You are on page 1of 2473

EDITED BY

Mhairi G. MacDonald MBChB, DCH, FRCP(E), FAAP, FRCPCH


Professor of Pediatrics
George Washington University
School of Medicine and Health Sciences
Washington, District of Columbia

Mary M. K. Seshia MBChB, DCH, FRCP(E), FRCPCH


Professor, Pediatrics, Obstetrics, Gynecology & Reproductive Sciences
University of Manitoba
Neonatologist, Division of Neonatal-Perinatal Medicine
Health Sciences Centre
Department of Pediatrics and Child Health
Winnipeg, Manitoba, Canada

Contributors
Nora I. Alfattoh MD
Consultant Pediatric Infectious Disease
Department of Pediatrics
King Abdulaziz Medical City/King Abdullah Children's Hospital
Riyadh, Kingdom of Saudi Arabia

Ruben E. Alvaro MD, FAAP


Associate Professor, Pediatrics, Obstetrics, Gynecology &
Reproductive Sciences
University of Manitoba
Medical Director, Division of Neonatal-Perinatal Medicine
St. Boniface General Hospital
Department of Pediatrics and Child Health
Winnipeg, Manitoba, Canada

Marianne Sollosy Anderson MD


Neonatologist
Sequoia Pediatrics Group
Kaweah Delta Medical Center Hospital
Visalia, California

Stephanie Andriole MS, CGC


Genetics Counselor
Comprehensive Genetics
Fetal Medicine Foundation of America
New York, New York

Jacob V. Aranda MD, PhD, FRCPC, FAAP


Professor
Department of Pediatrics and Ophthalmology
Director of Neonatology
Department of Pediatrics
State University of New York Downstate Medical Center
Brooklyn, New York

Kristian Aquilina MD, FRCS


Honorary Senior Lecturer
Institute of Child Health
University College London
Consultant Pediatrics Neurosurgeon
Department of Neurosurgery
Great Ormond Street Hospital for Children
London, United Kingdom

Robert J. Arceci MD, PhD


Professor, Department of Child Health
Director, Children's Center for Cancer and Blood Disorders
Department of Hematology/Oncology
Co-Director of the Ron Matricaria Institute of Molecular Medicine
Phoenix Children's Hospital
Phoenix, Arizona

Judy L. Aschner MD
Michael I. Cohen Professor and University Chair
Department of Pediatrics and Obstetrics, Gynecology and
Woman's Health
Albert Einstein College of Medicine
Chair and Physician-in-Chief
Department of Pediatrics
Children's Hospital at Montefiore
Bronx, New York

David J. Askenazi MD, MSPH


Associate Professor
Department of Pediatrics/Nephrology
University of Alabama at Birmingham
Attending Physician
Department of Pediatrics Nephrology
Children's of Alabama Hospital
Birmingham, Alabama

Maria Laura Avila MD


Clinical Research Fellow
Department of Haematology-Oncology
University of Toronto
The Hospital for Sick Children
Toronto, Ontario, Canada

Andrea T. Badillo MD
Assistant Professor, Department of Pediatric Surgery
George Washington University
Attending Surgeon, Department of Pediatric Surgery
Children's National Medical Centre
Washington, District of Columbia

Jillian M. Baker MD, MSc, FRCPC


Assistant Professor
Department of Pediatrics
University of Toronto
Staff Pediatrician and Hematologist
Department of Pediatrics
St. Michael's Hospital
Toronto, Ontario, Canada

Nancy M. Bauman MD
Professor, George Washington University School
of Medicine
Faculty
Otolaryngology, Head and Neck Surgery
Children's National Medical Center
Washington, District of Columbia

David A. Beckman PhD


Associate Professor
Department of Pediatrics
Thomas Jefferson University
Philadelphia, Pennsylvania
Nemours Research Programs
Alfred I. duPont Hospital for Children
Wilmington, Delaware

Edward F. Bell MD
Professor
Department of Pediatrics
University of Iowa
Attending Neonatologist
Department of Pediatrics
University of Iowa Children's Hospital
Iowa City, Iowa

Corinne Benchimol DO
Clinical Director, Pediatric Nephrology and Hypertension
Assistant Professor of Pediatrics
Department of Pediatrics
Icahn School of Medicine at Mount Sinai
Clinical Director, Pediatric Nephrology and Hypertension
Assistant Professor of Pediatrics
Department of Pediatrics
Kravis Children's Hospital at Mount Sinai
New York, New York

Judy C. Bernbaum MD
Professor of Pediatrics
Department of Pediatrics
Perelman Medical School
University of Pennsylvania
Medical Director
Neonatal Fellow Up Program
The Children's Hospital of Philadelphia
Philadelphia, Pennsylvania

Roee Birnbaum MD
Obstetrics and Gynecology Senior Resident
Department of Ultra-Sound in Obstetrics
and Gynecology
LIS Maternity hospital, Tel Aviv Sourasky
Medical Center
Tel Aviv, Israel

Carl L. Bose MD
Professor
Department of Pediatrics
University of North Carolina
Chapel Hill, North Carolina

Michael J. Boyajian MD
Assistant Professor
Department of Plastic and Reconstructive Surgery
George Washington University Medical Center
Attending Physician
Department of Plastic and Reconstructive Surgery
Children's National Hospital
Washington, District of Columbia

Robert J. Boyle MD
Professor Emeritus
Department of Pediatrics
University Virginia School of Medicine
Attending Neonatologist
Chair Ethics Committee
Neonatal Intensive Care
University of Virginia Health System
Charlottesville, Virginia

Robert L. Brent MD, PhD, Dsc


Distinguished Professor of Pediatrics, Radiology,
and Pathology
Department of Pediatrics
Jefferson Medical College
Philadelphia, Pennsylvania
Head, Clinical and Environmental Teratology
Laboratory
Nemours Research Programs
Alfred I. duPont Hospital for Children
Wilmington, Delaware

Luc P. Brion MD
Professor of Pediatrics
Director, Fellowship Training Program in Neonatal-Perinatal
Medicine
Department of Pediatrics
University of Texas Southwestern Medical Center
Attending Neonatologist
Department of Pediatrics
Parkland Health & Hospital System
Children's Health
William P. Clements Jr. University Hospital
Dallas, Texas

Laura D. Brown MD
Assistant Professor
Department of Pediatrics
Children's Hospital Colorado
University of Colorado School of Medicine
Aurora, Colorado

Barbara K. Burton MD
Professor of Pediatrics
Northwestern University Feinberg School
of Medicine
Clinical Practice Director
Division of Genetics, Birth Defects,
and Metabolism
Ann and Robert H. Lurie Children's Hospital
Chicago, Illinois

Anthony K. C. Chan MBBS, FRCPC


Professor of Pediatrics
McMaster University
Hamilton, Canada
Consultant
Coagulation Laboratory
Department of Pediatric Laboratory Medicine
The Hospital for Sick Children
Toronto, Ontario, Canada

Josef M. Cortez MD
Assistant Professor
Department of Pediatrics, Division of Neonatology
University of Florida Health Jacksonville
Associate Neonatologist
Department of Pediatrics, Division of Neonatology
Wolfson Children's Hospital
Jacksonville, Florida

John M. Costello MD, MPH


Associate Professor of Pediatrics
Department of Pediatrics
Northwestern University Feinberg School
of Medicine
Director, Inpatient Cardiology & Medical Director
Regenstein Cardiac Care Unit
Divisions of Cardiology and Critical Care Medicine
Ann & Robert H. Lurie Children's Hospital
of Chicago
Chicago, Illinois

Olaf Dammann MD, MS


Research Professor
Public Health and Community Medicine
Tufts University School of Medicine
Boston, Massachusetts

Jonathan M. Davis MD
Professor
Department of Pediatrics
Tufts University School of Medicine
Chief of Newborn Medicine
Department of Pediatrics and the Tufts Clinical
and Translational Research Institute
The Floating Hospital for Children at Tufts Medical Center
Boston, Massachusetts

Jaime R. Denning MD, MS


Assistant Professor
Department of Orthopedic Surgery
College of Medicine, University of Cincinnati
Staff Surgeon
Division of Orthopedic Surgery
Cincinnati Children's Hospital Medical Center
Cincinnati, Ohio

James G. Dinulos MD
Clinical Associate Professor
Department of Surgery (Dermatology)
Geisel School of Medicine at Dartmouth
Hanover, New Hampshire
Clinical Assistant Professor
Department of Dermatology
University of Connecticut
Farmington, Connecticut

Nicole R. Dobson MD
Assistant Professor
Department of Pediatrics
Uniformed Services University
Bethesda, Maryland
Chief, Neonatology
Department of Pediatrics
Tripler Army Medical Center
Honolulu, Hawaii

Yigal Dror MD, FRCPC


Associate Professor
Department of Pediatrics
Faculty of Medicine
Institute of Medical Sciences
Clinical Scientist
Department of Pediatrics
Division of Hematology and Oncology
The Hospital for Sick Children
Toronto, Ontario, Canada

Joanne E. Embree MD, FRCPC


Professor
Department of Pediatrics and Child Health,
Medical Microbiology
University of Manitoba
Consultant, Pediatric Infectious Diseases
Department of Pediatrics and Child Health
Children's Hospital, Health Sciences Centre
Winnipeg, Manitoba, Canada

Mark I. Evans MD
Professor, Obstetrics & Gynecology
Director, Comprehensive Genetics
President, Fetal Medicine Foundation
of America
Department of Obstetrics and Gynecology
Mount Sinai School of Medicine
New York, New York

Lynda B. Fawcett PhD


Assistant Professor of Pediatrics
Department of Pediatrics
Jefferson Medical College
Philadelphia, Pennsylvania
Assistant Professor
Nemours Biomedical Research
Alfred I. duPont Hospital for Children
Wilmington, Delaware

Ashanti L. Franklin MD
Pediatric Surgery Research Fellow
Children's National Health Systems
Surgery Resident
Howard University Hospital
Washington District of Columbia

Penny M. Feldman MD
Assistant Professor of Pediatrics
Department of Pediatrics
University of Massachusetts Medical School
Pediatric Endocrinologist
UMass Memorial Children's Medical Center
UMass Memorial Health Care
Worcester, Massachusetts

Michael F. Flanagan MD
Department of Pediatrics
Section of Pediatrics Cardiology
Dartmouth Medical School
Dartmouth-Hitchcock Medical Center
Lebanon, New Hampshire

Joseph T. Flynn MD, MS


Professor
Department of Pediatrics
University of Washington
Chief
Division of Nephrology
Seattle Children's Hospital
Seattle, Washington

M. Taylor Fordham MD
Pediatric Otolaryngology
George Washington University
Pediatric Otolaryngology
Children's National Medical Center
Washington, District of Columbia

Regan E. Giesinger MD, FRCPC


Assistant Professor
Department of Pediatrics
University of Toronto
Neonatologist
Department of Neonatal - Perinatal Medicine
The Hospital for Sick Children
Toronto, Ontario, Canada

Michael K. Georgieff MD
Professor, Vice Chair
Department of Pediatrics
University of Minnesota
Neonatologist
Department of Pediatrics
University of Minnesota
Amplatz Children's Hospital
Minneapolis, Minnesota

Harold M. Ginzburg MD, JD, MPH


Adjunct Professor of Psychiatry
Department of Psychiatry
Uniformed Services University of the Health Sciences School
of Medicine
Bethesda, Maryland
Associate Director for Research and Development
Department of Behavioral Medicine
Jack C. Montgomery Veterans Affairs
Medical Center
Muskogee, Oklahoma

Penny Glass PhD


Associate Professor
Department of Pediatrics
School of Medicine and Health Sciences
George Washington University
Director
Child Development Program
Psychology/Psychiatry & Behavior Science
Children's National Medical Center
Washington, District of Columbia

Sergio G. Golombek MD, MPH, FAAP


Professor of Pediatrics and Clinical Public Health
Department of Pediatrics
New York Medical College
Attending Neonatologist
The Regional Neonatal Center - Division of Newborn Medicine
Maria Fareri Children's Hospital at Westchester
Medical Center
Valhalla, New York

Stanley N. Graven MD
Professor, Community and Family Health
Department of Pediatrics
University of South Florida
Staff Physician
Department of Pediatrics
Tampa General Hospital
Tampa, Florida

Fahd Al Gurashi MBBS, FRCPC, MBA


Assistant Professor
Department of Anesthesia
University of Manitoba
Winnipeg, Manitoba, Canada

Torre Halscott MD
Fellow
Maternal-Fetal Medicine
Georgetown University School of Medicine
Fellow
Maternal-Fetal Medicine
Washington Hospital Center
Georgetown University Hospital
Washington, District of Columbia

Jane M. Hawdon MA, MBBS, MRCP, FRCPCH, PhD


Consultant Neonatologist
Clinical Academic Group Director, Women's
and Children's Health
Women's and Children's Health CAG
Barts Health NHS Trust
London, United Kingdom

William W. Hay Jr. MD


Professor
Department of Pediatrics
University of Colorado School of Medicine
Professor of Pediatrics (Neonatology)
Department of Neonatology
University of Colorado Hospital and Children's Hospital Colorado
Aurora, Colorado

Jeffrey C. Hellinger MD
Medical Director
New York Cardiovascular Institute
Department of Radiology
Lenox Hill Radiology and Medical Imaging
New York, New York

Carl E. Hunt MD
Research Professor
Department of Pediatrics
Uniformed Services University of the Health Sciences
Neonatologist
Department of Pediatrics
Walter Reed National Military Medical Center
Bethesda, Maryland

Sherwin J. Isenberg MD
Professor of Ophthalmology
Department of Ophthalmology
Jules Stein Eye Institute
University of California
Los Angeles, California

Amish Jain MBBS, MRCPCH


Assistant Professor
Department of Pediatrics
University of Toronto
Staff Neonatologist
Department of Pediatrics
Mount Sinai Hospital
Toronto, Ontario, Canada

Jennifer H. Johnston MD
Assistant Professor
Department of Diagnostic and Interventional Imaging
The University of Texas Medical School at Houston
Staff Radiologist
Department of Diagnostic and Interventional Imaging
Memorial Hermann Hospital - Texas Medical Center
Houston, Texas

George W. Kaplan MD, MS


Professor of Clinical Surgery and Pediatrics
Division of Urology
Department of Surgery
University of California, San Diego
Attending Pediatric Urologist
Department of Urology
Rady Children's Hospital San Diego
San Diego, California

Winston W. K. Koo MBBS


Professor
Department of Pediatrics
Chief of Neonatology
Department of Neonatology
Louisiana State University Health Sciences Center (LSUHSC)
Shreveport
Shreveport, Louisiana

Gideon Koren MD FRCPC, FACMT, FACCT


Director and Senior Scientist The Motherisk Program
The Hospital for Sick Children
Professor of Pediatrics,Pharmacology, Pharmacy and Medical
Genetics
The University of Toronto
Professor of Physiology/Pharmacology
The University of Western Ontario
Toronto, Ontario, Canada
Lajos Kovacs MDCM, FRCPC, FAAP
Associate Professor
Department of Pediatrics
McGill University
Neonatologist
Department of Neonatology
Jewish General Hospital
Montreal, Quebec, Canada

Helain J. Landy MD
Professor and Chief
Department of Obstetrics and Gynecology
Georgetown University School of Medicine
Chair
Department of Obstetrics and Gynecology
MedStar Georgetown University Hospital
Washington, District of Columbia

Mary Min-chin Lee MD


Professor of Pediatrics and Chair
Department of Pediatrics
University of Massachusetts Medical School
Physician-in-Chief
UMass Memorial Children's Medical Center
UMass Memorial Health Care
Worcester, Massachusetts

Shoo K. Lee MBBS, FRCPC, PhD


Professor
Department of Paediatrics, Obstetrics & Gynaecology
and Public Health
University of Toronto
Pediatrician-in-Chief
Department of Pediatrics
Mount Sinai Hospital
Toronto, Ontario, Canada

Judith Littleford MD, BSc, FRCPC


Associate Professor
Department of Anesthesia and Perioperative Medicine
University of Manitoba
Winnipeg, Manitoba, Canada

Liz M. Loewen RN, BFA, MN, CPHIMS-CA


Director
Coordination of Care
Manitoba eHealth
Winnipeg Regional Health Authority
Winnipeg, Manitoba, Canada

Ralph A. Lugo PharmD


Professor and Chair
Department of Pharmacy Practice
East Tennessee State University
Bill Gatton College of Pharmacy
Johnson City, Tennessee

Carolyn Lund RN, MS, FAAN


Neonatal Clinical Nurse Specialist
ECMO Coordinator
Neonatal Intensive Care Unit
Children's Hospital & Research
Oakland, California

Mhairi G. MacDonald MBChB, DCH, FRCP(E), FAAP, FRCPCH


Professor of Pediatrics
George Washington University
School of Medicine and Health Sciences
Washington, District of Columbia

M. Jeffrey Maisels MD, BCh, DSc


Professor
Department of Pediatrics
Oakland University William Beaumont School of Medicine
Rochester, Michigan
Chair Emeritus and Director, Academic Affairs
Department of Pediatrics
Beaumont Children's Hospital
Royal Oak, Michigan

Kathleen A. Marinelli MD, IBCLC, FABM


Associate Professor of Pediatrics
Department of Pediatrics
University of Connecticut School of Medicine
Farmington, Connecticut
Neonatology and Breastfeeding Medicine
Department of Neonatology
Connecticut Children's Medical Center
Hartford, Connecticut

Gilbert I. Martin MD
Clinical Professor
Department of Pediatrics
Loma Linda Medical Center
Loma Linda, California
Director Emeritus
Neonatal Intensive Care Unit
Citrus Valley Medical Center - Queen of the
Valley Campus
West Covina, California

Scott D. McLean MD
Associate Professor of Human and Molecular
Genetics
Associate Professor of Pediatrics
Baylor College of Medicine
Houston, Texas
Clinical Geneticist
Department of Genetics
Children's Hospital of San Antonio
San Antonio, Texas

Irene M. McAleer MD, JD, MBA


Health Science Clinical Associate Professor,
Department of Urology
University of California, Irvine
Attending, Pediatric Urologist,
Department of Pediatric Urology
Children's Hospital of Orange County
Irvine, California

Patrick McNamara MD
Professor of Pediatrics and Physiology
Department of Pediatrics
University of Toronto
Staff Neonatologist and Associate Scientist
Department of Pediatrics
Division of Neonatology
The Hospital for Sick Children
Toronto, Ontario, Canada

Nicole Mendelson
Research assistant
New York Cardiovascular Institute
Lenox Hill Radiology and Medical Imaging
New York, New York

Sarah C. Muttitt MD, FRCPC, MBA


Chief Information Officer and Vice President of Information
Management and Technology
The Hospital for Sick Children
Toronto, Ontario, Canada

Suhas M. Nafday MD, MRCP, FAAP


Associate Professor of Clinical Pediatrics
Albert Einstein College of Medicine
Director of Newborn Services
Division of Neonatology
Department of Pediatrics
Children's Hospital at Montefiore
Pediatric Hospital for Albert Einstein College of Medicine
Bronx, New York

Michael R. Narvey MD
Assistant Professor
University of Manitoba
Division Head, Neonatal-Perinatal Medicine
Health Sciences Centre and St. Boniface General Hospital
Department of Pediatrics and Child Health
Winnipeg, Manitoba, Canada

Nguyenvu Nguyen MD
Assistant Professor of Pediatrics
Department of Pediatrics
Northwestern University Feinberg School of Medicine
Attending Physician
Department of Pediatrics
Division of Cardiology
Ann & Robert Lurie Children's Hospital of Chicago
Chicago, Illinois

William Oh MD
Professor
Department of Pediatrics
Warren Alpert Medical School of Brown University
Attending Neonatologist
Department of Pediatrics
Women & Infants Hospital of Rhode Island
Providence, Rhode Island

Damjan Osredkar MD, PhD


Post-doctoral Research Fellow Institute of Basic Medical Sciences
University of Oslo
Oslo, Norway
Pediatric Neurologist
Department of Pediatric Neurology
University Children's Hospital Ljubljana
Ljubljana, Slovenia

Enrique M. Ostrea Jr. MD


Professor
Department of Pediatrics
Wayne State University
Attending Neonatologist
Department of Pediatrics
Hutzel Women's Hospital
Detroit, Michigan

T. Michael D. O'Shea Jr. MD, MPH


Professor of Pediatrics and Vice Chair for Research
Department of Pediatrics, Epidemiology and Prevention, and
Social Science and Health Policy
Wake Forest School of Medicine
Neonatology Section Chief, Department of Pediatrics
Brenner Children's Hospital
Winston-Salem, North Carolina

Eugenia K. Pallotto MD, MSCE


Associate Professor of Pediatrics
Department of Pediatrics
Division of Neonatology
University of Missouri-Kansas City School of Medicine
Medical Director, Intensive Care Nursery
Department of Pediatrics
Division of Neonatology
Children's Mercy Hospital and Clinics
Kansas City, Missouri

Apostolos N. Papageorgiou MD, FAAP


Professor
Department of Pediatrics
McGill University
Chief
Department of Pediatrics and Neonatology
Jewish General Hospital
Montreal, Quebec, Canada

Stephen W. Patrick MD, MPH, MS


Assistant Professor
Department of Pediatrics and Health Policy
Attending Physician
Department of Neonatology
Monroe Carell Jr. Children's Hospital at Vanderbilt
Vanderbilt University
Nashville, Tennessee

Rosemary J. Pauley-Hunter
Nurse Practitioner
Pediatric GI
Boystown National Research Hospital
Boystown, Nebraska

Ermelinda Pelausa MD
Assistant Professor of Pediatrics
McGill University
Montreal, Quebec, Canada
Neonatologist
Department of Neonatology
Jewish General Hospital
Montreal, Quebec, Canada

Mikael Petrosyan MD
Assistant Professor of Surgery
General and Thoracic Surgery
Children's National Medical Center
Washington, District of Columbia

Stacy L. Pineles MD
Assistant Professor of Ophthalmology
Department of Ophthalmology
Jules Stein Eye Institute
University of California
Los Angeles, California

J. Edgar Winston C. Posecion MD


Associate Professor
Department of Pediatrics
West Visayas State University College of Medicine
Chief, Newborn Intensive Care Unit
Department of Pediatrics
West Visayas State University Medical Center
Iloilo City, Philippines

Richard J. Powers MD
Regional Director of Quality Improvement
Department of Pacific Region
Pediatric Medical Group
Orange, California
Medical Director of Newborn Services
Department of Neonatology
Good Samaritan Hospital
San Jose, California

Gabriella Pridjian MD
Professor and Chairman
Department of Obstetrics and Gynecology
Tulane University School of Medicine
New Orleans, Louisiana
Professor and Chairman
Department of Obstetrics and Gynecology
Tulane Center for Women's Health
Metairie, Louisiana

Gloria S. Pryhuber MD
Professor
Department of Pediatrics
Department of Environmental Medicine
University of Rochester Medical Center
Rochester, New York

Virender K. Rehan MD
Professor of Pediatrics
Chief, Division of Neonatology
Director, Neonatal Intensive Care Unit
Director, Neonatal-Perinatal Fellowship Training Program
Co-Director
Perinatal Research Center Harbor UCLA Medical Center
David Geffen School of Medicine at UCLA
Torrance, California

Sara E. Ramel MD
Assistant Professor
Department of Pediatrics
University of Minnesota
Neonatologist/Attending Physician
Department of Pediatrics
University of Minnesota Amplatz Children's Hospital
Minneapolis, Minnesota

Mary E. Revenis MD
Assistant Professor
Department of Pediatrics
School of Medicine and Health Sciences
George Washington University
Attending Neonatologist
Department of Neonatology
Children's National Medical Center
Washington, District of Columbia

Ward R. Rice MD, PhD


Professor
Department of Pediatrics
University of Cincinnati College of Medicine
Director, Neonatology Fellowship Training
Department of Pediatrics
Cincinnati Children's Hospital Medical Center
Cincinnati, Ohio

Warren N. Rosenfeld MD
Professor
Department of Pediatrics
Stony Brook School of Medicine
State University of New York
Stony Brook, New York
Chairman
Department of Pediatrics
Children's Medical Center at Winthrop University Hospital
Mineola, New York

Paul J. Rozance MD
Associate Professor
Department of Pediatrics
University of Colorado School of Medicine
Aurora, Colorado

Samantha L. Russell MD, ChB, FCA


Fellow
Department of Obstetric Anesthesia
University of Manitoba
Winnipeg, Manitoba, Canada

Jeffrey M. Saland MD, MS


Chief, Division of Pediatric Nephrology and Hypertension
Director of Pediatric Kidney Transplantation
Associate Professor of Pediatrics
Department of Pediatrics
Icahn School of Medicine at Mount Sinai
Chief, Division of Pediatric Nephrology and Hypertension
Director of Pediatric Kidney Transplantation
Associate Professor of Pediatrics
Department of Pediatrics
Kravis Children's Hospital at Mount Sinai
New York, New York

Anthony D. Sandler MBCHB, FAAD, FACS


Professor, Surgery and Pediatrics
George Washington University
Surgeon-In-Chief
Joseph E. Robert Center for Surgical Care
Children's National Health System
Washington, District of Columbia

Georg M. Schmlzer MD, PhD


Postdoctoral Fellow
Department of Pediatrics
University of Alberta
Neonatologist
Department of Neonatology
Royal Alexandria Hospital, Alberta Health Services
Edmonton, Alberta, Canada

Jeffrey L. Segar MD
Professor
Department of Pediatrics
University of Iowa
Director
Division of Neonatology
Department of Pediatrics
University of Iowa Children's Hospital
Iowa City, Iowa

Mary M. K. Seshia MBChB, DCH, FRCP(E), FRCPCH


Professor, Pediatrics, Obstetrics, Gynecology & Reproductive
Sciences
University of Manitoba
Neonatologist, Division of Neonatal-Perinatal Medicine
Health Sciences Centre
Department of Pediatrics and Child Health
Winnipeg, Manitoba, Canada

Nalini Singh MD, MPH


Professor Pediatrics Global Health & Epidemiology
Department of Pediatrics
George Washington University
School of Medicine and Health Sciences
Division Chief Infectious Diseases
Children's National Health System
Washington, District of Columbia

Judith A. Smith MHA


President
Smith Hager Bajo, Inc.
Ashburn, Virginia

Ann R. Stark MD
Professor
Department of Pediatrics
Vanderbilt University School of Medicine
Attending Neonatologist
Division of Neonatology
Monroe Carell Jr. Children's Hospital at Vanderbilt
Nashville, Tennessee

Anne R. Synnes MDCM, MHSc, FRCPC


Clinical Professor
Department of Pediatrics
University of British Columbia
Neonatologist and Director
Neonatal Follow-Up Program
Division of Neonatology
British Columbia Women's Hospital and Health Center
Vancouver, British Columbia, Canada

Mark W. Thompson MD
Assistant Professor
Department of Pediatrics
Uniformed Services University
Bethesda, Maryland
Chief Consultant to Army Surgeon General
US Army Medical Command, Clinical Policy Services Division
Houston, Texas

Marianne Thoresen MD, PhD


Professor of Physiology and Pediatrics, Institute of Basic Medical
Sciences University of Oslo
Oslo, Norway
Consultant Neonatologist
Neonatal Intensive Care Unit, St. Michael's Hospital
Bristol, United Kingdom
Stephanie R. Thorn PhD
Assistant Professor
Department of Pediatrics
Division of Neonatology
University of Colorado
Aurora, Colorado

Reginald C. Tsang MBBS


Professor Emeritus of Pediatrics
Department of Pediatrics
Cincinnati Children's Hospital and Medical Center
Cincinnati, Ohio

William E. Truog MD
Professor and Associate Chair
Department of Pediatrics
School of Medicine
University of Missouri Kansas City
Sosland Endowed Chair in Neonatal Research
Children's Mercy Hospital
Kansas City, Missouri

Elizabeth C. Turney MD
Fellow
Department of Pediatrics
Division of Neonatal-Perinatal Medicine
University of North Carolina
Chapel Hill, North Carolina

Ma. Esterlita Villanueva Uy MD


Assistant Research Professor
Institute of Child Health and Human Development
National Institutes of Health, University of the
Philippines Manila
Associate Clinical Professor and Attending Neonatologist
Department of Pediatrics
Philippine General Hospital
Manila, Philippines

Bina Valsangkar MD
Adjunct Assistant Professor of Pediatrics
Department of Pediatrics
George Washington University
Washington, District of Columbia

Jon A. Vanderhoof MD
Lecturer
Department of Pediatrics
Harvard University
Attending
Pediatric Gastroenterology
Boston Children's
Boston, Massachusetts

Sally H. Vitali MD
Assistant Professor of Anaesthesia
Harvard Medical School
Associate
Division of Critical Care Medicine
Department of Anesthesia, Perioperative and Pain Medicine
Boston Children's Hospital
Boston, Massachusetts

Robert M. Ward MD, FAAP, FCP


Professor, Pediatrics; Adjunct Professor,
Pharmacology/Toxicology
Department of Pediatrics & Pharmacology/Toxicology
University of Utah
Attending Neonatologist
Department of Neonatology
Primary Children's Hospital
Salt Lake City, Utah

Jon F. Watchko MD
Professor of Pediatrics, Obstetrics, Gynecology,
and Reproductive Services
Department of Pediatrics
University of Pittsburgh School of Medicine
Senior Scientist
Department of Pediatrics
Magee-Womens Hospital of UPMC
Pittsburgh, Pennsylvania

Steven N. Weindling MD
Department of Pediatrics
Section of Pediatric Cardiology
Dartmouth Medical School
Dartmouth-Hitchcock Medical Center
Lebanon, New Hampshire

Howard J. Weinstein MD
R. Alan Ezekowitz Professor of Pediatrics
Department of Pediatrics
Harvard Medical School
Chief, Pediatric Hematology-Oncology
Massachusetts General Hospital for Children
Boston, Massachusetts

Dany E. Weisz MD
Assistant Professor
Department Paediatrics
University of Toronto
Neonatal Intensivist
Department of Newborn and Developmental Paediatrics
Sunnybrook Health Sciences Centre
Toronto, Ontario, Canada

Susan E. Wert PhD


Associate Professor of Pediatrics
University of Cincinnati College of Medicine
Associate Professor of Pediatrics
Division of Neonatology, Perinatal, and Pulmonary Biology
Perinatal Institute, Cincinnati Children's Hospital Medical Center
Cincinnati, Ohio

Robert D. White MD
Clinical Assistant Professor
Department of Pediatrics
Indiana University School of Medicine
Notre Dame, Indiana
Director
Regional Newborn Program
Memorial Hospital
South Bend, Indiana

Andrew Whitelaw MD, FRCPCH


Professor of Neonatal Medicine
School of Clinical Sciences
University of Bristol
Consultant Neonatologist
Neonatal Neuroscience, Level D, St. Michael's Hospital
Bristol, United Kingdom

Jeffrey A. Whitsett MD
Executive Director of the Perinatal Institute
Professor of Pediatrics
Department of Pediatric
Cincinnati Children's Hospital Medical Center
Cincinnati, Ohio

Craig B. Woda MD, PhD


Pediatric Hospitalist
Department of Pediatrics
Harvard Medical School
Boston Children's Hospital
Boston, Massachusetts

Karen S. Wood MD
Professor
Department of Pediatrics
University of North Carolina
Medical Director
Carolina Air Care
University of North Carolina Hospitals
Chapel Hill, North Carolina

Yuval Yaron MD
Assistant Professor
Department of Obstetrics and Gynecology
Sackler Faculty of Medicine
Tel Aviv University
Director
Prenatal Genetic Diagnosis Unit
Genetic Institute
Tel Aviv Sourasky Medical Center
Tel Aviv, Israel

Scott B. Yeager MD
Associate Professor
Department of Pediatrics
University of Vermont
Chief
Division of Pediatric Cardiology
Medical Center Hospital of Vermont
Burlington, Vermont

Dimitry Zilberman DO
MFM Attending
Department of Obstetrics and Gynecology
Division of Maternal Fetal Medicine
Medstar Georgetown University Hospital
Washington, District of Columbia
Dedication
We dedicate this book to those neonatal health care providers who place a premium on teaching; whether in
the classroom, at the bedside, or by publication. These individuals donate time, despite multiple other
professional duties, to an area of medicine that is frequently under-rewarded in the Halls of Academia.
But where is the man [woman] who counsel can bestow, Still proud to teach, and yet not proud to know.
Alexander Pope 1688-1744
The editors would also like to give special thanks to Ashley Fischer, Product Development Manager, for her
constant support throughout this, at times, daunting project.
Foreword
When I wrote the Preface for the First Edition of Neonatology in January, 1975, the specialty had not yet been
baptized. The first Sub-Board Examinations in Neonatal-Perinatal Medicine were given later that year. Nurseries
for sick newborns varied enormously in layout and equipment. Likewise, there was no uniformity in the training
and skills of those giving care to distressed fetuses, babies in the birth transition, and sick and distressed
newborn infants. Digging information out of basic science journals about these little beings, who were in a period
of rapid change and had life-threatening problems, was tedious. We were not all playing with the same deck of
cards.
Thus, a basic purpose of the book was to gather together relevant information about anatomy, physiology,
pathology, pharmacology, bacteriology, and genetics and couple it with a bestavailable approach to caring for
particular conditions. A special feature of distressed newborns was that they almost always had multiple
problems, which interacted like jack straws. Thus, treatment of disease conditions and support of vital bodily
functions were blended together.
In the 40 years since the First Edition, change has been continuous and dramatic. Genetics and biochemistry
have blossomed. Previously unheard of surgical interventions have become almost routine. Instrumentation to
support respiration, and even extracorporeal heart-lung machines, has proved effective and lifesaving.
Microchemistry has allowed the monitoring of blood chemistries using only tiny quantities of blood. Indwelling
catheters have served for both monitoring and intravenous alimentation. More than half the papers at Pediatric
research meetings, over these years, have concerned the fetus and newborn.
And so, practice has changed and become more complicated and expensive. Issues have arisen over the
resources to be devoted to neonatal intensive care. In addition, moral and procedural debates have sought to
define how much care was beneficial, and when this care became intrusive and futile.
Some of these changes in practice have been supported by good science: others have been the best judgment
of individuals, based on their own clinical experience. The truth is, good, clean clinical trials in the NICU are very
difficult and expensive. Over the 2- to 3-year period of a trial, many details of care are changing, apart from the
trial itself. Confounding variables are difficult to control in the face of life-threatening conditions and urgent
interventions. Getting statistically significant numbers often requires a multiinstitutional project, which
complicates project design, data collection, randomization, interpretation of results, and publication. These
projects are very costly, and the strong temptation is to load them with data collection on secondary questions.
The result is a lot of data dredging, rather than clean answers to a single research question which has a yes or
no answer. To paraphrase Thomas Jefferson's comment about democracy: multiinstitutional controlled trials are
the worst way to evaluate new treatments, except for all the others!
That being said, the NICHD Neonatal Research Network, the Cochrane Network, and the Vermont-Oxford
research trials have allowed many more of the clinical recommendations of the 7th Edition to be objective and
based on controlled trials, than was true in the past.
The 7th Edition is now adapted to the age of digital information. It can change fluidly as new studies and
discoveries emerge. It is a library where things can be quickly looked up, without dragging around what would
surely now be a two-volume tome. Most of the illustrations are now in color. It is no longer strictly a text BOOK. It
is a large, focused, digital database. Yet each chapter has been carefully organized, thought through, and written
by a recognized expert in his/her field. (No Wikipedia this.)
The explosion of knowledge is now so great, that research, bedside care, and indeed the entire neonatology
enterprise, is a collaborative venture. That is good, because we can help one another. But for myself, I hope that
the care of the individual child and the individual family will always remain intensely personal and caring.
Gordon B. Avery MD, PhD
Gordon B. Avery MD, PhD
Preface
It is now half a century since the subspecialty of neonatology was born in the United States. In the initial 20
years, pioneer efforts to rescue progressively less mature infants were laudable and remarkably successful but
at the same time fraught with serious side effects, long-term morbidity, and numerous ethical issues.
In a perfect world, the initial attempts to treat infants previously not recognized as potentially viable would take
the form of carefully designed, controlled clinical trials. However, in the early years of neonatology publications
introducing innovative therapeutic interventions were frequently little more than case reports, based upon small
numbers and lacking adequate follow-up. Once a treatment had been reported as successful, it became more
difficult, although not ethically interdicted, to deny treatment to a control group for whom the outcome might
approximate 100% mortality. An extreme example of this phenomenon can be traced back to abstracts presented
at Society for Pediatric Research Conferences in the mid-1980s. These presentations reported research that
included in the study cohorts small numbers of extremely immature (approximately 24 weeks of gestation)
premature infants who had been successfully resuscitated and admitted to the NICU. Adequate long-term
follow-up data were not provided; nevertheless, shortly thereafter in the United States, it became the standard
practice for many neonatologists to resuscitate infants of approximately 24 weeks of gestational age without
having obtained informed consent from the parents. In the intervening decades, the very poor prognosis for
these infants on the fringe of viability has shown marginal improvement at best despite an abundance of
technological and other advances in neonatal medicine.
Thirty years later, as noted by Dr. Gordon Avery in his foreword, we have entered the era of the multi-institutional
national and international research networks, which offer the major advantages of well-designed controlled
studies as well as large study cohorts. Thus, unless, as Dr. Avery cautions, the needle is lost in a datadredged
haystack, our clinical practices can now be based upon more robust research data. At the same time, it is critical
that neonatal health providers never lose sight of the importance of having a thorough knowledge of the
pathophysiology of disease processes and consider all data in terms of bioplausibility.
In recent years, the global aspects of neonatal care have been increasingly highlighted and addressed. The
reader is referred to the excellent review of global status presented in the new Chapter 1. It is sobering for those
of us who provide neonatal care in resource-rich countries to realize that one in five newborns who die in
developing countries could have been saved simply by being washed in clean water and cared for in a clean
environment by people who had washed their hands (WaterAid Charity report via the Thomson Reuters
Foundation, March seventeenth, 2015). Recent data from the United Nations show that 38% of health facilities in
54 developing countries do not have access to supplies of clean water. The World Health Organization reports
that nearly one-fifth of health facilities in these countries lack toilets and more than one-third do not have
anywhere for staff or patients to wash their hands with soap.
The 7th Edition is now in full color. There are 52 new authors and 4 chapters covering new topics. The print
edition is bundled with an interactive eBook edition offering tablet, smartphone, or online access. In addition to
the complete content of the print edition, there are additional online-only tables and figures, with state-of-the-art
navigation and search tools. Navigation cross-links pull information from the book content, the reader's notes,
and the Web site. The navigation system also offers highlighting tools, the ability to take and share notes with
colleagues, and quick reference tabbing to save selected content for future use. Throughout this edition, authors
have included links to multi-institutional research trials, which they have been asked to regularly update in the
eBook.
We wholeheartedly echo the sentiment expressed by Dr. Avery in the final sentence of his foreword. Modern
neonatal intensive care requires a team effort. The multidisciplinary members of the team must value the unique
professional skills of their colleagues and place a premium on maintaining optimal intra-team communication.
Above all, each member of the team must have the goal of providing excellent, individualized care to every
patient and their family unit.
Mhairi G. MacDonald MBChB, DCH, FRCP(E), FAAP, FRCPCH
Mary M. K. Seshia MBChB, DCH, FRCP(E), FRCPCH
Preface to the First Edition
Neonatology means knowledge of the human newborn. The term was coined by Alexander Schaffer, whose book
on the subject, Diseases of the Newborn, was first published in 1960. This book, together with Clement Smith's
Physiology of the Newborn Infant, formed cornerstones of the developing field. In the past 15 years, neonatology
has grown from the preoccupation of a handful of pioneers to a major subspecialty of pediatrics. Knowledge in
this area has so expanded that it now seems important to collect this material into a multiauthor reference work.
Although the perinatal mortality rate has declined over the past 50 years, the best presently attainable survival
rates have not been achieved throughout the world, and indeed the United States lags behind 15 other
countries, despite its vast resources. New knowledge and improvement in the coordination of services for mother
and child are needed to drive down perinatal mortality further. And finally, far greater emphasis must be placed
on morbidity, so that surviving infants can lead full and productive lives. One hopes that in the future, the
yardstick of success will be the quality of life and not the mere fact of life itself.
In this past decade, neonatology, as a recognized subspeciality of pediatrics, has come into being around the
intensive care premature nursery. Needless to say, the problems of prematurity are far from solved. But
neonatology is ripe for a broadening out from its prematurity-hyaline membrane disease beginnings. The
newborn is heir to so many problems, and his or her physiology is so unique and rapidly changing that all
conditions of the newborn should come within the concern of the new and expanding discipline of neonatology. It
has long since become standard practice to admit to premature nurseries other high-risk infants such as those of
diabetic or toxemic mothers. Here, the criterion is the need for intensive care. However, the neonatologist's
specialized knowledge should give him a significant role in the care of other infants in the first 2 to 3 months of
life, whether they require intensive care and whether they are readmitted for problems unrelated to prematurity
and birth itself. Detailed knowledge of newborn physiology can assist in the management of congenital
anomalies, surgical conditions of the neonate, failure to thrive, nutritional problems, genetic, neurologic, and
biochemical diseases, and a host of conditions involving delayed maturation. Thus, one can conceive of a
subspecialty sharply limited in age to early infancy but broad in its study of the interaction of normal physiology
and disease processes.
Neonatology must also grow in its relationship to obstetrics and fetal biology. In the best centers, an active
partnership has developed between obstetrics and pediatrics around the management of high-risk pregnancies
and newborns. Sometimes training has been cooperative, but in only a few instances have basic scientists
concerned with fetal biology been brought into this effort. Important beginnings have been made in studying the
fetomaternal unit, such as the endocrine studies of Egon Diczfalusy, the cardiopulmonary studies of Geoffrey
Dawes, and the immunologic studies of Arther Silverstein. But fundamental processes such as the controls of
fetal growth and the onset of labor are not understood at this time. Centers or institutes bringing together
workers of diverse points of view are needed to wrestle with the profound problems of fetal biology. At the clinical
level, the interdependence of obstetrics and neonatology is obvious. As an ultimate development, these two
specialties may one day be joined as a new entityperinatologyat least at the level of training and
certification. In the meantime, far greater mutual understanding and daily interaction are needed for the optimal
care of mothers and their infants.
This book is organized around problems as they occur, as well as by organ systems. It hopes to achieve a
balance between presentation of the basic science on which rational management must rest, and the advice
concerning patient care which experts in each subarea are qualified to give. Individual chapter authors have
approached their subjects in various ways, and no attempt has been made to achieve a completely uniform
format. In some instances, there is overlap of subject material, but the somewhat different viewpoints presented,
and the desire to spare the reader from hopscotching through the book after cross references, have persuaded
me to leave small overlaps undisturbed.
It is appreciated that no volume such as this can have more than a finite useful lifetime. Yet while its currency
lasts, I hope it will serve as a practical guide to therapy and an aid in the understanding of pathophysiology for
those active in the care of newborns.
Gordon B. Avery MD, PhD
1
The Scope and Organization of Neonatology: North American and Global
Comparisons
Judy L. Aschner
Stephen W. Patrick
Ann R. Stark
Shoo K. Lee

INTRODUCTION
In the past 50 years, we have witnessed extraordinary advances in perinatal medicine accompanied by dramatic improvements in
birth outcomes. The obstetrical subspecialty of maternal-fetal medicine has improved both maternal and neonatal outcomes for
women with high-risk pregnancies due to fetal disorders, maternal chronic diseases, or pregnancy-associated conditions, such as
toxemia and gestational diabetes. The emergence of the subspecialty of neonatology and the availability of dedicated neonatal
intensive care units with advanced technology designed for the newborn have improved the survival and outcomes of infants born
prematurely or with serious medical or surgical problems. In high-income, high-resource countries, an entire workforce has
evolved with expertise in the unique developmental physiology and congenital and acquired diseases of the newborn. This
convergence of expertise and resources describes most modern-day neonatal intensive care units in the United States (US),
Canada, and high-income countries in Europe and across the globe. Multidisciplinary care has become the norm, and families are
increasingly being integrated into the health care team.
The infrastructure, resources, and workforce realities are quite different in developing countries, particularly in Africa and
Southeast Asia but also in developing nations of North and South America. The disparities in maternal and neonatal mortality
remain striking despite recent investments and early signs of improvements in select regions. This chapter will explore the scope
and organization of perinatal and neonatal health care with a focus on (1) disparities that persist in many high-resource nations
based on race, ethnicity, income, and access to care and (2) the sobering perinatal outcomes statistics in low-resource,
developing nations that remain our collective challenge and are directly or indirectly the result of inequalities in resource
allocation, infrastructure, health literacy, and access.

INFANT, NEONATAL, AND PERINATAL MORTALITY


Valid comparisons of infant mortality over time or between different countries or geographic regions require acceptance and
application of standard definitions, complete and reliable data collection, and confidence in both the numerator and denominator.
The latter two represent challenges when attempting to compare outcomes in countries with vastly different resources,
infrastructure for data collection, and cultural expectations and values.

Standard Definitions
The infant mortality rate (IMR), as defined by the World Health Organization (WHO) and the National Center for Health
Statistics (NCHS) of the Centers for Disease Control and Prevention (CDC), is the number of deaths occurring within the first
year of life per 1,000 live births. Infant mortality can be further divided into neonatal mortality (death before 29 days of age)
and postneonatal mortality (death between 29 days and 1 year of age) (Table 1.1). Neonatal mortality is most often
attributable to poor maternal health or complications during pregnancy or at the time of delivery. Postneonatal mortality has a
broader range of etiologies, including infection, hypoxic ischemic encephalopathy, poor nutrition, trauma, and sleep-related
deaths. Perinatal mortality is variably defined but typically refers to a fetal death at or beyond 20 weeks of gestation or a
newborn death in the first 7 days of life. For purposes of international comparisons, perinatal mortality is a fetal or infant
death that occurs at 28 weeks of gestation to 7 days after birth (Table 1.1).
Preterm birth is defined as a live birth before 37 completed weeks of pregnancy and is divided into subcategories, based on
gestational age as shown in Table 1.1. Although prematurity and low birth weight (LBW) are interrelated, they are not
synonymous. Only about two-thirds of LBW infants are preterm. Term infants that are below the 10th percentile of the index
population's distribution of birth weights by gestation are considered small for gestational age. In addition to prematurity,
causes of LBW include fetal growth restriction and genetic syndromes. LBW infants at any gestational age are at increased
risk of mortality and morbidity. There are several subcategories of LBW (Table 1.1). Very-low-birth-weight (VLBW) infants
are more than 100 times as likely, and LBW babies are more than 25 times as likely to die in the first year of life compared
with infants who weigh 2,500 g or more at birth (1).

Infant Mortality in the United States and Other High-Resource Countries


The United States first began to measure and record the IMR in 1915. As shown in Figure 1.1, during the past 100 years, the US
IMR has fallen dramatically from about 100 deaths per 1,000 live births in 1915 to approximately 6 deaths per 1,000 live births in
2011 (2). Steady improvement in infant survival was interrupted by a plateau in 1955-1959 and again in 2000-2005 when the IMR
appeared stalled at approximately 6.9. However, by 2011, the IMR had dropped to 6.05, a 12% decrease from 2005 through 2011
(2). The progress observed over the past century has been attributed to improved nutrition and sanitation, economic growth,
advances in medical care and improved access to care (3,4).
Despite these encouraging statistics, 24,000 US infants died before their first birthday in 2011 (5). Moreover, the IMR in the
United States is higher than in many other developed countries. In 2010, the United States ranked 26th among the 29 countries in
the Organization for Economic Cooperation and Development
P.2
(OECD) ( Fig. 1.2), with an IMR of 6.1 (6). When births at gestational ages under 24 weeks were excluded to better ensure
international comparability, the IMR in the United States was 4.2 infant deaths per 1,000 live births, still about twice the IMR for
Finland, Sweden, and Denmark, the three countries with the lowest rates after excluding deaths under 24 weeks of gestation. For
infants born at 24 to 31 weeks of gestation, the IMR in the United States is comparable to the rates in most European nations;
however, the United States had among the highest IMR for preterm infants born between 32 and 36 weeks of gestation and the
highest rate of infant death at 37 weeks and above (2.2 per 1,000 live births in 2010) among the OECD countries (6).

TABLE 1.1 Standard Definitions of Key Perinatal Outcomes

Perinatal Outcome Definition

Infant mortality rate Number of deaths occurring within the first year of life per 1,000 live births

Neonatal mortality Number of deaths before 29 d of age per 1,000 live births
rate

Postneonatal Number of deaths between 29 d and 1 y of age per 1,000 live births
mortality rate

Perinatal mortality Number of fetal or infant deaths from 20 weeks of gestation (or 28 wk for international
rate comparisons) to 7 d after birth per 1,000 live births

Preterm birth A live birth before 37 completed weeks of pregnancy

Extremely Birth before 28 weeks of gestation


preterm

Very preterm Birth between 28 and 31 completed weeks of gestation

Moderate-to-late Birth between 32 and 36 completed weeks of gestation


preterm

Low birth weight Birth weight <2,500 g

Very low birth Birth weight <1,500 g


weight

Extremely low Birth weight <1,000 g


birth weight

FIGURE 1.1 Infant, neonatal, and postneonatal mortality rates in the United States: 1915-2011. U.S. Department of Health
and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau. Child health USA 2013.
Rockville, Maryland: U.S. Department of Health and Human Services, 2013.

Between 2005 and 2011, the decline in IMR was 16% among black mothers, a larger drop than that observed for other racial or
ethnic groups. Nonetheless, large disparities by race and ethnicity persist. The IMR for non-Hispanic black infants (12.40) is more
than twice that of non-Hispanic white infants (5.33), based on 2009 data (Fig. 1.3). Infants born to American Indian/Alaska Native
and Puerto Rican mothers also have higher rates of infant mortality (8.47 and 7.18, respectively) than do non-Hispanic white
infants (Fig. 1.3) (2).
Congenital malformations were the leading cause of infant mortality in the United States in 2011, followed by prematurity, LBW,
and sudden infant death syndrome (SIDS) (7). While birth defects have been identified as the leading cause of infant death for
more than two decades, prematurity/LBW is the leading cause of infant mortality among black infants (8). Prematurity is
associated with multiple causes of death, and when these are grouped together, preterm birth overtakes birth defects as the
leading cause of infant death in the United States, accounting for over a third of all infant deaths (2,9).
Between 2005 and 2011, there was a drop in mortality associated with congenital malformations, prematurity/LBW, SIDS, and
maternal complications, while the mortality from unintentional injuries rose slightly (6). A 20% decline in deaths attributed to SIDS
is worth noting given the education campaign to place infants on their backs to sleep and other recommendations related to the
sleep environment. However, some of this remarkable achievement could be related to changes in the way SIDS is diagnosed
and reported.
In 2011, neonatal mortality (death in the first 28 days) was 4.04 per 1,000 live births and accounted for about two-thirds of infant
deaths in the United States (2). Neonatal mortality is most often associated with congenital malformations, infection, perinatal
asphyxia, preterm birth, LBW, and other perinatal conditions related to prematurity. Similar to overall infant mortality, infants born
to non-Hispanic black mothers, followed by those born to Puerto Rican and American Indian/Alaska Native mothers, had the
highest rates of neonatal mortality (8.13, 4.76, and 4.38, respectively) (2).
P.3
FIGURE 1.2 Infant mortality rate ranking in 2010 of the United States and other countries in the Organization for
Economic Cooperation and Development. MacDorman MF, Mathews TJ, Mohangoo AD, et al. International comparisons of
infant mortality and related factors: United States and Europe, 2010; National vital statistics reports vol. 63, no. 5. Hyattsville,
MD: National Center for Health Statistics, 2014.

FIGURE 1.3 Infant, neonatal, and postneonatal mortality rates by race and ethnicity in the United States, 2009. U.S.
Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau.
Child health USA 2013. Rockville, MD: U.S. Department of Health and Human Services, 2013.

P.4
Postneonatal mortality is most often attributed to SIDS, other sleep-related deaths, congenital malformations, and unintentional
injuries. Once again, there are notable racial and ethnic disparities. Postneonatal mortality was more than twice as high for infants
of both non-Hispanic black and American Indian/Alaska Native mothers (4.27 and 4.09, respectively) than for non-Hispanic whites
(1.93) (2).
Perinatal mortality is another important measure of pregnancy health and takes into account fetal deaths as well as early (first
week of life) neonatal deaths. There are an estimated 1 million fetal deaths reported annually in the United States (8). Most fetal
deaths are caused by chronic asphyxia, congenital malformations, and pregnancy complications, such as placental abruption,
diabetes mellitus, and intrauterine infections (10). Although fetal deaths, including late fetal deaths at or beyond 28 weeks of
gestation, did not change significantly between 2006 and 2012, the perinatal mortality rate (fetal or infant deaths at 28 weeks of
gestation until 7 days after birth per 1,000 live births) declined 4% from 6.51 per 1,000 in 2006 to 6.26 in 2011 (11). There was an
8% decline in perinatal mortality for non-Hispanic black women to 10.8 in 2011, with no significant change for non-Hispanic white
and Hispanic women (11). The improvement in perinatal mortality was driven by an 8% drop in early neonatal deaths (deaths
under 7 days per 1,000 live births) from 3.55 in 2006 to 3.28 in 2011, seen primarily among non-Hispanic black infants in the
United States (11). Improved and more consistent reporting of fetal deaths is needed to better understand the risks factors
associated with perinatal death and develop strategies for prevention.

THE GLOBAL PERSPECTIVE ON INFANT MORTALITY


The dramatic decline in infant mortality during the past century was seen not only in developed countries but also in developing
countries and reflects improvements in socioeconomic, sanitation, and educational conditions and in population health, most
notably a reduction in malnutrition and infectious diseases. Indeed, the global IMR has declined from 152/1,000 live births in 1950
to 43/1,000 live births in 2010 (Fig. 1.4) (12). Although the disparity between developed and developing countries has narrowed,
the gap remains wide, with the IMR ranging from 5/1,000 live births in developed countries to 78/1,000 live births in Africa in 2010
(13).

FIGURE 1.4 Trends for global infant mortality rate by region, 1950-2050. United Nations World Population Prospects. The
2012 revisionUnited Nations development. Retrieved on December 26, 2014 from http://esa.un.org/unpd/wpp/index.htm

The causes of infant mortality vary by region (Fig. 1.5), but major causes include preterm birth (35%), neonatal infections (28%),
birth complications (23%), congenital anomalies (9%), and others (6%) (14). Preterm birth is the leading cause of infant deaths
worldwide, accounting for almost 1 million deaths in 2013. The rate of preterm births ranges from 5% to 18%, with the highest
incidence occurring in developing countries, but the incidence is increasing in almost all countries.

THE BURDEN OF PRETERM BIRTH: US AND GLOBAL PERSPECTIVES


The preterm birth rate is an important driver of a nation's IMR and a leading cause of childhood disabilities. In 2012, the March of
Dimes, the Partnership for Maternal, Newborn and Child Health, Save the Children, and the WHO published Born Too Soon:
The Global Action Report on Preterm Birth (15). This report provided estimates of preterm birth by country. Approximately 15
million babies, or about 1 in 10 infants, are born preterm annually. Over 1 million of these infants died in 2013, making preterm
birth complications the leading cause of death among children under 5 years of age. Across 184 countries, the rate of preterm
birth ranged from 5% to 18% of all live births (16).
Resource-poor regions of the world have excessive burdens of preterm birth, LBW, small size for gestational age, stillbirth, infant
mortality, and maternal mortality. More than 60% of preterm births occur in Africa and Southeast Asia (16). The countries with the
highest number and the highest rates of preterm birth are shown in Table 1.2.
After excluding infants born before 24 weeks of gestation, the United States had the highest preterm birth rate among the 19
countries shown in Figure 1.6 (6). The percent of preterm births in the United States was about 40% higher than in countries in
the United Kingdom and nearly 75% higher than in some Scandinavian countries (6). Recent US data indicate some progress,
with the rate of preterm birth falling annually from a peak of 12.8 in 2006 to 11.39 in 2013. Declines in preterm rates since 2006
were reported in 49 states and the District of Columbia (17). The reduction in the percent of US babies born prematurely is a
likely contributor to the improving infant mortality statistics because about two-thirds of all infant deaths occur among those born
preterm.
P.5

FIGURE 1.5 Estimated distribution of causes for 4 million neonatal deaths for the six WHO regions in 2000. Lawn JE,
Wilczynska-Ketende K, Cousens SN. Estimating the causes of 4 million neonatal deaths in the year 2000. Intern J Epidemiol
2006;35:706, by permission of the International Epidemiological Association. doi: 10.1093/ije/dyl043.

There are striking global inequalities in survival rates for infants born preterm. In low-income settings, mortality for infants born at
32 weeks is about 50%; almost all of these babies survive in high-income countries. These stark disparities are due to a lack of
basic and essential care, such as warmth, breastfeeding support, antibiotics, and supplemental oxygen. In low-income countries,
more than 90% of extremely preterm babies (<28 weeks) die in the first few days of life; less than 10% of these babies die in high-
income countries (15).
Most preterm births happen spontaneously, but in high-income countries, some preterm births are due to early induction of labor
or caesarean birth, for medical and nonmedical reasons. Late preterm births comprise the vast majority of all preterm births.
Compared to term infants, these infants have a higher incidence of morbidity, including respiratory distress syndrome,
temperature instability, and jaundice and have three times the IMR (1,18,19). Late preterm infants experience longer hospital
stays and are more likely to incur higher hospital costs associated with NICU admissions than do term infants (20). Even infants
born at 37 and 38 weeks of gestation have worse outcomes compared to infants born at 39 and 40 weeks of gestation. Despite a
low absolute risk of infant death, singleton infants born at 37 weeks had increased neonatal mortality rates, compared to infants
born at 40 weeks (0.66 and 0.34 per 1,000 live births, respectively) (21). Those born electively at 37 and 38 weeks had increased
rates of respiratory problems and were more likely to be admitted to a NICU, compared to those born at 39 weeks (22,23).

TABLE 1.2 Countries with the Greatest Number and Highest Rate of Preterm Births

Countries with the Greatest Number of Countries with the Highest Rate of Preterm Birth Per 100
Preterm Birth Live Births

India 3,519,100 Malawi 18.1

China 1,172,300 Comoros 16.7

Nigeria 773,600 Congo 16.7


Pakistan 748,100 Zimbabwe 16.6

Indonesia 675,700 Equatorial Guinea 16.5

United States 517,400 Mozambique 16.4

Bangladesh 424,100 Gabon 16.3

Philippines 348,900 Pakistan 15.8

Congo 341,400 Indonesia 15.5

Brazil 279,300 Mauritania 15.4

Blencowe H, Lee ACC, Cousens S, et al. Preterm birth-associated neurodevelopmental impairment estimates at regional
and global levels for 2010. Pediatr Res 2013;74:17.

A public education campaign launched by the March of Dimes, called Healthy Babies are Worth the Wait, and a similar
campaign called Healthy Start sponsored by the Department of Health and Human Services discourage scheduled deliveries
before 39 weeks. These measures aimed at the public have been accompanied by strong statements by professional
organizations including the American College of Gynecologists and the American Academy of Pediatrics to avoid nonmedically
indicated deliveries before 39 weeks. These recommendations have been reinforced by regional and statewide perinatal quality
collaboratives (PQCs) and initiatives championed by individual hospitals and hospital systems.

MAJOR NEONATAL MORBIDITIES


Preterm birth is associated with both short-term (infection, necrotizing enterocolitis, bronchopulmonary dysplasia, intraventricular
hemorrhage, and retinopathy of prematurity) and long-term complications. Infants born very preterm (<32 weeks of completed
gestation) are at greatest risk of death and long-term disability. Many preterm babies face a lifetime of disability, developmental
delay, learning disabilities, and neurosensory deficits, with the largest impact occurring in developing countries. Worldwide, it is
estimated that over 911,000 preterm survivors (7%) each year suffer
P.6
long-term neurodevelopmental disabilities, including 345,000 who are moderately or severely affected. For infants born under 28
weeks of gestation, 52% suffer some degree of neurodevelopmental impairment, compared to 24% for infants born at 28 to 31
weeks of gestation and 5% for infants born at 32 to 36 weeks of gestation. Table 1.3 (15) lists some of the major long-term
adverse outcomes suffered by preterm infants. Figure 1.7 shows the distribution of worldwide deaths and disabilities from
preterm births (16).
FIGURE 1.6 Percentage of preterm births: comparison of the United States with selected European countries, 2010.
MacDorman MF, Mathews TJ, Mohangoo AD, et al. International comparisons of infant mortality and related factors: United
States and Europe, 2010; National vital statistics reports vol. 63, no. 5. Hyattsville, MD: National Center for Health Statistics,
2014.

TABLE 1.3 Long-term Impact of Preterm Birth

Long-term Outcomes Examples Frequency in Survivors

Specific physical effects Visual impairment Blindness or high Around 25% of all
myopia after extremely preterm affected
retinopathy of Also risk in moderately
prematurity preterm babies especially if
Increased poorly monitored oxygen
hypermetropia and therapy
myopia

Hearing impairment Up to 5%-10% of extremely


preterm

Chronic lung disease From reduced Up to 40% of extremely


of prematurity exercise tolerance to preterm
requirement for home
oxygen
Increased hospital
admissions in
childhood for LRTI

Long-term Increased blood Full extent of burden still to


cardiovascular ill- pressure be quantified
health and Reduced lung
noncommunicable function
disease Increased rates of
asthma
Growth failure in
infancy, accelerated
weight gain in
adolescence

Neurodevelopmental/behavioral Mild Specific learning


effects (83) Disorders of impairments,
executive functioning dyslexia, reduced
academic
achievement

Moderate to severe Moderate/severe Affected by gestational age


Global developmental cognitive impairment and quality of care
delay Motor impairment dependent
Cerebral palsy

Psychiatric/behavioral Attention deficit


sequelae hyperactivity disorder
Increased anxiety
and depression

Family, economic, and societal Impact on family Psychosocial, Common varying with
effects Impact on health emotional, and medical risk factors,
service economic disability, socioeconomic
Intergenerational Cost of care (7) status
acute and ongoing
Risk of preterm birth
in offspring

March of Dimes, PMNCH, Save the Children, WHO. Born too soon: the global action report on preterm birth. In: Howson
CP, Kinney MV, Lawn JE, eds. Geneva, Switzerland: World Health Organization, 2012.

P.7
FIGURE 1.7 Worldwide deaths and disabilities for babies born preterm in 2010. Blencowe H, Lee ACC, Cousens S, et al.
Preterm birth-associated neurodevelopmental impairment estimates at regional and global levels for 2010. Pediatr Res
2013;74:17.

MATERNAL RISK FACTORS FOR POOR PREGNANCY OUTCOMES


Common causes of preterm birth include multiple pregnancies, infections, and chronic conditions, such as diabetes and high
blood pressure. There is also a genetic influence. However, often no cause is identified. The best predictors of having a preterm
birth are multifetal pregnancy or history of previous preterm labor/delivery. The major risk factors for poor pregnancy outcomes in
high-income countries are shown in Table 1.4.
In developing countries, maternal risk factors for poor pregnancy outcomes are often different from those in developed countries.
Teenage pregnancy, poverty, and access to health care are among the most important determinants of poor pregnancy outcomes
(24). In many countries, poverty is associated with poor nutrition, unhealthy lifestyles, obesity, and poor oral health, all of which
are associated with poor pregnancy outcomes (25). WHO estimates that 22% of the world population in 2008 live in absolute
poverty, with the highest rates occurring in South Asia (36%) and sub-Saharan Africa (47%) (26). Absolute poverty is defined as a
condition characterized by severe deprivation of basic human needs, including food, safe drinking water, sanitation facilities,
health, shelter, education, and information. It depends not only on income but also on access to services (27). Maternal
underweight (odds ratio 1.32) and overweight (odds ratio 1.07) are both risk factors for preterm birth and pregnancy complications
including hypertension, gestational diabetes, postpartum hemorrhage, stillbirth, and congenital anomalies (28). In rapidly
developing economies like the BRICS (Brazil, Russia, India, China, South Africa) countries, maternal obesity and diabetes are
emerging as significant health problems (29). Maternal diabetes before pregnancy increases the risk for pregnancy loss, stillbirth,
preterm labor, congenital anomalies, hypertension, and delivery by caesarean section (30). Folic acid deficiency is linked to
neural tube defects in newborn infants, and anemia increases the risk for maternal mortality, LBW, preterm birth, and child
mortality (31). Maternal depression also increases the risk for preterm birth, stillbirth, and peripartum and postpartum depression
(32). Use of addictive substances is another important risk. Tobacco smoking increases the risk for preterm birth, LBW,
respiratory problems, and risk for cognitive impairment, while alcohol use can cause fetal alcohol syndrome and intellectual
impairment (33,34). In some countries such as those in sub-Saharan Africa, infections such as malaria pose additional risks (35).

TABLE 1.4 Risk Factors for Preterm Birth and Poor Pregnancy Outcomes

Multifetal pregnancy Low prepregnant weight


History of prior preterm delivery Folic acid deficiency
Maternal age <17 or >35 y Obesity

Black race Infection

Low socioeconomic status Bleeding

Unmarried Anemia

Previous fetal or neonatal death Toxic stress

Three or more spontaneous fetal losses Lack of social supports

Uterine abnormalities Tobacco use

Incompetent cervix Illicit drug use

Genetic predisposition Alcohol abuse

P.8
Lack of prenatal care may arise from lack of health care facilities, lack of transportation to health care facilities, or lack of financial
resources to access care, and conditions are often worse in rural areas. In developing countries, 50% of births still take place
without a medically skilled attendant, and women in sub-Saharan Africa often depend on traditional birth attendants who lack
proper training. Improved maternal health is part of the United Nations Millennium Development Goals, which target reducing
maternal mortality by three-quarters by 2015, through a strategy of contraception, family planning, and use of skilled birth
attendants (36). Although these goals may not be reached in the target timelines, significant improvements have been made and
give hope for further improvements in the years ahead.

THE NEONATAL WORKFORCE


There are large differences in how countries employ health care resources to look after neonates (Table 1.5) (37). Thompson et
al. (37) report that the number of pediatricians varies from 20/10,000 live births in the United Kingdom to 144/10,000 live births in
the United States. The reverse is true for family practitioners, with 597 family practitioners/10,000 live births in the United
Kingdom, compared to 169 family practitioners/10,000 live births in the United States. These variations reflect differences in how
health care professionals are utilized and reimbursed in different countries. In the United States, primary care for infants is often
provided by pediatricians whereas in the United Kingdom, this service is mostly provided by family practitioners. Similarly,
neonatologists provide both level II and level III neonatal care in the United States while they provide mostly level III care in the
United Kingdom. Thompson et al. (37) also reported that greater neonatal intensive care resources were not consistently
associated with lower birth weight-specific mortality. The United States has high neonatal intensive care capacity, with 6.1
neonatologists per 10,000 live births; Australia, 3.7; Canada, 3.3; and the United Kingdom, 2.7. For intensive care beds, the
United States has 3.3 per 10,000 live births; Australia and Canada, 2.6; and the United Kingdom, 0.67. These variations reflect
differences in regionalization of care, geography and proximity to health care facilities, and organization of health care services.
Despite greater neonatal intensive care resources in the United States, the relative risk (United States as reference) of neonatal
mortality for infants less than 1,000 g was 0.84 for Australia, 1.12 for Canada, and 0.99 for the United Kingdom; for 1,000 to 2,499
g infants, the relative risk was 0.97 for Australia, 1.26 for Canada, and 0.95 for the United Kingdom.

TABLE 1.5 Physician Workforce and Hospital Capacity

United Australia Canada United


States Kingdom

Annual deliveries (in thousands) 3,942 256 349 636


Physicians* (per 10,000 live births):

Pediatricians 144.7 34.2 59.7 20.0

Neonatologists 6.1 3.7 3.3 2.7

Neonatologists per 1,000 LBW infants 8.0 5.7 5.5 3.7

Obstetricians/gynecologists 100.2 42.2 45.3 24.3

Maternal/fetal specialists 3.2 0.4 NA NA

Family practitioners 169.7 813.9 816.7 597.7

Number level III units* (per 10,000 live births) 1.21 0.90 0.72 2.92

Neonatal hospital beds* (per 1,000 live births) Intensive 3.3 NA NA 0.67
care beds

Intensive beds per 1,000 LBW 43.4 NA NA 9.3

Intensive + intermediate care beds 5.1 2.6 2.6 NA

Intensive + intermediate care beds per 1,000 LBW 67.1 39.4 44.8 NA
infants

*2 tests for differences across all 4 countries for each measure; all P values < .001.

From Thompson LA, Goodman DC, Little GA. Is more neonatal intensive care always better? Insights from a cross-
national comparison of reproductive care. Pediatrics 2002;109(6):1036, with permission.

The capacity of US hospitals to care for preterm or ill newborns is influenced by the availability of qualified physician
subspecialists. The American Board of Pediatrics sub-board of Neonatal-Perinatal Medicine conducted its first certifying
examination in 1975. Through December 2013, 5,552 neonatologists have been certified as Diplomates, 60% of whom are 50 to
70 years of age, with an average age of 56.2 years. The distribution of Diplomates varies widely among states, ranging from 0 in
Wyoming to 494 in California (38). The distribution is also not uniform when the number of certified neonatologists is compared to
the child population ( Fig. 1.8; American Board of Pediatrics).
The capacity to care for high-risk newborns was expanded with the introduction of neonatal nurse practitioners (NNPs) into the
workforce and formalized with certification beginning in the early 1980s. NNPs are registered nurses who have completed a
master's degree and advanced clinical training, and they perform many complex activities. A policy statement of the American
Academy of Pediatrics Committee of Fetus and Newborn recommended that care provided by NNPs be given in collaboration with
or under the supervision of a physician, usually a neonatologist (39). A survey of 394 NNPs in the United States found that the
distribution of primary practice site was a community hospital for 54%, an academic medical center for 37%, a private practice for
5%, and a managed care organization for 4% (40). These practitioners were most likely to practice with physicians with a
collaborative agreement (41%), supervised by a physician (33%) or in a multidisciplinary practice including physicians (24%);
independent NNP practice was rare (2%). Similar to neonatologists, distribution of NNPs varies regionally and is often not
matched to demand. Furthermore, due to factors including declining enrollment in programs, inadequate numbers of preceptors,
and plans of currently working NNPs to decrease their work hours, a gap exists between supply and demand (40,41,42). In some
regions of the United States, this gap is being filled by physician assistants and pediatricians working as NICU hospitalists, under
the supervision of a neonatologist.
P.9

FIGURE 1.8 Relative distribution of American Board of Pediatrics Neonatal-Perinatal Medicine Diplomates by state,
2013. American Board of Pediatrics. Workforce data 2013-2014. https://www.abp.org/sites/abp/files/pdf/workforcebook.pdf

AMERICAN ACADEMY OF PEDIATRICS RECOMMENDED LEVELS OF NEONATAL CARE


The American Academy of Pediatrics Committee on Fetus and Newborn published definitions for Levels of Neonatal Care in 2004
that were subsequently updated in 2012 (43). These updated definitions provide common terms that can be used to compare
outcomes, resource use, and health care costs. Standardized nomenclature is important for public health purposes, for health
care professionals who provide neonatal care, and for families making decisions about a delivery hospital. In addition, these
national designations can encourage uniform classification by hospitals, state governments and health departments, and other
organizations to promote improved perinatal care. The delineation of care levels and recommended capabilities and types of
medical providers for each of the four levels of care are shown in Table 1.6 (43). Importantly, all sites that deliver infants should
be capable of performing neonatal resuscitation at every delivery, with at least one provider available to be responsible solely for
the infant, consistent with the AAP Neonatal Resuscitation Program (44).

PERINATAL REGIONALIZATION
Perinatal regionalization is an organized system of care in a geographic area in which infants are born at or transferred to
hospitals that are able to provide the most appropriate care for each infant's needs. Hospitals within the region are designated by
their capability of providing basic or more highly specialized care. The highest level facilities have the most specialized providers
and advanced technology and equipment appropriate to care for the smallest, most critically ill, or most complex infants in order to
ensure the best outcomes. The intent of providing risk-appropriate care to the population within a region is to achieve the best
outcomes in the most cost-effective manner.
Through its leadership of three important publications, the March of Dimes has pioneered the effort for a rational approach to
perinatal services that would produce the best results. In 1976, the March of Dimes Committee on Perinatal Health published
Toward Improving the Outcome of Pregnancy (TIOP I), a coordinated effort of The American College of Obstetricians and
Gynecologists, The American Academy of Pediatrics, the American Medical Association, and the American Academy of Family
Physicians. TIOP I recommended a regionalized system of care with three levels of neonatal care (I, II, III) defined within the
system. High risk patients would be referred to centers with the appropriate resources and personnel to care for them. At the time,
most level III NICUs were at academic centers. TIOP II, published in 1993, adhered to the same principles of regionalization, but
changed the definitions of NICUs to Basic, Specialty, and Subspecialty units with expanded criteria. TIOP III, the most recent
revision, again promotes the principle of a coordinated continuum of perinatal services in a geographic region to increase survival
of high-risk infants (1). Concentrating relatively rare cases at a few locations allows clinical teams to develop expertise and
centralize expensive technologies. TIOP III promotes the use of model programs and quality and safety initiatives, including
measurement, transparency, and accountability to improve outcomes.
Numerous studies over more than 30 years have documented the increased risk of poor outcome for VLBW infants associated
with birth outside of a hospital with the highest level NICU (level III). A systematic review and meta-analysis of 41 US and
international studies that included more than 113,000 VLBW infants found a 62% increased odds of neonatal or predischarge
mortality for infants born at hospitals with non-level III compared to those with level III NICUs (45). Studies that identified extremely
LBW infants or those born at less than 32 weeks of gestation showed similar results. This differential effect on mortality was
sustained regardless of the decade of publication.
Mortality in VLBW infants with specific conditions of prematurity is also affected by the level and volume of the birth hospital NICU.
For example, in a study in California from 2005 to 2011, mortality was greater for VLBW infants with necrotizing enterocolitis
P.10
born at centers with low-volume level IIIA and IIIB NICUs than it was for those born at high-volume level IIIB and IIIC NICUs
(California definitions) (46). Morbidity is also greater in preterm infants born in lower-level settings and transferred to a tertiary
care center. In a study from the Canadian Neonatal Network, among infants born at less than 33 weeks of gestation during 1996-
1997, a greater proportion of infants born outside a tertiary center had severe intraventricular hemorrhage, chronic lung disease,
late-onset infection, and other morbidities compared to inborn infants, even after adjusting for perinatal risk factors and illness
severity at admission (47). Further study showed that this differential effect was related to gestational age and limited to infants
born at less than 30 weeks of gestation (48).

TABLE 1.6 Definitions, Capabilities, and Provider Types: Neonatal Levels of Care

Level of Capabilities Provider Typesa


Care

Level I Provide neonatal resuscitation at every delivery Pediatricians, family


physicians, nurse
practitioners, and other
advanced practice registered
nurses

Well Evaluate and provide postnatal care to stable term newborn infants
newborn Stabilize and provide care for infants born 35-37 wk of gestation who
nursery remain physiologically stable
Stabilize newborn infants who are ill and those born at <35 wk of
gestation until transfer to a higher level of care

Level II Level I capabilities plus: Level I health care providers


plus:

Special Provide care for infants born 32 wk of gestation and weighing 1,500 Pediatric hospitalists,
care g who have physiologic immaturity or who are moderately ill with neonatologist, and neonatal
nursery problems that are expected to resolve rapidly and are not anticipated nurse practitioners
to need subspecialty services on an urgent basis
Provide care for infants convalescing after intensive care
Provide mechanical ventilation for brief duration (<24 h) or continuous
positive airway pressure or both
Stabilize infants born before 32 wk of gestation and weighing <1,500 g
until transfer to a neonatal intensive care facility

Level III Level II capabilities plus: Level II health care providers


plus:
NICU Provide sustained life support Pediatric medical
subspecialists,b pediatric
anesthesiologists,b pediatric
surgeons, and pediatric
opthalmologistsb

Provide comprehensive care for infants born <32 wk of gestation and


weighing <1,500 g and infants born at all gestational ages and birth
weights with critical illness
Provide prompt and readily available access to a full range of pediatric
medical subspecialists, pediatric surgical specialists, pediatric
anesthesiologists, and pediatric ophthalmologists
Provide a full range of respiratory support that may include
conventional and/or high-frequency ventilation and inhaled nitric oxide
Perform advanced imaging, with interpretation on an urgent basis,
including computed tomography, MRI, and echocardiography

Level Level III capabilities plus: Level III health care providers
IV plus:

Regional Located within an institution with the capability to provide surgical Pediatric surgical
NICU repair of complex congenital or acquired conditions subspecialists
Maintain a full range of pediatric medical subspecialists, pediatric
surgical subspecialists, and pediatric anesthesiologists at the site
Facilitate transport and provide outreach education

aIncludes all providers with relevant experience, training, and demonstrated competence.

bAt the site or at a closely related institution by prearranged consultative agreement.

American Academy of Pediatrics Committee on Fetus and Newborn. Levels of neonatal care. Pediatrics 2012;130:387.

However, many factors may affect outcomes of preterm infants that may or may not be related to site of delivery. These include
characteristics of obstetric care (e.g., prenatal steroid use), the experience of nursing staff, nurse-to-patient ratios, or other issues
of practice, including approach to resuscitation at the border of viability. Other factors that may play a role in outcomes include
regional differences in race or health insurance status. Studies have shown conflicting results on the effect of volume of patients
on outcomes. For example, in a retrospective study of 48,237 VLBW infants delivered in California, compared to delivery at a
center with a high-volume (>100 VLBW infants per year) high-level NICU, the odds ratio for death increased with both lower
volume and lower NICU level (49). In a study of 165 neonatal units in England contributing data to a National Neonatal Research
Database, the odds ratio for neonatal mortality decreased in infants less than 33 weeks of gestation admitted to a high-volume
NICU (50). However, the odds ratio for death associated with admission to a tertiary-level NICU was reduced only in infants less
than 27 weeks of gestation. In contrast, although it seems logical that the experience derived from management of large numbers
of patients with similar conditions would be associated with improved outcomes, a study in the Vermont Oxford Network (VON)
showed that volume of admissions explained only 9% of the variation in mortality rate of VLBW infants among participating
hospitals (51). Furthermore, although infants with congenital anomalies are typically excluded from investigations of effects of
regionalization, studies suggest that delivery and care at a perinatal center with a level III NICU improves neonatal and obstetrical
outcomes of infants with prenatally diagnosed anomalies (52,53,54,55).
P.11
FIGURE 1.9 Trends in neonatal special care beds and US births, 1987-2008. Berns SD, ed. Toward improving the outcome
of pregnancy III: enhancing perinatal health through quality, safety and performance initiatives. Reissued edition. White Plains,
NY: March of Dimes Foundation, 2011.

In spite of evidence supporting perinatal regionalization and risk-appropriate care for newborns, changes in reimbursement
structure and policy in the past three to four decades led to changes in delivery site for VLBW infants. During the years 1987-
2008, the number of special care beds increased by 89.2% while the number of births in the United States increased by only
14.6% (Fig. 1.9) (1). Similarly, a retrospective study of data from US metropolitan areas during the period 1980-1995 showed that
while births increased by 18%, hospitals with NICU beds increased by 99% and the number of neonatologists increased by 268%,
with a low occupancy rate for NICUs (56). More recently, in California, the proportion of VLBW infants born at and initially
managed at hospitals with high volume and providing the highest level of care fell from 42.5% to 26.5% during the period 2005 to
2011 (46). Data from the Maternal Child Health Bureau show that the percent of VLBW infants delivered at facilities for high-risk
deliveries and neonates (Performance Measure 17) is still far from ideal ( Fig. 1.10).

FIGURE 1.10 National Performance Measure 17: percent of VLBW infants delivered at facilities for high-risk deliveries
and neonates. Maternal and Child Health Bureau Title V Block Grant:
https://mchdata.hrsa.gov/TVISReports/Charts/PMGMap.aspx?ReportType=NPM&MeasureType=Performance&PMNum=17
Multiple factors affect the ability to impact risk appropriate care for VLBW infants. Across the United States, large variation exists
in definitions and regulation of neonatal services and many units
P.12
self-designate their level (57). Clinical, structural, and social factors also impact site of delivery (58). For example, some mothers
of VLBW infants deliver prematurely and precipitously, making maternal transport infeasible. Similarly, mothers with a fetus that
has a lethal anomaly might benefit from supportive services in their own community. Providers at some nontertiary hospitals may
believe that they can adequately manage VLBW infants and that their own capability differs from published data. This is
compounded by competition among hospitals that encourage establishment of NICUs that do not meet all criteria for a tertiary
unit. This competition may hamper maternal transport when neonatal transport is available and efficient. Finally, pregnant women
may prefer to deliver locally at a community hospital with their known and trusted provider and may not agree to transfer.
Interestingly, in the realm of pediatric subspecialty services, there has been progress in the regionalization of care for children
with complex medical programs. This has been fueled by the success of large regional children's hospitals and a shortage of
pediatric subspecialists. For pregnant woman and high-risk neonates, reversal of deregionalization is unlikely to occur without
stateimposed regulatory requirements.

DIFFERENCES IN THE ORGANIZATION AND SCOPE OF NEONATAL CARE IN THE


DEVELOPED AND DEVELOPING WORLD
Developing countries have fewer resources than do developed countries and employ them quite differently. Table 1.7 compares
neonatal resources categorized by neonatal mortality (14). The 75 countries with the highest neonatal mortality account for 60%
of births and 85% of neonatal deaths in the world but have a median gross national income of US$1,045 compared with
US$4,645 and US$27,215 in countries with intermediate and low neonatal mortality, roughly corresponding to low-, middle-, and
high-income countries, respectively. Similarly, government spending on health care per capita is US$23 compared with US$200
and US$1452, respectively. Consequently, there is higher reliance on traditional birth attendants in low-income countries (20%)
compared with middle (9%) and high (0%) income countries. Only 60% of births in low-income countries have skilled attendants at
birth. There are fewer doctors, nurses, and midwives in low-income countries (2 doctors/10,000 population, 7 nurses and
midwives/10,000 population) compared with middle- (13 doctors/10,000 population, 20 nurses and midwives/10,000 population)
and high-income countries (29 doctors/10,000 population, 57 nurses and midwives/10,000 population). Access to care is
therefore a major issue in low-income countries, where only 3% of births have access to neonatal intensive care, compared with
98% in middle-income and 100% in high-income countries. There is also often lack of transport systems for rapidly and safely
moving infants to facilities that can provide appropriate care.

TABLE 1.7 Comparisons of Birth Outcomes, Infrastructure, and Economics Among Countries with Low,
Intermediate, and High Neonatal Mortality

NMR Group NMR Group 2, NMR Group 3,


1, Low Intermediate High
Mortality, Mortality, NMR 5 to Mortality,
NMR <5 <15 NMR 15

Birth outcomes Births 13,261,000 41,378,000 80,123,000

Number of countries 46 63 75

Neonatal deaths 42,000 437,000 2,589,000

Stillbirths 47,000 436,000 2,159,000

Total fertility rate (median) 1.7 2.3 4.3

Contextual Gross national income per capita 27,215 4,645 1,045


factors (US$, median)
Female literacy rate (% median) 99 91 62

Maternal Skilled attendant at birth, % (median) 100 98 60


newborn and
child care
services

Traditional birth attendant, % 0 9 20


(median)

Caesarean delivery, % (median) 24 21 4


(median coverage)

Access to NICU care, % of births 100 98 (IQR: 44%-99%) 3 (IQR: 2%-


(median) 19%)

Nurse and midwifery personnel per 57 20 7


10,000 population (median)

Total number of nurses and 8,187,000 6,710,000 3,354,000


midwives

% of the global total of nurses and 45% 37% 18%


midwives

Physicians per 10,000 population 29 13 2


(median)

Total number of physicians 3,266,000 4,344,000 1,513,000

% of global total 36% 48% 16%

Health system Government spending on health per 1,452 200 23


funding capita (US$, median)

Out-of-pocket expenditure on health 19 31 41


as percentage of total expenditure on
health (median %)

Official development assistance


($US) (median [min/max range])

To maternal/neonatal per live birth 5 (0-428) 24 (5-96)

To child health per child 2 (0-152) 14 (1-51)

Adapted from Lawn JE, Wilczynska-Ketende K, Cousens SN. Estimating the causes of 4 million neonatal deaths in the
year 2000. Int J Epidemiol 2006;35:706. doi: 10.1093/ije/dyl043.

It is therefore not surprising that different countries have adopted different standards for limits of viability and resuscitation. While
many high-income countries routinely provide active resuscitation for infants at 24 to 25 weeks of gestation and sometimes even
as low as 21 weeks of gestation, this is not necessarily the case in low-income countries.
P.13
ESTABLISHING INFRASTRUCTURE TO REDUCE MATERNAL AND INFANT MORTALITY
IN THE DEVELOPING WORLD
The Every Newborn Action Plan (59), Lancet Series on Neonatal Survival (60) and Preterm Birth (61,62,63), and the Born Too
Soon: The Global Action Report on Preterm Birth (15) propose actions for policy, programs, and research by all partners, from
governments to NGOs to the business community. Broad strategies of the Millennium Development Goals adopted by the United
Nations include eradicating extreme poverty and hunger; achieving universal primary education especially for girls; promoting
gender equality and empowering women; reducing childhood mortality; improving maternal health; combating HIV/AIDS, malaria,
and other communicable diseases; ensuring environmental sustainability; and developing a global partnership for development.
An integrated service delivery package ( Fig. 1.11) was proposed to provide health care services that cover the spectrum from
prepregnancy to birth and childhood. It is estimated that 84% of preterm maternal and newborn deaths can be averted by 2025
through universal adoption of a basic set of interventions ( Table 1.8) (14).
As noted earlier in this chapter, one reason for high mortality in resource-limited settings may be the limited numbers of
adequately trained health care professionals in community birth facilities (14). Training in basic newborn care and neonatal
resuscitation has been proposed as a low-cost intervention to reduce neonatal mortality. In 2010, a simplified, low-cost curriculum
for teaching newborn resuscitation in resource-limited areas, Helping Babies Breathe (HBB), was introduced by a consortium led
by the American Academy of Pediatrics (64). Using skill-based learning via simulation, peer teaching, and a pictorial action plan
that guides care, HBB has been shown to be effective in decreasing neonatal mortality and stillbirths (65,66). The success of
HBB led to the design of a simplified educational program, Essential Care for Every Baby (ECEB), based on the principles of
HBB, to teach providers knowledge and skills for essential newborn care. The guidelines for essential newborn care include
resuscitation at birth, early and exclusive breastfeeding, temperature regulation, hygiene, and prevention of infection (67). A study
testing the effectiveness of training birth attendants from rural communities in six countries using a simplified essential newborn
care educational program demonstrated that teaching a bundled newborn care curriculum reduces perinatal mortality (68).

FIGURE 1.11 Integrated service delivery package for maternal, newborn, and child health. Lawn JE, Wilczynska-Ketende
K, Cousens SN. Estimating the causes of 4 million neonatal deaths in the year 2000. Intern J Epidemiol 2006;35:706. doi:
10.1093/ije/dyl043.

NATIONAL AND REGIONAL QUALITY IMPROVEMENT NETWORKS


In 2000, the Institute of Medicine (IOM) highlighted that an estimated 98,000 people die each year as a result of medical errors
that occur in hospitals (69). To Err Is Human and Crossing the Quality Chasm served to galvanize national and local efforts to
improve the care
P.14
delivered in US hospitals (69,70). The IOM reports called attention to the problems of medical errors and created a construct to
emerge as a safer, more equitable health system by defining ideal health care as safe, effective, patient-centered, timely, efficient,
and equitable.

TABLE 1.8 Estimated Lives Saved of Premature Babies in Settings with Universal Coverage of
Interventions

Intervention Reaching 95% Also Saves Mothers or By 2015 By 2025


Coverage Other Babies
% Deaths Lives % Deaths Lives
Averted Saved Averted Saved

Family planninga M, SB, N 24 228,000 32 345,000

Antenatal corticosteroids N 40 373,000 41 444,000

Antibiotics for pPRoM N 9 85,000 9 101,000

Immediate assessment and N 5 44,000 5 53,000


simple care of all babies

Neonatal resuscitation N (SB) 7 65,000 7 77,000

Thermal care N 15 142,000 16 171,000

Kangaroo mother care N 48 452,000 48 531,000

Interventions implemented M, SB, N 81 757,000 84 921,000


together

Note: interventions marked with M will also save maternal lives, SB would avert stillbirth, and N will save newborns dying
from causes other than preterm birth.

aFamily planning scaled to 60% coverage or to a level whereby the total fertility rate is 2.5.

Note that obstetric care would also have an impact, but is not estimated separately.

Lawn JE, Wilczynska-Ketende K, Cousens SN. Estimating the causes of 4 million neonatal deaths in the year 2000.
Intern J Epidemiol 2006;35:706. doi: 10.1093/ije/dyl043.

In 2008, Don Berwick, then the President and Chief Executive Officer of the Institute for Healthcare Improvement (IHI), arguing
that effective health system change must occur in the continuum of care, introduced the Triple Aim with three interdependent
goals of care:
1. Improving the individual experience of care
2. Improving the health of populations
3. Reducing the per capita costs of care for populations
Berwick argued that improvement objectives must include all three laudable goals. For example, reducing per capita costs for
populations without taking into account their health could lead to systematic underutilization. Alternatively, reducing per capita
costs, while maintaining health, would ensure the sustainability of our health system and potentially create resources to improve
our population's overall health (71).

Improving Neonatal Care


Variations in practice (72) and outcomes (73,74) are well documented in neonatal care. The presence of variation and the
unacceptably high rate of care-related preventable complications (e.g., central line-associated bloodstream infections) created the
impetus for national, state, and local quality improvement efforts. The most established and well-known improvement organization
is the VON. VON began in the 1980s as a nonprofit organization with the goals of improving the quality and safety of medical
care for newborn infants and their families through a coordinated program of research, education, and quality improvement (75).
Through the years, VON has grown to include more than 800 neonatal intensive care units worldwide. VON's efforts include
collaboratives focused on specific topics including infection and chronic lung disease (76), and it serves as a peer network
providing feedback on process and outcome indicators. Participating centers collect data for VLBW infants and may also gather
data for all treated infants. Centers are able to compare their processes and outcomes to other similar units.
VON provides risk-adjusted outcomes for participating centers to compare their unit's performance. Risk adjustment enables the
centers to compare outcomes accounting for heterogeneity among neonates (e.g., comorbid conditions). This process enables
differences in outcomes to be better attributed to variations in practice (77). Institutions are presented observed or actual
outcomes (e.g., mortality) versus what would be predicted to occur based upon their patient case-mix. This feedback enables
institutions to identify areas of improvement (75).

State Collaboratives
States throughout the United States are creating PQCs to improve care delivered to infants in their states by advancing evidence-
based clinical practices and processes. PQCs include hospital leadership, pediatricians and neonatologists, obstetricians and
perinatologists, midwives, nurses, and state health department staff. State collaboratives are uniquely positioned to engage local
and state stakeholders to improve neonatal care and address issues that may be unique in their local environments. As of 2014,
33 states have perinatal collaboratives. State-based efforts have been effective in addressing specific problems, such as central
line-associated blood stream infections (78), and in some states, data collection efforts enabled comparisons of outcomes in the
setting of state-based system change (e.g., deregionalization) (46). There is no doubt that the care delivered in US neonatal
intensive care units is safer today than it was just a short time ago; however, there remains significant opportunity to decrease
variability in care and improve outcomes for this vulnerable population.

INTERNATIONAL EFFORTS IN QUALITY IMPROVEMENT


Many countries around the world have launched similar national and international efforts aimed at improving quality of care for
neonates, and several countries have established national networks for benchmarking neonatal outcomes. The International
Network for Evaluating Outcomes (iNEO) comprises nine countries (Australia, Canada, Israel, Japan, New Zealand, Spain,
Sweden, Switzerland, United Kingdom) that pool population-based data from their national networks for international comparison
of neonatal outcomes for benchmarking and quality improvement (79). Learning from other countries has yielded many benefits
and led to the development of several important initiatives such as kangaroo care (80) and familyintegrated care (81), both of
which have led to significantly improved patient outcomes. Canada pioneered the Evidence-based Practice for Improving Quality
(EPIQ) initiative aimed at using a more objective and evidence-based process for quality improvement and demonstrated 32%
reduction in nosocomial infection and 15% reduction in bronchopulmonary dysplasia in a cluster randomized controlled trial of 12
NICUs (82). This was followed by implementation at a national level, which resulted in 37% reduction in adjusted risk for
composite adverse outcomes (defined as death or any of five major morbidities, including nosocomial infection, necrotizing
enterocolitis, bronchopulmonary dysplasia, severe intraventricular hemorrhage, retinopathy of prematurity) for neonates born at
less than 29 weeks of gestation across Canada (83). EPIQ has now been adopted by many hospitals in Asia, Europe, and South
America.
P.15
FIGURE 1.12 National health expenditures, 2012. Martin AB, Hartman M, Whittle L, et al. National health spending in 2012:
rate of health spending growth remained low for the fourth consecutive year. Health Aff (Millwood) 2014;33(1):67.

HEALTH CARE EXPENDITURES AND THE ECONOMICS OF NEONATAL CARE


US National Health Expenditures
The United States spends more per capita on health care than does any other industrialized nation member of the OECD. In
2011, the United States spent an estimated $7,200 per person on health, nearly twice as much as the second highest nation,
Switzerland, which spent $4,300 per person. The United States leads other OECD nations in the proportion of Gross Domestic
Product spent on health, with the lowest share of public funds spent on health annually. However, when compared to other OECD
countries, the US investment in health produces uneven results, lagging behind other OECD countries in many indicators of
health, including infant mortality, and claiming the highest population rate among OECD countries without health coverage (84).
By 2012, aggregate US national health expenditures neared $2.8 trillion and accounted for 17.2% of the US economy (85).
Businesses, households, and other private venues were responsible for 56% of expenditures, compared to federal and state
governments accounting for 44%. The bulk of US health expenditures are attributed to hospital care (31%) and professional
services including physicians (27%) ( Fig. 1.12). In 2012, all facets of health spending grew, with the exception of investments in
noncommercial research which fell 3.1% (85).

TABLE 1.9 Average Length of Newborn Hospital Stays and Hospital Costs by Selected Clinical Outcomes,
2011

Outcomes Count Percent Average Length of Average Hospital Aggregates


Stay (Days) Costs (US $) Hospital
Costs
(Millions
US $)

All live hospital births 3,800,000 100.0 3.4 3,200 12,200

Preterm 321,900 8.5 14.3 21,500 6,900


Low birth weight 231,900 6.1 17.7 27,200 6,200

Weight <1,500 g 49,300 1.3 42.6 76,700 3,700

Weight 1,500 g 181,500 4.8 10.9 14,000 2,500

Respiratory distress 75,100 2.0 31.3 55,000 4,100


syndrome

Died after birth 10,900 0.3 7.4 28,600 300

Note: The categories for preterm, low birth weight, and respiratory distress syndrome overlap and are
not mutually exclusive.

Agency for Healthcare Research and Quality (AHRQ), Healthcare Cost and Utilization Project (HCUP),
Nationwide Inpatient Sample (NIS); Kowlessar NM, et al. Hospital Stays for Newborns, 2011. HCUP
Statistical Brief #163, Agency for Healthcare Research and Quality, Editor 2013, Rockville, MD: Agency
for Healthcare Research and Quality, 2013.

Expenditures for Newborn Care and Prematurity


Estimates of national health expenditures for the provision of care to newborns are difficult to obtain. An analysis of national
hospital billing data by the Agency for Healthcare Research and Quality (AHRQ) estimated national hospital costs of $12.2 billion
for the 3.8 million births that occurred in 2011. AHRQ also estimated that the 321,000 preterm infants born that year accounted for
57% of newborn expenditures. As expected, the most preterm infants accounted for the highest expenditures. In 2011, infants with
weights less than 1,500 g represented just 1.3% of births, but $3.7 billion (30%) in expenditures, or $76,700 per infant on average
(Table 1.9) (86). Importantly, such cost estimates likely underestimate actual costs as they include only hospital costs and not
professional fees.
The true cost of prematurity beyond the birth hospitalization is more difficult to measure. In the 2007, IOM report Preterm Birth:
Causes, Consequences, and Prevention estimated annual expenditures related to preterm birth to be $26.2 billion in 2005,
which, after adjusting for inflation, equates to $31.7 billion in 2014. More than half of annual expenditures are spent on maternal
care at delivery ($2.3B) and their children's hospitalization in the neonatal period and beyond ($20.4B). The IOM also estimated
other direct costs of prematurity including early intervention services ($700M), special
P.16
education ($1.3B), and indirect costs of lost productivity ($6.9B; Table 1.10). The IOM suggests their estimates are likely
conservative as they only include lifetime costs for four conditions (cerebral palsy, mental retardation, vision impairment, and
hearing loss) (87,88).

TABLE 1.10 Estimates Aggregate and Individual Costs Associated with Prematurity in 2014

Medical Care Costs Other Costs

Birth to 6Y Total Early Special Lost Maternal Total


Age 5 and Intervention Education Productivity Delivery
Y older (4 DDs) (4 DDs)
(4
DDs)

Aggregate $19,200 $1,200 $20,400 $700 $1,300 $6,900 $2,300 $31,700


(millions)

Per infant $37,800 $2,300 $40,200 $1,500 $2,600 $13,600 $4,600 $62,400
(dollars)

Institute of Medicine Committee on Understanding Premature Birth and Assuring Healthy Outcomes, Behrman RE, Butler
AS, eds. The National Academies Collection: reports funded by National Institutes of Health. Preterm birth: causes,
consequences, and prevention. Washington, DC: National Academies Press (US) National Academy of Sciences, 2007.

Paying for Newborn Care


In 2011, 48.7% of births were paid for by private insurance companies, 44.7% by state Medicaid programs, 3.6% were self-pay,
and 3.0% by other sources (e.g., Tricare). From 2008 to 2011, the proportion of births paid by Medicaid grew from 40.5% to
44.7% (86). There is substantial state variation in Medicaid paid births ranging from 69% in Louisiana to 24% in Hawaii (89).
Today, Medicaid, also known as Title XIX of the Social Security Act, is the single largest purchaser of health care for newborns.
The program, which began in 1965, was created to provide coverage for impoverished, women, children, and elderly (90). Since
its inception, Medicaid has expanded and now covers more than 61 million Americans accounting for $415B in annual
expenditures. When compared to other populations covered by Medicaid, children represent a relatively small share of total
Medicaid expenditures; in total, children account for 49% of Medicaid enrollment and 21% of expenditures (91). The program's
important role as a safety net for vulnerable populations has been clear during US economic downturns. The recent great
recession from 2007 to 2009 coincided with a rise in unemployment and child poverty, but not a rise in the number of uninsured
children in the United States. The program's design, as well as additional investment in the program through the American
Recovery and Reinvestment Act, ensured that Medicaid insured additional children (92).
Medicaid's role in covering a high proportion of neonates also means that the program has opportunities to manage the quality of
care delivered through the use of incentives and penalties, known as pay-for-performance. For example, the Patient Protection
and Affordable Care Act (PPACA) mandated that Medicaid not pay for certain hospital-associated infections (HAIs) including
central line-associated blood stream infections (93).
Another federal-state program that plays a critical role for neonates is the Maternal Child Health Grant, also known as Title V of
the Social Security Act. As opposed to Medicaid, which is an entitlement program, Title V is a block grant providing a fixed amount
to states for maternal and child health. The program was enacted in 1935 and provides funding for mothers, infants, and has a
specific focus on children with special needs. Combining both federal and state contributions to the program, a total of $6B is
spent annual on Title V activities. The program specifies that at least 30% are spent on preventive and primary care services for
children and at least 30% are allocated for services for children with special health care needs (94).

Patient Protection and Affordable Care Act


In March 2010, the PPACA was signed into law. The passage of the PPACA represented the largest change to the United States
health care system since the passage of Medicaid and Medicare in the 1960s. The basic premise of the PPACA is to expand
health care coverage through Medicaid expansions to previously ineligible groups (e.g., adults without children) through the
creation of federaland state-based health insurance exchanges and by mandating coverage. Importantly, the PPACA also created
standards for coverage that have implications for preterm infants. Prior to the PPACA, annual and lifetime expenditure caps were
not an uncommon feature of insurance policies, especially among those purchased on the individual market (i.e., not employer
sponsored). The PPACA eliminated caps for coverage, ensured that no one be denied coverage for preexisting conditions, and
ensured coverage for maternity care (95). Whether the PPACA will have a beneficial impact on maternal and child health will need
to be evaluated over time.

REFERENCES
1. Berns SD, ed. Toward improving the outcome of pregnancy III: enhancing perinatal health through quality, safety and
performance initiatives. Reissued edition. White Plains, NY: March of Dimes Foundation, 2011.

2. U.S. Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health
Bureau. Child health USA 2013. Rockville, MD: U.S. Department of Health and Human Services, 2013.

3. Guyer B, Freedman MA, Strobino DM, et al. Annual summary of vital statistics: trends in the health of Americans during the
20th century. Pediatrics 2000;106:1307.
4. Centers for Disease Control and Prevention. Advancements in public health, 1900-1999: healthier mothers and babies.
MMWR Morb Mortal Wkly Rep 1999;48:849.

5. National Center for Health Statistics. National Vital Statistics Reports (NVSR). Deaths: final data for 2011.

6. MacDorman MF, Mathews TJ, Mohangoo AD, et al. International comparisons of infant mortality and related factors:
United States and Europe, 2010. National vital statistics reports; vol. 63, no. 5. Hyattsville, MD: National Center for Health
Statistics, 2014.

7. Hoyert DL, Xu JQ. Deaths: preliminary data for 2011. National vital statistics reports; vol. 61, no. 6. Hyattsville, MD:
National Center for Health Statistics, 2012.

8. Horbar LD, Carpenter JH, Badger GJ, et al. Mortality and neonatal morbidity among infants 501-1500 grams from 2000 to
2009. Pediatrics 2012; 129:1019.

9. Mathews TJ, MacDorman MF. Infant mortality statistics from the 2009 period linked birth/infant death data set. National
vital statistics reports; vol. 61, no. 8. Hyattsville, MD: National Center for Health Statistics, 2013.

10. National Commission on Children. Beyond rhetoric: a new American agenda for children and families. Washington, DC:
National Commission on Children, 1991:127.

11. Gregory ECW, MacDorman MF, Martin JA. Trends in fetal and perinatal mortality in the United States, 2006-2012. NCHS
data brief, no. 169. Hyattsville, MD: National Center for Health Statistics, 2014.

12. United Nations World Population Prospects. The 2012 revisionUnited Nations development. Retrieved on December
26, 2014 from http://esa.un.org/unpd/wpp/index.htm

13. United Nations World Population Prospects. The 2008 revisionUnited Nations development. Retrieved on December
26, 2014 from http://en.wikipedia.org/wiki/Infant_mortality#mediaviewer/File:Infant_Mortality_Rate_by_Region_1950-2050.png

14. Lawn JE, Wilczynska-Ketende K, Cousens SN. Estimating the causes of 4 million neonatal deaths in the year 2000. Int J
Epidemiol 2006;35:706. doi: 10.1093/ije/dyl043

P.17

15. March of Dimes, PMNCH, Save the Children, WHO. Born too soon: the global action report on preterm birth. In: Howson
CP, Kinney MV, Lawn JE, eds. Geneva, Switzerland: World Health Organization, 2012.

16. Blencowe H, Lee ACC, Cousens S, et al. Preterm birth-associated neuro-developmental impairment estimates at regional
and global levels for 2010. Pediatr Res 2013;74:17.

17. Martin JA, Hamilton BE, Osterman MJK. Births in the United States, 2013. NCHS data brief, no. 175. Hyattsville, MD:
National Center for Health Statistics, 2014.

18. McIntire DD, Leveno KJ. Neonatal mortality and morbidity rates in late preterm births compared with births at term. Obstet
Gynecol 2008;111:35.

19. Tomashek KM, Shapiro-Mendoza CK, Davidoff MJ, et al. Differences in mortality between late-preterm and term singleton
infants in the United States, 1995-2002. J Pediatr 2007;151:450.e1.

20. NICHD Workshop. Optimizing care and long-term outcome of near-term pregnancy and near-term newborn infant. July
18-19. Bethesda, MD: NICHD Workshop, 2005.
21. Zhang X, Kramer MS. Variations in mortality and morbidity by gestational age among infants born at term. J Pediatr
2009;154:358.e1.

22. Clark SL, Belfort MA, Byrum SL, et al. Improved outcomes, fewer cesarean deliveries, and reduced litigation: results of a
new paradigm in patient safety. Am J Obstet Gynecol 2008;199:105.e1.

23. Tita AT, Landon MB, Spong CY, et al. Timing of elective repeat cesarean delivery at term and neonatal outcomes. N Engl
J Med 2009;360:111.

24. Timmermans S, Bonsel GJ, Steegers-Theunissen RPM, et al. Individual accumulation of heterogeneous risks explains
perinatal inequalities within deprived neighbourhoods. Eur J Epidemiol 2011;26(2):165.

25. Izugbara CO, Ngilangwa D. Women, poverty and adverse maternal outcomes in Nairobi, Kenya. BMC Womens Health
2010;10:33. doi: 10.1186/1472-6874-10-33

26. World Bank 2012. An update to the World Bank estimates of consumption poverty in the developing world. Retrieved on
December 26, 2014 from http://siteresources.worldbank.org/INTPOVCALNET/Resources/Global_Poverty_Update_2012_02-
29-12.pdf

27. United Nations 1995. World Summit for Social Development Programme of ActionChapter 2 eradication of poverty.
Retrieved on December 26, 2014 from http://www.un.org/esa/socdev/wssd/text-version/agreements/poach2.htm

28. Dean SV, Imam AM, Lassi ZS, et al. Preconception care: nutritional risks and interventions. Reprod Health 2014;11(suppl
3):S3. doi: 10.1186/1742-4755-11-S3-S3

29. WHO 2014. Obesity and overweight factsheet. Retrieved on December 26, 2014 from
http://www.who.int/mediacentre/factsheets/fs311/en/

30. Rosenberg TJ, Garbers S, Lipkind H, et al. Maternal obesity and diabetes as risk factors for adverse pregnancy
outcomes: differences among 4 racial/ethnic groups. Am J Public Health 2005;95(9):1545. doi: 10.2105/AJPH.2005.065680

31. Czeizel AE, Dudas I, Vereczkey A, et al. Folic acid deficiency and folic acid supplementation. Nutrients 2013;5(11):4760.

32. Grote NK, Bridge JA, Garvin AR, et al. A meta-analysis of depression during pregnancy and the risk of preterm birth, low
birth weight, and intrauterine growth restriction. Arch Gen Psychiatry 2010;67:1012.

33. Parazzini F, Chatenoud L, Surace M, et al. Moderate alcohol drinking and risk of preterm birth. Eur J Clin Nutr
2003;57(10):1345.

34. Shiono PH, Klebanoff MA, Nugent RP, et al. Fetus-placenta-newborn: the impact of cocaine and marijuana use on low
birth weight and preterm birth: a multicenter study. Am J Obstet Gynecol 1995;172(1 Pt 1):19. doi: 10.1016/0002-
9378(95)90078-0. PMID 7847533

35. van den Broek NR, Jean-Baptiste R, Neilson JP. Factors associated with preterm, early preterm and late preterm birth in
Malawi. PLoS One 2014;9(3):e90128. doi: 10.1371/journal.pone.0090128

36. United Nations Development Programme. Millennium development goals. Retrieved on December 26, 2014 from
http://www.undp.org/content/dam/undp/library/MDG/english/UNDP_MDGReport_EN_2014Final1.pdf

37. Thompson LA, Goodman DC, Little GA. Is more neonatal intensive care always better? Insights from a cross-national
comparison of reproductive care. Pediatrics 2002;109(6):1036.
38. American Board of Pediatrics. Workforce data 2013-2014. https://www.abp.org/sites/abp/files/pdf/workforcebook.pdf

39. Wallman C; American Academy of Pediatrics Committee on Fetus and Newborn. Advanced practice in neonatal nursing.
Pediatrics 2009;123: 1606 (reaffirmed January 2014).

40. Freed GL, Dunham KM, Lamarand KE, et al. Neonatal nurse practitioners: distribution, roles and scope of practice.
Pediatrics 2010;126:856.

41. Cusson RM, Buus-Frank ME, Flanagan VA, et al. A survey of the current neonatal nurse practitioner workforce. J
Perinatol 2008;12:830.

42. Pressler JL, Kenner CA. The NNP/DNP shortage: transforming neonatal nurse practitioners into DNPs. J Perinat
Neonatal Nurs 2009;23:272.

43. American Academy of Pediatrics Committee on Fetus and Newborn. Levels of neonatal care. Pediatrics 2012;130:387.

44. Textbook of neonatal resuscitation. 6th ed. 2011.

45. Lasswell SM, Barfield WD, Rochat RW, et al. Perinatal regionalization for very low-birth-weight and very preterm infants: a
meta-analysis. JAMA 2010;304:992.

46. Kastenberg ZJ, Lee HC, Profit J, et al. Effect of deregionalized care on mortality in very-low-birth-weight infants with
necrotizing enterocolitis. JAMA Pediatr 2015;169(1):26. doi: 10.1001/jamapediatrics.2014.2085

47. Chien LY, Whyte R, Aziz K, et al. Canadian Neonatal Network. Improved outcome of preterm infants when delivered in
tertiary care centers. Obstet Gynecol 2001;98:247.

48. Lee SK, McMillan DD, Ohlsson A, et al. The benefit of preterm birth at tertiary care centers is related to gestational age.
Am J Obstet Gynecol 2003;188(3):617.

49. Phibbs CS, Baker LC, Caughey AB, et al. Level and volume of neonatal intensive care and mortality in very-low-birth-
weight infants. N Engl J Med 2007;356:2165.

50. Watson SI, Arulampalam W, Petrou S, et al. The effects of designation and volume of neonatal care on mortality and
morbidity outcomes of very preterm infants in England: retrospective population-based cohort study. BMJ Open
2014;4:e004856.

51. Rogowski JA, Horbar JD, Staiger DO, et al. Indirect vs direct hospital quality indicators for very low birth-weight infants.
JAMA 2004;291:202.

52. Audibert F. Regionalization of perinatal care: did we forget congenital anomalies? Ultrasound Obstest Gynecol
2007;29:247.

53. Calisti A, Oriolo L, Giannino G, et al. Delivery in a tertiary center with co-located surgical facilities makes the difference
among neonates with prenatally diagnosed major abnormalities. J Matern Fetal Neonatal Med 2012;25:1735.

54. Nasr A, Langer JC; Canadian Paediatric Surgery Network. Influence of location of delivery on outcome in neonates with
gastroschisis. J Pediatr Surg 2012;47:2022.

55. Nasr A, Langer JC, Canadian Paediatric Surgery Network. Influence of location of delivery on outcome in neonates with
congenital diaphragmatic hernia. J Pediatr Surg 2011;46:814.
56. Howell EM, Richardson D, Ginsburg P, et al. Deregionalization of neonatal intensive care in urban areas. Am J Public
Health 2002;92:119.

57. Blackmon LR, Barfield WD, Stark AR. Hospital neonatal services in the United States: variation in definitions, criteria and
regulatory status, 2008. J Perinatol 2009;29:788.

58. Freeman VA. Very low birth weight babies delivered at facilities for high-risk neonates: a review of Title V national
performance measure 17. 2010; http://mchb.hrsa.gov/grants/natlperformmeasure17rpt.pdf (Accessed 11-16-14).

59. WHO 2014. Every newborn: an action plan to end preventable deaths. Retrieved on December 26, 2014 from
http://www.everynewborn.org/Documents/Full-action-plan-EN.pdf

60. Horton R. Newborn survival: putting children at the centre. Lancet 2005; 365(9462):821.

61. Goldenberg RL, Culhane JF, Iams JD, et al. Epidemiology and causes of preterm birth. Lancet 2008;371(9606):75.

62. Iams JD, Romero R, Culhane JF, et al. Primary, secondary, and tertiary interventions to reduce the morbidity and mortality
of preterm birth. Lancet 2008;371(9607):164.

63. Saigal S, Doyle LW. An overview of mortality and sequelae of preterm birth from infancy to adulthood. Lancet
2008;371(9608):261.

64. Singhal N, Lockyer J, Fidler H, et al. Helping Babies Breathe: global neonatal resuscitation program development and
formative educational evaluation. Resuscitation 2012;83(1):90.

65. Goudar S, Somannavar M, Clark R, et al. Stillbirth and newborn mortality in India after helping babies breathe training.
Pediatrics 2013;131(2):e344. doi: 10.1542/peds.2012-2112

66. Msemo G, Massawe A, Mmbando D, et al. Newborn mortality and fresh stillbirth rates in Tanzania after helping babies
breathe training. Pediatrics 2013;131(2):e353.

67. World Health Organization. Pregnancy, childbirth, postpartum and newborn care: a guide for essential practice, 2nd ed.
Geneva, Switzerland: World Health Organization, 2006.
http://www.who.int/reproductivehealth/publications/maternal_perinatal_health/924159084X/en/

68. Carlo WA, Goudar SS, Jehan I, et al. Newborn-care training and perinatal mortality in developing countries. N Engl J Med
2010;362(7):614.

69. Institute of Medicine Committee on Quality of Health Care in A. In: Kohn LT, Corrigan JM, Donaldson MS, eds. To err is
human: building a safer health system. Washington, DC: National Academies Press (US); Copyright 2000 by the National
Academy of Sciences. All rights reserved, 2000.

70. Institute of Medicine Committee on Quality of Health Care in A. Crossing the quality chasm: a new health system for the
21st century. Washington, DC: National Academies Press (US); Copyright 2001 by the National Academy of Sciences. All
rights reserved, 2001.

71. Berwick DM, Nolan TW, Whittington J. The triple aim: care, health, and cost. Health Aff (Millwood) 2008;27(3):759.

72. McCormick MC, Escobar GJ, Zheng Z, et al. Place of birth and variations in management of late preterm (near-term)
infants. Semin Perinatol 2006; 30(1):44.
P.18

73. Sankaran K, Chien LY, Walker R, et al. Variations in mortality rates among Canadian neonatal intensive care units. Can
Med Assoc J 2002; 166(2):173.

74. Vohr BR, Wright LL, Dusick AM, et al. Center differences and outcomes of extremely low birth weight infants. Pediatrics
2004;113(4):781.

75. Horbar JD, Soll RF, Edwards WH. The Vermont Oxford Network: a community of practice. Clin Perinatol 2010;37(1):29.

76. Horbar JD, Rogowski J, Plsek PE, et al. Collaborative quality improvement for neonatal intensive care. NIC/Q Project
Investigators of the Vermont Oxford Network. Pediatrics 2001;107(1):14.

77. Patrick SW, Schumacher RE, Davis MM. Methods of mortality risk adjustment in the NICU: a 20-year review. Pediatrics
2013;131(suppl 1):S68.

78. Fisher D, Cochran KM, Provost LP, et al. Reducing central line-associated bloodstream infections in North Carolina
NICUs. Pediatrics 2013; 132(6):e1664.

79. Shah P, Lee SK, Lui K, et al.; iNEO. The International Network for Evaluating Outcomes of very low birth weight, very
preterm neonates (iNeo): a protocol for collaborative comparisons of international health services for quality improvement in
neonatal care. BMC Pediatr 2014;14:110.

80. Ramanathan K, Paul VK, Deorari AK, et al. Kangaroo mother care in very low birth weight infants. Indian J Pediatr
2001;68:1019.

81. O'Brien K, Bracht M, Macdonell K, et al. A pilot cohort analytic study of Family Integrated Care in a Canadian neonatal
intensive care unit. BMC Pregnancy Childbirth 2013;13(suppl 1):S12.

82. Lee SK, Aziz K, Singhal N, et al. Improving the quality of care for infants: a cluster randomized controlled trial. CMAJ
2009;181(8):469.

83. Lee SK, Shah P, Singhal N, et al.; Canadian EPIQ Study Group. Association of a quality improvement program with
neonatal outcomes in extremely preterm infants: a prospective cohort study. CMAJ 2014;186(13):E485.

84. Lorenzoni L, Belloni A, Sassi F. Health-care expenditure and health policy in the USA versus other high-spending OECD
countries. Lancet 2014; (9937):83.

85. Martin AB, Hartman M, Whittle L, et al. National health spending in 2012: rate of health spending growth remained low for
the fourth consecutive year. Health Aff (Millwood) 2014;33(1):67.

86. Kowlessar NM, et al. Hospital Stays for Newborns, 2011. HCUP Statistical Brief #163, Agency for Healthcare Research
and Quality, Editor 2013, Rockville, MD: Agency for Healthcare Research and Quality, 2013.

87. Institute of Medicine Committee on Understanding Premature Birth and Assuring Healthy Outcomes, Behrman RE, Butler
AS, eds. The National Academies Collection: reports funded by National Institutes of Health. Preterm birth: causes,
consequences, and prevention. Washington, DC: National Academies Press (US) National Academy of Sciences, 2007.

88. Bureau of Labor Statistics. Consumer price index. 2014 [cited 2014 July 15]; Available from: http://www.bls.gov/cpi/

89. Markus AR, Andrus E, West KD, et al. Medicaid covered births, 2008 through 2010, in the context of the implementation of
health reform. Womens Health Issues 2013;23(5):e273.
90. Patrick SW, Freed GL. Intergenerational enrollment and expenditure changes in Medicaid: trends from 1991 to 2005. BMC
Health Serv Res 2012;12:327.

91. State Health Facts. 2014 [cited 2014 December 4]; Available from: http://www.statehealthfacts.org

92. Patrick SW, Choi HJ, David M. Increase in federal match associated with significant gains in coverage for children through
Medicaid and CHIP. Health Aff (Millwood) 2012;31(8):1796.

93. Patrick SW, Kawai AT, Kleinman K, et al. Health care-associated infections among critically ill children in the US, 2007-
2012. Pediatrics 2014;134(4):705.

94. Title V Maternal and Child Health Services Block Grant Program. 2014 [cited 2014 December 2]; Available from:
http://mchb.hrsa.gov/programs/titlevgrants/

95. Summary of the Affordable Care Act. 2013 [cited 2014 December 2]; Available from: http://kff.org/health-reform/fact-
sheet/summary-of-the-affordable-care-act/
2
Newborn Intensive Care Unit Design: Scientific and Practical
Considerations
Gilbert I. Martin
Robert D. White
Judith A. Smith
Stanley N. Graven

INTRODUCTION
As of 2015, there are approximately 1,100 neonatal intensive care units (NICUs) in the United States and 40 in
Canada. There are currently over 5,000 board-certified neonatologists and many more pediatricians who are
practicing neonatology (1). Add to this figure the growing number of neonatal nurse practitioners, which makes
the personnel caring for the sick neonate a major force. Over time, the definition of the levels of neonatal care
has been revised, so that there are now four levels: Level I (basic), Level II (specialty), Level III and IV
(subspecialty). Each of these care levels has well-defined capabilities and unit staffing requirements for the
NICUs providing the care (2).
In order to be successful, most hospitals today need to have full capabilities to care for the mother and infant.
Advances in technology and changing patterns in community demographics have mandated redesigned, state-of-
the-art perinatal services, and in particular neonatal intensive care.
Although specific design issues, once anecdotal and now often evidence based, have progressed, the basic
mission statement governing design and redesign of an NICU remains the same: the provision of high-quality
and compassionate care, which includes the following:
Care that honors the racial, ethnic, cultural, religious, and socioeconomic diversity of family and staff
Education, information, and emotional support
Access to the most current effective therapies
Integrated treatment plans emphasizing coordination throughout the continuum of care
Encouragement of family support and involvement
Provision of a state-of-the-art, integrated, family-centered approach to neonatal care (3).
When consensus is reached on a mission statement, specific goals and objectives can be defined and applied to
local demographics, care practices, and competition. Defining these goals will be the first step toward decisions
on bed capacity, types of equipment needed, and changes in care practices. The goals and objectives should be
measurable (e.g., survival and morbidity rates compared to regional and national standards, staff experience and
turnover, parental satisfaction ratings, cost per patient day) and realistic, so that the overall value of the project
can be weighed against initial cost projections and reviewed on an ongoing basis after NICU construction is
complete. In order to accomplish the above, a team concept approach is necessary.

THE TEAMS
The Strategic Planning Team
The strategic planning team will continue to develop the vision and goals that led to the decision to pursue new
construction. Team members should include, at a minimum, an administrator, a neonatologist, and a nursing
director. This group will be responsible for reviewing utilization and demographic information (available from state
and local planning and health agencies, the insurance industry, and the Census Bureau) in order to define the
service area and appropriate number of beds for the NICU. Total NICU days for a defined region will
approximately 1.25 patient-days per live birthif a region has 10,000 live births per year, it will generate
approximately 13,000 NICU patient days, or an average census of 35 babies, with an average length of stay of
20 to 25 days.
The strategic planning team also should make some basic calculations regarding staffing patterns, especially if
this is to be a new service for the hospital. Depending on patient mix, overall staffing patterns may require up to 4
to 6 nurses and 2 support staff (inclusive of nursing administration, respiratory therapy, developmental therapy,
social work, unit clerk, and housekeeping staff) per shift for every 10 babies in the average daily census.
The strategic planning team also will need to assess the impact of the new or renovated NICU on other hospital
departments, especially obstetric, maintenance, and supply, laboratory, and imaging services.

The Financial Planning Team


This group is composed of the hospital's chief financial and operational officers, nursing management, and any
other individuals representing areas of the hospital whose budgets will be significantly affected. In addition, the
financial planning team should utilize consultants who are familiar with the coding and reimbursement process
that will ultimately affect the bottom line financial health of the project.

The Care Practices Team


This large group represents caregivers in the NICU. These individuals include the following disciplines:
neonatology, nursing management and staff, respiratory therapy, social work, pharmacy, laboratory, radiology,
infection control, nutrition, and housekeeping. It is also essential to have parents represented on this team.
Reevaluation of current care practices utilizing evidence-based information when possible will assure a state-of-
the art approach. The goal is to provide optimal care for babies, working environment for staff, and integration of
families.

The Design Team


When the initial goals of the project are completed, an architect group should be interviewed and hired. The
architectural firm chosen should have all required engineering and interior design specialists on staff, as well as
an equipment representative and a neonatal nurse planner. The entire architectural team should be familiar with
the latest trends in NICU design and the scientific principles behind the design process. Once chosen, the
architectural group and the strategic planning team can develop a timetable for planning and construction of the
new design or redesign (3).

SITE VISITS
Touring other NICUs can be a valuable investment for many reasons, whether planning for a renovation or new
construction. Visits provide a firsthand look at design features that have or have not been successful.
The typical purpose of a site visit during the early stages of planning is to stimulate and evaluate ideas. Many
NICU teams find it useful to tour other units early in the process and to continue their visits during various stages
of planning and construction (3).

SPECIFIC DESIGN ISSUES


Location within the Hospital
The NICU shall be a distinct area within the facility and if possible in close proximity to the labor and
delivery unit. If the services are on separate floors, there needs to be an elevator adjacent to the
P.20
units that is only utilized for service between labor and delivery and the NICU. A separate fully equipped
resuscitation room or a resuscitation area in each delivery room is necessary.
Many NICUs, particularly in small or medium-sized hospitals, share staff and responsibilities with the well-
baby nursery or pediatric ICU. When these areas are contiguous, much of the support space (e.g., family
lounge, staff lockers, equipment storage) can be shared, and the opportunities for staff to assist one
another are enhanced.
Traffic patterns for infants who leave the NICU for procedures should be identified and private hallways
created wherever possible, so that ill infants and their attendants do not have to use public areas.
Security Considerations
The NICU should be designed as a component of an overall security program that will protect the physical
safety of infants, families, and staff and minimize the risk of infant abductions. The number of entrances and
exits should be limited, and control station(s) should allow for direct visualization of all visitors. The need for
security must be balanced with the need for comfort and privacy. New technology, including cameras and
other electronic detection systems, will allow for a safe yet intimate NICU experience.
Fire exits should be carefully planned in the initial design, and clearly marked, as should the location of fire
extinguishers. The fire marshal should be given the first draft of the design documents so that any problems
can be corrected early.
Reception Area and Family Support Space
With the additional emphasis on family-centered care, the reception area will introduce the first impression
of the NICU for the family. The size and layout of this space is often dependent on not only the size of the
NICU but its own individual culture. The means to communicate with an NICU staff member should be
provided in the reception area. If possible, there should be a hospital-based individual in charge of this
reception desk. In addition to the reception area, a family lounge should be designed that provides
comfortable seating but is not conducive to overnight sleeping. This lounge area should have a television
set, reading material for families, and a toy box for children. Access to the Internet can be provided via a
computer station in the family lounge area, and there should be space for families to secure valuables.
Public restrooms and telephones should be available nearby.
In addition to generous provision of space at the bedside and in the family lounge, parents need space to
stay overnight, to meet in private with staff to discuss their baby or to grieve, and to breastfeed. Depending
on the size of the NICU and its local practices, some of these functions can be combined, but none can be
ignored.
Breastfeeding of premature or ill infants is poorly accommodated in many existing NICUs. Mothers should
be able to breastfeed babies at the bedside without compromising their privacy. A single-family room design
alleviates the need for additional breast-feeding rooms. However, if the design is not a single-family room
model, private breast pumping areas are required.
Signage
The location and content of signs are often overlooked when planning an NICU. Some thought should be
given to traffic patterns for families and the public from the hospital entrance(s) and how signage will be
used to direct them clearly to the NICU. Information on signs should be phrased warmly, in a way that will
make families feel welcome, rather than sternly, in a way that could make them feel like outsiders and
intruders.
Unit Configuration
NICUs have changed from wardlike configurations to specialized pods, two-bedded rooms, and now to
the individual single bed patient room. This design is now most popular since families want continuous
access to their babies and desire to stay at the bedside, but it raises the potential for isolation of both
families and caregivers. Rooms must be large enough to allow parents the ability to sleep in. Storage and
communication capabilities must be upgraded so that each room is self-sufficient. The family space area
should include a reclining chair suitable for kangaroo/skin-to-skin care, a parent bed, enough outlets for
electronic devices, and adequate storage space.
Certain principles can be established for all direct patient care area plans, regardless of whether a large
multi-bed room, multiple smaller rooms, or private rooms are chosen as the model. First, each patient bed
position must have sufficient space for families to stay for extended periods without interfering with staff
duties. Second, each patient bed position must have individualized lighting, data entry, and communications
systems. Third, traffic patterns must be well planned, with sufficient aisle widths to accommodate diagnostic
equipment and personnel. Nursing functions should be separated from the bedside whenever possible.
There should be adequate space for both direct patient care and nondirect patient care (charting, giving
reports, telephone calls).
Floor Space Requirements
The complete infant space includes clear floor space, aisle space, storage space, and available areas that
allow for necessary furnishings. NICUs have historically been undersized, but now horizons are expanding
to accommodate new practices (especially increased parental access).
There are two methods of defining floor space/bed. The first method recommends a minimum of 120 square
feet for multi-bed rooms and 165 square feet for single-bed rooms, excluding hand-washing stations,
columns and aisles. There shall be an aisle adjacent to each infant space with a minimum width of 4 feet. In
addition, an adjacent aisle of at least 8 feet will allow for passage of equipment and personnel. The second
method utilizes square footage per bed. In the past, 150 to 200 departmental (complete) gross square feet
per bed were recommended. At present, the recommendation has increased to 600 to 800 departmental
gross square feet per bed, which includes patient care areas, office space, support space and space
required for restrooms.
General Support Space
General support space includes clean and soiled utility areas, medical equipment storage and unit
management services. The soiled utility area stores used and contaminated material prior to removal. This
room shall contain a counter and hands-free hand-washing station separate from any utility sinks. The
hand-washing station is controlled by a hands-free mechanism. The soiled utility area should be situated to
allow removal of materials without passing through the infant care area. This soiled room shall have a
ventilation system with negative air pressure. Provision for charting space is also included in general
support space. The technology in this area must be upgraded to allow for electronic medical records and
maintain patient confidentiality. Storage areas should have a generous supply of electrical outlets and
shelving so that battery-powered devices can be recharged. Additional specific considerations are outlined
in each state's code documents.
General Storage Areas
A three-zone storage system is required. The first storage area is the central supply department. The
second storage area is the clean utility room described above. Routinely used supplies (diapers, formula,
linen, cover gowns, information booklets) are stored in this
P.21
space. The space allocated for each infant's equipment should be 18 square feet per infant in intermediate
care and 30 square feet per infant in critical or intensive care. The third storage zone is for items frequently
used at the infant's bedside. In addition, cabinet storage should be 16 cubic feet per infant intermediate care
and 24 cubic feet per infant in critical or intensive care.
Laundry Room Area
It is best to include a separate laundry room where infant clothing, cloth covers, and toys utilized in the
NICU can be cleaned. Space for a commercial-grade washer and dryer should be provided, with the dryer
vented through an outside wall.
Support Space for Ancillary Services
Space for preparation of specialized formulas and additives to breast milk and formula should be in a
separate area. This room should allow for storage for supplies, formulas, and refrigerated and frozen breast
milk. To minimize contamination the ventilation system should have a minimum filtration of 90% based on
the American Society of Heating, Ventilation, and Air Conditioning Engineers standards or have a high-
efficiency particulate air (HEPA) forced air filtration system. All water supplied for feeding preparation should
meet Federal Standards and be commercially sterile.
Other areas for ancillary services such as respiratory therapy, laboratory, pharmacy, radiology, and
developmental therapy should be provided. Although pharmacies are not required to use a laminar flow
hood to prepare oral medication, it is preferable to include one when choosing equipment. The laminar flow
hood prevents contamination of biologic samples. Equipment should include an UV-C germicidal lamp to
sterilize the shell and contents when not in use.
Family Transition Room(s)
Family-infant room(s) shall be provided adjacent to the NICU that allows families and infants extended
private time together. The room(s) shall have a sink, toilet, shower, emergency call, and linkage with the
NICU staff. Sleeping facilities should include at least one parent bed and sufficient space for the infant and
all necessary equipment. Provision of family-infant rooms encourages overnight stays by parents and allows
for better transition to home. If the unit configuration includes only single-patient rooms, family transition
rooms may not be necessary.
Staff Support Space
Staff support space should meet their professional, personal, and administrative needs efficiently. Lockers,
a lounge, toilet facilities, and on-call rooms are necessary. Some charting space, especially for the nursing
and respiratory therapy staff, should be allocated within this area. Additional space, especially for physician
and nurse practitioner charting and discussion, should be provided adjacent to the patient care area. The
communications systems (phone, computer terminal, printer) that link the NICU with the hospital laboratory,
pharmacy, and central supply generally will also be situated in this area.
Several disciplines should have administrative office space immediately adjacent to the NICU, including
social work, medical and nursing administration, and developmental and respiratory therapy. When parent
support or research staff are actively involved in unit activities, they also will need nearby office space. On-
call rooms and a conference room should be situated within this complex, with phone and computer links,
including digital x-ray transmission, and restrooms with showers.
Infection Control and Hand Washing
Hand hygiene is a key component in the prevention and in the reduction of infection in the NICU. A hands-
free hand-washing station shall be provided within 20 feet of every infant bed in multi-bed rooms and in
each patient room when single family rooms are provided. Hand-washing stations should be no closer than
3 feet from an infant bed, supply, or counter/work surface unless a splash guard is provided. Hand-washing
sinks shall be large enough to control splashing and to avoid standing/retained water. Space for hand-
washing instructions, soap and towel dispensers, trash receptacles, and an available timer for the required
three minute hand washing should be provided. Sinks should be large and deep, so that a full surgical
scrub can be performed with minimal splatter. The walls and floor surrounding the sinks should have easily
cleanable surfaces. Porcelain sinks generally are more attractive and quieter in use than stainless steel.
Faucets should operate hands-free; and soap, hand-drying supplies, and large trash receptacles should be
easily accessible. These trash receptacles should be designed to avoid cross-contamination, to be easily
cleanable, and to minimize noise production. At least some sinks should be provided for children and
individuals with handicaps. Signage above each sink should contain written and pictorial hand-washing
instructions. Alcohol-based hand rub dispensers should be placed at many sites in and around the NICU so
that they can be utilized by staff and families. As noted previously, an Infection Control Practitioner will be
an important member of the design team during the discussion of the layout of the NICU and the choice of
surface finishes.
Lighting
Planning appropriate lighting for the NICU requires consideration of the disparate needs of both the babies
and staff. In general, babies need very little light, but exposure to moderate levels of illumination during part
of the day may help establish circadian rhythmicity.
The lighting will be different in many areas in the NICU as illumination of a procedure area will vary from
other ambient lighting situations. Flexibility in lighting levels is required as infants' needs change at different
stages of development. Control of illumination should be accessible to both staff and families, and the
design should include multiple light switches. If linear fluorescent lamps are used, the color designation
should be RE80. All fixtures should have filters or shields that block ultraviolet radiation and minimize the
risk to babies and staff if a bulb should shatter.
Staff need moderate levels of illumination at the bedside to evaluate babies and to perform charting and
manual tasks. At times, intense levels of illumination are necessary to perform procedures and for
phototherapy of hyperbilirubinemia. It is doubtful that babies need natural lighting, but studies of adult office
workers and hospital patients document the benefit of windows for staff and families. A multilevel lighting
scheme should be considered by the design team:
1. Ambient lighting in infant care areas.
Lighting levels are adjustable through a range of 10 to 600 lx (1 to 60 foot-candles) as measured on a
horizontal plane. A control thermistor should allow for immediate darkening if necessary. Electric light
sources should have a color rendering index (CRI) of no less than 80 and a gamut area index (GA) of no
less than 80 and no greater than 100. The optical reflectors in the light fixture shall have a neutral finish
and unnecessary ultraviolet or infrared radiation shall be avoided. Any ambient lighting utilized should
avoid the infant's direct line of sight.
2. Procedure lighting in infant care areas.
Separate procedure lighting should be present at each infant bed and be capable of providing no less
than 2,000 lx. In addition, the light output must be framed to exclude extension beyond its illumination
field. Temporary increases in illumination may be necessary for clinical evaluation or performing a
procedure. It is important not to increase lighting levels for adjacent babies. Intense light may be harmful
to the developing retina. It is best to permanently mount a procedure light than to have a free-standing
floor light, so that space can be maximized and accidents avoided.
P.22
3. Illumination of support areas.
All support areas (e.g., charting, medication preparations, hand washing, reception) shall conform to
current Illuminating Engineering Society of North America (IES) specifications. At times the areas in the
NICU may overlap; if infant care areas are close to charting space, it is important to make sure that the
lighting does not reach adjacent infants.
4. The importance of daylight
At least one source of natural daylight shall be visible from an infant's space or room. If an exterior
window provides this light, the window shall be glazed with insulating glass to minimize heat gain or loss
and shall be situated at least 2 feet away from any part of an infant's bed. Shading devices with neutral
colors are necessary for external windows. Windows providing daylight provide an important
psychological benefit to both staff and families; however, radiant heat loss or gain may occur if babies are
placed too close to an external window.
Acoustic Environment
We have learned a great deal concerning the acoustic environment and the effect of noise on the
development of newborn hearing. The entire NICU environment has both operational sound and
background noise. It is a challenge to design a facility where the combination of background noise and
operational sound should not exceed an hourly Leq of 45 dB and an hourly L10 of 50 dB in infant rooms
and adult sleep areas. In other parts of the NICU (staff, family and lounge), these levels can be somewhat
higher.
Mechanical sources of noise (not including medical equipment) include heating, air-conditioning, plumbing,
vacuum tubes, freezers, refrigerators, and communication systems. Remember too, that pipes, ducts, and
other conduits can produce noise. Airflow through the heating and cooling ducts can produce considerable
background noise in an NICU, but this can be reduced by appropriate sizing and baffling of the ducts.
These issues must be addressed in the design process, because it is prohibitively expensive to correct a
poor design after construction begins.
Today there are Sound Transmission Class (STC) criteria that delineate these factors. In addition,
absorptive surfaces (see Surface Finishes section) should be considered in all plans. The acoustic
environment of the NICU should allow for Speech Intelligibility. There are Intelligibility ratings as defined by
the International Organization for Standardization (IOS).
Some NICUs are situated in noisy communities, which require extra insulation in the external walls to
minimize the impingement of outside sounds into the NICU. Audible telephone noises, alarms (even fire
alarms) and water flow through pipes can also increase the amount of background noise.
Traffic patterns also play a role in determining the level of noise to which babies and staff are exposed. To
the greatest extent possible, traffic flow should be designed so that an echocardiogram, ultrasound, x-ray,
or electroencephalographic technician can get to each baby's bedside as directly as possible, without
wheeling the equipment past several other bed positions. Vibration isolation pads are recommended under
permanent equipment and appliances. As noted previously, support areas should be designed so that
restocking functions can be accomplished without creating unnecessary bedside traffic.
Noise production also should be a prime consideration in design of the monitoring and communication
systems and in the selection of equipment. Whenever possible, equipment should be selected with a noise
criterion rating (NCR) of less than 40.
We believe an acoustical engineer should be part of the design team to assure that the acoustic
environment satisfies all current standards.
Once all unnecessary sources of sound are minimized, the next design consideration is the abatement of
unavoidable sound such as voices, equipment noise, and anything that might disturb a sleeping baby. Here,
there is no substitute for adequate space, and another cogent argument for individualized environments
becomes apparent. Increasing the distance between beds will diminish noise transfer from one baby's
bedside to another, as will higher ceilings, especially those that are angled to reflect sound laterally rather
than back to the bedside. Obviously, floor, wall, and ceiling materials are crucial in this regard (see the
section, Surface Finishes).
Finally, care practices should be evaluated as part of the design process to see whether sources of noise
produced by the staff can be diminished or eliminated. Radios, pagers, rounds, and reports are examples of
care practices that may create considerable noise at the bedside and that can be modified or eliminated
(3,4).
Surface Finishes
In the past, selection of surface finishes was given little attention in NICU design, which focused primarily on
integration of the newest technology. The choice of ceiling, wall, and floor finishes is important, however, for
reasons of aesthetics, noise abatement, and infection control.
Ceiling
The ceiling is the largest available area for sound absorption. Ceiling materials should be designated with a
noise reduction coefficient (NRC) of at least 0.90 for 80% of the entire surface area or an average NRC of
0.85 for the whole ceiling including solid and acoustically absorptive surfaces. Ceilings in infant rooms and
adult sleep rooms shall be specified with a ceiling articulation class (CAC)-29. The CAC provides a barrier
effect that offers protection from sounds transmitted from adjacent areas. Ceiling finishes shall be free of
substances known to be teratogenic, mutagenic, carcinogenic or otherwise harmful. Volatile organic
compounds (VOCs) and persistent bioaccumulative toxins (PBTs) such as cadmium are often found in
paints and ceiling tiles and should be avoided. Many states are now allowing the use of certain types of
nonfriable acoustical ceiling tile that helps with noise abatement. The method of cleaning the ceiling and
changing lights should be considered in the design process so that this can be accomplished with minimal
disruption to patient care.
Walls
Wall finishes increasingly include quilts or soft sculpture to provide aesthetic and sound-absorbent qualities.
Extensive use of railings or moldings is necessary throughout the NICU, as walls are easily damaged by
portable equipment. The wall finishes shall be durable and easily cleanable. Wall coverings contain
polyvinyl chloride (PVC) can affect indoor air quality and should be avoided.
Floors
Floor surfaces shall be easily cleanable and shall minimize the growth of microorganisms. Perhaps the most
controversial design issue in this regard is the choice between carpet and hard flooring. Hard flooring (e.g.,
vinyl, rubber, or linoleum) is easily cleaned, is durable, and provides little resistance to wheeled equipment.
Carpeting provides noise abatement and may be more attractive and more comfortable to those who are on
their feet for many hours a day. The differences between these two choices have started to blur in recent
years, as carpet has become more durable and cleanable, and hard flooring has become more resilient and
sound absorbent. It seems clear that vinyl or rubber flooring is the ideal for patient care areas, for clean and
soiled utility areas, and around sinks. Carpeting may be desirable for some high-traffic public areas to limit
the effect of noise and improve aesthetics.
Headwalls
The area surrounding the baby's bedside containing service outlets, shelving, and bedside storage is
commonly referred to as the headwall. It is the focal point for creating a self-contained
P.23
workstation at each bedside. This area must be easily adaptable to changes in census and acuity and to
future changes in care practices. The system should include easy access for electrical conduits and gas
piping. Flexibility should allow for modification or upgrades as necessary. The headwall must support and
provide easy access to necessary equipment, to supplies, as well as to the baby. The headwall design also
should contain a comfortable working area for the staff and provide space for the family to personalize the
baby's surroundings. There are several headwall systems that can be built on-site, using either moveable or
fixed-rail systems that can be customized.
A complete headwall system might include the following items and capabilities:
Dimmer (rheostat)-controlled task lighting
Three oxygen ports
Three compressed air ports
Three vacuum ports
Twenty to 30 electrical outlets
Telephone jack
Computer terminal jack
All electrical, vacuum, and gas outlets need to be simultaneously and conveniently accessible so that the
use of one outlet cannot obstruct other outlets, even when equipment contains oversized plugs. Some
electrical outlets should provide normal power, whereas others should be on emergency power, because
either system could temporarily become incapacitated.
A fixed or moveable shelf to contain monitoring equipment should be located as near to eye level as
possible and within easy reach. A countertop should provide a work surface, with at least 24 cubic feet of
cabinet space below to hold supplies. Raceways within this cabinetry to hold wiring and gas lines can
minimize the danger and unattractiveness of a clutter of cords, but should be easily accessible for repairs or
modifications. Support beams should be placed to avoid visual obstruction or access problems. A ledge at
the base of the headwall will prevent moveable equipment, such as incubators or warmers, from damaging
the wall.
The goal of a headwall is to allow for an efficient, well-organized, and self-contained work station that
supports the infant.
Heating and Cooling Systems
Heating, ventilation, and air-conditioning (HVAC) is perhaps the most mundane segment of NICU design,
and the one in which most members of the design team feel they have the least expertise; yet it is important
to consider for several reasons. A minimum of six air changes per hour is required, with at least two air
changes being outside air. These values need to be specified for patient care areas in general, and for the
isolation and procedure areas in particular. Minimums usually are dictated by State code. In the case of the
isolation area(s) and soiled utility rooms, a negative air pressure design, with 100% of the air exhausted to
the outside, is imperative. In all cases, a high-efficiency filtering system is needed to remove particulate
matter from the air. A high-efficiency (HEPA) filtration system can also provide improved infection control.
Control of temperature and humidity is particularly important when designing the HVAC system for an NICU.
The system should be able to maintain ambient temperature in the NICU between 72F and 78F
throughout the year, even at the extremes of outside temperatures for that particular locale. A relative
humidity of 30% to 60% should also be maintained. Maintaining temperature and humidity within these
guidelines will minimize heat and water loss for the babies and discomfort for the staff. Heat sources if near
the exterior wall should be placed to decrease the cold wall condition that can lead to convection drafts.
Delivery of airflow into the unit requires considerable forethought. Return ducts should be situated near the
floor so that particulate matter is not carried upward. Supply ducts should be located where drafts will not
be a problem and should be generous in number, so that high-velocity air flow is avoided. Placement of
supply ducts near external walls and windows should be carefully planned to avoid condensation and to
minimize convective heat loss or gain to other babies. The fresh air intake into the hospital HVAC system
should be planned carefully to avoid areas that will contain exhaust fumes from vehicles, nearby buildings,
or from the hospital itself. Ventilation air delivered to the NICU should follow Facilities Guidelines Institute
(FGI) specifications. A regular maintenance program should be organized to assure the proper functioning
of the entire heating and cooling system (3,4).
Communication Systems
Communication systems may constitute the segment of NICU design that requires the greatest anticipation
of future developments. Computer-based systems producing an electronic medical record are common in
today's NICU environment. The daily notes that allow for shared information with physicians, nurses,
respiratory therapists, and other ancillary personnel provide for communication of data and information but
tend to become repetitive if not updated frequently. A recent innovation that is quickly becoming standard is
computerized physician order entry (CPOE). This new technology reduces medical errors and supports
evidence-based practice. There is improved communication between the NICU, the pharmacy, and the
radiology department (5). Intercomlinked audiovisual displays for both families and staff have been shown to
enhance infection control in the NICU (6).
Communication among NICU staff needs to be timely and sensitive to the acoustic environment. Hands-free
devices allow connection between individuals providing care in the NICU. In addition, information can be
transmitted to electronic devices (phones, tablets), which allows for exchange of information.
One aspect of communication between staff deals with improving hand-offs (sign-outs) at the end of a
patient encounter. The Agency for Healthcare Research and Quality (AHRQ) and the Accreditation Council
for Graduate Medical Education (ACGME) has identified hand-offs as a priority for patient safety. Residency
programs are required to emphasize optimal one-on-one communication during the hand-off process (7).
Those planning an NICU should anticipate digital transfer of virtually all information. Computerized
electronic medical records work areas within the patient care area should be designed with adequate space
to add terminals. Ergonomics and lighting should be considered, so that staff can work at these terminals
with minimal strain. Monitoring systems will be interfaced with all the equipment supporting the baby, such
as the incubator, ventilator, and intravenous pumps, and with the patient chart. This interfacing will provide
the obvious benefit of faster and more accurate data acquisition and also new alternatives for patient alarm
systems. Currently, many NICUs still depend on audible alarms to alert staff that a baby needs attention,
but, in the future, these alarms can be transmitted digitally to the staff via headsets, pagers, or other
devices that will improve the acoustic environment.
An emergency system is required so that there is immediate access from delivery rooms, from surgical
suites, and even within the NICU should an untoward event occur. Because using these systems requires a
considerable adjustment in the local culture of the NICU, the design process should include discussion with
end users so that the system chosen will address their needs and concerns.
In addition, communication between higher and lower level NICUs through telemedicine allows for the
expertise of individuals without their physical presence. For example, in addition to sharing clinical patient
information, radiographic and echocardiographic images can be transmitted (8) (see Chapter 7).
Handwritten notes, orders, and even the writing of prescriptions will slowly disappear, and all
communication will become electronic. Although less personal, the reduction of medical
P.24
errors and improved data collection within Health Insurance Portability and Accountability Act (HIPAA)
guidelines will improve NICU care.
Designing for Renovations
Unless the pace of change slows dramatically, all NICUs will be faced with the desire to upgrade their
facility every few years. Many of the suggestions (listed previously) are practical to implement without a
major building program (e.g., reduced ambient lighting; some noise-control measures; better, more
welcoming signage). Others can be accomplished through a renovation-in-place project (e.g., adding
carpeting, wall hangings, and acoustical tiles; providing more rooming-in space for families), but some will
be impossible without new construction and significant increases in available floor space. In the course of a
new construction project, it will be impossible to anticipate all the changes that might occur in neonatal care
and technology in the future, but there are general principles that should be considered.
First, there is no substitute for adequate space. The square footage needed for state-of-the-art NICU care
has increased, yet is still less per patient than that allotted to pediatric or adult ICUs. Most NICU
construction projects completed in the last 20 years have been forced to accept reductions in floor space
from the ideal due to fiscal considerations that often proved to be short-sighted as renovations were
required only a few years later. It is hard to imagine new developments that would reduce the floor space
requirements for NICUs, but it is easy to suggest those that might increase them, such as increased
parental access and continued development of new technology to improve monitoring, diagnosis, and
thereby survival and outcome of extremely low-birth-weight infants.
Second, we are in the midst of a dramatic transition from the NICU as a high-tech, sterile (in every sense of
the word) environment, to one that resembles a baby's bedroom, with all the implications that carries for
parental involvement. This does not mean technology will disappear, or that many bedsides will not
continue to resemble an operating room setup. It does suggest that this latter situation should be the
exception, and that NICU design teams will need to consider the norm to be an area where each baby is
surrounded by family in a nurturing environment (3).

EQUIPMENT SELECTION
Equipment selection is an integral part of the planning process, whether for renovation or new construction.
It is important to recognize that equipment features change rapidly and that, with advancing technology,
specific plans for space allocation and cost must be easily modifiable. Categories of equipment that will
need to be specified include the following:
Environmental (incubators, radiant warmers)
Life support (ventilators, extracorporeal membrane oxygenation)
Monitors
Diagnostic (x-ray, ultrasound, electronic scales)
Treatment (infusion pumps, phototherapy, suction)
Communications (telephone, computer terminals, printers, wireless communication systems)
General support (breast milk freezers, pharmacy refrigerators, formula preparation equipment)
All users, as well as consultants familiar with the process of equipment inventory, planning, procurement,
and installation, and with maintenance of the equipment, should be part of the planning team.
The first step of the equipment selection process involves preparing a list of all fixed and portable
equipment that will be needed. Next, existing equipment should be evaluated to determine which items can
be used in the newly constructed NICU. At this point, dimensions and general space and equipment-
mounting requirements should be transmitted to the design team so that design of the patient care and
storage areas can proceed while decisions are being made regarding purchase of new equipment.
The choice and procurement of new equipment is itself a several-step process. After deciding exactly what
equipment will be needed and the budget available, the selection team should become familiar with options
available in the market. If a considerable amount of new equipment is anticipated, it is wise to organize an
exhibitors' day when all the major vendors can demonstrate their products to the largest number of staff
possible. Alternatively, most large medical and nursing conferences have displays by the major vendors.
When evaluating a new product, considerations should include ease of use, durability, ease of
maintenance, the ability to interface easily with computer and monitor systems, hazards such as noise and
electromagnetic radiation, size and portability, ability to upgrade, and cost. After all this information is
gathered, the equipment consultant should organize it into a report that can be given to all interested parties
(users, maintenance, and procurement staff) for comment. Procurement then can proceed with a request for
bids, and a final purchasing decision can be made when these are available. A delivery schedule should be
developed in coordination with the design and construction teams so that equipment will arrive in sufficient
time to be assembled, tested, and installed before the NICU opens, but not so far in advance that upgrades
and modifications in the technology occur while the equipment is sitting in storage.
After selecting the equipment, the financial planning team will need to consider decisions regarding
purchase versus lease and service contracts on each item. Although many hospitals have standing policies
for such decisions, certain factors may still be worth reviewing. If a piece of equipment is a newer model of
an item from a manufacturer with which the hospital has had considerable experience, the biomedical
maintenance department may feel quite comfortable with assuming the responsibility for repair without the
benefit of a service contract. Purchase of the item usually will be less expensive than leasing in the long run
(although the chief financial officer should confirm this based on the specific terms offered). However, if the
piece of equipment is an entirely new device and only one or two are being acquired, lease or purchase
with a service contract has considerable value in that repairs will be made by experienced technicians, and
faulty equipment may be replaced more readily. In either case, the hospital should have a very clear
understanding of how quickly service will be available, and whether replacements will be timely if repairs
cannot be made promptly, especially if the equipment is crucial to the management of a critically ill child.
These commitments should be obtained in writing, and their reliability should be confirmed by calling other
units currently using similar equipment from each manufacturer.
Before installation occurs, the equipment consultant should review architectural, mechanical, electrical, and
plumbing drawings to ensure that all specifications are current and compatible with the equipment that has
been selected. There is nothing more frustrating than trying to install a piece of equipment that does not fit
or has inadequate electrical or plumbing support. There are also many human dynamics that are crucial to
this process, and the equipment consultant must be able to work with each of the specialists involved and
organize a process that will be well structured and successfully implemented. Again, the construction of a
full-sized, functional mock-up of a patient care area should be considered as part of this process before final
approval of the blueprints (3).

REVIEW AND APPROVAL PROCESS


After several months of planning, the architects will review the completed plans with the design team for final
comment and approval. The hospital administration will be asked to sign off on a series of
P.25
construction documents before submitting them to contractors for bids. Although it is common knowledge to
architects and construction managers, many members of the design team may be unaware that changes after
this time usually are expensive and sometimes impossible. A design that has considerable flexibility built in will
become an obvious benefit, so that interim changes in care practices or equipment can be accommodated easily.
When bids on the project are received, however, some major changes may still be required, because financial
projections by the architects are, of necessity in such one-of-a-kind projects, only rough estimates.
Before finalizing the blueprints, the design team should spend one more session exploring all the possible
sources of unanticipated problems. Any device or system that could break or malfunction should be reevaluated
to see if a change in the design would minimize the impact of such an event. For example, is there anything in, or
attached to, the ceiling that could break or come loose and fall on a baby? Is there any wiring, plumbing, or
ductwork hidden in a wall to which access might be very difficult or disruptive? Is there any passageway that
might be obstructed by a piece of equipment just when a resuscitation became necessary? Architects cannot
anticipate all of these events, which usually are not discussed in the planning process, and staff have difficulty
visualizing the impact of such events from looking at blueprints, so this process usually is best done with several
members of each discipline committed to brainstorming together for several hours. Visits to other NICUs can be
particularly valuable to one who is interested in the flaws as well as the positive features of the design.
After a bid is accepted (a process that, like the choice of an architect, should be based not only on cost but on
the extent and quality of the contractor's previous experience), a meeting should be held with the contractor and
members of all the planning teams to be sure the construction team has caught the vision so carefully thought
out over the previous months. Far from being simply contractors, the construction team can offer many helpful
suggestions along the way if they understand the planning team's desires, especially with regard to specifics
such as easily accessible wiring, minimizing noise generation from HVAC and plumbing supplies, and so forth.
Walkthroughs should be performed regularly throughout the construction process, as multiple fine-tuning issues
that were not anticipated in advance undoubtedly will occur. Any resulting changes from the architectural
drawings should be documented carefully on as is drawings, so that future renovations are not hampered by
unpleasant surprises (3).

Staff Integration: A Prerequisite to Success


In renovation projects, construction often must proceed in stages, while patient care continues to be provided in
the existing unit. With either new construction or renovation, the implementation of new equipment and care
practices can be both exciting and stressful, and it is not unusual for turnover of staff to increase around the time
of the move. There are two contrasting strategies that have been used to ease the transition to a new unit. One
school of thought suggests that introducing new equipment and practices to the greatest extent possible before
the move is valuable to minimize the culture shock of transition. An alternative strategy is based on the concept
that acceptance of new practices is most successful when undertaken wholesale, especially if some issues that
are anxiety provoking (e.g., increased access for families) are balanced by others about which the staff will be
excited (more space, better equipment). In practice, the transition period requires belief of both philosophies,
because some changes may not be possible until the new unit is built (e.g., rooming in for parents), whereas
others will be desirable to implement as soon as possible (e.g., a new ventilator).
Certainly the most important strategy in this regard is to integrate the staff as fully as possible into the planning,
design, and construction process. The staff must understand and buy into the conceptual changes intended by
the mission statement and around which the design process proceeded. Attendance at committee meetings and
posting blueprints are helpful in this regard, especially if comments are encouraged and used. Additionally,
working with a full-scale mock-up of a patient care area and occasional visits to the construction site are very
helpful to those who have difficulty visualizing two-dimensional renderings (3).

Conclusion: The NICU, a Work in Progress


The final step of any NICU construction is a commitment to change-in-place. We are just beginning to
understand the biologic effects of the environment on premature infants, especially the positive and negative
effects of light, sound, touch, movement, and smell at each gestation stage. Likewise, a better recognition of the
role of parents and staff in the care and nurture of their babies will lead to improved care practices, and
technologic improvements are sure to continue. Each of these trends will influence our concept of the optimal
NICU design and should be incorporated to the greatest extent possible on an ongoing basis within an existing
structure, rather than waiting until new construction again becomes feasible. An agreement by all members of the
planning process is that the NICU will be considered a work in progress, rather than a finished edifice, will
enhance the readiness of all disciplines to implement change when the need becomes apparent and will drive
the design teams to build in as much flexibility as possible.

REFERENCES
1. Newborn Intensive Care Units (NICUs) and Neonatologists of the USA & Canada. Directory 2011. Section
on Perinatal Pediatrics. American Academy of Pediatrics. Sponsored by Mead Johnson Nutrition. (Published
by the American Academy of Pediatrics.)

2. Committee on Fetus and Newborn Pediatrics. Levels of neonatal care. Pediatrics 2012;130:587.

3. White RD, Martin GI, Smith J, et al. Newborn intensive care unit design: scientific and practical
considerations. In: MacDonald MG, Seshia MK, Mullett M, eds. Avery's neonatology: pathophysiology and
management of the newborn, 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2005, Chapter 6.

4. White RD, Smith JA, Shepley MM, et al. Recommended standards for newborn ICU design, eighth edition.
J Perinatol 2013;33:S2.

5. Cordero L, Kuehn L, Kumar R, et al. Impact of computerized physician order entry on clinical practice in a
newborn intensive care unit. J Perinatol 2004;24:88.

6. El-Kafrawy U, Taylor RJ, Francis N, et al. Effectiveness of a neonatal intensive care unit access intercom
linked audiovisual display monitor highlighting infection control procedures. Am J Infect Control
2013;41(8):749.

7. Starmer AJ, Sectish TC, Simon DW et al. Rates of medical errors and preventable adverse events among
hospital children following implementation of a resident handoff bundle. JAMA 2013;310(21):2262.

8. Garingo A, Friedlich P, Tesoriero I, et al. The use of mobile robotic telemedicine technology in the
neonatal intensive care unit. J Perinatol 2012;32:55.
3
Organization of Care and Quality in the NICU
Richard J. Powers
Carolyn Lund

INTRODUCTION
The delivery of care in the neonatal intensive care unit (NICU) is a complex process involving numerous
disciplines and personnel. Neonatologists, nurses, respiratory therapists, social workers, developmental care
specialists, pharmacists, clinical dieticians, and occupational and physical therapists have roles to play in
planning, implementing, and evaluating care for infants and their families in the NICU. The day-to-day
management is important to the overall organization and keeps operations in motion. However, to continually
improve practice and reduce medical errors, a system of continuous quality improvement (CQI) is needed.
In recent decades, medicine has witnessed a rapid expansion of knowledge and technology. This expansion has
occurred in parallel with financial pressures brought on by relentless increases in per capita costs of health care
in the United States and limitations in financial resources available to the health care system. These forces are
especially applicable to intensive care subspecialties such as neonatology on which a significant amount of
research and technology is focused and for which delivery of care can be extremely costly.
The volume of available research for practitioners to review and integrate into practice has increased
exponentially in recent years. In 1966, approximately 100 articles were published annually in all fields of medicine
from randomized controlled trials (RCTs), and current estimates are more than 10,000 RCTs published annually
(1). In pediatrics alone, there are 191 specialty-specific journals with more than 22,000 pediatric articles
published in 2010 (2). In addition to the volume of published research, the health care industry has been
challenged to provide more accountability in light of public information such as the Institute of Medicine (IOM)
report estimating 98,000 preventable hospital deaths per year in the United States and other reports of excessive
errors, medical errors, and adverse events (3,4,5).
The field of neonatology is especially vulnerable to the occurrence of adverse events due to the rapid
development of technology with limited evidence supporting widespread adoption (6). The small size, critical
medical conditions, and longer length of stay of NICU patients compound their risk of exposure to complications.
The Harvard Medical Practice Study reported that 1.2% to 1.4% of NICU patients experience a medical error
during their hospitalization (7,8). Ligi reported the incidence of iatrogenic events in the NICU at 25.6 per 1,000
patient-days and that over one-third of the iatrogenic events in the NICU are preventable (9).
In the face of this mounting information on clinical efficacy from RCTs of various treatments, the rapid infusion of
technology, and the high prevalence of preventable complications, neonatal practitioners and institutions face
major challenges. Neonatal practitioners in health care organizations need to efficiently evaluate new
interventions and adopt the most compelling ones in a timely manner, in order to provide optimal patient care and
avoid preventable complications. It is through the principles of quality improvement (QI), along with organizational
adaptability, that continuous integration of research, technology, and improved patient care outcomes is
accomplished.

BACKGROUND OF QUALITY IMPROVEMENT


Quality of care is defined by the IOM as the degree to which health services for individuals and populations
increase the likelihood of desired health outcomes and are consistent with current professional knowledge. (10)
This definition, first proposed in 1990, is now widely accepted and is still considered the best definition of health
care quality today. The concept of health services for individuals and populations is especially important in
neonatology, where evaluation of treatment is often determined by population data such as infant and neonatal
mortality rates or the incidence of neurologic deficits among a specific subgroup such as extremely low-birth-
weight survivors.
The IOM definition also emphasizes that quality care increases the likelihood of beneficial outcomes, a
reminder that quality is not merely the achievement of positive outcomes. Poor outcomes occur despite excellent
care because diseases vary in severity and can defeat even the best efforts. Conversely, patients may do well
despite poor quality of care. Assessing quality thus requires attention to both processes and outcomes of care.
The last part of the definition of quality, consistent with current professional knowledge, highlights the dynamic
and evolving body of knowledge available to health care professionals and the need to revise and update
measures of quality as new interventions become standards of care.
Problems in quality of health care can be classified in three categories: underuse, overuse, and misuse (11).
Underuse is the failure to provide a health care service when it has a significant likelihood of producing a
favorable outcome, for example, failure to provide surfactant in a timely manner after the delivery of an extremely
low-birth-weight infant with respiratory distress syndrome. Overuse occurs when a health care service is
provided despite the fact that its potential for harm exceeds its possible benefit. The widespread use of postnatal
steroids for chronic lung disease, popular in the 1990s, is an example of overuse in neonatology. Misuse occurs
when a preventable complication arises during administration of an appropriately selected treatment. Misuse
includes many of the common medical errors that occur during hospitalization or other health care encounters.
Medical errors have been extensively discussed (3), driving numerous initiatives by governmental and regulatory
agencies aimed at understanding the human and systems factors that contribute to errors (see also Chapter 9).
External reporting systems that collect information on adverse events and errors are important in the reduction of
future errors by alerting practitioners to new hazards, using the experience of individual hospitals applying new
methods to prevent errors and revealing trends that require attention (12). In neonatology, medical errors have
been collated and classified as part of an anonymous error-reporting project in conjunction with the NIC/Q
Quality Improvement Collaborative of the Vermont Oxford Network ( Fig. 3.1) (13).
Regulatory agencies, in conjunction with federal and state governments, have traditionally been charged with the
task of motivating health care professionals and organizations to maintain and improve quality. The Joint
Commission on Accreditation of Healthcare Organizations (JCAHO), formed in 1951, initially developed
standards for hospitals and evaluated compliance to these standards, hypothesizing that compliance with these
standards would correlate with quality care and positive outcomes for patients in hospitals. In accreditation,
quality is evaluated by monitoring adherence to accepted standards and measuring outcomes. Standards used
by accreditation organizations are derived from a variety of sources, including government (via regulatory
agencies at both the federal and state levels), as well as professional and community-based standards of
practice.
P.27
FIGURE 3.1 Vermont Oxford Network Key Performance Measures: The columns display the total number of
infants (N), mean incidence (%), and the values of the lower quartile (Q1) and upper quartile (Q3). A rate lower
than the Q1 means at least 75% of hospitals had a higher rate. A rate higher than Q3 means at least 75% of
hospitals had a lower rate.

Regulation is, for the most part, successful in establishing minimal standards of performance and is an important
means of protecting the public from egregiously poor providers. It has, however, numerous limitations. Standards
are difficult to enforce uniformly, and regulation tends to be inflexible with difficulty in adapting quickly as
knowledge changes. Regulation also fails to stimulate organizations to integrate new technologies or
developments and does not motivate them to continuously improve. CQI can supplement the deficiencies of
regulation alone, while providing an impetus for individuals and organizations to strive for the highest quality of
care.

CONTINUOUS QUALITY IMPROVEMENT


The health care industry has learned from the industrial sector that CQI is an effective system to reduce errors. It
motivates good performers to excel, emphasizes identification of potentially successful change opportunities, and
facilitates change implementation throughout all levels of the organization. CQI provides the framework for
organizations to keep abreast of current knowledge and innovations, identify appropriate changes, and
implement them in a timely manner. There are three components to CQI: measurement, benchmarking, and
action.
P.28
CQI Measurement
An important fundamental in bringing about change is the creation of urgency, a motivational force leading to
alignment of goals within an organization. This provides an answer to the question of what do we need to
change? The basic element in identifying the priorities for QI is the acquisition of data. Data acquisition drives
information, which in turn drives action. Over the past three decades, numerous systems of quality measurement
have been developed, encompassing the areas of outcomes, processes, and patient satisfaction.
Outcome measures represent the most objective and often the most meaningful data for health care
organizations. When applied to populations, outcome measures provide essential feedback to leaders charged
with resource allocation, managers charged with developing successful and efficient organizations, and
individual health care providers.
Due to variability in disease severity among patients from different socioeconomic and cultural backgrounds, as
well as differences in the type of patients cared for in highly specialized tertiary centers compared to community
health facilities, data based on outcomes alone can be inaccurate or misleading. Process measures are also
important in evaluating overall quality. Measures of process are needed to determine that accepted standards of
care are being met regardless of good or bad outcomes.
Several mandatory measures are required of hospitals in order to maintain accreditation and receive
reimbursement. These include the JCAHO Core Measure Sets addressing care in the areas of acute myocardial
infarction, heart failure, pneumonia, and surgical and perinatal care. The perinatal care measure set became a
requirement for all hospitals with greater than 1,100 births per year in January 2014. There are five perinatal
care measures: elective delivery, cesarean section, antenatal steroids, health care-associated bloodstream
infections in newborns, and exclusive breast-feeding. Bloodstream infections, essentially central line-associated
bloodstream infections (CLABSIs), are one of the primary measures used in NICU quality improvement work and
are tracked in most NICU databases using various definitions. The Centers for Disease Control and Prevention
(CDC) Neonatal Health Services Network (NHSN) definition is the most commonly used definition for CLABSIs
(14).
A third category of measurement in assessing quality of care is patient and family satisfaction. This is the result
of applying traditional marketing techniques to the health care industry, and it has accompanied the adoption of
CQI principles from industry to health care. It is also a natural result of a larger movement throughout health care
that recognizes the autonomy and accountability of the patient and family. As the health care community expands
its expectations that patients and their families take on a larger part of the responsibility to maintain their own
health and wellness, the feedback obtained from patients and families regarding their interaction with the health
care system is crucial. Table 3.1 shows examples of neonatology quality measures in the three areas of
outcomes, processes, and patient satisfaction.
Hospitals have collected information on patient and family satisfaction using internal tools or through various
outside vendors. More recently, the Center for Medicare and Medicaid Services of the U.S. Department of Health
and Human Services has required the administration of a national standardized survey of patients' perspectives
of hospital care. This survey, the Hospital Consumer Assessment of Healthcare Providers and Systems
(HCAHPS), was designed to provide a national standard for collecting and publicly reporting information about
patient experience that allows valid comparisons to be made across hospitals locally, regionally, and nationally.
The HCAHPS survey provides limited information on the NICU family experience, thus many NICUs still rely on
other standardized tools for obtaining feedback and benchmarking outcomes related to the family experiences
during the NICU hospitalization of their newborn (15,16).

TABLE 3.1 Typical Quality Improvement Measures

Process measures

Intrapartum antibiotics for mothers with positive group B strep cultures

Timely placement of a pulse oximeter probe in the delivery room

Antenatal steroid use

Use of breast milk in extremely low-birth-weight infants

Postnatal steroid use

Surfactant administration

Incidence of hypocarbia (PaCO2 < 30)

Outcome measures

Mean 1-min and 5-min Apgar scores

Survival rate

Length of stay

Incidence of chronic lung disease

Incidence of retinopathy of prematurity

Incidence of intraventricular hemorrhage

Nosocomial infection rate

Patient satisfaction measures (16)

Information

Care and treatment


Organization

Parental participation

Professional attitude

CQI Benchmarking
Use of Comparative Databases
The second step in the process of CQI is benchmarking of outcomes. Opportunities for benchmarking are
numerous due to the recognition of the contribution it brings to CQI. A number of regional, national, and
international databases have been organized in neonatology, providing benchmarking opportunities through
voluntary participation and confidential reporting of individual center outcomes.
One of the first neonatology databases, and currently the largest, is the Vermont-Oxford Neonatal (VON)
Network. Initiated in 1990 with 36 hospitals, this network has grown to 750 centers (17,18,19). The network
includes data on more than 53,000 very-low-birth-weight (VLBW, 1,500 g) infants each year from U.S. and
international participating institutions. The network also maintains an expanded database of 241 hospitals
reporting on over 106,000 infants greater than 1,500 g.
In the VON Network, centers report outcomes, including survival and length of stay for infants admitted to the
NICU. They also report incidence of chronic lung disease and complications, nosocomial infection,
pneumothorax, necrotizing enterocolitis, intraventricular hemorrhage, retinopathy of prematurity, and other
conditions. All participating centers receive a confidential annual report showing their performance compared
with the database as a whole. Each center can evaluate how they rank with all other centers and with centers
that are grouped in similar categories by number and type of NICU admissions. In the VON Network, all of the
variables are reported in aggregate form showing the mean incidence rate and highest and lowest quartile of
each measure. The mortality rate and length of stay for each center are also adjusted for patient acuity.
Other databases have been formed at regional and national levels. The National Institute of Child Health and
Human Development Neonatal Research Network provides a venue for participating institutions to submit
outcome measures; their aggregate data have been published to serve as a reference for other centers to
compare their performances. These include general survival and complication rates in VLBW infants (20) and
rates of neurologic abnormalities
P.29
(21,22). The Children's Hospitals Neonatal Database (CHND) contains demographic, treatment, and outcome
data on NICU patients from more than 25 U.S. children's hospital NICUs. This database was designed to
develop benchmarking data for the care of infants who are typically cared for in Children's Hospitals, particularly
infants requiring surgical care or management by multiple pediatric sub-specialists. Examples include infants with
surgical anomalies, such as gastroschisis, tracheoesophageal fistula, and necrotizing enterocolitis, or those with
severe bronchopulmonary dysplasia (23).
The Pediatrix Medical Group Clinical Data Warehouse (CDW) captures aggregate data from over 20,000 VLBW
infants annually through the integration of the electronic medical record used for bedside documentation by
neonatal clinicians. The passive acquisition of data in the CDW allows a much broader scope of analysis, since
the variables are not limited to preselected data sets (24).
The passive acquisition of data has been made possible through development of functional electronic medical
record systems that are designed to store data as it is being entered, rather than the retrospective extraction of
data from the written or transcribed elements of the patient chart. The inherent efficiencies of passive acquisition
in real time, with the elimination of retrospective chart review and additional steps of data entry, can lead to a
more comprehensive database. A major concern about passively acquired data is the challenge of maintaining
data accuracy when compared to retrospective data entry following rigorous criteria for defining clinical variables.
The Canadian Neonatal Network receives input for all level III NICUs in Canada. It has also published reports
tracking overall outcomes and complications in infants of all gestational ages (25). In the United States,
numerous statewide databases have been formed by neonatology organizations to promote local participation in
benchmarking and collaborative QI projects. The California Perinatal Quality Care Collaborative (CPQCC) was
the first of these to be organized and has been the model for many other states (26).

Variability in NICU Outcomes


Variability in neonatal outcomes becomes apparent when data from multiple NICUs are analyzed in comparative
databases. Annual VON reports provide the distribution of outcomes among centers by ranking the data and
calculating the 25th and 75th percentiles for mean incidence of the given outcome from each center. Percentile
ranking of mean values from individual centers represents a simple and effective means of illustrating one
center's ranking among the entire sample of participants. Figure 3.1 shows recent data from VON, illustrating this
methodology for selected outcomes (27).
Investigators also report variation in outcomes as part of multicenter prospective interventional trials or
retrospectively as multicenter independent research. Brodie and associates (28) studied nosocomial bloodstream
infections in VLBW infants in six NICUs in the Boston area from 1994 to 1996; mean incidence of infections was
19.1% for the whole group but varied from 8.5% to 42% among the six units. After adjusting for patient- and
treatment-related variables, significant variation persisted. Variation in blood transfusions has been studied,
showing a mean total transfused volume ranging from 95.5 (highest) to 35.0 mL/kg (lowest) (29). Avery and
associates (30) described the variability in incidence of chronic lung disease among eight units surveyed. Later,
an in-depth review of the practices related to respiratory support for infants with respiratory distress syndrome
was undertaken at the eight centers, triggering the study and dissemination of a number of innovations in
respiratory care practices from the unit reporting the best outcome. Wide variation in outcomes among centers is
often found when centers participate in comparative outcome studies. Even when the data have been adjusted
for confounding risk factors, marked variability still exists in many cases. Explanations for this persistent variation
include differences in case mix, data quality, and case finding. However, the final and most important factor is
often variation in effectiveness of clinical practice.
One of the major benefits of participating in the comparative analyses of outcome measures lies in the
understanding that changing clinical care practices truly can influence their outcomes. In most cases, individual
units find outcomes in the lowest quartile for only a few variables of their data set, with the majority falling within
the interquartile range (25th to 75th percentile) or even exceeding the 75th percentile. The lowest quartile
outcomes provide target areas to which focused improvement efforts can be directed. Furthermore, centers in
the database that report better outcomes can be used as resources to identify practices that may benefit centers
in the lowest quartile.
Published data can also be used as a benchmark when concurrent data are not available. It is important to
review the methodology and data definitions in the published benchmark paper to allow for consistency in data
acquisition before any extrinsic comparison can be made. An example is the CLABSI rate published by the
National Healthcare Safety Network, which is used by many NICUs to analyze their infection prevalence (14).
This benchmark definition, used as the gold standard for most public reporting systems, has been periodically
modified to provide better precision in generating comparative data.
Evidence-Based Medicine
Evidence-based medicine is defined as the conscientious, explicit, and judicious use of current best evidence in
making decisions about the care of individual patients (31). In the context of CQI, the definition is expanded
beyond the individual patient to decisions regarding institutional policies in the care of multiple patients with
similar diagnoses. In both applications, the principles are the same: an answerable clinical question is
formulated, the best evidence is located, and the evidence is critically appraised. These steps are essential
whether answering a question regarding treatment for a VLBW infant with a patent ductus arteriosus or
identifying best practices to be implemented in the management of certain VLBW admissions who meet criteria
for the diagnosis of patent ductus arteriosus.

Randomized Controlled Trials


RCTs represent the highest level of evidence and are the foundation for establishing the effectiveness of
interventions. The methodology of RCTs is meant to minimize selection bias by the random allocation of study
subjects at the time of enrollment. This unique characteristic of the RCT establishes it as the basis for traditional
statistical comparisons in both clinical and basic science research. Sound scientific methodology must also
address other potential biases that can impact validity of the results. These include performance bias, or
nonuniform exposure to the intervention; exclusion bias, or incomplete follow-up in obtaining postintervention
data; and assessment bias, or inaccurate measurement of outcomes.

Meta-analyses
Although RCTs are considered the best single methodology to evaluate an intervention, there are often more
than one RCT for a given intervention. Multiple RCTs can be summarized and analyzed via systematic reviews,
either qualitatively where data are summarized without further statistical analysis or quantitatively where
statistical methods are used to combine the results of multiple RCTs. The quantitative systematic reviews are
also known as meta-analyses. Pooling results of similar RCTs can increase the statistical power lacking in
multiple small RCTs or provide more support for decision making when conflicting results are reported in
separate studies on the same treatment. The techniques used in performing meta-analysis are rigorous. The
methodology of meta-analysis has been formally outlined and includes five stages: (a) specify the objectives of
the review, (b) identify and select studies, (c) assess validity, (d) combine results of independent studies, and (e)
make inferences (32).
P.30
Estimates of treatment effect or lack of effect are much stronger in meta-analyses in which there is less
heterogeneity. Heterogeneity refers to the observation of large differences in point estimates among the included
studies. The ability to make inferences from a meta-analysis depends on the methodologic quality of the primary
trials on which the review was based, degree of consistency of results among the trials contributing to the
review, and degree of confidence that the search for all trials relevant to the review was comprehensive (32).
The process of meta-analysis has its critics; attempts at pooling results from various studies not only
incorporates the biases of the primary studies but may add additional bias attributable to study selection and
heterogeneity of the selected studies (33). In addition, pooling data from small studies not adequately powered
cannot answer questions about the potential side effects of new therapies, as compared to larger RCTs.

Evidence-Grading Systems
Although RCTs and meta-analyses of RCTs represent the best sources of evidence, such high-quality evidence
is not always available. Multiple systems for grading the strength of evidence have evolved to account for
published sources other than RCTs. The majority of grading systems place the most value on inferences from a
systematic review of RCTs, with evidence from an individual RCT second, followed by evidence from well-
designed trials without randomization, evidence from nonexperimental studies, and, finally, opinions of respected
authorities or reports of expert committees (34). Sources that may not meet the gold standard of an RCT are
nonetheless important when this gold standard is either not yet available or is likely not achievable. Table 3.2
represents the most recent evidence rating system used by the U.S. Preventative Services Task Force.
Despite the obvious value of evidence provided by RCTs and meta-analyses, quantitative and qualitative
examples of other forms of validation can be found in collaborative benchmarking processes. The Vermont
Oxford Quality Improvement Collaborative for Neonatology reported that one of the most fundamental
components in reducing nosocomial infection in the NICU observed consistently in best-performing units was
staff accountability for nosocomial infections (35). Implementation of this valuable insight was critical for
collaborative participants desiring to reduce infections in their individual units. Although not meeting the usual
criteria for high-grade evidence since it has not been formally studied scientifically, this form of observational
data nevertheless represents an important category of evidence, that is, evidence gained from collaborative
benchmarking.

CQI Action
Tools for Quality Improvement (see Chapter 9)
Fundamental to CQI is the identification and recruitment of improvement teams composed of individuals working
together to solve problems or improve processes in which they are key players. In this approach, a process
needing revision is analyzed using a series of tools, with multiple group discussions among the team,
progressing through specific phases of analysis. This diagnostic journey progresses through description,
cause-effect analysis, elaboration of potential improvements, and prioritization of these improvements based on
research and study. The end result is the identification and implementation of a specific intervention or bundle of
interventions that will have the most constructive impact on the related process.

Aim Statement
The identification of a process needing change leads to the need for a clear articulation of the end result. This is
best summarized by providing answers to the following questions What are we going to change and How will
we know when we've made the change? Members of CQI teams need to share a common understanding of the
problem and what the outcome of the CQI project means in objective terms. A clearly worded goal or aim
statement has proven to be essential. The aim statement must be realistic and specific, with definition of
objective measures representing attainable improvements. Failure of CQI projects has often been traced to
poorly worded aim statements with vaguely defined goals (36,37).

TABLE 3.2 U.S. Preventative Services Task Force Grade Definitions After July 2012

What the Grades Mean and Suggestions for Practice

Grade Definition Suggestions


for Practice

A There is high certainty that the net benefit is substantial. Offer or provide
this service.

B There is high certainty that the net benefit is moderate or there is Offer or provide
moderate certainty that the net benefit is moderate to substantial. this service.
C There is at least moderate certainty that the net benefit is small. Offer or provide
this service for
selected
patients
depending on
individual
circumstances.

D The USPSTF recommends against the service. Discourage the


use of this
service.

I Evidence is lacking, of poor quality, or conflicting, and the balance of If the service is
benefits and harms cannot be determined. offered,
patients should
understand the
uncertainty
about the
balance of
benefits and
harms.

Levels of Certainty Regarding Net Benefit

Level of Description
Certaintya

High The available evidence usually includes consistent results from well-
designed, well-conducted studies in representative primary care
populations.

Moderate The available evidence is sufficient to determine the effects of the


preventive service on health outcomes, but confidence in the
estimate is constrained by such factors as:
The number, size, or quality of individual studies
Inconsistency of findings across individual studies
Limited generalizability of findings to routine primary care practice
Lack of coherence in the chain of evidence

Low The available evidence is insufficient to assess effects on health


outcomes. Evidence is insufficient because of:
The limited number or size of studies
Important flaws in study design or methods
Inconsistency of findings across individual studies
Gaps in the chain of evidence
Findings not generalizable to routine primary care practice
Lack of information on important health outcomes

aThe USPSTF defines certainty as likelihood that the USPSTF assessment of the
net benefit of a preventive service is correct. The net benefit is defined as benefit
minus harm of the preventive service as implemented in a general, primary care
population. The USPSTF assigns a certainty level based on the nature of the
overall evidence available to assess the net benefit of a preventive service.
http://www.uspreventiveservicestaskforce.org/uspstf/grades.htm

Process Description and Analysis


One of the tools used early in the analysis of a problem is process description or flowcharting, which allows
teams to depict the sequence of activities that make up the process. Charting the sequence of activities in a
process can help to identify problem areas that become potential opportunities for improvement. Subsequent
analyses using another tool, the cause-effect diagram, allow the team to attribute causes to the problem areas in
the process being studied. The determination of causes often requires analysis and chart review, with group
discussion and consensus determining
P.31
the likelihood of a cause-effect relationship. The cause-effect analysis is aided by categorization of potential
causes in generic terms comprising common factors including procedures, people, equipment, environment,
materials, etc. The fishbone diagram is helpful in illustrating the contribution of specific factors in these
categories (see example of fishbone diagram in Figure 3.2).

FIGURE 3.2 Fishbone diagram. This skeleton of a fishbone diagram shows the basic categories of potential
causes representing barriers to the implementation of a clinical guideline. Additional factors within each category
are added as the analysis proceeds.

After group consensus and further study using a Pareto analysis (based on the 80-20 rule, ascribing 20% of
the causes to account for 80% of the problems), the next step is to elaborate the relationship between potential
problems identified and their relative effect on the global process under review. The goal in this stage is to
identify the relationship between factors identified with the adverse outcome of the process under review as well
as to systematically describe those factors that have the most potential to favorably influence the process under
review.
Traditional CQI incorporates several tools for ongoing data collection and analysis. Data are essential in
establishing the baseline activity or outcome of a process, ascertaining the relative role of various potential
interventions and measuring the process to document the desired improvement. Examples of data collection
tools include simple check sheets, data sheets, interviews, audits, and surveys. Analysis of the data is often
performed using bar charts, histograms, line graphs, and scatter diagrams. Another important and more
sophisticated tool useful in this stage is the process control chart (38,39,40,41) in which data are plotted over
time; analyzing variation in the data allows the distinction of special-cause variation from common-cause
variation. This distinguishes variation caused by the influence of extrinsic factors from normal variation intrinsic to
the process itself (see Fig. 3.3).

FIGURE 3.3 Statistical process control (SPC) chart. This SPC chart or p-chart is from a random sample of
pharmacy orders for which patients had to wait more than 10 minutes, shown as percent of total orders. The
mean percentage (Avg.) is 18.6%; the upper control limit (UCL) and lower control limit (LCL) are 3 standard
deviations from the mean and would represent special cause variation from some extrinsic factors not a part of
normal variability. From Plsek PE. Quality improvement methods in clinical medicine. Pediatrics 1999;103(1
Suppl E):209 (Fig. 4).
FIGURE 3.4 Annotated run chart. Time is plotted on the x-axis, and measurement is plotted on the y-axis.
When changes are implemented, the chart is annotated to illustrate a temporal link between the various changes
and the measurement. From Ellsbury DL, Ursprung R. A primer on quality improvement methodology in
neonatology. Clin Perinatol 2010;37(1):93 (Fig. 4).

Rapid-Cycle Continuous Quality Improvement


Rapid-cycle CQI entails the use of small tests of change, commonly referred to as PDSA cycles, incorporating
the sequence of Plan (P), Do (D), Study (S), and Act (A). Originally designed by Nolan et al. (36), rapid-cycle
CQI involves the identification of changes chosen as goals by an organization; introduction of change concepts
in small cycles, or tests of change; and measurement of the effects of the change. Interventions based on
credible evidence in the experimental setting may have variable results when applied to a specific neonatal unit
with elements interacting in ways that are unique to that setting. Cycles of intervention, with one potentially better
practice at a time followed by measurement to assess the effects of each implementation, provide the necessary
feedback to members of the QI team. Adoption of individual interventions with small tests of change becomes a
highly efficient means of bringing about meaningful change that is consistent with the overall aim of the project.
An annotated run chart, with a linear display of an outcome over time showing discrete interventions displayed at
their point of implementation, is a helpful tool to gauge the value of different tests of change (42) (see Fig. 3.4).
Rapid-cycle CQI has several basic prerequisites. Organizations with smaller, less hierarchical leadership
structures are best suited for this methodology (43). The use of smaller sample size in initial assessment is
another important element as it allows for more rapid feedback regarding the success or failure of a test of
change. Rapid-cycle CQI works best when potential solutions or successful change concepts are readily
available through prior applications in other settings. However, when no potential solutions are apparent, rapid-
cycle CQI can also be effective in testing new approaches
BUNDLES AND CHECKLISTS
The term bundle refers to multiple interventions disseminated nearly simultaneously to address one or
more interrelated clinical and/or administrative problems. The concept was popularized by the Institute for
Healthcare Improvement (IHI) (44) and has served as an important means to further innovation (45). The
potential efficacy derives from three streams of evolving understandings about the challenges in
implementing change: (a) the appreciation that consensus statements or guidelines are an effective way to
translate complex knowledge into effective practices; (b) the perspective that there are many contributing
factors to each specific outcome targeted in a CQI effort, and (c) the demonstrated utility
P.32
of bundled interventions in the NICU to promote multiple, simultaneous, and effective changes in practices
(46,47,48,49).
Another valuable adjunct to CQI action is the use of checklists. Implementation of bundles and individual
practice changes are facilitated by the use of checklists. Successful complex industries use simple
checklists to make the reliable management of complexity a routine (50). Examples of early successes with
the use of checklists in health care involve elimination of wrong-site surgery and coordination of care in the
ICU to reduce bloodstream infections (51). In health care settings and successful industries involving
complexity, the role of the simple checklist is essential to the routine and reliable completion of everyday
complex tasks.

COLLABORATION
The concept of institutions collaborating with each other for the purpose of improving overall quality of care
has become commonplace and is felt to be essential in successfully and efficiently changing health care.
Comparative databases with institutions prospectively reporting outcomes to identify opportunities for
improvement are an example of how collaboration can benefit organizations. Collaboration is equally
important for implementing practices that enable institutions to adopt new technology and bring about
improved methods of delivering care.
Institute for Healthcare Improvement
The IHI implemented its collaborative model, the Breakthrough Series, in 1995 to address a gap between
available science supporting improved health care and reduced costs and the actual implementation of this
science in the daily work of organizations (52). The Breakthrough Series was developed with the goal of
helping organizations improve the value of their activity by making breakthrough improvements in quality
while reducing costs. The model is based on a short-term learning system that brings together teams from
multiple hospitals to seek improvement in a focused area over a 6- to 15-month period of time. The model
incorporates a standardized sequence of activity, starting with 1. Topic Selection, chosen by IHI leaders
based on known gaps between science and practice; 2. Faculty Recruitment, the identification of content
experts whose role is to develop a list of evidence-based change practices and to serve as facilitators to the
participants throughout the duration of the collaborative; 3. Enrollment of participating organizations; 4.
Learning Sessions, traditional face-to-face meetings bringing together multidisciplinary teams and the expert
faculty to exchange ideas; and 5. Action Periods, during which teams implement changes locally and share
their monthly progress with conference calls, peer site visits, and Web-based discussions that enable them
to share information. During the Action Periods, the Breakthrough Series emphasizes the local use of the
Model for Improvement described previously and summarized in Figure 3.5.
The Breakthrough Series collaborative model has been highly successful and has been widely adopted.
The first Breakthrough Series collaboratives were on reducing cesarean section rates, improving asthma
management, and reducing delays and wait times in the Emergency Department. Since these were
introduced in 1996, the IHI has sponsored over 50 projects involving over 2,000 teams from 1,000
organizations. The model has spread throughout the world and has been adopted by multiple other groups
of health care institutions spinning off collaborative projects under the guidance of the parent organization
(52).
Vermont Oxford Network
The Vermont Oxford Network, with its extensive database has been a natural source of collaborative QI,
motivated by the fertile ground laid by center-to-center variation reported in clinical neonatal outcomes. The
Network has focused on QI activity for over 15 years, starting with its first collaborative, the Neonatal
Intensive Care Quality (NICQ) Collaborative in 1995, based on the successful model of the New England
Cardiovascular Disease Study Group (NNECVSG). The VON model emphasizes the extensive collaboration
between participating sites, involving a discovery process to review and categorize the evidence for
potentially better practices and the sharing of knowledge and experience among centers with face-to-face
meetings, benchmarking site visits, and regular conference calls. VON has sponsored six intensive NICQ
collaboratives and nine Internet-based (iNICQ) collaboratives (53).
FIGURE 3.5 The model for improvement. Adapted from Plsek PE. Quality improvement methods in
clinical medicine. Pediatrics 1999;103(1 Suppl E):206 (Fig. 2).

Pediatrix Medical Group


The natural connection between acquiring patient-specific data, dissemination of center-based aggregate
data, and fostering local identification of variability has been the motivation for other organizational efforts.
The Pediatrix Medical Group electronic medical record, populating the Pediatrix CDW, is another example.
Recognizing the power and potential benefit of this data set, Pediatrix has developed through its Center for
Research and Education a system of collaborative and center-based QI projects under its 100,000 Babies
and Quality Steps programs. The 100,000 Babies program identifies five critical clinical practices and
procedures used in the NICU with the goal of improving the quality of care in each: enhancing nutrition,
optimizing central line use, minimizing mechanical ventilation, improving medication use, and reducing
hypothermia on admission. The Quality Steps program provides centers with QI tools to facilitate local
measurement, implementation of changes, and tracking of progress (24).
California Perinatal Quality Care Collaborative
The CPQCC is an example of a regional network combining a data set with a successful QI system. The
CPQCC was established in 1997 as a regional outgrowth of VON. In addition to the database
P.33
providing risk-adjusted outcomes benchmarked against other California NICUs, the CPQCC has engaged a
Perinatal Quality Improvement Panel, a multidisciplinary committee that defines indicators and benchmarks,
recommends QI objectives, and provides models for performance improvement (54). The CPQCC quality
improvement activity was originally centered on a model of developing a toolkit of activities to drive
improvement processes by a panel of experts and dissemination of the toolkits through a series of
workshops. This method has been shown to be successful (55) but has been replaced by the more
structured approach of the IHI Breakthrough Series Model starting in 2008. The CPQCC has had three
successful collaboratives since then, addressing central line infections (48), breast milk feeding (56), and
delivery room management (62) and is in the midst of a fourth collaborative on reducing length of stay.
Several other successful regional collaboratives have been developed based on the Breakthrough Series
model (49,54,57,58).
Reports of multicenter collaborative QI projects in adult and pediatric medicine settings have demonstrated
improvements over time when comparing outcomes at the end of the collaborative with baseline
measurement (48,49,56,59,60). A limitation of these studies is the lack of prospective controls, since
comparisons are made with the historical cohort. Most reports of successes are based on a pre-post cohort
design using the center as its own control. A recent systematic review evaluating the effectiveness of QI
collaboratives points out the limited number of studies based on a prospective controlled design (61). In this
review, that included 72 published reports of collaborative QI in the health care setting, there were only 12
reports involving 9 studies with a controlled design and only 2 randomized studies. This illustrates the value
of a rigorous design and the identification of control centers in the database when reporting the successes
of QI endeavors. In a recent study by the CPQCC, the IHI model was compared with a less intensive center-
based noncollaborative model in a prospective study. They showed the most improvement in the IHI
collaborative model group, with more limited gains in the center-based group, but also showed significant
improvement in several measures in the control group. In the current environment of networking and open
communication among different organizations, secular trends are often occurring simultaneously with active
improvement efforts, thus a prospective controlled design is critical if a goal is to demonstrate effects
attributed to the QI intervention apart from secular trends (62).

ROBUST PROCESS IMPROVEMENT


In this chapter, we have discussed the Model for Improvement that has been an essential component of the
majority of CQI efforts in the NICU community over the past decades. It includes four key elements of successful
improvement: specific and measurable aims, measures of improvement that are tracked over time, key changes
that will result in the desired improvement, and a series of testing cycles during which teams learn how to apply
key change ideas to their own organization. Significant results have been documented in controlled trials where
this methodology has been used.
There has been criticism of the Model for Improvement based on the lack of progress made by the U.S. health
care industry in reducing major morbidity and mortality resulting from preventable medical errors. Fourteen years
after the release of the IOM report, To Err is Human, policy makers and leaders in health care regulation are
concerned with the ongoing occurrence of health care-associated infections and harmful medication errors
(63,64). Lessons from High Reliability Organizations (HROs) such as the nuclear power and aviation industries
illustrate some basic differences between these groups and the health care industry. Chassin identifies the
qualities driving HROs as (a) preoccupation with failure, (b) avoidance of simplification of their observations and
experiences of their environment, (c) sensitivity to operations, (d) commitment to resilience, and (e) placement of
decision-making authority in the hands of the person or group having the most expertise when confronted with a
threat to safety (65). In this evaluation, Chassin states that health care organizations still behave as if they
accept failure as an inevitable feature of their daily work rather than having a preoccupation with avoiding failure.
Another challenge noted is the inordinate amount of intimidating behavior that suppresses the reporting of safety
problems by health care workers.
Chassin discusses changes in the principles of QI in health care proposed by the JCAHO to enable better
alignment of the goals of the health care industry with those of HROs. These would include three major changes:
(a) the leadership's commitment to the ultimate goal of zero patient harm, (b) the incorporation of all the principles
and practices of a safety culture throughout the organization, and (c) the widespread adoption and deployment of
the most effective process improvement tools and methods. The JCAHO has introduced a new model for process
improvement incorporating elements of Lean, Six Sigma, and Change Management, called Robust Process
Improvement or RPI (66). Lean is a set of well-defined tools intended to eliminate waste and create flow
throughout the value stream. Six Sigma is a statistical model that measures a process in terms of defects and
provides a set of strategies, tools, and methods designed to improve processes so that less than 3.4 errors per
million opportunities occur (representing six standard deviations from the mean). Change Management is a set of
principles designed to increase the success and accelerate the implementation of organizational change efforts.
The JCAHO has adopted this system of Robust Process Improvement internally and has preliminary evidence of
its enhanced effects in four collaborative projects where it was used. These included improved hand hygiene
and handoff communication, and decreased surgical site infections and wrong site surgery risks (66).

ORGANIZATIONAL CHANGE AND UNIT CULTURE


Because of the challenge to improve health care issued by the JCAHO and the IOM, as well as the identification
of hierarchical leadership and failure to include the front-line members of the health care team in QI efforts, a
significant effort to understand organizational change and unit culture is imperative. Change, and an
organization's readiness for change, has been the topic of numerous treatises in the field of industrial
psychology. Although the traditional view of change management is that change begins at the top of an
organization pyramid, recent views emphasize that change must occur at all levels within the organization,
especially on an individual level (43). Three elements in the change process are (a) energizers or motivators that
compel people to take action, (b) impediments to change, and (c) action steps needed for change to occur.
Examples of energizers include objectives, benefits, and negative consequences. Barriers to change include
apathy, errors, disturbed relationships, lost power, status, and money. Action steps are the steps taken to
overcome barriers and resistance to change. It is important in CQI to understand common obstacles and the
unique interrelationships that characterize different organizations in their quests for providing the same quality of
care (67).
One major component to overcoming barriers and resistance to change is the understanding of the importance of
unit or organizational culture. Every organization has a culture that is unique, representing the individuals and
relationships that make up that organization. As Baker has expressed: teams and organizations
P.34
that attempt to implement QI often identify organizational culture as an important barrier to or facilitator of
success (43). It is likely that sustained improvement in quality and safety has more to do with the culture of the
NICU than various improvement techniques, isolated training programs and interventions (68,69). The foundation
for successful QI involves strong motivation, teamwork, and leadership that values the role of understanding and
improving unit culture. In addition, after successful quality goals have been attained, maintaining the gain
becomes just as important as initiating new practice changes, and unit culture is a vital part of this process (70).
CONCLUSION
NICU patients deserve not to be harmed. Improving safety is vital, as is quality of care in helping our
patients to maintain health, cope with illness, and hopefully improve or attain the best quality of life (71).
The integration of comparative databases and benchmarking, evidence-based medicine principles, along
with rapid-cycle processes that are compatible with concepts such as unit culture and change cycles, will
help an individual unit to maintain quality in the face of technologic advances and organizational change.
Participation in collaborative CQI projects can enhance effectiveness, creating a community of practice to
share successful strategies and motivate center participation. Finally, incorporation of additional QI models
utilized by HROs is useful especially when striving to eliminate errors and improve safety.

REFERENCES
1. Ellsbury DL. Crossing the quality chasm in neonatal-perinatal medicine. Clin Perinatol 2010;37(1):1.

2. Quinn N, Hensey O, McDowell DT. A historical perspective of pediatric publications: a bibliometric


analysis. Pediatrics 2013;132(3):406.

3. Kohn LT, Corrigan JM, Donaldson MS. To err is human: building a safer health system. Washington, DC:
National Academy Press, 2000.

4. Kohn L. To err is human: an interview with the Institute of Medicine's Linda Kohn. Jt Comm J Qual Improv
2000;26(4):227.

5. Leape LL, Berwick DM, Bates DW. What practices will most improve safety? Evidence-based medicine
meets patient safety. JAMA 2002;288 (4):501.

6. Sekar KC. Iatrogenic complications in the neonatal intensive care unit. J Perinatol 2010;30(suppl):S51.

7. Brennan TA, Leape LL, Laird NM, et al. Incidence of adverse events and negligence in hospitalized
patients. Results of the Harvard Medical Practice Study I. N Engl J Med 1991;324(6):370.

8. Leape LL, Brennan TA, Laird N, et al. The nature of adverse events in hospitalized patients. Results of the
Harvard Medical Practice Study II. N Engl J Med 1991;324(6):377.

9. Ligi I, Arnaud F, Jouve E, et al. Iatrogenic events in admitted neonates: a prospective cohort study. Lancet
2008;371(9610):404.

10. Lohr K. Medicare: a strategy for quality assurance. Washington, DC: National Academy Press, 1990.

11. Chassin MR, Galvin RW. The urgent need to improve health care quality. Institute of Medicine National
Roundtable on Health Care Quality. JAMA 1998;280(11):1000.

12. Leape LL. Reporting of adverse events. N Engl J Med 2002;347 (20):1633.
13. Suresh G. Don't believe everything you read in the patient's chart. Pediatrics 2003;111(5 Pt 1):1108.

14. Edwards JR, Peterson KD, Andrus ML, et al. National Healthcare Safety Network (NHSN) Report, data
summary for 2006 through 2007, issued November 2008. Am J Infect Control 2008;36(9):609.

15. Conner JM, Nelson EC. Neonatal intensive care: satisfaction measured from a parent's perspective.
Pediatrics 1999;103(1 Suppl E):336.

16. Latour JM, Duivenvoorden HJ, Hazelzet JA, et al. Development and validation of a neonatal intensive
care parent satisfaction instrument. Pediatr Crit Care Med 2012;13(5):554.

17. Horbar JD. The Vermont Oxford Network: evidence-based quality improvement for neonatology.
Pediatrics 1999;103(1 Suppl E):350.

18. Horbar JD. The Vermont-Oxford Neonatal Network: integrating research and clinical practice to improve
the quality of medical care. Semin Perinatol 1995;19(2):124.

19. Horbar JD, Carpenter JH, Badger GJ, et al. Mortality and neonatal morbidity among infants 501 to 1500
grams from 2000 to 2009. Pediatrics 2012;129(6):1019.

20. Stoll BJ, Hansen NI, Bell EF, et al. Neonatal outcomes of extremely preterm infants from the NICHD
Neonatal Research Network. Pediatrics 2010;126(3):443.

21. Laptook AR, Shankaran S, Ambalavanan N, et al. Outcome of term infants using Apgar scores at 10
minutes following hypoxic-ischemic encephalopathy. Pediatrics 2009;124(6):1619.

22. Natarajan G, Shankaran S, Laptook AR, et al. Apgar scores at 10 min and outcomes at 6-7 years
following hypoxic-ischaemic encephalopathy. Arch Dis Child Fetal Neonatal Ed 2013;98(6):F473.

23. Pallotto EK, Hunt PG, Durand DJ, et al. Topics in neonatal informatics: infants and data in the electronic
health record era. NeoReviews 2013; 14:e57.

24. Spitzer AR, Ellsbury DL, Handler D, et al. The Pediatrix Baby Steps Data Warehouse and the Pediatrix
Quality Steps improvement project system tools for meaningful use in continuous quality improvement.
Clin Perinatol 2010;37(1):49.

25. Ge WJ, Mirea L, Yang J, et al. Prediction of neonatal outcomes in extremely preterm neonates.
Pediatrics 2013;132(4):e876.

26. Gould JB. The role of regional collaboratives: the California Perinatal Quality Care Collaborative model.
Clin Perinatol 2010;37(1):71.

27. Vermont Oxford Network Database of very low birth weight infants. Vermont Oxford Network, 2013.
Generated from Nightingale Internet Reporting System on 12/9/13.
28. Brodie SB, Sands KE, Gray JE, et al. Occurrence of nosocomial bloodstream infections in six neonatal
intensive care units. Pediatr Infect Dis J 2000;19(1):56.

29. Bednarek FJ, Weisberger S, Richardson DK, et al. Variations in blood transfusions among newborn
intensive care units. SNAP II Study Group. J Pediatr 1998;133(5):601.

30. Avery ME, Tooley WH, Keller JB, et al. Is chronic lung disease in low birth weight infants preventable? A
survey of eight centers. Pediatrics 1987;79(1):26.

31. Sackett DL, Rosenberg WM, Gray JA, et al. Evidence based medicine: what it is and what it isn't. BMJ
1996;312(7023):71.

32. Sinclair JC, Bracken MB, Horbar JD, et al. Introduction to neonatal systematic reviews. Pediatrics
1997;100(5):892.

33. Soll RF. Evaluating the medical evidence for quality improvement. Clin Perinatol 2010;37(1):11.

34. AHRQ. U.S. Preventive Services Task Force, Grade Definitions.


http://www.uspreventiveservicestaskforce.org/uspstf/grades.htm. Accessed December, 2013.

35. Kilbride HW, Powers R, Wirtschafter DD, et al. Evaluation and development of potentially better practices
to prevent neonatal nosocomial bacteremia. Pediatrics 2003;111(4 Pt 2):e504.

36. Langley G, Nolan K, Nolan T, et al. The improvement guide: a practical approach to enhancing
organizational performance. San Francisco, CA: Jossey-Bass, 2009.

37. Nelson EC, Batalden PB, Godfrey MM. Quality by design: a clinical microsystems approach. San
Francisco, CA: Jossey-Bass, 2007.

38. Plsek PE. Quality improvement methods in clinical medicine. Pediatrics 1999;103(1 Suppl E):203.

39. Carey R, Lloyd RC. Measuring quality improvement in healthcare: a guide to statistical process control
applications. New York: Quality Resources, 1995.

40. Schulman J. Evaluating the processes of neonatal intensive care. London, UK: BMJ Publishing Group,
2004.

41. Lloyd RC. Navigating in the turbulent sea of data: the quality measurement journey. Clin Perinatol
2010;37(1):101.

42. Ellsbury DL, Ursprung R. A primer on quality improvement methodology in neonatology. Clin Perinatol
2010;37(1):87.

43. Baker GR, King H, MacDonald JL, et al. Using organizational assessment surveys for improvement in
neonatal intensive care. Pediatrics 2003; 111(4 Pt 2):e419.

44. Nolan T, Berwick DM. All-or-none measurement raises the bar on performance. JAMA
2006;295(10):1168.

45. Levy MM, Pronovost PJ, Dellinger RP, et al. Sepsis change bundles: converting guidelines into
meaningful change in behavior and clinical outcome. Crit Care Med 2004;32(11 suppl):S595.

46. Kilbride HW, Wirtschafter DD, Powers RJ, et al. Implementation of evidence-based potentially better
practices to decrease nosocomial infections. Pediatrics 2003;111(4 Pt 2):e519.

47. Golombek SG, Rohan AJ, Parvez B, et al. Proactive management of percutaneously inserted central
catheters results in decreased incidence of infection in the ELBW population. J Perinatol 2002;22(3):209.

48. Wirtschafter DD, Pettit J, Kurtin P, et al. A statewide quality improvement collaborative to reduce neonatal
central line-associated blood stream infections. J Perinatol 2010;30(3):170.

P.35

49. Schulman J, Stricof R, Stevens TP, et al. Statewide NICU central-line-associated bloodstream infection
rates decline after bundles and checklists. Pediatrics 2011;127(3):436.

50. Gawande A. The checklist manifesto how to get things right. New York: Henry Holt and Company, 2009.

51. Pronovost P, Needham D, Berenholtz S, et al. An intervention to decrease catheter-related bloodstream


infections in the ICU. N Engl J Med 2006;355(26):2725.

52. The breakthrough series: IHI's collaborative model for achieving breakthrough improvement. IHI
Innovation series white paper. Boston, MA: Institute for Healthcare Improvement, 2003.

53. Horbar JD, Soll RF, Edwards WH. The Vermont Oxford Network: a community of practice. Clin Perinatol
2010;37(1):29.

54. Billett AL, Colletti RB, Mandel KE, et al. Exemplar pediatric collaborative improvement networks:
achieving results. Pediatrics 2013;131(suppl 4):S196.

55. Wirtschafter DD, Powers RJ, Pettit JS, et al. Nosocomial infection reduction in VLBW infants with a
statewide quality-improvement model. Pediatrics 2011;127(3):419.

56. Lee HC, Kurtin PS, Wight NE, et al. A quality improvement project to increase breast milk use in very low
birth weight infants. Pediatrics 2012;130(6):e1679.

57. Kuehn BM. Hospitals slash central line infections with program that empowers nurses. JAMA
2012;308(16):1617.

58. Kaplan HC, Lannon C, Walsh MC, et al. Ohio statewide quality-improvement collaborative to reduce late-
onset sepsis in preterm infants. Pediatrics 2011;127(3):427.

59. Mills PD, Weeks WB. Characteristics of successful quality improvement teams: lessons from five
collaborative projects in the VHA. Jt Comm J Qual Saf 2004;30(3):152.

60. Lannon CM, Peterson LE. Pediatric collaborative improvement networks: background and overview.
Pediatrics 2013;131(suppl 4):S189.

61. Schouten LM, Hulscher ME, van Everdingen JJ, et al. Evidence for the impact of quality improvement
collaboratives: systematic review. BMJ 2008; 336(7659):1491.

62. Lee HC, Powers RJ, Bennett MV, et al. Implementation methods for delivery room management: a quality
improvement comparison study. Pediatrics 2014;134:e1378.

63. Aspden P WJ, Bootman L, Cronenwett L. Preventing medication errors. 2007.


http://www.nap.edu/openbook.php?record_id=11623&page=Ri. Accessed December 8, 2013.

64. Klevens RM, Edwards JR, Richards CL Jr, et al. Estimating health care-associated infections and deaths
in U.S. hospitals, 2002. Public Health Rep 2007;122(2):160.

65. Chassin MR, Loeb JM. High-reliability health care: getting there from here. Milbank Q 2013;91(3):459.

66. Joint Commission Center for Transforming Healthcare. Robust process improvement.
http://www.centerfortransforminghealthcare.org/about/rpi.aspx. Accessed November 2013.

67. O'Connor E, Fiol CM. A roadmap for managing change: energizers, barriers and action steps. In: Lowery
JE, ed. Culture shift: a leader's guide in heath care. Chicago IL: American Hospital Publishing Inc., 1997:39.

68. Khatri N, Brown GD, Hicks LL. From a blame culture to a just culture in health care. Health Care Manage
Rev 2009;34(4):312.

69. Ohlinger J, Brown MS, Laudert S, et al. Development of potentially better practices for the neonatal
intensive care unit as a culture of collaboration: communication, accountability, respect, and empowerment.
Pediatrics 2003;111(4 Pt 2):e471.

70. Leonard M, Graham S, Bonacum D. The human factor: the critical importance of effective teamwork and
communication in providing safe care. Qual Saf Health Care 2004;13(suppl 1):i85.

71. Woolf SH. Patient safety is not enough: targeting quality improvements to optimize the health of the
population. Ann Intern Med 2004;140(1):33.
4
The Vulnerable Neonate and the Neonatal Intensive Care
Environment
Penny Glass

Technologic advances in care of the sick newborn infant have led to major improvement in mortality rates over
the past three decades, especially for the very-low-birth-weight newborn. However, morbidity among survivors
remains a significant and ongoing problem. Throughout early childhood, developmental and behavioral
differences exist between preterm and healthy full-term infants, even when matched for conceptional age. The
preterm infant often shows manifestations of altered brain organization, including disrupted sleep, difficult
temperament, hypo- and hypersensitivity to sensory input, prolonged attention to redundant information,
inattention to novel stimuli, and poor quality of motor function (1,2,3,4,5). These precursors of learning
disabilities and social/emotional problems at school age may occur in more than 50% of Neonatal Intensive Care
Unit (NICU) survivors and are not fully explained among preterm infants by either the severity of illness or the
subsequent home environment (2). Increasing evidence points to the vulnerability of the immature human brain to
pain and stress and to abnormal sensory and social environments. Altogether, this extensive knowledge base
supports the ongoing impetus to change the intensive care nursery experience for both the neonate and the
family.
In virtually every respect, the NICU is different from the prenatal environment of the fetus and from the home
environment of the full-term newborn. The birth of a newborn who requires intensive care is immediately followed
by physical separation from the mother and her cohesive protective role, impacting the mother, the infant, and
their relationship. The NICU experience includes frequent aversive procedures, excess handling, disturbance of
rest, noxious oral stimulation, noise, and bright light. These events and conditions are all sources of stress and
physiologic instability. Noxious stimuli disrupt sleep, which can also have biologic consequences for the neonate.
Even some medical complications commonly associated with prematurity per se, such as bronchopulmonary
dysplasia and necrotizing enterocolitis, may be, in part, stress-related diseases (6).
Appropriate sensory input is essential during maturation. The most vulnerable period occurs during rapid brain
growth and neuronal differentiation, which corresponds to 28 to 40 weeks of gestation for the human fetus
(7,8,9). Alterations in postnatal brain development have been well demonstrated in the preterm population. It is
assumed that for a fetus, the optimal environment is within the womb, and this environment changes as gestation
advances. Although it is not practical or feasible to replicate the womb, it provides a point of departure for
developing a better sensory, physical, and social environment in the NICU.
The purpose of this chapter is to lend a framework to developmental interventions in the NICU by summarizing
the maturation of each sensory system during fetal life and how this relates to the developing neonate in the
NICU environment.

NEONATAL SENSORY SYSTEMS


Maturation of all the sensory systems begins during the latter part of embryo gestation. The process is neither
unitary nor fixed, in that to some extent, sensory input drives maturation (10). The rate of maturation of each
sensory system varies, with the onset of function generally on the following order: tactile, vestibular, gustatory-
olfactory, auditory, and visual (11). These sensory systems are also interrelated in a hierarchical manner
stimulation of early maturing senses (e.g., tactile, vestibular) has a positive influence on development of later-
maturing ones (e.g., visual) (12). Recent research also indicates that untimely, or precocious, stimulation (e.g.,
visual) may disrupt the normal maturational process of another sensory system (e.g., auditory) (Philbin MK,
Personal communication, 1998).

Tactile System
It is widely known that cortical representation of tactile stimuli is somatotopic and contralateral to the stimulated
side. It is also important to note that increased stimulation to an area of the body can alter the pattern of
representation in the sensory cortex. Tactile hypersensitivity, or tactile defensive behavior, is described in clinical
reports of children with developmental delay, many of whom were born preterm. It is also seen in infants and
children who otherwise appear normal. It appears as an overreaction to touch, generally the hands or oral-facial
regions. With oral hypersensitivity, the infant may withdraw, gag, or retch when touched, even around the
outside of the mouth. Some children are intolerant of normal clothing and even may avoid body contact.
Hypersensitivity and hyposensitivity adversely affect parent-infant bonding.
The tactile sense, like the vestibular system, develops very early in fetal life. Receptor cells are present in the
perioral region by 8 weeks postconception and spread to all skin and mucosal surfaces by 20 weeks. Response
to stroking the lip region occurs first (8 weeks) (13) followed by stimulation of the palmar surface, with most of the
body sensitive to touch as early as 15 weeks. The somatosensory cortical pathway is intact by 20 to 24 weeks.
Tactile threshold is very low in the preterm infant, increasing by term. Prior to 30 weeks of gestation, a preterm
infant responds with leg withdrawal to a pressure that is 1/3 of the pressure necessary to elicit the same
response in a term infant (14). An important, qualitative shift in tactile sensitivity occurs around 32 weeks
postconception. Younger preterm infants respond to repeated stimulation with sensitization and diffuse
behavioral response, whereas after 32 weeks, infants show habituation to the same stimuli.
Classic studies (15) have demonstrated the profound importance of contact comfort for normal development. In
parallel fashion, preterm infants seek and maintain contact with a physical object in their incubator, and more so
if the tactile source contains rhythmic stimulation (16).

Early Fetal Experience


The fetus is housed in a thermoneutral, fluid-filled space that is a source of cutaneous input from the entire body
surface. Fetal movement provides tactile self-stimulation and, perhaps even more importantly, often evokes a
contingent maternal response. As term approaches and the intrauterine space becomes more constraining, the
normal posture of flexion facilitates hand-mouth, skin-to-skin, and body-on-body tactile feedback. The effect is
progressive, gradually changing throughout gestation. After a normal term birth, a ventral-to-ventral position is
preferred by both mother and infant, with touch followed by slow stroking (17). Traditionally, the infant is then
swaddled and held. Human proximity is universal under normal circumstances.

Touch and Handling in the NICU


The type and frequency of tactile stimulation imposed on a sick newborn in the NICU would be overwhelming
even for a healthy adult. They may be handled by more than 10 different nurses in the course of a month, in
addition to physicians, occupational or physical therapists, laboratory and x-ray technicians, and the
P.37
parents. Handling occurs more often among the sickest infants, typically is related to procedures, generally is
disturbing, and often is painful. Sleep has important biologic and immunologic consequences (18,19) and may be
disrupted by frequent intrusions. In addition, excess handling may have other negative physiologic
consequences, such as effects on blood pressure, cerebral blood flow, and oxygen saturation.
More benign manipulations, such as those that occur during neurodevelopmental assessment, are associated
with elevated cortisol levels (20,21). It is not clear whether this is a response to the assessment itself or to stress
associated with crying, but handling per se appears stressful even for a stable preterm infant.

Tactile Intervention in the NICU


The two general approaches to tactile intervention in the NICU provide either a reduction of general handling or
a provision of planned touch experiences. Touch may be pressure alone from an adult's open palm at rest, or
may include stroking. The neonates may be acutely ill or medically stable. These distinctions are important.
As part of an individualized approach to developmental care of acutely ill preterm infants, Als and colleagues (22)
provided a model of minimal handling and clustering of routine procedures, in addition to positioning devices and
techniques to improve postural flexion. Their approach triggered a significant change in the role of the nurse, to
that of a caregiver.
More specific to the tactile modality, Jay (23) evaluated the effects of gentle touch for 12-minute periods four
times a day on acutely ill preterm infants. This intervention consisted of hands on contact, but no stroking or
manipulating, and was associated with a lower required fraction of inspired oxygen (FIO2) after 5 days compared
to a similar nonintervention group.
A different approach has been successful employed by Field et al., and Scafidi et al., with preterm infants who
were beyond their acute phase, stable, and growing (24,25). The treatment involved infant massage (i.e.,
stroking and passive limb movement). Compared to nonintervention groups, the massaged infants showed
greater weight gain (on the same amount of formula), spent more time awake, showed better performance on the
Brazelton neonatal assessment, were discharged home 6 days sooner, and had better performance on
developmental assessment at 8 months past term.
Although the overall effects of the tactile interventions have been positive, the response of individual infants is
variable, and the timing of the intervention may be less than optimal when protocols are used. An unintended
response, such as oxygen desaturation, may occur during the intervention or afterward, and may not be
adequately monitored. Individual differences also exist among caregivers. Teaching the parent a gradual
approach, which begins early with steady/hands-on, is likely to have additional benefit in terms of bonding and
helps to encourage the parenting role during visits.
Soft swaddling and soft clothing provide tactile input in a more sustained fashion and are more widely accepted.
Early swaddling should provide a loose boundary for the limbs, rather than the more traditional firm swaddling.
The use of swaddling in the NICU is necessary if the parent is to retain that option at home as a means of
calming and supporting sleep. Swaddling is difficult to reinitiate after a period when the infant is unswaddled.
Once discharged home, NICU parents are apt to place their baby prone to sleep because they sleep longer, in
spite of the Back to Sleep teaching and literature that is made available.

Nonnutritive Sucking
Nonnutritive sucking (NNS) is an important oral-tactile intervention that supports both feeding and behavioral
regulation. It represents an early endogenous rhythm and manifestation of sensorimotor integration (26), is
reported to occur in the fetus (27), and is observed in the preterm newborn prior to 28 weeks of gestation. The
number of sucks per burst increases with maturation, whereas the duration of burst remains fairly stable.
NNS experience may facilitate important physiologic and behavioral mechanisms that potentially reduce cost of
care (28,29). It has a positive effect during gavage feeding by improving gastrointestinal transit time, improving
suck pressure and number of sucks per burst, and decreasing sporadic sucks. NNS is associated with earlier
onset of bottle feeding, better weight gain, and shorter hospital stays. However, having a pacifier continuously
available may not be beneficial and may encourage inappropriate sucking patterns, particularly in the chronically
ill neonate.
NNS also serves as a behavioral organizer by increasing quiet/alert state and decreasing motor activity, which in
turn facilitates social interaction. In addition, NNS dampens an infant's behavioral response, but not the cortisol
response, after a painful procedure (i.e., heel stick, circumcision) (20,30,31). It is noteworthy, however, that
sucking on a pacifier before and during repeated painful procedures may be inappropriate, because aversive
conditioning to the pacifier could result. This cautionary note is also included in recent studies showing a similar
decreased behavioral response to heel stick during nursing.

Vestibular System
The vestibular system responds to movement and directional changes in gravity. It is situated in the nonauditory
labyrinth of the inner ear and connects to the cerebellum, rather than the cortex. Lack of normal vestibular
stimulation in a developing organism is thought to affect general neurobehavioral organization (12).
Initial vestibular development is concurrent with auditory development, emanating from the same otocyst early in
gestation. The three semicircular canals reach morphologic maturity by 14 weeks postconception and full size by
week 20 (10). Response to vestibular stimulation has been observed by 25 weeks (32). The traditional vertex
presentation of the fetus at term is thought to result from fetal activity induced in response to vestibular input.

Intrauterine Vestibular Experience


The fetus experiences both contingent and noncontingent vestibular stimulation, which varies during gestation.
Fetal movement is first reported by mothers around 16 weeks postconception. After 28 weeks of gestation, with
the decrease in the relative amount of amniotic fluid, the movement of the fetus becomes constrained by the
more limited physical space. Vestibular experience is thereafter less contingent upon self-activation and more
related to normal maternal activity and position change, which often occurs in response to fetal activity. In
general, maternal activity level slows as parturition approaches.
After birth, the infant normally is held. Movement is slow, and change of position is gradual, even in the arms of
an experienced parent. Thereafter, the infant experiences multiple episodes of slow movement in space and
changes of position with each feeding/diapering episode, or when carried from room to room.
Vestibular stimulation per se is typically used to influence statemoving upright to increase arousal, or
monotonous side-to-side rocking and parental pacing to induce sleep. More vigorous position changes are
usually initiated when an infant is crying. Stimulation in this manner is highly contingent on the infant's behavior
and response, with variation in rate, rhythm, and duration.

Vestibular Experience in the NICU


In the NICU, the infant is typically nursed on a stationary surface, thus vestibular stimulation is limited to efficient
manipulation or turning of the neonate by the caregiver and clearly lacks any of the temporal qualities or
contingencies that the fetal or typical postnatal environment would have provided. Spontaneous limb
P.38
movement is generally diffuse, often unrestricted, and typically disorganizing in its effect. Infants requiring
mechanical ventilation experience even less position change.

Vestibular Intervention in the NICU


Like the tactile sense, the early development of the vestibular system provides a theoretical basis for primary
intervention within the NICU environment, but implementation has varied. Initial studies supported the use of an
oscillating waterbed or air mattress to reduce apnea of prematurity, improve sleep state organization/alertness,
decrease irritability, enhance motor behaviors, and improve somatic growth (33,34,35,36). Importantly, these
studies were limited to medically stable preterm infants. A subsequent clinical trial (37) failed to confirm a
reduction in apneic episodes or changes in neurobehavioral responses; however, the study cohorts included
infants requiring ventilator support. More recent findings have demonstrated a positive effect of vestibular
stimulation on apnea and oxygen saturation (38). Thus, it would seem reasonable to consider a trial on an
oscillating water bed/air mattress, for an otherwise stable infant with apnea/oxygen desaturations, before
assuming that pharmacologic intervention is required.
Other sources of vestibular stimulation, such as swings, hammocks, and rocking/vibrating seats, have not been
investigated formally. A rocking chair for an adult belongs at each infant's bedside. Swings are questionable,
given the excessive upright position of the baby and the standard rate of oscillation (too fast and invariant),
especially for an infant with a feeding issue. A crib has been devised that provides controlled motion that is
designed to be similar to a woman in late pregnancy walking. The rate of oscillation of this crib appears too rapid
for a preterm infant, but the device has shown some effect in modulating fussiness in full-term infants. The
duration of the motion may be individually controlled and proportionally reduced over time (39).

Positioning
The physical position of an infant is a part of the NICU tactile-vestibular experience. In the past, nursing care for
sick infants was routinely provided with the infant in supine position and exposed, thus simplifying management,
but not optimal for the infant. More recently, management in supine has incorporated small devices to reduce the
shoulder and hip abduction effects and to incorporate more natural upper and lower limb flexion, which may have
long-term benefits (40). This nesting approach has the added effect of visually improving the physical
appearance of the infant, an important benefit for both parents and staff.
Prone positioning in the NICU has been strongly supported physiologically, with resulting improved gastric
emptying, more quiet sleep, and less crying, in addition to higher PAO2 (41,42,43,44). The evidence suggests
that, when possible, the infant with respiratory compromise should be nursed in a prone position, with a slight
elevation of the head of the bed. Elevation of the head is particularly important if enteral feedings have been
initiated.
The dilemma is that, due to the increased risk for sudden infant death syndrome (SIDS), the prone sleep position
is contrary to the recommendation by the American Academy of Pediatrics (AAP). Parents are highly influenced
by the manner in which their infant is cared for in the NICU. In spite of the information provided to them prior to
NICU discharge about Back to Sleep, they are apt to continue to place their infant prone to sleep once home,
stating that the baby sleeps better.

Kangaroo Care
Kangaroo care, which evolved primarily in South America in an effort to discharge small or near term infants
earlier from hospital, is widely used in Level III NICUs. Traditionally, the infant is clad only in a diaper and
nested, upright, under the mother's clothing between her breasts, remaining there according to the mother's
comfort and the infant's physiologic stability, and feeding on demand. The technique provides familiar,
multimodal stimulation: tactile, vestibular, proprioceptive, olfactory, taste, and auditory. Kangaroo care appears to
be safe for medically stable larger preterm or full-term infants, in whom it demonstrates the greatest benefits in
the facilitation and maintenance of lactation and enhancement of the maternal sense of competency.
More caution should be used with infants who are less than 32 weeks of postconceptual age (PCA) or still
requiring mechanical ventilation. The primary consideration should be whether the increased stimulation and
additional handling might overstress the immature or sick infant. It is important to note that the vast majority of
research reporting the benefits of skin-to-skin contact did not use other forms of holding/cuddling by a parent as
a comparison, but compared infants receiving kangaroo care to infants lying stationary in an incubator or crib
(45).
Successful kangaroo experience by the mother in the NICU also has a major caveat. Clinical reports have shown
that it may encourage prone sleeping on the mother's chest after discharge home (exhausted parents will tend to
also fall asleep), contrary to APA recommendations regarding SIDS prevention.

Chemical Senses
Chemoreceptors include taste and olfaction. Taste receptors are in the taste buds, which are located primarily in
the papillae of the tongue but are also found on the soft palate and epiglottis (10,46). Taste stimuli (i.e., sweet,
sour, bitter, salt) transmit to the brainstem, and the hypothalamus. Cortical regions are involved in learned taste
preferences. Olfactory receptors are located in the lining of the olfactory epithelium in the posterior portion of the
nasal passage. The afferent pathway has no cortical projection area but connects directly to the limbic system.
Olfaction also is an integral part of infant/maternal attachment and may be as important to maternal bonding with
her infant as it is in reverse (47).
Chemoreceptors are well developed within the first trimester (10,46). Taste buds appear around 8 to 9 weeks,
and receptors are present by 16 weeks, increasing by term to adult levels. Taste discrimination at term is
sufficiently sensitive to detect a 0.1 mol/L concentration of NaCl in water (48). Full-term neonates, even
anencephalic infants, demonstrate reliably discriminable facial expressions to sweet, bitter, sour, and salt (49). In
a behavior described as savoring, normal newborns discriminate between different concentrations of sucrose
and even among various sugars (48).
In the fetus, taste receptors are functional by 34 weeks, with a differential behavioral response to distinct tastes
injected into the amniotic fluid: increased swallowing with sweeter taste and decreased swallowing with bitter
taste (10). By 30 weeks of gestation, preterm infants show stronger sucking in response to glucose, compared to
plain water (50). Behavioral response to formula, or breast milk, administered to the tip of the tongue has been
documented in preterm infants prior to 28 weeks of gestation (Zorc L, Unpublished doctoral dissertation, 2000).
Stimulation of taste receptors has important implications for early feeding and behavioral regulation. Smotherman
and Robinson (51) hypothesized that tastes of milk activate a centrally mediated endogenous opioid system in
newborn term infants, consistent with their animal model. Thus, in normal development, the mechanism to
support early feeding might very well extend beyond maintenance of chemical or caloric balance and become
feeding to thrive.
The human olfactory system is composed of four anatomically distinct but integrated subsystems, each of which
differentiate very early in gestation and are nearly mature prior to term birth (10,52). Epithelia in the nasal
cavities are linked to the olfactory bulbs and then to the ventral wall of the forebrain. Functional onset of human
olfaction is presumed to occur prenatally, having been demonstrated in the rat model. Rat fetuses exposed to a
specific
P.39
odorant (citral) in the amniotic fluid will selectively attach postnatally to the nipple of the same scent (53). Human
prenatal olfactory function is inferred from the sophistication present by term, including behavioral discrimination
and preference for the odor of their mother. Infants less than 12 hours old exhibit different facial expressions, in
response to pleasant or aversive odors, which are discriminable by adults (49). By 28 to 32 weeks of gestation,
the majority of preterm infants show reliable behavioral response to olfactory input. Finally, repeated exposure to
a novel odor can lead to preference for that odor (47).

Orogustatory Disorders
Feeding disorders are reported commonly among preterm infants, particularly those with chronic lung disease
and infants with central nervous system (CNS) damage. The cause in preterm infants is generally attributed to
frequent stressful procedures around the mouth (such as suctioning) as well as poor coordination of suck and
swallow. However, the orogustatory environment in the NICU deviates markedly from that in utero. No studies
have attempted to identify whether deficits in taste or smell are present in infants with feeding disorders;
however, it seems reasonable that in the NICU, consideration be given to the potential impact of highly odorous
substances, such as some skin adhesives, and agents used for environmental cleaning.

Intrauterine Experience
The amniotic fluid is a complex solution that changes in chemical composition during the same gestational period
when orogustatory maturation is taking place (10,52,54). As early as 18 weeks of gestation, more than 120
compounds have been identified in single amniotic fluid samples. The mother contributes to the chemical content
of amniotic fluid through her hormones and even the types of food she consumes. The fetus contributes to the
chemical status through urination and oral mucosal and lung secretions. More directly, fetal respiratory
movements, sucking, and swallowing cause pulsatile displacement of the amniotic fluid in contact with their
chemoreceptors so that receptor cells habituate less readily (52). A link has been proposed between the
intrauterine orogustatory experience and the selective behavior preference of the newborn for their mother's
breast milk, that is, the fetus is learning the smell and taste of its mother.

Orogustatory Experience in the NICU


The NICU environment has not been described in terms of gustatory or olfactory content, but it clearly is not well
adapted to this important developmental process. The necessary restriction of oral feeding in sick or extremely
premature neonates presents a marked deviation from the rich fetal orogustatory environment. The chemical
composition of initial feedings (breast milk or formula) is different from amniotic fluid or colostrum. Common
changes in formula composition (brand) concentration (dilute or hypercaloric), and temperature (too cool)
introduce unintended variability. The addition of noxious oral medications and electrolyte supplements to the
feeding typically activate a gag response in the newborn. Negative experiences, temporally associated with
feeding, can lead to aversive conditioning. Finally, in contrast to the healthy full-term neonate, sick full-term and
preterm infants in the NICU are exposed to multiple caregivers and lack the stable olfactory source that is
provided by sustained body contact with a consistent caregiver at home.

Orogustatory Intervention in the NICU


For any newborn, especially if medically fragile, the presence of the mother can be partially maintained when she
needs to leave the bedside, by having her provide a breast pad to be placed near the infant's head. The impact
on infant is not known but is unlikely to be negative. The important impact on the mother is her recognition that,
even under circumstances of duress, she is able to provide something special for her baby that no one else can.
Other direct stimulation of smell and taste in the NICU also has significant implications for enhancing care. For
example, a period of familiarization with the odor of breast milk or formula, before oral introduction, can facilitate
acquisition of oral feeding skills (47). Most infants who are restricted from oral feeds can safely tolerate a small
drop of breast milk (or formula) on the lips or tongue tip. Small tastes of formula or (ideally) breast milk before the
introduction of the nipple may foster behavioral organization and facilitate the onset of feeding (54). The limited
research available suggests that gut priming in the extremely preterm or sick full-term infant probably should
not bypass the mouth entirely. In addition, animal research suggests that surfactant and colostrum may have
previously unsuspected roles in the initiation of human feeding (51).
The taste of sucrose, usually by a nipple, administered prior to pain-associated procedures has been extensively
studied and has become widely accepted in neonatal care units (55), although the mechanism of action is not
clear. The reported effect has been primarily on damping the behavioral response to pain, with less effect on
physiologic measures of pain (e.g., heart rate, oxygen saturation, cortisol level). The long-term effects of this
early and frequently used taste intervention have not been studied.
Auditory System
Development of the auditory system begins around 3 to 6 weeks of gestation, and by 25 weeks, all the major
structures of the ear are essentially in place (56,57). The cochlear nucleus has differentiated sufficiently to be
functional, although microscopically the cochlea is still not mature, even at term. Frequency-specific hair cells of
the basilar membrane are organized tonotopically (different sound frequencies cause the basilar membrane to
vibrate at different locations; hence, the frequencies of auditory stimuli are encoded topographically on the
auditory sensory organ; this is referred to as the tonotopic map) and are systematically shifting their location
during this early period (56), likely in response to stimulation. The afferent pathway from the cochlea to the
auditory cortex is complete, and myelination of the auditory pathway is present.
Functionally, both cortical auditory evoked responses and brainstem auditory evoked responses can be elicited
by 25 to 28 weeks (58,59); however, wave morphology is different from that recorded in the full-term infant, and
the latency is prolonged. A blink response to vibroacoustic stimulation has been obtained in human fetuses of 24
to 25 weeks of gestation. A more complex behavioral response to sound occurs by 28 weeks, but readily
fatigues. The maximum rate of electrophysiologic change occurs in the cortical auditory evoked response and
brainstem auditory evoked response between 28 and 34 weeks of gestation, and this is likely to be a period of
increased vulnerability. Orienting behavior toward soft sound can also be elicited by 34 weeks.
Maturation of the fetal auditory system is also marked by an increase in spectral sensitivity and decrease in
auditory threshold (56,60,61). Auditory thresholds in a preterm infant at 25 weeks of gestation are approximately
65 dB, compared to 25 dB at term. The range of auditory sensitivity by the third trimester is fairly restricted, 500
to 1,000 Hz, compared to 500 to 4,000 Hz at term and 30 to 20,000 Hz by adulthood.
Evidence for a functional auditory system in the fetus is strong. Specific anatomic sites are present in the cortex
that are responsible for processing complex sounds, such as language. A biologic predisposition to respond to
the specific acoustic patterns of speech is already present in full-term neonates. For example, they have lower
thresholds for sound within the most important range for speech perception (i.e., 500 to 3,000 Hz) (62). Within
this frequency range, they respond differently to speech and nonspeech stimuli. There are even hemispheric
differences in auditory evoked potentials that support this language sensitivity (63).
P.40
Finally, healthy full-term neonates demonstrate a preference for sounds that they were exposed to in utero.
Research shows that 2- to 4-day-old neonates prefer their mother's voice, compared to another female voice,
and prefer a recording of a story read by their mother prenatally, to a recording of a story read by their mother
that they were not exposed to prenatally (64,65,66,67,68).

Auditory Deficits
Preterm and sick full-term infants are at increased risk for sensorineural hearing loss (particularly high frequency)
and developmental language disorders (8). Language disorders may be receptive or expressive dysfunctions.
Receptive language disorders are often referred to as auditory processing deficits. These deficits primarily
include phonemic-based disorders that involve discrimination between speech sounds, such as ba versus pa,
short-term memory deficits, and difficulty in interpreting the meaning of words implied by grammatical structure.
Expressive language problems may include disorders of speech (as in articulation or fluency), wordfinding
difficulty, and deficient or disordered sentence structure. Disorders result from direct damage to central brain
structures or can be incidental to more general brain dysfunction. They occur in children with normal hearing
thresholds and otherwise normal intelligence. They occur more commonly among NICU graduates and are not
directly related to severity of illness (69).

Intrauterine Experience
The auditory system during fetal life is developing within an environment that includes rhythmic, structured, and
patterned sound emanating predominantly from the mother. Internal sounds include maternal respirations,
borborygmi, placental and heart rhythms, and the like. Maternal speech transmits both externally and internally.
Prosody (i.e., intonation rhythm, stress) is probably the most salient aspect of speech available to the fetus. The
intensity of internally recorded sound within the amniotic fluid is approximately 70 to 85 dB, with a predominance
of low frequency (Fig. 4.1) (70). External sound also is transmitted to the fetus, but is attenuated by the time it
reaches the intrauterine cavity, more so at higher frequencies (i.e., 70 dB at 4,000 Hz), than lower frequencies
(i.e., 20 dB at 50 Hz) (71). Given these considerations, the fetus probably is minimally exposed to frequencies
lower than 1,000 Hz (72). The available frequencies in utero also parallel cochlear development (65).
The auditory environment in the womb likely provides the most appropriate substrate for normal development of
the sensory system. After a normal, full-term birth, the auditory environment is quiet by contrast to the intrauterine
environment. This may serve to increase the salience of the human voice, and the mother's voice in particular.

NICU Auditory Environment


The acoustic environment in the NICU differs from the intrauterine environment in peak intensity, spectral
characteristics, and pattern (Fig. 4.1). Ambient noise is generated by motors, fans, ventilator equipment,
personnel, alarms, hand-washing areas, trash lids, pagers, and intercoms, to name a few. The intensity of
background noise is around 50 to 60 dB and, therefore, not necessarily louder than in utero, but episodic bursts
of higher-intensity (even 100 dB) sound occur. The NICU experience varies depending on whether the infant is
in a private room versus an NICU with several infants per room, or in an incubator versus an open crib. The
incubator has the potential to attenuate high-frequency room noise but produces its own background noise in the
range of 50 dB. Overall, it would appear (Fig. 4.1) that speech input for the neonate is selectively masked by the
NICU auditory environment.
Exposure to aberrant noise levels in the NICU may cause sensory-neural damage, induce stress, and contribute
to language or auditory processing disorders in the preterm neonate. Schulte and colleagues (8) reported nearly
12% hearing loss in a follow-up of preterm infants. Damage to the outer hair cells of the cochlea of newborn
guinea pigs was reported after exposure to noise ranges similar in intensity and frequency to noise in the NICU
(73). In other research, the combination of noise and ototoxic drugs commonly given to sick preterm infants (e.g.,
aminoglycosides, diuretics) was found to have a potentiating effect on hearing loss (74,75,76). Data further
suggest that the immature cochlea may be most susceptible to damage during its final stages of anatomic
development and differentiation (76).
FIGURE 4.1 Comparison of the frequency-specific auditory environment of the fetus in utero and the
preterm infant in the neonatal intensive care unit. SPL, sound pressure level. From Walker D, Grimwade J,
Wood C. Intrauterine noise: a component of the fetal environment. Am J Obstet Gynecol 1970;109:91, with
permission; and Otho Boone, Personal communication, December 1992, with permission.

In addition to possible sensory nerve damage, loud noise could have physiologic consequences in the newborn
preterm infant in the form of stress, leading to alterations in corticosteroid levels and autonomic changes.
Decreases in oxygen saturation, increases in intracranial pressure, and peripheral vasoconstriction are reported
in preterm infants after exposure to sudden noise (77). Furthermore, sleep is disrupted.
Abnormal auditory environmental conditions could contribute to language problems. Auditory habituation patterns
were impaired in chicks reared in an NICU-like sound environment (78). Delayed cortical auditory evoked
responses among healthy preterm infants have been reported, in addition to deficits in brainstem response to
linguistic stimuli (69,79).

Auditory Intervention in the Neonatal Intensive Care Unit


Auditory interventions in the NICU include efforts both to reduce ambient noise and to introduce patterned
auditory input. Noise reduction protocols have been routinely put into place by NICU staff. Quiet times during
each shift are prevailing.
Evidence suggests the potential value of caring for the smallest and sickest preterm infant inside an incubator,
as a shield against environmental noise. However, initially, the steady incubator noise may induce sleep in the
infant (80), but this continuous white noise also masks more socially relevant auditory stimuli. Occluding
P.41
the infant's ears as a noise reduction procedure has been suggested (81), but it has the potential to do harm and
has not been adequately researched.
Auditory stimulation, as a single modality, has been studied in the preterm infant. Heartbeat sounds were shown
to lengthen the duration of the first quiet sleep period (80). Quiet sleep is a more stabilized state that reflects
CNS maturity. Some NICUs use sound as a protective window for the infantwhen music is played, the infant
will not be disturbed. This approach responds to the potential for conditioning in the preterm infant. Soothing
sounds are soft, simple, repetitive, and harmonic, with a limited dynamic range. Nonetheless, speech and
nonspeech stimuli differentially stimulate the cerebral hemispheres (63). Availability of speech sounds is likely to
be more critical for the infant than music, so any music should be turned off when anyone is present. The quiet
background encourages speech from nearby adults and also makes the speech sounds more audible to the
infant.
Healthy preterm infants (28 to 32 weeks of gestation) exposed to recorded speech until reaching 36 weeks PCA
showed improved auditory and visual responses, along with better motor development (82). This study highlights
once more that intervention in one sensory modality may affect another modality. Even so, recorded speech
lacks the essential social, contingent, and reciprocal nature of normal adult-infant communication, and therefore
should not take precedence over social interactions.
Finally, a lot of attention has been paid to studies highlighting the importance of reading to infants (83). In the
NICU, the reading experience may encourage parents to be more verbal around their baby, but the visual aspect
associated with the reading should be the parent's face, rather than pictures in the book.

Visual System
The eye, as an outgrowth of the brain, is said to be like a window to the brain, since it contains two thirds of the
afferent nerve fibers in the CNS. The amount of light necessary to stimulate a single photoreceptor cell in the
retina is extremely small1 quantum (84). Similarly, on a clear dark night, the adult eye can detect a single
candle flame 10 miles away. Representation in the cortex is topographic, but upside down and reversed.
By 24 weeks of gestation, gross anatomic structures of the eye are in place and the visual pathway is complete
from the retina to the cortex. A visual evoked response to bright light can be obtained, but it appears as a long
latency negative wave, which readily fatigues. Behavioral response to bright light consists of lid tightening, but
this also fatigues quickly. Pupillary reflex is absent.
Important functional changes occur at around 32 weeks of gestation. A bright light will cause immediate lid
closure, and the response sustains. The eyes may open spontaneously, and the infant may briefly fixate. This
has been described as the beginning of attention (85). Attention as such may be best elicited with a large, high-
contrast form held closer to the eyes than would be necessary at term, but under similar conditions of low
illumination (i.e., 5 footcandles).
By 36 weeks, alertness is less sustained than at term, but the infant now shows a spontaneous orientation
toward a soft light and can track an object horizontally and vertically. As does the full-term infant, the 36-week
preterm infant shows a preference for a patterned versus a nonpatterned surface.
Relative to other sensory systems, the visual system is the least mature by term birth, with considerable
development continuing over the next 6 months (86). Newborns are photophobic; thus, visual attention is
facilitated under low illumination, that is, approximately 5 footcandles (ft-c).
In spite of acuity estimates in the range of 20/200 Snellen equivalents, the term newborn can attend to form,
object, and face. Specifically, he or she can fixate on relatively fine detail (a 0.2-cm black line on a white
background at a distance of 30 cm) and can show preference for patterns along dimensions of brightness and
complexity (87). Attention to the human face can be partially explained as a predisposition to contrast (e.g., eyes,
open mouth) or to edge (e.g., hairline), to slow movement (e.g., nodding), and to contingent stimulation (e.g.,
adult's soft voice).

Visual Deficits
It is generally agreed that the visual system of the preterm infant is particularly susceptible to insult. Risk for any
visual disorder is inversely related to gestational age. The most well-known visual disorder is retinopathy of
prematurity (ROP), a proliferative vascular disease of multifactorial origin most strongly associated with degree of
immaturity of the retina (88,89,90,91). Other disorders of the eye (i.e., poorer acuity, high myopia, strabismus,
and blue-yellow color deficits) are also more common among children born preterm (92,93,94).
In addition, children born preterm are at higher risk for visual information processing problems, which are more
cortically mediated. Poorer performance on tests of visual attention, visual pattern discrimination, visual
recognition memory, and visual motor integration repeatedly indicates particular vulnerability among infants and
children born preterm (1,4,5,44,95).
Parents of NICU graduates commonly report their infant's fascination with ceiling lights or electronic sources of
light at home; in preference to looking at their parent's face.

Intrauterine Environment
The womb generally is dark, but under certain conditions, light can transmit to the fetus. A behavioral response
by a fetus to light has been described (96). Transmission through the tissue layers is limited to small amounts of
red, or long-wavelength, light. Probably only 2% of incident light reaches the interior of the uterus (Sliney D,
Personal communication, 1992). It is unlikely that light exposure is a necessary condition for the fetus, or that
periodic exposure to low levels of long-wavelength light is harmful. Aspects of the light-dark cycle that reach the
fetus are probably mediated more by maternal responses such as rest-activity cycles and hormones, than by
light directly.

NICU Environment
Ambient light in the NICU during the 1980s was reported to be high, in the general range of 30 to 150 ft-c, with
peaks of more than 1,500 ft-c from sunlight (97,98). Over the past two decades, ambient lighting has been
markedly reduced in most Level III nurseries with the recognition of both the potential photobiologic effects of
light (99,100,101,102,103,104,105,106,107,108,109,110) and the shift toward a general developmental
approach to NICU nursing care, spearheaded by Als and her colleagues (111).
For the individual infant, light exposure is affected by location in the room, open crib versus incubator,
size/location/compass direction of the windows, season of the year, and the weather (i.e., sunny vs. hazy). The
duration of exposure is generally 24 hours a day, but efforts are being made to incorporate dim/dark cycling.
Unlike open cribs, incubators are often shielded from overhead light with a cover. The physical position of the
infant assumes increased importance, because they may be facing a light source across the room, even though
shaded from overhead ambient light. The infant may be in prone position, in which case one eye may be
inadvertently occluded.
Intervention in the NICU: Light and Pattern
The optimal level of NICU lighting has not been determined, but light in an infant's face is stressful and
influences behavior, whether sleep or awake (109). Lower ambient light is associated with more mature sleep
state organization and improved eye opening/awake periods. Increased eye opening under dim light, generally in
the range of 5 ft-c, makes the infant more socially available to a parent
P.42
or other caregiver. Cycled lighting may be beneficial as the infant approaches discharge.
Ambient light levels may produce sensory overload. Under bright light, an infant will show an aversive response
to an auditory stimulus but will show an orienting response to the same auditory stimulus under a dimmer light
(112). This evidence has very important implications for successful parent/infant interaction.
Light source is particularly important when an infant is being held for feeding or social engagement. Positioning
the light source behind the infant may enhance the visibility of the parent's face. A light source placed behind the
parent's face tends to cast their face in a shadow, and the infant is visually drawn to the light.
Whether, or when, to provide patterned visual stimulation is particularly important. An infant's ability to respond to
a level of stimulation does not necessarily mean that he or she should be stimulated at that level. For example,
infants visually regard high-contrast black-and-white stimuli, compared to pastel, but there is no evidence to
indicate that high-intensity visual input is beneficial. For comparison, although infants can more easily hear a
louder sound, higher-intensity sound would not be advocated merely because the infant hears it better. Indeed,
animal research has shown that the nature of the visual environment influences the propensity of the visual
cortex to respond selectively to the nature of the visual input (i.e., spots of light, black/white grating).
Furthermore, longer attention to black/white checkerboard pattern in early infancy was associated with lower IQ
at 4 years of age (113).
Overall, the evidence suggests that optimal visual environment for neonates would provide low or dim ambient
light in the daytime and dimmer light at night, but would avoid spots of light such as overhead fixtures, monitors,
TV screens, mirrors, and light from crib mobiles. An object placed within the infant's visual field should be pastel,
and no more interesting than the parent's face. The base of a mobile should be at least 30 cm from an infant's
face and oriented over the tummy, rather than over the face. The infant should be able to choose to look, rather
than be compelled to look.

CONCLUSION
Multiple aspects of the NICU environment are potential sources of stress and overt damage to the brain of
the preterm infant or sick full-term infant. The well-established long-term goal of intact survival among NICU
survivors has acquired a broader meaning; rather than sensory, motor, and cognitive competence alone,
intact survival now also includes the child's social/emotional development and the relationship between the
parent/s and child.
Identifying for each individual infant the nature of the physical, sensory, and social environment, and how to
incorporate that into the overall plan of medical and nursing care, is an essential need for the infant and the
family. All infants at admission to a NICU should be provided protective care with a developmental care
plan, which includes efforts to modulate the impact of maternal separation. The plan should evolve over the
course of NICU stay and include repeated encouragement of parent participation and input.
A cogent example involves one of the most fundamental experiences in the NICU for the parent and infant,
the feeding process. In addition to quieting the visual and auditory input and providing optimal experiences
with olfaction (the mother), taste (breast milk), nonnutritive sucking, and even kangaroo care, there are
other experiences that may require special attention in individual infants. For example, it is noteworthy that
even a medically stable infant who requires NG tube feedings is less likely to be held routinely than an
infant feeding normally, especially if the feeds are continuous rather than bolus. Appropriate developmental
care includes being held, cuddled, and socially engaged in the manner of normal oral feeding by a familiar
adult: every 3 hours during the day and at least once over night, and for a duration similar to that
experienced by an infant feeding normally.
In addition, formal therapeutic interventions provided by Physical, Occupational, or Speech Therapy should
always involve an integrated care plan across disciplines and should routinely provide repeated modeling
and instruction for both the parents and the nurse/caregivers throughout the NICU stay. Goals and
objectives should be individualized and based on expectations for the infant's conceptional age and medical
stability, but also specifically address how the integrated therapeutic plan reduces sources of stress and
best supports the infant's feeding and social needs. This ongoing plan provides important details for
continuity of care after NICU discharge both for the parents and for community resources.
The modern NICU is a miracle, with its technology, staff, and ability to rescue partially developed fetuses
and repair many lethal defects. What is needed is a second miracle: a nurturing environment in the midst of
this apparent chaos, which is neither a uterus nor a warm, quiet nursery bedroom at home yet manages to
meet the needs of the sensitive, developing nervous system of the tiny patient, who becomes a baby, and
then a child with a lifetime ahead.

ACKNOWLEDGMENTS
I thank Ms. Teresa Bullen for substantial contributions to this revised chapter and Richard D. Walk, PhD, my
former mentor.

REFERENCES
1. Kopp C, Sigman M, Parmelee A, et al. Neurological organization and visual fixation in infants at 40 weeks
conceptional age. Dev Psychobiol 1975;8:165.

2. Parmelee AH, Sigman M. Development of visual behavior and neurological organization in pre-term and
full-term infants. In: Minnesota symposium on child psychology, vol. 10. Minneapolis, MN: University of
Minnesota Press, 1976:119.

3. Sostek AM, Quinn PO, Davitt MX. Behavior, development and neurologic status of premature and full term
infants with varying medical complications. In: Field TM, Sostek A, Goldberg S, et al., eds. Infants born at
risk. New York: Spectrum, 1979:281.

4. Caron A, Caron R. Processing of relational information as an index of infant risk. In: Friedman S, Sigman
M, eds. Preterm birth and psychological development. New York: Academic Press, 1981:219.

5. Rose SA. Enhancing visual recognition memory in preterm infants. Dev Psychol 1980;16:85.

6. Gorski PA. Developmental intervention during neonatal hospitalization. Pediatr Clin North Am
1991;38:1469.

7. Weisel TN, Hubel DH. Single cell response in striate cortex of kittens deprived of vision in one eye. J
Neurophysiol 1963;26:1003.

8. Schulte FJ, Stennert E, Wulbrand H, et al. The ontogeny of sensory perception in preterm infants. Eur J
Pediatr 1977;126:211.

9. Dobbing J. Later development of the brain and its vulnerability. In: Davis JA, Dobbing J, eds. Scientific
foundations of Paediatrics. London, UK: Heinemann, 1974:565.

10. Bradley RM, Mistretta CM. Fetal sensory receptors. Physiol Rev 1975; 55:352.

11. Gottlieb G. The psychobiological approach to developmental issues. In: Mussen PH, ed. Handbook of
child psychology, vol. II, 2nd ed. New York: John Wiley and Sons, 1983:1.

12. Turkewitz G, Kenny PA. The role of developmental limitations of sensory input on sensory/perceptual
organization. J Dev Behav Pediatr 1985;6:302.

13. Humphrey T. Correlation between appearance of human fetal reflexes and development of the nervous
system. Prog Brain Res 1964;4:93.

14. Fitzgerald M, Shaw A, MacIntosh N. Postnatal development of the cutaneous flexor reflex: comparative
study of preterm infants and newborn rat pups. Dev Med Child Neurol 1988;30:520.

15. Harlow H, Harlow M. The effects of rearing conditions on behavior. Bull Menninger Clin 1962;26:213.

16. Thoman EB, Ingersoll EW, Acebo C. Premature infants seek rhythmic stimulation, and the experience
facilitates neurobehavioral development. J Dev Behav Pediatr 1991;12:11.

17. Klaus MH, Kennell JH. Maternal-infant bonding. St. Louis, MO: CV Mosby, 1976.

18. Adam K, Oswald I. Sleep helps healing. BMJ 1984;289:1400.

19. Sassin JF, Parker DC, Mace JW, et al. Human growth hormone release: relation to slow-wave sleep and
sleep-waking cycles. Science 1969;165:513.

20. Gunnar MR. Reactivity of the hypothalmic-pituitary-adrenocortical system to stressors in normal infants
and children. Pediatrics 1992;90:491.

P.43

21. Kuhn CM, Schanberg SM, Field T, et al. Tactile-kinesthetic stimulation effects on sympathetic and
adrenocortical function in preterm infants. J Pediatr 1991;119:434.

22. Als H, Lawhon G, Brown E, et al. Individualized behavioral and environmental care for the very-low-birth-
weight preterm infant at high risk for bronchopulmonary dysplasia: neonatal intensive care unit and
developmental outcome. Pediatrics 1986;78:1123.
23. Jay S. The effects of gentle human touch on mechanically ventilated very short gestation infants. PhD
thesis. Pittsburgh, PA: University of Pittsburgh, 1982.

24. Field TM, Schanberg SM, Scafidi F, et al. Tactile/kinesthetic stimulation effects on preterm neonates.
Pediatrics 1986;77:654.

25. Scafidi FA, Field TM, Schanberg SM, et al. Massage stimulates growth in preterm infants: a replication.
Infant Behav Dev 1990;13:167.

26. Hack M, Estabecek M, Robertson S. Development of sucking rhythm in preterm infants. Early Hum Dev
1985;11:133.

27. Birnholz J, Stephens J, Faria M. Fetal movement patterns: a possible means of defining neurologic
developmental milestones in utero. AJR 1978;130:537.

28. Bernbaum JC, Pereira GR, Watkins JB, et al. Nonnutritive sucking during gavage feeding enhances
growth and maturation in premature infants. Pediatrics 1983;71:41.

29. Field T, Ignatoff E, Stringer S, et al. Nonnutritive sucking during tube feedings: effects on preterm
neonates in an intensive care unit. Pediatrics 1982;70:381.

30. Dixon S, Syder J, Holve R, et al. Behavioral effects of circumcision with and without anesthesia. J Dev
Behav Pediatr 1984;5:246.

31. Field T, Goldson E. Pacifying effects of nonnutritive sucking on term and preterm neonates during
heelstick procedures. Pediatrics 1984;74:1012.

32. Hooker D. The prenatal origin of behavior. New York: Hafner, 1969.

33. Korner AF. The use of waterbeds in the care of preterm infants. J Perinatol 1986;6:142.

34. Cordero L, Clark DL, Schott L. Effects of vestibular stimulation on sleep states in premature infants. Am J
Perinatol 1986;3:319.

35. Kramer LI, Pierpont ME. Rocking waterbeds and auditory stimuli to enhance growth of preterm infants. J
Pediatr 1976;88:297.

36. Pelletier JM, Short MA, Nelson DL. Immediate effects of waterbed flotation on approach and avoidance
behaviors of premature infants. In: Ottenbacher KJ, Short-DeGraff MA, eds. Vestibular processing
dysfunction in children. Binghamton, NY: Haworth Press, 1985:81.

37. Saigal S, Watts J, Campbell D. Randomized clinical trial of an oscillating air mattress in preterm infants:
effect on apnea, growth, and development. J Pediatr 1986;109:857.

38. Bloch-Salisbury E, Ludic P, Bednarek F, et al. Stabilizing immature breathing patterns of preterm infants
using stochastic mechanosensory stimulation. J Appl Physiol 2009;107(4):1017.

39. Gatts JD, Fernbach SA, Wallace HD, et al. Reducing crying and irritability in neonates using a continuous
controlled learning environment. J Perinatol 1995;15(3):215.

40. Grenier A. Prvention des dformations prcoches de hanche chez les nouveau-ns cerveau ls:
maladie de Little sans ciseaux? Ann Pediatr (Paris) 1988;35:423.

41. Yu VYH. Effect of body position on gastric emptying in the neonate. Arch Dis Child 1975;50:500.

42. Henderson-Smart DJ, Read DJ. Depression of intercostal and abdominal muscle activity and vulnerability
to asphyxia during active sleep in the newborn. In: Guilleminault C, Dement W, eds. Sleep apnea syndromes.
New York: Alan R. Liss, 1978:93.

43. Martin RJ, Herrell N, Rubin D, et al. Effect of supine and prone positions on arterial oxygen tension in the
preterm infant. Pediatrics 1979;63:528.

44. Wagaman MJ, Shutack JG, Moomijian AS, et al. The effects of different body positions on pulmonary
function in neonates recovering from respiratory disease. Pediatr Res 1978;12:571 (abstract).

45. Blois M. Birth: care of infant and mother: time sensitive issues. In: Gordon W, Trafton J, eds. Best
practices in the behavioral management of health from preconception to adolescence. Los Altos, CA:
Institute for Dis Manag, 2007:108.

46. Mistretta CM, Bradley RM. Development of the sense of taste. In: Blass EM, ed. Handbook of behavioral
neurobiology. Vol.8: developmental psychobiology and developmental neurobiology. New York: Plenum
Press, 1986:205.

47. Porter RH, Balogh RD, Makin JW. Olfactory influences on mother-infant interaction. In: Rovee-Collier C,
Lipsitt LP, eds. Advances in infancy research. Camden, NJ: Ablex, 1988:39.

48. Werner JS, Lipsitt LP. The infancy of human sensory systems. In: Gollin ES, ed. Developmental
plasticity: behavioral and biological aspects of variations in development. New York: Academic Press,
1981:35.

49. Steiner JE. Human facial expressions in response to taste and smell stimulation. Adv Child Dev Behav
1979;13:257.

50. Tatzer E, Schubert MT, Timischl W, et al. Discrimination of taste and preference for sweet in premature
babies. Early Hum Dev 1985;12:23.

51. Smotherman WP, Robinson SR. Milk as the proximal mechanism for behavioral change in the newborn.
Acta Paediatr Suppl 1994;397:64.

52. Schaal B, Orgeur P, Rognon C. Odor Sensing in the human fetus: anatomical, functional, and
chemoecological bases. In: Lecanuet J-P, Fifer WP, Krasnegor NA, et al., eds. Fetal development: a
psychobiological perspective. Hillsdale, NJ: Erlbaum, 1995:205.

53. Pedersen PE, Greer CA, Shepherd GM. Early development of olfactory function. In: Blass EM, ed.
Handbook of behavioral neurobiology. Vol.8: developmental psychobiology and developmental
neurobiology. New York: Plenum Press, 1986:163.

54. Smotherman WP, Robinson SR. Dimensions of fetal investigation. In: Smotherman WP, Robinson SR,
eds. Behavior of the fetus. Caldwell, NJ: Telford, 1988:19.

55. Harrison D, Beggs S, Stevens B. Sucrose for procedural pain management in infants. Pediatrics
2012;130:918.

56. Rubel EW. Auditory system development. In: Gottlieb G, Krasnegor N, eds. Measurement of audition and
vision in the first year of postnatal life: a methodological overview. Camden, NJ: Ablex, 1985:53.

57. Parmelee HP, Sigman MD. Perinatal brain development and behavior. In: Mussen PH, ed. Handbook of
child psychology, vol. II, 2nd ed. New York: John Wiley and Sons, 1983:95.

58. Birnholz JC, Benacerraf BR. The development of human fetal hearing. Science 1983;222:516.

59. Querleu D, Renard X, Boutteville C, et al. Hearing by the human fetus? Semin Perinatol 1989;13:409.

60. Aslin RN, Pisoni DB, Jusczyk PW. Auditory development and speech perception in infancy. In: Mussen
PH, ed. Handbook of child psychology, vol. II, 2nd ed. New York: John Wiley and Sons, 1983:573.

61. Hecox K. Electrophysiological correlates of human auditory development. In: Cohen LB, Salapatek P,
eds. Infant Perception: from sensation to cognition. Perception of space, speech, and sound, vol. II. New
York: Academic Press, 1975:151.

62. Berg KM, Smith M. Behavioral thresholds for tones during infancy. J Exp Child Psychol 1983;35:409.

63. Molfese D, Freeman R, Palermo D. Ontogeny of brain lateralization for speech and non-speech stimuli.
Brain Lang 1975;2:356.

64. Fifer W, Moon C. Psychobiology of newborn auditory preferences. Semin Perinatol 1989;13:430.

65. Fifer WP, Moon C. Auditory experience in the fetus. In: Smotherman WP, Robinson SR, eds. Behavior of
the fetus. Caldwell, NJ: Telford, 1988:175.

66. DeCasper AJ, Fifer WP. Of human bonding: newborns prefer their mothers' voices. Science
1980;208:1174.

67. DeCasper AJ, Spence MJ. Prenatal maternal speech influences on newborn's perception of speech
sounds. Infant Behav Dev 1986;9:133.
68. Spence M, DeCasper A. Newborns prefer a familiar story over an unfamiliar one. Infant Behav Dev
1987;10:133.

69. Kurtzberg D, Stapells DR, Wallace IF. Event-related potential assessment of auditory system integrity:
implications for language development. In: Vietze PM, Vaughan HG, eds. Early identification of infants with
developmental disabilities. Philadelphia, PA: Grune & Stratton, 1988:160.

70. Gerherdt K. Characteristics of the fetal sheep sound environment. Semin Perinatol 1989;13:362.

71. Armitage SE, Baldwin BA, Vince MA. The fetal sound of sheep. Science 1980;208:1174.

72. Walker D, Grimwade J, Wood C. Intrauterine noise: a component of the fetal environment. Am J Obstet
Gynecol 1970;109:91.

73. Douek E, Dodson HC, Bannister LH, et al. Effects of incubator noise on the cochlea of the newborn.
Lancet 1976;2:1110.

74. Falk SA. Combined effects of noise and ototoxic drug. Environ Health Perspect 1972;2:5.

75. Walton JP, Hendricks-Munoz K. Profile and stability of sensorineural hearing loss in persistent pulmonary
hypertension of the newborn. J Speech Hear Res 1991;34:1362.

76. Carlier E, Pujol R. Supra-normal sensitivity to ototoxic antibiotic of the developing rat cochlea. Arch
Otorhinolaryngol 1980;226:129.

77. Long JG, Lucey JF, Philip AGS. Noise and hypoxemia in the intensive care nursery. Pediatrics
1980;65:143.

78. Philbin MK, Ballweg DD, Gray L. The effect of an intensive care unit sound environment on the
development of habituation in healthy avian neonates. Dev Psychobiol 1994;27:11.

79. Salamy A, Mendelson T, Tooley WH, et al. Differential development of brainstem potentials in healthy
and high-risk infants. Science 1980;210:553.

80. Schmidt K, Rose SA, Bridger WH. Effect of heartbeat sound on the cardiac and behavioral
responsiveness to tactual stimulation in sleeping preterm infants. Dev Psychol 1980;16:175.

81. Zahr LK, de Traversay J. Premature infant responses to noise reduction by earmuffs: effects on
behavioral and physiologic measures. J Perinatol 1995;15:448.

P.44

82. Katz V. Auditory stimulation and developmental behavior of the premature infant. Nurs Res 1971;20:196.

83. Lariviere J, Rennick J. Parent picture-book reading to infants in the neonatal intensive care unit as an
intervention supporting parent-infant interaction and later book reading. J Dev Behav Pediatr 2011;32:146.

84. Gregory RL. Eye and brain: the psychology of seeing, 4th ed. Princeton, NJ: Princeton University Press,
1990.

85. Hack M, Mostow A, Miranda S. Development of attention in preterm infants. Pediatrics 1976;58:669.

86. Abramov I, Gordon J, Hendrickson A, et al. Light and the developing visual system. In: Marshall J, ed.
Vision and visual dysfunction. Boca Raton, FL: CRC Press, 1991.

87. James L, Lanman J. History of oxygen therapy and retrolental fibroplasia. Pediatrics 1976;57:590.

88. Lucey J, Dangman B. A reexamination of the role of oxygen in retrolental fibroplasia. Pediatrics
1984;73:82.

89. Johns KJ, Johns JA, Feman SS, et al. Retinopathy of prematurity in infants with cyanotic congenital heart
disease. Am J Dis Child 1991; 145:200.

90. Inder TE, Clemett RS, Austin NC, et al. High iron status in very-low-birth-weight infants is associated with
an increased risk of retinopathy of prematurity. J Pediatr 1997;131:541.

91. Fledelius T. Prematurity and the eye. Acta Ophthalmol 1976;128:3.

92. Hoyt C. Long-term visual effects of short-term binocular occlusion of at-risk neonates. Arch Ophthalmol
1980;98:1967.

93. Dobson V, Quinn GE, Abramov I, et al. Color vision measured with pseudoisochromatic plates at five-
and-a-half-years in eyes of children from the CRYO-ROP study. Invest Ophthalmol Vis Sci 1996;37:2467.

94. Miranda SB. Visual abilities and pattern preferences of premature infants and full-term neonates. J Exp
Child Psychol 1970;10:189.

95. Siegel L. The prediction of possible learning disabilities in preterm and full-term children. In: Field T,
Sostek A, eds. Infants born at risk: physiological, perceptual, and cognitive processes. New York: Grune &
Stratton, 1983:295.

96. Brazelton TB, Field TM. Introduction. In: Gunzenhauser N, ed. Advances in touch: new implications in
human development. Skillman, NJ: Johnson & Johnson Consumer Products, 1990:xiii.

97. Glass P. Light and the developing retina. Doc Ophthalmol 1990;74:195.

98. Landry RJ, Scheidt PC, Hammond RW. Ambient light and phototherapy conditions of eight neonatal care
units: a summary report. Pediatrics 1985;75:434.

99. Lanum J. The damaging effects of light on the retina: empirical findings, theoretical and practical
implications. Surv Ophthalmol 1978;22:221.

100. Stefansson E, Wolbarsht ML, Landers MB. In vivo O2 consumption in rhesus monkeys in light and dark.
Exp Eye Res 1983;37:251.

101. Glass P, Avery GB, Subramanian KN, et al. Effect of bright light in the hospital nursery on the incidence
of retinopathy of prematurity. N Engl J Med 1985;313:401.

102. Hommura S, Usuki Y, Takei K, et al. Ophthalmic care of very low birth-weight infants, report 4: clinical
studies of the influence of light on the incidence of ROP. Nippon Ganka Gakkai Zasshi 1988;92:456.

103. Reynolds JD, Hardy RJ, Kennedy KA, et al. Lack of efficacy of light reduction in preventing retinopathy
of prematurity. N Engl J Med 1998;338:1572.

104. Fielder AR, Robinson J, Shaw DE, et al. Light and retinopathy of prematurity: does retinal location offer
a clue? Pediatrics 1992;89:648.

105. Monos T, Rosen SD, Karplus M, et al. Fundus pigmentation in retinopathy of prematurity. Pediatrics
1996;97:343.

106. Bhatia J, Mims L, Roesel R. The effect of phototherapy on amino acid solutions containing
multivitamins. J Pediatr 1980;96:284.

107. Maurer H, Fratkin M, McWilliams N, et al. Effects of phototherapy on platelet counts in low-birthweight
infants and on platelet production and life span in rabbits. Pediatrics 1976;57:506.

108. Rosenfeld W, Sadhev S, Brunot V, et al. Phototherapy effect on the incidence of patent ductus
arteriosus in premature infants: prevention with chest shielding. Pediatrics 1986;78:10.

109. Glass P, Sostek A. Sleep organization in preterm infants: the effect of nursery illumination. Presented
at the International Conference of Infancy Studies (poster session), New York, April 21, 1984.

110. Glotzbach SF, Rowlett EA, Edgar DM, et al. Light variability in the modern neonatal nursery:
chronobiologic issues. Med Hypotheses 1993;41 (3):217.

111. Als H. Developmental care in the newborn intensive care unit. Curr Opin Pediatr 1998;10(2):138.

112. Haith MM. Rules that babies look by. Hillsdale, NJ: Erlbaum, 1980.

113. Sigman M, Cohen S, Beckwith L, et al. Infant attention in relation to intellectual abilities in childhood.
Dev Psychol 1986;22:788.
5
Neonatal Transport
Karen S. Wood
Elizabeth C. Turney
Carl L. Bose

HISTORY
Neonatal transport began in 1900 with the development of the first mobile incubator for premature infants by Dr.
Joseph DeLee of the Chicago Lying-In Hospital (1). This hand ambulance provided warmth while transporting
premature infants to the hospital following home birth. The development acknowledged the need to create a
controlled environment for the transport of infants that simulated the inpatient setting. In 1934, the first dedicated
neonatal transport vehicle in the United States was donated to the Chicago Department of Health by Dr. Martin
Couney (2), following the closure of the Chicago World's Fair where the vehicle was used to transport premature
babies to the exhibit. The first organized transport program in the United States began in 1948 with the
development of the New York Premature Infant Transport Service by the New York Department of Health in
conjunction with area hospitals (3,4). This remarkable system, created more than a decade before the evolution
of neonatal intensive care units (NICUs), incorporated many of the features of modern neonatal transport
programs, including around-the-clock staffing by specially trained nurses, dedicated vehicles, a clerk to receive
referral calls, and equipment designed specifically for neonatal transport. During a 2-year period, this program
transported 1,209 patients, of whom 194 weighed less than 1,000 g (4).
Neonatal transport took to the air in 1958 with the first fixed-wing transport of a newborn infant by the Colorado
Air National Guard (2). The 1967 flight of a premature baby to St. Francis Hospital in Peoria, Illinois, using the
Peoria Journal Star helicopter, marked the first rotor-wing neonatal transport (2). Routine use of air
transportation for neonatal patients began in 1972 with Flight for Life of Denver's St. Anthony Hospital (5).
Proliferation of organized transport programs occurred in the late 1970s, in conjunction with regionalization of
perinatal care. Regionalization initially minimized the number of infants requiring transport by promoting maternal-
fetal transport. Regionalization also shifted the responsibility for transporting infants born in community hospitals
to referral centers. Subsequently, the next decade saw improvements in perinatal mortality (6) and neonatal
morbidity (7) as the percentage of very-low-birth-weight (VLBW) infants delivered in referral centers increased.
Since the late 1980s patterns of referral dictated by schemes of regionalization have deteriorated in many areas
(8), coincident with an increase in community hospitals capable of providing some degree of neonatal intensive
care. As a result, increasing numbers of infants deliver at centers without subspecialists or the necessary
support services demanded by some VLBW infants. Community-based neonatal intensive care creates a need to
transport infants at a critical time in their illness, occasionally while receiving therapies such as high-frequency
ventilation or inhaled nitric oxide, which are not easily portable. Even in areas where regionalized perinatal care
persists and prenatal risk assessment is routine, unpredictable, emergent events may precipitate the delivery of
an infant in an unsuitable hospital. Collectively, these situations mandate increasingly sophisticated neonatal
transport systems.

ORGANIZATION AND ADMINISTRATION


Neonatal transport can be performed by either the community hospital referring the patient (one-way transport)
or the referral center receiving the patient (two-way transport). Two-way transport offers an economic
advantage, offers generally more skilled and experienced transport personnel (9), and may result in improved
survival (10,11). The American Academy of Pediatrics supports two-way transport (12), and in most perinatal
regions, referral centers have assumed this responsibility. The major disadvantage of two-way transport is the
time delay to get the transport team to the community hospital. In the remainder of this chapter, two-way
transport is discussed exclusively.

Administrative Personnel
The components of a transport program include those related to medical care and the nonmedical components
such as transportation, communications, finances, and marketing. The medical components must fall under the
direction of a physician who is credentialed to supervise the patients served by the program. Direction of the
nonmedical components often rests with a hospital administration staff person (Fig. 5.1). The following is a brief
discussion of each potential contributor to the administration of a transport program (13).

Hospital Administrator
Generally, a hospital administrator manages aspects of the program that are not directly related to patient care.
Many decisions regarding program operation require a cost-benefit analysis. While medical personnel are relied
on to provide an estimate of benefit, the hospital administrator must assess financial impact. Therefore, the
hospital administrator should be prepared to receive advice from medical personnel and develop the nonmedical
components of the program in consideration of the financial resources of the institution.

Medical Director
The medical director of a neonatal transport program is usually a neonatologist with expertise or a special
interest in transport. The medical director is ultimately responsible for the quality of care provided by the
transport team; this is particularly true if physicians do not participate directly in transport. The medical director
assumes responsibility for developing and updating training programs, equipment procurement, and treatment
protocols. The medical director, in conjunction with the coordinator of nonphysician personnel, must ensure that
all personnel have completed training requirements successfully and have satisfied the regulations of the
agencies that govern the various professional groups. The medical director also must develop and maintain a
system for reviewing the quality of care provided during transport.

Coordinator of Nonphysician Personnel


Each group of professionals (e.g., nurses, respiratory therapists, paramedics) on the transport team should have
a designated coordinator. The coordinator supervises the selection and training of personnel and develops
systems of peer review. Additional coordinator responsibilities include scheduling personnel, organizing
continuing medical education, ordering supplies and equipment, monitoring documentation standards, promoting
effective internal dynamics, and identifying team member needs. It is advisable to designate a single person to
coordinate team activities who will interface with the medical director and, in some programs, the hospital
administrator as well.

Consulting Neonatologists and Other Subspecialists


During the transport of a patient, it is important, and often mandated by state law, that a physician provides
consultation to the transport team. This physician, known as the medical control officer (MCO), is typically the
physician receiving the patient and often has already discussed the patient's care with the referring/community
physician
P.46
and made recommendations about interim management. Given this broad consultative role to both the
community physician and to the transport team, the MCO should be a person with extensive training, at a level in
excess of that available in the community hospital, such as a neonatologist, a trained pediatric subspecialist, or a
postdoctoral fellow. In addition, the MCO must be aware of the handicaps and hazards imposed by the transport
environment and must be familiar with the operational aspects of the program.

FIGURE 5.1 The administrative structure of a typical neonatal transport program.

The Advisory Board


A neonatal transport program should be considered an extension of the inpatient unit to which it delivers patients.
Therefore, the operation of the program should be reviewed periodically by representatives of all services
interfacing with the inpatient unit. These representatives, comprising the advisory board, might include the
following:
Medical director of the NICU
Neonatal division chief
Respiratory therapy administrator
Nursing administrator
Outreach education coordinator
Director of public relations
Representatives of community hospitals involved in transport
Parent representative
Advice should be solicited from the advisory board about all major program changes because of the impact these
changes may have on their respective services.

THE TRANSPORT TEAM


A variety of personnel participate in the inpatient care of infants, and all should be considered candidates for
caretakers during neonatal transport. These personnel include the following:

Neonatologists
Neonatal subspecialty residents (fellows)
General pediatric residents
Nurse practitioners
Transport nurses
Respiratory therapists
NICU staff nurses
Paramedics
The selection of the type of personnel used by each program is based on the unique aspects of that program;
however, some general principles apply that determine the relative desirability of various professionals. As the
number of transports increases, it becomes less practical to send physicians on transport. Neonatologists rarely
have the time to devote to frequent transports, and reimbursement is not sufficient to justify their presence.
Although participation in transport can be educational, in high-volume programs, time spent on transport by
pediatric residents and neonatal fellows competes with other aspects of training. In addition, the interest in
participation and expertise may vary considerably among trainees. This is a particular problem if participation is
mandated. Pediatric residents who participate in transport should be senior-level trainees under close
supervision.
Most high-volume programs choose to use nonphysician personnel as attendants during transport. The use of
neonatal nurse practitioners offers an attractive alternative to physician attendance (14,15). Nurse practitioners
are highly skilled in neonatal stabilization and care and provide a consistency of expertise not usually
encountered in other professional groups. They are licensed in most states to perform all of the diagnostic and
therapeutic procedures required during transport. The greatest disadvantages to the use of neonatal nurse
practitioners are scarcity of workforce in certain locations and cost. In addition, neonatal nurse practitioners are
rarely trained, or willing, to transport patients other than neonates.
As a cost-effective alternative to nurse practitioners, many centers train NICU staff nurses to participate in
transport. In addition, most states permit nurses to perform invasive procedures as an extension of their inpatient
nursing role under guidelines and protocols approved by the Boards of Nursing. Therefore, NICU staff nurses
can be trained to provide all the care required by a critically ill neonate during transport. This training often is
extensive, however, because the cognitive knowledge necessary to diagnose disorders and the experience to
perform invasive procedures must be mastered. This extensive training must be considered when estimating the
cost of using staff nurses as compared to nurse practitioners. The requirement of training is particularly
burdensome when there is a high personnel turnover rate.
Most patients transported to the NICU either have respiratory failure requiring mechanical ventilation or are
receiving supplemental oxygen. Respiratory therapists should be considered when selecting transport personnel
because of their expertise in the use and maintenance of respiratory care equipment. The therapists' ability to
adapt this equipment to the unique environment of transport can be lifesaving, particularly in circumstances when
unexpected events occur. The disadvantage of using therapists is the narrow focus of their usual training.
Further education and crosstraining allows their scope of practice to be expanded.
Eliminating physicians from attendance during transport can create problems that must be anticipated. For
example, leadership of the team is not defined by the usual medical model in which a physician assumes this
role. Designating one member of the transport team as the leader, accountable for communication, decision
making, and documentation can solve this problem.
P.47
Advisory personnel at the referral center, particularly physicians, often are unwilling to endorse a patient care
program that does not mandate initial evaluation by a physician. This resistance usually stems from a concern for
the well-being of the patient and can be overcome by the selection and training of competent non-physician
personnel. The support and endorsement of an involved medical director may also be critical. A similar attitude
may prevail in community hospitals. Community physicians may find it unacceptable to relinquish care of a critical
patient to nonphysician personnel. In an environment in which referral centers compete for patients, this may be
a motivation for maintaining physician attendance during transport. Most community physicians, however, are
concerned only with transferring their patients in a safe and timely fashion. Anecdotal experience, as well as
retrospective and prospective studies, suggests that properly selected and trained nurses provide a level of care
during transport that approximates the level provided by physicians (5,16,17,18). Once a nonphysician team
demonstrates competence and efficiency, the concerns of most community physicians vanish. Because the use
of specially trained nonphysician personnel represents both a safe and economic alternative to physician
participation in neonatal transport, most programs now rely on nonphysician personnel for patient care.
Transport personnel must be proficient in cognitive knowledge of neonatal diseases, management principles of
acute problems, and technical skills. The method and extent of training necessary to reach proficiency will
depend on the type of personnel; however, the pattern of preparation will be similar for all professionals (19).
Cognitive knowledge is best provided in didactic sessions in conjunction with self-study exercises. Management
principles may also be taught in a didactic setting, but refinement of these skills requires repeated experiences in
the inpatient setting. Laboratory simulation of technical skills and patient care scenarios is essential. These skills
can then be refined in the inpatient setting under supervision. Demonstration of proficiency in these areas should
be ensured by examination or observation by a qualified supervisor. After initial preparation, a period of training
should be provided, during which the trainee accompanies a more experienced team member on transport. Final
certification of competence should be awarded by both the medical director and the coordinator for the trainee's
professional group.

COMMUNICATION
The quality of the communication system that supports a transport program may be the key determinant of its
success. The communication system serves two basic functions: to provide a point of access for community
physicians, and to coordinate the activities of the transport team (20). A single call by the community physician
should provide access to all of the neonatal services of the referral center. The use of a transfer center or a toll-
free hot line, often associated with a memorable acronym, is favored by some institutions (21). Alternatively,
community physicians can call the NICU directly. If consultation is requested, the community physician should be
connected in a timely fashion with a consultant of appropriate training. If transfer is requested and deemed
appropriate, an available bed in the NICU of the referral center or an alternative should be identified. Bed
procurement and all subsequent details of the transport should then occur without additional calls by the
community physician.
Once the decision is made to transport the patient and an NICU referral center bed is located, the role of the
communication system shifts to dispatching the team and disseminating information regarding the transport. In
this role, the system is best served by a communication center that is staffed and equipped for emergency
medical service functions. The community hospital should be informed of the estimated time of arrival and of any
necessary preparations for the arrival of the vehicle. The receiving NICU should be notified and be provided with
medical information necessary for admission of the patient.
During the conduct of the transport, periodic communication between the dispatch center and the vehicle
operator is advisable. Unexpected delays or mishaps are identified promptly, and appropriate action is taken.
Most high-volume transport programs use satellite-tracking systems to monitor the movement of their transport
vehicles, which can be useful if diversion is necessary. When the transport team does not include a physician,
the team should have the capability of communicating directly with the consulting physician at all times. The
nurse practice acts in some states mandate this level of communication.
Communication capabilities are usually a trivial problem while the team is in the community hospital; however,
they can present a challenge during transit. The gravity of this problem declines each year with the evolution of
telecommunications equipment. Cellular phones are typically used during ground transport. VHF, UHF, and SHF
radios are utilized for communication in rotor-wing aircraft with air traffic control, medical control, and general
communications using separate frequencies. Fixed-wing aircraft generally use satellite communication systems.
Many communication centers are equipped with automated devices that record all communications. Although not
essential, the recorded transmissions may be valuable educational tools and aids in identifying system errors; in
addition, they are often critical for medicolegal questions.
Communication should not end with the conclusion of the transport. The transport team should contact both the
patient's family and the community facility to relate the events of the transport. The receiving physician should
update the community physician following admission and give further follow-up information at regular intervals,
including at the patient's discharge. This update should be expedited if an acute event occurs and should occur
immediately in the event of death. Failure to effectively communicate subsequent patient information remains one
of the most common criticisms of referral centers.

FINANCIAL CONSIDERATIONS
Subjecting a transport program to periodic cost-benefit analyses is a critical aspect of the program's operation.
The following elements should be included in the cost of operation:

1. Medical components
Personnel salaries/benefits
Salary support of the medical director
Equipment and supplies
Medication
Expenses related to education of personnel
2. Nonmedical components
Administrative overhead
Vehicle operation, maintenance, insurance
Communications
Educational and marketing material

Identifying the costs associated with the program may be difficult if its operation is financially integrated into the
operation of the NICU. For example, personnel costs often are difficult to quantify because, except in high-
volume programs, transport personnel usually contribute to inpatient services during transport duty time.
Therefore, the cost allocated to the transport program should be discounted based on this contribution. The
proportion of time devoted by the medical director is even more difficult to quantify and often ignored in the
financial analysis. The cost of equipment is usually separable from the cost of inpatient equipment as transport
equipment rarely is used for other purposes. Included in estimates of equipment costs should be allowances for
depreciation and maintenance.
The nonmedical components of a program are often more costly than the medical components because of
expenses related to transportation. This is particularly true when air transportation is used.
P.48
Sharing resources with other hospitals or agencies can minimize these expenses. Ground ambulances can be
shared with local emergency medical service agencies or be used for convalescent transport. Aircraft can be
used by a consortium of hospitals. The major disadvantage of the shared approach is the possible vehicle
unavailability at the time of transport request; however, the potential for this occasional conflict may be far
outweighed by the cost reduction.
The revenue of a transport program comes from three general sources: reimbursement, support from
governmental agencies, and support from other extramural organizations (22). Support from government and
charitable organizations are unusual in the United States, and hospitals are increasingly dependent on
reimbursement to support transport programs. Most third-party payers will reimburse the majority of the initial
transport as long as the care rendered at the referral center was unavailable at the community hospital.
Reimbursement for back transport is less consistent. In general, the cost of a transport program exceeds its
revenue. Subsistence of the program, therefore, depends on financial assistance from the sponsor hospital.
The decision to fund a transport program usually is based on a favorable cost-benefit analysis. Benefit can be
quantified by reductions in mortality, morbidity, and length of hospital stay. Among low-birth-weight (LBW) infants
with respiratory disease, it has been demonstrated that the services of a hospital-based neonatal transport team
reduce hypothermia and acidosis, the greatest prognostic indicators of mortality (10). However, little other
evidence supports the advantage of specialized neonatal transport teams. In an attempt to quantify the utility of a
neonatal transport program, the most prudent approach may be to scrutinize the type of patients requiring
transport to ensure potential benefit. These medical benefits should be combined with nonmedical benefits to the
institution, such as improved public relations and the recruitment of new patients. Ultimately, many institutions in
the United States elect to support a neonatal transport program, despite its financial disincentives, in order to
increase occupancy of NICU beds.
A potential economy for transport programs may be to combine services, either within a program or between
programs. An example of the former would be to cross-train members of specialty transport teams (e.g., pediatric,
neonatal, and adult) such that the total number of personnel can be reduced. This strategy invariably results in
some loss of expertise but may be necessary to ensure financial viability. Collaboration between programs may
include sharing vehicles or teams. Smaller institutions may benefit from outsourcing entirely by contracting with
larger medical centers for the provision of all transport services.

TECHNICAL ASPECTS
The Transport Environment
The principles of care provided during transport are the same as the principles of inpatient care. Any differences
in practice arise from the unique features of the transport environment (23). Many features, including excessive
noise, vibration, improper lighting, variable ambient temperature and humidity, changes in barometric pressure,
confined space, and limited support services, can create problems during transport. The impact of these
environmental factors relative to the mode of transportation is summarized in Figure 5.2.

Noise
High sound levels, in the range of 60 to 70 dB, are inherent to the NICU (24,25,26); but levels recorded on
transport are significantly higher, on the order of 90 to 110 dB (27,28). The effects of exposure to high sound
levels on the neonate are not known, but the possibility of physiologic changes is suggested by studies of
hospitalized infants (29,30). Brief exposure to high sound levels probably has little long-term effect on transport
personnel; however, repeated exposure over time may result in hearing loss. Personnel should protect
themselves from exposure by using sound-attenuating devices. Probably the most significant problem resulting
from high sound levels is the inability to use auscultation to assess the patient. This handicap must be
recognized before transport, and alternative methods for assessing heart rate and respiratory sufficiency must be
available during transport.

FIGURE 5.2 Environmental factors and their impact relative to different modes of transportation.

Vibration
Vibration exposure is a problem unique to the transport environment (28,31,32). The physiologic consequences
in patients of this exposure are not known. Animal studies and investigations using healthy adults suggest that
negative effects on the autonomic and central nervous system may occur (33,34,35). The vibrational effects on
transport personnel are potentially important. For example, a typical helicopter transport results in vibration
exposure associated with reduced personnel efficiency (36). The overt symptoms of motion sickness resulting
from low-frequency vibration may be incapacitating. A more subtle manifestation of motion sickness, termed the
sopite syndrome, also may affect transport team members (37,38). The symptoms associated with this syndrome
include drowsiness, inability to concentrate, and disinclination to communicate with others. The sopite syndrome
is common among crew members during transport, regardless of the mode of transportation (39). The impact on
patient care is poorly understood but may be significant.
The effect of vibration on equipment also poses a major problem. Monitor artifact is a common phenomenon.
Personnel should be familiar with monitor artifact and with the use of alternative monitoring techniques. The
selection of equipment should be made in consideration of resistance to the effects of vibration. Premature
failure of equipment secondary to vibrational damage should be anticipated, and preventive maintenance should
be on an accelerated schedule.

Poor Lighting
Improper lighting in transport vehicles is a common problem. The patient care compartment should have
illumination to 400 lx (40). In addition, high-intensity directional lighting (1,000 to 1,500 lx) should be available for
procedures. The eyes of the patient, as well as those of the driver or pilot, should be screened from these light
sources.

Heat Loss
The difficulties in maintaining a neutral thermal environment are accentuated during transport because of the
increased opportunities for heat loss. Hypothermia can be a significant problem during transport and has been
linked to increased mortality (10). Heat loss in the transport environment usually occurs by two mechanisms:
convection and radiation. Heat loss can be minimized by use of a doublewalled incubator, avoiding opening the
incubator unnecessarily, heating the transport vehicle, and creating barriers between the incubator wall and cold
surfaces.
P.49
Variable Humidity
Transport teams often elect not to humidify respiratory gases both for simplicity and to eliminate the negative
effects of water vapor pressure on infants in respiratory failure. This is a reasonable approach assuming short
transport times; however, long-term effects of poor humidification include dehydration and increased tenacity of
secretions. Therefore, gas humidification and close attention to hydration are desirable for transports longer than
2 hours.

Variable Altitude
Changes in altitude that occur during air transport present a potential hazard to an acutely ill neonate because of
the phenomena that occur during ascent. As altitude increases, the following occur:
Air temperature decreases.
Partial pressure of gases decreases.
Total atmospheric pressure decreases.
For the change in altitude to be clinically important, ascent must be of significant magnitude (in excess of 5,000
feet). At constant temperature, gas volume expands as atmospheric pressure decreases:
P1V1 = P2V2

Where P1 = initial pressure, P2 = final pressure, V1 = initial volume, and V2 = final volume.

Therefore, gases contained in spaces not in continuity with the atmospheresuch as those in cuffed
endotracheal tubes, sinuses, middle ear canals, pneumothoraces, pneumatoses, intrapulmonary cysts,
pulmonary interstitial emphysema, intracranial and intraocular air, and air spaces distal to obstructed bronchi
can all expand as atmospheric pressure declines. Attempts should be made to ventilate closed-space gas to the
atmosphere when significant changes in altitude are expected. Also, the impact of gas expansion during ascent
can be minimized by the use of pressurized aircraft if a significant change in altitude during the conduct of a
transport is anticipated.

Confined Space
Space limitations in transport vehicles may impact care. The recommended minimum floor space for the care of a
critically ill neonate in an NICU is 150 square feet (41). A standard ambulance has approximately 47 square feet,
and aeromedical helicopters have 22 to 36 square feet of workspace. Personnel must remain seated and
restrained while the vehicle is in motion; therefore, typically only one provider has any access to the patient in
transit.

Minimizing the Impact of the Transport Environment


An appreciation of the problems created by the transport environment and strategies to minimize their impact are
essential for safe transport. Some general principles include the following:
Prepare the transport vehicle. The vehicle should be retrofitted to simulate the inpatient environment as much
as is possible and practical. This generally requires the addition of supplemental lighting and sound insulation.
Assess and stabilize the patient extensively before transport. Other than surgical emergencies, neonates
have problems that can be managed adequately by the transport team. There is rarely urgency in returning to
the tertiary center, and time spent in the community hospital preparing the patient for transport is not time
wasted. Stabilization will prepare the patient for the highest risk period, the time in transit between hospitals.
Monitor electronically all possible physiologic parameters. Because of the dynamic nature of the diseases in
most transported patients and the inability to assess patients by physical examination in transit, electronic
monitoring is critical to the identification of significant changes in physiology.
Anticipate deterioration. All possible forms of deterioration should be anticipated before transport, and
strategies to support the patient in the event of deterioration should be planned. Application of this principle
can result in the performance of procedures or therapies that may not be necessary in the inpatient setting, for
example, intubation of an infant requiring continuous positive airway pressure or thoracostomy tube placement
for a pneumothorax of borderline significance.

Equipment
Major pieces of equipment used during neonatal transport include the following:
1. Essential equipment
Portable incubator
Mechanical ventilator
Cardiorespiratory monitor
Blood pressure transducer
Noninvasive blood pressure monitor
Transcutaneous O2 monitor or pulse oximeter
Intravascular infusion pumps
Air-oxygen blender
Suction apparatus
2. Desirable equipment
Body temperature monitor
Transcutaneous CO2 or end-tidal CO2 monitor
Airway humidification system
Blood gas/glucose analyzer
Although these devices can be purchased individually and either carried separately or attached to the
incubator, it usually is advisable, and often more economical, to purchase a modular incubator that includes
many of the devices listed. Modular transport incubators have been designed to minimize space and weight.
They also use a common battery power supply for most devices. Several transport incubators are
commercially available. The logical choice for each program often depends on the size, weight, and heating
capability of the unit.
With the increased use of inhaled nitric oxide, many transport teams have acquired the ability to provide this
gas during transport. The inhaled nitric oxide delivery system can be cumbersome. Some transport teams
use a small, portable, commercially available device, while other teams have constructed their own system
for transport (42).
Small accessory equipment and supplies can be divided into respiratory care supplies and nursing supplies.
These supplies can be carried in packs or equipment bags (Tables 5.1, 5.2, 5.3, and 5.4). They should be
organized in a recognized and reproducible fashion. This technique will aid in rapidly locating an item during
transport and assist in restocking after use.

Transport Vehicles
An essential component of neonatal transport is rapid, safe transportation. The types of vehicles in use include
standard ambulances, specially prepared ground ambulances, rotor-wing aircraft, and fixed-wing aircraft. The
selection of one or more of these vehicles to support a neonatal transport program usually is based on patient
population, resources, geography, and practical issues, such as the use of the vehicle by other hospital-based
services (43,44).
Ambulances are economical, available, and least affected by weather; however, they generally require retrofitting
to make them acceptable for neonatal transport. Extensive retrofitting, including the addition of radiant heat and a
blood gas analyzer, improves patient care capabilities but dramatically increases costs and decreases
usefulness for other services. The major disadvantage of ground ambulance transport is time consumption,
which can be prohibitive if frequent long transports are anticipated or traffic congestion is inherent.
P.50

TABLE 5.1 Neonatal Nursing Pack

Equipment Amount

Chest tube insertion kit 1

Omphalocele bag, sterile 1

Sterile lancets 4

Angiocatheter

18G 2

22G 2

24G 9

Intraosseous needles 2

IV limb board 2

Rubber bands 6
Tape

Silk 1 roll

Dermaclear 1 roll

Stethoscope

IV fluids

D10W One 500-mL bag

D5W One 500-mL bag

LR One 1,000-mL bag

NS One 100-mL bag

D5NS One 500-mL bag

D5NS One 500-mL bag

Masks 2

Syringes

20-mL Luer lock 2

60-mL Luer lock 4

Transilluminator 1

Gloves, sterile

Size 6 2 pairs

Size 7 2 pairs

Suction catheters, sterile

Size 6 Fr 2

Size 8 Fr 2
Stockinette for caps 2
Cotton balls 4

Feeding tubes

8 Fr 2

5 Fr 2

Gowns 2

Blood gas syringe 2

Tegaderm 1

Protocol manual 1

Disposable transducer 2

Scissors 1

Hemostat 1

Tape measure 1

Lubricant 2

Disposable blood pressure cuffs sizes 2, 3, 4, and 5 1 each

Pacifier 1

Bulb syringe 1

Sterile gauze 2

Stopcocks 2

Extension tubing 1

Thoracostomy tubes

10 Fr 2

12 Fr 2
Digital thermometer 1

Umbilical catheters

3.5 Fr 2

5.0 Fr 2

Heimlich valves 2

Alcohol and Betadine swabs 10 each

IV extension T-connectors 2

Butterfly needles

21G 2

23G 2

25G 3

Syringes

10 mL 3

3 mL 9

1 mL 9

60-cc catheter tip syringe 1

60-cc Luer lock syringe 4

Needles, 19G 10

Tubing adapters (blunt needles) 2

Replogle, 10 Fr 2

Tubing

Mini-volume extension 1
Low-volume extension 4
Blood component and filter set 1

IV ext. double T-connector 1

cc, cubic centimeter; D5W, 5% dextrose in water; D10W, 10% aqueous dextrose solution; IV,
intravenous; LR, lactated ringer; NS, normal saline.

Rotor-wing aircraft minimize transit time and, within a 150-mile radius, usually provide the fastest service. The
major disadvantages of helicopter transportation are the constraints of the patient care environment, the high
cost of operation, and the inherent safety risks (45,46) of helicopter flight. The cost of rotor-wing transportation
usually cannot be justified unless the vehicle can be shared by other emergency medical services.
Fixed-wing aircraft are more economical, spacious, quiet, and efficient compared to rotor-wing aircraft; however,
they must travel between airports, and therefore, additional patient transfers are required. These shuttles
between the hospital and airport often are troublesome and increase the likelihood of mishap. For these reasons,
transportation by fixed-wing aircraft usually is advantageous only for distances between hospitals in excess of
150 miles.

DOCUMENTATION
Transport programs typically maintain record-keeping systems that are distinct from the inpatient record. An
accurate, thorough record of each transport is essential to provide permanent documentation of the care
rendered. The transport record should adhere to the standards of documentation of the sponsor institution. The
record also is a valuable tool for quality assurance and education. The critical components of a typical transport
record include the following:
Medical necessity documentation/referral form
Transport medical record with run-sheet of timed medical interventions/protocols/orders
Parental consent form
Billing form
P.51

TABLE 5.2 Neonatal Respiratory Therapy Pack

Equipment Amount

Exterior Pockets

Oxygen tubing 2

Infant nasal cannula 2

Complete ventilation setup with exhalation 1 valve (plus one in isolette)


Spare exhalation valve 1

Face tent 1

Treatment setup 1

Space blanket 1

Earmuffs 2

Thermal hats 2

Pulse oximeter sensors (N-25 and I-20) 2 each

Suction catheters 6 Fr, 8 Fr, 10 Fr 3 each

NCPAP prongs (#1,#2) 1 each

Lukens trap 1

Interior of Bag

Airway supplies

Laerdal masks

No. 0 2

No. 1 2

No. 2 1

Infant McGill forceps 1

Laryngoscope handle 1

Laryngoscope handle fiberoptic 1

Blades, Miller/Shaw #00,#0,#1 1 each

Other equipment

Benzoin applicators 6

Alcohol preps 4
Adjustable wrench 1

E-tank wrench 1

Cable ties 1

Scissors 1

9-V battery 1

Assorted laryngoscope bulbs 4

Adjustable Venturi 2

Silicone adapter 2

O2 flowmeter nipple 2

One-way valve 1

Set of EKG lead wires 2

23G, 25G, -inch butterfly 3 each

Breath Tracker 1

Albuterol 1

Racemic epinephrine 1

Heliox 1

Tape measure 1

Infant MVB bag, O2 tubing, PEEP valve 1 (plus one in isolette)

Infant oral airway 2

Normal saline 4 vials

Silk tape 1 roll

Oxygen connectors 2
Hemostat 1

Briggs T-adapters 2

15-mm adapter 2

O2 connectors (NCG, OES, P-B) 1 each

Air connectors (NCG, P-B) 1 each

EKG lead pads 3

Three-way stopcock 2

E-Z Heat hot packs 4

Stethoscope 1

1-mL and 3-mL syringe 3 each

Cooling blanket and setup 1

Esophageal temperature probe 1

ABG kit 3

Low-volume extension tubing 4

Full set Mini-Med Tubing 1

Endotracheal tubes

2.5 mm 3

3.0 mm 3

3.5 mm 3

4.0 mm 3

4.5 mm 3

Pedi-Cap detector 2
End Tidal Monitors 2
T-com 1

Istat Pack 1

ABG, arterial blood gas; EKG, electrocardiogram; Fr, French; MVB, manual ventilation bag; NCPAP,
nasal continuous positive airway pressure; PEEP, positive end-expiratory pressure.

As hospital systems utilize computerized medical records, transport documentation must also be electronic,
although no vendor product designed specifically for neonatal transport patients currently exists. Neonatal
transport systems therefore must create their own electronic template, that can be subsequently scanned into the
computerized medical record, or customize an existing adult electronic transport record. Along with electronic
record-keeping systems come the challenge of the charting devices, laptops or personal digital assistants, and
the accompanying efficiency problem of connectivity during transport.

QUALITY ASSURANCE
Performance review of the transport program should be a continual process. All activities of the program should
be reviewed periodically to ensure that standard operating procedures are being observed. These reviews are
best conducted by people directly related to the program activities. For example, the medical care provided by
the team should be scrutinized for adherence to protocols and quality assurance. For nonphysician teams, the
medical director or a physician designate should conduct this level of review. In addition, to assure that every
chart is reviewed and problems discovered in a timely fashion, peer review in the immediate posttransport period
is valuable. These review processes have been streamlined by electronic medical record systems.
Quality assurance activities should be closely linked to education and research. Review of individual transport
records can be an extremely valuable method of identifying transport personnel in need of further education and
training. The compilation of reviews and the monitoring of patient outcomes provide an assessment of the
efficacy of existing protocols and procedures and may identify a need to alter program activities. In addition, new
therapies and equipment can be evaluated using existing quality assurance techniques.
General guidelines for developing quality assurance programs have been published (47,48). Guidelines, specific
for air transport, have
P.52
been produced by the Association of Air Medical Services (49). The Commission on Accreditation of Medical
Transport Systems (CAMTS) provides external quality assurance reviews for transport programs and allows
programs to benchmark themselves against measurable standards (50). A number of states require CAMTS
accreditation, and some states use CAMTS certification in lieu of state regulations (51).

TABLE 5.3 Neonatal Medicine Pack

Medications Amount

Antibiotics

Acyclovir 1
Ampicillin, 500 mg 1

Ampicillin, 250 mg 1

Ceftriaxone, 1 g 1

Clindamycin 1

Gentamicin, 20 mg 1

Metronidazole 1

Vancomycin 1

Cardiovascular Medications

Atropine 1

Adenosine, 6 mg 2

Amiodarone 2

Alprostadil 1

Digoxin 1

Dobutamine 1

Dopamine 1

Epinephrine 1:1,000, 1 mL 1

Epinephrine 1:1,000, 30 mL 1

Hydralazine, 20 mg 1

Lidocaine 1% 1

Nitroprusside 1

CNS Medications

Acetaminophen 1

Etomidate 1
Flumazenil 1

Naloxone 2

Mannitol 1

Magnesium 1

Phenytoin 2

Rocuronium 1

Succinylcholine 1

Sodium chloride, 3% 1

Vecuronium 1

Respiratory Medications 1

Curosurf 1

Nitric oxide 1

Steroids

Dexamethasone 1

Methylprednisolone 1

Miscellaneous

Artificial tears 1

Benadryl 1

Calcium gluconate 1

Calcium chloride bristojet 1

D50W 1

Furosemide 1
Heparin lock flush 2

Ondansetron 1

Lidocaine 2% bristojet 1

Sterile water vials, 20 mL 4

Sodium bicarbonate 1

Vitamin K, 1 mg/mL 1

Neonatal Narcotic Pack

Morphine 2 mg/mL 2

Midazolam 5 mg/mL 1

Phenobarbital 130 mg/mL 1

Fentanyl 50 g/mL 1

Lorazepam 2 mg/mL 1

D50W, 50% dextrose water.

PSYCHOSOCIAL CONSIDERATIONS
Psychological Impact on the Family
Neonatal transport creates countless stressors for a family. Although it is impossible to eliminate these anxieties,
a few techniques exist that may help families in coping with this difficult situation. The transport team should
provide the family with as much information as possible about the nature of their child's illness, the therapies and
equipment that will be used, the NICU to which the infant will be transported, and the professionals who will
provide care. A member of the community hospital staff should be in attendance during this discussion in
preparation for dealing with questions that may arise after the departure of the transport team (52). This
information should be provided in both verbal and written form in lay language that is sensitive and easily
understood. Many teams use brochures that describe their service and provide relevant phone numbers and
directions to the referral center and the NICU.
Parents should see their infant before departure from the community hospital. The benefit of this interaction
outweighs any delay in departure (53). This contact should be encouraged prior to the transport of even the most
critically ill infant or when parents are reluctant to view their child. When possible, an opportunity for the family to
photograph their infant should be offered. On arrival at the referral center, the transport team should call the
family immediately to reassure them that their child has arrived safely and discuss any pertinent changes in their
child's status since transport. The transport team should alert the referral center staff to any unusual problems
the parents might have in coping with their child's illness.

Relationships with Referring Hospital Personnel


Transporting a neonate from a community hospital to a referral center has the potential to either improve
dramatically the relationship between institutions or to cause irreparable damage. Each transport represents an
opportunity for success or failure. To ensure success, community hospital personnel must have easy access to
and a quick response time from the transport team. Rapidity of response often is critical from a public relations
standpoint, even when the infant's medical condition does not mandate speed.
Even the most responsive service will fail to satisfy personnel in the community hospital if the team does not
conduct itself appropriately. The team must understand the psychological milieu surrounding a transport. The
event is often emotionally charged because of the acute nature of the infant's illness and feelings of inadequacy
on the part of the community hospital personnel. These feelings seem to arise even when excellent,
comprehensive care is provided. Community hospital personnel may be sensitive to criticism, and any critique of
care, unless requested, should be deferred until a later time. The team should seek information about
P.53
the history and condition of the infant before their arrival, and they should ask for assistance from community
hospital personnel when practical. Members of the transport team should clearly state appreciation for the
contribution made by the community hospital staff. The need for performing all procedures should be explained,
this being particularly important when community hospital personnel have made the decision not to perform a
procedure because of their lack of understanding of the transport environment. Nonphysician teams must avoid
conflicts with community physicians over the need for therapies or procedures. Any disagreements should be
resolved through discussion between the community physician and the consulting physician in the referral
center.

TABLE 5.4 Back Transport Pack

Equipment or Drug Amount

Equipment

Istat Pack 1

Lancets, sterile 5

Stethoscope 1

Syringes

1 mL 3

3 mL 3

10 mL 1

20 mL 1
60 mL 3

Needles, 19G 10

Bulb syringe 1

Feeding tubes

5 Fr 1

8 Fr 1

Butterfly needles

23G 2

25G 2

ET tubes, 2.5 through 5.0 2 each

Stylets 2

Laryngoscope and blades

(Miller 0, 1) 1 each

Face masks, assorted sizes 1 each

Face tent 1

Manual ventilation bag 1

Nebulizer setup 1

Venturi tubing 2

Nasal cannula 2

Alcohol and Betadine swabs 10 each

Thermometer 1

Suction catheters
6 Fr 2

8 Fr 2

10 Fr 2

Yankauer suction 1

Three-way stopcock 2

IV tubing

Low-volume extension 2

Mini-volume extension 1

Minimed full set 1

Minimed half set 1

Oximeter probes 2

EKG electrodes 6

Hot packs

Small 1

Large 1

Silver thermal hats

Small 1

Large 1

IV limb board 1

Intraosseous needle 1

BP cuffs (2,3,4,5) 1 each

Scissors 1

Hemostat 1
T-connector 2

Angiocatheter, 24G 4

Safety pins 6

Tape 2 rolls

Benzoin 1

Sterile gloves

Size 6 2

Size 7 2

Diapers 3

Pacifiers 1

Oxygen tubing 2

Medications

Isotonic saline 2

Heparin flush 2

Sodium bicarbonate 2

Epinephrine 1:10,000 bristojet 1

Atropine bristojet 1

Sterile water 2

D10W, 500 cc 1

D5W, 100 cc 1

D5/0.2 NaCl, 250 cc 1

Normal saline, 250 cc 1


BP, blood pressure; cc, cubic centimeters; D5/0.2 NaCl = dextrose 5% injection in 2% sodium chloride;
D5W, 5% dextrose in water; D10W, 10% aqueous dextrose solution; EKG, electrocardiogram; ET,
endotracheal; Fr, French; IV, intravenous.

LEGAL CONSIDERATIONS
Emergency medical services personnel may be held to a different standard of care while rendering aid at the
scene of an accident or in a transport vehicle compared to performing the same tasks in the inpatient setting
(54). Neonatal transport, however, is more closely associated with inpatient intensive care and less likely to be
considered an emergency service. Therefore, neonatal transport personnel should assume that they have the
same risk of litigation as other perinatal caretakers.
Although there are few regulations and minimal case law defining the legal obligations of transport services,
understanding the principles that are likely to govern legal decision making will help guide programs in
establishing sound practices and limit litigation risk (55). The principles of respondeat superior define the
hospital as the party responsible for governing the protocols and procedures followed by its personnel (56).
These principles apply to mobile services as well as inpatient care. Therefore, the hospital that sponsors a
transport program is responsible for selecting and training the personnel and defining their scope of practice.
Logically, the medical director, as the
P.54
medical professional delegated to ensure the quality of care, also is liable for the governance of the team. Team
members assume personal liability only if they perform outside their enfranchised scope of practice.
Each transport program should construct a manual of operations clearly denoting standard procedures. The
method used for selecting, training, and certifying personnel should be documented. Similarly, protocols and
procedures should be recorded and approved by the medical director. Activities of nonphysician personnel that
exceed their usual scope of practice in the inpatient setting should be listed, approved by the respective
governing bodies (e.g., Board of Nursing) and skills verified and updated routinely. All documentation should be
kept on permanent file.
During the conduct of a transport, the team should adhere to established protocols and procedures (57) unless
the patient's medical needs dictate an abridgement. In this situation, advice from a consulting physician should
be sought, and the recommendations of this physician should be carefully documented in the patient record.
Community hospitals referring patients have both ethical and legal responsibilities to patients requiring
interhospital transfer, with the latter responsibilities outlined primarily in the Consolidated Omnibus Budget
Reconciliation Act (COBRA) of 1985 (58). This federal legislation assigns to the referring/community hospital the
responsibility to adequately stabilize the patient prior to transport. The community hospital also must establish an
agreement with a hospital to receive the patient and must ensure that the receiving hospital/referral center is
capable of providing for the predicted needs of the patient. The 1989 Amendment of this act added the
requirement that referring hospitals make an effort to obtain written consent from parents of a minor patient prior
to transport. Failure to comply with these requirements is considered medical abandonment.
Referring and receiving hospitals, and their personnel, have distinct responsibilities to the patient at varying
points in time during the conduct of a transport. There is no single point at which the responsibility shifts
absolutely from referring to receiving hospital. From the time of a transport call to the arrival of the patient in the
receiving hospital, there is a stepwise decline in the responsibility of the referring hospital (Fig. 5.3). Critical
events that shift responsibility include
Arrival of the transport team in the referring (community) hospital
Assumption of direct patient care by the transport team
Departure of the team and patient from the referring (community) hospital
Arrival in the receiving (referral center) hospital (59)

FIGURE 5.3 Changing levels of legal responsibility for patient care. From Brimhall DC. The hospital
administrator's perspective. In: MacDonald MG, ed., Miller MK, assoc. ed. Emergency transport of the perinatal
patient. Philadelphia, PA: Little, Brown and Co., 1989:148, with permission.

The scope of nursing practice is usually established by two sets of regulations, the rules and regulations set
forth by their employing hospital or agency and the nurse practice acts in the state in which they practice.
Transport creates unique problems regarding nursing scope of practice because transport nurses often provide
care in a hospital other than their sponsoring institution, and sometimes they may provide patient care in a state
other than the one in which they are licensed (59). Nurses in general are not permitted to practice under the
supervision of a physician not credentialed by their employing hospital. Therefore, while providing care in the
community hospital and sharing responsibilities with the community physician, a transport nurse must follow
protocols and procedures established by the sponsoring institution, or receive verbal orders from the referral
center's MCO. In anticipation of problems during interstate transport, many adjacent states have established
reciprocal relationships for licensure. Transport teams may circumvent this problem entirely by admitting the
patient to their home hospital at the time of first contact with the patient and thus team members are considered
to be practicing in their licensed state.
Vendors who provide transportation for medical personnel and patients are governed by either state or federal
legislation. The Emergency Medical Services Act of 1973 places the responsibility for the governance of ground
transportation under the umbrella of state emergency medical service regulations. Air transportation services
must comply with Part 135 of the Federal Aviation Administration regulations, which govern medical air
operations.

BACK TRANSPORT
The return transport of convalescing infants to community hospitals before discharge home is referred to as back
transport. Benefits of back transport include the following:
Reserves referral center resources for critically ill patients, decreasing overcrowding in these units (60,61)
Improves use of community hospital resources and helps prepare their personnel for the care of acutely ill
patients
Improves relationships between referral centers and their community hospitals
Familiarizes primary care physicians with infants before discharge home
Promotes family visitation and family-infant bonding
Reduces the total cost of medical care (62,63)
There are also potential disadvantages associated with back transport, including the following:

Parental anxiety and loss of continuity of care caused by the change of caretakers
Occasional requirement for readmission to the referral center (64)
Lost opportunity by referral center personnel to participate in convalescent care
Hazards and cost of transport
Lack of reimbursement by third party payers for the transport (63)
Back transport should be considered an option for all infants who no longer require the unique resources of the
referral center and for whom the referral center is not the site of subsequent primary care (64).

REFERENCES
1. DeLee JB. Infant incubation, with the presentation of a new incubator and a description of the system at
the Chicago Lying-In Hospital. Chic Med Rec 1902;22:22.

2. Butterfield LJ. Historical perspectives of neonatal transport. Pediatr Clin North Am 1993;40(2):221.

3. Losty MA, Orlofsky I, Wallace HM. A transport service for premature babies. Am J Nurs 1950;50:10.

P.55

4. Wallace HM, Losty MA, Baumgartner L. Report of two years experience in the transportation of premature
infants in New York City. Pediatrics 1952;22:439.

5. Pettett G, Merenstein GB, Battaglia FC, et al. An analysis of air transport results in the sick newborn
infant: Part I: the transport team. Pediatrics 1975;55(6):774.

6. Cifuentes J, Bronstein J, Phibbs CS, et al. Mortality in low birth weight infants according to level of
neonatal care at hospital of birth. Pediatrics 2002;109(5):745.

7. Hohlagschwandtner M, Husslein P, Klebermass K. Perinatal mortality and morbidity: comparison between


maternal transport, neonatal transport and inpatient antenatal treatment. Arch Gynecol Obstet 2001;265:113.

8. Richardson DK, Reed K, Cutler JC, et al. Perinatal regionalization versus hospital competition: the
Hartford example. Pediatrics 1995;96(3):417.

9. Bose CL. Organization and administration of a perinatal transport service. In: MacDonald MG, Miller MK,
eds. Emergency transport of the perinatal patient. Boston, MA: Little, Brown and Co., 1989:43.

10. Hood JL, Cross A, Hulka B, et al. Effectiveness of the neonatal transport team. Crit Care Med
1983;11(6):419.

11. Chance GW, Matthew JD, Gash J, et al. Neonatal transport: a controlled study of skilled assistance. J
Pediatr 1978;93(4):662.

12. American Academy of Pediatrics, Committee on Fetus and Newborn, and American College of
Obstetricians and Gynecologists, Committee on Obstetric Practice. Guidelines for perinatal care, 7th ed.
Evanston, IL: American Academy of Pediatrics and American College of Obstetricians and Gynecologists,
2007:58.

13. Brimhall D. Developing administrative support for the transport system. In: McCloskey K, Orr R, eds.
Pediatric transport medicine. St. Louis, MO: CV Mosby, 1995:56.

14. Mitchell A, Watts J, Whyte R, et al. Evaluation of graduating neonatal nurse practitioners. Pediatrics
1991;88(4):789.

15. Karlowicz MG, McMurray JL. Comparison of neonatal nurse practitioners' and pediatric residents' care of
the extremely low-birth-weight infants. Arch Pediatr Adolesc Med 2000;154(11):1123.

16. Thompson TR. Neonatal transport nurses: an analysis of their role in the transport of newborn infants.
Pediatrics 1980;65(5):887.

17. Aylott M. Expanding the role of the neonatal transport nurse: nurse-led teams. Br J Nurs 1997;6(14):800.

18. Cook LJ, Kattwinkel J. A prospective study of nurse-supervised versus physician-supervised neonatal
transports. JOGN Nurs 1983;12(6):371.

19. American Academy of Pediatrics, Task Force on Interhospital Transport. Guidelines for air and ground
transport of neonatal and pediatric patients, 3rd ed. Elk Grove Village, IL: American Academy of Pediatrics,
2007:37.

20. Conn AKT, Bowen CY. The communications network for perinatal transport. In: MacDonald MG, Miller
MK, eds. Emergency transport of the perinatal patient. Boston, MA: Little, Brown and Co., 1989:93.

21. Perlstein PH, Edwards NK, Sutherland JM. Neonatal hot line telephone network. Pediatrics
1979;64(4):419.

22. Risemberg HM. Financing a perinatal transport program in the United States. In: MacDonald MG, Miller
MK, eds. Emergency transport in the perinatal patient. Boston, MA: Little, Brown and Co., 1989:85.
23. Bose CL. The transport environment. In: MacDonald MG, Miller MK, eds. Emergency transport of the
perinatal patient. Boston, MA: Little, Brown and Co., 1989:195.

24. Philbin MK, Gray L. Changing levels of quiet in an intensive care nursery. J Perinatol 2002;22(6):455.

25. Kellman N. Noise in the intensive care nursery. Neonatal Netw 2002;21(1):35.

26. Robertson A, Cooper-Peel C, Vos P. Peak noise distribution in the neonatal intensive care nursery. J
Perinatol 1998;18(5):361.

27. Shenai JP. Sound levels for neonates in transit. J Pediatr 1977;90(5):811.

28. Campbell AN, Lightstone AD, Smith JM, et al. Mechanical vibration and sound levels experienced in
neonatal transport. Am J Dis Child 1984;138:967.

29. Gadeke R, Doring B, Keller R, et al. The noise level in a children's hospital and the wake-up threshold in
infants. Acta Paediatr Scand 1969;58:164.

30. Blackburn S. Environmental impact of the NICU on developmental outcomes. J Pediatr Nurs
1998;13(5):279.

31. Shenai JP, Johnson GE, Varney RV. Mechanical vibration in neonatal transport. Pediatrics
1981;68(1):55.

32. MacNab A, Chen Y, Gagnon F, et al. Vibration and noise in pediatric emergency transport vehicles: a
potential cause of morbidity? Aviat Space Environ Med 1995;66(3):212.

33. Floyd WN, Broderson AB, Goodno JF. Effect of whole-body vibration on peripheral nerve conduction time
in the rhesus monkey. Aerosp Med 1973; 44(3):281.

34. Clark JG, Williams JD, Hood WB, et al. Initial cardiovascular response to low frequency whole body
vibration in humans and animals. Aerosp Med 1967;38(5):464.

35. Ando H, Ishitake T, Miyazaki Y, et al. The mechanism of a human reaction to vibration stress by palmar
sweating in relation to autonomic nerve tone. Int Arch Occup Environ Health 2000;73(1):41.

36. Adey WR, Winters WD, Kado RT, et al. EEG in simulated stresses of space flight with special reference
to problems of vibration. Electroencephalogr Clin Neurophysiol 1963;15:305.

37. Graybiel A, Knepton J. Sopite syndrome: a sometimes sole manifestation of motion sickness. Aviat Space
Environ Med 1976;47:873.

38. Lawson BD, Mead AM. The sopite syndrome revisited: drowsiness and mood changes during real or
apparent motion. Acta Astronaut 1998;43 (3-6):181.
39. Wright MS, Bose CL, Stiles AD. The incidence and effects of motion sickness among medical attendants
during transport. J Emerg Med 1995; 13(1):15.

40. Patient compartment illumination. Federal specifications for the Star of Life ambulance KKK-A-1822F.
Washington, DC: National Automotive Center, General Services Administration, 2007. Para 3.8.4.1.

41. American Academy of Pediatrics, Committee on Fetus and Newborn, and American College of
Obstetricians and Gynecologists, Committee on Obstetric Practice. Guidelines for perinatal care, 5th ed.
Evanston, IL: American Academy of Pediatrics and American College of Obstetricians and Gynecologists,
2002:45.

42. Kinsella JP, Griebel J, Schmidt JM, et al. Use of inhaled nitric oxide during interhospital transport of
newborns with hypoxemic respiratory failure. Pediatrics 2002;109(1):158.

43. Schneider C, Gomez M, Lee R. Evaluation of ground ambulance, rotor-wing, and fixed-wing aircraft
services. Crit Care Clin 1992;8(3):533.

44. Brink LW, Neuman B, Wynn J. Air transport. Pediatr Clin North Am 1993;40(2):439.

45. King BR, Woodward GA. Pediatric critical care transportthe safety of the journey: a five-year review of
vehicular collisions involving pediatric and neonatal transport teams. Prehosp Emerg Care 2002;6(4):449.

46. DeLorenzo RA. Military and civilian emergency aeromedical services: common goals and different
approaches. Aviat Space Environ Med 1997;68(1):56.

47. Council on Medical Service. Guidelines for quality assurance. JAMA 1988;259(17):2572.

48. Joint Commission on the Accreditation of Hospitals and Health Organizations. Examples of monitoring
and evaluation in emergency services. Chicago, IL: JCAHHO, 1988:13.

49. Eastes L, Jacobson J, eds. Quality assurance in air medical transport. Orem, UT: WordPerfect
Publishers, 1990.

50. Commission on Accreditation of Medical Transport Systems. Accreditation standards of the commission
on accreditation of medical transport systems, 9th ed. Anderson, SC: Commission on Accreditation of
Medical Transport Systems, 2012.

51. Frazier E. How many state EMS agencies require CAMTS accreditation for air ambulance services. Air
Med J 2012;20(1):8.

52. McBurney B. The role of the community hospital nurse in supporting parents of transported infants.
Neonatal Netw 1988;6:60.

53. MacNab AJ, Gagnon F, George S, et al. The cost of family-oriented communication before air medical
interfacility transport. Air Med J 2001; 20(4):20.

54. Reimer-Brady JM. Legal issues related to stabilization and transport of the critically ill neonate. J Perinat
Neonatal Nurs 1996;10(3):62.

55. Ginzburg HM. Legal issues in medical transport. In: MacDonald MG, Miller MK, eds. Emergency transport
of the perinatal patient. Boston, MA: Little, Brown and Co., 1989:152.

56. Tonsic v Wagner, 458 Pa. 246; 329 A 2d 497;1974.

57. American Academy of Pediatrics, Task Force on Interhospital Transport. Guidelines for air and ground
transport of neonatal and pediatric patients, 3rd ed. Elk Grove Village, IL: American Academy of Pediatrics,
2006,16.

58. Ross M, Hayes C. Consolidated Omnibus Budget Reconciliation Act of 1985. Soc Secur Bull
1986;49(8):22.

59. Brimhall DC. The hospital administrator's perspective. In: MacDonald MG, Miller MK, eds. Emergency
transport of the perinatal patient. Boston, MA: Little, Brown and Co., 1989:147.

60. Jung AL, Bose CL. Back transport of neonates: improved efficiency of tertiary nursery bed utilization.
Pediatrics 1983;71:918.

61. Zarif MA, Rest J, Vidyassagar D. Early retransfer: a method of optimal bed utilization of NICU beds. Crit
Care Med 1979;7:327.

62. Bose CL, LaPine TR, Jung AL. Neonatal back transport: cost effectiveness. Med Care 1985;23(1):14.

63. Phibbs CS, Mortensen L. Back transporting infants from neonatal intensive care units to community
hospitals for recovery care: effect on total hospital charges. Pediatrics 1992;90(1 Pt 1):22.

64. Lynch TM, Jung AL, Bose CL. Neonatal back transport: clinical outcomes. Pediatrics 1988;82(6):845.
6
Radiologic Imaging of the Neonate
Jeffrey C. Hellinger
Nicole Mendelson
Mary M.K. Seshia
Mhairi G. MacDonald

INTRODUCTION
Radiologic imaging is an essential clinical tool for investigating and monitoring neonatal disease. These
diagnostic data are fundamental to prompt bedside management and decision making, particularly in a critically ill
neonate in the intensive care unit (ICU). Goals are to obtain reliable and reproducible images that meet or
exceed established standards for neonatal imaging. Images of sufficient quality should readily afford analysis,
understanding, and accurate interpretation. While high diagnostic quality is a prerequisite for meaningful use and
confident decision making, anatomical and functional neonatal imaging algorithms should always strive for the
highest safety of the neonate.
Safety considerations in neonatal imaging include the type of imaging modality, the location of imaging, and the
effectiveness of communication between all providers in the care of the neonate. Plain film radiography,
ultrasound, fluoroscopy, computed tomography (CT), and magnetic resonance imaging (MRI) are common
modalities utilized for neonatal imaging. Nuclear scintigraphy is less commonly used. Each modality has intrinsic
benefits, risks, and limitations, which must be considered when choosing an imaging strategy and applying the
interpretive findings. A primary risk consideration in neonatal imaging is radiation exposure (1). Radiography,
fluoroscopy, CT, and nuclear scintigraphy are dependent upon radiation to generate images, while ultrasound
and MRI are not. Another risk consideration is the transportation for and performance of imaging outside of the
neonatal unit. Radiography and ultrasound can be performed routinely at the bedside, minimizing risks extrinsic
to the type of modality. Fluoroscopy, MRI, and nuclear scintigraphy require transportation of the neonate to a
dedicated radiology department, increasing the potential risks. While in select centers it is possible to perform
CT at the bedside with a portable CT unit, in most neonatal centers CT is also performed in a dedicated imaging
department.
Effective communication among bedside clinicians, radiologists, imaging technologists, and other health care
providers is important to ensure appropriate use and performance of these modalities in neonatal imaging. When
modalities are applied and operated appropriately, the potential for diagnostic yield, clinical impact, and neonatal
safety is optimized. Effective communication is achieved through a collaborative team approach to share a
neonate's clinical data. In this manner, imaging protocols and strategies are best tailored to the neonate's clinical
presentation and known or suspected disease process, while the duration and volume of imaging are potentially
minimized. It is equally important that there is clear and timely reporting of findings to optimize timely and
appropriate bedside decision making. Full-proof protocols for conveying urgent imaging diagnoses from the
radiologist to the NICU team must be in place and, in reverse, ready access to a radiologist for consultation. In
the current digital era, it is strongly recommended that the neonatal imaging team, with all relevant health care
providers, reserve direct personal time for regular consultative discussions and reviews of patients, their
pathologic processes, clinical management, radiologic studies, and other diagnostic data. Strong emphasis on
communication and teamwork is also paramount in order to minimize risks to the neonate during transportation
for radiologic studies outside the neonatal unit.
Imaging of the neonate is distinguished by the small size of the patient, immature nature of organs, distinct
physiology, range of potential multiorgan congenital disorders, and unique in utero and early postpartum
acquired pathology. These unique characteristics present technologic and interpretive challenges for the imaging
team as it seeks optimization of performance, patient safety, and diagnostic yield, along with minimization of cost
and potential short- and long-term iatrogenic sequelae from imaging. These challenges further highlight the
importance of strong teamwork, communication, and consideration of the risks and benefits of the radiologic
modalities. Addressing these challenges and successfully utilizing multimodality diagnostic imaging for neonatal
clinical management require fundamental understanding of neonatal imaging principles, the imaging modalities,
and key modality findings for neonatal pathology. This chapter provides a review of imaging principles and
modalities and an overview of imaging findings for neonatal pathology using a body system approach. Also
included is a review of the radiologic evaluation of neonatal catheters and other support devices.

PRINCIPLES OF NEONATAL IMAGING


Neonatal organs are highly sensitive to radiation, relative to more mature organs in older children and
adolescents and the fully developed organs in adults. Organs, however, have variable radiosensitivity. Targeted
imaging with the radiation-dependent modalities results in an additional source of variable organ exposure.
Despite neonatal organs having variable exposure and susceptibility, one must remember that radiation exposure
is cumulative (2). Each time a neonate undergoes an examination with a radiation-dependent modality, the
output effective radiation dose is added to the total effective exposure. The neonate's total effective radiation
dose is then measured against natural background exposure that the neonate may experience from the
environment. The natural radiation exposure averages approximately 2.5 millisieverts (mSv) per year (1).
A fundamental principle for neonatal imaging is to restrict radiation exposure to not more than 2.5 mSv per year.
This may not be possible for all neonates as a neonate's disease process and clinical management may dictate
frequent imaging and higher utilization of radiation-dependent modalities (2). Nevertheless, every attempt should
be made to control a neonate's exposure to radiation from medical imaging and reduce the potential adverse
end-organ sequela.
Restricting a neonate's medical radiation exposure requires that neonatologists and radiologists adhere to the
principles of ALARA As Low As Reasonably Achievable (3). Foremost to achieving ALARA in one's clinical
practice is to selectively limit the use and volume of studies dependent upon radiation. Based upon the clinical
need, this mandates primary consideration of first employing a nonradiation imaging modality (e.g., ultrasound or
MRI) or a nonimaging solution for diagnosis. If there is no alternative to a radiation-dependent modality,
performance should follow established guidelines in an effort to keep exposure low (3,4). Core tenets to these
guidelines are that imaging should always be based upon sound clinical indications, a high pretest diagnostic
yield probability, and moderate-to-high potential clinical impact from the imaging findings. Repeat (e.g., follow-up
head CT) and routine examinations (e.g., daily chest radiographs) should be limited and performed only when
the benefits of radiation exposure will outweigh the risks of added effective radiation exposure. When imaging is
performed with a radiation modality, core technical strategies are employed to reduce neonatal radiation
exposure. These include (a) weight-based protocols for appropriate radiation imaging parameters (e.g.,
kilovoltage, kV; milliamperage, mAs) and (b) body and selective organ-protective shielding (3,4).
While it is always possible to image at the lowest possible kV and mAs parameters, the penalty may be
nondiagnostic quality images. Nondiagnostic images can also result from incorrect neonatal positioning, neonatal
motion, and overlying support devices (Fig. 6.1). The result in these instances is a nondiagnostic exam and
ineffective utilization of health care resources and time. More importantly for neonatal safety, these
nondiagnostic exams also result
P.57
in unnecessary radiation exposure, which has no clinical value and only adds to the neonate's cumulative
effective dose and risk. In other instances, the radiation settings may render an exam of diagnostic quality for the
radiologist to interpret, but of insufficient quality for the bedside clinician to confidently make decisions. The
result is also an ineffective use of resources and an unnecessary exposure to radiation; the exposure does not
lead to decision making and potential changes in the neonate's medical management. At the other end of the
spectrum, complete avoidance of radiation-dependent modalities and reliance solely upon ultrasound and MRI
are not cost-effective, nor workflow efficient. For some disease processes, a radiation-dependent modality will
have a higher diagnostic performance. Not utilizing a radiation modality because of concern about radiation
exposure could then also adversely impact resource utilization and clinical management.

FIGURE 6.1 Multiple overlying support devices may detract attention from the Nasogastric tube
doubled on itself in the stomach with its tip in the esophagus (arrow) and the ET tube bevel too close
to the carina resulting in over ventilation of the right lung and under ventilation of the left lung. From
MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA:
Lippincott Williams & Wilkins, 2013:282.

Quality control measures are critically important to balancing the appropriate use of radiation-dependent
modalities and adhering to ALARA. Radiology departments in collaboration with the neonatologists should have
pathology-based protocols, outlining recommended modality workflow algorithms. This is based upon scientific
performance and the modalities' risks, benefits, and alternatives. Radiologists should have recommended
positioning and radiation exposure parameters (e.g., kV, mAs) for technologists to apply when employing a
radiation-dependent modality. Instructions for general body and selective organ shielding (based upon the
modality and region scanned) are additional radiation control measures. Finally, when preparing for the exam,
control efforts should also focus on reducing or eliminating potential sources of poor image quality. Support
devices nonessential to the imaging goals should be excluded from the field of view if possible. Neonatal motion
can be controlled by swaddling or use of sucrose in some examinations (5). For other examinations, sedation is
key to controlling motion. Sedation protocols should be established in collaboration with neonatologists and
pediatric anesthesiologists for optimum results and patient safety. Third-party products and software are
available to record and monitor a neonate's cumulative exposure. Knowledge of a neonate's actual cumulative
dose can help restrict medical radiation exposure and guide imaging strategies.
Continuous quality reviews are fundamental to ensuring appropriate modality utilization and compliance with
control measures. They are also important to ensuring that exams are of diagnostic quality and are of sufficient
quality for rendering both interpretations and confident clinical decisions. Reviews should assess all aspects that
may impact exam quality including but not limited to radiation exposure settings, positioning, support devices,
and motion. Three hundred sixty-degree input and feedback from the entire extended imaging team are essential
to assessing the quality of the neonatal imaging program, identifying areas for improvement, and implementing
changes that will provide optimal neonatal clinical care and safety.
A final set of general principles for neonatal imaging regards interpretation of the study. For the radiologist and
neonatologist, a systematic analytical approach to each type of imaging study will ensure complete assessment
of neonatal anatomy and identification of potential abnormal pathology and pathophysiology. Evaluation begins
with image quality, proceeds to the support devices in the imaged field of view, and then finally the imaged
anatomy. Recognition of factors that may degrade image quality is a primary step as these factors will potentially
negatively impact the exam's sensitivity, specificity, and positive and negative predictive values. Knowing the
limitations of the exam helps establish a level of confidence in detecting support devices, depicting and
identifying abnormal anatomy, and making subsequent management decisions. Determinants of image quality
will vary by modality. Support devices are recommended as the second step so that these catheters, tubes, and
wires are not overlooked in the diagnostic analysis. Recognition of a malpositioned device and potential
iatrogenic neonatal complication can only be made by actively searching. Support devices may also provide
insight to a neonate's congenital disorder, postpartum acquired disease, clinical management, or a combination
thereof. In the third step, neonatal anatomy is evaluated. Core review addresses size, shape, and contour of the
imaged anatomy. More specific anatomical and functional reviews and findings will vary by the modality and
disease process.

Radiography
Radiography is a two-dimensional (2D) static x-ray imaging technique that depicts structures based upon relative
densities (e.g., air, fat, water, soft tissue, bone). Radiodense structures (e.g., bone), devices, and foreign bodies
are well depicted. Visceral silhouettes and soft tissue lines are depicted with limited detail based upon the
adjacent differential densities.
Radiography is the most commonly used modality in neonatal imaging and has historically been and remains one
of the first steps in radiologic investigations for most neonatal disease processes. The advantages of
radiography are that it is a portable exam, which is easy to perform, process, and interpret in a rapid manner.
This makes it a highly effective method to rapidly screen for neonatal pathology and monitor medical and
postprocedural treatment responses involving the neck, chest, abdomen-pelvis, and skeletal structures. The
evaluation of support device positioning is a further key indication for obtaining radiographs in the neonate. In
each of these applications, anatomy outside of the region(s) of interest should always be shielded.
For some clinical presentations, single-anatomical-level radiography is appropriate. For example, a noncyanotic
neonate with intermittent tachypnea will initially undergo an anteroposterior (AP) chest radiograph. A neonate
with abdominal distention and vomiting but no respiratory distress will initially undergo an AP abdominal-pelvis
radiograph. A hemodynamically stable neonate with a suspected osseous congenital anomaly will undergo
targeted bone imaging (e.g., forearm for radial array anomaly). For more critical neonates with greater time-
sensitive diagnostic needs, multilevel radiography is appropriate. For example, for a neonate in respiratory
distress, a combined AP chest and abdominal-pelvis radiograph is performed. This facilitates evaluation of
targeted anatomy with categorization of visceral situs, recognition of potential cardiovascular and
noncardiovascular congenital disease, and confirmation of appropriate positioning of all support devices placed
during initial neonatal resuscitation and evaluation. Subsequent radiographs can then be targeted to a single
level of concern, for example, chest or abdomen-pelvis radiograph. Follow-up chest radiographs should extend to
at least the upper abdomen to account for variable degrees of inspiration and confirm stability
P.58
of support devices. For example, in Figure 6.1, had the upper abdomen not been included in this well-centered
chest x-ray, the tip of the doubled back nasogastric tube might have been mistaken for the tip of an umbilical
arterial catheter (UAC). The acute clinician would recognize, however, that the UAC if properly placed runs
parallel with the left side of the spine on an AP film and would not deviate to the right as shown. When support
devices require complete follow-up evaluation, coverage should again extend across multiple anatomical levels
to include their entire anticipated locations. At times, lateral projections may be obtained in conjunction with the
standard AP radiographs to evaluate anatomy, suspected pathology, and/or confirm positioning of support
devices ( Figs. 6.2 and 6.3). Diagnostic quality of radiographs
P.59
may be degraded by incorrect positioning, motion, overlying devices, and the degree of under- or overexposure (
Fig. 6.4B). In addition, low inspiratory volumes may degrade the evaluation of chest radiographs.

FIGURE 6.2 (A) UAC in satisfactory high position at the level of the ninth thoracic vertebral body on AP and (B)
Lateral projections. From MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology.
5th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2013:159.

FIGURE 6.3 Effect of abdominal mass simulating catheter misplacement. Anteroposterior (A) and lateral
(B) films show displacement of a UAC by a giant hematocolpos in a 1 day old infant. From MacDonald MG,
Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA: Lippincott Williams
& Wilkins, 2013:168.
FIGURE 6.4 Sequential radiographs in a patient with right pneumothorax. An air collection in supine
neonates (A) is mostly effectively treated with an anteromedial chest tube (B,C). The medial extension is falsely
exaggerated by the slight right posterior oblique rotation of the chest. Pulling this tube back might put the side
holes outside the pleural space. There is a pneumomediastinum, most evident on the lateral view, not drained by
the pleural tube. Note the nuchal air on all three films. From MacDonald MG, Ramasethu J, Rais-Bahrami K.
Atlas of procedures in neonatology. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2013:261.

Appropriately used radiography exposes the neonate to the least amount of radiation, as compared to other
radiation-dependent modalities. The typical dose from an AP chest radiograph is 0.02 mSv, while the dose from
a combined AP chest-abdominal-pelvis radiograph is up to 0.12 mSv (2). These values can be applied as a
means to understand a neonate's dose exposure from other radiation modalities and also when assessing a
neonate's cumulative dose. In this manner, dose exposure is expressed as an equivalent number of radiographs
(e.g., chest radiographs). While this approach may provide a measure for communicating and understanding a
neonate's exposure and risk for single and cumulative exams, it is not always an accurate means for reporting
exposure. As previously discussed, organs may have differential exposure, depending upon coverage, organ
radiosensitivity, and shielding.

Ultrasound
Ultrasound is a modality that utilizes high-frequency sound waves to generate anatomical images and functional
data. B-mode gray-scale real-time sonography serves as a primary method to investigate neonatal congenital
and acquired pathology across most anatomical regions. This includes assessment of intracranial, intra-
abdominal-pelvis, cardiovascular, and musculoskeletal pathologies (Fig. 6.5). Additional use of gray-scale
ultrasound includes image guidance for cardiovascular and noncardiovascular interventions (Fig. 6.6). Color and
pulse-wave Doppler sonography are supplemental techniques that afford evaluation of cardiovascular
physiology. Doppler sonography is also used to evaluate ureteral flow and exclude uterovesicular obstruction. M-
mode sonography can be applied along with gray-scale sonography to evaluate diaphragmatic excursion and
quantify the degree of
P.60
motion. Three-dimensional (3D) ultrasound techniques provide volumetric data and sophisticated morphologic
displays, complementary to standard 2D displays.

FIGURE 6.5 Echocardiogram image of preterm infant with pericardial effusion and central venous line
in left atrium. From MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed.
Philadelphia, PA: Lippincott Williams & Wilkins, 2013: pg 274.

FIGURE 6.6 Echocardiogram images of pericardiocentesis. A: Echocardiogram image of pericardial


effusion. B: Tip of needle in pericardial space. C: Pericardial effusion partially drained. From MacDonald MG,
Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA: Lippincott Williams
& Wilkins, 2013:276.

The key advantage of ultrasound is that it is does not require ionizing radiation to generate images and
physiologic data. In addition, it is widely available, is portable, and can be performed rapidly at the bedside in the
neonatal ICU. The sonographic window may be degraded by catheters, metallic devices, bone, anatomical
deformities, soft tissue edema, body fluid, and normal and abnormal regions of air. Sonography may also be
limited by operator skill, emphasizing the importance of continuous quality control and review.

Fluoroscopy
Fluoroscopy is a rapid sequential 2D x-ray imaging technique that affords dynamic real-time imaging. As with
static radiography, fluoroscopy depicts structures based upon the relative densities of air, fat, water, soft tissue,
and bone. Radiodense structures (e.g., bone), devices, and foreign bodies are well identified, while viscera and
soft tissues are depicted with limited structural detail. Oral, intracavity, and direct intravascular iodinated water-
soluble contrast are administered to enhance lumens, generate new density interfaces, and increase structural
depiction and detail. Multiple specific technical radiation reduction strategies are used simultaneously during
fluoroscopy, in conjunction with the previously described core strategies. These include (a) using intermittent,
pulsed x-ray beams (as opposed to constant and continuous fluoroscopy) of narrow width and low rates; (b)
capturing the majority of the exam with last image hold and fluoroscopic save options (as opposed to actual
radiographic images); (c) using x-ray beam and field-of-view filters; and (d) selecting appropriate larger field of
views and greater radiation source to skin distances. (3) In addition, antiscatter grids should be used on a limited
basis.
Common neonatal applications of fluoroscopy include evaluations of the gastrointestinal (GI) tract and
genitourinary system. Typically, these exams follow an ultrasound of the abdomen, pelvis, or both. Fluoroscopic
evaluation of the GI tract requires intraluminal contrast opacification. Once opacified, GI tract shape, luminal
integrity, course, caliber, and contour can be assessed. For the upper and mid-GI tract, oral contrast is
administered (e.g., barium swallow, esophagram, upper GI series, or small bowel follow-through); the transit of
contrast is monitored in an antegrade direction. For the lower GI tract, a contrast enema is administered in
retrograde fashion toward the cecum (e.g., barium enema). Performance of the appropriate fluoroscopic GI exam
and selection of the correct type and volume of contrast will depend upon the neonate's clinical presentation and
suspected pathology. Neonatal dose exposures from an upper GI series and barium enema may be as low as 0.5
and 0.4 mSv, respectively (2).
The neonatal genitourinary system is evaluated under fluoroscopy, most commonly with voiding
cystourethrography (VCUG). For this examination, contrast is instilled into the bladder in a retrograde fashion via
a catheter. Images are acquired during bladder filling, maximal distention, and voiding to assess bladder and
urethra morphology and exclude ureteral reflux, urethral obstruction, and congenital anomalies involving the
bladder, ureters, and urethra (Fig. 6.7). VCUG catheterization should always be performed by a skilled provider
using proper, sterile technique. Neonatal dose exposure from a VCUG may be as low as 0.1 mSv (2).
Historically, fluoroscopy has also been used to evaluate diaphragmatic motion, airway morphology, and the
cardiovascular system. Fluoroscopy combined with endoscopy has also been used
P.61
for diagnostic evaluation of neonatal biliary and pancreatic ducts (e.g., endoscopic retrograde
cholangiopancreatography [ERPC]). Fluoroscopy is still an option to evaluate a neonate's diaphragm and airway.
However, ultrasound and MRI are now more common, nonradiation alternatives to evaluate the diaphragm, while
both MRI and CT can be used to evaluate the neonatal airway. In most neonatal centers, MR
cholangiopancreatography has replaced ERCP, while ultrasound, MRI, magnetic resonance angiography (MRA),
and CT angiography (CTA) are now principle noninvasive cardiovascular imaging modalities. Fluoroscopic-
based invasive catheter angiography is performed selectively in neonates to investigate congenital heart disease
(CHD), congenital vascular disorders (CVD), and acquired cardiovascular pathologies (see Chapter 30, Figs.
30.22, 30.24, and 30.26).

FIGURE 6.7 A: Cystourethrogram shows dilated posterior urethra (arrows) secondary to posterior urethral
valves. B: Subsequent film shows perforation of the bladder with free contrast in the peritoneal cavity. From
MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA:
Lippincott Williams & Wilkins, 2013:118.

Fluoroscopy remains a primary technique for image-guided cardiovascular and noncardiovascular interventions
in the neonate. Fluoroscopic guidance may be combined with ultrasound to improve technical guidance and
potentially decrease radiation exposure. Hybrid CT-fluoroscopy and MRI-fluoroscopy technologies are also
available and can be applied for neonatal image-guided interventions. Cardiovascular applications include
vascular access and interventions for CHD, CVD, and acquired pathologies. Potential noncardiovascular
applications include enteric access (e.g., feeding and percutaneous gastrostomy tube placements),
percutaneous transhepatic cholangiography and biliary drainage, percutaneous cholecystostomy, and
percutaneous nephrostomy. Noncardiovascular applications also include intraoperative guidance in select
surgical procedures. Radiation exposure during procedures will depend upon the complexity of the interventional
procedure, extent of disease, comorbid risk factors, and the operator's technique.

Computed Tomography
CT is a multiprojectional x-ray beam technique that generates 2D cross-sectional images in the z-axis direction
across all body systems. With current CT scanners, submillimeter sections can be acquired. These datasets
afford multiprojectional 3D anatomical reconstructed displays that enhance interpretation, anatomical
understanding, and treatment planning. High spatial resolution and superior tissue characterization, combined
with frequent exam availability, rapid scan times, and ease of patient access make CT a desirable modality in
select neonatal pathologies. CT scan times in a neonate may range from less than 1 second to 5-8 seconds
depending upon the CT scanner technology, range of coverage, and prescribed technique. While motion can
degrade CT image quality, more rapid CT acquisitions may afford imaging of the neonate independent of motion
and without the need for sedation.
The radiation risks posed by CT are the greatest deterrents to its use in neonates. However, submillisievert
exposure is possible when utilizing core and advanced CT-specific radiation reduction strategies (4). CT often
follows ultrasound or fluoroscopic diagnostic evaluations. Based upon its advantages, CT imaging is indicated in
the neonate for emergent evaluations (e.g., head CT for investigation of intracranial hemorrhage [ICH], ischemia,
or trauma); for superior anatomical characterization (e.g., chest CT for congenital lung lesions) (see Chapter 41,
Fig. 41.15); and for a neonate who has high sedation or general anesthesia risks. CT is used to define choanal
atresia (see Chapter 41, Fig. 41.5A). CT is also indicated when MRI is not available, is contraindicated (e.g.,
during extracorporeal membrane oxygenation), is nondiagnostic (e.g., ferromagnetic materialspatent ductus
arteriosus [PDA] clip), or has a high pretest probability of yielding a nondiagnostic exam. CT may be performed
without or with iodinated intravenous contrast, depending upon the exam indications. Intravenous contrast-
enhanced CT images may be acquired during the arterial phase, venous phase, or visceral phase, yielding CT
arteriography, CT venography, or routine CT scans, respectively. An abdominal-pelvis CT may be performed in a
neonate without or with oral water-soluble iodinated contrast.

Magnetic Resonance Imaging


MRI is an imaging technique that uses high magnetic fields and radio waves to generate 2D and 3D anatomical
displays at any targeted plane for an imaged body system. Functional sequences can also be acquired to
generate physiologic data. The lack of radiation and iodinated contrast exposure along with the ability to
inherently delineate and characterize tissues with high contrast resolution should clearly make MRI an ideal
choice for neonatal imaging.
P.62
In clinical neonatal practice, however, MRI is used as a secondary modality, typically following ultrasound or CT.
In part, this is related to potentially long scan times and limited availability. More importantly, the majority of MRI
exams in a neonate require conscious sedation or (less commonly) general anesthesia to control neonatal
motion. This places added risks on the neonate. Not only can monitoring be challenging during the exam but the
mechanical ventilation, oxygen, and anesthetics can profoundly alter the neonate's circulatory physiology.
Furthermore, anesthetics have been associated with potential neuroapoptotic effects in the brain of young
patients (6) (see Chapter 53). In addition to motion, MRI quality may be degraded by metallic (e.g.,
ferromagnetic) structures and air. Preexam screening is essential to plan for sedation or general anesthesia,
discuss the added risks with the family, and identify metallic objects and devices that may degrade image quality
(see Chapter 46, Figs. 46.12, 46.14, 46.16, 46.17, 46.18, 46.19 and 46.20).

Nuclear Scintigraphy
Nuclear imaging is a technique that uses radioactive material (e.g., radiopharmaceutical) to map out cellular
activity in 2D and 3D anatomical displays. The radiopharmaceutical is delivered to target a set of organs and
organ system based upon cellular properties. Radiation is emitted from the organs to form an image, directly
proportional to the organ uptake and metabolic activity. This affords an ability to evaluate normal and abnormal
cellular physiology and indirectly, anatomic morphology. Primary considerations in the neonate include the
diagnosis of biliary atresia (e.g., hepatobiliary scan) and the localization of a suspected intrapancreatic insulin
producing tumor (e.g., positron emission tomography [PET]-CT exam). While evaluations of the brain, endocrine
system, heart, lungs, kidneys (Fig. 40.16D), GI tract, and skeletal system are possible, other applications in the
neonate are on a selective basis given the radiation exposure and ability of ultrasound, MRI, and low-dose CT to
reliably provide a diagnosis. As an example of the potentially high radiation exposure with nuclear scintigraphy, a
hepatobiliary scan in a neonate may result in exposure to 6 to 7 mSv (2). Because of high radiation exposure,
nuclear scintigraphy is infrequently used for the neonate.

NEONATAL RADIOLOGIC FINDINGS


Catheters and Support Devices
Clinical care of a neonate in the ICU routinely leads to the placement of several different types of catheters,
tubes, and other devices. Core neonatal ICU devices include endotracheal tubes (ETTs), UAC, venous access
catheters, enteric access catheters, and intercostal catheters. Following placement of a device and before it is
used, confirmation of suitable positioning is an immediate priority. Prompt recognition of an abnormally positioned
device is critical for rapid management of a potential iatrogenic injury and prevention of short- and long-term
sequelae.
While there are several clinical bedside measures which the clinician can employ to affirm appropriate
positioning (e.g., auscultation and carbon dioxide monitoring for ETT placement), radiography serves an
essential primary diagnostic role in device management. Device positioning can change during the clinical
course of the neonate. Thus, periodic follow-up radiography serves a key secondary role in device management.
Beyond these core clinical functions, radiographic assessment of support devices also serves an important
ancillary role in the recognition and evaluation of congenital cardiovascular and noncardiovascular disease The
abnormal course of several different devices can offer insight into presence of situs inversus, heterotaxy,
esophageal atresia, congenital diaphragmatic hernia (CDH), abdominal wall defects, patent foramen ovale, an
atrial septal defect, a persistent left superior vena cava (SVC), a persistent left inferior vena cava (IVC), a right
aortic arch, and a circumflex descending aorta.
In addition to the general imaging principles previously discussed, there are a few radiographic principles specific
for imaging devices. First, to ensure adequate depiction of the devices, kV and mAs radiation parameters may
need to be adjusted from the typical neonatal settings. Second, the field of view should completely cover the
internal as well as external segments of the device. This may require imaging multiple anatomical regions of the
neonate (e.g., chest, abdomen, and pelvis). Third, all external segments should be isolated in a straight course,
separate from other devices and aligned at an angle different from the expected internal course. For an AP
radiograph, isolating external from internal segments may require manually repositioning the external segment or
obliquely positioning the neonate. Fourth, all monitoring leads and other objects not related to the support device
should be removed from the field of view. Fifth, when the course and final position of a device are indeterminate
based upon a diagnostic-quality AP radiograph, an additional oblique or lateral projection must be obtained (Fig.
6.8A, and B). Alternatively, suboptimal image quality may preclude diagnostic assessment of the device. In this
instance, the AP radiograph can be repeated with proper adjustments to correct for potential inappropriate
radiation technique, positioning, overlying leads, or a combination thereof.

Endotracheal Tubes
Whether placed from an oral or nasal route, an ETT courses along the spine, ipsilateral to the neonate's chin
and is positioned ideally with the tip approximately 1 cm above the carina (7). The carina is readily identified on
an AP chest radiograph and is used as a reference point to measure distances for required ETT advancement or
retraction. When radiographically assessing the level of the ETT tip, consideration should be given to the
neonate's position. This is of particular importance in the extremely low-birth-weight infant. The ETT tip position
will vary based upon neutral versus lateral positioning of the head and upward versus downward tilt of the chin.
Lateral head and downward chin positioning each will result in the ETT tip located higher than neutral
positioning, while an upward chin tilt will yield a lower ETT position. Aside from determining the ETT tip position,
it is important radiographically to exclude bronchus intubation, esophageal intubation, and traumatic injury.
Bronchus intubation more commonly will occur into the right main bronchus, as it has a straight takeoff and
course relative to the distal trachea (Fig. 6.9). The more horizontal alignment of the left main bronchus creates
natural resistance should the ETT advance to the carina. With a right bronchus intubation, it is not uncommon for
the ETT to extend further into the bronchus intermedius and potentially the right lower or middle lobes (Fig.
6.10). Critical ancillary findings on a chest radiograph may include left lung and right upper lobe atelectasis.
Clinically, esophageal intubation should be readily detected. With ventilation, there will be an absence of chest
expansion, coinciding with a rise of the abdominal wall, a decrease in oxygen saturation, and an absence of
exhaled carbon dioxide (8). AP chest radiographic depiction of esophageal intubation is facilitated by positioning
the neonate's head to the side. By offsetting the anterior trachea from the posterior esophagus, the ETT will be
shown to course along the spine contralateral to the anterior chin.
During ETT insertion, mucosal tears or perforations to the pharynx or esophagus may occur. These traumatic
injuries may not always be recognized at the bedside. Injury may be suggested radiographically when the ETT
has an atypical course and location within the mediastinal silhouette. Perforation is diagnosed radiographically
when there is pneumomediastinum or subcutaneous emphysema following ETT insertion. Another manifestation
of airway injury is illustrated in Figure 6.11.

Vascular Catheters
Neonatal vascular access catheters that require radiologic imaging include UAC, umbilical venous catheters
(UVC), and percutaneously inserted central venous catheters (PICC). UACs are critical to monitoring the
neonate's physiologic parameters and making management decisions. An UAC enters the umbilical
P.63
artery and courses retrograde into an internal iliac artery and then into the ipsilateral common iliac artery to lie
either in the supramesenteric thoracoabdominal aorta (T6-11) or in the infrarenal abdominal aorta (L3-4).
Thoracic and lumbar spine landmarks are used radiographically to confirm appropriate positioning. UVC and
PICC lines are placed for delivery of fluids, medications, and parenteral nutrition in addition to taking blood for
laboratory analysis. UVCs are utilized in the early days following birth, while PICCs are used for more long-term
venous access solutions. UVCs course from the umbilical vein to left portal vein and then cross into the IVC via
the ductus venosus. The tip of the catheter ideally should be at the IVC-right atrial (RA) junction. This is readily
P.64
identified radiographically. PICC lines are typically inserted in a peripheral upper extremity brachial, basilic, or
less commonly a cephalic vein and course centrally to terminate at the SVC-RA junction. If the catheter cannot
be advanced to the SVC-RA junction, the ipsilateral brachiocephalic vein is also acceptable. When
radiographically assessing an upper extremity PICC line, it is important to keep in mind that the catheter tip
position will vary depending upon arm abduction and adduction and the accessed vein. PICC lines may also be
inserted via the common femoral vein. In this instance, the catheter courses centrally to terminate at the IVC-RA
junction.
FIGURE 6.8 A: Lateral view, peripheral intravenous central catheter PICC inserted in the saphenous vein
entering the vertebral venous plexus via an ascending lumbar vein. There is also an UAC in place. Note that the
malposition is rendered more difficult to detect because the PICC line is minimally radio-opaque. B: In this case,
the AP x-ray was interpreted as showing a well-placed UAC, despite the fact that the line was in the vertebral
venous system and not in the aorta, was minimally radiopaque, was of a caliber significantly smaller than an UAC
and did not follow a pelvic course typical of an UAC. If there is question consider low volume administration of
radio-opaque material and obtain lateral film. This infant suffered severe neurologic damage as the result of
receiving parenteral nutrition through the misplaced PICC for several days prior to recognition of the etiology of
the associated clinical deterioration. From MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures
in neonatology. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2013:209 & 170.

FIGURE 6.9 A: Radiograph suggesting that the ETT is in the right mainstem bronchus. Note the gaseous
distension of the stomach. The wavy tube over the right hemithorax is external. B: In the lateral view, the same
ETT is easily seen to be in the esophagus (arrowheads) posterior to the trachea (arrows). From MacDonald MG,
Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA: Lippincott Williams
& Wilkins, 2013:248.
FIGURE 6.10 Radiograph demonstrating an ETT malpositioned in the bronchus intermedius, with
resulting atelectasis of the right upper lobe and of the left lung. There is marked over aeration of the right
middle and lower lobes but no pneumothorax. From MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of
procedures in neonatology. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2013:247.

FIGURE 6.11 Radiographic magnification high-kilovoltage film (x2) demonstrating an abrupt cutoff of
the right bronchus intermedius (arrow) due to an endobronchial granuloma, with secondary volume
loss at the right lung base. Although these granulomas may be due to ETT trauma, in this area they are more
likely related to suction tube injury. The ETT is just entering the right bronchus. From MacDonald MG,
Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA: Lippincott Williams
& Wilkins, 2013:246.
FIGURE 6.12 UAC in pelvic artery. From MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures
in neonatology. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2013:166.

For each vascular access catheter, a radiograph may show coiling, retrograde looping, and inadvertent course at
any level from its cutaneous entry site to its target location. UACs can potentially track into mesenteric and renal
aortic branch arteries as well as the contralateral common iliac artery and lower extremity arterial tree (Fig. 6.12).
It is not uncommon for a UVC to course into the left or right portal vein and (Fig. 6.13). An upper extremity PICC
line could extend into an ipsilateral thoracodorsal
P.65
vein, internal jugular vein, contralateral brachiocephalic vein, or the azygous vein, while a lower extremity PICC
line could extend into a lumbar or renal vein (Fig. 6.8 A and B). If a radiograph shows that a left-sided upper
extremity PICC line extends into a persistent left SVC (L-SVC), retraction and use of the catheter from the mid L-
SVC is appropriate.

FIGURE 6.13 UVC in right portal vein with secondary air embolization into portal venous system. From
MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA:
Lippincott Williams & Wilkins, 2013:178.
FIGURE 6.14 Tube coiled in the oropharynx and upper esophagus simulating esophageal atresia. From
MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA:
Lippincott Williams & Wilkins, 2013:282.

Perforation, hemorrhage, in situ thrombosis, and thromboembolism may occur with placement of UACs, UVCs,
and PICC lines (Table 6.1). Venous catheters extending into the right cardiac chambers can lead to arrhythmias,
chamber perforation, and pericardial tamponade (Fig. 6.5). For suspected catheter-related vascular injuries,
duplex (i.e., gray-scale plus color) arterial or venous sonography should be performed emergently. As indicated
based upon the sonographic findings, low-dose CTA is the next diagnostic modality of choice.

Enteric Catheters
Enteric catheters include nasogastric, oral-gastric, and jejunal feeding tubes. Each requires to traverse the
pharynx and esophagus via either a nasal or oral cavity insertion. Resistance to advancement and catheter
looping or retrograde coiling may occur at any level even with a normal pharynx and upper GI tract.
Accordingly, radiographs for enteric catheters cover the neonate's neck, chest, and upper abdomen.

TABLE 6.1 Diagnosis of Vascular Thrombosis

Site Clinical Signs Diagnostic


Imaging

CVL-associated Malfunction of CVL, SVC syndrome, chylothorax,


venous thrombosis swelling, and livid discoloration of extremity,
dilatation of collateral veins over trunk or abdomen
in chronic cases

Inferior vena cava Lower limbs cool, cyanotic, edematous Contrast


thrombosis angiography

Superior vena Swelling of upper limbs and head, chylothorax Doppler


cava thrombosis ultrasonography
Renal vein Flank mass, hematuria, thrombocytopenia, Real-time 2D
thrombosis hypertension ultrasonography

Aortic or renal Systemic hypertension, hematuria, oliguria


arterial thrombosis

Peripheral or Pallor, coldness, weak or absent peripheral


central (aorta or pulse(s), discoloration, gangrene
iliac) arterial
thrombosis

Right atrial Congestive heart failure Echocardiography


thrombosis

Pulmonary Respiratory failure Lung perfusion


thromboembolism scan

CVL, central venous line; SVC, superior vena cava.

From MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology, 5th ed.
Philadelphia, PA: Lippincott Williams & Wilkins, 2013:225.

FIGURE 6.15 Feeding tube in the left main-stem bronchus. From MacDonald MG, Ramasethu J, Rais-
Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins,
2013:282.

As briefly discussed, the course and position of enteric catheters can indicate the presence of congenital
disorders. A fixed obstruction combined with a looped catheter in the pharynx or esophagus would indicate
esophageal atresia (Fig. 6.14). After coursing to the diaphragm, an enteric catheter with a final position over the
thorax would be highly suggestive for a diaphragmatic hernia, while a catheter coursing to the right upper
abdomen would suggest either situs inversus or heterotaxy. After traversing the stomach and entering the
duodenum, a feeding tube that remains ipsilateral in the abdomen and does not course back across the lumbar
spine would suggest bowel malrotation.
Radiographic interpretation of enteric catheters must also exclude tracheal insertion and traumatic injury.
Tracheal insertion is diagnosed radiographically when the catheter courses ipsilateral to the chin and within the
intrathoracic tracheal and right or left main bronchial silhouettes (Fig. 6.15). Traumatic injury to
P.66
the pharynx or esophagus during enteric catheter insertion may result in an atypical catheter location,
pneumomediastinum, subcutaneous emphysema, pleural effusion, or a combination thereof (Fig. 6.16). Tracheal
insertion could result in a traumatic pneumothorax and/or pneumomediastinum.

FIGURE 6.16 Chest radiograph showing esophageal perforation by an orogastric tube. From MacDonald
MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA: Lippincott
Williams & Wilkins, 2013:283.
FIGURE 6.17 A: AP radiograph demonstrates ventral air over the over the right hemidiaphragm and around the
heart (arrowheads). The sometimes difficult question of pneumothorax versus pneumomediastinum is answered
in the decubitus films. B: The left lateral decubitus film (right side up) shows that the right-sided gas is a
pneumothorax (arrowheads). C: The right decubitus film indicates that the adventitial air fails to completely cover
the lung and is located in the mediastinum (arrowheads). This important distinction is made obvious by the
decubitus films. From MacDonald MG, Ramasethu J, Rais-Bahrami K. Atlas of procedures in neonatology. 5th
ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2013:260.

Intercostal Catheters
Intercostal thoracic catheters (chest tubes) are placed in the neonate for management of pleural effusions and
pneumothoraces. Regardless of the source of pleural fluid (e.g., postoperative, chylous effusion, hemothorax),
the thoracic catheter is directed posteriorly for simple nonloculated effusions. The neonate will be in a supine
position and fluid will collect dependently. In distinction, a thoracic catheter is directed anteriorly for management
of a pneumothorax. Ideally, it is directed toward the anteroinferior medial pleural space, where most air will
collect in a supine neonate (Figs. 6.4 and 6.17). Initial and serial radiographs are obtained, monitoring catheter
positions and the amount of fluid or pleural air present. A persistent effusion or pneumothorax may suggest
catheter malposition, catheter dysfunction, or loculation ( Fig. 6.18). A persistent effusion or pneumothorax may
warrant thoracic ultrasound or CT to assess the catheter position, exclude loculations and septations, and plan
an appropriate intervention (Fig. 6.5).
P.67

FIGURE 6.18 The thoracostomy tube is completely outside the pleural space on this slightly oblique
chest film. Note that the long transpyloric tube is not in an appropriate position for feeding. Life support devices
may dislodge during transport for imaging or during imaging procedures. From MacDonald MG, Ramasethu J,
Rais-Bahrami K. Atlas of procedures in neonatology. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins,
2013:268.

Central Nervous System


Conditions in the neonatal central nervous system (CNS) warranting radiologic imaging include hypoxic-ischemic
injury (HII), ICH, trauma, congenital malformations, neurocutaneous disorders, congenital infections, masses, and
metabolic disorders. Utilization of imaging and radiologic findings vary depending upon the age of the neonate
and the presence of comorbid congenital disorders and physiologic stressors. For structural abnormalities
detected prenatally (e.g., Chiari malformation), brain and/or spine MRI may be the first radiologic test (see
Chapter 47, Fig. 47.5B). For many neonatal neurologic disorders with an initial postnatal presentation and
predictable imaging appearance (e.g., periventricular leukomalacia [PVL], intraventricular hemorrhage [IVH],
spinal dysraphism), evaluation begins with a transcranial or spinal ultrasound. For acute clinical presentations
and indeterminate pretest findings imaging may first proceed with CT (e.g., trauma) or MRI (e.g., profound
hypoxia). Following ultrasound or CT, further diagnostic assessment may require MRI. Surveillance imaging may
utilize ultrasound, CT, MRI, or a combination thereof, depending upon the pathology, prior radiologic findings,
comorbid disease, and clinical stability.

Hypoxic-Ischemic Injury
HII of the brain is defined as irreversible cellular and parenchymal brain damage resulting from cerebral
hypoperfusion and hypoxemia. The pattern of brain injury depicted by radiologic imaging will vary depending
upon the degree of brain maturity (e.g., preterm vs. term infant), duration of hypoxia, and timing of imaging (9).
In a term infant with mild-to-moderate ischemia, HII will be seen in the metabolically less active parasagittal
watershed cortex and subcortical white matter, as cerebral autoregulation seeks to preserve blood flow to the
more metabolically active regions of the brain (10), (see Chapter 46, Fig. 46.9A and B). With severe
hypoperfusion and ischemia, regulatory measures cannot adequately compensate flow to the high metabolic
regions. In this instance, the term infant will demonstrate HII in the deep gray matter (thalami, basal ganglia,
brainstem), cerebellum, perirolandic cortex, and corticospinal tracts, with progression to the global cortex and
white matter involvement as hypoxia persists (10).
In a preterm infant with mild-to-moderate hypoxia, HII manifests as PVL, in which there are cytokine-mediated
inflammation and cellular damage to the poorly vascularized, watershed periventricular white matter. Susceptible
regions are white matter dorsal and lateral to the lateral ventricles, centrum semiovale, optic radiations, and
acoustic radiations (11). Inflammation and tissue necrosis can lead to cavitation, cyst formation (e.g., cystic PVL),
and parenchymal destruction. Cysts may coalescence to form larger cysts. Alternatively, cysts may dehisce into
the adjacent ventricles forming porencephalic cysts. Progressive parenchymal destruction leads to loss of white
matter, secondary ex vacuo ventriculomegaly, and irregular margins of the ventricles (e.g., end-stage PVL). With
severe ischemia, in addition to possible PVL, there will likely be a HII pattern similar to term infantswith the
exception of less involvement of the basal ganglia and perirolandic cortex (11). Finally, as discussed in the next
section, a preterm infant with hypoxia of any degree may present with concomitant germinal matrix (GM) IVH.
Infrequently, HII in a neonate may present with obstructive arterial (e.g., middle cerebral artery) or venous
territory ischemia and/or infarction (Fig. 6.19). Underlying etiologies may be multifactorial. Common causes
include thromboembolism, coagulopathies, and volume depletion.
On ultrasound, PVL is depicted as hyperechoic white matter in the characteristic locations (see Chapter 46, Fig.
46.16). On MRI, the affected white matter will show T2 hyperintensity and diffusion weighted image (DWI)-
restricted diffusion. Cysts, white matter volume loss, ventriculomegaly, and irregular ventricular margins are all
readily demonstrated by ultrasound, MRI, and CT (see Chapter 46, Figs. 46.12, 46.14, 46.15, 46.16, 46.17,
46.18, 46.19 and 46.20). Other patterns of HII will manifest on ultrasound as nonspecific increased echogenicity.
The location and pattern of restricted diffusion and T2 hyperintensity on MRI will be required to accurately
diagnose the HII and assess its severity.

Intracranial Hemorrhage
ICH in a neonate is an emergent condition for which imaging serves a critical role in diagnosis and management.
Underlying ICH etiologies and ancillary clinical considerations differ for the preterm versus term infant, leading to
different imaging strategies.
For a preterm infant (<34 weeks), ICH pathogenesis is fundamental to imaging strategies and interpreting
radiologic exams. Most commonly, preterm ICH presents as GM IVH with or without hemorrhagic PVL and/or
isolated ischemia-related intraparenchymal hemorrhage (IPH). The GM is a highly vascularized region in the
subependymal lateral ventricles (adjacent to the caudate, thalamus, and caudate-thalamic groove) containing
proliferating neuron and glial cell precursors. Its fragile capillaries are highly susceptible to physiologic stressors
(e.g., hypoxia, hypertension, hypotension, metabolic acidosis, reperfusion) that impact cerebral blood flow
autoregulation. Depending upon the immaturity of the GM capillaries and the severity and duration of physiologic
stress, the capillaries can rupture with blood either confined to the GM or spreading within the ventricular
system. Coagulopathy and fibrinolytic abnormalities are additional risk factors compounding a GM hemorrhage
(11).
Thrombus formation following GM hemorrhage can obstruct flow of cerebrospinal fluid, leading to hydrocephalus.
Venous obstruction may also occur, leading to periventricular white matter ischemia and hemorrhagic infarctions
(e.g., hemorrhagic PVL). A periventricular white matter hemorrhage will often lead to
P.68
porencephaly, similar to PVL without hemorrhage. When cerebral ischemia is prolonged and severe,
parenchymal arterial hemorrhagic infarctions may also occur, leading to IPH remote from IVH and hemorrhagic
PVL.
FIGURE 6.19 A: Computed tomographic scan of a full-term newborn with acute, focal infarction involving the
territory of the left middle cerebral artery. B: CT scan of an infant at 18 months of age showing porencephalic
cyst in location of earlier cerebral infarction. C: MRI of infant performed at age 2 years with abnormal signal in
location of infarction. Note that, if available, MRI would have been the imaging modality of choice for images A
& B to minimize radiation exposure.

Head ultrasound is performed in the preterm infant during the first week of life to screen for IVH. Timing (e.g., day
1, 3 or 7) of the ultrasound will depend upon the clinical course and risk factors. Blood is depicted as coalescent
echogenicity within the GM, ventricle, parenchyma, or a combination thereof and graded as I-IV. Follow-up
imaging proceeds with ultrasound, CT, and/or MRI as dictated by the initial imaging findings and clinical course.
If no IVH is detected during the initial screening period, additional surveillance imaging is typically performed with
ultrasound. However, the subsequent clinical course and comorbid neurologic clinical pathophysiology may
demand CT or MRI.
In the term infant, ICH is most commonly related to trauma. Other less common etiologies include hemorrhagic
transformation of a cerebral infarction (e.g., middle cerebral artery territory) and a rare aggressive, malignant
congenital intracranial tumor. It is important to keep in mind that while these causes of ICH are infrequent in the
preterm infant, they nonetheless can occur in a preterm infant.
With regard to traumatic injury, ICH may be accidental or non-accidental. Depending on the mechanism of head
injury, a supraor infratentorial subdural hematoma (SDH) or less commonly an epidural hematoma may present
with a concomitant subarachnoid
P.69
hemorrhage (SAH), IPH, parenchymal contusion, scalp hematoma, and/or a skull fracture. When there is
nonaccidental trauma (NAT), an SDH may present with concomitant HHI and SAH. As birth trauma and child
abuse (e.g., shaken baby) are the two main causes of NAT SDH, clinical history and potentially, additional
imaging (e.g., skeletal radiographs), are mandatory.
CT is the initial modality of choice to accurately and timely diagnose a traumatic ICH and associated potential
fractures, mass effect, and midline shift. MRI is useful to age hemorrhage (e.g., NAT) and identify other potential
injuries (e.g., HII). To minimize radiation exposure, MRI should be used in conjunction with CT to monitor the
injury and the response to therapy. Alternatively, ultrasound can be performed to monitor a moderate-tolarge
SDH (echogenic extra-axial collection) and potential mass effect and shift.

Congenital Malformations
CNS developmental anomalies warranting imaging in the neonatal period include neural tube defects (open and
closed), midline malformations, posterior fossa abnormalities, and neuronal migration abnormalities. In many
presentations, antenatal diagnosis has been established by prenatal ultrasound and MRI. In other instances,
diagnosis is first suspected following birth, by a combination of the clinical presentation and an initial ultrasound
exam. In both scenarios, postnatal MRI with multiplanar T1- and T2-weighted sequences in addition to DWI and
DTI sequences provides the most comprehensive approach for evaluating and accurately characterizing these
anomalies. CT may complement the workup, by providing a more detailed depiction of potential concomitant
defects involving the calvarium and/or skull base (e.g., cephalocele).

Neurocutaneous Disorders (Phakomatoses)


Among the large number of neurocutaneous disorders, tuberous sclerosis complex (TSC), Sturge-Weber
syndrome, and PHACE syndrome commonly lead to radiologic imaging in a neonate.
TSC is an autosomal dominant multisystem disorder. Intracranial manifestations in a neonate include cortical
tubers, subependymal hamartomas, subependymal giant cell tumors, and white matter lesions (12). Early
calcification may be present in the subependymal hamartomas. It is the extracranial manifestations of disease,
however, that typically lead to a suspected diagnosis. These include cardiac rhabdomyomas, arterial aneurysms
and stenotic-occlusive disease, renal cysts, and rarely retinal hamartomas and cutaneous lesions (e.g., adenoma
sebaceum) (13,14). Brain MRI is recommended as the primary modality consideration. CT is a secondary
consideration for what is typically a nonemergent workup. If there is a known cardiac rhabdomyoma or
concomitant vascular disease (e.g., from prior echocardiography or vascular duplex sonography), cardiovascular
MR or CT should also be considered.
Sturge-Weber syndrome is defined by a cutaneous capillary malformation (in the trigeminal nerve distribution)
and an intracranial leptomeningeal capillary-venous malformation, associated with enlargement of the ipsilateral
choroid plexus and deep medullary veins (15). Involvement is typically unilateral. Additional features include
gyriform cortical calcifications, brain hemiatrophy, and skull thickening. However, these are not typical in a
neonate. MRI with arterial- and venous-phase MRA provides a comprehensive assessment of the intracranial
vascular malformation. Head CT without contrast followed by CTA is also an option.
PHACE syndrome is composed of Posterior fossa malformations (e.g., Dandy-Walker malformations),
Hemangiomas, Arterial anomalies (e.g., cervical and cerebral agenesis, hypoplasia, or dolichoectasia), Cardiac
anomalies and Coarctation of the aorta, Eye anomalies, and ventral, midline developmental defects. Head and
neck infantile hemangiomas are commonly found; intracranial, thoracic, and extremity hemangiomas are also
possible. Multisystem MRI with MRA is the modality of choice for complete cardiovascular and noncardiovascular
evaluation. Low-dose multilevel CTA is also a consideration (16).

Congenital Infections
Transplacental and transvaginal transmitted infections that can impact the CNS include Toxoplasmosis gondii ,
Rubella, Cytomegalovirus, and Herpesvirus (TORCH infections). Neurologic manifestation of disease may
include intrauterine growth restrictions, microcephaly, seizures, low muscle tone, and sensorineural hearing loss.
Other systemic nonneurologic manifestations may also occur (e.g., bone, cardiac, and ophthalmic congenital
abnormalities). Key findings identified on CT or MRI are calcifications, cortical malformations, white matter
lesions, volume loss, hydrocephalus, and ex vacuo dilatation of the ventricles secondary to volume loss. The
pattern and constellation of these findings along with cultures, titers, and other nonneurologic imaging findings
will support the diagnosis (17).

Congenital Masses
Benign and malignant congenital masses in the brain or spine occur infrequently in a neonate. In some
instances, prenatal ultrasound and MRI have detected a mass. Postnatal ultrasound of the brain and/or spine
can be performed at the bedside to confirm the mass and evaluate for potential secondary complications (e.g.,
hydrocephalus) and associated anomalies (e.g., anorectal and genital malformations, spinal dysraphism in the
setting of sacrococcygeal teratoma). In other instances, nonspecific symptoms (e.g., increased head
circumference, vomiting, seizures; hypotonia, constipation) may lead to diagnostic evaluation. MRI or CT with
contrast is necessary for diagnosis. Radiologic diagnosis is based upon the location and imaging features of the
mass.
Common benign congenital cystic lesions in a neonate include arachnoid cyst, choroid plexus cyst (CPC),
epidermoid, and dermoid. Arachnoid cysts (filled with clear cerebrospinal fluid) will appear anechoic on
ultrasound, hypointense on CT, and T1 hypointense, T2 hyperintense on MRI. No enhancement will be present.
When these extra-axial cysts (e.g., middle cranial fossa, suprasellar cistern, posterior fossa) are of sufficient size,
localized compression on the developing brain and possibly secondary calvarial developmental scalloping will
occur. A CPC (e.g., lateral ventricular atria) is also sonolucent, but may have an echogenic rim, secondary to
surrounding choroid plexus. On CT, the CPC (with lipid laden histiocytes) will appear iso- to hyperdense and on
MRI, have iso- to hyperintense T1 and hyperintense T2 signal. Rim or nodular enhancement may be seen.
Epidermoids (composed of cellular debris, keratin, water, and cholesterol) are hypointense on CT and iso- to
slightly hyperintense on T1- and T2-weighted MR imaging, reflecting the fluid and lipid contents. Calcification
infrequently occurs; CT will more readily depict this calcification. Although not common, rim enhancement may be
seen on MRI. While an intracranial off-midline location is most prevalent (e.g., cerebellopontine angle, fourth
ventricle, and sellar-parasellar locations), an epidermoid may less commonly develop in the spine. An intracranial
dermoid is almost exclusively midline (e.g., sellar, parasellar, frontonasal, midline posterior fossa). In distinction
to the other intracranial cysts, it is composed of primarily of fat (e.g., liquid cholesterol) and dermal appendages;
its wall is thick and often calcified. The lesion will have fat attenuation on CT and have hyperintense T1 and
heterogeneous hypo- to hyperintense T2 signal on MRI. These lesions are typically uncomplicated in a neonate
and, as such, show no enhancement. In both epidermoid and dermoid, a dermal sinus tract is possible. This can
be defined both on CT and MRI (18).
Teratomas are the most common congenital brain and spine tumors arising in a neonate. Other common
neonatal brain tumors are primitive neuroectodermal tumors, astrocytomas, glioblastoma
P.70
multiforme, and choroid plexus papilloma. Primary intraspinal (extradural) neuroblastoma may also be
encountered in a neonate. The majority of intracranial tumors in a neonate are supratentorial; symptoms and
secondary imaging findings (e.g., midline shift, hydrocephalus) will be reflective for the tumor size and degree of
compression and displacement of these structures. Teratomas most commonly are sacrococcygeal teratoma
(SCT) but may also present as intraspinal, extradural masses in the lumbar and thoracic spine. Imaging of spinal
tumors also aims to define secondary sequelae (e.g., localized mass effect and abdominopelvic extension in a
SCT). Primary pre- and postcontrast primary radiologic characteristics for brain and spine congenital tumors will
be dependent upon the tumor histology. The majority of teratomas (with ectoderm, mesoderm, and endoderm
germ layers) have benign, mature histology, presenting as a cystic lesion containing fat, fluid, soft tissue, and
calciumall readily distinguished on ultrasound, MRI, and CT. Less commonly, teratomas will have immature
cellular neuroectodermal elements and present as a heterogeneous solid mass (e.g., intracranial teratoma). In a
similar manner to these malignant teratomas, CT density, MRI signal, and enhancement pattern of the
intracranial neuroepithelial tumors will vary based upon the degree of tumor cellularity and tissue necrosis.
Tumor vascularity, arterial supply, and venous drainage can all readily be defined with MRI and CT to assist
surgical planning (19).

Metabolic Disorders
Metabolic encephalopathy in a neonate may result as a by-product from primary and secondary metabolic
pathway dysfunctions: metabolic acidosis (e.g., nonketotic hyperglycinemia; renal failure), hyperammonemia
(e.g., ornithine transcarbamylase deficiency; liver disease), or hypoglycemia (e.g., hepatic and mitochondrial
enzyme deficiencies; nesidioblastosis). Recognition of the metabolic derangement and diagnosis of the specific
etiology are typically made clinically, based upon presenting symptoms, other organ involvements, and
laboratory analysis (e.g., enzyme assays). However, brain MRI with spectroscopy and DWI can assist in the
diagnosis (e.g., elevated glycine peak on spectroscopy) and can detect brain parenchymal abnormalities (e.g.,
restricted diffusion, edema, myelinopathy) contributing to prompt and accurate treatmentparticularly for the
primary inborn errors of metabolism.

Pulmonary System
Disorders affecting the neonatal pulmonary system warranting radiologic investigations encompass an array of
congenital and acquired pathology. Chest radiography and CT are critical to their diagnoses and management.

Developmental
Developmental anatomical abnormalities impacting neonatal pulmonary function include agenesis, hypoplasia,
bronchopulmonary foregut malformations, and CDH (9,20). Unilateral pulmonary agenesis with bronchovascular
atresia is a primary developmental abnormality, often associated with other congenital anomalies. Chest
radiography will show complete hemithorax opacification with ipsilateral cardiomediastinal shift. Pulmonary
hypoplasia has multiple possible extrinsic etiologies leading to abnormal pulmonary development, in addition to
intrinsic developmental errors. These include in utero renal agenesis and oligohydramnios (e.g., Potter
syndrome), neuromuscular disorders, skeletal dysplasias (leading to a small thorax), and CDH. A postnatal chest
radiograph will demonstrate reduced unilateral or bilateral lung volumes associated with hemidiaphragm
elevation and rib crowding. If unilateral, ipsilateral cardiomediastinal shift will also be present. Bronchopulmonary
foregut malformations are a spectrum of tracheobronchial tree developmental anomalies including bronchial
atresia, congenital lobar hyperinflation, bronchial cysts, congenital pulmonary airway malformation, and
sequestration. CDH is a diaphragmatic developmental defect leading to herniation of abdominal viscera and
mesentery into the thoracic cavity. Chest radiography may demonstrate ipsilateral chest opacification,
contralateral cardiomediastinal shift, and decreased or absent intra-abdominal bowel gas. With a left CDH and
gastric herniation, an enteric tube will overly the left hemithorax. When air has reached bowel loops, herniation of
bowel loops will readily be identified in the chest (see Chapter 41, Fig. 41.16).

Surfactant Deficiency Disease


Surfactant deficiency disease (SDD) is a disorder in which there is either abnormal surfactant production (e.g.,
primary SDD) or impaired surfactant function (e.g., secondary SDD) leading to neonatal respiratory distress.
Primary SDD is typically found in a preterm infant while secondary SSD is encountered in a term infant who may
be exposed to perinatal asphyxia, aspiration (e.g., meconium) pneumonitis, and/or infection. The classic
radiographic features are ground-glass reticulogranular interstitial opacities with low lung volumes, atelectasis,
and air bronchograms (see Chapter 26, Fig. 26.4). The radiographic appearance may vary depending upon
severity of disease and secondary risk factors (e.g., aspiration, pneumonia).

Acquired Pulmonary Pathology


Transient tachypnea of the newborn (TTN), aspiration, pneumonia, pulmonary hemorrhage, chronic lung
disease/bronchopulmonary dysplasia (CLD/BPD) (10), pleural effusions (11), and air leaks are important
acquired pathologies that are primarily diagnosed and monitored with chest radiography (21,22). Depending
upon the clinical course of disease and response to treatments, further management decisions for pneumonias,
CLD, pleural effusions, and air leaks may require supplemental imaging (e.g., ultrasound or CT).

TTNprolonged resorption of physiologic fetal fluid may result in short-term respiratory distress (e.g., 24 to 72
hours). Chest radiography is distinguished by interstitial to alveolar pulmonary edema, hyperinflation, pleural
fluid (fissural vs. small dependent effusions), and potentially cardiomegaly. Follow-up radiographs will show
clearance to resolution in a peripheral to central and upper to lower lung zone pattern, corresponding to
clinical improvement.
Aspirationintrauterine stress (e.g., asphyxia, sepsis) may lead to aspiration of amniotic fluid into the infant
lung, most commonly with meconium (meconium aspiration syndrome, MAS). Radiographically, simple
amniotic fluid aspiration may manifest as interstitial or evolving air space opacities. MAS has typical
radiographic features (see Chapter 26, Fig. 26.6). Airway obstruction with elevated alveolar tension and
chemical pneumonitis leads to hyperinflation, air trapping, subsegmental atelectasis, coarse opacities, and
potentially air leaks. Secondary SDD most typically also occurs, contributing to coalescent multifocal opacities
and complete parenchymal opacification.
Pneumoniaexposure to pathogens in utero (e.g., TORCH infections), delivery (e.g., group B streptococcus),
and postdelivery (rhinovirus, respiratory syncytial virus, influenza, enterovirus; staphylococcus, Escherichia
coli , enterococcus; Candida) leads to potential early versus late neonatal presentations of pulmonary
infection. Chest radiographs may show granular, coarse, or fine interstitial patterns, often indistinguishable
from SSD, MAS, or TTN, respectively. Patchy distributions, persistent or progressive opacities, hyperinflation,
and pleural effusions can aid in radiologic diagnosis of pneumonia.
Pulmonary hemorrhagerisk factors include respiratory stress and states of high pulmonary blood flow (e.g.,
PDA). Chest radiography will show progressive coalescent air-space opacification, potentially evolving to
complete lung opacification, depending upon the severity of hemorrhage.
P.71
CLDmechanical ventilation pressure, high oxygen tension, and low surfactant in high-risk neonates with
immature lungs (e.g., preterm) can lead to inflammation with secondary irreversible bronchopulmonary
damage, bronchial stenosis, and parenchymal cysts and fibrosis (bronchopulmonary dysplasia). Pulmonary
interstitial emphysema may be seen during mechanical ventilation and its presence increases the likelihood of
subsequent development of BPD (see Chapter 26, Fig. 26.9). Clinically, the neonate will require supplemental
oxygen for at least 28 days, to a corrected age of 36 weeks (21). Radiography can confirm the diagnosis:
coarse reticular interstitial opacities, cystic lucencies, hyperinflation, and heterogeneous aeration with
atelectasis. CT 3D airway reconstructions (e.g., virtual bronchoscopy) is useful to define extent of
parenchymal disease (see Chapter 27, Fig. 27.4A and B) and noninvasively define bronchial stenoses with
potential tracheomegaly.
Pleural effusionsfluid in the pleural space in a neonate may be congenital (e.g., hydrops, chylothorax) or
acquired (e.g., iatrogenic injury, postoperative, infection, heart failure) (22). Radiography may show blunted
costophrenic sulci, hazy lung field attenuation, and obscured ipsilateral hemidiaphragms, associated with
ipsilateral partial to complete atelectasis and contralateral mediastinal shift, depending upon the size of the
effusion(s) and underlying cardiopulmonary conditions. Ultrasound is useful to confirm an effusion, while both
ultrasound and CT can be utilized to assess for septation and loculation.
Air leak syndromebaro- and volutrauma may cause air to leak outside the tracheobronchial tree and alveoli. Air
tracks and localizes, potentially presenting as pulmonary interstitial emphysema, pneumothorax,
pneumomediastinum, pneumoperitoneum, and pneumopericardium (see Chapter 26, Figs. 26.10, 26.11, and
26.12). Supplemental lateral and decubitus radiographs may be required for diagnoses.

Cardiovascular System
Neonatal cardiac pathology includes pericardial, myocardial, and cardiac chamber structural abnormalities.
Cardiac chamber structural abnormalities can be subdivided into right-sided obstructive lesions, left-sided
obstructive lesions, left-to-right shunt lesions, and morphologic admixture lesions. Vascular disorders include
those abnormalities involving the coronary and peripheral arterial and venous systems. The majority of these
disorders in the neonate have an underlying congenital etiology. For cardiac pathology, chest radiography and
echocardiography are first tiers of diagnostic imaging, while for vascular disorders, it is most typically vascular
duplex sonography and radiography targeted to the clinical region of concern (e.g., chest, abdomen, or
extremity). MRI-MRA and CTA are secondary modality considerations for diagnostic imaging of the
cardiovascular system.

Pericardium
Pericardial pathology in the neonate includes congenital absence of the pericardium (CAP), pericardial cysts,
pericardial effusions, and pneumopericardium. The radiographic appearance for CAP will vary depending upon
whether it is partial or complete and unilateral or bilateral. Chest radiography may show shifts in the cardiac
position. (e.g., levoposition with upward rotation in left complete absence), hilar bulging (e.g., protrusion of main
pulmonary artery, left atrium, or left atrial appendage), or lung interposed with cardiovascular structures and/or
the diaphragm. Echocardiography and as-needed MRI or CT serve important roles to confirm CAP, evaluate for
associated anomalies, and exclude herniation and strangulation. A pericardial cyst is typically an incidental
finding in a neonate; it is detected while imaging for other disease processes. Chest radiography demonstrates a
paracardiac mass or mass-like opacity, while echocardiography, MRI, and CT will show a fluid-filled,
hypovascular, well-circumscribed paracardiac mass. Pericardial effusions (e.g., fluid in the pericardial space)
most commonly are seen postoperatively. Less commonly, neonatal congestive heart failure (e.g.,
cardiomyopathy) and iatrogenic trauma (e.g., central line perforation) may result in effusions. Radiography will
show a globular enlarged cardiomediastinal silhouette with extension to the aortic arch and obscuration of hilar
structures. Echocardiography, MRI, and CT will show widening of the pericardial space with fluid characteristics
(Figs. 6.5 and 6.6). Pneumopericardium (e.g., air in the pericardial space) may be seen postoperatively or as part
of an air leak syndrome in a mechanically ventilated preterm neonate. Radiography will demonstrate a
radiolucent halo (halo sign) surrounding the heart, crossing the hemidiaphragms and extending to but not
beyond the superior margin of the aortic arch (see Chapter 26, Fig. 26.12).

Myocardium
Cardiomyopathies and cardiac tumors are two main groups of pathologies in which the myocardium may be
abnormal in the neonate. For cardiomyopathies, imaging findings will vary depending upon the type, degree of
cardiac dysfunction, and comorbid pathology. Chest radiography may show regional or global cardiomegaly,
pulmonary venous congestion (PVC), pleural effusions, and a pericardial effusion. Echocardiography and as-
needed MRI serve important diagnostic roles to distinguish the type of cardiomyopathy, assess function and the
severity of disease, and recognize comorbid congenital anomalies.
Cardiac tumors are rare in the neonate, with the majority being benign. The most common tumor is a
rhabdomyoma; others include teratoma, fibroma, vascular tumors, and myxoma. Depending upon the tumor
location and degree of intracardiac obstruction, chest radiographs may show oligemia (e.g., right outflow tract
obstruction) or PVC (e.g., left outflow tract obstruction); heart size may be normal or enlarged. Echocardiography
is often the first modality performed following chest radiography and can detect a neonatal cardiac tumor with
high sensitivity. MRI (or alternatively CT) is often required for more detailed tumor characterization along with
assessment of myocardial function.

Right-Sided Intracardiac Obstructive Lesions


This group of CHD lesions includes Ebstein anomaly (see Chapter 30, Fig. 30.29), tricuspid atresia, tetralogy of
Fallot, and pulmonary atresia and stenosis. They are distinguished by a mechanical or functional obstruction
involving the tricuspid valve, right ventricular outflow tract (infundibulum, RVOT), pulmonary valve, or a
combination thereof. The obstruction leads to diminished antegrade pulmonary blood flow, accentuated by an
obligatory intracardiac right-to-left shunt. These lesions are distinguished radiographically by oligemia and
variable degrees of cardiomegaly. Echocardiography, MRI, and CT offer direct characterization of the lesions.
Pressure gradients across the structural obstructions can be quantified with echocardiography and MRI.

Left-Sided Intracardiac Obstructive Lesions


Left-sided obstructive cardiac lesions include cor triatriatum, congenital mitral stenosis and atresia, hypoplastic
left heart syndrome, and congenital aortic stenosis. These lesions result in decreased systemic aortic perfusion.
An intracardiac left-to-right shunt may be present to unload the left heart. Radiographically, all will demonstrate
variable PVC. Heart size may range from normal to markedly enlarged. If an intracardiac shunt is present, there
may be a component of increased pulmonary vascularity. Echocardiography will define the lesion with high
sensitivity and specificity. MRI and CT can also define the structural lesion.
P.72
Left-to-Right Shunt Lesions
Excessive shunting of blood from the left-to-right heart circulation in the neonate may occur independently in the
setting of intra- and extracardiac shunt lesions. Intracardiac shunts include atrial and ventricular septal defects
and a PDA. Intrathoracic, extracardiac shunts include pulmonary venous anomalies. Shunting leads to increased
pulmonary blood flow, coinciding with the fall in pulmonary vascular resistance. The ensuing respiratory distress
and tachypnea (without cyanosis) will lead to a chest radiograph which is distinguished by variable
cardiomegaly, central pulmonary artery enlargement, and pulmonary overcirculation, dependent upon the
location and size of the shunt. Radiographic analysis of the enlarged cardiac chambers and pulmonary vein
silhouette often leads to a reliable working differential diagnosis. Diagnosis is typically confirmed with
echocardiography (e.g., intrathoracic shunts) or peripheral vascular ultrasound (e.g., extrathoracic shunts). MRI
or CTA are useful to evaluate the complete pulmonary venous drainage pattern. Cardiac MRI is particularly
useful to quantify the hemodynamic severity of the shunt.

Morphologic Admixture Lesions


This group of CHD lesions are defined by structural abnormalities that lead to direct admixture of deoxygenated
and oxygenated blood. Cyanosis is the hallmark as a result of the admixture. Lesions include double-inlet
ventricle (single ventricle), transposition of the great arteries, double-outlet right ventricle, and truncus arteriosus.
Associated septal, pulmonary, aortic, and coronary anomalies may be present, depending upon the lesion.
Echocardiography can define the primary and associated congenital lesions with reliable detail. Cardiac MRI and
CTA may be indicated to further assess anatomy and associated anomalies. MRI may also be indicated to
quantify ventricular and valvular function and ventricular volumes for operative planning.

Coronary Artery Anomalies


There is a wide range of possible anomalous origins for the coronary arteries. Some do not impact coronary
perfusion while others lead to diminished coronary artery flow. Among the latter, anomalous right (ARCAPA) and
left (ALCAPA) coronary arteries arising from the pulmonary artery may present in a neonate. Potential symptoms
include feeding intolerance, irritability, pallor, respiratory difficulty, and congestive heart failure. Chest
radiographs will demonstrate cardiomegaly and PVC. Diagnosis and recognition of associated anomalies can be
acheived with echocardiography, ultra-low-dose CTA, or MRI. Rarely, Kawasaki disease may present in a
neonate. Following echocardiography, ultra-low-dose CTA is recommended for complete evaluation of the
coronary artery caliber, lumen, and wall and exclusion of coronary aneurysms, stenoses, and wall thickening.

Pulmonary Vascular Pathology


Imaging of the pulmonary arteries in the neonate is indicated for suspected unilateral aplasia or hypoplasia,
pulmonary arteriovenous malformation (PAVM), pulmonary embolism, and pulmonary hypertension (PHTN) (23).
The developmental aplastic and hypoplastic anomalies may be defined by echocardiography, MRI-MRA, and/or
CT pulmonary angiography (CTPA). A PAVM will result in a right-to-left shunt and decreased oxygenation. High-
resolution CT and MR angiography can readily define the nidus, inflow, and outflow. Pulmonary embolism in a
neonate most commonly is iatrogenic related to venous catheters and thromboembolism. Depending upon the
clinical presentation and presence of venous catheters, diagnostic evaluation should proceed with duplex
venography and/or ultra-low-dose CTPA. PHTN may be primary (e.g., idiopathic) or secondary (e.g., MAS,
CHD). Echocardiography is indicated to exclude CHD, define the central pulmonary vasculature, and assess
right heart strain and pulmonary pressure. CTPA and MRA are excellent means to thoroughly evaluate central
and peripheral pulmonary arteries and veins and morphologic criteria for right heart strain. MRI can also evaluate
physiologic hemodynamics to support the diagnosis of PHTN. Invasive catheter angiography should be reserved
for direct physiologic interrogation and instances where cardiopulmonary anatomy is not well defined by the
other modalities.

Aorta and Branch Arteries

Obstructive Aortic Disease


Neonatal obstructive aortic disorders can be categorized into three groups. The first includes pathologies in
which there is intrinsic aortic arch luminal obstructionaortic arch hypoplasia, interruption of the aortic arch, and
coarctation. These lesions result primarily in decreased systemic-aortic perfusion, similar to cardiac left-sided
obstructive lesions. Intracardiac CHD may also be present, including septal defects with left-to-right shunting.
The neonatal appearance on chest radiography may be indistinguishable from other left-sided obstructive CHD,
namely cardiomegaly and PVC with or without increased pulmonary vascularity. Echocardiography will define the
obstructive arch lesion with high sensitivity and specificity. MRI or CT may be obtained for more comprehensive
evaluation of concomitant CHD (see Chapter 30, Figs. 30.35). Echocardiography and MRI are useful to quantify
the pressure gradient across the obstruction.
The second group of obstructive aortic disease includes anomalies in which the aortic arch, aortic arches, aortic
branch arteries, pulmonary branch arteries, ductus arteriosus, and/or ligamentum arteriosum surround and either
compress or efface structures extrinsic to the thoracic aortafor example, trachea, esophagus, or both. These
lesions include vascular rings (complete or incomplete), pulmonary slings, and innominate artery compression.
The anomalous arch anatomy most commonly is a double aortic arch or right-sided aortic arch; less commonly a
left aortic or cervical aortic arch. Radiographic detection of obstructive aortic arch anomalies requires recognition
whether the aortic arch is right or left sided and the level and location of tracheal narrowing. Air trapping is a key
supportive finding. Echocardiography will demonstrate whether the aortic arch is right or left sided and the
branch artery origins, but may not completely depict distal arterial segments nor tracheal morphology. MR or
CTA can provide definitive comprehensive diagnoses and are particularly useful to exclude potential
tracheomalacia and tracheal rings. Rarely, feeding intolerance predominates as a presenting symptom. In this
instance, an esophagram may initially be obtained. Recognition of esophageal effacement raises the suggestion
for a vascular ring or pulmonary sling. Further evaluation should proceed with MR or CTA.
The third group of aortic obstructions is midaortic stenosis, in which there is intrinsic obstruction of the abdominal
aorta. MAS may be primary or secondary (e.g., fibromuscular dysplasia, neurofibromatosis Type 1, tuberous
sclerosis, William syndrome, arterial calcification of infancy). Clinical presentation includes hypertension and
potentially mesenteric and lower extremity ischemia. Duplex sonography, MRA, or CTA will demonstrate diffuse
or segmental narrowing of the abdominal aorta, often with involvement of the renal arteries. Celiac axis and
superior mesenteric artery involvement occurs less frequently. Surveillance of other vascular territories may be
required, depending upon the diagnosis (e.g., fibromuscular dysplasia, tuberous sclerosis, William syndrome,
arterial calcification of infancy).

Aneurysmal Disease
Aortic and branch artery aneurysmal disease rarely presents in a neonate. Common congenital considerations
include Marfan, Ehlers-Danlos, and Loeys-Dietz syndromes in which there are abnormal vessel collagen, elastin,
and/or smooth muscle development and arrangement. Marfan syndrome commonly manifests as aortic root
P.73
aneurysmal enlargement with associated aortic insufficiency, Ehlers-Danlos with aortic and branch artery
aneurysms and dissections, and Loeys-Dietz with tortuous, ectatic and aneurysmal large and medium arteries.
Other differential considerations for a neonate with an arterial aneurysm include UAC iatrogenic injury with a
pseudoaneurysm and a mycotic aneurysm. Echocardiography, cardiac MRI, and vascular MRA or CTA may be
required for complete diagnostic evaluation. For the congenital aortopathies, findings should be correlated with
clinical phenotypes and genetic testing.

Extremity Vasculature
Indications for radiologic evaluation of neonatal extremity vasculature include ischemia (e.g., UAC iatrogenic
arterial injury), deep venous thrombosis (e.g., venous catheter related), and extremity malformations. Duplex
sonography is the initial modality of choice followed by potentially CT or MR angiography, dependent upon
sonographic findings and management. In evaluating for lower extremity arterial ischemia, evaluation of the aorta
is also recommended (in addition to inflow and outflow arteries) to exclude concomitant aortic injury and
thrombus. In the evaluation of lower extremity venous and iliocaval thrombus, attention should be given to the
renal veins to exclude concomitant involvement.

Vascular Malformations and Vascular Tumors


Vascular malformations are composed of low-flow capillary, venous, and lymphatic malformations (AVM) and
high-flow arteriovenous fistulas and malformations. Most common presentations in a neonate are (a) lymphatic
malformations, such as a cystic hygroma in the neck, and (b) visceral (e.g., Vein of Galen malformation, Fig.
47.11) and extremity high-flow AVM. Vascular tumors that may present in a neonate include congenital and
infantile hemangiomas, hepatic hemangioendothelioma, and kaposiform hemangioendothelioma (KHE).
Postnatal imaging strategies for vascular malformations and tumors primarily focus on radiography, duplex
sonography, and MRI-MRA; however, CTA can also be utilized. On chest radiography, a large neck hygroma will
result in extrathoracic tracheal deviation and neck enlargement. Extracardiac, extrathoracic, high-flow vascular
malformations and tumors may cause significant left-to-right shunting with ensuing cardiomegaly, central
pulmonary enlargement, and pulmonary overcirculation indistinguishable from intrathoracic shunts.

Genitourinary System
Neonatal urogenital imaging may be warranted based upon prenatal clinical findings (e.g., fetal hydronephrosis)
and diagnoses, postnatal pathophysiology, or a combination thereof. Radiologic anatomical and functional
evaluations are essential to investigating developmental abnormalities, cystic kidney disease, obstructive
uropathy, infection, nephrolithiasis, renal masses, and adrenal hemorrhage. Ultrasound is the most important first
step in direct radiologic evaluation. A VCUG is indicated to exclude vesicoureteral reflux and lower urinary tract
obstructions (Fig. 6.7A and B). Based upon the initial clinical and imaging evaluations, magnetic resonance
urography may be indicated to anatomically and functionally assess the complete urogenital system. CT is used
selectively for anatomical urogenital evaluations while renal scintigraphy is reserved for functional evaluation of
the kidneys (See Chapter 40).

Developmental Abnormalities
Embryologic development of the kidney and upper and lower collecting system is a coordinated process in which
a ureteral bud and mesodermal cells form a primitive metanephros in the pelvis with a ventral hilum (24). The
developing kidney undergoes relative ascent to the expected retroperitoneal fossa with 90-degree rotation. The
bladder develops from ventral cloaca; during fetal development, the allantois (caudal end of the yolk sac)
connects the bladder to the umbilicus. Abnormal progression of urogenital development can lead to a spectrum
of anomalies including renal agenesis, hypoplasia, and dysplasia; ureteropelvic duplication; renal ectopia and
fusion; urachal anomalies; and the bladder extrophy-epispadia complex (see Chapter 40, Figs. 40.3, 40.4, and
40.5).

Cystic Kidney Disease


Two main groups of neonatal renal cystic disease include cystic-dysplastic kidney disease (CDKD) and
polycystic kidney disease (PKD). CDKD is type of renal dysplasia in which there is variable connective tissue
and cyst replacement of normal renal parenchyma. Cysts do not communicate with the collecting system.
Ultrasound will show cysts along with echogenic parenchyma. The important step is to distinguish whether the
CDKD is a mild form of disease in which there is preservation of some degree of identifiable renal parenchyma
and function versus a severe form of disease (e.g., multicystic dysplasia) in which there is complete replacement
of renal parenchyma and absence of function. A renal scan is useful to distinguish reduced versus absence of
renal function. PKD includes two entities: autosomal recessive polycystic kidney disease (ARPKD) and
autosomal dominant polycystic kidney disease (ADPKD). Both may be diagnosed prenatally with fetal ultrasound.
If undetected prior to birth, ARPKD will typically present during the neonatal period, while ADPKD will more
commonly present during childhood or adulthood. Sonographic features for ARPKD include enlarged echogenic
kidneys, dilated renal tubules, and small cysts. Should ADPKD be detected during the neonatal period, renal
sonography will typically show an enlarged echogenic kidney with micro- and macrocysts, associated with renal
contour distortion (secondary to the cysts). For both types of PCKD, MRI may be indicated to further define renal
parenchyma and the cysts (see Chapter 40, Figs. 40.6, 40.7, and 40.9).

Obstructive Uropathy
Ureteropelvic junction (UPJ) obstruction and posterior urethral valves (PUVs) are two anatomical obstructive
uropathies in the neonate for which radiologic imaging is critical. UPJ obstruction occurs as a stenosis at the
transition between the renal pelvis and ureter and may be caused by intrinsic errors in ureteral development
versus extrinsic compression (e.g., crossing vessels). PUVs are congenital obstructing urethral membranes (of
connective tissue and muscle) located in the posterior urethra (Fig. 6.7A and B). Both UPJ obstructions and
PUVs may be diagnosed pre- or postnatally. On neonatal ultrasound, UPJ obstruction manifests as
hydronephrosis transitioning at the UPJ, associated with diminished or absent ureteral jets. Renal function may
be diminished; a renal scan may show delayed washout of the radiotracer. PUVs are distinguished on ultrasound
by a dilated, keyhole posterior urethra associated with bladder wall thickening, likely ureterectesis and
pelvicalyectasis, and potentially renal dysplasia. VCUG will show a dilated posterior urethra with abrupt caliber
change between the posterior and anterior urethra, associated with bladder wall thickening, bladder neck
hypertrophy, vesicoureteral reflux, and potentially a bladder diverticulum (see Chapter 40, Figs. 40.10, 40.12,
40.14, 40.18, 40.20, and 40.24).

Infection
Pyelonephritis (PN) may occur in the neonate, often related to underlying urogenital structural anomalies (e.g.,
congenital obstruction) and functional abnormalities (e.g., reflux). Imaging is not necessarily required to diagnosis
PN, but rather to assess urogential structure and function. On ultrasound, findings for PN include parenchymal
congestion with abnormal focal or multifocal echogenicities corresponding to regions of infection, associated with
diminished or absent blood flow. Perinephric fluid or phlegmon may be present. VCUG is indicated to exclude
reflux,
P.74
while a renal scan should be considered to evaluate renal parenchymal inflammation and potential scar.

Nephrolithiasis
Secondary medullary nephrocalcinosis in the neonate may result from a variety of etiologies, including
medications (e.g., furosemide), endocrine disorders, ARPKD, and renal tubular acidosis. It is depicted on
ultrasound as renal pyramid coalescent hyperechogenicity. Large calcifications will typically demonstrate
posterior shadowing.

Renal Masses
Neonatal renal masses are relatively rare. Clinical symptoms (e.g., abdominal distention) or abnormal urinalysis
or renal chemistries may lead to an ultrasound examination in which the mass is detected. Diagnostic
considerations would include mesoblastic nephroma, nephroblastomatosis, Wilms tumor, rhabdoid tumor, and
clear cell sarcoma. Further radiologic imaging may proceed with either CT or MRI.

Adrenal Hemorrhage
Neonatal adrenal hemorrhage is hypothesized to result from perinatal stress (e.g., birth trauma, hypoxia, blood
pressure changes, and sepsis), resulting in blood being shunted from the adrenal glands. This leads to a
spectrum from parenchymal congestion to hemorrhagic infarction. Adrenal hemorrhage is depicted on ultrasound
as a nonvascular echogenic adrenal mass, potentially with mass effect on the adjacent kidney. Over time,
liquefaction will result in anechoic regions, while chronic organization will lead to hyperechogenicity with or
without calcification (see Chapter 40, Fig. 40.25).

Gastrointestinal System
Neonatal GI pathologies, which require imaging, are a diverse group involving the GI tract, hepatobiliary system,
and pancreas. Abdominal radiographs serve an important initial diagnostic role. Bowel gas pattern and visceral
shadows are assessed with the goal of excluding obstruction, displaced viscera, pneumatosis,
pneumoperitoneum, and perforation. Diagnostic imaging may typically proceed with ultrasound, fluoroscopy, CT,
MRI, or a combination thereof, depending upon the clinical presentation and initial radiographic findings.

Gastrointestinal Tract
Three main neonatal pathologic conditions of the GI tract warrant radiologic imaging. These include suspected
obstruction, necrotizing enterocolitis, and bowel perforation. The spectrum of possible obstruction includes
esophageal atresia (with tracheoesophageal fistula), hypertrophic pyloric stenosis, duodenal stenosis and
atresia (see Chapter 41, Fig. 41.18), small bowel atresia, meconium ileus, small bowel malrotation, colonic
atresia, anal atresia, and Hirschsprung disease. While radiography may be suggestive for the cause, diagnosis
is confirmed with ultrasound or fluoroscopy depending upon the pathology.
Necrotizing enterocolitis occurs almost exclusively in a preterm neonate. Central perquisites in the pathogenesis
include GI tract ischemia, bacterial colonization, and enteral feeding. Abdominal radiography may show dilated
loops of small bowel, wall thickening, pneumatosis intestinalis, portal venous gas, and pneumoperitoneum (see
Chapter 41, Fig. 41.20). Sonography may demonstrate bowel wall thickening, decreased or absent bowel
perfusion, bowel wall echogenic foci (corresponding to pneumatosis), portal venous gas, and
pneumoperitoneum. If there is mucosal sloughing, regions of wall thinning will be demonstrated.
Bowel perforation may occur in utero or postnatally. In utero perforation occurs most typically in the setting of
bowel obstruction (e.g., meconium ileus, bowel atresia) and/or ischemia. The radiographic and sonographic
hallmarks for in utero perforation are peritoneal calcifications, corresponding (see Fig. 6.20A) to intraperitoneal
spillage of enteric meconium and secondary sterile peritonitis. Nonsealed in utero perforations will lead to
postnatal pneumoperitoneum (Fig. 6.20B). Bowel perforations that occur exclusively in the postnatal period may
occur in the setting of high-grade bowel obstruction and NEC. Perforations will result in pneumoperitoneum,
peritonitis, and potentially intra-abdominal fluid and fluid collections. On AP radiographs, free air may be seen
around bowel loops (e.g., Rigler sign) and the falciform ligament (e.g., football sign, see Fig. 6.21), while on
lateral decubitus view, air is seen anterior to the liver edge. Ultrasound is useful to confirm peritonitis (e.g.,
peritoneal hyperemia) and free and loculated fluid.

Hepatobiliary
Imaging of the liver and biliary system may be indicated in the setting of obstructive jaundice, hepatomegaly, and
ARPKD. The workup of obstructive jaundice includes ultrasound and possibly MR cholangiopancreatography
(MRCP) and nuclear scintigraphy. Using this approach, biliary atresia and choledochal cysts are both diagnosed
with high accuracy, along with exclusion of other differential possibilities (e.g., bile plug). While hepatomegaly
may be secondary to hepatic congestion (e.g., right heart obstructive lesion) or obstructive jaundice, initial
sonographic screening may detect a hepatic mass (e.g., hepatic infantile hemangioma). Depending upon the
sonographic features, CT or MRI with angiographic imaging is an appropriate next radiologic step for a hepatic
mass. ARPKD is associated with periportal fibrosis and ectatic intrahepatic bile ducts. Liver parenchyma and the
bile ducts in the setting of ARPKD are readily assessed with ultrasound and as needed MRCP.

Pancreas
In addition to other described pathology, ADPKD also manifests with pancreatic cysts while ARKD may rarely
result in pancreatic fibrosis. This pathology can be assessed during sonographic and MRI evaluations for PCKD.
Similarly in the evaluation of suspected biliary ductal disorders, the pancreas is routinely evaluated during
abdominal ultrasound and MRCP to exclude ductal anomalies (annular pancreas). As mentioned previously, an
abdominal PET-CT may be indicated to evaluate for a suspected pancreatic insulin producing tumor.

Musculoskeletal System
Developmental deformities, skeletal dysplasias, metabolic diseases, congenital and acquired infections, and
trauma are major categories of neonatal skeletal disorders. For these pathologies, radiography serves as the
primary diagnostic imaging modality. In some instances, radiographs may initially be obtained for nonskeletal
indications. As an example, in a neonate with difficulty swallowing secretions, a chest-abdominal radiograph
obtained to verify positioning of a feeding tube may reveal looping of the catheter over the mediastinum as wells
as vertebral body anomalies. Findings would be indicative of esophageal atresia with VACTERL syndrome. In
other instances, bone radiographs are obtained specifically with the intent to investigate skeletal pathology (e.g.,
trauma). CT (with multiplanar reconstructions and 3D volume rendering) and MRI are utilized as secondary,
complementary modalities for diagnosis, management, and surveillance. In select pathologies, ultrasound (e.g.,
developmental dysplasia of the hip) and bone scintigraphy (e.g., osteomyelitis) are utilized.

Developmental
Developmental deformities presenting in the neonatal period may involve the axial or appendicular skeleton.
More common developmental deformations leading to imaging include developmental dysplasia of the hip (Fig.
48.13), limb anomalies, vertebral anomalies, and craniosynostosis.

Dysplasias
Genetic mutations leading to abnormal fibroblast and connective tissue cellular development can result in a
multitude of rare
P.75
skeletal dysplasias impacting the axial and appendicular skeleton. Severe dysplasias most commonly lead to in
utero or perinatal demise, often from a restricted thoracic cavity and pulmonary hypoplasia (e.g., thanatophoric
dysplasia). Diagnosis of nonlethal dysplasias encountered in the neonatal period relies upon a combination of
genetic testing, clinical phenotype, and radiographic findings.

FIGURE 6.20 A: Transverse image of a 33-week gestation fetal abdomen demonstrates multiple dilated loops of
bowel (black arrowheads). Echogenic focus (white arrow) is consistent with a peritoneal calcification. B:
Following delivery at term, a water-soluble enema demonstrates a normalcaliber colon with dilated air-filled small
bowel loops (arrow). Jejunal atresia was found at surgery.
Two of the more common skeletal dysplasias seen in a neonate are achrondroplasia and osteogenesis
imperfecta (OI). Achrondroplasia is the most common nonlethal skeletal dysplasia. The differential includes
hypochondroplasia, homozygous achondroplasia, and a less severe form of thanatophoric dysplasia. OI
manifests with variable severity (e.g., types I to VIII) of fragile bones, multiple fractures (including those of
variable age), and deformed limbs, associated with specific clinical features, including blue sclera, joint laxity,
and a soft skull.

Metabolic Bone Disease


In the neonate, abnormal bone metabolism may present clinically as two distinct entities: osteopenia of
prematurity and rickets. In osteopenia of prematurity, inadequate mineral storage compounded by inadequate
supplementation leads to osseous demineralization. Radiographs will show marrow lucency and cortical thinning
with or without fractures. Rickets occurs in term neonates from vitamin D deficiency. Characteristic neonatal
radiographic findings include indistinct trabeculae, metaphyseal cupping and fraying, widened epiphyseal plates,
poorly mineralized epiphyseal centers, periosteal reaction, and osseous deformities. Findings are best identified
in the knees and wrists.
Metabolic lysosomal storage disorders can also impact bone structure and may result in radiographic features
overlapping with dysplasias (e.g., OI), rickets, and osteopenia of prematurity (if preterm). Lysosomal storage
disorders which may present in the neonatal period include type 2 Gaucher disease, galactosialidosis, GM1
gangliosidosis, and I-cell disease (mucolipidosis II). Distinguishing radiographic features for type 2 Gaucher
disease are lytic bone lesions. Dysostosis multiplex can be seen radiographically with the latter three lysosomal
storage disorders (25).

Infection
Congenital (e.g., transplacental and transvaginal transmissions) and acquired (e.g., hematogenous transmission)
osteomyelitis in the neonate have specific radiographic features. Rubella virus and CMV are common congenital
infections. Both may exhibit long bone metaphyseal radiolucent striations (celery stalk sign). Syphilis occurs
less commonly but the incidence may be increasing. Metaphyseal bands, metaphyseal edge fraying, diaphyseal
periostitis, and bone destruction are distinctive radiographic features; proximal tibia medial metaphyseal bone
destruction is pathognomonic (Wimberger sign)
Acquired osteomyelitis typically is bacterial in origin. Radiographs will show metaphyseal osseous destruction,
periosteal reaction, and soft tissue swelling. If not initially diagnosed and treated, as the osteomyelitis progresses
radiographs may depict an involucrum and sequestrum. Metaphyseal involvement in the neonate will lead to
septic arthritis and potentially joint effusions. Ultrasound can be performed to evaluate for septic arthritis and
joint
P.76
effusions, as well as a periosteal or soft tissue abscess. In select cases, a MRI or bone scan can be obtained.
FIGURE 6.21 Supine abdominal x-ray film demonstrates a massive intraperitoneal air collection. The air
is seen as a large central bubble on which is superimposed a dense linear opacity produced by the falciform
ligament. The falciform ligament forms the lace for the football sign.

Trauma
Accidental skeletal trauma may occur during birth. The clavicle, humerus, and femur are common locations for
birth-related fractures (Fig. 48.16). Cranial skull fractures may also occur, but less commonly. Diagnosis is
readily made with targeted radiographs. Ultrasound and CT are additional modality considerations.
Suspected nonaccidental skeletal (NAT) trauma requires complete axial and appendicular skeletal radiographic
interrogation. The following are patterns of fractures which would support NAT in a neonate: multiple fractures,
fractures of variable age, long bone diaphyseal spiral fractures, transmetaphyseal fractures, rib fractures
(typically posterior-medial, paraspinal), and skull fractures (linear to complex). When there is suspected
intracranial, intrathoracic, or intraabdominal NAT, CT should be performed for complete skeletal and visceral
assessment. When encountering fractures of variable age in a neonate, the reader is reminded that this finding
can also be seen with OI. Clinical and other radiographic findings will distinguish NAT from OI.

SUGGESTED READING
Kirpalani H, Epelman M, Mernagh JR, eds. Imaging of the newborn, 2nd ed. United Kingdom: Cambridge
University Press, 2011.

REFERENCES
1. International Commission on Radiological Protection (ICRP). Radiation and your patient: a guide for
medical practitioners. A web module produced by Committee 3 of the ICRP.
http://www.icrp.org/docs/Rad_for_GP_for_web.pdf

2. Glatz AC, Purrington KS, Klinger A, et al. Cumulative exposure to medical radiation for children requiring
surgery for congenital heart disease. J Pediatr 2014;164:789.

3. Khong PL, Ringertz H, Donoghue V, et al. ICRP publication 121: radiological protection in paediatric
diagnostic and interventional radiology. Ann ICRP 2013;42(2):1.

4. Hellinger JC, Pena A, Poon M, et al. Pediatric CT angiography: imaging the cardiovascular system gently.
Radiol Clin North Am 2010;48 (2):439.

5. Blass EM, Hoffmeyer LB. Sucrose as an analgesic for newborn infants. Pediatrics 1991;87:215.

6. Creeley CE, Olney JW. The young: neuroapoptosis induced by anesthetics and what to do about it.
Anesth Analg 2010;110(2):442.

7. Schmolzer GM, O'Reilly M, Davis PG, et al. Confirmation of correct tracheal tube placement in newborn
infants. Resuscitation 2013;84(6):731.

8. American Heart Association. 2005 American Heart Association (AHA) guidelines for cardiopulmonary
resuscitation (CPR) and emergency cardiovascular care (ECC) of pediatric and neonatal patients: pediatric
basic life support. Pediatrics 2006;117(5):e989.

9. Blankenberg FG, Loh NN, Bracci P, et al. Sonography, CT, and MR imaging: a prospective comparison of
neonates with suspected intracranial ischemia and hemorrhage. Am J Neuroradiol 2000;21(1):213.

10. Chao CP, Zaleski CG, Patton AC. Neonatal hypoxic-ischemic encephalopathy: multimodality imaging
findings. Radiographics 2006;26(Suppl 1):S159.

11. Papile LA, Burstein J, Burstein R, et al. Incidence and evolution of subependymal and intraventricular
hemorrhage: a study of infants with birth weights less than 1,500 gm. J Pediatr 1978;92(4):519.

12. Brouwer AJ, Groenendaal F, Koopman C, et al. Intracranial hemorrhage in full-term newborns: a hospital-
based cohort study. Neuroradiology 2010;52(6):567.

13. Isaacs H. Perinatal (fetal and neonatal) tuberous sclerosis: a review. Am J Perinatol 2009;26(10):755.

14. Salerno AE, Marsenic O, Meyers KE, et al. Vascular involvement in tuberous sclerosis. Pediatr Nephrol
2010;25(8):1555.

15. Vogl TJ, Stemmler J, Bergman C, et al. MR and MR angiography of Sturge-Weber syndrome. Am J
Neuroradiol 1993;14(2):417.

16. Epelman M, Johnson C, Hellinger JC, et al. Vascular lesionscongenital, acquired, and iatrogenic:
imaging in the neonate. Seminars of CT, US, and MRI. 2015. [In press]

17. Del Pizzo J. Focus on diagnosis: congenital infections (TORCH). Pediatr Rev 2011;32(12):537.
18. Osborn AG, Preece MT. Intracranial cysts: radiologicpathologic correlation and imaging approach.
Radiology 2006;239(3):650.

19. Buetow PC, Smirniotopoulos JG, Done S. Congenital brain tumors: a review of 45 cases. Am J
Neuroradiol 1990;11(4):793.

20. Epelman M, Kreiger PA, Servaes S, et al. Current imaging of prenatally diagnosed congenital lung
lesions. Semin Ultrasound CT MR 2010;31:141.

21. Hellinger JC, Daubert M, Lee E, et al. Congenital thoracic vascular anomalies: MDCT and MRI
evaluation with advanced imaging. Radiol Clin North Am 2011;49(5):969.

22. Rocha G, Fernandes P, Rocha P, et al. Pleural effusions in the neonate. Acta Paediatr 2006;95(7):791.

23. Shennan AT, Dunn MS, Ohlsson A, et al. Abnormal pulmonary outcomes in premature infants: prediction
from oxygen requirement in the neonatal period. Pediatrics 1998;82(4):527.

24. Glodny B, Peterson J, Hofmann KJ, et al. Kidney fusion anomalies revisited: clinical and radiological
analysis of 209 cases of crossed fused ectopia and horseshoe kidney. BJU Int 2009;103(2):224.

25. Staretz-Chacham O, Lang TC, LaMarca ME, et al. Lysosomal storage disorders in the newborn.
Pediatrics 2009;123(4):1191.
7
Telehealth in Neonatology
Sarah C. Muttitt
Mary M. K. Seshia
Liz M. Loewen

Health care is facing increasing pressure to improve access and quality while reducing the administrative and
financial burdens of providing care. There is a growing expectation that technology has a key role to play in
meeting these demands. Telehealth, one of several eHealth-related applications gaining use in the health care
sector, is defined as the use of information and communications technology (ICT) to deliver health services,
expertise, and information over barriers of distance, geography, time, and culture (1). Another term often used is
telemedicine, and the American Telemedicine Association considers these to be synonymous, with both defined
as the use of remote health care technology to deliver clinical services (2).
Telehealth is in use across a wide spectrum of health system service areas including clinical diagnosis,
treatment, and prevention of disease, continuing education of health professionals and consumers, and
research. When well integrated into routine clinical practice, telehealth can improve the efficiency and cost-
effectiveness of the health system by moving people and information virtually rather than physically (1). Although
not new, telehealth has experienced rapid expansion over the past two decades and is being utilized in a
growing number of medical specialties including dermatology, oncology, radiology, surgery, cardiology, mental
health, and home health care. While telehealth still has not reached the volume and maturity required for large-
scale randomized studies, the value of telehealth has been well accepted by consumers and health care
providers alike (1,2,3).
Telehealth implementation addresses three major issues: access to health care services; retention, recruitment,
and support of rural physicians and other health care providers; and potential cost savings to the health care
system and/or patients and their families. In order to access specialty care, residents of rural areas are often
forced to travel long distances at significant cost, inconvenience and, in some cases, aggravation of underlying
medical conditions. Although some tertiary care centers provide itinerant specialty clinics, these services may not
be available where and when a patient requires specialist advice. Physician travel for itinerant clinics also has
associated risks and costs, including the loss of valuable time while traveling between clinics. Increasingly,
telehealth is being used to support home-based care where access barriers are less about geographic distance
and relate more to mobility and access to primary care services locally (3). Regardless of distance between
patient and provider, telehealth can provide access to a broader range of comprehensive primary, secondary,
and tertiary health care services, more timely intervention, earlier repatriation, and improved continuity of care for
patients. Physicians and health care providers in rural areas have limited direct access to peers, specialists,
education, and opportunities to participate in health care administration or professional association activities.
This sense of professional and social isolation often contributes to clinicians leaving positions prematurely and
the inability to recruit skilled practitioners, leaving rural communities largely underserviced. Access to education
and peer support through telehealth may impact retention and recruitment and allow health care providers in
rural settings to work to the full potential of their scope of practice as well as provide more complex care closer to
home.
As with any new technology, much attention has been given to the telehealth business case. Cost avoidance
and cost savings are constantly sought to offset the substantial costs of telehealth implementation and
operations. Although there are cost savings associated with a reduced number of unnecessary medical
transports, earlier patient discharge to community hospitals or home, and decreased education and
administrative travel, the longer-term savings associated with more timely access to care resulting in less
consumption of health care resources and improved health outcomes have yet to be measured on a systemic
basis (1,4). The ability of telehealth to contribute to greater efficiencies within the system may offer the
opportunity to redirect any savings toward improved patient services. Even if telehealth does not result in a
reduction in total health expenditures, improved access to quality health care services should be of high
importance to patients, providers, and health care funders (1).

HISTORY OF TELEHEALTH
Health care at a distance, or telehealth, has been practiced for decades using less sophisticated
communications technologies than we currently associate with telehealth. The National Aeronautics and Space
Agency (NASA) played an important role in the early development of telehealth. The remote monitoring of crew,
spacecraft, and environmental health has been an integral part of NASA operations. Similarly, the U.S. military
has been actively involved in telehealth research and applications as a means of bringing medical expertise to
those injured in battle with less risk of injury to health care personnel. In the 1960s, the Nebraska Psychiatric
Institute became one of the first facilities in the United States to develop a two-way link via microwave technology
to provide education and consultations between specialists and general practitioners. NASA's efforts at
enhancing satellite communications provided the opportunity to pilot telehealth in more remote locations such as
Alaska in the early 1970s (5). Satellite technology also facilitated the development of the Telemedicine Centre at
Memorial University of Newfoundland (MUN), Canada, in 1977. Using simple, low-cost audio-conferencing
technology, the MUN program linked hospitals, community colleges, university campuses, high schools, town
halls, and education agencies throughout the province for educational programs and transmission of medical
data (6). In the 1980s, there was a flurry of telehealth activity in North America as well as around the world, with
new projects in Australia, New Zealand, the United Kingdom, France, and Norway. Early projects were largely
focused on the technical feasibility of telehealth and often were developed around a single application and single
clinical champion. Because of the high capital costs of these projects, they were also extremely dependent on
grant funding. Many came to an end when funding expired or the clinical champion moved on to other areas of
research interest.
In the late 1980s and early 1990s, telehealth technology became more robust and less expensive, making
telehealth a more viable alternative for health care service delivery. The past decade has seen rapid expansion
of telehealth sites and applications. The American Telemedicine Association reports there are about 200
telemedicine networks (3,500 service sites) in the United States (2). A better understanding of the human factors
associated with telehealth success has allowed improved planning, deployment, and management of telehealth
programs. However, the majority of telehealth activity worldwide still remains grant funded or hospital supported
with consequent vulnerability to annual funding cycles. It is becoming apparent that telehealth solutions have to
be integrated within the traditional health care system to be sustainable. With careful attention to cost-
effectiveness and quality services, telehealth is destined to become a standard component of health service
delivery.
P.78
TECHNOLOGY
There are two main types of communications in telehealth. Asynchronous, or store and forward, involves the
capture and later transmission of data or images for dissemination or interpretation. Teleradiology, the sending of
radiographs, computed tomography (CT) scans, or other digital scans, is the most common store-and-forward
application of telehealth in use today and is often integrated into larger picture archiving and communication
systems (PACS). Pathology and dermatology are other specialties that typically use store-and-forward
technology for remote diagnosis. Synchronous, or real time, implies the transmission of information instantly and
is primarily associated with the use of interactive videoconferencing to support face-to-face consultation between
a patient in one location and a provider in another. Almost all medical specialties have found an application for
the use of videoconferencing technology and with the addition of appropriate peripheral medical devices, such
as stethoscopes, otoscopes, and examination cameras, a comprehensive examination can be conducted
remotely. Some telehealth applications use a combination of store-and-forward and videoconferencing
technologies to allow both the review of still images and interactive consultation with peers and patients. In all
cases, the clinical requirements must drive the technical solution. The price and performance of telehealth
technology has improved dramatically over recent years, and in many cases, off-the-shelf hardware now
provides the necessary functionality at much lower cost than systems specifically designed for telehealth. All
equipment should comply with accepted technical standards to ensure quality, flexibility, and compatibility
between systems.
In addition to end points, telehealth requires a telecommunications network to facilitate the exchange of
information. Although telecommunications infrastructure in urban settings has developed remarkably over the
past decade, the primary focus of telehealth has been to serve rural and remote populations where connectivity
continues to be a considerable challenge. Requirements for bandwidth (communication channel capacity) vary
depending on the application. The higher the bandwidth, the more information can be sent in a measured time
period. POTS (plain old telephone system) may be appropriate for transmitting low volumes of non-urgent x-ray
images between two destinations for a teleradiology service. Larger volumes of images or a need for urgent
interpretation would require a higher bandwidth solution. Similarly, higher bandwidth is required to support
quality interactive videoconferencing for clinical applications. Although urban sites may be able to choose
between a number of suitable solutions such as ISDN (Integrated Services Digital Network), DSL (digital
subscriber lines), or high-speed cable, geographically remote communities may only have access to the
necessary bandwidth through satellite or other high-cost wireless solutions. Telecommunications costs for rural
education and health care networks are subsidized in some jurisdictions, and sharing infrastructure costs with
other sectors, such as education, justice, or industry, may improve the viability of telehealth in a small, remote
community. The availability of low-cost telecommunications solutions is critical to the expansion and
sustainability of telehealth in many of the neediest areas, including developing countries where cellular networks
are also showing potential use in some applications.
The advent of IP (Internet Protocol) videoconferencing impacted the design and operations of telehealth
networks everywhere. Traditional copper-based networks required dedicated connections, and so telehealth was
often limited to a single site or suite within a health care facility. With line installation, monthly line rentals, and
long-distance charges associated with each session, telecommunications costs often accounted for as much as
15% to 25% of total telehealth costs (2). With advances in digital video compression, composite audio and video
signals can now be carried over typical IP network circuits on a LAN (local area network) within a health center,
across a broader WAN (wide-area network), or private network. With nearly ubiquitous access, telehealth can be
available on every physician's desktop, at the patient bedside, and throughout every hospital and primary health
care facility, thus providing access wherever and whenever health care services are delivered. Although there is
a fixed cost associated with an IP network, there is little or no additional cost associated with actual use. As a
result, the actual per session cost for telehealth declines with increasing utilization. In addition to long-term cost
savings, the convergence of voice, video, and data onto a single network will allow telehealth to interface with
other health information, including PACS and electronic health records. Issues surrounding network quality,
bandwidth requirements, and security continue to be refined, but telehealth over IP networks is becoming an
attractive option for many programs.
Wireless or mobile (mHealth) applications are increasingly being considered within the spectrum of telehealth
applications. This emerging area includes Web-based interactive videoconferencing and mobile apps, which can
be accessed through smartphones and tablets as well as other applications that connect patients with reminders
and health advice. Choosing the right technology for telehealth is complicated in the face of declining equipment
and telecommunications costs, inevitable capital depreciation, and rapid technical innovation. Clinical users of
the equipment must be involved in purchasing decisions, as clinical and operational requirements will directly
determine technical specifications; such involvement will also foster acceptance by the users. In general, a
telehealth program should purchase the highest specification equipment available to meet user expectations at
the lowest possible cost. Similarly, decisions regarding telecommunications infrastructure should be based on file
size, immediacy, and volume of usage balanced by fiscal realities and whether or not funding or revenue streams
can offset the associated capital and operating costs. Telehealth programs must also plan for equipment and
network maintenance, support, and upgrading. Vendor relationships are crucial to the success of telehealth
programs. In addition to price and technical specifications, appropriate service level agreements should be
instituted to ensure high-quality, reliable, and state-of-the art telehealth operations.

MEDICOLEGAL AND REGULATORY ISSUES


The clinician's duty of care and clinical case responsibility in the telehealth setting adhere to the same principles
found in face-to-face encounters. The ethical and quality standards of practice governing clinicians are not
altered by the use of telehealth. Proper documentation, including written consent when applicable, should be
maintained for all telehealth encounters. The advantage of telehealth is that it permits health care to be delivered
anywhere, with no recognition of borders; however, this inherent distinction from traditional in-person care also
raises new issues surrounding regulations and policy related to health care practice (7) (see also Chapter 9).
Licensing of health professionals is typically a jurisdictional responsibility. If physicians and patients are located
in different provinces or states, it is important to determine whether the locus of accountability will be the patient
site or provider site. If the service is deemed to occur at the patient site, the physician may be required to obtain
appropriate licensing and credentialing as if practicing in person at that site. A number of policy options to
overcome these licensing issues have been proposed including universal licensure, a special-purpose license
for telehealth, and mutual agreements, but there is still a significant need to harmonize standards to support
cross-jurisdictional telehealth activities (8).
The absence of policies regarding physician reimbursement for telehealth consultations has historically been a
significant barrier to the widespread adoption of telehealth. Early telehealth
P.79
initiatives were often pilot projects or clinical trials located at hospitals or universities, and physician
reimbursement was not a major concern as most physicians treated their participation as a research initiative or
were compensated through alternative payment arrangements (salary or capitation). However, as more
practitioners incorporate telehealth into routine practice, compensation has become a pivotal issue. Although
many insurance plans still require patients to be seen in person by a physician in order to bill, others have
developed specific fee codes for telehealth services; however, these often have significant limitations related to
geographic location, specific facilities, number and types of services, and professional category. Canada's
publicly administered system is also inconsistent, with some provinces allowing direct reimbursement for
telehealth services and others not. There is slow progress toward expanding telehealth reimbursement, but
health care organizations should determine the policies in their jurisdiction regarding payment prior to
implementing telehealth services.
All physicians engaging in telehealth consultations should verify with their insurance providers that telehealth is
included within their malpractice insurance policies. To date, there has been very little litigation associated with
telehealth, but there are some specific issues to be considered. Not all consultations are appropriate for
telehealth. Providers must use their best clinical judgment to determine whether services can be safely and
effectively provided by telehealth. In addition, a backup process must be established to ensure that patients
receive appropriate and timely care in the event of technical failure. Practitioners may require specialized training
and expertise for telehealth and demonstrate acceptable technologic competence prior to providing telehealth
services. Specific clinical protocols and guidelines may be necessary to ensure consistent, high-quality
telehealth applications in some settings. At all times, telehealth services must adhere to basic quality assurance
and professional standards of care (8).
Privacy of personal information related to the use of ICTs in health has been a developing issue over the last
decade. Concerns over the use of technology to track everything from health care services to spending habits
have spurred the development of policies to regulate the protection of individual privacy. Standards for
maintaining the privacy of health information in a telehealth context do not differ from those in a face-to-face
encounter; however, the introduction of technology adds privacy and security considerations (9). In addition to
maintaining privacy through more traditional measures such as a private physical environment and organizational
processes, delivery of telehealth requires attention to security of data during transmission and, in some cases,
storage. Ensuring security in an ever-changing technologic environment requires a proactive and evolving
approach (10). Ensuring confidentiality in a telehealth setting can be more challenging given the potential risks
for interception, potential for a permanent video record, and additional people involved in each care session.
This is compounded by the variety of equipment and the complexity of transmitting images between two settings
(10). As telehealth moves from single-room stand-alone applications to integration within direct patient care
areas, such as the neonatal intensive care unit (NICU), the complexity of ensuring privacy increases.

ECONOMICS AND EVALUATION OF TELEHEALTH


The body of scientific literature related to the application of telehealth has been growing steadily over the last 40
years. While utilization of telehealth continues to grow internationally and the volume of scientific literature
grows, there remain few conclusive studies demonstrating telehealth effectiveness and the economic impacts of
telehealth. Several recent literature reviews have examined the current state of research knowledge related to
telehealth, and most have determined that while telehealth shows promise, research and evaluation of telehealth
is still maturing with few scientifically conclusive studies completed to date. The reviews completed to date have
identified small numbers of studies that meet criteria for inclusion ranging from 7 for those requiring a randomized
controlled trial to 50 when inclusion criteria were broadened to include any controlled design (11,12). The
strongest studies examining clinical impacts have demonstrated support for telehomecare initiatives,
management of chronic illness, psychiatry, dermatology, cardiology, teleradiology, transmission of digital images
for neurosurgery consultation, and echocardiographic image transmission (11,12,13,14,15).
While telehealth is often identified as a means of reducing costs, the literature in this area remains divided.
Economic indicators for telehealth include costs related to travel and travel time for patients and providers,
patient transport, equipment and telecommunications, shift of care from larger centers, and recruitment and
retention related to access to continuing education. In a 2009 review, Davalos et al. (16) found a lack of
evidence in the literature demonstrating the economic impact of telehealth. A more recent analysis completed in
Canada estimated cost avoidance for the health system (beyond personal cost savings to patients) of
approximately $55 million per year attributable to the use of telehealth (17). This discrepancy may relate to the
measures used; an earlier systematic review of telemedicine cost-effectiveness studies from 1966 to 2000 found
612 articles that included some economic analysis; most were recorded as small-scale or short-term evaluations
with less than ideal economic analysis and limited transferability (18). Only 55 included actual cost data, of which
24 met the requirements for a full review. In neonatology, Armfield et al. (19), in Australia, demonstrated a strong
economic benefit in addition to clinical value, for the use of telemedicine to replace phone-based consultation for
acute neonatal consultation between remote and tertiary hospitals with the majority of cost benefit resulting from
avoided neonatal patient transfers.
While the reviews done to date have demonstrated the feasibility of telehealth, telehealth research has not yet
included large-scale randomized studies (11). An additional challenge in the evaluation of telehealth is whether
face-to-face care is, in fact, the gold standard against which any new service delivery method should be tested
(11). As telehealth moves toward a more integrated model, evaluation approaches must also use an integrated
and systemic approach rather than focusing on limited indicators to measure the impact of the technology. The
potential for increased access to health care resulting from telehealth applications may have long-term systemic
benefits not easily captured in a single study. A review examining the socioeconomic impact of a variety of
telehealth applications found benefits to patients, clinicians, and the health care system (1).

TELEHEALTH APPLICATIONS IN NEONATOLOGY


In 1970, regionalization of perinatal-neonatal care was advocated following the observation that neonatal
mortality was highest in those hospitals delivering small numbers of infants compared with that of larger hospitals
with neonatal referral units. Additionally, since that time, the care of the sick neonate has become increasingly
technologically dependent, providing further justification for regionalization of neonatal care. This has resulted in
dislocation of families during complicated pregnancies, limited access to specialized resources for sick neonates,
and difficulties in ensuring access to follow-up resources.
In this same time period, technologic advances have resulted in the ability to deliver health care effectively over
distance. Those practicing neonatal-perinatal medicine must harness this technology and incorporate it into daily
care. Teleconsultation services (both emergent and elective), televisitation for families, and supportive care to
level II facilities following transfer from a level III unit should be part of neonatal health care delivery. This section
P.80
demonstrates how telehealth has been used effectively through the perinatal-neonatal continuum, both for the
delivery of care and for education of health care providers.

Antepartum
Telehealth has been used successfully for a number of antepartum applications including genetic counseling and
tele-ultrasound. In Queensland, Australia, a weekly connection, developed in 1997, allows maternal-fetal
medicine specialists to provide direction to a sonographer at the patient site while viewing the fetal ultrasound in
real time. At the end of the consultation, the subspecialist counsels the parents on diagnosis, prognosis, and
management and prepares a report for the referring physician. In addition to increasing patient access to the
subspecialist, communication between the two hospitals is improved, the consultation can be interdisciplinary,
and knowledge transfer between health care providers is facilitated. A review of the program found only one
missed fetal diagnosis out of 120 cases, and patients were highly satisfied with the consultative process. The
real-time interaction is considered a key component of the success of this project (20,21). A limitation to
mainstream use of this technology is the availability of a sonographer at the remote site. As robotics advance,
use of a robot to perform the ultrasound remotely may become feasible.

Neonatal
Health care professionals caring for neonates know that even a low-risk pregnancy can result in a high-risk
situation for the neonate in 2% to 4% of deliveries. Additionally, newborn infants who initially appear well can
deteriorate rapidly, particularly from sepsis, respiratory problems, and congenital heart disease. Timely access to
specialists in newborn care can be problematic for remote and isolated health facilities. In 2002, in Manitoba,
Canada, a telehealth link was established between a tertiary NICU and the newborn nursery in a general
hospital 760 km north of the NICU. Physicians at the remote site were able to consult the receiving NICU on an
emergent basis for assistance with management and stabilization. In this integrated system, both the remote and
receiving staff were able to operate the equipment and relied on technical support only when problems were
encountered. Supported by MBTelehealth, the NICU can now link to six sites delivering newborns in Manitoba
and two in Northwestern Ontario. There is a virtual bed within the NICU allowing for full integration with workflow
patterns and also enabling multidisciplinary interaction. Image resolution is high. Interoperability of the systems
allows the NICU health care professionals to operate the remote camera, thus ensuring that the remote staff
members are not distracted from the care of the newborn. In some situations, a parent is present at the remote
site. The interaction between the NICU staff, which frequently includes not only the neonatologist but also nurses
and respiratory therapists, and the referring physician and nursing staff, not only supports clinical care but also
provides a conduit for ongoing education in neonatal care; importantly, the parents are comforted by the ready
availability of a specialist for their newborn. This link has also allowed parents and extended family at the remote
location to visit the neonate and participate in management decisions in situations when they are unable to
travel to the NICU.
While technology is not going to replace the need for neonatal units, it allows for improved access to care, and in
addition, it provides an opportunity for education, which improves the confidence of those relatively isolated
health care providers. NICU staff must continue to incorporate other applications into enhancing neonatal care.
Yager et al. (22) found that telemedicine was also a viable tool for allowing on call medical staff to provide
support to bedside (resident) staff from home during evening hours avoiding the need for staff to travel to the site
provide support. In addition to the support already available through telephone, staff were now able to undertake
a more comprehensive assessment to confirm fellow assessments, communicate more effectively with the
patient/parent, and provide team teaching and communications. Telehealth has also shown promising outcomes
as a means of providing support to distant emergency departments for management of moderate trauma in the
general patient population with the use of telehealth resulting in decreased time to discharge and decreased use
of transportation without any negative impact on patient outcomes (23).

Telecardiology
Digital echocardiography has enabled the transmission of echocardiograms from remote sites to pediatric
cardiologists both by store-and-forward and real-time, synchronous transmission. Real-time transmission allows
for continuous live contact between the cardiologist, sonographer, and other health professionals and also family
members at the remote site. Although this technology is used for all age groups, Finley et al. (24) found that 51%
of urgent examinations were for newborn infants. Webb et al. (25), in a multicenter study of neonates less than 6
weeks of age where a pediatric cardiologist was consulted for suspect CHD, demonstrated that those patients
in whom a telecardiology consultation occurred were less likely to be transported and less likely to receive
inotropic support with no differences in mortality. In addition to diagnosis, the utilization of interactive
videoconferencing can support case conferencing and echocardiogram review between referring sites and
remote surgical teams for infants being referred to a cardiac surgical center. Families can participate in this
process, increasing their confidence in the care of their infant.
With appropriate bandwidths, images can be of clinical quality. Ideally, the sonographer at the remote end should
have pediatric cardiology experience to ensure that more difficult diagnoses, such as total anomalous pulmonary
venous return and coarctation of the aorta, are not missed (26,27,28). Although in many situations the neonate
will still have to be transported to the tertiary center, with telecardiology, some transports can be avoided or
undertaken more electively and more appropriate management decisions can be made.

Retinal Telephotoscreening
Retinopathy of prematurity (ROP) is a complication of surviving preterm low-birth-weight infants, with
approximately 65% of infants less than 1,300 g at birth and up to 80% of infants less than 1,000 g developing
ROP. A recent review estimated that in 2010, worldwide, ROP occurred in an estimated 185,000 surviving
preterm infants with 53,000 progressing to visual impairment (29).
Most guidelines suggest that preterm infants born at less than 32 weeks of gestation should be screened; the
gold standard for screening is indirect ophthalmoscopy by a pediatric ophthalmologist with expertise in ROP
management. Such subspecialists are few in number and with the geographical diversity of levels II and III
NICUs, providing this service is challenging. These infants require frequent retinal screening, especially once
ROP is diagnosed, if blindness is to be prevented. Telehealth technology, using a digital retinal camera
equipped with an ROP lens, has been investigated as an alternative method for retinal screening. The camera
system provides an immediate wide-angle view of 130 degrees and also produces a real-time image on a
computer monitor (Fig. 7.1), which is then stored, uncompressed, on a digital videodisc (Fig. 7.1). Two studies
concluded that the sensitivity was insufficient for recommendation as a screening tool for ROP (30,31). However,
Schwartz et al. (32) suggested that this telemedicine strategy might be useful for assessing whether the
neonate's eye required the urgent attention of a physician capable of evaluating and managing threshold ROP.
Ells et al. (33) subsequently adopted a pragmatic approach; rather than using this technology to differentiate
between stages 1 and 2 ROP, she focused on whether this technology could identify those eyes requiring
treatment, that is, referral-warranted ROP (RW-ROP). Using this approach, digital photography had a sensitivity
of 100% and a specificity of 96% in detecting RW-ROP. A recent large multicenter National Eye Institute-funded
study (34) provided further strong support for the use of a telemedicine system for detecting
P.81
RW-ROP and importantly provides evidence for the use of trained nonphysician imagers to detect RW-ROP in
infants at risk. As training programs are developed and personnel become certified, the use of digital retinal
photography will become a useful adjunct to hospitals in developed countries providing level II care, as well as to
hospitals in developing countries. The primary purpose of the e-ROP study (35) was to determine the specificity
and sensitivity of detecting RW-ROP by trained readers of image sets obtained by certified retinal imagers
(including nonphysicians) when compared to indirect ophthalmologic examination of the eye by an
ophthalmologist experienced in ROP examination. This well-designed study provides us with insight of the
potential future of ROP screening.

FIGURE 7.1 Retinal telephotoscreening of the right eye for ROP taking place in the NICU using the
RetCam. Left: normal retina; Right: stage 3 ROP in zone 2 with plus disease (pretreatment). A: Increased
tortuosity and dilatation of retinal vessels at the optic disc. B: Fovea. C: Avascular peripheral retina. D: Increased
vascular branching posterior to the ridge. E: Stage 3 ROP at the demarcation line between the vascular and
avascular retina. (Courtesy Dr. Ian Clark.)

Televisitation
Televisitation is best described as the virtual transportation of the patient's family to the bedside. It can provide a
connection to the neonate's home setting both before and after discharge of the infant. Interactive
videoconferencing can provide a link between the mother and her family. Gray et al. (36) developed a Web-
based solution designed to reduce the costs of care as well as providing enhanced medical, informational, and
emotional support to families of very-low-birth-weight infants in the NICU. Families can link to the NICU at any
time to obtain information about their infant, as well as obtain educational information and information on
experiences of other families. Also incorporated, and perhaps the most exciting aspect for families, was the
interactive videoconferencing capability. Parents can see their infant and receive information and support from
staff at times when they cannot visit. In a small, randomized study of this technology, hospital stay was shorter
and families experienced greater satisfaction with the care received. The system requires the installation of an
easy-to-use computer in the home. Given that access to the Internet has been adopted more rapidly than any
other technologic advance in history, the use of this technology as a means of providing family-centered care
should be expanded. With length of stay in NICUs for some neonates ranging from weeks to months, the
importance of virtual bedside visits by family members, particularly those from remote communities, cannot be
overemphasized.

Telehomecare
Telehomecare is a growing application with considerable potential to support neonatology. Telehomecare
applications link patients from their home environment to hospital or community-based providers with ongoing
monitoring of vital signs, and when indicated, videoconferencing contact on a scheduled or urgent basis.
Reviews of these applications in the nonneonatal population have demonstrated improved control of chronic
conditions such as diabetes and chronic heart failure, along with high levels of patient satisfaction (1,13). Within
the neonatal setting, telehomecare can be a support to parents following discharge, a time when families may
feel inadequate and may be reluctant to, or have difficulty in accessing care. Morgan et al. (37) successfully
demonstrated acceptability of telehomecare for postdischarge follow-up of patients with severe congenital heart
disease. Telehomecare may include regular vital sign monitoring as well as videoconferencing to assess color or
respiratory status in conditions such as congenital heart disease or bronchopulmonary dysplasia.
Postdischarge telehealth applications can include improved access to a wide range of disciplines and support
systems, including follow-up developmental assessments, feeding assessments, speech and language therapy,
genetic counseling, and many others. For neonates with ongoing health issues, telehealth can also provide
access to patient education and support for families in rural locations, who may not have regular access to other
families supporting a newborn with a chronic condition. Telehealth can provide key support for neonates as they
transition out of the institutional setting, and it may facilitate earlier discharge and improved patient outcomes.

Continuing Education
The education of health care professionals involved in newborn care is critical to maximizing telehealth's
potential to improve neonatal care. Continuing education is challenging, particularly for professionals working at
a site removed from a tertiary center. It is recommended that all hospitals delivering newborns should have an
individual certified in the Neonatal Resuscitation Program present at the delivery. In addition to initial certification,
recertification is required every 2 years. Cronin et al. (38) demonstrated that instruction via interactive
videoconferencing, including the testing of practical skills, can be provided satisfactorily. Similarly, Loewen et al.
(39) have demonstrated that the neonatal stabilization program, S.T.A.B.L.E. (sugar, temperature, artificial
breathing, blood pressure, lab work, and emotional support), can be delivered effectively. In a study of 56 health
professionals randomized to receive the program either in person or by videoconferencing, both groups, with
similar pretest scores, had a similar but significant increase in their posttest scores.
P.82
In Canada, the Neonatal-Perinatal Fellowship programs meet virtually each month for an academic session;
these sessions are highly interactive and include discussion of rare clinical cases as well as state-of-the art
presentations by expert faculty.

PRACTICAL STEPS IN GETTING STARTED


As telehealth moves away from pilot projects to operational telehealth networks, there have been many lessons
learned and some consensus on the critical factors for success. This background of experience has provided a
framework for developing new telehealth services or programs.

Needs Assessment
Experience has proven that time and resources spent on a needs assessment will result in the ability to design
systems that truly meet the user requirements. The needs assessment process includes obtaining information
and ideas from many groups involved in the delivery of health services to determine the goals, objectives, and
priorities for telehealth. The clinical needs identified will drive the goals of the telehealth program and system
design. Engaging clinical staff early and promoting buy-in will ensure that clinical needs remain the primary focus
of telehealth programs and will begin the change management process necessary to integrate telehealth into
clinical workflow. A well-structured telehealth needs assessment not only will define the clinical direction of a
potential telehealth application or network but will identify strategies to resolve issues that could potentially add
long delays and increased costs to telehealth implementation.

Readiness Assessment
Prior to telehealth investment, there is a clear need to determine the telehealth readiness of communities and
organizations to reduce the risk of failure and losses in time, money, and effort. Although there is a fundamental
requirement for sufficient bandwidth to support telehealth, nontechnical organizational factors are equally
important to successful telehealth implementation. The perception of need and the ability and willingness of
users to adapt to the changes associated with the introduction of telehealth can have enormous impact on
adoption and utilization. Telehealth success depends upon selecting communities, organizations, and programs
that are aware of telehealth and its benefits, have a genuine need for and commitment to telehealth, and can
provide or acquire the capacity to support and resource telehealth beyond implementation.

Telehealth Business Plan


To successfully deploy and evolve a telehealth program, it is important to develop a telehealth business plan.
The business plan serves as a guide for the organization and as a communications tool for external stakeholders
and funders. This plan should be generated in collaboration with a range of staff and be revisited frequently in
response to changes in the environment. The plan should fully describe the telehealth opportunity, products, and
services to be provided; organizational structure; operational and technical requirements; implementation plan;
marketing and business development strategy; and financial analysis. There is increased pressure for telehealth
programs to become viable commercial enterprises. Even in publicly funded health systems, telehealth is
expected to show a return on investment realized through cost savings and improved health outcomes. The goal
of the financial analysis is to define potential funding/revenue sources, expenses (capital and operating), and
cost recovery, as well as to identify and mitigate financial risk. Projections should contain best-case, expected-
case, and worst-case scenarios based upon a mix of alternative assumptions related to growth and use of the
telehealth network, cost of operations, cost recovery, and sources of funding/revenue. Careful attention to
longer-term sustainability is critical from the outset. Guidelines for developing a telehealth business plan can be
found in several of the listed references (2,40).

Program Management
Successful telehealth initiatives are built upon a robust operational infrastructure that ensures efficient delivery of
telehealth services on a daily basis. In addition to the obvious need for technical training and support for users,
there are many other functions required for effective telehealth program management including scheduling,
policy and standards development, workflow and process design, marketing and communications, research and
evaluation, and financial management. Strategic clinical leadership is also essential to ensure buy-in of key
clinical providers, validate clinical telehealth applications, and develop clinical policies and risk management
strategies. Understanding these functions and the related skills and competencies will allow a telehealth program
to implement an appropriate organizational structure and human resource plan to support operations. The
optimal organizational model will also be determined by the degree of telehealth integration into existing
organizational structures and functions.

Evaluation and Quality Assurance Plan


Evaluation and quality assurance are vital to ensuring quality telehealth activities. To date, telehealth evaluation
has focused on technical accuracy and reliability, diagnostic quality and effectiveness, impact on clinical
management, user satisfaction, and impact on service or program costs (10,11). There is still great need for
standardization of evaluation tools, measures, and key indicators to assess the impact of telehealth on cost,
quality, and accessibility of care. An effective evaluation plan will provide evidence to funding agencies, health
care administrators, providers, and patients and will significantly contribute to the strategic development and
sustainability of a telehealth program.
Health care organizations, including telehealth programs, are adopting a focus on quality improvement in
response to increased demands for accountability, heightened consumer expectations, resource limitations, and
fundamental changes in health service delivery models (41). There is also an increasing focus on the concept of
patient safety across multiple sectors using information systems, and this is also gaining hold in the health field.
The focus is on the advancement of safety practices across the solution lifecycle from software development, to
implementation, to ongoing operations to avoid, as much as possible, unintended risks with the use of
technology through controlled and managed processes. An example of this in Canada is the recent release of the
eSafety Guidelines established by COACH: Canada's Health Informatics Association (42). Management tools
such as the balanced scorecard are increasingly being adapted to the health care setting to guide ongoing
evaluation and quality improvement initiatives and can be tailored to include telehealth and other ICTs
(43,44,45,46). Regardless of the approach chosen, telehealth services should be continually monitored through
a quality assurance process that allows rapid and responsive improvements to performance and the
maintenance of quality practices. When possible, both evaluation and quality assurance processes should be
aligned with broader program or organizational initiatives.

IMPLICATIONS FOR THE FUTURE


The introduction of advanced ICT is inherently changing the way that medicine is practiced. With the introduction
of health information systems, clinical decision support tools, and online learning, medical professionals are
being provided the tools to support high-quality, evidence-based practice. Telehealth and the electronic health
record allow access to health care services and information across sites, providers, and the continuum of care,
removing the
P.83
traditional organizational boundaries and building a foundation for integrated service delivery. These
technologies are transforming the way that health care professionals and patients interact with the health care
system. IP-based technologies will provide a convergence of data with images, video, and voice in a media-rich
environment. Wireless network solutions will enable mobile access to the health care system and these
supporting technologies.
Drotar et al. (47) identify a number of key issues to be considered when introducing technologies into the care of
children and adolescents. It can be daunting for clinicians to understand the range of considerations and identify
best practices for integrating telehealth into their practice. In the past, health care providers who were early
adopters of technology needed to look to other sectors to build technology into their workflow. Fortunately, this is
no longer the case as an increasing body of academic and experiential knowledge now exists. Clinicians entering
the field are encouraged to engage with relevant local associations focusing on telemedicine and/or health
informatics. They provide a wealth of knowledge about current and emerging trends as well as opportunities to
network with colleagues facing similar practice challenges. Examples include the American Telemedicine
Association or HIMSS in the United States, COACH: Canada's Health Informatics Association, the Australasian
Telehealth Society, and other similar groups existing in most of the developed and developing world (2,48,49).
Telehealth is about the relationships and processes that allow health care services to be delivered virtuallyit is
not just about technology. Limits to telehealth expansion have less to do with access to bandwidth and
equipment costs than do the human factors. Telehealth initiatives must have the necessary time and resources
committed to address not only the technical solution but also the process redesign and change management
necessary to encourage more widespread adoption of telehealth. Unless those involved with telehealth programs
develop their skill and expertise in these areas, the programs will run the risk of limited user acceptance and poor
network use. With the relatively high cost of implementation and operations, low utilization threatens the
sustainability of any telehealth program.
Neonatology has been accused of focusing on the high tech. Telehealth is also viewed by some as yet another
application of technology. Ultimately, the fundamental purpose of both remains grounded in patient care and
achieving the best possible outcomes for patients. Telehealth improves access to health care, which means
access to specialist consultation for the neonate; improves access to education of health care professionals in
the care of the neonate; and facilitates family involvement with their newborn infant over distance. Telehealth
applications in neonatology can range from low-tech home care applications to high-end intensive care and
wireless transport communications. For both, the ultimate goal of improved patient care remains. To achieve this
goal, regardless of the technical solution chosen, the need for building user acceptance and confidence, as well
as simplifying the telehealth process to support integration into the workplace, is essential.

REFERENCES
1. Jennett PA, Scott R, Hailey D, et al. Socio-economic impact of telehealth: evidence now for health care in
the future. Volume one: state of the science report. Calgary, AB: Health Telematics Unit, University of
Calgary, 2003.

2. Telemedicine frequently asked questions [homepage on the internet]. Washington, DC: American
Telemedicine Association; n.d. [cited January 13, 2014]. Available from:
http://www.americantelemed.org/learn/what-is-telemedicine/faqs

3. COACH; Canada's Health Informatics Association Canadian telehealth report: based on the 2012
telehealth survey. Toronto, ON: COACH, 2013.

4. Darkins AW, Cary MA. Telemedicine and telehealth: principles, policies, performance and pitfalls. New
York: Springer Publishing Co., 2000.

5. B.O.H.M. Telemedicine Centre [homepage on the internet]; Brown N. A brief history of telemedicine.
[updated May 30, 1995, cited November 30, 2014]. Available from: http://www.bestohm.com/index.php?
option=com_content&task=view&id=52&Itemid=66

6. Elford R. Telemedicine activities at memorial University 1975-1997. 2002. Available at


http://www.med.mun.ca/telemed/telehist/telemulti.htm. Accessed September 29, 2004.

7. Hasham S, Akalu R, Rossos PG. Medico-legal implications of telehealth in Canada. Telehealth Law
2003;4:9.

8. Blum JD. Telemedicine poses new challenges for the law. Health Law Can 1999;20:115.

9. COACH; Canada's Health Informatics Association. Guidelines or the protection of health information.
Toronto, ON: COACH, 2013.

10. National Initiative for Telehealth (NIFTE). National initiative for telehealth framework of guidelines.
Ottawa, ON: NIFTE, 2003.

11. Currell R, Urquhart C, Wainwright P, et al. Telemedicine versus face to face patient care: effects on
professional practice and health care outcomes. Cochrane Database Syst Rev 2000;(2):CD002098.

12. Roine R, Ohinmaa A, Hailey D. Assessing telemedicine: a systematic review of the literature. CMAJ
2001;165:765.

13. Hersh WR, Helfand M, Wallace J, et al. Clinical outcomes resulting from telemedicine interventions: a
systematic review. BMC Med Inform Decis Mak 2001;1:5.

14. Ohinmaa A, Hailey D, Roine R. The assessment of telemedicine: general principals and a systematic
review. Alberta, Canada: Finish Office for Health Care Technology Assessment and Alberta Heritage
Foundation for Medical Research, 1999.

15. Hersch W, Helfand M, Wallace J, et al. A systematic review of the efficacy of telemedicine for making
diagnostic and management decisions. J Telemed Telecare 2002;8:197.

16. Davalos ME, French MT, Burdick AE, et al. Economic evaluation of telemedicine: review of the literature
and research guidelines for benefit-cost analysis. Telemed J E Health 2009;15(10):933.

17. Praxia, Gartner (Report commissioned by Canada Health Infoway). Telehealth benefits and adoption:
connecting people and providers across Canada. Toronto, 2011.

18. Whitten PS, Mair FS, Haycox A, et al. Systematic review of cost-effectiveness studies of telemedicine
interventions. BMJ 2002;324:1434.

19. Armfield NR, Donovan T, Bensink ME, et al. The costs and potential savings of telemedicine for acute
care neonatal consultation: preliminary findings. J Telemed Telecare 2012;18:429.

20. Chan FY, Soong B, Watson D, et al. Realtime fetal ultrasound by telemedicine in Queensland. A
successful venture? J Telemed Telecare 2001;7 (suppl 2):7.

21. Soong B, Chan FY, Bloomfield S, et al. The fetal tele-ultrasound project in Queensland. Aust Health Rev
2002;25:67.

22. Yager PH, Cummings BM, Whalen MJ, et al. Nighttime telecommunication between remote staff
intensivists and bedside personnel in a pediatric intensive care unit: a retrospective study. Crit Care Med
2012; 40(9):2700.

23. Westbrook JI, Coiera EW, Brear M, et al. Impact of an ultrabroadband emergency department
telemedicine system on the care of acutely ill patients and clinicians' work. Med J Aust 2008;188(12):704.

24. Finley JP, Sharratt GP, Nanton MA, et al. Paediatric echocardiography by telemedicinenine years'
experience. J Telemed Telecare 1997;3:200.

25. Webb CL, Waugh CL, Grigsby J, et al. American Society of Echocardiography Telemedicine
Collaborators' Group. Impact of telemedicine on hospital transport, length of stay, and medical outcomes in
infants with suspected heart disease: a multicenter study. J Am Soc Echocardiogr 2013; 26(9):1090.

26. Casey FA. Telemedicine in paediatric cardiology. Arch Dis Child 1999;80:497.

27. Sable C. Digital echocardiography and telemedicine applications in pediatric cardiology. Pediatr Cardiol
2002;23:358.

28. Widmer S, Ghisla R, Ramelli GP, et al. Tele-echocardiography in paediatrics. Eur J Pediatr
2003;162:271.

29. Blencowe H, Lawn JE, Vazquez T, et al. Preterm-associated visual impairment and estimates of
retinopathy of prematurity at regional and global levels for 2010. Pediatr Res 2013;74(suppl 1):35.

30. Roth DB, Morales D, Feuer WJ, et al. Screening for retinopathy of prematurity employing the retcam 120:
sensitivity and specificity. Arch Ophthalmol 2001;119:268.

31. Yen KG, Hess D, Burke B, et al. Telephotoscreening to detect retinopathy of prematurity: preliminary
study of the optimum time to employ digital fundus camera imaging to detect ROP. J AAPOS 2002;6:64.

32. Schwartz SD, Harrison SA, Ferrone PJ, et al. Telemedical evaluation and management of retinopathy of
prematurity using a fiberoptic digital fundus camera. Ophthalmology 2000;107:25.

33. Ells AL, Holmes JM, Astle WF, et al. Telemedicine approach to screening for severe retinopathy of
prematurity: a pilot study. Ophthalmology 2003;110:2113.

34. Graham EQ; e-ROP Cooperative Group. Telemedicine approaches to evaluating acute-phase
retinopathy of prematurity: study design. Ophthalmic Epidemiol 2014;21(4):256.
P.84

35. Quinn GE, Ying GS, Daniel E, et al.; e-ROP Cooperative Group. Validity of a telemedicine system for the
evaluation of acute-phase retinopathy of prematurity. JAMA Ophthalmol 2014;132(10):1178.

36. Gray JE, Safran C, Davis RB, et al. Baby CareLink: using the internet and telemedicine to improve care
for high-risk infants. Pediatrics 2000;106:1318.

37. Morgan GJ, Craig B, Grant B, et al. Home videoconferencing for patients with severe congenital heart
disease following discharge. Congenit Heart Dis 2008;3:317.

38. Cronin C, Cheang S, Hlynka D, et al. Videoconferencing can be used to assess neonatal resuscitation
skills. Med Educ 2001;35:1013.

39. Loewen L, Seshia MM, Fraser Askin D, et al. Effective delivery of neonatal stabilization education using
videoconferencing in Manitoba. J Telemed Telecare 2003;9:334.

40. Health Resources and Services Administration: Rural Health [homepage on the Internet]. [updated
November 20, 2012; cited November 30, 2014]. Available from http://telehealth.hrsa.gov/welcome.htm

41. Harrigan M. Quest for quality in Canadian health care: continuous quality improvement, 2nd ed. Ottawa,
ON: Minister of Public Works and Government Services Canada, 2000.

42. COACH eSafety Guidelines [homepage on the Internet]. [Updated June 2013; cited January 26, 2014].
Available from: https://www.coachorg.com/en/practices/eSafety_Guidelines.asp

43. Baker GR, Pink GH. A balanced scorecard for Canadian hospitals. Healthc Manage Forum 1995;8:7.

44. Kaplan RS, Norton DP. The balanced scorecard: translating strategy into action. Boston, MA: Harvard
Business School Press, 1996.

45. Kaplan RS, Norton DP. The strategy-focused organization: how balanced scorecard companies thrive in
the new business environment. Boston, MA: Harvard Business School Press, 2001.

46. Castaneda-Mendez K, Mangan K, Lavery AM. The role and application of the balanced scorecard in
healthcare quality management. J Healthc Qual 1998;20:10.

47. Drotar D, Greenley R, Hoff A, et al. Summary of issues and challenges in the use of new technologies in
clinical care and with children and adolescents with chronic illness. Child Health Care 2006;35(1):91.

48. Australasian Telehealth Society [homepage on the Internet]. [cited December 1, 2014]. Available from:
http://aths.org.au/

49. COACH: Canada's Health Informatics Association. [homepage on the Internet]. [cited December 1, 2014].
Available from: http://www.coachorg.com/
8
Current Moral Priorities and Decision Making in Neonatal-Perinatal
Medicine
Robert J. Boyle

GOALS OF NEONATAL-PERINATAL MEDICINE


In the first part of this chapter, broad goals and moral priorities of neonatal-perinatal medicine (NPM) are
discussed in relation to patients and society in developed and developing nations. In the second part, some
specific ethical issues that frequently arise in clinical decision making in the neonatal period are reviewed.
Medicine is a goal-oriented profession. One approach might argue that healing is the sole and overriding goal of
medicine. This view is unconvincing, because some valid goals of medicine (e.g., prevention) cannot be
collapsed into healing. The practice of NPM illustrates that medicine has multiple, complex, and sometimes
conflicting goals:
To save life and cure disease
To relieve pain, suffering, and disability
To rehabilitate and restore function
To prevent disease
To improve the quality of living and dying
To seek new knowledge

SOCIETAL AND PROFESSIONAL ISSUES


Medicine is accountable to society and to the international community. Critics may validly argue that American
medicine and public policy have neglected primary prevention and fostered the technologic imperative. To
counter these criticisms, the moral priorities of NPM specialists need to include advocacy for comprehensive
prenatal care, improved nutrition, prevention of premature delivery, and appropriate family support and services
for the infant after discharge. NPM in the United States is also part of a global community of science, medicine,
and humanitarian outreach. The moral priorities of the field ought to include commitment to global infant health.
Nations where newborns are in greatest danger of death or lifelong disability can benefit from transferable
knowledge and technology, for example, maternal folic acid supplementation, prevention of HIV transmission,
vitamin K prophylaxis, eye prophylaxis for gonorrhea, and prevention of neonatal tetanus.
For the United States, epidemiologic data (see also Chapter 1) highlight moral issues that relate to public policy,
program funding, and professional guidelines:
Increased rates of preterm delivery, especially in births 32 to 36 weeks of gestation
Dramatic differences in prematurity rates for various racial and ethnic groups
Increasing use of assisted reproductive technologies leading to an increase in multiple gestation and preterm
delivery
Significantly higher rates of prematurity and mortality compared to other industrialized nations
Absent or inadequate health insurance for women of childbearing age
Decreased access to family planning and prenatal and postnatal care compared to other industrialized nations
Infertile individuals who want their own genetic child suffer psychologically and spiritually. Fertility treatments
have allowed literally millions of women to have children who otherwise would not have been able to conceive.
However, there are high costs, risks, and potential harms associated with these treatments. To date, reliance on
professional self-regulation has not been effective in reducing multifetal pregnancy in the United States. There is
currently a debate about whether social controls of infertility treatment and research are fitting for a pluralistic
society that highly values reproductive liberty.
Following Buchanan, we propose that in a pluralistic democracy, the relation between society and medicine
ought to be understood as a self-correcting contract or bargain (1). Instead of resting on one supreme value
easily captured by ideology, the contract reflects a commonwealth of values. These values govern the complex
goals of medicine and interests of society in health. A commonwealth has a primary locus of loyalty. In this
society, the preeminent value is fidelity to patients or loyalty to the patient at hand. Using the right procedural
principles, patient-centered medicine is not inconsistent with viewing the patient in a population with needs for a
finite supply of expensive community health care resources (2). Within constraints of fidelity to patients and their
rights, physicians ought to promote the welfare of the many, to practice fairness in access and distribution of
resources, and to be efficient and effective in their practices. Physicians in this society are also members of a
scientific community with a high standard of evidence in practicing medicine. Guided by these values, society
grants the profession a privileged place, permits its members to earn high incomes, and subsidizes their training.
In return, society expects the net health benefits of pursuit of the goals of medicine to outweigh the net costs.
Over the past 15 to 20 years, there has been a dramatic increase in the number of neonatal intensive care units
(NICUs) in the United States. NICUs are often significant profit centers for a hospital, and in an academic
institution, the income from the NICU may be a major source of income for the entire Pediatric Department.
However, the increasing investment in perinatal-neonatal care has not produced proportional improvements in
crude infant survival and low birth weight.

ETHICS AND MORAL DELIBERATION


Ethics is a body of practical knowledge composed of principles and values, judgments about cases and policies,
and beliefs and theories about the world and persons (3). John Dewey's view of the main purpose of ethics is
integration of beliefs about the world with beliefs about the values and purposes that should direct human
conduct (4). Decision making for infants and children should follow the same general approaches taken for all
patients who are unable to make decisions for themselves.
Making judgments in novel situations can lead to change of background beliefs and practices. Since the advent
of modern neonatal care in the 1960s, rapid advances in technology and aggressive care of smaller and sicker
neonates have focused philosophical, theologic, professional, legal, consumer, and media attention (both
positive and negative) on the use of technology and the processes for decision making in life or death choices
for newborns. One can trace changes in background beliefs and values in the history of ethics in neonatal
decision making using landmark cases in which medical decisions for infants have been scrutinized by the
United States government or the legal system. Table 8.1 provides a sample of important cases, events, articles,
and studies in that history.
Neonatologists, nurses, and parents of newborns today stand on the cumulative moral experience of the past.
The process for decision making in NPM is more transparent and shared between clinicians and parents than in
the past. However, it is never immune from confusion, especially when cultural beliefs about medicine
P.86
collide. Practitioners are arguably better trained to identify ethical issues, participate in shared decision making,
and seek help with ethical problems. For example, most try to be empathetic, nonbiased, and honest in
disclosing a poor or uncertain prognosis to anxious parents.
TABLE 8.1 Landmark Cases in Neonatal Ethics

Year Name of Clinical Issues Outcome


Case, Event

1963 Hopkins Babya Trisomy 21, duodenal atresia; Died after 15 d of no


parental refusal of surgery. feeding/hydration.

1974 Baby Houleb,c Multiple malformations; parental Court orders surgery; infant died.
refusal.

1973 Duff and Selective nontreatment of Controversial report from an NICU.


Campbelld multiple infants with a variety of
medical conditions.

1981 The Danville Siamese twins, joined at the The parents and physicians were
Twinse abdomen, with three legs and a found not guilty.
common pelvis. The parents and
physicians were criminally
charged with ordering that the
infants not be fed.

1981 Stinson Babyf Premature infant; 800 g, 26 wk; Infant dies at 6 mo.
parents object to unwanted
treatment.

1982 Bloomington Trisomy 21, tracheal esophageal Indiana Supreme Court rules for
Baby (Baby fistula; parental refusal of parents.
Doe)g surgery.

1983 Baby Jane Spina bifida, hydrocephalus, and NY Court of Appeals rules for parents.
Doeh microcephaly; parental refusal of
surgery.

1983 Baby Doe U.S. Dept. of HHS issues Requires life-sustaining treatment for
regulationsi regulations. every infant.

1983 President's Clarifies decisions to forgo More moderate than U.S. Dept. of
Commissionj treatment in newborns. HHS regulations.

1984 Child Abuse Federal law. To receive federal funds for child
Protection Actk protection, states must have
procedures for such cases.

1990 Baby Kl,m,n Anencephaly. Mother demands life supports, and


federal courts rule for her.

1994 Messengero,p,q Premature infant; 780 g, 25 wk. Father disconnects respirator, and
Parents request no resuscitation. jury finds him not guilty of
manslaughter.

1999 Montalvor Premature infant; 23 wk, 679 g. Court ruled parents do not have the
After 3 y, parents sue alleging right to withhold life-sustaining
they were not advised about treatment in the absence of persistent
risks. vegetative state. Also implied parents
were not wholly responsible for
these decisions.

2000 HCA v. Millers,t Premature (629 g, 23 wk) infant Court of Appeals reverses prior
resuscitated against parents' judgment awarding parents $60
wishes. million.

2004 Sun Hudsonu Thanatophoric dysplasia Texas court supported hospital's


request to withdraw the ventilator
against mother's wishes; Texas'
Advance Directives Act

aGustafson JM. Mongolism, parental desires, and the right to life. Perspect Bio Med 1973;16:524.

bMaine Medical Center v Houle, No 74-145, 1974 (Super. Ct. Cumberland Co. Me. Feb. 14, 1974).

cMcCormick RA. To save or let die: the dilemma of modern medicine. JAMA 1974;229:172-176.

dDuffRS, Campbell AGM. Moral and ethical dilemmas in the special care nursery. N Engl J Med
1973;289:890.

eStinson R, Stinson P. The long dying of Baby Andrew. Boston, MA: Little Brown, 1983.

fMurray TH, Caplan AL. Beyond Babies Doe. In: Murray TH, Caplan AL, eds. Which babies shall live:
humanistic dimensions of the care of imperiled newborns. Clifton, NJ: Humana Press, 1985:3.

gState ex rel. Infant Doe v Baker, No. 482 S 140 (Ind. May 27, 1982).

hWeber v Stony Brook Hosp, 476 NY.S. 2d 685, 686 (App. Div.); Bowen v American Hospital
Association, 476 US. 610 at 611(1986).

iU.S.Department of Health and Human Services. Nondiscrimination on the basis of handicaps:


procedures and guidelines relating to health care for handicapped infants. Fed Regist 1984;49: 622-
654.

jU.S.
President's Commission for the Study of Ethical Problems in Medicine and Biomedical and
Behavioral Research. Seriously ill newborns, in deciding to forego life-sustaining treatment: a report on
the ethical, medical, and legal issues in treatment decisions. Washington, DC: U.S. Government
Printing Office, 1983:197.

kChild Abuse Protection Act, 42 U.S.C. 5103 (1982).

lIn re Baby K, 832 F. Supp. 1022 (E.D. Va. 1993); In re Baby K, 16 F. 3d 5900 (4th Cir.).

mAnnas G. Asking the courts to set the standard of emergency carethe case of Baby K. N Engl J Med
1994;330:1542.

nParis JJ,
Crone RK, Reardon FE. Physician refusal of requested treatment: the case of Baby K. N Engl
J Med 1990;322:1012.

oState v Messenger, file 94-67694-FY, Clerk of the Cir. Ct. County of Ingram, Mich.

pClark FI. Making sense of State v Messenger. Pediatrics 1996;97:579.

qParis JJ.
Manslaughter or a legitimate parental decision? The Messenger case. J Perinatol
1996;16:60.

rMontalvo v Borkovec, 647 NW 2d 413(Wis App 2002).

sHCA v Miller, 2000 WL 1867775, Tex. App. Hous. (Dec. 28, 2000).

tParis JJ,
Schreiber MD, Reardon F. The emergent circumstances exception to the need for consent:
the Texas Supreme Court ruling in Miller v HCA. J Perinatol 2004;24:337.

uHopper L. Houston Chronicle. January 26, 2005, section A, page 01.

HHS, Health and Human Services; NICU, neonatal intensive care unit.

Neonatologists, nurses, and their colleagues are fallible. They have made serious mistakes in excluding parents
from decisions and imputing guilt to them for wanting to withdraw treatment (5). Some events and cases test the
limits of moral concepts: the best interests of the infant, parental autonomy, professional integrity, futility, fairness
in use of resources, or quality of life. These cases brew storms that can throw experienced neonatologists,
nurses, other staff, parents, and administrators off balance and into conflict with one another. Ethics helps
human beings to keep enough balance, when taking hazardous, puzzling, or new paths in social and personal
life, to maintain moral insight and equilibrium.
P.87
CULTURAL AND RELIGIOUS DIVERSITY
Ethics is a self-correcting, constantly evolving body of practical knowledge. Therefore, open societies require a
public process of debate about continuity and change in the moral aspects of social and professional practices.
The United States is now the world's most diverse religious and cultural society. These differences are very
difficult to sort out in debate about ethics. To change public policy in this society, faith-based reasons standing
alone are insufficient. Secular and presumably rational reasons are required to persuade a court, legislature, or
commission. In debatable health care decisions, we see cases involving newborns that break down the limits of
rationality. For example, should physicians continue to respect parents' faith-based rejection of futility of
treatment when the baby has total intestinal necrosis or renal agenesis and pulmonary hypoplasia? In our view,
the case of Baby K (6) shows that the federal courts can approve parental faith-based demands as a legally
justified reason to continue futile treatment of an anencephalic newborn, because the society has been unwilling
to ration resources for this category of newborns.
Cross-cultural issues deeply complicate debate on ethics. For example, should any moral weight be given to a
request to withdraw life support if the parents of a neonate are immigrants from a society with very different moral
beliefs about medicine and what constitutes a seriously impaired newborn? Should weight be given to parents'
religious views when the infant is in pain and not benefiting from additional procedures and the parents flatly
reject the concept of futility, relying instead on faith in miracles?
This chapter presents a broadly pragmatic approach to ethics and to cross-cultural issues in ethical debates.
Moral norms and ways of life are, in fact, relative to culture and personality. However, cultural and psychological
relativity does not drain ethics of content or moral force. Although ethics is a phenomenon within and not
independent of human experience, it has objectivity in two senses. First, ethics is objective as a growing and
evolving body of knowledge about practices in society and the professions. Secondly, the basic imperatives of
ethics are no less objective than law or medicine (3) when it comes to destructive acts like murder, torture,
genocide, rape, cruelty for its own sake, and so forth. Such acts violate our most basic convictions about the
intrinsic value of human life and community. As Benjamin writes, If these things aren't wrong, nothing is (3).
Some argue that moral judgments are not binding across cultural boundaries or that ethics is merely a matter of
personal taste. If ethics is merely subjective or relative, there is no basis for a moral judgment about any horrific
act that occurs in subcultures or beyond one's own culture, such as forcing children to wage war against their
own people or to suffer loss of limb or life. The case of Baby K ought not be judged to exemplify horrific acts,
because the parents' intentions are loving and not destructive. The moral failure in these cases is societal in its
failure to assume responsibility for setting democratically debated limits on health care resources. Societal
failures can result in horrific consequences, but the parents and staff involved in these cases do not deserve
moral blame.

Working through Ethical Problems


Although important moral values and principles are binding, there is no prefabricated way to work through ethical
problems in cases or events when principles collide. When compared with highly theoretical or case-by-case
approaches, the method of wide reflective equilibrium (7) has proven useful in bioethics. In this approach, one
weighs ethical problems in cases by creating a dialogue (internally or externally) that moves between three
interactive elements: the values and principles at stake, the problem at hand, and relevant background beliefs
and theories.
Principalism is a dialectical method used widely in ethics courses and the literature of neonatal ethics, which
analyzes ethical problems in a framework of prima facie principles of biomedical ethics (8). However, a method
that moves only between values or principles and the problem at hand lacks larger constraints and correctives.
Cultural influences and personal bias are at work in selecting principles and putting them to work in cases.
Judgments made in past cases, such as Baby K (6) and Baby Doe (9), are fallible and need evaluation.
Background beliefs and theories are sources of critical distance and constraintbeliefs and theories about the
nature of personhood, community, the world as revealed by science and metaphysics, human psychology,
sociology, and political and economic behavior; the nature of nonhuman animals, and so forth. In short, our uses
of principles and judgments in cases ought to make sense in terms of intelligible background beliefs and in-depth
knowledge of the issues at hand.
Current neonatal intensive care practice is a complex, multidisciplinary, multispecialty approach that can be
confusing to both clinicians and families: frequent changes of nursing, resident, and attending staff; frequent shift
turnovers; poor communication among team members. The clinical team should have a plan of care or possible
options for care agreed upon before discussion with the family. Clarity, transparency, and openness to family
input are important to communication.
When parents and clinicians are unable to reach agreement on an approach to care, additional resources should
be available to assist the process:
Social worker.
Chaplains.
Parent support groups.
Ethics CommitteesCommittee process varies widely from institution to institution in terms of membership,
involvement of family, and decision-making models (10).
Ethics Consultation Serviceavailable to review the situation with those involved, provide background or
policy information, facilitate communication, and, when necessary, bring the parties together to work toward a
consensus. In most institutions, the committee or consultation service recommendations are advisory rather
than binding (10).

Moral Status of the Newborn


Part of the evolution in clinical ethics in NPM has involved ascribing greater moral status and protection at birth
to seriously ill newborns. There has been a dramatic transition from frequent nontreatment of trisomy 21 (11) to
the Baby Doe Rules (12) and support for appropriate assessment of extremely-low-birth-weight (ELBW) infants
(13). However, there remains a philosophical impression that decisions to withhold or withdraw treatment for
newborns, especially the extremely premature newborn, may be less troublesome than are decisions for older
children and adults, because the newborn does not have the same moral worth or is not fully a person. Tooley
(14) argued that because a newborn lacks advanced brain function or has no self-consciousness, ability to
suffer, or sense of the future, it should not be considered a person. Engelhardt (15) agrees that neonates are not
persons in the strict sense at the time but affirms that they are persons in a social and cultural sense. Others
have further defined this view, identifying the bonding, affection, and care given newborns by parents and other
adults (16). Janvier has documented that neonatologists, as well as other physicians, lawyers, and ethicists,
would withhold resuscitation or aggressive care at the request of parents of an extremely premature infant more
readily than for an older infant, young child, or adult with far worse prognoses for survival and/or long-term
function. The decision for the premature is similar instead to that for the very elderly, demented patient
(17,18,19). These authors suggest this reflects a greater sense of responsibility, guilt, or moral culpability in
the case of the disabled newborn patient. The older child, already here, would possibly die without therapy,
but any disability was due to his or her disease. The premature may be seen as not yet here in terms
P.88
of relationship to others, interactions, etc. If the premature infant survives with significant disability, the clinician
may perceive that But for our actions, there would be no disabled child. They have defined a perception of
saving vs creating (20).
While detailed plans for conception, antenatal ultrasound, and fetal movement may support parental attachment
early in and progressing through gestation, it cannot be denied that these emotions are not as strong in the
perinatal period, especially when the infant is extremely preterm, as they are at several months or years of age.
In addition, many preterm infants are the result of unplanned, often unwanted, or sometimes unknown
pregnancies. Blustein describes the neonate as not born into the family circle so much as outside it, awaiting
inclusion or exclusion. The moral problem the family must confront is whether the child should become part of the
family unit (21). Ross notes that respect for the person is owed to all individuals on the basis of the individual's
personhood (and developing personhood) proportionate to the actualized capacities of the individual and his
or her potential to attain full personhood (22). It is therefore problematic that the infant's moral worth is variable,
dependent on acceptance by its family rather than on any innate socially or even legally accepted character.
This worth may change over a short period of time, and this change may then affect differences in or difficulties
with decision making prior to birth versus at a week or month of life. Decisions made in the labor room regarding
a child who has never been seen by the parent and who may have an increased risk of mental retardation
become much more difficult when the 6-month-old child has confirmed severe developmental delay.

Decision Making
Sound clinical decision making should be based on sound data, a careful and thorough diagnostic assessment,
and, based on that diagnostic assessment, accurate prognostic estimates. Sometimes, this is relatively easily
accomplished: confirmed trisomy 13 has a well-defined natural history. In other situations, the diagnosis may not
be well defined or the prognosis is uncertain. For both the ELBW infant about to be born or the premature infant
with grade III intraventricular hemorrhage, there is an increased statistical risk of developmental delay, but it is
not certain how this particular infant will progress. Rhoden (23) has defined strategies that have been or might
be used when there is uncertainty about prognosis:

Wait until certain: Continue until the patient is actually dying or will survive but with definite severe disability.
Some very critically ill infants might survive with aggressive care. There is little attention paid to suffering,
burden-benefit ratios, or the number of infants needed to be treated for one additional intact survivor.
Statistical prognosis: Use statistical cutoffs and aggressively treat all those selected. This might be described
as the evidence-based approach. Selection might be by birth weight or gestational age. This approach may
be used when resources are limited. Professional, regional, or national guidelines might exist to define these
cutoffs (e.g., resuscitation at 23 to 24 weeks). This approach ignores individual variation and may sacrifice
some potentially normal infants who may behave outside of the norm. It relies on data that may or may not
accurately reflect the clinical situation at hand. Decision making is psychologically easier, because it is
allegedly objective.
Individualized prognosis: Decide for each infant using the available data, the present condition, and a benefit-
burden analysis. This approach allows for clinical change, evaluation and reevaluation, and ongoing
communication. There is more of a role for the family in decision making. It also can be a source of confusion,
uncertainty, error, and agony. However, Rhoden believes that this is justified, given the tragic nature of the
situations. Fischer and Stevenson (24), and Kraybill (25), have expanded on this approach beginning with a
nonprobabilistic paradigm of attempting to save every ELBW infant's life (provisional intensive care for all)
modified by an individualized prognostic strategy when prognosis could be better defined. The American
Academy of Pediatrics has endorsed this approach (26).

Withholding/Withdrawing
Most agree that it is ethically superior to withdraw a therapy compared to withholding it (27,28,29). If therapy is
begun and is effective, the patient benefits. If the therapy is begun but is not effective, it can be stopped. If the
therapy is never initiated, the patient can never benefit. Initiation of therapy also provides the clinician with
additional time to collect data, which may lead to a more accurate diagnosis and therefore more reliable
prognosis and allows the family more time to understand the situation. While a preference to withdrawing rather
than withholding is sound philosophically, in actual clinical settings, there are often emotional responses and, in
some settings, religious restrictions to withdrawal of a therapy. It is much easier emotionally to be passive than to
make an active decision to withdraw aggressive care. Pulling the plug and killing my baby are not infrequently
heard. There is a potential for weeks or months of care, and possible pain and suffering, before the infant's
death or an eventual decision to withdraw. The clinical situation may reach the point that there is no aggressive
care to withdraw, and the outcome is poor.
Clinically, the withdrawal of life-sustaining therapy does not require that the patient have multiorgan system
failure or meet criteria for brain death. If the organ that has failed or been irreversibly injured is a vital one,
decision making should be based on the infant's prognosis for recovery, long-term survival, quality of life, and so
forth. Technology would be withdrawn, and the infant would be allowed to die. Brain death or death by
neurologic criteria is a clinical and legal definition of a type of death. Brain death is not a requirement for
withdrawing life-sustaining treatment in most situations. Criteria for brain death in the newborn are somewhat
different than for older children and adults (30).
The term euthanasia generates a great deal of confusion and debate in legal, legislative, media, and clinical
spheres. Active versus passive euthanasia, voluntary versus involuntary euthanasia, physician-assisted suicide,
and other descriptors have created unfortunate ambiguity about the actual issue at hand. If one defines
euthanasia or active euthanasia as directly and actively causing the death of a patient who may not be
imminently dying or is dependent on life-sustaining technology, usually by administering a lethal dose of a drug,
most state law and policies of the American Medical Association and the American Academy of Pediatrics would
prohibit that action (31,32). How often active euthanasia of neonates actually occurs in the United States is not
known. Confusion arises when the term passive euthanasia is used for decisions to withdraw life-sustaining
therapy with the expectation that the patient will die (33). Nonescalation of care, that is, continuing current
treatment but not adding additional aggressive therapy is a newer, commonly used approach that softens the
need for more difficult decisions. The use of medication to treat symptoms of pain or dyspnea or other suffering
in the context of comfort or palliative care further compounds the confusion, in spite of an ethical duty to provide
this type of care. Some would argue that the overall intention may be the same as or may certainly blur into
active euthanasia. However, others would suggest that intention is an important determinant (34). Describing
what actually is being considered as a plan of care and avoiding the terminology may prevent the confusion and
the associated emotion.

The Role of Parents


Parents are the decision makers for their children. This role is understood to have social, legal, and ethical
facets; some are better defined than others, and some are potentially in conflict with others. Parents should be in
the best position to judge what is in their child's best interest. They will be the ones who will continue
P.89
to raise the child after the newborn period. Outcome studies document the potential difficulties families may have
to face following the birth of an infant who will have long-term medical and educational needs (see Chapter 56).
They will be the ones who will have to deal with the consequences of the decisions. Ethically, this right and
responsibility of parents has been the focus of considerable analysis and commentary since the Baby Doe era.
The extremes of no one, including parents, should make these decisions and an absolute right of parents to
make medical decisions are simplistic and unrealistic. Parents should be seen as guardians of their child's
welfare, not owners.
Bartholome (35) suggested using the language of permission, a term somewhat less rigid than consent. He
sees the parents' role as a duty rather than a right, a duty to ensure the provision of necessary medical care.
Parental permission for interventions into children's lives must not be seen as the unconditional right to demand
or refuse a particular intervention because it is a proper exercise of parental authority over the lives of children.
That children are largely dependent, at least for a time, on their parents is to be affirmed, but that dependency
does not warrant the secondclass social standing implied by a parental right of consent in decisions affecting
the health care of their children. Child abuse and neglect statutes frequently result in challenges to parental
decision and control.
Weir (36) proposes that the parents as decision makers should:
Have relevant knowledge and information about medical facts, prognosis, and family setting
Be impartial
Be emotionally stable
Be consistent
This final quality should assure that the process ends with the same result in similar cases. However, what
information the parents receive and from whom may clearly impact their decision. The clinician has an obligation
to present accurate, up-to-date information. Several studies document the different prognoses presented by
obstetricians versus neonatologists for the extremely premature infant (37). Others reflect major differences in
clinical approach and parental counsel between intensivists and rehabilitation physicians for children who are or
might become ventilator dependent (38).
Other issues in the newborn period complicate the process relative to the parents' emotional stability and
consistency:

Prolonged, geographic separation.


Transfer to an NICU before the parents have seen the infant.
Maternal medication or illness.
Lack of support from other family members.
The father of the baby may not be involved.
Intense feelings of grief, depression, denial, and fear.
Language barriers
Time constraints in the delivery room or for emergency procedures

Standards for Decision Making


The usual broad standard for decision making for this population is to decide what is in the child's best interests.
However, defining best interests can be difficult and reflects a basic problem with the use of terms that may
carry very different meanings for different individuals.
Weir and Bale (39) suggest eight variables for evaluating best interest:
The severity of the patient's medical condition
The achievability of curative or corrective treatment
The important medical goals in the case (such as prolongation of life, relief of pain, or amelioration of disabling
conditions)
The presence of serious neurologic impairments
The extent of the infant's suffering
The multiplicity of other serious medical problems
The life expectancy of the infant
The proportionality of treatment-related benefits and burdens
Is the best-interests standard not, in fact, quite subjective; potentially defined differently by clinicians and family?
How do we define how much potential pain and suffering is acceptable for a given outcome, especially when the
outcome may not be precisely predicted? How do we define an acceptable outcome? Is being alive but
vegetative acceptable? Is a 20% or 40% or 70% risk of death or developmental disability acceptable? Is it in the
best interests of a child with trisomy 21 to have surgery for duodenal atresia, or the child with trisomy 18 to have
open heart repair for congenital heart disease? How differently should one consider a 25% risk of a poor
outcome (e.g., for the very-low-birth-weight [VLBW] infant) versus a known, defined poor prognosis (e.g., for
trisomy 13)? Silverman (40) proposed abandoning the term best interests and adopting the concept of a
standard of reasonableness proposed by Veatch (41). Here, the issue is how far the technology is pursued to
accomplish reasonable ends defined by those most directly affected by the decisionsthe parents.
Are best interest or reasonableness standards the same as quality of life standards? The latter term is
often viewed negatively because of the even greater subjective element. How does one define a good or bad
quality of life? Does defining a quality of life that is poor reflect potential for discrimination against individuals with
disabilities? In response to the death of an infant with trisomy 21 whose parents refused surgery for
tracheoesophageal fistula, the federal government in 1982, under pressure from right-to-life advocates and
advocacy groups for the handicapped, proposed regulations (12) to prohibit hospitals from withholding care from
newborns, specifically excluding clinical judgments based on quality of life.
Certain conditions, however, were identified as not requiring treatment, including anencephaly, trisomy 13, and
ELBW.
What one clinician sees as a good quality of life may be unacceptable to another professional or a parent. A
family may see the life of a child who is profoundly visually handicapped but with normal intelligence as
qualitatively poor. Although many families do not feel burdened with a moderately mentally retarded child, others
consider learning disability with normal intelligence unacceptable. Clinicians who work with developmentally
impaired children often have a very different appraisal than do laypersons of the quality of life in this patient
population.
However, it may be possible to use quality of life as a basis for decision making when considering more
fundamental issues. Richard McCormick (42) proposed a minimal condition for defining quality: the capacity
for experience or social interrelating. If the condition is not met, as with anencephaly, treatment is not required.
Coulter and associates (43) define interests that would constitute a minimal quality of life as:
Freedom from intractable pain and suffering. Mental retardation, paralysis, or cerebral palsy would not be
considered physical suffering; dyspnea or intractable physical pain would.
Capacity to experience and enjoy lifethe ability to enjoy food, warmth, or the caring touch of another; the
ability to give or receive love.
Expectation of continued lifeheroic treatment, when death will likely occur in a few weeks or months, may be
cruel.
Others approach the issue from a nonmaleficence point of view: avoiding an intolerable life (44) or avoiding harm
(45).
There has been considerable debate about how much attention should be paid in the decision-making process
to interests other than the child's. Parents may be overwhelmed with the prospects of
P.90
chronic medical care, financial burdens, difficulty in raising a handicapped child, need for special education, and
harm to other children in the family. Some parents may focus on their own psychological and financial interests,
protecting their lifestyle and other children at home. Fost (46) suggests: The history of childhood is one that
does not support idyllic notions of parents as decision makers for their children. It is naive to posit an identity of
interest between infant and parent [in all situations]. Parents guard their own interests, those of the family as a
unit, and those of current and future siblings all of which may be gravely threatened by the newborn (47).
Most would argue that the parents should not refuse treatment that would be in the infant's interests in order to
avoid burdens to the family (36,48). Others suggest that the impact of a decision on the welfare of the family may
or should be taken into account. Silverman (49) comments, parents of a badly damaged baby often resent that
their family is required to pass a sacrifice test to satisfy the moral expectations of those who do not live, day by
day, with the consequences of diffuse idealism. It is easy to demand prolongation of each life that requires
none of [the clinician's] own resources to maintain that life later. Ross (50), using the concept of the intimate
family, proposes a model of constrained parental autonomy, where the parent should be guided by the child's
well-being, but is not obligated to disregard all personal interests of themselves or other children in order to fulfill
the child's needs and interests. Others agree that the baby's interests should not invariably take precedence
over those of the family (44). This is an issue that requires sensitivity to the parents and family situation, but at
the same time balancing the short- and long-term needs of the child.
What is the clinician's role in decision making for the infant? Do pediatric practitioners have a stronger
responsibility for the decisions made for the child than the clinician caring for an adult patient? The Academy of
Pediatrics defines providers' responsibilities as follows:
Proxy consent poses serious problems for providers. [They] have legal and ethical duties to their child
patients to render competent medical care based on what the patient needs, not what someone else expresses
The pediatrician's responsibilities to his or her patient exist independent of parental desires or proxy
consent (51).

Futility
Conflict also may arise when the parents demand care that the clinician feels is inappropriate, futile, and
potentially harmful to the child. The clinician should not be required to provide care that he or she considers
harmful or unethical. What is futile care? Who defines futility? Types of futility have been defined (52):
Physiologicinterventions that are inappropriate because they have zero chance of being effective or have
demonstrated a lack of effect in the clinical situation at hand
Quantitativea treatment that is only effective in x%where should the threshold be set?
Qualitativeprobably the most common situation. What may be futile in the eyes of the clinician may be
beneficial for the child from the parents' point of view. Being kept alive on the ventilator with no chance of
recovery and minimal or no social interaction may be sufficient for the parents to continue. Do benefits
outweigh the burdens to the patient?
A family may request nonrecommended treatment for multiple reasons
Distrustof the limits of prognostication, due to a prior medical error, access to other sources of information,
of the medical system in general (especially prevalent among African-Americans and Hispanics)
Cognitive issuesfailure to understand the clinical status of the patient due to the complexity of the language
used to explain the situation, pressure to make a decision, confusion over uncoordinated or conflicting
information from different professionals
Psychological and emotional issues (discussed earlier)
Values, religion, and miracles (52)
The case of Baby K complicated this question even further when the federal court upheld the mother's demand
for resuscitation and aggressive treatment of her anencephalic infant (6). Again, ethics consultation or ethics
committee involvement may facilitate resolution. The clinician may transfer the patient to another clinician (or
institution) who is willing to provide the care. Some institutions have developed procedures for case review that
may conclude that life-sustaining treatments can be withdrawn, even against the objections of the family (53).

The Extremely-low-birth-weight Infant


Infants who are born at gestational ages of less than 27 weeks or who weigh less than 800 g have been the
focus of considerable professional, legal, and media debate about withholding resuscitation in the delivery room,
parental requests for aggressive care or no resuscitation, mortality rates, and quality of outcome (both short term
into later infancy and long term to school age) (44,54,55). There are hundreds of outcome studies and reviews
regarding this population, many of which are discussed in other chapters of this book. The problems of decision
making (discussed previously) are painfully apparent. For example, the clinician has an obligation to accurately
inform the parents expecting an ELBW infant about the clinical situation, prognosis, anticipated clinical course,
and so forth. While there is certainly no lack of outcome data, it is clear that there are wide variations in outcome
from one study to another. The studies are also faulted by the sample size of infants at any gestation, by the
selection criteria (were all live born infants included or only those admitted to the NICU, inborn or outborn, etc.),
and by decisions about viability and nonresuscitation that then impact mortality of the sample as a whole.
Mortality will, by definition, be higher if most infants were not resuscitated because of clinician or family
preference. Drop out and incomplete followup, small numbers, and variation in definitions of morbidity impact the
usefulness of long-term outcome studies. With such a wide range of study results, there is a risk or tendency for
an individual clinician to choose those that support his or her philosophical approach (conservative or
aggressive). Fortunately, there are several large, collaborative studies now available that lessen the impact of
uncontrolled variables and provide more reliable outcome data (56,57,58). Ideally, clinicians should have
mortality and morbidity data for their own institution in addition to national statistics to share with the parents.
Use of gestational criteria for decision making is reasonable; each additional gestational week adds a significant
increase in survival percentage. However, in many cases both obstetrical estimates using ultrasound and
neonatal estimates using physical exam scoring are not accurate enough to define 23 versus 24 or 24 versus 25
weeks of gestation (59,60,61). The current practice of quoting gestational age in fractions of a week (e.g., 23
5/7) provides a sense of accuracy that is not justified by the existing data and can be misleading both to parents
and clinicians.

Guidelines for Resuscitation/Care


Several professional groups have developed criteria or guidelines for decision making for the ELBW population.
In 1994, the Canadian Paediatric Society and the Society of Obstetricians and Gynaecologists of Canada
defined relatively specific recommendations:
At 22 completed weeks of gestation, they suggest treatment should be started only at the request of fully
informed parents or if it appears that the gestational age has been underestimated. At
P.91
23 to 24 completed weeks of gestation, they emphasize a significant role for parental wishes, the option of
resuscitation, and the importance of discussion with parents about the need for flexibility in deciding to start or
withhold resuscitation, depending on the infant's condition at birth. Finally, at 25 weeks of gestation, they state
resuscitation should be attempted for all infants without fatal anomalies (62).
Of note, these guidelines have not been revised.
Tyson and Stoll (63) have suggested developing fairly detailed guidelines based on outcome data. Female
and/or small-for-gestational-age infants would be resuscitated at lower birth weights than male and/or
appropriately grown infants. Antenatal steroids would lower the recommended weight further. They recommend
mandatory resuscitation when the data reveal a greater than 50% chance of survival without severe sequelae
and optional resuscitation when the chance is 25% to 49%. The Neonatal Resuscitation Program, which is
supported by the American Academy of Pediatrics and the American Heart Association, suggests that
noninitiation of resuscitation for newborns of less than 23 weeks' gestational age and/or 400 g in birth weight is
appropriate (64). Interestingly, clinicians have overinterpreted these criteria as either demanding resuscitation at
23 weeks or 400 g or as stating that it is inappropriate to resuscitate at less than 23 weeks or 400 g (63).
A Working Party from the Nuffield Council on Bioethics developed a very detailed report on this issue from both
an obstetrical and neonatal perspective. They proposed that:
Below 22 weeks, no baby should be resuscitated
Between 22 weeks 0 days and 22 weeks 6 days, standard practice should be not to resuscitate a baby. Only if
parents request resuscitation after a thorough discussion with an experienced pediatrician about the risks and
long-term outcomes, should resuscitation be attempted.
Between 23 weeks 0 days and 23 weeks 6 days, it is very difficult to predict the outcome for an individual
baby. Preferences should be given to the wishes of the parents regarding resuscitation and treatment.
Between 24 weeks 0 days and 24 weeks 6 days, normal practice should be that a baby would be offered full
intensive care unless the parents and clinicians are agreed that, in light of the baby's condition or likely
condition, it is not in his or her best interests to start intensive care.
At 25 weeks of gestation or above, intensive care should be initiated.
They also recommend that professional bodies who choose to produce similar guidelines should review those
guidelines regularly and revise them to reflect any changes in outcomes for this population (44).
Discussions with the family should include
the uncertainty of gestational age if that is at issue
the advantage of assessing and then making decisions
the possibility of withdrawal of support if it is apparent that there will be a bad outcome
the importance of ongoing assessment and ongoing communication
To avoid confusion and conflict, the obstetrician and the neonatologist should coordinate their approaches. For
the extremely immature infant, the parents should be aware that there may be nothing that can be done. There
may be physical limitations to the resuscitation. In most circumstances, firm decisions about how to proceed
should be avoided. Likewise, vague terms such as no heroic measures or do everything can cause confusion
and conflict. Several recent papers have discussed the importance of and techniques for antenatal and
intrapartum discussions (65,66,67). When possible, the use of educational materials, tours of the NICU, or
videos of NICU experiences may further the parents' understanding of the situation.

Infants with Severe Encephalopathy


Infants with perinatal hypoxic-ischemic injury may have lethal multisystem failure. However, more often they are
able to survive despite severe central nervous system (CNS) injury. Prolonged, difficult-to-control seizures,
hypotonia; poor feeding; apnea; and inability to maintain body temperature are poor prognostic signs for
developmental outcome. Imaging studies (computed tomography [CT], magnetic resonance imaging [MRI]) as
well as an electroencephalogram (EEG) may add additional prognostic data. Some infants may be ventilator
dependent because of poor respiratory drive. Others may require gastrostomy feedings. Prognosticating may be
difficult early after the insult, but in some circumstances, the clinical data convincingly show that the prognosis is
extremely poor. Do not resuscitate orders and/or withdrawal from the ventilator are appropriate decisions at this
time. This approach based on an anticipated extremely poor prognosis is separate from and does not require a
determination of brain death.

Infants with Severe Congenital Malformations/Chromosomal Anomalies


The approach to children with severe anomalies has changed dramatically in the history of neonatology,
especially in the past 10 years. In a series of articles and commentaries beginning in 1973, pediatricians Duff
and Campbell (11) reported on a practice of selective nontreatment at Yale-New Haven Hospital that resulted in
the deaths of 43 infants over a period of 30 months. Physicians and parents decided together, with the
physicians sometimes yielding to parental wishes. Surveys at that time confirmed that many pediatricians and
pediatric surgeons frequently accepted selective nontreatment of seriously impaired newborns, including infants
with trisomy 21 (68,69). With the Baby Doe case, advocates for the handicapped, developmental pediatricians,
and family support groups promoted a change in approach for infants with trisomy 21 or meningomyelocele.
Treatment and approaches to rehabilitation and special education have improved; the current standard of care in
the United States is that infants with trisomy 21 receive the same treatment that is indicated for a child with a
similar medical or surgical condition who does not have trisomy 21.
There are other conditions associated with extremely poor prognoses for survival or for reasonable quality of
life, including anencephaly and other severe CNS developmental anomalies. Most of these infants will not
survive beyond the first few months of life. Those who do survive have severe neurodevelopmental delays. Many
have life-threatening conditions in the immediate newborn period and require life-sustaining treatment to prolong
survival. The Neonatal Resuscitation Program guidelines in 2000 suggested that noninitiation of resuscitation is
appropriate for confirmed diagnoses of anencephaly or trisomies 13 or 18 (70).
In the latter situations, every parent of such an infant should have appropriate counseling about the diagnosis.
When the diagnosis is made in utero, a definite plan for care in the delivery room (including cesarean section for
fetal distress, endotracheal intubation, aggressive resuscitation) and during the newborn period can be very
helpful and comforting to the parent. Again, the guideline does not state that it is inappropriate to resuscitate.
When a condition is not diagnosed until after birth, there is the usual obligation for the clinician to provide the
parents with appropriate, accurate information in a timely fashion. Some families wish to pursue aggressive
treatment, and their wishes should be considered.
Since 2000, there has been an ongoing reassessment of the care for infants with trisomy 13 and 18 (71,72,73).
Internet sites and parent support groups have expanded the options available to families
P.92
for treatment (74). The term lethal condition has been criticized as misleading, since some of these infants will
survive into early childhood and beyond. Parents are requesting C-sections to increase the chances of a live
birth. While cardiac surgery for infants with trisomy 18 was formerly quite uncommon, there are now increasing
requests for such procedures (75,76). Trisomy 13 and 18 are no longer listed in the NRP suggested conditions
for noninitiation of resuscitation (64). The issues of parental autonomy, best interests, and burdens of therapy as
discussed above are critical. Parents desire empathy, continuity of care, good communication, and a sense that
the clinician values the pregnancy, fetus, and newborn (77). If surgery is considered for a life-threatening
anomaly, a plan should be developed regarding how to proceed if the infant does not tolerate the procedure,
becomes ventilator dependent, and so forth. Parents and clinicians should always be open to modifying any plan
of care as the situation changes. If the clinician is not willing to offer this level of care, efforts should be made to
transfer care to another physician or facility.

Artificial Nutrition and Hydration


While most agree that withdrawing artificial nutrition and hydration from hopelessly ill, persistently vegetative or
dying patients may be appropriate (78,79), this issue becomes less well defined in the newborn population, who
are all dependent on others for feeding and where tube feeding is the standard of care for many until they
develop the ability to feed. There are circumstances, however, where discontinuing intravenous nutrition or tube
feedings may be appropriate (44,80,81,82). If the infant is actively dying, there is no benefit to feeding or
hydration, and experience would suggest that there may be less suffering from excess secretions if the patient is
somewhat dehydrated (78). Severe neurologic impairment in the newborn is, unfortunately, not rare. However,
exact prognostication about recovery is difficult, and some infants who need feeding support initially may develop
the ability to feed normally after a period of recovery. When clinical evaluation strongly suggests an extremely
poor neurologic outcome, it may be reasonable to withhold feedings (81). Factors to consider include whether
the infant is able to feed at all or whether the infant demonstrates hunger or satiety. Infants may have medical
conditions that preclude enteral feeding (e.g., extreme short gut syndrome), and, therefore, they are dependent
on total parenteral nutrition (TPN). TPN has many potential complications including loss of venous access,
infection, and liver toxicity. If the possibility of slow recovery of absorptive ability or successful bowel transplant is
poor, withdrawal of TPN may be an option after consideration of the criteria discussed earlier. Withdrawal of
feeding often presents more psychological and emotional angst than withdrawal of other life-sustaining
technology because of the cultural connotations of feeding and starving. Many clinicians are more reluctant
to consider discontinuing artificial nutrition than mechanical ventilation (29), and some religious groups conclude
that nutrition and hydration are treatments that must be provided in almost all circumstances.

Palliative/Comfort Care and Pain Control


When aggressive care or life-sustaining technology is withheld or withdrawn, care does not stop. The clinician
has a strong ethical obligation to the patient and the parents to provide comfort care, relief of symptoms,
adequate pain control, warmth, feedings, if desired, and emotional support (83). Catlin and Carter (84) have
developed a protocol for neonatal end-of-life palliative care. Palliative care should be available for an infant with
a life-threatening condition, for example, trisomy 13, or with a refractory or chronic debilitating condition for which
there is little hope of long-term survival. Palliative care functions on a multidisciplinary model that includes the
parents and family. When possible, the approach may continue at home, progressing into a community-based
hospice program. The availability of palliative and hospice care should be discussed with parents antenatally
when a fetus is diagnosed with a condition likely to be incompatible with postnatal prolonged survival (85,86).

High-Technology Home Care


With technologic advances in the hospital has come the use of this same technology in the hometube
feedings, oxygen, and mechanical ventilators. This development has raised numerous ethical issues both on an
individual patient basis and related to the general issues of resource allocation, funding, and health planning
(86,87). How a family and the clinician reach the conclusion to proceed or not proceed with long-term home care,
especially mechanical ventilation, requires careful attention to the details of adequate information, parent
education and training, planning for professional assistance in the home, adequate support and funding,
opportunity for respite care, and so forth. Studies suggest that there is wide variation in physician practice
regarding offering home ventilation, with families then receiving unbalanced information (38). With the growing
incidence of ventilator use in the home, parent support groups and the Internet offer a very positive bias.
Physicians may feel that this is fiscally inappropriate, or that the child's quality of life may be poor, or that the
family will be unable to manage at home. Home ventilation for older children and adults with a variety of diseases
provides a great deal of satisfaction for many of them (88). Every child and family is unique in their clinical
situation, prognosis, family goals and stamina, and resources. On the other hand, just because the technology
for home ventilation is now available does not require its use in every circumstance. If the family is unable or
unwilling to provide less intensive care in the home (e.g., tube feeding or oxygen), other options must be
identified. It is inappropriate to discharge an infant with heavy ongoing health care needs home to a family that is
ill prepared and does not have adequate support from outside services.

SUBSTANCE ABUSE IN PREGNANCY


Substance abuse during pregnancy has incited a great deal of media and legal attention. Prosecutors and
governmental agencies have charged mothers with homicide and child abuse, imprisoned them for the duration
of their pregnancies, and removed their children from their custody (89,90,91). Complicating the ethical and legal
issues is a wide range of scientific opinion about the short- and long-term effects of drug exposure in utero on
the child (92,93). Recent studies suggest that the rearing environment is much more critical for the child's
development (92). Routine drug screening of pregnant women without their consent has been ruled
unconstitutional (94). Punitive approaches in general are counterproductive, potentially keeping women away
from prenatal care (89). Routine screening of newborns should be limited to situations in which there is a clinical
history or signs or symptoms compatible with drug exposure or withdrawal. Testing infants solely to identify
substance-abusing mothers is ethically controversial: why is the test being done, what are the criteria for who is
tested, and does testing require parental consent? The approach to mothers and babies should be supportive
and nonpunitive (95).

GENETIC TESTING
In the past two decades, there has been a dramatic increase in knowledge about the human genome and the
ability to screen for the presence of certain genetic diseases, carrier states, traits, or predispositions to disease.
Genetic testing of a newborn raises different issues than routine medical testing performed as part of clinical
care. The genetic information relates not only to the individual tested but also to other members of the family.
Results of testing may have psychological (guilt, anxiety), social (stigmatization, discrimination), and financial
impact (insurability, employment
P.93
considerations) with long-term consequences. Some genetic information defines risks only and does not predict
with any certitude a specific condition or outcome. Finally, many defined conditions do not necessarily have
effective therapy. For these reasons, concerned professional organizations have developed policies and
guidelines related to these issues. Testing should involve effective counseling, informed consent, and attention
to confidentiality. Genetic testing to confirm a medical diagnosis would be an appropriate component of medical
care, for example, DNA analysis for cystic fibrosis in an infant with meconium ileus or chromosomal analysis for a
newborn with clinical features of Down syndrome. Testing for conditions that may benefit from monitoring,
prophylaxis, or treatment in an otherwise healthy individual (e.g., familial hyperlipidemia) may also be in the
child's best interest. However, carrier screening for diseases with no risk to the pediatric patient should be
avoided. Likewise, screening for adult-onset conditions should be deferred until adulthood or until the mature
adolescent is able to consent. Recent changes in policy have recognized, however, the traditional deference
given to parents about how they raise their children and that the interests of the child are embedded in and
dependent on the interests of the family unit. After careful genetic counseling, it may be ethically acceptable to
proceed with predictive genetic testing to resolve disabling parental anxiety or to support life-planning decisions
that parents sincerely believe to be in the child's best interests. (96)

MAKING DECISIONS REGARDING THE APPROPRIATENESS OF


INTERINSTITUTIONAL TRANSFER
The transfer of neonates from referring to referral hospitals potentially adds a layer of ambiguity to the process of
ethical decision making, particularly if there is uncertainty on the part of the NPM team at the referral hospital
regarding the level of treatment received by the infant since birth. For example, a 25-week gestation premature
infant who has been set aside as nonviable but is found gasping 1 hour later and resuscitated is unlikely to
benefit from transfer to another institution for aggressive support.
A regional network of hospitals can reduce this problem by having a common set of criteria for delivery room
practices and interinstitutional referral. Fletcher and Paris (97) suggested the following mnemonics to assist in
formulating these policies:
1. ACUTE (Acute, Critical, Unexpected, Treatable, and Easily diagnosed). Assuming appropriate immediate
postnatal care at the referring facility, infants included in this group are those with prematurity and respiratory
distress syndrome; ELBW infants of gestational age known to be 25 weeks or greater; term or preterm infants
with sepsis, pneumonia, or meningitis; and infants with surgically correctable malformations.
The authors place these infants in a definite category for transfer to an NICU capable of providing the level
of care required.
2. UNSURE (UNknown disease, SUspected REsponse). This group includes preterms of 23 to 24 weeks of
gestational age or with VLBW and uncertain gestational age, infants with severe birth asphyxia, and any infant
with an unexplained disease or syndrome that requires further diagnostic efforts. Within this group, there will
be a significant number of infants for whom the response to treatment will be unpredictable. These infants
should be given full medical care until the diagnosis has been made or the response to treatment is clear;
decisions to omit care or not to transfer should not be made precipitously.
3. KNOT (Known, NOt Treatable). Although only a small number of infants fit into this category, treatment
decisions for this group frequently take a disproportionate amount of time. This group includes neonates with
anencephaly and those with lethal genetic disorders such as trisomy 13 and 18.
Transfer of neonates with anencephaly for aggressive support is not indicated; transfer of those with lethal
genetic defects is not indicated if there are facilities for accurate diagnosis and appropriate care and counseling
at the hospital of birth. When diagnostic facilities are not available at the birth hospital, on-site consultation by a
specialist from the referral hospital is an appropriate alternative to transferring the infant.

REFERENCES
1. Buchanan AE. Is there a medical profession in the house? In: Spece RG, Shimm DS, Buchanan AE, eds.
Conflict of interest in clinical practice and research. New York: Oxford University Press, 1996:105.

2. Emanuel EJ. The ends of human life: medical ethics in a liberal polity. Cambridge, MA: Harvard
University Press, 1991.

3. Benjamin M. Philosophy and this actual world. Lanham, MD: Rowman & Littlefield, 2003:112.

4. Dewey J. Human nature and conduct. Carbondale, IL: Southern Illinois University Press, 1988:164.

5. Stinson R, Stinson P. The long dying of Baby Andrew. Boston, MA: Little Brown, 1983.

6. In re Baby K, 832 F. Supp. 1022 (E.D. Va. 1993); In re Baby K, 16 F. 3d 5900 (4th Cir.).

7. Brown-Ballard J. Consistency, common morality, and reflective equilibrium. Kennedy Inst Ethics J
2003;13:231.

8. Beauchamp TL, Childress JF. Principles of biomedical ethics, 7th ed. New York: Oxford University Press,
2013.

9. State ex rel. Infant Doe v Baker, No. 482S 140 (Ind. May 27, 1982).

10. Smith ML. Mission, vision, goals: defining the parameters of ethics consultation. In: Hester DM, Schonfeld
T, eds. Guidance for healthcare ethics committees. New York: Cambridge University Press, 2012:32.

11. Duff RS, Campbell AGM. Moral and ethical dilemmas in the special care nursery. N Engl J Med
1973;289:890.

12. U.S. Department of Health and Human Services. Nondiscrimination on the basis of handicaps:
procedures and guidelines relating to health care for handicapped infants. Fed Regist 1984;49:622.

13. Paris JJ, Schreiber MD, Reardon F. The emergent circumstances exception to the need for consent:
the Texas Supreme Court ruling in Miller v HCA. J Perinatol 2004;24:337.

14. Tooley M. Abortion and infanticide. Philos Public Aff 1972;2:37.

15. Engelhardt HT. The foundations of bioethics. New York: Oxford University Press, 1986:116.

16. May WF. Parenting, bonding, and valuing the retarded. In: Kopelman LM, Moskop JC, eds. Ethics and
mental retardation. Dordrecht, The Netherlands: D. Reidel, 1984:141.

17. Janvier A, Leblanc I, Barrington KJ. The best-interest standard is not applied for neonatal resuscitation
decisions. Pediatrics 2008;121:963.

18. Laventhal N, Spelke MB, Andrews B, et al. Ethics of resuscitation at different stages of life: a survey of
perinatal physicians. Pediatrics 2011;127:e1221.

19. Fontana MS, Farrell C, Gauvin F, et al. Modes of death in pediatrics: differences in the ethical approach
in neonatal and pediatric patients. J Pediatr 2013;162:1107.

20. Janvier A, Mercurio MR. Saving vs creating: perceptions of intensive care at different ages and the
potential for injustice. J Perinatol 2013;33: 333.

21. Blustein J. The rights approach and the intimacy approach: family suffering and care of defective
newborns. Mt Sinai J Med 1989;56:164.

22. Ross LF. Children, families and health care decision-making. Oxford, UK: Clarendon Press, 1998:47.

23. Rhoden NK. Treating Baby Doe: the ethics of uncertainty. Hastings Cent Rep 1986;16:34.
24. Fischer AF, Stevenson DK. The consequences of uncertainty: an empirical approach to medical decision
making in neonatal intensive care. JAMA 1987;258:1929.

25. Kraybill EN. Ethical issues in the care of extremely low-birth-weight infants. Semin Perinatol
1998;22:207.

26. Committee on Fetus and Newborn, American Academy of Pediatrics. The initiation or withdrawal of
intensive care for high-risk newborns. Pediatrics 2007;119:401.

27. Beauchamp TL, Childress JF. Principles of medical ethics, 7th ed. New York: Oxford University Press,
2013:158.

28. Fletcher JC. The decision to forgo life-sustaining treatment when the patient is incapacitated. In: Fletcher
JC, Spencer E, Lombardo PA, eds. Introduction to clinical ethics. Frederick, MD: University Publishing
Group, 2005:212.

29. Feltman DM, Du H, Leuthner SR. Survey of neonatologists' attitudes toward limiting life-sustaining
treatments in the neonatal intensive care unit. J Perinatol 2012;32:886.

P.94

30. Nakagawa TA, Ashwal S, Mathur M, et al. Guidelines for the determination of brain death in infants and
children: an update of the 1987 Task Force recommendations. Pediatrics 2011;128:e720.

31. Council on Ethical and Judicial Affairs, American Medical Association. Euthanasia. In: Code of medical
ethics: current opinions with annotations. Chicago, IL: American Medical Association, 2012:114.

32. Section on Hospice and Palliative Medicine, and Committee on Hospital Care, American Academy of
Pediatrics. Pediatric palliative care and hospice care commitments, guidelines, and recommendations.
Pediatrics 2013;132:966.

33. Sklansky M. Neonatal euthanasia: moral considerations and criminal liability. J Med Ethics 2001;27:5.

34. Edwards SJ. The distinction between withdrawing life-sustaining treatment under the influence of
paralyzing agents and euthanasia. The doctrine of double effect is difficult but not impossible to apply. BMJ
2001;323:390.

35. Bartholome WG. The child-patient: do parents have the right to decide. In: Spicker SF, Healey JM,
Engelhardt HT, eds. The law-medicine relation: a philosophical exploration. Boston, MA: Reidel, 1981:271.

36. Weir R. Selective treatment of handicapped newborns: moral dilemmas in neonatal medicine. New
York: Oxford University Press, 1984.

37. Haywood JL, Goldenberg RL, Bronstein J, et al. Comparison of perceived and actual rates of survival
and freedom from handicap in premature infants. Am J Obstet Gynecol 1994;171:432.
38. Hardart MKM, Truog RD. Spinal muscular atrophytype 1. Arch Dis Child 2003;88:848.

39. Weir RF, Bale JF Jr. Selective nontreatment of neurologically impaired neonates. Neurol Clin
1989;7:807.

40. Silverman WA. Medical decisions: an appeal for reasonableness. Pediatrics 1996;98:1182.

41. Veatch RM. Abandoning informed consent. Hastings Cent Rep 1995;25:5.

42. McCormick RA. To save or let die: the dilemma of modern medicine. JAMA 1974;229:172.

43. Coulter DL, Murray TH, Cerreto MC. Practical ethics in pediatrics. Curr Probl Pediatr 1988;18:168.

44. Nuffield Council on Bioethics. Critical care decisions in fetal and neonatal medicine: ethical issues.
London, UK: Nuffield Council on Bioethics, 2006.

45. Hester DM. Ethical issues in pediatrics. In: Hester DM, Schonfeld T, eds. Guidance for healthcare ethics
committees. New York: Cambridge University Press, 2012:119.

46. Fost N. Parents as decision makers for children. Prim Care 1986;13:285.

47. Dellinger AM, Kuszler PC. Infants: public-policy and legal issues. In: Reich WT, ed. Encyclopedia of
bioethics. New York: Simon & Schuster and MacMillan, 1995:1214.

48. Beauchamp TL, Childress JF. Principles of biomedical ethics, 7th ed. New York: Oxford University
Press, 2013:173.

49. Silverman W. Overtreatment of neonates? A personal perspective. Pediatrics 1992;90:971.

50. Ross LF. Children, families and health care decision-making. Oxford, UK: Clarendon Press, 1998:51.

51. Committee on Bioethics, American Academy of Pediatrics. Informed consent, parental permission, and
assent in pediatric practice. Pediatrics 1995;95:314.

52. Pope TM. Medical futility. In: Hester DM, Schonfeld T, eds. Guidance for healthcare ethics committees.
New York: Cambridge University Press, 2012:89.

53. Truog RD. Futility in pediatrics: from case to policy. J Clin Ethics 2000; 11(2):136.

54. Berlinger N, Jennings B, Wolf SM. The Hastings center guidelines for decisions on life-sustaining
treatment and care near the end of life. New York: Oxford University Press, 2013:79.

55. Lantos JD. The Lazarus case: life-and-death issues in neonatal intensive care. Baltimore, MD: Johns
Hopkins University Press, 2001.
56. Neonatal Research Network, NICHD. http://neonatal.rti.org

57. Extremely Preterm Outcomes Data, Neonatal Research Network, NICHD.


http:\\www.nichd.nih.gov/about/org/der/branches/ppb/programs/epbo_case.aspx

58. Moore T, Hennessy EM, Myles J, et al. Neurological and developmental outcome in extremely preterm
children born in England in 1995 and 2006: the EPICure studies. BMJ 2012;345:e7961.

59. Haidet KR, Kurtz AB. Routine ultrasound evaluation of the uncomplicated pregnancy. In: Spitzer AR, ed.
Intensive care of the fetus and neonate. St. Louis, MO: Mosby-Year Book, 1996:45.

60. American Academy of Pediatrics, Committee on Fetus and Newborn, American College of Obstetricians
and Gynecologists, Committee on Obstetric Practice. Perinatal care at the threshold of viability. Pediatrics
1995;96:974.

61. Donovan EF, Tyson JE, Ehrenkranz RA, et al. Inaccuracy of Ballard scores before 28 weeks gestation. J
Pediatr 1999;135:147.

62. Fetus and Newborn Committee, Canadian Paediatric Society; Maternal Fetal Medicine Committee,
Society of Obstetricians and Gynaecologists of Canada. Management of the woman with threshold birth of
an infant of extremely low gestational age. CMAJ 1994;151:547.

63. Tyson JE, Stoll BJ. Evidence-based ethics and the care and outcome of extremely premature infants.
Clin Perinatol 2003;30:363.

64. American Academy of Pediatrics. Ethics and care at the end of life. In: Kattwinkel J, ed. Textbook of
neonatal resuscitation, 6th ed. Elk Grove Village, IL: American Academy of Pediatrics, 2011:288.

65. Guillen U, Suh S, Munson D, et al. Development and pretesting of a decision-aid to use when counseling
parents facing imminent extreme premature delivery. J Pediatr 2012;160:382.

66. Janvier A, Lorenz JM, Lantos JD. Neonatal counseling for parents facing an extremely preterm birth:
limitations of the medical evidence. Acta Paediatr 2012;101:800.

67. Govande VP, Brasel KJ, Das UG, et al. Prenatal counseling beyond the threshold of viability. J Perinatol
2013;33:358.

68. Todres ID, Krane D, Howell MC, et al. Pediatricians' attitudes affecting decision-making in defective
newborns. Pediatrics 1977;60:197.

69. Treating the defective newborn: a survey of physicians' attitudes. Hastings Cent Rep 1976;6:2.

70. American Academy of Pediatrics. Special considerations. In: Braner D, Kattwinkel J, Denson S, et al.,
eds. Textbook of neonatal resuscitation, 4th ed. Elk Grove Village, IL: American Academy of pediatrics, 2000:
Lesson 7.

71. McGraw MP, Perlman JM. Attitudes of neonatologists toward delivery room management of confirmed
trisomy 18: potential actors influencing a changing dynamic. Pediatrics 2008;121:1106.

72. Wilkinson DJ. Antenatal diagnosis of trisomy 18, harm and parental choice. J Med Ethics 2010;36:644.

73. Merritt TA, Catlin A, Wool C et al. Trisomy 18 and trisomy 13: treatment and management decisions.
NeoReviews 2012;13:e40.

74. Support Organization for Trisomy 18, 13 and Related Disorders. Available online at www.trisomy.org

75. Boss RD, Holmes KW, Althaus J, et al. Trisomy 18 and complex congenital heart disease: seeking the
threshold benefit. Pediatrics 2013;132:161.

76. Yates AR, Hoffman TM, Shepherd E, et al. Pediatric sub-specialist controversies in the treatment of
congenital heart disease in trisomy 13 or 18. J Genet Couns 2011;20:495.

77. Janvier A, Farlow B, Wilfond BS. The experience of families with children with trisomy 13 and 18 in social
networks. Pediatrics 2012;130:293.

78. Lynn J, Childress JF. Must patients always be given food and water? Hastings Cent Rep 1983;13:17.

79. American Academy of Pediatrics, Committee on Bioethics. Guidelines for forgoing life-sustaining medical
treatment. Pediatrics 1994;93:532.

80. Nelson LJ, Rushton CH, Cranford RE, et al. Forgoing medically provided nutrition and hydration in
pediatric patients. J Law Med Ethics 1995;23:33.

81. Carter BS, Leuthner SR. The ethics of withholding/withdrawing nutrition in the newborn. Semin Perinatol
2003;27:480.

82. Rapoport A, Shaheed J, Newman C, et al. Parental perceptions of forgoing artificial nutrition and
hydration during end-of-life care. Pediatrics 2013;131:861.

83. Field MJ, Behrman RE, eds. When children die: improving palliative and end-of-life care for children
and their families. Washington, DC: The National Academies Press, 2003.

84. Catlin A, Carter B. Creation of a neonatal end-of-life protocol. J Perinatol 2002;22:184.

85. Carter BS. Comfort care principles for the high-risk newborn. NeoReviews 2004;5:e484.

86. Lantos JD, Kohrman AF. Ethical aspects of pediatric home care. Pediatrics 1992;89:920.

87. Goldberg AI, Faure EAM, O'Callaghan JJ. High-technology home care: critical issues and ethical choices.
In: Monagle JF, Thomasma DC, eds. Health care ethics: critical issues for the 21st century. Gaithersburg,
MD: Aspen Publications, 1998:146.

88. Bach JR, Campagnolo D, Hoeman S. Life satisfaction of individuals with Duchenne muscular dystrophy
using long-term mechanical ventilatory support. Am J Phys Med Rehabil 1991;70:129.

89. Acuff K. Perinatal drug use: state interventions and the implications for HIV-infected women. In: Faden
RR, Kass NE, eds. HIV, AIDS and child-bearing: public policy, private lives. New York: Oxford University
Press, 1996:214.

90. American Academy of Pediatrics, Committee on Substance Abuse. Drug-exposed infants. Pediatrics
1995;96:364.

91. DeVille KA, Kopelman LM. Substance abuse in pregnancy: moral and social issues regarding pregnant
women who use and abuse drugs. Obstet Gynecol Clin North Am 1998;25:237.

P.95

92. Jos PH, Marshall MF, Perlmutter M. The Charleston policy on cocaine use during pregnancy: a
cautionary tale. J Law Med Ethics 1995; 23:120.

93. Frank DA, Augustyn M, Knight WG, et al. Growth, development, and behavior in early childhood following
cocaine exposure: a systematic review. JAMA 2001;285:1613.

94. Chavkin W. Cocaine and pregnancytime to look at the evidence. JAMA 2001;285:1626.

95. Ferguson v Charleston 532 U.S. 67 (2001).

96. Ross LF, Saal HM, David KL, et al. Technical report: ethical and policy issues in genetic testing and
screening of children. Genet Med 2013;15:234.

97. Fletcher AB, Paris JJ. Bioethical issues surrounding transport of neonates. In: MacDonald MG, eds,
Miller MK, assoc. ed. Emergency transport of the perinatal patient. Boston, MA: Little Brown & Company,
1989:173.
9
Law, Regulation, Quality Assurance, and Risk Management
Harold M. Ginzburg
Mhairi G. MacDonald

BASIC LEGAL AND REGULATORY CONCEPTS


Introduction
Those who cannot remember the past are condemned to repeat it, is an insight often attributed to George
Santayana, a 19th to 20th century philosopher, essayist, poet, and novelist. Edmund Burke wrote, in the 18th
century, Those who don't know history are doomed to repeat it. Thus, a brief historical overview is provided in
this chapter.
The Pharaohs had their physicians, the slaves did not. Throughout recorded history, the affluent had either sole
or better access to medicinal herbs and health care advisors. Disappointed leaders banished or executed their
unsuccessful health care providers. The Babylonian Code of Hammurabi, estimated to have been written nearly
4,000 years ago, is generally considered the first codified legal document that addressed medical issues,
including wages and scaled punishments (1). The entire document consisted of 282 laws chipped into stone and
clay tablets. The Affordable Care Act, 2010, (also known as Obamacare) is more than 1,000 pages in length
and is but one of thousands of legislative acts or laws that govern the 21st century practice of the healing arts
within the United States (2). Each state functions as a sovereign nation and may have its own legal codes and
regulations governing the healing arts and professions and the medications prescribed and dispensed. As
medicine evolved from the domain of the priest or shaman herbalists to that of physician specialists, the
remedies for medical injury adjudicated by the legal system also evolved, in Western Society, from the Code of
Hammurabi (an eye for an eye/and/or coin(s) of silver and gold for permanent injury, untoward or unexpected
consequences or death), to confinement for criminal activities such as assault and battery (unwanted or
unauthorized touching of a patient), to pecuniary or monetary damages for reported present and future emotional
and physical damages.
Community standards for what was considered the minimum threshold of care below which the conduct of a
health care professional would be sanctioned were developed. In the Middle Ages, in England, compensation
was to be paid for errors of medical judgment or perceived less than satisfactory results. In other domains, if a
physician or barber-surgeon were found responsible for poor medical care, he or she could be deformed in a
similar manner to the damaged patient. Thus, physicians and barber-surgeons were not motivated to treat
patients with complicated illnesses unless the patient and his or her extended family clearly understood that
treatment would be palliative at best.
Today, inadequate (from the patient or patient's family's perspective) communication, coupled with unrealistic
expectations for treatment outcome, produces family anger and guilt and may lead to litigation. The family
expects success, as they define it. The neonatologist's definition of success in an individual case may differ
substantially from that of the family. Litigation may be initiated when negligence has actually occurred or when it
is perceived to have occurred. Poor communication and failure to convey empathy toward the family members
can be as self-destructive for the health care provider as a faulty knowledge base, performing in an
unprofessional manner, or functioning in an impaired capacity. Good medical knowledge and practical clinical
skills alone are not sufficient to preclude involvement in a malpractice litigation.
Large institutions with a substantial number of health care providers can be perceived as being impersonal. If, in
addition, the communication of members of the health care team with the patient or family members is
inadequate, the lack of understanding that results may form the primary basis for litigation.
Patients, their families, and the public at large need to understand that health care providers are not able to
succeed all the time. Sometimes, the nature and extent of the disease is too life threatening or debilitating;
sometimes, the state-of-the-art knowledge of the pathophysiology and/or treatment is not yet adequately
developed; sometimes, the treatment options are fraught with significant risks; sometimes, errors in judgment and
skill are made. Over the past few years, some states (within the United States) have passed laws that are
referred to as I am sorry laws, which allow health care providers to speak frankly with a patient and/or his or
her family and explain what may have gone wrong in a manner that promotes dialogue and compassion and
potentially avoids subsequent litigation. Coincident with the writing of this chapter, in March 2014, the National
Health Service in the United Kingdom introduced regulations that mandate that health care providers reveal and
explain all significant medical errors. In addition, the Joint Commission on Accreditation (TJC) in the United
States now requires health care organizations to disclose unanticipated injuries or complications, perform root
cause analyses as to what occurred and why it occurred, and institute an action plan to prevent future
occurrences (3). In similar fashion, the Joint Commission International (TJCI) publishes the International
Accreditation Standards for Hospitals in nations willing to accept their accreditation standards (see Accreditation
of Health Care Activities) (4). It appears that regulatory agencies are developing an understanding that this level
of transparency should be the gold standard, and frank communication has been shown to decrease the
incidence of malpractice litigation (5). Prior to the middle of the 20th century, members of the healing profession
functioned as an integral part of their local community. Since the 1940s, there has been a progressive separation
of health care providers from the communities they serve; this process has been accelerated by the advent of
highly subspecialized, expensive intensive care. Neonatologists function in a crisis environment with little or no
prior knowledge of their patients' family unit.
Isolation of patients from health care providers, except in times of crisis, can lead to poor or limited
communication and unrealistic expectations.

Responsibility and Liability


The mundane aspects of health care and treatment, such as scheduling of appointments, documentation of
procedures, and understanding of the federal, state, and local guidelines, procedures, policies, regulations, and
laws, are frequently the basis for confrontations between members of the medical and legal professions.
The vast majority of health care education, within the United States, pertains to understanding basic sciences
and providing clinical services. Little formal systematic attention and education, within the healing arts, are given
to the myriad of governmental policies, procedures, and regulations that control all aspects of health care
delivery.
Medical care is a legal contract between the health care professional and the patient. In almost all instances, if
the patient is unable, because of age and/or illness and/or linguistic and/or cultural perspective, to render
informed educated consent, then others must provide such consent. Thus, as a patient is registered into a health
care system, examined, or interviewed, law and medicine become intimately intertwined.
P.97
The basic legal considerations relating to the care and treatment of any patient, and particularly, a neonate, flow
from the following four concepts:
1. The duty to act. When does the health care professional-patient or health care facility-patient relationship
commence?
2. Knowledge and application of hospital policies and local, state, and federal mandates. What resources are
available to facilitate information transfer to health care facilities and service providers?
3. Responsibility and accountability of the health care provider (individual or organization) to provide adequate
care and treatment. Who is responsible for the decisions made in the provision of health care? Who monitors
the quality of the services provided? Who ensures that the services provided are consistent with hospital
policies and local, state, and federal mandates?
4. Information transfer to patients and their families or guardians. Who obtains educated informed consent, in
what manner, and with what documentation? Who is responsible for providing ongoing medical information to
the families or guardians of neonates and ensuring that the information, and the implications of the
information, are understood? State and federal legislation, such as the Health Insurance Portability and
Accountability Act (HIPAA) of 1996 (6) and the Patient Protection and Affordable Care Act of 2010
(OBAMACARE) (2), do not set standards for the manner in which a clinician may or should communicate with
a patient, parent, or guardian.

Defensive Medicine
Defensive medicine has become a medical term of art. It should connote a thoughtful systematic approach to
health care, rather than the demonstration of poor judgment such as the excessive ordering of investigatory
studies because of anticipatory fear of litigation for malpractice. Medical malpractice lawsuits are based on the
principle of negligence. Negligence implies some wrongful act of commission or omission. The essence of
negligence is unreasonableness.

Due Care
Due care is simply reasonable conduct (7). In order for negligence to be demonstrated in a courtroom, the injured
person/plaintiff must demonstrate that (a) there was a legal duty owed to him or her; (b) there was a breach of
that duty (a deviation from the accepted standard of care); (c) as a result of the duty and the breach thereof,
damages or an injury occurred; and (d) the damages or injury can be determined to have been caused by, or
shown to have flowed from, the care or lack of care provided by the health care provider(s) and/or organization
responsible for the environment in which the health care was provided ( Fig. 9.1).
FIGURE 9.1 The elements of negligence. There must be an unbroken chain for successful litigation. If any
link is not proved, the plaintiff will lose the case. From Ginzburg HM. Legal issues in patient transport. In:
MacDonald MG, ed. Emergency transport of the perinatal patient. Philadelphia, PA: Little, Brown and Company,
1989:163, with permission.

Quality assurance and risk management aspects of medical care are relatively recent innovations designed to
improve patient care and outcome. Quality assurance activities accept the legal and medical position that a
health care provider owes a duty to the patient to provide reasonable medical care, consistent with available
resources. There are inherent, irreducible risks in the delivery of health care and treatment, and quality
assurance and risk management assessments are designed to identify and limit the risks.
Informed consent that includes a relative risk assessment of potential complications can be used to document
educated (8), informed consent. The informed consent provides a written mechanism to explain to the patient
and his or her family and guardians that there are always inherent risks involved in a medical intervention, which
must be weighed against the inherent risks involved in not rendering that same medical intervention. The
balance of relative risks needs to be understood by the health care provider and the patient and/or
parent/guardian.

The Duty to Act


The duty to act is determined when the health care professional-patient or organization-patient relationship
commences (7). A duty is a legal and ethical responsibility. There is no legal duty, under most circumstances,
for a health care provider or institution to accept a patient for care unless they hold themselves out as providing
emergency care or they are required to do so by law, regulation, or contract. The federal government, through
the Emergency Medical Treatment and Active Labor Act (EMTALA), states that if a medical center, hospital, or
physician represents themselves, to the public, as a source of emergency medical care and/or specialty care,
and the community has come to expect such care, then a patient cannot be arbitrarily denied such services (9).
Once a health care service is initiated, a health care provider/institution-patient relationship exists, a duty is
created, and there is then a legal and moral obligation not to abandon the patient. Further, the care that is being
provided must be adequate under the circumstances. The moral and legal obligation that attaches precludes
abandonment or dumping of the patient (9). A referring hospital transferring an infant to another institution for
further care is not perceived as abandoning the patient as long as the reason for transfer is medical and not
financial. The senior medical person responsible for the transport, whether stationed at a hospital or directly
providing patient care during transport, is deemed to be supervising the health care until the transport team
transfers care to the clinical staff at the receiving medical facility.
The complexity of health care responsibility and liability increased rapidly during the 20th century. Public health
care facilities have existed since the Middle Ages. Commencing in the 13th century, the Hotel Dieu in Paris
provided indigent care for many centuries. In the United States, city, municipal, state, and federal public hospitals
provided, and continue to provide, care for those unable to obtain it elsewhere. Historically, these physicians and
hospitals were not held liable for the outcome of care that was provided free of charge. This doctrine of
charitable immunity protected hospitals from legal liability if medical negligence occurred within their boundaries.
However, an individual's inability to pay for medical care no longer affects his or her ability to demand and
receive services that are commensurate with those provided to patients who pay for their care directly or through
third-party payment systems. Thus, providing care to patients who are unable to pay no longer protects a health
care provider or medical institution from liability for negligence or malpractice. Physicians, other health providers,
suppliers, and manufacturers of equipment, medical devices, and medicines can now all be sued for negligence
and be held individually or jointly liable for their own actions, the actions of those that they supervise, and actions
performed by members of their health care team.
P.98
Licensure; Interstate-International Practice
In the United States, health care providers (physicians, nurses, emergency medical technicians, respiratory
therapists, etc.) may be licensed in more than one state. These health care providers, in general, must be
licensed in the state in which they maintain their primary office or place of employment. The authorities in most,
but not all, states are not concerned about whether a health care provider who enters the state solely to
transport a patient to another health care facility is licensed in that state; however, they are concerned that the
individuals involved in the transport are competent to perform their job.
An individual who enters a state, regardless of the reason, will be subject to the laws of that state. The most
obvious analogy is that if a driver is involved in an accident, he or she is subject to the laws of the state in which
the accident occurred, not those of the state that issued the driving license; this legal principle also applies to
medical transport vehicle operators (10). Failure to obtain informed consent for transport may result in litigation in
the state in which it was inadequately obtained or in the state to which the patient was transferred (see also
Chapter 5).
Patients and their guardians may initiate medical malpractice litigation in the state in which they reside, the state
in which the alleged negligence occurred, the state in which the hospital is located, or the state in which the
physician resides. If the patient/plaintiff can show that his or her residence is in a different state from that of the
defendant/hospital and defendant/health care provider, then the plaintiff may commence the litigation in a federal
court because the matter involves diversity of jurisdiction, that is, opposing parties are located in two or more
states. The defendant may also request that the matter be removed to the federal court system because of
diversity of jurisdiction (11). Most plaintiffs prefer state courts, especially if the defendant is from a different state.
Some state courts are known for their large awards to plaintiffs, whereas others are known to be more
sympathetic to defendants/health care providers; forum shopping does occur.
Advances in what was previously called telephone-linked care (TLC) and is now referred to as telemedicine,
include videoconferencing and sharing of electronic information across state, national, and international borders
(see also Chapter 7). In the United States, final revisions to telemedicine standards, from The Centers for
Medicare & Medicaid Services (CMS), have hastened the expansion of data sharing. CMS has adopted new
rules (and regulations) addressing credentialing for physicians involved in telemedicine (12). Home-bound
patients can be brought into the electronic era with passive remote monitoring and active data access to their
medical records. In the United States, the patients are generally perceived as owning their medical records.
Licensing and scope of practice issues are beginning to transcend state and national borders, resulting in
licensing and quality of care issues for state licensing authorities. For more than 15 years, throughout the world,
telemedicine has been integrated into direct patient care, monitoring patient progress, and serving as an
expander for specialized medical expertise and technology (e.g., the interpretation of neonatal radiologic or
cardiologic films and tracings at a remote site with the results being electronically communicated back to the
source hospital) (13). The American Academy of Pediatrics Council on Clinical Information Technology (COCIT)
was established in 2002 and was instrumental in the development of the Academy's Child Health Informatics
Center (CHIC) whose goal it is to support the development of health information technology (HIT) and use of
electronic health records (EHRs).
Telemedicine, as one would expect, shares the same issues that face-to-face medicine must address: regulatory
issues including standard and quality of care, credentialing and licensure, liability and scope of practice,
informed consent, confidentiality, privacy, and reimbursement.
Telemedicine has been practiced for as long as there has been the telephone. The neonatologist, especially at a
tertiary medical center, can regularly be expected to engage in telehealth. This includes providing consultation,
arranging transportation, and interpreting radiographic, cardiologic, and other data, as well as professional
education, community health education, and public health activities. The American Medical Association and
American Telemedicine Association have urged medical specialty societies to develop appropriate practice
standards. U.S. federal health care agencies, such as the Indian Health Service and the Department of Veterans
Affairs, and nongovernmental managed care organizations have embraced telemedicine. Louisiana, in 1995,
became the first state to enact legislation dealing with telemedicine reimbursement (14) that specifies a certain
reimbursement rate for physicians at the originating site and includes language prohibiting insurance carriers
from discriminating against telemedicine as a medium for delivering health care services.
At the present time, issues relating to cross-state licensure are still perceived as potential barriers to the
expansion of telemedicine (15), especially now that reimbursement is possible. States license physicians and
other health care providers within their boundaries, but the federal government and, in particular, the Department
of Health and Human Services (DHHS) and Centers for Medicare & Medicaid Services (CMS) have the authority
to prepare national licensure standards as they relate to national programs such as Medicaid and Medicare. In
the future, there may be alternative approaches to state licensure for health care professionals. Regardless of
the legal issues surrounding telemedicine, neonatologists increasingly cross state and international boundaries.
Thus, the need to appreciate that the laws of political jurisdictions other than their home state or country may
significantly impact the manner in which they practice and the associated liability.

Medical Torts and Contracts


The legal system is divided into two broad areascivil litigation and criminal prosecution. Civil litigation is based
on the need to correct or remedy a wrong between one individual, corporation, or partnership and another
individual, corporation, or partnership. Criminal prosecution is instituted to correct a wrong against the
community. In a civil litigation matter (or case), the plaintiff is the party initiating the lawsuit and alleging the
wrong; the lawsuit is filed against the party (defendant) who is accused of causing the damage. In a criminal
prosecution, the plaintiff is the government (local, state, federal) alleging that the community has been harmed by
the action or inaction of a party (also known as the defendant). Civil matters resulting in litigation generally are
either contract disputes or torts. A contract dispute occurs when two or more parties have entered into an
agreement and one or more parties believe that the terms and conditions of the agreement, either an oral or a
written contract, have not been met. It is important to appreciate that, in a court, in general, most oral contracts
have the same weight as written contracts (there are notable exceptions when it comes to the transfer of
ownership of real property).
A personal tort is an injury to a person or his or her reputation or feelings that directly results from a violation of a
duty owed to the plaintiff (in medical malpractice cases, this is usually the patient) and produces damage. The
remedy in any civil matter, after the nature and extent of the damages have been proved to the court (a judge
with or without a jury), is determined by a preponderance of evidence (more than 50.01%) (16). Thus, to a
reasonable medical degree of certainty means that it is more likely than not true and it is that standard upon
which there are usually monetary damages awarded if the matter is found in favor of the plaintiff. The
preponderance of the evidence rule is a threshold test (17). In general, either the plaintiff proves that the
damages were more likely to have been caused by the defendant than by
P.99
any other source and that he or she is, therefore, entitled to full compensation, or he or she fails to meet the
burden of proof and is entitled to nothing (18). In most instances in the United States, each side pays for its own
legal services, regardless of the outcome of the case. In other countries, such as in the United Kingdom, this is
not usually the case.

Battery
Battery is a tort; it is an intentional and volitional act, without consent, which results in touching that causes harm
(e.g., the touching of a patient's body without consent). A technical battery can occur when there is no actual
harm but touching occurred without consent. Patient care, even with a beneficial outcome but without informed
consent, is considered to be a battery.
Plaintiffs may sue for an injury that occurred as a result of negligence or a tort (physical or mental harm), or both.
Because the criminal court usually will not award monetary damages to the victim of a crime and because the
standard of proof for conviction, in the United States, is beyond a reasonable doubt (quantitatively, this can be
conceptualized as at least 95% certain), plaintiffs usually prefer to sue for injuries from a tort in civil court. In civil
litigation, monetary damages may be awarded, and if the injury is determined to be egregious, punitive damages
as a punishment and example to others may also be awarded.

Professional Negligence
Negligence is conduct, and not a state of mind (19), and it involves an unreasonably great risk of causing
damage (19) and is conduct which falls below the standard established by the law for the protection of others
against unreasonable risk of harm (20,21).
Professional negligence, or medical malpractice, is a special instance or type of negligence. The medical
profession is held to a specific minimum level of performance based on the possession, or claim of possession,
of special knowledge or skills that have been accrued through specialized education, training, and experience.
Ely and associates (22) found that when family physicians recalled memorable errors, the majority fell into the
following categories: physician distracters (hurried or overburdened), process of care factors (premature closure
of the diagnostic process and thus failure to identify the appropriate diagnosis), patient-related factors
(misleading normal laboratory results, incorrect or inadequate medical/psychosocial history), and physician
factors (lack of knowledge, inadequately aggressive patient management). Understanding the common causes of
errors alerts the practitioner to situations when and where, in the evaluation and treatment process, errors are
most likely to occur.

The Elements of a Malpractice Case


To establish a prima facie medical malpractice case (one that still appears obvious after reviewing the medical
evidence), the patient/plaintiff must demonstrate (Fig. 9.1) that (a) there is a duty on the part of the
defendant/health care provider and/or defendant/health care facility to the patient/plaintiff, (b) the defendant
failed to conform his or her conduct to the standard of care required by the relationship, (c) an injury to that
patient/plaintiff resulted from that failure, (d) the injury was the proximate cause, without other extraneous
interventions and as a result of that injury, and (e) quantifiable damages may be calculatedthen a negligence
verdict, in favor of the plaintiff, may be rendered (23).
Generally, in order for the plaintiff to establish a claim of medical malpractice, the plaintiff must establish by
medical expert testimony (a) what the applicable standard of care/knowledge base was at the time of the injury,
(b) how the defendant breached or violated that standard of care, and (c) that the breach or violation (also
referred to as the negligence) was the proximate cause of the injury.
A medical malpractice action can only proceed if the court determines that there is a genuine issue of material
fact and if damages are quantifiable (e.g., the future costs of treatment, economic lost value of productive
activities, etc.).
The most difficult element to prove is whether or not the care was adequate. The plaintiff usually must provide
expert witnesses to establish what a prudent health care provider in similar circumstances might have done; what
would be considered an acceptable standard of care rather than ideal or extraordinary care.
More than 130 years ago, in Massachusetts, it was held that a physician in a small town was bound to have only
the skill that physicians of ordinary ability and skill in similar localities possessed. The court believed that a small-
town physician should not be expected to have the skill of a physician practicing the same specialty in a large
city (24). It was also held that a physician was required to use only ordinary skill and diligence, the average of
that possessed by the profession as a body, and not by the thoroughly educated (25). However, in the age of the
Internet, and with continuing medical education classes available locally and at national medical conferences, a
health care provider is no longer excused for failing to keep up with medical progress in his or her specialty area.
Courts admit medical evidence based upon rules of evidence. In 1993, the United States Supreme Court in
Daubert v Merrill Dow Pharmaceuticals, Inc. ruled that the Federal Rules of Evidence standards for acceptance
of evidence would be used (26). In Daubert, the Supreme Court focused upon the admissibility of scientific expert
testimony. It pointed out that such testimony is admissible only if it is both relevant and reliable. In 1996, Kumho
Tire Corporation v. Carmichael , the Supreme Court addressed the issue of how Daubert applies to the testimony
of engineers and other experts who are not scientists (27). The Supreme Court, in Kumho, concluded that
Daubert's general holdingsetting forth the trial judge's general gatekeeping obligationapplies not only to
testimony based on scientific knowledge but also to testimony based on technical and other specialized
knowledge.
The Daubert case was an attempt to prevent junk science from distracting the jury. The court held that scientific
(medical) evidence had to be grounded in relevant scientific principles. The four criteria the court established are
as follows: (a) whether the theory or technique has been tested; (b) whether the theory or technique has been
subjected to peer review and publication; (c) the known or potential rate of error of the method used and the
existence and maintenance of standards controlling the technique's operation; and (d) whether the theory or
method has been generally accepted by the scientific community. Thus, publication in a peer-reviewed or peer-
refereed journal alone is not sufficient qualification for acceptance of evidence in a courtroom. The state and
federal district (trial) court judges maintain the latitude to permit or exclude experts, based on the perceived
scientific merit of the information they intend to provide to the court and, thus, to the jury. The fundamental issue
for a clinician is not an understanding of the rules of evidence and the workings of the civil justice system but
practicing medicine and acting in a professional manner, as documented in a patient's medical record. If there is
missing information, the presumption is usually made that the clinical data was not obtained or the procedure
was not performed.
Many states have medical peer-review panels in place. In these states, before a medical malpractice case may
be heard in a court, the facts of the case are presented to the medical review panel on behalf of both the plaintiff
and defendant. The medical facts often are buttressed by the opinions of retained medical experts for both sides.
In some states, the medical review panel is composed of attorneys and physicians; in other states, the medical
review panel is chaired by an attorney and comprises physicians in the same or similar medical specialty as the
physician defendant. Even when there is a finding for the defendant by the medical review panel,
P.100
the plaintiff may continue litigation in the local court. However, the findings of the medical review panel are
admissible on behalf of either the plaintiffs or defendants.

Res ipsa loquitur


There are circumstances in which no expert witness is required to corroborate the findings of negligence. The
doctrine of res ipsa loquitur essentially means that the thing or deed speaks for itself. Under such
circumstances, the negligence is inferred from the act itself, that is, proof from circumstantial evidence. In the
classic case Ybarra v Spangard, a patient was well prior to being anesthetized for an appendectomy, and when
he woke up, he had an injury to his arm (28). Clearly, the patient could not determine how his arm was injured;
the operating room and recovery room staff either could not or would not explain the etiology of the injury. The
court found for the injured plaintiff without the introduction of any expert witnesses because (a) the plaintiff had
not done anything that in any way could have contributed to the injury, (b) the injury could not have occurred
unless someone was negligent, and (c) the instrumentalities (hospital staff and physicians) that allegedly caused
the injury were at all times under the control of the defendant hospital.

Informed Consent
Informed consent requires that sound, reasonable, comprehensible, and relevant information be provided by a
health care professional to a competent individual (patient or parent/guardian) for the purpose of eliciting a
voluntary and educated decision by that patient (or guardian) about the advisability of permitting one course of
clinical action as opposed to another (20). Physicians and other health care providers are held to have a
fiduciary duty to their patients. Such a duty exists when one individual relies on another because of the unequal
possession of information. The failure to obtain proper informed consent may result in the defendant/health care
provider (often the physician) or defendant/hospital being sued for battery in some states or for negligence in
others.
According to the battery theory, the defendant is to be held liable if any deliberate (not careless or accidental)
action resulted in physical contact. The contact must have occurred under circumstances in which the
plaintiff/patient did not provide either express or implied permission and the defendant/health care provider knew
or should have known that the action was unauthorized. If the scope of consent obtained from the patient is
exceeded, a claim of battery is proper. The plaintiff in Mohr v Williams consented to have surgery performed on
her right ear (29). During the procedure, the surgeon determined that the right ear was not sufficiently diseased
to require surgery, but the left ear required surgery. Because the patient was already anesthetized, the surgeon
performed the operation. The operation was a success, but the patient successfully sued for battery. The court
held that there was no informed consent for an operation to the left ear. Thus, it is not necessary for injury to
occur for damages to be awarded; demonstration that there was unauthorized touching may be sufficient. In this
instance, the court found that there was no medical emergency that would have threatened the plaintiff/patient if
the surgery had not immediately commenced. If there had been evidence of a medical emergency, the court's
decision might have been different.
Failure to specifically identify the risks that accompany a surgical procedure also can result in a successful claim
of battery. In Canterbury v Spence, the plaintiff/patient successfully proved that he was not informed of the risks
attendant to the surgical procedure and that had he known them he would not have given permission (30). The
court held that the physician has a duty to disclose all reasonable risks of a surgical procedure and, because he
failed to perform that duty, the court held him liable for damages to the patient. The court noted that the concept
of informed consent might be more appropriately replaced with the concept of educated consent (8). The court
also articulated an objective standard that could be used in legal cases involving informed consent. This
objective standard is based on what a reasonable person in circumstances similar to that of the patient would
have decided if he or she had been provided with an adequate amount of information. Therefore, the central
issue in a medical battery case is whether an educated, effective, or valid consent was given for the procedure
that actually was performed.
A physician is not required to disclose every possible risk to a patient for fear of being guilty of battery (31). The
court in Cooper v Roberts held that [t]he physician is bound to disclose only those risks which a reasonable
man would consider material to his decision whether or not to undergo treatment (32). Thus, the court stated
that such a standard creates no unreasonable burden for the physician. However, the physician must disclose
risks that are material and feasible alternatives that are available. The information should be provided in a
language and manner that reflects the emotional and educational status of the patient or, when the patient is a
neonate, the parent(s) or guardian(s). In Davis v Wyeth Laboratories, the Court held that any medical
complication or risk that has a probability of greater than 1:1,000 should be included in the informed consent
(33).
When a therapeutic procedure is for the benefit of a minor, the decision to proceed usually belongs to the parent
or legal guardian. The failure of the parent or guardian to consent to blood transfusions or antibiotic treatment
(even if the refusal is based on sincere religious convictions), or other now routine procedures for a small child
that are clearly medically indicated and required for the maintenance of life, can be overridden, in the United
States, by the physician and/or hospital petitioning a court of appropriate jurisdiction for the appointment of a
temporary legal guardian (34) who is answerable to that Court.
The unavailability of a parent in a life-threatening circumstance should not preclude therapeutic action. Just as
informed consent is imputed or attributed rational behavior to an unconscious accident victim who has a life-
threatening condition that requires immediate surgery, such rational behavior can be imputed to the absent
parent in the case of an acutely ill neonate. However, in such circumstances if time permits, detailed
documentation and consultation with the hospital administration is recommended.
Informed consent in neonatal/perinatal medicine is not an empty gesture to reduce liability, but rather an
opportunity for health care providers and parents or guardians to be partners in the decision-making process.
Informed consent documents are intended to support decision makers in their choices, rather than merely to
provide ratification of medical decisions already made (35). Informed consent documents need to be routinely
reviewed to determine that the reading level required to understand them is consistent with the educational,
linguistic, and cultural experiences of those being asked to read, understand, and sign or acknowledge the forms
(36).
The informed consent process can be extremely complex, with several legal gray areas. For example, are
maternal rights any more definitive in making critical decisions for a fetus or neonate than paternal rights?
Conflicts can arise even when the putative or alleged father is not the legal spouse of the mother. Emergency
hearings in front of local judges may be required to resolve conflicting opinions, especially when the decision of
one parent may predictably lead, to a medical degree of certainty (more likely than not), significant adverse
consequences to the infant. In January 2014, a Texas Court ordered a local hospital to accept that a pregnant
mother was brain-dead and that the husband, not the hospital, could decide to end life support (37).
It is one of the ironies of the law, in most states, that an unwed teenage mother has the ultimate legal
responsibility for the care of her child, unless the court is petitioned to appoint an alternative guardian. In many
states, the live birth of a child, regardless of the age of the mother, results in the mother being declared an
P.101
emancipated minor. In contrast, a nonpregnant teenager, living at home and attending high school or college,
under the age of majority (usually age 18) may not have the legal right to make decisions about many aspects of
her own medical care.
The disclosure of risks in the informed consent process tends to underscore a parent's sense of helplessness
and to portray the physician as somewhat helpless as well. The powerlessness of the parents and their wish that
the physician be omnipotent creates unrealistic expectations for the outcomes of procedures and treatment.
Gutheil and associates (38) suggest that the physician acknowledge the parent's wish for certainty and
substitute the mystical with a physician-parent alliance in which uncertainty is accepted as an element in medical
care.

Advanced Care Planning


Decision making in life and death situations is not easy. Advanced care planning efforts initially evolved in the
care of the elderly (39). Advanced care planning, or contingency medical care planning, is no longer generally
reserved for adults. The American Academy of Pediatrics Committee of Bioethics developed a policy statement
about parental permission and informed consent (40). A distinction is made in this public policy statement and in
case law in the United States between emergency medical treatment, life support efforts, and elective surgical
procedures, such as circumcision or removing a kidney from one child to aid a sibling (41,42). Neonates need
others to make decisions about their treatment and viability. Their treatment in a neonatal intensive care unit
(NICU) is rarely a series of leisurely scheduled elective procedures. There must be a designated person with
whom the health care providers regularly communicate and who is held to provide consensus about treatment.
When potential conflicts in decision making are recognized, a conference among the concerned individuals, with
representation from the hospital administration, may be a worthwhile endeavor to clarify who actually has the
final decision-making authority. There may be times in the treatment of a patient when one person has to render
an immediate decision, even if that decision is not by consensus. Decision-making issues are often confounded
when the mother of the neonate is an adolescent herself and not married to the father.
While all 50 states in the United States and the District of Columbia have passed legislation on advance
directives, reinforcing the fact that adherence to such directives is mandatory rather than optional continues to
be problematic (39). The majority of states place restrictions on proxy decision making. If identification of the
responsible party for decision making is not clear or keeps shifting, legal consultation, initiated by the health care
providers, is recommended. Most jurisdictions have the ability to hold emergency judicial proceedings when an
impasse has been reached and a medical decision must be made before irreversible damage or death occurs
(34).

Accreditation of Health Care Activities


The Joint Commission (TJC), formerly known as the Joint Commission on Accreditation of Healthcare
Organizations (JCAHO) and prior to that known as the Joint Commission on Accreditation of Hospitals (JCAH), is
a nonprofit organization that accredits more than 20,000 health care organizations and programs in the United
States (4). The TJC does not hold a complete monopoly on hospital and health care assessments. In September
2008, the CMS granted reviewing authority for hospitals to Det Norske Veritas Healthcare Inc. (DNVHC), a
Norwegian international company. In addition, there are other health care accreditation organizations in the
United States that are unrelated to both TJC and DNV.
The stated mission of TJC is To continuously improve health care for the public, in collaboration with other
stakeholders, by evaluating health care organizations and inspiring them to excel in providing safe and effective
care of the highest quality and value (4). Accreditation standards and patient safety goals, along with the results
of the hospital reviews, are posted on the TJC Web site so that their assessments are transparent to all
stakeholders. Stakeholders range from institutions, to practitioners, to patients, and their guardians.
The purpose of The Joint Commission's National Patient Safety Goals (NPSG) is to promote specific
improvements in patient safety. Problematic areas in health care are identified, and expertbased approaches to
solutions are presented. The goals highlight problematic areas in health care and describe evidence and
expertbased solutions to these problems on a system-wide basis.
While medical tourism is less of an issue in neonatology than in some other specialties, international health care
accreditation has grown in importance when tertiary centers with unique skills and equipment may be in a
neighboring or even a distant nation. The Joint Commission International (JCI), a component of the TJC,
currently accredits hospitals in Asia, Europe, the Middle East, and South America. Australia, Canada, India, and
the United Kingdom have their own accreditation organizations (5).

Medical Records
The Federal statute known as HIPAA (6) and individual state regulations recognize that medical records are legal
documents. Medical and hospital records are designed to be a contemporaneous record of the available clinical
information and medical and other decisions that flow from the clinical information and interactions with the
patient/patient's significant others. Records provide an opportunity for adequate documentation. Documentation
is the key to management of patients, and it is the key to protecting physicians against malpractice litigation,
especially in the instances in which patients have a complex clinical presentation and course. The course of
treatment and whether or not therapeutic goals are met should be noted in the hospital chart. Treatment options,
including the option of no treatment when pertinent, should be explained to the patient's family and, if necessary,
others potentially involved in the decision-making process; these interactions should be documented in the
patient's chart. The degree of the patient's family's understanding, or lack thereof, of the various treatment
options also should be noted, especially if there are divergent views among family members. Ultimately, one
family member or guardian has to be acknowledged by the family and health care providers as the decision
maker. Identifying such an individual in the medical record will facilitate treatment decisions and posthospital
treatment care and management. Such family consensus meetings with health care providers, and the resultant
documentation, may preclude the need for judicial intervention.
Adequate medical records will document that the risks of a given procedure and the risks of the failure to perform
the given procedure have been shared with the patient's decision makers. A record that indicates that specific
known adverse side effects, or rare but serious untoward events, were discussed with a patient's family members
or guardians helps protect the clinician should one of these untoward events actually occur. No medical
procedure is risk free, and although families may be informed of the relative risks of the various procedures and
pharmacologic interventions, the stress of the moment may shorten their attention span, concentration, and
recall. For example, prescriptions at the time of discharge are often for a limited period of time, with follow-up
care being provided in either a hospital outpatient clinic or private clinical environment. Prescriptions must be
written clearly, identifying the patient, date, dose, dose schedule, and route of administration; if electronic
prescriptions are used, a patient-provided paper copy will be useful in explaining the need for each specific
medication. The parent or guardian to whom the prescription is provided needs to understand why the
medication is being prescribed, adverse side effects, therapeutic effects of the medication, and consequences to
the infant if the medication is not provided. Medical record documentation, including discharge instructions and
prescriptions, at least provides a contemporaneous record of what information actually was
P.102
provided. Written copies of instructions for medical management and postdischarge follow-up, with points of
contact, will minimize but never eliminate a family's failure to follow through.
Medical records provide the basis for reimbursement of the costs for patient care and treatment. A description of
the severity of the condition, justification for laboratory and other investigations, need for consultations, and
manner in which the consultative advice is incorporated into patient care should be present within a patient's
medical record. In the spring of 1998, the U.S. Justice Department announced the hiring of 250 Federal Bureau
of Investigation (FBI) agents for the purpose of investigating Medicare and Medicaid fraud; 15 years later,
Medicare and Medicaid fraud continue to cost the health care system multiple millions of dollars. The FBI has
estimated that 10% of the money paid out for health care services under the Medicaid program is the result of
fraudulent billing (43). Not all the money paid for fraudulent services is recovered; in 2002, $1.6 billion was
collected in connection with health care fraud cases (44), but Medicaid (the U.S. insurance program primarily,
but not exclusively, for children) and Medicare (the U.S. insurance program for the elderly and the disabled of all
ages) are plagued by a reported $60 billion in fraud annually, the Justice Department estimates. While Medicaid
is run by states, more than half of the costs are paid by the federal government, which spent about $270 billion in
2011. Anti-fraud efforts involving Medicaid were left to states until 2005, when a federal law aimed at reducing
the deficit created the Medicaid Integrity Group (45).
The absence of consistent, comprehensive reimbursement policies is frequently cited as one of the most serious
obstacles to total integration of telemedicine into health care practice. This lack of an overall telemedicine
reimbursement policy reflects the multiplicity of payment sources and policies within the current U.S. health care
system. The United States Affordable Care Act of 2010 does not address reimbursement issues.
Adequate health care documentation facilitates medical audits, permits those who prepare invoices for
reimbursement or payment to justify the categories or International Classification of Diseases codes placed on
the universal billing forms (sometimes identified as CMS-1500 forms and formerly known as HCFA-1500 forms),
and prevents errors that may result in the appearance of fraud (46).

Medical Confidentiality of Oral and Handwritten Communications


The Hippocratic oath states, in part, Whatever, in connection with my professional practice or not in connection
with it, I see or hear, in the life of men, which ought not to be spoken of abroad, I will not divulge, as reckoning
that all should be kept secret (47). This oath, taken by many physicians upon graduating from medical school,
has been codified by state and federal law. The patient information obtained by the physician is considered to be
privileged, that is, only specified individuals for specific purposes shall be allowed to review the medical
records in question (48). The privilege, however, belongs to the patient or his or her guardian; it does not belong
to the physician or other health care provider. A court may order a health care provider to breach medical
confidentiality; legislation and state health regulations may require it, especially as it relates to infectious
diseases and (child) abuse and neglect.
As the delivery of medical care becomes more complex, individuals not generally thought of as direct health care
providers are requiring access to confidential medical information. The legitimate needs, for example, of physical
therapists, occupational therapists, hospital administrative personnel, and medical insurance personnel may
appear to conflict with the principle that medical information should be restricted only to direct health care
providers such as physicians. The ethical basis for confidentiality derives from the concept that assurance of
confidentiality encourages patients to seek the medical care that they require and to be candid with their health
care providers. The legal basis for confidentiality is derived from statutes, which have been enacted in
essentially all states within the United States.
There is significant variation among the states as to what classes of health care providers (doctors, nurses,
social workers, etc.) may assert that they cannot share medical information provided to them without the patient's
express permission or a court order directing them to do so.
Legislatures create state disease-reporting requirements that require medical confidentiality to be breached
because, historically, the courts have found that the state has a compelling need to protect its citizens from
certain infectious diseases (49). Certain behaviors, such as dangerousness, also have resulted in reporting
requirements that compel a health care professional to notify authorities (50,51). Dangerous behaviors include
spousal and child abuse.
Medical records also are protected by state and federal legislation. The U.S. Public Health Service Act provides
for the explicit protection of medical records dealing with treatment for drug (52) and alcohol abuse (53).
However, even the contents of substance abuse treatment records may be released to medical personnel to the
extent necessary to meet a bona fide medical emergency (54,55). If release of the medical records is authorized
by a court order, then appropriate safeguards need to be put into place to preclude unauthorized disclosure.
The courts recognize that they must balance the public interest and the need for disclosure against injury to the
patient, physician-patient relationship, and treatment services (56,57). A hospital medical record is the property
of the hospital, but it is kept for the benefit of the patient, the physician, and the hospital (47). A patient's medical
record is preserved to document events in a contemporaneous manner for later use. Later use includes further
medical care and treatment, documentation for financial payment by third-party payers, and defense of medical
malpractice claims. While the medical record may be the property of the hospital, the patient or his or her
guardian does have the ability to access the record and obtain a copy of the record.
The health care facility medical record librarian becomes the custodian of the medical records. The American
Association of Medical Record Librarians has a code that is similar to the Hippocratic Oath taken by physicians.
Hospital policies and procedures, consistent with state and federal statutes and regulations, prevent a medical
record from being released without either a patient release or a court order. Under certain defined
circumstances, medical records can be admitted as evidence in a court of law. They can be authenticated as
business records. They also can be used to refresh a doctor's memory and document his or her actions during a
deposition (the taking of a sworn statement) or during trial testimony (taken under oath).
The American Hospital Association, TJC, De Norske Veritas, and other national and international health care
professional organizations have asserted that a patient's medical records are to be protected from unauthorized
and unnecessary access. Their positions are generally consistent with current HIPAA and state regulations.
Hematology, blood chemistry, urinalyses, and radiographic, sonographic, and electrodiagnostic findings are all
considered to be encompassed within a patient's authorization or court order for release of medical information.
Third-party payers, as a condition of insurance, demand and almost invariably are given access to patient
medical records, as are state and federal auditors.
The privilege of medical confidentiality does not extend to third parties present who are not part of the health
care being delivered. That is, police officers present during an evaluation or treatment cannot be prevented from
sharing with other law enforcement personnel or the courts any information that they obtained under such
circumstances. When an individual engages in litigation in which his or her physical or mental status is an issue,
that individual cannot assert the privilege to prevent unfavorable information from
P.103
reaching the ear of the court. Thus, in a lawsuit for malpractice, the patient/plaintiff has waived his or her right to
medical confidentiality of any oral, written, or electronic communication concerning his or her medical history,
diagnosis, treatment, or prognosis. Medical confidentiality statutes were and are designed to protect a patient's
privacy and to encourage treatment for conditions that may bear social or other stigmas. The laws were not
made to give the moving party, the patient/plaintiff in a medical malpractice case, an unfair advantage by
allowing the individual to select only those records considered testimony favorable to his or her case. A medical
record cannot be used as both a sword and a shield.
In 1991, the Institute of Medicine (IOM) advocated the adoption of the computer-based patient record as
standard medical practice in the United States (58); two decades later, computer-based patient records that are
accessible to multiple providers, at different unaffiliated facilities, is the exception rather than the general rule.
The United States federal government has provided funds to facilitate hospitals and practitioners developing and
utilizing electronic records; the failure to use such a system may result in a loss of revenue for Medicare and
Medicaid providers. A computer-based record is perceived as a continuous chronologic history of a patient's
medical care. The medical care record can be linked to various aids, including reminders and alerts to clinicians
and clinical decision-making instruments. A computer-based record increases access to a patient's record and
increases the array of data maintained in a single record (59). However, the hacking of a large computer base
can result in huge quantities of personal health information being exposed. The more information compressed
into an easily accessible single location, the greater the precautions needed to prevent misuse. Annas, 25 years
ago, wrote that in a setting of private practice, medical information that identifies a patient is supposed to be
transferred from physician to physician only with the patient's written informed consent (60). In contrast, he
explains, within a medical institution, information is generally passed around on a perceived, usually self-
designated, need to know basis without first obtaining a patient's informed consent. Individuals who receive
their medical care through managed care facilities and integrated health care delivery systems that have multiple
treatment sites and use computer-based patient records can anticipate that the traditional standards of medical
confidentiality will be diminished. Even a minor error, such as dialing an incorrect fax number and sending an
electronic report to an unintended recipient, can result in damage to the patient and potentially costs assessed to
the individual and/or organization that authorized the report to be transmitted in error.
A release of medical information request can be general or rather specific, depending on the clinical and social
circumstances, needs of the treating health care providers, and instructions of the patient or guardian. In general,
medical information is released upon written instruction; however, oral instructions frequently are sufficient and
necessary in a medical emergency. Documentation of oral permission for release of medical information is
recommended.

Health Insurance Portability and Accountability Act (HIPAA) of 1996


HIPAA (6) is concerned, in general, about the written word and not oral communications, especially those
provided in an ongoing manner in the course of clinical treatment. HIPAA set national standards for the protection
of health information, as applied to the three types of covered entities: (a) health plansan individual or group
plan that provides or pays the cost of medical care that includes the diagnosis, cure, mitigation, treatment, or
prevention of disease; health plans include private entities and government organizations dealing with health-
related issues such as Medicaid, Medicare, Department of Defense, Department of Health and Human Services,
and the Department of Veterans Affairs; (b) health care clearinghousesa public or private entity, including a
billing service or community health information system that processes medical, financial, or other data; and (c)
health care providers who conduct certain health care business transactions electronically. Health care providers
such as physicians, hospitals, and clinics are covered entities if they transmit health information in electronic
form in connection with a transaction for which a HIPAA standard has been adopted by the DHHS. The
Affordable Care Act, and its amendments, does not alter the substance of HIPAA.
The HIPAA compliance date for health care providers and health care entities has passed; for larger entities, it
was April 14, 2003, and for smaller entities, it was April 14, 2004. If HIPAA is more stringent than a state's law, it
governs; if the state law is more stringent than HIPAA, the state law governs. Generally, covered entities are
required to comply with both HIPAA and state law whenever possible. HIPAA preempts any contrary provision of
state law, including state law provisions that require written records rather than electronic ones. State law,
however, is not preempted in the following circumstances: regulation of insurance or health plans; prevention of
fraud and abuse; reporting on health care system operations and costs; regulations concerning controlled
substances; when a state law requires the reporting of disease or injury; child abuse, birth, or death; public
health surveillance, investigation, or intervention; or when a provision of state law is more stringent than the
requirements of HIPAA.
As with every issue of conflict between state and federal laws, there are exceptions. Exceptions mean health
care lawyers need to be consulted when a clinician is unsure as to his or her course of action with regard to
HIPAA and state confidentiality laws. There are other federal laws that may be relevant to parental conduct, such
as the federal Drug and Alcohol Confidentiality Regulations (52) that govern the release of medical records that
contain information about drug and alcohol use, and the terrorism provisions of HIPAA that allow access to
medical records if matters of national security may be involved.
HIPAA was designed to protect medical records and their confidentiality. Other laws have existed to do this. The
U.S. Congress believed, in consultation with many patient advocates, health care providers, and other
commentators, that more than 1,000 additional pages of laws and regulations were required. If HIPAA serves to
demonstrate two basic principles that health care professionals have long recognized, it is that first, medicine is
no longer a one-on-one relationship, and second, medicine has evolved with all its disciplines, providers, and
equipment to be a major economic force and component of every developed or developing nation, whether
agrarian based or industrial based or a combination thereof.
HIPAA has forced health care professionals to better communicate with patients and their relatives. Ironically, the
destruction of the World Trade Center Towers, in New York City, on September 11, 2001, and ongoing terrorist
activities, has resulted in a demand for better communication by health care professionals with potential patients,
the general public, politicians, and the media. Risk communication has been transformed from an arcane
technique used by military and emergency preparedness personnel to a staple of daily communication. The
basic principles of risk communication are simple and totally applicable to the practice of medicine, especially
neonatology: Tell what you knowclearly, succinctly, and in a manner that every sixth grader can understand.
Tell what you do not know clearly, succinctly, and in a manner that every sixth grader can understand. Do not
guessinformation provided has to be data driven. It is better to say we do not know now, and when we do, we
will share it with you, rather than to provide incorrect information because incorrect information causes credibility
to suffer, believability to suffer, cooperation to suffer, and ultimately there is more confusion, anxiety, and poorer
outcomes. Poorer outcomes refer to poorer clinical outcomes and increased possibility of litigation because of
expectations not met, whether realistic or not.
HIPAA delegates to local health departments the task of collecting vital statistics that include birth, death, and
marriage information. These documents generally are protected to some degree. Autopsy reports usually are not
released beyond the treating
P.104
physician and/or coroner/medical examiner, unless there is authorization to do so. Civil or criminal courts can
order the contents of an autopsy revealed if it would assist a party in asserting his or her civil claim, criminal
prosecution, or defense from prosecution.

Legal Issues Specific to Managed Care


Although the incidence of malpractice litigation against health professionals has increased exponentially in the
United States since the 1970s, it is not a recent phenomenon. Documented medical professional liability cases
date to 1374, when a surgeon in England was sued for negligent treatment of a wound (61). However, a hybrid
of lawsuits against health care providers, focusing on managed care issues, have evolved since the beginning of
the 21st century (62,63). Profit is the bottom line in today's health care system; not-for-profit or nonprofit
hospitals use their surplus to contribute to increased salaries and capital expenditures. Managed care
developed in response to a perception on the part of both government and society in general that there were
unnecessary medical procedures being performed with an associated overutilization of medical resources and
services and an unrestrained lack of uniformity of cost in health care. Managed care filled a vacuum; it was
developed and marketed because of a perceived need to control by those who paid costs (the employers) and
those who managed health care dollars (health care insurance companies). Health care providers did not fully
understand and thus did little to inform their constituencies of the potential problems associated with managed
care.
Managed care may be defined as any entity capable of negotiating to deliver health care to a group of recipients
at a predetermined rate on a per capita basis (64). The American Association of Health Plans claims that 150
million Americans were enrolled in health maintenance organizations (HMOs) or other managed care entities in
1995 (65). Prior to the Affordable Care Act, almost 75% of those who received health insurance through their
employers were covered by some type of managed care plan (66). The Affordable Care Act of 2010 (2) is
designed to increase the numbers of individuals covered by managed care plans in 2014, by allowing those not
enrolled in a health care plan to afford to do so, with government subsidies as needed. Under the Act there are
tax penalties for failing to enroll in a health care plan.
Managed care has become a major corporate entity with the administrative costs and profit reportedly deriving in
major part from reported cost savings and efficiencies and lowered reimbursement rates. The focus of the
decision maker to authorize or perform a service or procedure or order a medication now has an aspect of a real
or perceived profit motive: There has been considerable media coverage of managed care programs that firmly
control costs to the detriment of those paying premiums and expecting the best services available. In many
cases, unqualified personnel are placed in a position of second-guessing and overruling physician decision-
making responsibilities and obligations. Delays in receiving permission for expensive procedures or outright
denials for critical but costly procedures occur. In some instances, so-called gag orders had been inserted in
physician contracts, which effectively prohibited physicians from disclosing expensive, and potentially more
effective, alternative treatments to their patients (64). However, in February 1997, then President Clinton
requested that the DHHS send a letter to all state Medicaid directors informing them that gag rules are
prohibited for Medicaid HMOs. He also supported national legislation to ban gag rules for all managed care
organizations within the United States. Approximately half the states in the United States have enacted or
introduced their own laws regulating gag rule provisions in medical care policies. In Moore v Regents of the
University of California, the California Supreme Court held that the concept of informed consent is broad enough
to include the physicians' duty to inform their patients that they have an economic interest, which might affect that
physician's professional judgment (67).
Although managed care attempts to reduce costs by creating economies of scale and coordinating care among
health care providers and resources, they also attempt to reduce costs by eliminating unnecessary care, as they
define it. In some instances, a managed care program's criteria for emergency or extensive and expensive care
may conflict with those held by the physician. The physician's contract may have a provision in which a
percentage of fees are paid only if utilization goals are met. The physician's contract also may have a capitation
provision, which means that the physician is provided a fixed amount regardless of the level of service provided
to each patient. In the latter circumstances, the physicians essentially become coinsurers and may have a
perceived conflict of interest in the manner and degree to which they provide services to their patients.
The most unique feature of an HMO is that an enrolled patient pays a prepaid, fixed fee for medical services.
This is in contrast to fee for services, in which the patient pays a separate fee for each service rendered by the
independent physician. Preferred provider organizations (PPOs) differ from HMOs in that a PPO is an organized
group of health care providers offering their services at a discount. Services are provided on a predetermined
fee-for-service basis. Members may choose physicians regardless of whether they are in the members' plan but
receive discounts only from physicians within the plan. PPO physicians have relatively minimal financial
incentive to limit services, and physicians practicing under such an arrangement would appear to have greater
control of their practice and potential liability.
The HMO may contract directly with physicians and pay them a salary (staff model), or they may contract with a
group of physicians. The physician group may or may not devote a majority of its time to serving the needs of a
specific HMO. It may offer a mixture of financial arrangements to its patients, including fee for service. The HMO
may contract with an individual practice association (IPA), usually a partnership or corporation of physicians, to
provide health care services to the HMO membership. The IPA then contracts with its physicians to provide
services to the HMO. The fundamental difference between the IPA and the staff and group physician
arrangements is that IPA physicians usually work in their own facilities, use their own equipment, and keep their
own records. The HMO pays the IPA a capitation fee (a specific amount per enrollee or subscriber); the IPA pays
the treating physicians on a fee-for-services basis (68).
Physicians and their managed care carriers are being sued jointly. Allegations of malpractice now are joined with
allegations of bad faith, and contract theories of liability are being added to the more traditional
negligence/medical malpractice theories of liability (62,63,69). Under the doctrine of respondeat superior, HMOs
have been found responsible for the alleged negligence of their employeephysicians (70). The doctrine of
respondeat superior states that an employer is vicariously liable for negligence of an employee acting within the
scope of his or her employment (71). The doctrine does not apply if the negligent party is a true independent
contractor (72). The distinction between an employee and an independent contractor is control or independence;
that is, an employee is subject to the immediate direction and control of the employer, and independent
contractors use their own judgment and are not subject to direct control. In some instances, an HMO can be held
vicariously liable for the negligence of a consultant requested by the HMO's physician (73). This issue may be
decided on the wording in the promotional material provided to subscribers. A managed care contract can
increase a physician's obligations to a patient; the contract cannot decrease or even limit the physician's
obligations and legal liability to the patient. When a physician employed by an HMO makes a treatment decision,
that treatment decision has financial consequences to the HMO (62). In a malpractice suit, there are now
assertions that the HMO-paid physician's treatment decisions were economically motivated rather than being
made in the
P.105
best interests of the patient (62). Before a physician accepts a contractual obligation, whether it is the product of
an employment contract with a hospital, group practice, HMO, or other form of managed or prepaid care
program, it is strongly suggested that they seek legal advice regarding the terms of the contract and the
consequences of those terms.
Regardless of the employment status of the health care provider, the hospital has an obligation to oversee the
quality of patient care and services (74). Ultimately, however, the courts have held that it is the physician's
responsibility to uphold good medical practice in the face of improper or incorrect cost-containment procedures
put forth by HMOs or other managed care organizations. The California Court of Appeals has held that, while
we recognize, realistically, that cost consciousness has become a permanent feature of the health care system, it
is essential that cost limitation programs are not permitted to corrupt medical judgment (75). That court found for
the plaintiff because the physician did not protest the health insurer's determination that a prolonged
hospitalization for his patient was not necessary, with severe negative consequences for the patient (75). In
2013, when designating providers and provider medical centers, under the Affordable Care Act, some insurance
providers, in Washington State, restricted access to many of the highest ranked hospitals, including specialized
pediatric medical centers (76). Thus, many insurance companies have selectively entered the medical exchange
networks established under the Affordable Care Act because they are concerned that the patient pool signing up
for services will include a large percentage of risky, high-utilization patients who previously wanted but could not
afford insurance. These populations clearly include women who, when they become pregnant, might be
considered high risk and require more intensive care and treatment during pregnancy, at the time of delivery, and
also postpartum neonatal care for their offspring.
The Employee Retirement Income Security Act (ERISA) of 1974 (77) supersedes any and all state laws insofar
as they may now or hereafter relate to any employee benefit (78). In Shea v Esensten, a federal court found that
ERISA requires HMOs to disclose to their enrollees the compensation agreement between the HMO and its
physicians (79). That is, there is an affirmative duty by the HMOs to inform their subscribers of any financial
incentives that the health care providers may receive as they manage their patients' care. These financial
incentives must be disclosed and the failure to do so is a breach of ERISA's fiduciary duties (79). Physicians
who have challenged managed care decisions about patient care have been removed from the HMO panels.
Suits for reinstatement on the grounds that they were removed from the panel without good cause and in
violation of public policy and the implied covenant of good faith and fair dealing traditionally read into contracts
have had mixed results (80,81). Just as the practice of medicine, and in particular neonatology, has evolved
significantly during the past several decades, so too has the related health law. Both will continue to change, and
their progress, conflicts, and resolutions will be documented in the media, professional journals, legislature, and
courts.

TABLE 9.1 Liability Risk and Risk Reduction for Neonatologists Working with Nonphysician
Providers

Areas of Risk Risk Reduction

Inadequate Check NPP credentials before hiring and maintain documentation


supervision by Establish, and review at least annually, written policies, protocols, and
the physician procedures for:
NPPs working patient examination
beyond their treatment
scope delegation
Parent unhappy supervision
about access to the patient's/parent's right of access to the physician
their newborn's Instruct NPP to adhere as closely as possible to patient care protocols,
physician seeking physician input when significant deviation may be required; the
Physician rationale for deviations should be documented
viewed as deep Document completion of skills inventory check
pocket by Document current competency and establish a quality monitoring system
plaintiff's bar Educate other staff about the role and limits of the NPP
Apparent Understand and keep current regarding statutory requirements for NPPs
physician delay Use name tags to identify the professional status of the NPP
in seeing a Introduce NPP to parents and explain role
critical patient Review NPP's charts on a regular basis and cosign orders in a timely fashion
Establish protocol for the physician to see the patient and parents at set
intervals
Obtain adequate liability insurance for NPP; physician's liability insurer should
be informed that they are supervising NPPs
Ensure that NPP complies with hospital credentialing requirements and has
sufficient time allotted for continuing professional education; require that
copies of licensure renewal and certification of continuing education are kept
on file
Inform physicians covering call of NPP's role

Physician Liability Arising from Nonphysician Providers


Legal liability exposure arising from the apparent implicit or explicit supervision of nonphysician providers (NPPs)
(e.g., neonatal nurse practitioner, clinical nurse specialist, physician assistant) is relatively new. It can be
assumed, however, that as NPPs increase their roles, there will be a concomitant increase in malpractice
allegations against them and the physicians working with and supervising them, based upon the fact that the
NPP is acting as an agent for the physician.
To the extent that NPPs exercise control over their professional activities, they will be independently held
responsible for their negligent acts; but, physicians and medical centers have deeper financial pockets. Under
the old captain of the ship (respondeat superior) doctrine, the physician was presumed to be responsible for
the activities of all the NPPs working with them. Contemporary courts have moved away from this theory and
have placed responsibility on those professionals exercising direct supervision over their employees and their
employers. However, this does not eliminate the liability risk for the supervising physician. The supervising
physician does not have to be on site when the adverse event in question occurs to be named in litigation.
Potential areas of risk for neonatologists working with NPPs and methods for reducing risk are listed in Table
9.1.
P.106
The National Practitioner Data Bank
Some malpractice insurance carriers retain the right to settle lawsuits independent of the wishes or sentiments of
the health care provider. The federally mandated and operated National Practitioner Data Bank contains
information, available to hospitals and individuals, and lists settled medical malpractice claims (82). General
medical malpractice carriers may not always be knowledgeable regarding the specific nuances of specialty
practice.
Health professionals should do more than comparison shop the price of a malpractice policy. They need to
determine the financial soundness of the malpractice carrier, their own rights to control settlement, and to
determine the sophistication and knowledge of the legal representation provided by their insurance carrier.
Three critical questions to be asked are: (a) Do I have the right to not settle a case in favor of going to trial? (b)
May I chose the attorney to represent me or is that decision made by the malpractice insurance carrier? (c)
Will my malpractice carrier force me to accept their choice of attorney or if I select my own attorney, will I then be
responsible for the legal fees?

RISK MANAGEMENT AND QUALITY IMPROVEMENT


The Growth of Institutional Oversight of Health Care
In the United States, risk management and quality improvement programs grew out of changes in the medical
malpractice insurance market (insurance became more difficult and more expensive to obtain). As the cost of
liability insurance increased, the establishment of risk management programs made health care facilities more
attractive to insurance underwriters. When institutions formed their own self-insurance trust funds, risk
management programs provided some assurances to hospital board members that the financial liability of the
institution would be limited to the greatest extent possible by their own risk managers. Depending upon the size
and complexity of the health care institution involved, there may be one set of professionals who provide risk
management/quality improvement guidance relative to standards of care and a second set of professionals,
under the leadership of a compliance officer, who assure compliance with the requirements of regulatory bodies.
Ensuring statutory and regulatory compliance requires a thorough knowledge of applicable standards and a
commitment of time specifically directed toward understanding compliance and related issues. In smaller
institutions, the compliance officer and risk manager may be the same individual.
Risk is the possibility of harm, the uncertainty of danger, and the probability of loss. The essence of a Risk
Management or Quality Improvement program is that they are attempting to maximize the areas in which there is
some control over the outcome while minimizing the areas in which there is absolutely no control over the
outcome. The existence of risk does not imply that there are always alternatives.
The terms Risk Management and Quality Improvement are used to describe a process of examining any potential
alternatives and thereby influencing, in a positive manner, the level of risk to which patients, health care
providers, and institutions are exposed. An outcome of managing risk is the identification of a level of harm or
danger that is the irreducible minimum. However, Risk Management and Quality Management differ in the order
of priority of their primary goals. In the health care setting, harm or danger expressed in human terms pertains to
the safety of patients, visitors, and employees, and to health care delivery practices; however, harm or loss can
also be expressed in financial terms, such as losses in settlements, jury verdicts, legal expenses, and payment of
increased insurance premiums. Quality Improvement is the term used for the process in which the primary
expected reduction is expressed in human terms. Risk Management is the term used when the primary expected
reduction in risk is expressed in financial terms. Risk reduction is achieved using a process that involves
investigating, evaluating, planning, organizing, and implementing procedures. The resulting benefit of risk
reduction is improved quality and understanding of patient care and also preservation of the financial resources
necessary to provide optimal patient care when there are fewer claims made and fewer lawsuits instituted.
Financial and psychological benefits also are realized when malpractice cases are dismissed or settled for
reduced amounts, based on the strength of the defense. The following discussion will concentrate on the
definition of Risk Management v. Quality Improvement; a detailed discussion of the mechanism of a Quality
Improvement program in the NICU is provided in Chapter 3.
The level of risk exposure in the health care environment is inversely proportional to the control maintained by
the health care service providers. Total control is not possible in the face of complex disease processes and
complex care and treatment managed in complex patient care delivery systems. In the critical care environment,
a risk reduction program must be established that anticipates, identifies, and responds to risk. When it is possible
in the process of delivering care to patients, fail-safe systems are established to prevent errors from affecting the
patient.
Risk Management and Quality Improvement are systems approaches that are protocol and data driven to
identify, evaluate, and address problems involving patient care and treatment. These approaches go beyond
direct patient care and may address plant (hospital/medical center) infrastructure operations, general and risk
management communications, and staff/employee relations. Peer Review for Quality Improvement is designed to
improve efficiency and effectiveness of health care interventions. Risk Management and Quality Management
are ongoing processes; a form of preventive medicine that constantly and critically examines operational and
clinical activities. Risk Management and Quality Improvement examine medical support operations, including
building maintenance and operations, as well as the actual services provided by the health care providers and
their ancillary and support staff. Risk Management and Quality Improvement, while patient centric, focus on the
effect the physical plant may have on patient care and family attitudes, values, and beliefs. Every employee,
whether a critical care specialist, a licensed health care professional, or a member of the janitorial service,
represents the institution when they interact, directly or indirectly, with patients and friends and family. A wound
infection, an unmarked wet floor, and a defective chair can all contribute to actual and perceived poor patient
and visitor care and services.
Health care errors rarely are due to a single mistake made by an individual; they are usually an indication of
failure of a health care system. An effective Quality Improvement team works in close cooperation with the Risk
Management team; both work closely with the hospital administration. The ultimate goal is improved care and
safety of patients and lowered liability exposure.
There are two fundamental types of reviews: Quality Improvement reviews are confidential and protected by
federal statute (83). Risk Management reviews are nonprotected and may be used for administrative and other
nonquality assurance purposes. A nonprotected review can be subpoenaed as part of ongoing litigation,
including discovery prior to trial.
Protected reviews must be so identified in advance. Records and documents must be designated in writing as
being for Quality Improvement/Resource Utilization, prior to the commencement of the review. Quality
Improvement work products cannot then be used for administrative decisions such as credentialing or privileging.
Protected reviews or their productmedical quality assurance recordsare considered to be confidential,
meaning nondiscoverable in litigation, if the records and documents produced as part of this process are used to
improve quality of care or alter utilization of health care resources in the health care facilities. The essential
elements of Peer Review for Quality Improvement include (a) concern about a selected episode of care; (b)
examination of the
P.107
background, specific actions, and consequences involving the episode of care; (c) open privileged
communication with the providers and others involved in the selected episode of care provision; (d) confidential
communications, including findings and recommendations, to the providers being reviewed; and (d) identification
of systems and process issues that may require further investigation/administration action, possibly by the
Quality Improvement Team. A peer is defined as a health care professional with comparable education, training,
experience, licensure, clinical privileges, and scope of practice. A peer reviewer should be able to provide a fair
and credible assessment of the actions taken by the provider in the specific episode of care under review. Thus,
the peer reviewer must (a) have the necessary clinical expertise to make accurate judgments about the decisions
in the episode of clinical care under review, (b) make a fair and credible assessments of the actions taken by
those involved in the episode under scrutiny, (c) have the knowledge of current relevant evidence based on
standards of care relevant to the case, and (d) have adequate training regarding the peer review process and
responsibilities, including associated legal and ethical requirements entailed in the peer review process.
There are various aspects or criteria for a review of care, not all may be relevant in any given circumstance: (a)
choice of diagnostic tests and the timely ordering of diagnostic tests, (b) performance of a procedure or
treatment, (c) addressing the results of abnormal diagnostic tests, (d) timeliness of diagnosis, (e)
appropriateness of diagnosis, (f) timeliness of treatment, (g) appropriateness of treatment, (h) skill level during
procedures, (i) recognition of and communication of critical clues to patient's condition during a period of clinical
deterioration to other health care staff and family members/guardians, (j) health record documentation, (k)
adequacy of supervision of health profession trainees and supervisees, and (l) other relevant aspects of clinical
and nonclinical care.
It is recommended that the following actual or similar language be placed on Quality Assurance Documents:
The documents, records and other information contained herein, which resulted from (PLACE NAME OF
SPECIFIC QUALITY PROGRAM OR RESOURCE UTILIZATION ACTIVITY HERE) are confidential and
privileged under the provisions of 38 USC 5705, and its implementing regulations. This material cannot be
disclosed to anyone without authorization provided for by that law or its regulations. The statute provides for
fines up to $20,000 for unauthorized disclosures.
Management, including Risk Management, reviews are not considered a peer review for quality improvement.
These reviews are not protected as confidential and privileged by 38 USC 5705 (83). Nonconfidential reviews
can be disclosed for administrative and legal purposes. The findings from these reviews may be used in
administrative actions, for example, a change in privileges, decisions about the competencies of individual
providers. The findings must be kept administratively separate from protected reviews. Management reviews
include the following: focused Professional Practice Evaluations (FPPE), Administrative Investigative Boards
(AIB), Medical Advisory Opinions (MAO), Ongoing Professional Practice Evaluation (OPPE), and Peer
Recommendations for credentialing and privileging purposes. While a Peer Review for Quality Improvement and
a Management Review may be performed for the same event and either may occur before, concurrent, or
subsequent to the other, the protected and nonprotected information and processes must be kept separate.
When transitioning between these two different types of reviews, only the initial report or summary of the
occurrence can be shared when the other review commences.
Risk managers are attempting to improve the quality of care by minimizing adverse patient outcomes. Adverse
patient outcomes and even favorable patient outcomes may lead to malpractice claims and/or claims of
substandard or inappropriate care and/or excessive cost of care. Such assertions may result in emotional stress
and distress to the health care providers and have a financial impact on the institution and health care providers.
The rapid rise in health care costs in the United States during the past three decades has prompted a significant
increase in governmental scrutiny. The results of government audits have created a dramatic change in public
opinion regarding the level of waste, fraud, and abuse in the health care system. This has led to the passage of
legislation that creates a trust fund for investigations and expands governmental prosecutorial powers and also
has markedly increased penalties for noncompliance. The False Claims Act imposes liability on persons and
companies who defraud federal governmental programs (84). For instance, a claim for payment for medical
services to Medicaid or Medicare that is deemed to be fraudulent under the False Claims Act requires payment
of three times the amount of the overpayment, plus a mandatory $5,000 to $10,000 fine per claim. Also, since
each individual service billed for is a claim under the definitions used in the statute, penalties can become
enormous. On the other hand, erroneous claims (i.e., those resulting from innocent errors) require only the
return of the amount of the overpayment. Fraudulent claims result from three circumstances: (a) actual
knowledge that the claim is false, (b) reckless disregard of the truth or falsity of the claim, and (c) deliberate
ignorance of the truth or falsity of the information. To mitigate the potential and corresponding penalties for
noncompliance, voluntary compliance programs have become commonplace and necessary in the health care
world. The voluntary compliance programs recommended for health care providers are designed to detect and/or
prevent illegal activity through self-policing.
Providers meeting the requirements of an effective compliance program, listed below, demonstrate their
commitment to creating an environment in which payment claims are accurate; fraudulent behavior does not
occur; improper practices are prevented, detected, or rectified; wrongdoing is reduced; administrative liability is
mitigated; and the mental state of reckless disregard is negated. Although all compliance programs do not need
to be alike and the degree to which each element needs to be addressed varies among practice types,
compliance programs should address the following seven basic elements (85):

1. Establish written standards of conduct, policies, and procedures


2. Designate a compliance officer or contact
3. Provide mandatory training and education
4. Create and publish accessible lines of communication
5. Audit and monitor compliance with guidelines
6. Enforce through clear disciplinary guidelines
7. Respond to violations and take corrective action
The U.S. Department of Health and Human Services, Centers for Medicaid and Medicare, the Department of
Defense, and the Department of Veterans Affairs are the principal payers for medical care in the United States.
The Department of Justice (DOJ) seeks out health care fraud violators and prosecutes them. DOJ reports that
$2.6 billion in health care fraud recoveries occurred in fiscal year 2013. This was the fourth consecutive year that
recoveries exceeded $2 billion in cases involving health care fraud (86). DOJ has achieved success against
pharmaceutical firms for deceptive practices and as well as against those who violate the Federal Food, Drug
and Cosmetic Act or the Stark Act, and individual clinicians receiving kickbacks. Amgen paid the federal
government $762 million, including $598.5 million under the False Claims Act for its illegal promotion of Aranesp,
a drug used to treat anemia, in doses not approved by the FDA and for off-label use to treat non-anemia-related
conditions (86). Ranbaxy USA Inc. paid $505 million to settle allegations of false claims to federal and state
health care programs for adulterated drugs distributed from its facilities in India. The Civil Division of the
Department of Justice's Consumer Protection Branch obtained 16 criminal convictions and more than $1.3 billion
in criminal fines, forfeitures, and disgorgement under the Federal Food, Drug and Cosmetic Act (FDCA) (87). The
FDCA
P.108
protects the health and safety of the public by ensuring, among other things, that drugs intended for use in
humans are safe and effective for their intended uses and that the labeling of such drugs bears true, complete,
and accurate information.
The DOJ pursues claims under the Stark Act, a federal act that places limitations on certain physician referrals
for Medicare and Medicaid (relevant for neonatologists) patients if the physician or an immediate family member
has a financial interest in the medical facility (88). A referral is defined as a request by a physician for the item or
service for clinical laboratory services, physical therapy services, occupational therapy services, radiology,
including magnetic resonance imaging (MRI), computerized axial tomography (CAT) scans, ultrasound services,
radiation therapy services and supplies, durable medical equipment and supplies, parenteral and enteral
nutrients, equipment, and supplies; prosthetics, orthotics, and prosthetic devices, home health services and
supplies, outpatient prescription drugs; and inpatient and outpatient hospital services. In other words, what might
at first glance seem to be a series of irrelevant federal laws and their regulations cover every aspect of clinical
neonatology and the business of neonatology, even if the neonatologist or other health care provider is not in
private practice but is an employee of a health care entity. A significant example of the power embedded within
the Stark Act is the Department of Justice's recovering $26.3 million, in a settlement, from a dermatologist
practicing in Florida, to resolve allegations that he entered into an illegal kickback arrangement with a pathology
laboratory that resulted in increased claims to Medicare (86).
Although neonatal practitioners are not expected to be experts in law or regulation, they need to have a good
working knowledge of relevant legal and regulatory requirements that relate directly to their duties and
responsibilities. Regulations often are complex, ambiguous, and sometimes silent on key issues. Whenever a
doubt about a legal or administrative issue is raised, it is important for the practice or practitioner to consult with
an expert before proceeding.
FIGURE 9.2 The risk management investigation process begins with the receipt of notice that an
adverse event has occurred. An attorney directs some investigations in jurisdictions where the risk
management activities are not protected from discovery in litigation. The personal notes of health care providers
are not protected from discovery. After many rounds of questions, opportunities for improvement are reported to
quality improvement councils. In a separate activity, the results of investigation directed by the defense attorney
are sent to him or her.

Staff Education
Personnel involved in Risk Management or Quality Improvement are educators. Each committee meeting,
exchange with a staff member, or participation in new employee orientation, is an opportunity to dispel
misinformation and increase understanding of the risk management process.
The fundamentals of risk management are not highlighted in medical and nursing schools but are necessary to
prepare future health care providers for dealing with risk issues that arise in the clinical setting. The development
of a risk management curriculum gives structure to educational efforts and provides a framework to cover
essential topics, ranging from the orientation of new employees, staff physicians, and residents to seminars for
advanced practitioners, department chairpersons, and administrators.
Risk Management and Quality Improvement core curriculums should address the following concepts (85):

1. Explain the concepts of risk, risk reduction, and sentinel events


2. Understand the basic elements of a risk reduction protocol
3. Describe the functions of a risk manager, including when to contact them
4. Recognize that procedural changes need to be monitored to determine if they are really, operationally, more
effective and efficient than the prior interventions or procedures
5. Understand the relationship between risk management and professional liability carriers
All health care providers, including nurses, nurse practitioners, residents, house staff, and ancillary staff, such as
respiratory therapists and clinical nutritionists, should know the process a risk management investigation will
follow (Figs. 9.2 and 9.3).
P.109

FIGURE 9.3 Decision-making process. From the U.S.NRC 24th Annual Regulatory Information Conference,
March 30, 2012 (http://www.nrc.gov/about-nrc/organization/commission/comm-george-apostolakis/apostolakis-
03-13-2012-ric.pdf).

Health care professionals should avoid making editorial comments in patient records and should not keep
personal notes about clinical events. Objective clinical impressions belong in a patient's medical record;
subjective personal comments do not. Physicians, nurses, and other health care professionals should
understand that investigations take time and that the findings may be very different from the initial views of the
staff. Speculation about the causes of adverse events or discussions of the events outside the peer
review/quality improvement areas should be discouraged.
With an understanding of the fundamentals of risk reduction/management, the health care provider should be
able to examine the safety and effectiveness of the systems and processes that are involved in providing patient
care. The risk manager analyzes the frequency or severity of adverse events or unexpected outcomes and the
findings during the course of discovery in lawsuits, and then identifies topics that can be presented in an
advanced risk reduction curriculum. These include: retroactive peer review (e.g., clinicopathologic review,
regular review of perinatal or neonatal patient care statistics), documentation, communication, supervision,
monitoring and assessment, coordination of care, medication administration, system failure(s) and human error,
and changes in local, state, or national regulations or laws.

Adverse Events
Adverse sentinel events are defined by TJC as unexpected occurrences involving a risk of or actual death or
serious physical or psychological injury. The term sentinel reflects the perception that when one event occurs,
similar events may follow. A root cause analysis permits an intensive assessment of the event in question and a
determination of the actions needed to prevent and/or reduce similar events in the future. This type of analysis
permits an understanding of (a) what occurred, (b) why it occurred, (c) what factors were involved that allowed
the event to occur, (d) how the events could have been prevented, and (e) what changes should be
recommended.
In some instances, it is not possible to sort out the exact sequence of events. Interruptions in the system of care
delivery that are identified should be repaired or redesigned to prevent further breakdowns. Adverse,
unexpected, or poor patient outcomes do not necessarily indicate that a mistake has been made. It is important
to withhold drawing conclusions and instituting change or discipline until all the data have been presented and
analyzed.
Receiving an incident report and investigating problems that have occurred or potentially might occur is viewed
by the risk manager as an opportunity for improvement in the system of care delivery. Realistically, the stigma of
punishment or retaliation in relation to incident reports exists and may be a deterrent to obtaining the information
need for understanding and rendering improvement in health care delivery.

Accountability versus Blame


System and practice patterns are the focus of a risk management investigation. Investigations may reveal
systems and patterns that do not provide sufficient or effective safety checks to prevent human error or
miscommunication. In addition to identifying system or process flaws that cause and perpetuate poor outcomes,
adverse event investigations assist in the identification of potential high-risk clinicians as well as high-risk
populations. Risk identification leads to risk mitigation. Fewer complications generally are associated with
improved clinical care, fewer appearances before risk management and quality improvement committees, and
fewer lawsuits.

Communication
Less than optimal care is often perceived as being the result of inattention, carelessness, and/or negligence.
Risk Management and Quality Improvement may demonstrate that some events were not preventable, not
predictable, and not caused by human or mechanical failure. Risk communication translates the analyses of the
events in question to others in a coherent, comprehensible nonaccusatory manner. Alternative treatment or
intervention options, including the option of inaction, are presented. Addressing the patient's status, in terms of
changed circumstances, forces the discussion to go beyond recriminations and accusations. An examination of
what may occur next provides an option to look to the future. Confrontation should be avoided. Questioning
should be encouraged and not perceived as a challenge to the health care provider's knowledge, treatment
interventions, plans, or communication skills. It is axiomatic that NICUs are highly emotionally charged
environments, both for the parents and concerned family members and the health care providers. Risk
communication needs to be unhurried and held in a nonhostile environment. Patients, their families, clergy,
administrators, and clinicians should meet in a private, quiet room. In many cases, lawsuits are not the result of
malpractice but are instituted because the patient or patient's family feels that information was withheld from
them or that they were not told the truth. Effective communication skills used in data collection, developing
relationships, and dealing with parents' emotions help to prevent anger and possible malpractice litigation.
The perception by the family of the competency of the physicians, nurses, and other health care team members
is drawn from their interactions with them. Ideally, contact with the family inspires trust, confidence, and
openness. Positive contact with the family of a neonate is based upon the fundamentals of communication: body
language that conveys attention to the family and infant, taking time to answer questions, asking for input from
the family regarding their impressions of the baby's progress, and speaking in terms that can be understood by
the family. These basic clinical and social skills represent the foundation of positive defensive medicine
techniques. It is important to convey that team members taking care of the infant are communicating with each
other, are fully aware of the treatment plan, and are coordinated in their approach to the care of the infant.
Judgments and preconceived opinions about the family should be avoided, even in the face of socially
unacceptable behaviors and angry statements. Communication style requires compassion in a socially, culturally,
and morally neutral manner.
Tertiary medical centers and teaching facilities have complex hierarchies that are unknown and threatening to
families. Just as the family needs to have an identified spokesperson and communicator, so too does the hospital
or NICU need to identify one individual or office where family members can go and ask questions and receive
information and emotional support. Policies and procedures need to be explained so that they are not perceived
as being arbitrary and capricious and for the benefit of the hospital rather than the patient. In general, physicians
and other health
P.110
care professionals in training should not be designed the spokesperson; given their usual limited stay on any
given unit or within any given clinic. Spokespersons for the health care facility should understand that stating
Good question, I don't know the answer. Let me find the answer and get back to you is a positive action and
not any reflection on their competence.

Risk Communication
Risk communication is a multidisciplinary, multidimensional, interactive ongoing process that includes discussion
rather than didactic presentation of information. Risk communication is an educational opportunity. Risk
communication includes various levels of involvement in decisions, actions, or policies aimed at managing or
controlling health or environmental risks. Risk communication, in medicine, directly involves those affected by the
events in question. Risk communication becomes an opportunity for conflict resolution. Effective risk
communication is an exchange of information; it is a two-way process. Conflict resolution may be included in risk
communication. Family members and hospital staff, clinical and administrative, may disagree about the nature or
severity of the illness. Risk communication is able to support a consensus-building process; it is not designed to
eliminate dissent. According to the National Research Council (89), risk communication is a process that can be
considered successful only to the extent that it, first, improves or increases the base of accurate information that
decision makers use and, second, satisfies those involved that they are adequately informed within the limits of
available knowledge.
Risk communication may improve decision making. Personal and institutional anxieties and concerns can be
reduced or increased, depending on the inherent quality of the information provided. The risk communication
process requires increased communication between the sources of the information and recipients. It also
requires increased communication among the parties involved. Families need to be educated as to the meaning
of relative risk. The concept of probabilities needs to be explained. Medical terms need to be explained.

GENERAL PRINCIPLES OF RISK COMMUNICATION (90)


1. Accept and involve the public as a legitimate partner.
2. Plan carefully and evaluate your efforts.
3. Listen to the public's specific concerns.
4. Be honest, frank, and open.
5. Coordinate and collaborate with other credible sources.
6. Meet the needs of the media.
7. Speak clearly and with compassion.
These general principals are affected by individual perceptions of risk. A family's perception of a 5% risk, a risk
of 5 chances in a 100, is different from that of the neonatologist proposing the procedure in question. The
potential loss for the family is greater than that for the clinician. The family must yield control. The neonatologist
must accept control. The consequence or outcome is not evenly shared.
Risk communicators must ask themselves questions that are different from those that they are going to address.
The communicators need to know who their audience is, and what the family wants to hear. They must decide
on the message, the approach to be taken, and consider how it will be received and what their responses will be.
They need to anticipate linguistic, cultural, social, and economic issues. They must emphasize that health care
provider, patient, and the family are part of a single team, each bringing something unique to the arena.
Essential for optimal risk communication and discussion is an understanding that while there is an academic
distinction between a bad outcome due to disease progression and a bad outcome due to unfortunate
circumstances/errors/negligence, the family often cannot see the distinction when they look at their child.
Anxiety over the uncertainty inherent in the care of the seriously ill may evoke feelings of helplessness.
Outcomes that are expected by the physician but not clearly conveyed to the family may be a shock for the
family. The nonoccurrence of expected outcomes leaves the parents feeling that a mistake has been made.
Families who experience disappointments in clinical outcomes may take out their grief and despair on the health
care provider. The health care provider can never be fully prepared for the parents' anger and distrust.
Appearing uncertain, handling questions improperly, apologizing for oneself or the team, not knowing the
available information, not involving the family in the decision-making process, not establishing rapport, appearing
disorganized, and providing the wrong information (such as discussing the wrong patient) can all lead to a
breakdown in communication. Acknowledgment of hostility is acceptable; this indicates that the health care
provider recognizes the reality of the situation. Anxiety undercuts confidence, concentration, and momentum.
Listen to what others have to say, even if you disagree with them; recognize expressions of frustration. State
conclusions and then provide supporting data. Above all, do not lie.
If an adverse event occurs, the honest, sincere, and compassionate response of the health care team to the
family will do more than reduce the family's view that there is a need to bring a lawsuit. A family often needs
reassurance that they accepted the risk in good faith and with trust. Saying that you are sorry that the outcome
was not a good one is not unreasonable, and now, in many states will not be construed as an admission of guilt
or responsibility but rather compassion. Without burdening the family with personal feelings of inadequacy,
regret, or vague misgivings, it is helpful to show concern and to express empathy regarding the patient outcome.
Risk communication is a philosophical approach that when incorporated into daily activities improves
communication within and among the medical team and the overall hospital or medical center facility. Improved
communication includes better communication between physicians, nursing staff, and ancillary support staff. The
application of risk communication principles can decrease clinical errors. Clear oral and written communication is
vital in an NICU. A medical record alone cannot be relied upon to adequately convey urgent information between
members of a health care team.
The medical record should be designed to achieve clear communication with and among the members of the
health care team. Clinical information, in a medical chart, is focused on the patient more than the patient's family.
Decision making is reliant on repeated assessments, analyses, judgments, and actions aimed at achieving
specific goals. Documentation in the patient record allows ongoing evaluation of patient progress and review of
the clinical management plan. The individual contributions of the members of the health care team are not
always recorded. Both oral and written communication between and among treatment team members is a basis
for planning each new step in patient care. This documentation of the exchange of information among the team
and the recording of the outcome of each team member's analysis of the information helps to support the team's
choices and judgments, regardless of outcome. Documenting patient care, contemporaneous with the events,
provides the clinical picture that will be used in the defense of the care provided should an adverse event occur.
If the clinical record has the appearance of being a battleground for warring or defensive factions of the team, the
record will reflect a lack of team cohesion and direction that is difficult to defend. The record should contain the
facts of the event, assessments of the patient, the decision-making processes, and the interventions undertaken.
P.111
Any documentation of an abnormality in the medical record should be accompanied by the reassuring factors that
support the overall interpretation of the clinical findings. All factors that explain decisions made in the face of
abnormal finding(s) should be recorded. In fact, documentation of reassuring factors should accompany the
recording of abnormal factors.
Clinical findings, if possible, are best graded using numeric values. Modifiers, such as the words extreme,
severe, or massive do not provide objective information and will feed into the drama of a courtroom
presentation. Contributors to the evaluation and care of the patient must avoid inflammatory language, markings,
or punctuation that attempt to draw attention to the writer and imply that the team may be inattentive to the
remarks if not emphasized.
It is possible that an infant's condition is changing so rapidly that thorough, contemporaneous notes in the
medical record cannot be made. Although not documented, not done is a well-known legal motto, there is much
that occurs that cannot be documented in the patient record at the time of a critical event (91). It is often
hindsight that focuses the care provider on events not recorded in contemporaneous records. A decision must be
made as to whether an addendum to the medical record would contribute to patient care or whether the
additional information relates to system issues and should be conveyed to the risk manager by completing an
incident report. References to the completion of the incident report or discussions with the risk manager are not
appropriate for, and should not appear in, the medical record (92); see discussion above dealing with the
distinction between confidential and protected quality improvement activities (83) and risk management (not
protected) records. An incident report relates to an adverse event; the patient's record reflects care and
treatment delivered, the patient's response, and the further plan of care.
When additional information, identified after an adverse event, is necessary to the care of the patient,
communication of this information is achieved in an addendum to the medical record. Such entries must include
the date and time that they were written. Under no circumstances should an attempt be made to make this entry
appear contemporaneous with the adverse event. The addendum must be placed sequentially in the medical
record in the next available space, not on a separate page, and never inserted into the text of previous entries.
The purpose of an addendum is not to provide a defense or to make excuses.

Communication with Consultants


The curbside consultation between colleagues is a thing of the past. This type of consultation tends to be
documented poorly, if at all, and can lead to damaging finger pointing in a court of law. Guidelines for the
consultation process are provided in Table 9.2.

TABLE 9.2 Steps in the Consultation Process

Identify when it is appropriate to obtain consultation


Choose the type of consultant (sequencing of a variety of consultants)
Share the purpose of the consultation with the family
Decide what information is necessary to provide to the consultant, including the purpose of the
consultation and provide as a written consultation request
Arrange a formal consultation or designate this responsibility to a specific individual
Give a time frame within which the consultation is to be done
Agree with the consultant on how the consultation will be reported and who will discuss the findings
with the family/parent(s)
Share the outcome of the consultation with the family
Tell the family what will be done with the resulting information
Document in the medical record the reasons for requesting a consultation, discussions with the family,
and plan of treatment, including the reasons why the recommendations of the consultant will or will
not be followed

When a consultant is contacted for a specific patient with a formal request for evaluation and/or management,
both the primary physician and consultant should interact in such a way as to limit unnecessary risk for both.

CONCLUSION
Fifty years ago, there was no organized subspecialty field now known as neonatology. Forty years ago,
telemedicine generally referred to the use of a telephone. Thirty years ago, rotary and fixed-wing transport
of the high-risk pregnant woman became a regular occurrence across this country. Twenty years ago,
electronic data access and data transfer, remote interpretation of radiographic and other diagnostic studies,
became integrated into patient care. Ten years ago, paper textbooks continued to be replaced with CDs,
DVDs, and Internet Web sites. Science evolves, technology develops, and treatment regimens become
more complex, but human communication skills and human nature have not kept pace. Health care
providers need to maintain and develop their specialty skills while learning more about the environment in
which they practice. The practice of any medical discipline or medical specialty is more effective when the
legal, ethical, social, and cultural perspectives are fully understood. Communication with patients and their
families is the first and last step in treating a patient. The quality of the experience is the shared
responsibility of all who come in contact with the patient and family.

REFERENCES
1. http://avalon.law.yale.edu/ancient/hammper-asp. Accessed on January 28, 2014.

2. Public Law 111-148Patient Protection and Affordable Care Act of 2010 (commonly referred to as
Obamacare).

3. 2014 Hospital Accreditation Standards, Joint Commission Resources (JCAHO), Oakbrook Terrace, IL.

4. Joint Commission on International Accreditation Standards for Hospitals, Joint Commission International,
5th ed., Oakbrook Terrace, IL, 2013.

5. Pearlmutter M. Physician apologies and general admissions of fault: amending the federal rules of
evidence. Ohio State Law J 2011;72:688.

6. Public Law 104-191Health Insurance Portability and Accountability Act of 1996 (commonly referred to as
HIPAA).
7. Dobbs DB, Keeton RE, Owen DG. Prosser and Keeton on the law of torts, 5th ed. Eagan, MN: West
Group, 1984:356.

8. Inglefinger FJ. Informed (but uneducated) consent. N Engl J Med 1972; 287:465.

9. The Emergency Medical Treatment and Active Labor Act (EMTALA), passed in 1986 is part of the
Consolidated Omnibus Budget Reconciliation Act (COBRA) at 42 USC 1395(dd).

10. Hess v Paulowski , 279 US 352 (1927).

11. 28 USC 1332

12. 2012 Update 1 to the Comprehensive Accreditation Manual for Hospitals and the Comprehensive
Accreditation Manual for Critical Access Hospitals, Oakbrook Terrace, IL, 2012.

13. Public Law 104-104, Telecommunications Reform Act of 1996.

14. Senate Bill 773, 1995 Reg. Sess. (La. 1995).

15. http://www.ntia.doc.gov/reports/telemed/index.htm

16. Jackson v Johns-Manville Sales Corp., 727 F2d 506,516 (5th Cir 1984).

17. McCormick C. McCormick on evidence, 2nd ed. St. Paul, MN: West Publishing Co., 1972:339.

18. Morgan E. Basic problems of evidence, 4th ed. Philadelphia, PA: Joint Committee on Continuing Legal
Education, 1963:24.

19. Terry HT. Negligence. Harv Law Rev 1915;29:40.

20. Zebarth v Swedish Hospital Medical Center, 81 Wash 2d 12,499 (P2d 1 1972).

21. Second Restatement of Torts, 282

22. Ely JW, Levinson W, Elder EC, et al. Perceived causes of family physicians' errors. J Fam Pract
1995;40:337.

23. Oelling v Rao, 593 NE2d 189 (1992).

24. Small v Howard, 128 Mass 131 (1880).

25. Peck v Hurchinson, 88 Iowa 320, 55 NW 511.

26. Daubert v Merrill Dow Pharmaceuticals, Inc. 509 US 579 (1993).


27. Kumho Tire Co. v. Carmichael , 526 U.S. 137 (1999).

28. Ybarra v Spangard, 25 Cal App 2d 486, 154 P2d 687 (1944).

29. Mohr v Williams, 104 NW 12, 5 Ct (1905).

30. Canterbury v Spence, 464 F2d 772 (DC Cit 1972), cert denied, 409 US 1064 (1972).

P.112

31. Getchell v Mansfield, 260 Or 174, 4P2d 953 (1971).

32. Cooper v Roberts, 286 A2d 647,650 (1971).

33. Davis v Wyeth Laboratories, Inc., 399 F2d 121 (1968).

34. Application of President & Directors of Georgetown College, 331 F2d 1000 (DC Cir), cert denied, 377 US
978 (1964).

35. http://www.latimes.com/science/sciencenow/la-sci-sn-brain-dead-mother-fetus-survival-munoz-
20140114,0,1320584.story

36. King NM. Transparency in neonatal intensive care. Hastings Cent Rep 1992;22:18.

37. Paasche-Orlow MK, Taylor HA, Brancati FL. Readability standards for informed-consent forms as
compared with actual readability. N Engl J Med 2003;348:721.

38. Gutheil TG, Bursztajn H, Brodsky A. Malpractice prevention through the sharing of uncertainty. Informed
consent and the therapeutic alliance. N Engl J Med 1984;311:49.

39. Gillick MR. Advance care planning. N Engl J Med 2004;350:7.

40. Committee on Bioethics, American Academy of Pediatrics. Informed consent, parental permission, and
assent in pediatric practice. Pediatrics 1995;95:314.

41. Little v Little, 576 SW2d 493 (1979).

42. In Re Richardson, 284 So2d 185 (1973).

43. Medicaid frauda multibillion-dollar crime. Available at:


http://www.newsmax.com/archives/articles/2002/12/30/170723s.html

44. U.S. Department of Health and Human Services and U.S. Department of Justice Health Care Fraud and
Abuse Control Program. Annual Report for FY 2002. Washington, DC: U.S. Government Printing Office,
2003.
45. http://www.bloomberg.com/news/2012-06-14/medicaid-fraud-audits-cost-five-times-amount-u-s-
found.html

46. Federal crackdown puts risk managers in hot seat [Editorial]. Health Care Risk Manage 1997;19:49.

47. Hayt E, Hayt LR, Groeschel AH. Law of hospital, physician, and patient, 2nd ed. New York: Hospital
Textbook Co., 1952:637.

48. Beck JC. Confidentiality versus the duty to protect: foreseeable harm in the practice of psychiatry.
Washington, DC: American Psychiatric Press, 1990.

49. Reisner v Regents of the University of California 31 Cal App 4th 1195, 37 CalRptr2d518 (1995).

50. Tarasoff v Regents of University of California, 108 Cal Rptr 878 (Cal App 1973), superseded by Tarasoff
v Regents of University of California, 13 Cal 3d 177, 118 Cal Rptr 129,529 P2d 553 (1974), subsequent op
on reh Tarasoff v Regents of University of California, 17 Cal 3d 425, 131 Cal Rptr 14,551 P2d 334 (1976).

51. Lipari v Sears Roebuck & Co., 497 F Supp 185 (D Neb 1980).

52. 42 USC 290ee-3

53. 42 USC 290dd-3

54. 42 USC 290ee-3(b)(2)(A)

55. 42 USC 290dd-31(b)(2)(A)

56. 42 USC 290ee-3(tb)(2)(C)

57. 42 USC 290dd-3(b)(2)(C)

58. Dick RS, Steen EB, eds. The computer-based patient record: an essential technology for health care.
Washington, DC: National Academy Press, 1991.

59. Woodward B. The computer-based patient record and confidentiality. N Engl J Med 1995;333:1419.

60. Annas GJ. The rights of patients: the basic ACLU guide to patient rights, 2nd ed. Carbondale, IL:
Southern Illinois University Press, 1989:178.

61. Kramer C. Medical Malpractice 5 (1976), citing History of Reported Medical Professional Liability Cases,
30 Temple LQ 367 (1957).

62. Pegram v Herdrich, 530 US 211 (2000), 154 F3d 362, reversed.
63. Aetna Health Inc. v Davila, 542 US (2004), 307 F3d 298, reversed and remanded.

64. Malone TW, Thaler DH. Managed health care: a plaintiff's perspective. Tort Insur Law J 1996;32:123.

65. Clifford RA. Physician's liability in a managed care environment. Health Lawyer 1997;10:5.

66. Bodenheimer T. The HMO backlashrighteous or reactionary? N Engl J Med 1996;335:1601.

67. Moore v Regents of the University of California, 793 P2d 479, 51 Cal 2d 120 (1990).

68. Kanute M. Evolving theories of malpractice liability in HMOs. Loy Univ Chic Law Rev 1989;20:841.

69. Fox v Health Net, Civ No 21962 (Riverside County Super Ct, Cal 1993).

70. Sloan v Metropolitan Health Council, 516 NE2d 1104 (Ind Ct App 1987).

71. Restatement (second) of Agency 216, 1958.

72. Restatement (second) of Agency 250, 1958.

73. Schleier v Kaiser Foundation Health Plan of the Mid-Atlantic States, Inc., 876 F2d 174 (DC Cir 1989).

74. Darling v Charleston Community Memorial Hospital, 211 NE2d 253 (1965), cert denied, 383 US 946
(1966).

75. Wickline v State, 192 Cal App 3d 1630, 239 Cal Rptr 810 (1986).

76. http://seattletimes.com/html/localnews/2022371201_exchangenetwork-sxml.html

77. 29 USC 1001-1461

78. 29 USC 1144(a)

79. Shea v Esensten, 107 F3d 625 (8th Cir 1997).

80. Harper v Healthsource New Hampshire, 674 A2d 962 (NH 1996).

81. Texas Medical Association v Aetna Life Insurance Co., 80 F3d 153 (5th Cir 1996).

82. 42 USCA 11131-11137 (West Supp 1995).

83. 38 USC 5705

84. 31 USC 3729-3733


85. Kohn LT, Corrigan JM, Donaldson MS, eds. To err is human: building a safer health system. Washington
DC: National Academy Press, 2000.

86. http://www.justice.gov/opa/pr/2013/December/13-civ-1352.html

87. Food Drug and Cosmetic Act 21 USC 301 et seq

88. 42 USC 1395nn

89. http://odphp.osophs.dhhs.gov/pubs/prevrpt/Archives/95fm1.htm

90. Seven Cardinal Rules of Risk Communication. Pamphlet drafted by Vincent T. Covello and Frederick H.
Allen. U.S. Environmental Protection Agency, Washington, DC, April 1988, OPA-87-020.

91. Hartline JV Smith CG. Risk management in medical consultation. In: Donn SM, Fisher CW, eds. Risk
management techniques in perinatal and neonatal practice. Armonk, NY: Futura Publishing, 1996:617.

92. Chilton JH, Shimmel TR. Inappropriate word choice in the labor and delivery and newborn medical
record. In: Donn SM, Fisher CW, eds. Risk management techniques in perinatal and neonatal practice.
Armonk, NY: Futura Publishing, 1996:603.
10
Prenatal Diagnosis and Management in the Molecular AgeIndications, Procedures, and Laboratory
Techniques
Mark I. Evans
Stephanie Andriole
Roee Birnbaum
Yuval Yaron

INTRODUCTION
In the beginning of the 20th century, leading causes of infant mortality (approximately 150/1,000) included infectious diseases. With the development of antibiotics and increasingly
sophisticated medical and surgical therapies, the primary causes of child mortality shifted to genetic and congenital disorders (1). Particularly, for syndromic conditions, the
pediatrician is commonly the first medical provider to raise the issue of future pregnancies and the possibilities for prenatal diagnosis.
Over the course of the past four decades, there have been revolutionary changes in our approach to prenatal diagnosis and screening (2). In the 1960s and 1970s, we evolved from
merely wishing patients good luck to then asking how old are you? Maternal age was, and still is, a cheap screening test for aneuploidy, but there has been an explosion of
techniques that have dramatically enhanced the statistical performance of screening tests to identify high-risk patients. Screening for elevated maternal serum alpha-fetoprotein
(MSAFP) for neural tube defects (NTDs) began in the 1970s, and low MSAFPs were discovered to be associated with Down syndrome (DS) and trisomy 18 in the 1980s (3). Nuchal
translucency (NT) and several other ultrasound (US) markers then emerged, which increased the efficacy of both US screening and detection of anomalies. Biochemical and now
molecular markers have moved to the forefront of screening and diagnostic testing and have both revolutionized our abilities and challenged some of the basic tenets of the past
decades (2).
We see a pendulum swinging back and forth between screening and testing primacy as new technologies have been developed (Fig. 10.1). Overall, prenatal diagnosis has moved
along two parallel paths that sometimes converge (i.e., imaging and tissue diagnoses) (see also Chapters 12 and 35). In many instances, clinicians are experts in one diagnostic
modality or the other; there are a very limited number who are experts in both. As a result, there is often huge variability in approach to screening and diagnosis depending upon by
whom and where a patient is seen. We can, oversimplistically, divide overall approaches into basic, selective, and comprehensive. As they internalize reproductive risks, both
physicians and patients have to decide how much effort they are willing to exert to evaluate those risks and what they would do with the results ( Fig. 10.2). As with all advances in
medicine and science and culture within any society, there is a gradual acceptance and incorporation of new technologies that proceed at highly different paces in different locales.
The Internet has hastened the dissemination process; sophisticated patients even from remote areas can now access information on what is available if they are willing to travel to
get it. At tertiary/quaternary centers such as ours, a significant proportion of patients travel considerable distances for services that are not available at home (4,5).
Thousands of papers and hundreds of textbooks have been written over the past decades about the subjects addressed in this chapter, but only a miniscule percentage of the
available literature can be cited here. We will provide a summary of key points, but no short chapter can possibly do justice to the enormous technical advances in multiple
disciplines that have contributed to our abilities to screen, diagnose, and treat fetal conditions. In this chapter, we concentrate on classic genetics, while in Chapter 12, the focus is
on US and other imaging techniques.

GENETIC COUNSELING
As the complexity of genetic information has increased massively in scope and amount, the need to explain it has skyrocketed in parallel. The best analogy is to computers, for which
Moore law predicted that capabilities would double every 18 to 24 months and cost would decrease by 50%. At least the first half of the equation has applied in genetics. The
situation has been made even more challenging because currently advances in clinical medicine are often beholden to technologic advances in disciplines that are outside the
culture of medicine (6). For example, many of the noninvasive screening techniques rely upon the ingenuity and intellectual capabilities of electrical engineers and venture
capitalists who do not necessarily adhere to the ethos of putting patient care above all else and who have pushed to introduce tests into practice without sufficient testing, medical
peer review, and user/patient education. Numerous direct-toconsumer genetic companies have emerged that provide frequently alarming informationoften without context.
Likewise, shopping mall US boutiques have emerged to supply baby pictures (7). In both instances, patients often believe they have received complete services when in fact
important questions about their specific situation have neither been asked nor answered (7).
Genetic counseling is the foundation of educating patients as to the risks of reproduction, opportunities to investigate those risks, and options for dealing with the information
obtained. There is no one standard for what genetic counseling is in practice. Numerous studies have documented that the education of most obstetricians in genetics is suboptimal;
thus, relatively few physicians caring for pregnant women are in a position to have a substantive discussion about complex genetic issues (8). There are approximately 200
obstetrician-gynecologists in the United States who are also trained and board certified in genetics, so that the approximately 2,000 maternal-fetal medicine subspecialists perform a
disproportionate proportion of genetic evaluations. However, while they often have considerably more knowledge than general obstetricians, perinatologists are commonly very time
challenged and may not maintain a state-of-the-art understanding of the rapid changes
P.114
in screening and testing technology options or have the time for in-depth, unrushed discussions with patients.

FIGURE 10.1 Generations of screening and testing choices. CVS, chorionic villus sampling; ff, free fetal.
Genetic counseling as a profession emerged over the past few decades. Genetic counselors are master's trained individuals who have both an in-depth knowledge of genetic
fundamentals and an understanding of screening principles and testing options. Their credo includes respect for one of the key tenets of genetics, that is, nondirective presentation
of information. In many settings, the counselors have far more understanding of genetic issues than does the attending physician, which can be the cause of quality of patient care
issues if care is not regarded as a team effort. The authors, all of whom have received formal training in genetics, believe that it is optimum to have a coordinated team approach to
patient care. What too frequently occurs is that a vending machine selection of possible testing options is offered to the patient without adequate guidance. Only when there is an
abnormal result does the primary medical provider call for help to explain to an often panicked patient what the results actually mean. When possible, we believe that dedicated
centers providing the continuum of genetic counseling, diagnosis, and treatment are optimal. Alternatively, in this digital age, it should be possible to create a hierarchy of services
from networked providers that approximates the kind of care that would be available in a comprehensive center.

FIGURE 10.2 Flowchart for prenatal genetic evaluation and management. PAPP-A, pregnancy associated plasma protein-A; ff, free fetal; MPSS, massive parallel shotgun
sequencing; CVS, chorionic villus sampling.

INDICATIONS
Prenatal Screening
Mendelian Disorders
To the pediatrician, screening for mendelian disorders has been central to care for decades (9). The routine use of newborn heel-stick blood sample screening is nearly
universal in the United States and many developed countries and continues to rapidly expand in both utilization and quantity of tests available (10). In the 1970s, prenatal
screening for mendelian disorders was simple: sickle cell for Africans, Tay-Sachs for Ashkenazi Jews, -thalassemia for Mediterraneans, and -thalassemia for Asians. Since
then, there has been an explosion of testing possibilities, and a serious disconnect has developed between the individual risk for a specific disorder and the availability
P.115
of screening tests. For example, the Ashkenazi panel has increased from 1 to 3 available tests in the 1970s to 18 current routine test offerings (11), and more tests are
currently under development. For many of the diseases tested for, the incidence in the Jewish population is actually no higher than in other ethnic groups, and the incidence of
some is less than 1/100,000. Several companies are now offering panethnic screening for dozens of disorders (12). Although there have been some serious problems in the
implementation of these screens, including confusion regarding the actual risks of a disease for individual couples, it may ultimately prove more cost-effective to offer screening
for everything to everyone. However, such expansion of screening will require an increase in the understanding of genetic testing and the ability to appropriately
communicate the information far beyond the level currently available. We frequently see new patients who state that they have already been screened for everything and who
are very upset to learn that there is no such thing.
Public policy will also have to catch up with technologic reality (13). At the same time that there has been at least partial acceptance of screening for rare disorders such as
Usher syndrome, national organizations such as the American College of Obstetrics and Gynecology (ACOG) have declined to endorse universal screening for disorders such
as fragile X and spinal muscular atrophy (SMA) whose incidences are far higher ( Table 10.1). With rapid advances in testing technologies, the cost/benefit analyses of how
much it is worth doing can be expected to change many times over the next several years.

TABLE 10.1 Expanded Mendelian Disorder Panela

Gene Variants Detection


Rates

ABCC8-related ABCC8 (3): F1388del, V187D, 3992-9G>A South Asian


hyperinsulinism <10%,
Ashkenazi
Jewish
90%.

Alpha-mannosidosis MAN2B1 (1): R750W South Asian


<10%,
Ashkenazi
Jewish
32%.
Ataxia-telangiectasia ATM (8): R35X, Q1970X, 7517del4, 5762ins137, 2546_2548del, 3245ATC>TGAT, K1192K, E1978X South Asian
<10%,
Ashkenazi
Jewish
65%.

Autosomal recessive PKHD1 (4): Leu1965fs, T36M, R496X, V3471G South Asian
polycystic kidney <10%,
disease Ashkenazi
Jewish18%.

Bardet-Biedl BBS1 (1): M390R South Asian


syndrome, BBS1- 79%,
related Ashkenazi
Jewish
79%.

Bardet-Biedl BBS10 (1): C91fs South Asian


syndrome, BBS10- 46%,
related Ashkenazi
Jewish
46%.

Biotinidase deficiency BTD (4): G98:d7i3, D252G, Q456H, R538C South Asian
45%,
Ashkenazi
Jewish
45%.

Bloom syndrome BLM (1): 2281del6ins7 South Asian


<10%,
Ashkenazi
Jewish
99%.

Canavan disease ASPA (4): E285A, Y231X, A305E, IVS2-2A>G South Asian
53%,
Ashkenazi
Jewish
98%.

Carnitine CPT1A (1): G710E South Asian


palmitoyltransferase IA <10%,
deficiency Ashkenazi
Jewish
<10%.

Carnitine CPT2 (3): Q413fs, S113L, R124X South Asian


palmitoyltransferase II 80%,
deficiency Ashkenazi
Jewish
80%.

Cartilage-hair RMRP (1): g.70A>G South Asian


hypoplasia 48%,
Ashkenazi
Jewish
48%.

Citrullinemia type 1 ASS1 (2): IVS6-2A>G, G390R South Asian


20%,
Ashkenazi
Jewish
20%.

CLN3-related neuronal CLN3 (1): 461_677del South Asian


ceroid lipofuscinosis 96%,
Ashkenazi
Jewish
96%.

CLN5-related neuronal CLN5 (1): 2467AT South Asian


ceroid lipofuscinosis <10%,
Ashkenazi
Jewish
<10%.
Cohen syndrome VPS13B (1): 3348_3349delCT South Asian
<10%,
Ashkenazi
Jewish
<10%.

Congenital disorder of PMM2 (4): V231M, F119L, R141H, P113L South Asian
glycosylation type Ia <10%,
Ashkenazi
Jewish
72%.

Congenital disorder of MPI (1): R295H South Asian


glycosylation type Ib <10%,
Ashkenazi
Jewish
<10%.

Congenital Finnish NPHS1 (2): 121_122del, R1109X South Asian


nephrosis <10%,
Ashkenazi
Jewish
<10%.

Costeff optic atrophy OPA3 (1): 143-1G>C South Asian


syndrome <10%,
Ashkenazi
Jewish
<10%.

Cystic fibrosis CFTR (99): G85E, R117H, R334W, R347P, A455E, G542X, G551D, R553X, R560T, R1162X, W1282X, N1303K, South Asian
F508del, I507del, 2184delA, 3659delC, 621+1G>T, 711+1G>T, 1717-1G>A, 1898+1G>A, 2789+5G>A, 54%,
3120+1G>A, 3849+10kbC>T, E60X, R75X, E92X, Y122X, G178R, R347H, Q493X, V520F, S549N, P574H, Ashkenazi
M1101K, D1152H, 2143delT, 394delTT, 444delA, 1078delT, 3876delA, 3905insT, 1812-1G>A, 3272- Jewish
26A>G, 2183AA>G, S549R(A>C), R117C, L206W, G330X, T338I, R352Q, S364P, G480C, C524X, 97%.
S549R(T>G), Q552X, A559T, G622D, R709X, K710X, R764X, Q890X, R1066C, W1089X, Y1092X,
R1158X, S1196X, W1204X(c.3611G>A), Q1238X, S1251N, S1255X, 3199del6, 574delA, 663delT, 935delA,
936delTA, 1677delTA, 1949del84, 2043delG, 2055del9>A, 2108delA, 3171delC, 3667del4, 3791delC,
1288insTA, 2184insA, 2307insA, 2869insG, 296+12T>C, 405+1G>A, 405+3A>C, 406-1G>A, 711+5G>A,
712-1G>T, 1898+1G>T, 1898+5G>T, 3120G>A, 457TAT>G, 3849+4A>G, Q359K/T360K

D-Bifunctional protein HSD17B4 (2): G16S, N457Y South Asian


deficiency 35%,
Ashkenazi
Jewish
35%.

Familial dysautonomia IKBKAP (2): IVS20+6T>C, R696P South Asian


<10%,
Ashkenazi
Jewish
>99%.

Familial Mediterranean MEFV (4): M694V, V726A, M680I, M694I South Asian
fever <10%,
Ashkenazi
Jewish
75%.

Fanconi anemia type C FANCC (3): IVS4+4A>T, 322delG, R548X South Asian
<10%,
Ashkenazi
Jewish
99%.

Fragile X syndrome FMR1 (1): NM_002024.4:c.1-131CGG[1_n].


(triplet repeat detection
only)

Galactosemia GALT (8): S135L, Q188R, F171S, L195P, K285N, IVS2-2A>G, T138M, Y209C South Asian
80%,
Ashkenazi
Jewish
80%.

Gaucher disease GBA (10): N370S, L444P, 84GG, IVS2+1G>A, V394L, R496H, D409H, D409V, R463C, R463H South Asian
60%,
Ashkenazi
Jewish
95%.
GJB2-related DFNB1 GJB2 (7): 35delG, 167delT, 235delC, E120del, W24X, W77R, L90P South Asian
nonsyndromic hearing <10%,
loss and deafness Ashkenazi
Jewish
84%.

Glutaric acidemia type GCDH (1): R402W South Asian


1 12%,
Ashkenazi
Jewish
40%.

Glycogen storage G6PC (7): R83C, Q347X, Q27fsdelC, 459insTA, R83H, G188R, Q242X South Asian
disease type Ia 30%,
Ashkenazi
Jewish
99%.

Glycogen storage SLC37A4 (2): 1211delCT, G339C South Asian


disease type Ib <10%,
Ashkenazi
Jewish
46%.

Glycogen storage AGL (3): 1484delT, Q6X, 17delAG South Asian


disease type III 45%,
Ashkenazi
Jewish
45%.

GRACILE syndrome BCS1L (1): S78G South Asian


<10%,
Ashkenazi
Jewish
<10%.

Hb beta chain-related HBB (28): Hb S, K17X, Q39X, Phe41fs, Ser9fs, IVSII-654, IVS-II-745, IVS-II-850, IVS-I-6, IVS-I-110, IVS-I-5, IVS-I- South Asian
hemoglobinopathy 1(G>A), -88C>T, -28A>G, -29A>G, Lys8fs, Phe71fs, IVS-II-849(A>C), IVS-II-849(A>G), Gly24 T>A, -87C>G, 86%,
(including beta Hb C, W15X, Gly16fs, Glu6fs, Hb E, Hb D-Punjab, Hb O-Arab Ashkenazi
thalassemia and sickle Jewish
cell disease) 83%.

Hereditary fructose ALDOB (3): A149P, N334K, A174D South Asian


intolerance <10%,
Ashkenazi
Jewish
75%.

Herlitz junctional LAMA3 (1): R650X South Asian


epidermolysis bullosa, <10%,
LAMA3 related