Chapter 39

1. In infants with Bochdalek type congenital diaphragmatic hernia the severity of pulmonary hypoplasia and the resultant pulmonary hypertension are key determinants of survival. Barotrauma and hypoxia should be avoided.

2. During initial management of an infant with esophageal atresia and distal tracheoesophageal fistula, every effort should be made to avoid distending the gastrointestinal tract, especially when using mechanical ventilation. The patient should be evaluated for components of the VACTERL (vertebral, anorectal, cardiac, tracheoesophageal, renal, limb) anomalies. Timing and extent of surgery is dictated by the stability of the patient.

3. Although malrotation with midgut volvulus occurs most commonly within the first few weeks of life, it should always be considered in the differential diagnosis in a child with bilious emesis. Volvulus is a surgical emergency; therefore in a critically ill child, prompt surgical intervention should not be delayed for any reason.

4. When evaluating a newborn infant for vomiting, it is critical to distinguish between proximal and distal causes of intestinal obstruction utilizing both prenatal and postnatal history, physical examination and abdominal radiographs.

5. Risk factors for necrotizing enterocolitis (NEC) include prematurity, formula feeding, bacterial infection, and intestinal ischemia. Critical to the management of infants with advanced (Bell stage III) or perforated NEC is timely and adequate source control of peritoneal contamination. Early sequelae of NEC include perforation, sepsis, and death. Later sequelae include short-bowel syndrome and stricture.

6. In patients with intestinal obstruction secondary to Hirschsprungâs disease a leveling ostomy or endorectal pull through should be performed using ganglionated bowel, proximal to the transition zone between ganglionic and aganglionic intestine.

7. Prognosis of infants with biliary atresia is directly related to age at diagnosis and timing of portoenterostomy. Infants with advanced age at the time of diagnosis or infants who fail to demonstrate evidence of bile drainage after portoenterostomy usually require liver transplantation.

8. Infants with omphaloceles have greater associated morbidity and mortality than infants with gastroschisis due to a higher incidence of congenital anomalies and pulmonary hypoplasia. Gastroschisis can be associated with intestinal atresia, but not with other congenital anomalies. An intact omphalocele can be repaired electively, while gastroschisis requires urgent intervention to protect the exposed intestine.

9. Prognosis for children with Wilmsâ tumor is defined by the stage of disease at the time of diagnosis and the histologic type (favorable vs. unfavorable). Preoperative chemotherapy is indicated for bilateral involvement, a solitary kidney, or tumor in the inferior vena cava above the hepatic veins. Gross tumor rupture during surgery automatically changes the stage to 3 (at a minimum).

10. Injury is the leading cause of death in children older than 1 year of age. Blunt mechanisms account for the majority of pediatric injuries. The central nervous system is the most commonly injured organ system and the leading cause of death in injured children.

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