[UNIT #3 SURGICAL DISORDERS IN CHILDHOOD THAT AFFECT

RESPIRATORY CARE]
[PROF. MARK KELWIN DAGUIAO, RTRP [03/14/23] LECTURE #3

1) Generalized Macroglossia
I. CHOANAL ATRESIA - Associated with other congenital disorders
II. MACROGLOSSIA - Beckwith-Wiedemann syndrome, inborn errors
III. MANDIBULAR HYPOPLASIA
IV. LARYNGEAL CLEFT
of metabolism, and hypothyroidism, each of
V. ESOPHAGEAL ATRESIA AND which may carry its own complicating medical
TRACHEOESOPHAGEAL ATRESIA issues.
VI. TRACHEOMALACIA
VII. MEDIASTINAL TUMORS 2) Relative Macroglossia
VIII. CONGENITAL DIAPHRAGMATIC HERNIA
IX. DIAPHRAGMATIC EVENTRATION
- Comparatively large tongue relative to
X. CONGENITAL PULMONARY AIRWAY abnormally small craniofacial structures.
MALFORMATION - Trisomy 21 and other disorders
XI. BRONCHOPULMONARY SEQUESTRATION
XII. CONGENITAL LOBAR EMPHYSEMA 3) Focal Macroglossia
XIII. BRONCHOGENIC CYST
XIV. PECTUS EXCAVATUM
- Arise from tumors of the tongue, including
XV. ASPHYXIATING THORACIC DEFICIENCY vascular or lymphatic malformations.
XVI. SCOLIOSIS AND KYPHOSOCOLIOSIS
XVII. GASTROESOPHAGEAL REFLUX SYMPTOMS
XVIII. ABDOMINAL COMPARTMENT SYNDROME ➢ Stridor speech impairment
XIX. OMPHALOCELE
➢ Feeding intolerance
XX. GASTROSCHISIS
➢ Drooling
I. CHOANAL ATRESIA TREATMENT AND MANAGEMENT
➢ Unilateral or bilateral obstruction of the posterior ➢ Oral airway placement to intubation
nasal apertures. ➢ Surgical intervention: tongue reduction and
➢ Possible causes: disordered neural crest cell tracheostomy
migration and abnormal persistence of membranes
or other structures.
III. MANDIBULAR HYPOPLASIA
➢ Most common cases: obstructive bony choanal
plate ➢ AKA micrognathia
➢ Minority cases: membranous obstruction ➢ Craniofacial abnormality resulting in a small jaw →
respiratory distress
TYPES ➢ Leads to relative macroglossia
1) Bilateral Choanal Atresia ➢ Accompanies other developmental anomalies:
➢ early severe respiratory distress or feeding difficulties - Pierre Robin Sequence → most common
➢ inserting in oral airway - Treacher Collins Syndrome
➢ Polysomnographic studies using oximetry
2) Unilateral Choanal Atresia - Hypoxia and carbon dioxide retention can occur
➢ Two-thirds of cases ➢ Normal respiration in prone positioning
➢ Chronic unilateral rhinorrhea without frank respiratory ➢ Normal feeding
distress
TREATMENT AND MANAGEMENT
ASSOCIATED DISEASE/SYNDROME ➢ Tracheostomy
➢ CHARGE syndrome → most common ➢ Surgical procedures
- Coloboma of the iris or choroid - Hold the tongue forward (tongue – lip adhesion)
- Congenital heart defects
- Atresia of choanae IV. LARYNGEAL CLEFT
- Retarded growth and development ➢ An abnormal opening between the larynx and the
- Genitourinary anomalies esophagus through which food and liquid can pass
- Ear and hearing anomalies through the larynx into the lungs.
➢ Craniofacial Defects ➢ Be suspected → endotracheal tube repeatedly falls
into the esophagus, despite visualized placement
TREATMENT between the cords, resulting in an inability to
➢ Resection of the posterior nasal septum, with or ventilate → ECMO
without stent placement.
➢ Standard folded ET tube is used to stent the TYPES
choanae in a U configuration. 1) TYPE 1
➢ Suction and saline are used to ensure patency in the - Mildest form of laryngeal cleft
stent. - Gap between the larynx and the esophagus is
➢ Prophylactic antibiotics and steroid drops. located above the vocal cords.
➢ Stent is removed when the infant is medically ready
2) TYPE II
II. MACROGLOSSIA - Laryngeal cleft extends into the lower cartilage
➢ Enlarged tongue of the voice box, below the vocal cords.
➢ Caused by pharyngeal obstruction
3) TYPE III
THREE SUBTYPES - Laryngeal cleft extends beyond the voice box
and into the trachea (windpipe)
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[PROF. MARK KELWIN DAGUIAO, RTRP [03/14/23] LECTURE #3

e) Type E – H-type tracheoesophageal fistula without

4) TYPE IV esophageal atresia → least common
- Most severe from laryngeal cleft
- Gap extends even further down into the RESPIRATORY DISTRESS MAY DEVELOP FROM:
windpipe, and it may go all the way to the ➢ Aspiration of secretions from the blind proximal
bottom of the trachea. pouch.
➢ Aspiration of gastric contents via the fistula.
SIGNS AND SYMPTOMS ➢ Loss of ventilation through the fistula.
Minor Cases:
➢ Cyanosis with feeding DIAGNOSIS
➢ Aspiration with recurrent pneumonia ➢ Radiographic evaluation: demonstrate a coiled
➢ Stridor orogastric tube
➢ Increased secretions ➢ Renal ultrasound
➢ Spinal ultrasound
ASSOCIATED DISEASES OR SYNDROMES ➢ Spinal plain film for completion of VACTERL workup.
➢ VACTERL Syndrome
- Vertebral defects, anal atresia, cardiac defects, TREATMENT AND MANAGEMENT
tracheoesophageal fistula, renal anomalies, and ➢ Mechanical ventilation (HFV)
limb abnormalities ➢ Surgical ligation of the fistula (tying it closed) and
➢ Pallister – Hall Syndrome reconnection of the interrupted esophagus
➢ CHARGE Syndrome
VI. TRACHEOMALACIA
DIAGNOSIS ➢ Excessive tracheal collapse from anterior
➢ Direct laryngoscopy cartilaginous abnormalities, posterior coaptation, or
➢ Bronchoscopy extrinsic compression from aberrant vasculature.
➢ Contrast study of the esophagus ➢ Commonly associated with esophageal atresia
➢ Mild form: chronic cough (barking), recurrent
TREATMENT AND MANAGEMENT infection and poor clearance of secretions.
➢ Mechanical Ventilation ➢ Severe form: stridor, respiratory distress, or acute
➢ Surgical Repair life-threatening events

V. ESOPHAGEAL ATRESIA AND DIAGNOSIS

TRACHEOESOPHAGEAL FISTULA ➢ Bronchoscopy while spontaneously breathing
➢ Collapse of >50% during expiration
Esophageal Atresia
➢ Birth defect in which part of a baby’s esophagus (the TREATMENT
tube that connects the mouth to the stomach) does ➢ Suspending the aorta (and thus the attached anterior
not develop properly wall of the trachea) by aortopexy and by posterior
tracheopexy.
Tracheoesophageal Fistula
➢ Abnormal connection in one or more places between VII. MEDIASTINAL TUMORS
the esophagus (the tube that leads from the throat to ➢ Compressing the airways and are unrecognized
the stomach) and the trachea (the tube that leads ➢ light sedation can result in obstruction and an airway
from the throat to the windpipe and lungs emergency
SIGNS AND SYMPTOMS TYPES
➢ Frothing and bubbling → nose and mouth 1) Anterior
➢ Choking - Behind the sternum, medial to the pleura and
➢ Drooling anterior to the pericardium, great vessels,
➢ Coughing episodes and trachea.
➢ Wheezing - Houses the thymus, as well as lymph nodes,
➢ Recurrent respiratory infections fatty tissue, and sometimes thyroid tissue.
➢ Abdominal distention → excessive air moving from - Half of mediastinal tumors arise in the anterior
the respiratory tract to GIT compartment and found to be lymphomas (B-
➢ Inability to pass an orogastric tube into the stomach Cell, T-Cell, Hodgkin), germ cell tumors, tumors
→ confirms the diagnosis or cysts of the thymus, or thyroid goiters.
2) Middle
TYPES - Beneath the anterior compartment and
a) Type A – pure esophageal atresia anterior to the anterior border of the spine
b) Type B – esophageal atresia with proximal - Heart and great vessels are contained here as
tracheoesophageal fistula well as the trachea, major bronchi, and hilar
c) Type C – esophageal atresia with distal lymph nodes.
tracheoesophageal fistula → most common - Commonly lymphomas (Hodgkin and Non-
d) Type D – esophageal atresia with proximal and distal Hodgkin) bronchogenic cysts, pericardial
tracheoesophageal fistula cysts, germ cell tumors, or vascular anomalies.

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[PROF. MARK KELWIN DAGUIAO, RTRP [03/14/23] LECTURE #3

➢ Inhaled Nitric Oxide → PPHN

3) Posterior
- Contains the esophagus, aorta, and IX. DIAPHRAGMATIC HERNIA
sympathetic chain and lymphatics.
➢ Abnormal intrusion of the diaphragm into the
- Neuroblastoma, ganglioneuroma, and foregut
thoracic space on one or both sides
cysts.
➢ Congenital cases: normal musculature is either
thinned or completely absent, resulting in doming
SIGNS AND SYMPTOMS
of the diaphragm
➢ Dyspnea
➢ Acquired eventration: phrenic nerve injury during
➢ Stridor
cardiothoracic surgery or birth trauma
➢ Wheezing
DIAGNOSIS
VIII. CONGENITAL DIAPHRAGMATIC HERNIA ➢ Ultrasound or Fluoroscopy
➢ Diaphragm, the muscle that separates the chest from - Demonstrating paradoxical movement of the
the abdomen, fails to close during prenatal affected side
development. ➢ Surgical Treatment: involves diaphragm plication
➢ Opening allows contents of the abdomen or resection, and repair is often in symptomatic
(stomach, intestines, and/or liver) to migrate into the congenital cases
chest, impacting the growth and development of the
lungs. X. CONGENITAL PULMONARY AIRWAY
➢ Pathophysiologic Mechanism: lung hypoplasia, MALFORMATION
decreased alveolar count and decreased pulmonary
➢ Cystic mass of abnormal lung tissue
vasculature, pulmonary hypertension, and unusual
➢ Overabundance of terminal respiratory bronchioles
anatomy of the inferior vena cava
→ abnormal alveoli
➢ Asymptomatic (rare) and severe life-threatening
disease (frequent)
TWO SUBTYPES
1) Macrocystic Lesions
TYPES
- Affect one lobe with a dominant cyst and smaller
1) Bochdalek Hernia
daughter cysts
- Most common
- Not true cysts
- Left side
- Communicate with airways
- Posterolateral defect
2) Microcystic Lesions
2) Morgagni Hernia
- More uniform in appearance and confer a worse
- Right side
overall prognosis, in part because of their
- Retrosternal or peristernal, or central
association with other developmental anomalies
- Less severe → liver can block the defect and
- Not true cysts
decrease the volume of abdominal contents that
- Communicate with the airways
can enter the thorax
SYMPTOMS
SIGNS AND SYMPTOMS
➢ Discovered prenatally → asymptomatic at birth
➢ Tachypnea
➢ Air trapping and compression of normal surrounding
➢ Scaphoid abdomen (because the abdominal
lung
contents are in the thorax)
➢ Large lesions → require intervention in utero
➢ Decreased breath sounds on the side of lesion
→ expanding lesion: associated with hydrops and
➢ Displaced heart sounds (because the heart is
fetalis and myocardiopathy → poor prognosis
pushed away from the hernia)
➢ Severe cyanosis (from lung hypoplasia and
TREATMENT
pulmonary hypertension)
➢ Resection of the lesion with a lobectomy →
thoracoscopically
DIAGNOSIS
➢ Prenatal Ultrasonography
➢ Physical Examination XI. BRONCHOPULMONARY SEQUESTRATION
➢ Chest Radiography → confirmatory → bowel in the ➢ Nonfunctioning lung tissue that lacks a bronchial
chest connection to the normal tracheobronchial tree
➢ Some cases be aerated through collateral
TREATMENT channels → pores of Kohn
➢ Resuscitation → delivery room → prevents hypoxia ➢ Most commonly located in the posterior basal
or significant hypercapnia and acidosis segment of the left lower lobe
➢ Large orogastric tube is placed to decompress the
stomach and gastrointestinal tract TYPES
➢ Mechanical Ventilation → cuffed endotracheal tube 1) Intralobar Sequestrations
➢ HFV (high-frequency oscillatory ventilation and high- - Venous drainage is usually into the pulmonary
frequency jet ventilation) veins
➢ ECMO - Have a systemic arterial blood supply
➢ Surgery → delayed for several days to allow the - Presentation typically occurs later in life with
natural decrease in PVR recurrent infection of an occult lesion

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 [UNIT #3 SURGICAL DISORDERS IN CHILDHOOD THAT AFFECT
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[PROF. MARK KELWIN DAGUIAO, RTRP
2) Extralobar Sequestrations
- Drainage is into systemic veins (azygos or
hemiazygos veins, inferior vena caba, or right
atrium)
- Have a systemic arterial blood supply
- Separate visceral pleural envelope and may
even lie within the abdomen
- Associated with other congenital anomalies:
cardiac disease, congenital diaphragmatic
hernia, and vertebral deformities
EARLY SYMPTOMS
➢ Related to vascular shunt through the sequestration
→ heart failure if severe
DIAGNOSIS
➢ Chest radiography: homogeneous dense lesion
➢ CT angiogram: diagnostic and define the vascular
anatomy for operative planning
TREATMENT
➢ Curative resection → symptomatic lesions as well as
patients with large lesions
➢ Elective resection → asymptomatic sequestrations
→ infectious risk
➢ Thoracoscopic resection
XII. CONGENITAL LOBAR EMPHYSEMA
➢ Overdistention of one or more lobes of the lung
most frequently the left upper lobe
➢ Air enters but cannot escape because of
obstruction of a segmental or lobar bronchus
➢ Due to weakness in the bronchial cartilage (a variant
of bronchomalacia), leading to air trapping
➢ Aberrant artery or vascular ring may alternatively
compress the bronchus during expiration
➢ Progressive lobar distension leads to
emphysematous changes of the parenchyma
➢ Severe cases: massive overdistention
→ prevent normal gas exchange in the surrounding
lung
→ cardiovascular collapse by obstructing systemic
venous return to the right atrium
➢ Treatment of symptomatic lesions → resection
➢ Severe cases: thoracotomy in the newborn →
release compression of surrounding structures by a
quickly expanding lesion
➢ Asymptomatic patients: nonoperative management
XIII. BRONCHOGENIC CYST
➢ Most common foregut duplication cyst
➢ Intrathoracic (bronchial wall, the pleura, the
mediastinum, or the lung parenchyma) or
Extrathoracic (trachea and bronchi)
➢ Lined by respiratory epithelium
➢ Lumen is not connected to the bronchial tree, and
the lesions are nonfunctional
➢ Related to other types of foregut cysts: esophageal
duplication cysts, gastric and duodenal duplication
cysts, and neurenteric cysts
SYMPTOMS
➢ Dyspnea
➢ Wheezing
➢ Recurrent pneumonia
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[03/14/23] LECTURE #3
DIAGNOSIS
➢ Plain Film Radiographs → circular or ovoid mass
with smoot edges
➢ CT Imaging → confirm the suspected diagnosis
➢ Magnetic Resonance Imaging → vertebral
anomalies
→ rule out neurenteric cyst (i.e., foregut cyst with
extension into the spinal canal), which would require
a multidisciplinary team, including neurosurgery, for
adequate treatment
TREATMENT
➢ Resection → thoracoscopically
XIV. PECTUS EXCAVATUM
➢ most common congenital disorder of the chest wall
➢ deformity of the cartilaginous ends of the ribs →
inward curvature of the sternum
➢ decreased AP diameter of the chest → lungs may
noy fully expand
➢ male-to-female ratio of 4:1
➢ Marfan Syndrome, Ehlers – Danlos Syndrome, and
Osteogenesis Imperfecta
➢ Pseudopectus Excavatum
- Neonates → chest wall retraction during
respiration as a result of the pliability of the
chest wall
- Disappears by 6 months of age
SYMPTOMS
➢ Infancy → asymptomatic
➢ Chest wall becomes more rigid and activity is
increased symptoms subsequently may develop
COMPLICATIONS
➢ Reduce ventricular filling and stroke volume
➢ Decreasing exercise tolerance
➢ Arrhythmias
TREATMENT
➢ Surgical repair (after 13to 14 years of age)
→ passing a retrosternal bar between the sternum
and heart to apply anterior pressure on the sternum
XV. ASPHYXIATING THORACIC DYSTROPHY
➢ Aka JEUNE SYNDROME
➢ Rate osteochondrodystrophy with an autosomal
recessive inheritance pattern
➢ Inherited disorder of bone growth characterized by a
narrow chest, short ribs, shortened bones in the
arms and legs, short stature, and extra fingers
and toes (polydactyl)
➢ Thoracic insufficiency syndrome
→ Chest cavity is decreased in both the
anteroposterior and superoinferior orientations
➢ Leads to pulmonary hypoplasia
➢ After birth the lungs are not able to fully expand
➢ Hypoplastic iliac wings, and fixed clavicles, heart,
renal, and airway abnormalities, subglottic stenosis
➢ Surgery: augmenting the volume of the thoracic
cavity for greater lung expansion
→ expansion thoracoplasty
→ anterior and posterior rib osteotomies
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➢ The spring – loaded portion is placed in the

XVI. SCOLIOSIS AND KYPHOSCOLIOSIS
➢ Decrease lung capacity and lead restrictive
pulmonary function
➢ Severe form, kyphoscoliosis may lead to secondary
chest wall deformities
➢ Treatment: bracing or surgery
XVII. GASTROESOPHAGEAL REFLUX
➢ Child with a history of reflux → considered for
aspiration precautions with elevation of the head of
the bed, right lateral decubitus positioning, acid
blockade, and/or decompression of the stomach to
avoid aspiration
➢ Respiratory symptoms exacerbated by GER:
reactive airway disease and chronic cough
➢ Passage of gastric contents into the esophagus
➢ Mild symptoms: feeding intolerance and feeding
irritability
➢ Reflux leading to aspiration → pneumonias and
chronic lung disease
abdominal cavity.
➢ Sequential tightening of the sac reduces the viscera
until they are contained within the abdominal cavity,
which then allows definitive closure of the abdominal
wall defect.
➢ Bianchi and colleagues
- reducing the abdominal contents without
anesthesia
- avoids the complications of endotracheal
intubation and mechanical ventilation
➢ Sandler et al.
- Sutureless closure
- Eviscerated contents are reduced until they can
be contained within the abdominal cavity, and
the defect is closed with the umbilical cord used
as a biological patch
- Adhesive dressing is then applied

XVIII. ABDOMINAL COMPARTMENT SYNDROME

➢ Intraabdominal pressure of >20 mmHg with
associated organ dysfunction
➢ Symptoms: acute kidney injury related to
proinflammatory cytokines, venous compression, and
local renal compression, as well as competition with
diaphragmatic excursion.
➢ Abdominal pressures: measured via the bladder
using a foley catheter transducer

TREATMENT
➢ Relieving intraabdominal hypertension by positioning
(flat and supine) gastric and rectal tube placement,
neuromuscular blockade, peritoneal drainage
catheter placement, or decompressive laparotomy

XIX. OMPHALOCELE
➢ Occurs at the umbilicus and the eviscerated
abdominal contents are covered by peritoneal sac
(although rupture may occur)
➢ Occurs early during fetal development, at the time of
organogenesis, and other defects are common

TREATMENT AND MANAGEMENT

➢ Large omphaloceles or associated with heart disease
→ include the liver, which should be handled with
care to prevent liver laceration or hepatic vein injury.
→ delayed repair or “paint and wait” approach is
commonly adopted.
→ sac is treated with silver sulfadiazine cream to
allow it to thicken, and repair is performed months to
years later, when it is safer and the necessary
abdominal domain has been gained.

XX. GASTROSCHISIS
➢ Herniation occurs to the right or the umbilicus
➢ Develops later in gestation
➢ Not covered by peritoneum
➢ Associated defects: intestinal atresia
➢ Infant should be placed in a sterile bowel bag to
prevent evaporative fluid loss and protect the bowel
➢ Too Distended Abdominal Contents: spring –
loaded silo is placed over the intestines
Trans: MABBORANG, MARIA PATRICIA LEI B. EDITOR: MABBORANG, MARIA PATRICIA LEI B. NEONATAL LAB