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Abdominal Wall Defects
• Omphalocele - central defect in umbilicus, covered by a membrane
• Gastroschisis - cleft in abdominal wall to right of umbilicus. Not protected by membrane. External loops of bowel are thickened, covered by a fibrinous peel
Abdominal Wall Defects Interventions
• Protection and support of viscera are most important.
• Nasogastric tube for decompression of bowel• Thermal regulation• Fluids and electrolytes• Prevention of infection - prophylactic antibiotics• Surgical interventions
Abdominal Wall Defects Problems
• Associated defects– Trisomy 13 or 18– Urinary tract abnormalities– Beckwith-Wiedemann syndrome: includes macrosomia,
macroglossia, omphalocele, and hypoglycemia– Congenital heart defects– Pentalogy of Cantrell: omphalocele, as well as defects
in diaphragm, sternum, heart, and pericardium
Abdominal Wall Defects Problems
• Reduced abdominal cavity• Malrotation of bowel (omphalocele)• Bowel atresias,strictures, adhesions,
stenoses (gastroschisis)• Difficulty in ventilation when bowel is
compressed surgically into abdomen
Congenital Diaphragmatic Hernia
• Occurrence: 1 in 3000 births• Description: displacement of abdominal
contents through diaphragm into thoracic cavity - most often left-sided
Congenital Diaphragmatic HerniaRecognition
• Respiratory distress• Scaphoid abdomen• Presence of bowel sound and/or absence of
breath sounds in all or portion of chest• Displaced heart sounds - away from
affected side - most often, dextrocardia
Congenital Diaphragmatic HerniaInterventions
• Do not bag-and-mask ventilate• Give 100% oxygen• Intubate if respiratory distress is profound• Ventilate with small tidal volumes/ minimize peak airway
pressures (which will tend to be high)• Watch for pneumothorax• Decompress stomach with orogastric tube (if possible)• Transport with affected side down• Surgical intervention
Congenital Diaphragmatic HerniaProblems
• Pulmonary hypoplasia• Pneumothorax/barotrauma• Persistent fetal circulation
Tracheo-Esophageal Fistula/ Esophageal Atresia
• Occurrence: 1 in 4500 births• Description: various interruptions in
esophagus and abnormal connections to the trachea
Tracheo-Esophageal Fistula/ Esophageal Atresia
• Esophageal atresia without fistula (5-7%)• Esophageal atresia with distal fistula (85%)• Esophageal atresia with proximal fistula• Esophageal atresia with proximal and distal
fistula• T-E fistula without esophageal atresia (H-
type) (5%)
Tracheo-Esophageal Fistula/ Esophageal Atresia
Recognition• Polyhydramnios• Excess salivation and drooling• Episodes of choking, gagging, and dyspnea, especially
with feeding• Crying or coughing leads to distended abdomen• Chest X-ray may reveal pneumonia, pneunonitis,
atelectasis, elevated diaphragm. Dilated esophageal pouch. Presence or absence of air in abdomen
• Inability to pass a large catheter into esophagus
Tracheo-Esophageal Fistula/ Esophageal Atresia
Interventions• Maintain in 30 degree, upright position to minimize
chances of gastric reflux• Insert nasogastric tube into esophageal pouch and suction
to remove excess, pooled secretions• Humidification, CPT, oxygen, and antibiotics may be
added in the treatment of aspiration pneumonitis• Feeding can be accomplished via gastrostomy tube when
surgical correction is delayed• Surgical intervention
Tracheo-Esophageal Fistula/ Esophageal Atresia
Problems• Cardiac (37%): most common (1) VSD, (2)
PDA, (3) Tetrology of Fallot• Gastrointestinal (21%)• VACTERL syndrome (7%): vertebral, anal,
cardiac, trachea, esophageal, renal, and limb anomalies
Choanal Atresia
Descriptions• Choanae: two openings in the posterior
portion of the nasal cavity that allow airflow from the nose to pharynx
• Choanal atresia: blockage of these openings from choanal stenosis, a bony septum, or membranous obstruction
Choanal AtresiaRecognition
• Newborns are “obligate nasal breathers” first two months of life– Respiratory distress - cyanosis and retractions
• resolves when the baby cries• worsens when the baby sucks
– Failure to pass a 6 Fr suction catheter through nares– Visualization of region by nasopharyngoscope– Unilateral choanal atresia may have less severe to
nonexistent respiratory distress, inspiratory stridor may be heard
Choanal AtresiaInterventions
• Placement of oral airway• Topical decongestant in case obstruction
caused by nasal edema rather than choanal atresia
Choanal AtresiaProblems
• 20 to 50% have assciated defects• CHARGE syndrome
– Colobomata of the eyes– Heart defects– Atresia of the choanae– Renal anomaly– Growth and mental retardation, gastresophageal reflux– Ear deficits
Pierre-Robin Syndrome
• Description: Glossoptosis and micrognathia. Tongue is large in comparison to mandible, reduced oropharynx. Often includes cleft palate. Tongue is more posterior and falls back in hypopharynx to cause airway obstruction.
• Recognition: reduced mandible. Mild-to-severe respiratory distress to complete obstruction