Index of Suspicion in the Nursery
3 Late Preterm Infant with Respiratory Distress
AUTHOR DISCLOSURE Dr Rhine has
disclosed that he owns stock in SonarMed,
Nfant, and Novonate. Drs Kim and Arain have
disclosed no financial relationships relevant to
this article. This commentary does not contain
a discussion of an unapproved/investigative
use of a commercial product/device.
Downloaded from http://neoreviews.aappublications.org/ at Indonesia:AAP Sponsored on April 17, 2021
Michael Kim, DO,* Yassar Arain, MD,† William Rhine, MD†
*Department of Pediatrics, Medical College of Wisconsin Affiliated Hospitals, Milwaukee, WI
†
Division of Neonatal and Developmental Medicine, Department of Pediatrics, Stanford University,
Palo Alto, CA
PRESENTATION
A 2.7-kg male neonate is delivered at 36 weeks’ gestation by a 33-year-old gravida 1,
para 1 woman via cesarean section. The cesarean delivery was indicated for non-
reassuring fetal heart tones and arrest in the first stage of labor. The mother’s
pregnancy had been complicated by gestational diabetes (managed with metfor-
min), chronic hypertension, and preeclampsia. The family history is not signif-
icant for any congenital cardiac defects or genetic syndromes. Fetal echocardiography
suggested a coarctation of the aorta at 34 weeks of gestation. Fetal ultrasonography
at 28 weeks’ and 34 weeks’ gestation did not show any anomalies.
After an uncomplicated delivery (Apgar scores of 8 and 9 at 1 and 5 minutes,
respectively), the infant develops mild respiratory distress, which requires con-
tinuous positive airway pressure (CPAP). He is admitted to the NICU for
anticipatory screening for coarctation and management of his respiratory dis-
tress. Chest radiography performed 1 day after birth shows no significant signs
of respiratory distress syndrome and normal chest anatomy, with no defects
(Fig 1). His respiratory distress subsides within 1 day after birth and feeding is
initiated on the same day. Postnatal evaluation, including 4 extremity blood
pressure measurements, pre- and postductal saturations, and physical findings,
are reassuring within the first 72 hours, though cardiac imaging is yet to be
performed. Four days after birth, the neonate develops bilious emesis and
respiratory distress, prompting reinitiation of CPAP. Subsequent imaging
studies confirm the diagnosis (Fig 2).
DISCUSSION
Respiratory distress with bilious emesis in the neonate should prompt immediate
evaluation and workup. Differential diagnosis in a 3-day-old late preterm neonate
includes sepsis, respiratory distress syndrome, malrotation, volvulus, anatomic
defects or variants including diaphragmatic hernias, and less commonly, necro-
tizing enterocolitis. For this neonate’s presentation, chest radiography showed
concern for loops of bowel in the left chest cavity (Fig 2). With subsequent chest
ultrasonography and lateral table tilt chest radiography, the findings were most
concerning for a congenital diaphragmatic hernia (CDH) (Fig 3).
Based on his prior studies and otherwise normal chest radiograph on the first
day after birth, late-presenting CDH in the neonate was suspected. We suspected
a hernia sac covered most of the defect, and gradual increasing abdominal
pressures subsequently displaced portions of the confirmed bowels into the
chest cavity.
Vol. 20 No. 9 S E P T E M B E R 2 0 1 9 e527
 Figure 1. Normal anteroposterior chest radiograph first day after birth
taken for respiratory distress.

Figure 3. Lateral cross-table chest radiograph confirming loops of bowel

CDH is an opening that forms a communication between in the left chest cavity (arrows).
the peritoneal and pleural cavities. CDH is one of the most
common structural defects occurring in 1 to 4 in 10,000
defined as older than 30 days of age at detection. (5)(6)(7)
births. (1)(2) If it occurs in utero, embryologic conse-
Some retrospective studies speculate that the lower percent-
quences, including lung hypoplasia and cardiac complica-
age is attributed to the improvement of prenatal screenings
tions, can lead to poor postnatal outcomes, including death.
and treatment, while rural areas may report higher values
Speculation on its pathogenesis is thought to be a failure in
because of limited resources. (7) Among these studies,
the embryologic development and closure of pleuroperito-
data on the diagnosis of late CDH in the neonatal period
neal folds early in gestation around 4 to 10 weeks. (3)(4)(5)
are limited and a smaller fraction of the late-presentation
There has not been a clear association with sex, but reports
groups may be included, because most of these cases may
suggest that it occurs in boys more than in girls. (1) Approx-
be asymptomatic. (6)(7)
imately 2.5% to 25% of CDH cases present “late,” which is
CDH screening occurs at 24 weeks’ gestation with pre-
natal ultrasonography (at least 50% of cases diagnosed), or
on rare occasion using fetal magnetic resonance imaging.
(8) Postnatal signs and symptoms that should raise suspi-
cion include a scaphoid abdomen in combination with
respiratory distress. A CDH can be detected postnatally

Figure 2. Anteroposterior chest radiograph demonstrating loops of Figure 4. Thorascopic view of the diaphgramatic hernia demonstrating
bowel in the left pleural space (arrows). small bowel loops (arrows) in the chest cavity.

e528 NeoReviews
Downloaded from http://neoreviews.aappublications.org/ at Indonesia:AAP Sponsored on April 17, 2021
with a chest radiograph that displays herniation of abdom-
inal contents into the chest cavity. (8)
A CDH is characterized based on the type of defect and
its location. Left-sided hernias are more common, consist-
ing of almost 85% of all reported CDH cases. Locations
are labeled as posterolateral (Bochdalek), anterior (Morgag-
ni), or central anterior (septum transversum of the Cantrell
type). The defect can also vary within those respective
locations and are identified by a letter classification system as
described by Ackerman et al. (3) Posterolateral hernias are
the most commonly reported type in literature. (4)
Management of CDH is a surgical urgency. Despite the
good outcomes of CDH repair with delayed presentation
in a neonate, missing the diagnosis could prove fatal.
Herniation of the contents with fully developed lungs and
vasculature can lead to respiratory distress and cardiac
failure. CDH repairs involve thoracoscopic or minimally
invasive surgery and the outcomes are usually favorable if
detected early. Respiratory support in addition to bowel
decompression is suggested to decrease intra-abdominal
pressure and to limit the bowel dilation. Enteral nutritional
support is withheld before operative repair, because the
bowel location in the chest precludes enteral feeds. If
prolonged medical resuscitation is anticipated, intravenous
access and parenteral nutrition should be initiated.
Patient Course
The neonate underwent a thoracoscopic diaphragmatic
hernia repair. Initial thoracoscopy confirmed a diaphrag-
matic hernia with the presence of small bowel loops and
stomach in the thoracic cavity (Fig 4). Once gently reduced
into the abdomen, a type A defect in the diaphragm was
identified. In addition, there was a hernia sac encompass-
ing the contents. After mobilization of the hernia sac, the
diaphragmatic defect was repaired using interrupted per-
manent sutures. A pigtail catheter was left for chest drain-
age and the patient was taken back to the NICU for
postoperative care. A repeat chest radiograph was ob-
tained, which showed complete repair of the defect.
The neonate recovered well from the operation and
he reached full feeds 12 days after birth and was placed
on H2-blocker prophylaxis. He was discharged the same day
and had no complications.
Lessons for the Clinician
• Respiratory distress with bilious emesis in the neonate
should warrant an evaluation with imaging and the
Downloaded from http://neoreviews.aappublications.org/ at Indonesia:AAP Sponsored on April 17, 2021
differential should include anatomic defects such as a
late-presenting CDH.
• Although late-presenting CDH may have favorable out-
comes, timely management and repair are essential to
prevent cardiorespiratory compromise in the patient.
American Board of Pediatrics
Neonatal-Perinatal Content
Specifications
• Recognize the clinical features of extrapulmonary causes of
respiratory distress.
• Recognize the imaging features of extrapulmonary causes of
respiratory distress.
• Plan appropriate therapy for an infant with extrapulmonary causes
of respiratory distress.
References
1. Deprest J, Brady P, Nicolaides K, et al. Prenatal management
of the fetus with isolated congenital diaphragmatic hernia in
the era of the TOTAL trial. Semin Fetal Neonatal Med. 2014;19(6):
338–348
2. McGivern MR, Best KE, Rankin J, et al. Epidemiology of congenital
diaphragmatic hernia in Europe: a register-based study. Arch Dis
Child Fetal Neonatal Ed. 2015;100(2):F137–F144
3. Ackerman KG, Vargas SO, Wilson JA, Jennings RW, Kozakewich HP,
Pober BR. Congenital diaphragmatic defects: proposal for a new
classification based on observations in 234 patients. Pediatr Dev
Pathol. 2012;15(4):265–274
4. Bagłaj M, Dorobisz U. Late-presenting congenital diaphragmatic
hernia in children: a literature review. Pediatr Radiol.
2005;35(5):478–488
5. Scahill MD, Maak P, Kunder C, Halamek LP. Anterolateral congenital
diaphragmatic hernia with omphalocele: A case report and literature
review. Am J Med Genet. 2013;161A:585–588.
6. Cigdem MK, Onen A, Otcu S, Okur H. Late presentation of
bochdalek-type congenital diaphragmatic hernia in children: a 23-
year experience at a single center. Surg Today. 2007;37(8):
642–645
7. Kitano Y, Lally KP, Lally PA; Congenital Diaphragmatic Hernia Study
Group. Late-presenting congenital diaphragmatic hernia. J Pediatr
Surg. 2005;40(12):1839–1843
8. Bebbington M, Victoria T, Danzer E, et al. Comparison of ultrasound
and magnetic resonance imaging parameters in predicting survival
in isolated left-sided congenital diaphragmatic hernia. Ultrasound
Obstet Gynecol. 2014;43(6):670–674
Vol. 20 No. 9 S E P T E M B E R 2 0 1 9 e529
 Case 3: Late Preterm Infant with Respiratory Distress
Michael Kim, Yassar Arain and William Rhine
NeoReviews 2019;20;e527
DOI: 10.1542/neo.20-9-e527

Updated Information & including high resolution figures, can be found at:
Services http://neoreviews.aappublications.org/content/20/9/e527
References This article cites 8 articles, 1 of which you can access for free at:
http://neoreviews.aappublications.org/content/20/9/e527.full#ref-list-
1
Subspecialty Collections This article, along with others on similar topics, appears in the
following collection(s):
Pediatric Drug Labeling Update
http://classic.neoreviews.aappublications.org/cgi/collection/pediatric
_drug_labeling_update
Permissions & Licensing Information about reproducing this article in parts (figures, tables) or
in its entirety can be found online at:
https://shop.aap.org/licensing-permissions/
Reprints Information about ordering reprints can be found online:
http://classic.neoreviews.aappublications.org/content/reprints

Downloaded from http://neoreviews.aappublications.org/ at Indonesia:AAP Sponsored on April 17, 2021

 Case 3: Late Preterm Infant with Respiratory Distress
Michael Kim, Yassar Arain and William Rhine
NeoReviews 2019;20;e527
DOI: 10.1542/neo.20-9-e527

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://neoreviews.aappublications.org/content/20/9/e527

Neoreviews is the official journal of the American Academy of Pediatrics. A monthly publication,
it has been published continuously since 2000. Neoreviews is owned, published, and trademarked
by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village,
Illinois, 60007. Copyright © 2019 by the American Academy of Pediatrics. All rights reserved.
Online ISSN: 1526-9906.

Downloaded from http://neoreviews.aappublications.org/ at Indonesia:AAP Sponsored on April 17, 2021