Operative management of a newborn with the chest X-ray shown in Fig. below
The diagnosis of congenital diaphragmatic hernia (CDH) is made by chest X-ray, with the vast majority of infants developing immediate respiratory distress and pulmonary hypertension. CDH care has improved considerably through effective use of improved methods of ventilation and timely cannulation for extracorporeal membrane oxygenation. In the past, correction of the hernia was believed to be a surgical emergency, and patients underwent surgery shortly after birth. It is now accepted that the presence of persistent pulmonary hypertension that results in right-to-left shunting across the patent foramen ovale or the ductus arteriosus and the degree of pulmonary hypoplasia are the leading causes of cardiorespiratory insufficiency. Current management therefore is directed toward managing the pulmonary hypertension, which is usually seen within 7 to 10 days, but in some infants, may take up to several weeks.
Which of the following is most consistent with pyloric stenosis?
Infants with hypertrophic pyloric stenosis (HPS) develop a hypochloremic, hypokalemic metabolic alkalosis. The urine pH level is high initially, but eventually drops because hydrogen ions are preferentially exchanged for sodium ions in the distal tubule of the kidney as the hypochloremia becomes severe (paradoxical aciduria). The diagnosis of pyloric stenosis usually can be made on physical examination by palpation of the typical "olive" in the right upper quadrant and the presence of visible gastric waves on the abdomen. When the olive cannot be palpated, ultrasound (US) can diagnose the condition accurately in 95% of patients. Criteria for US diagnosis include a channel length of over 16 mm and pyloric thickness over 4 mm.
An infant presents to the emergency room with bilious emesis and irritability. Physical examination is notable for abdominal tenderness and erythema of the abdominal wall. Abdominal X-ray demonstrates dilated proximal bowel with air-fluid levels. What is the most appropriate next step in management, after resuscitation?
The cardinal symptom of intestinal obstruction in the newborn is bilious emesis. Prompt recognition and treatment of neonatal intestinal obstruction can truly be lifesaving. Bilious vomiting is usually the first sign of volvulus, and all infants with bilious vomiting must be evaluated rapidly to ensure that they do not have intestinal malrotation with volvulus. The child with irritability and bilious emesis should raise particular suspicions for this diagnosis. If left untreated, vascular compromise of the midgut initially causes bloody stools, but eventually results in circulatory collapse. Additional clues to the presence of advanced ischemia of the intestine include erythema and edema of the abdominal wall, which progresses to shock and death. It must be re-emphasized that the index of suspicion for this condition must be high, since abdominal signs are minimal in the early stages. Abdominal films show a paucity of gas throughout the intestine with a few scattered air-fluid levels. When these findings are present, the patient should undergo immediate fluid resuscitation to ensure adequate perfusion and urine output followed by prompt exploratory laparotomy.
Which of the following statements regarding Hirschsprung disease is FALSE?
Hirschsprung disease is characterized by the absence of ganglion cells in Auerbach plexus and hypertrophy of associated nerve trunks. It is thought to result from a defect in the migration of neural crest cells, which migrate from cephalad to caudad. In children who do not respond to nonoperative treatment, a decompressive stoma is required. It is important to ensure that this stoma is placed in ganglion-containing bowel, which must be confirmed by frozen section at the time of stoma creation. The hypertrophied, dilated portion of the intestine in Hirschsprung disease contains normal ganglion cells, and it is in the narrow segment of the colon distal to the dilated portion that ganglion cells are absent.
The most common form of esophageal atresia (EA) is:
The five major varieties of esophageal atresia (EA) and tracheoesophageal fistula (TEF) are shown in Fig. below. The most commonly seen variety is EA with distal TEF (type C), which occurs in approximately 85% of the cases in most series. The next most frequent type is pure EA (type A), occurring in 8 to 10% of patients, followed by TEF without EA (type E). This occurs in 8% of cases and is also referred to as an H-type fistula, based on the anatomic similarity to that letter (Fig. below). EA with fistula between both proximal and distal ends of the esophagus and trachea (type D) is seen in approximately 2% of cases, and type B, EA with TEF between proximal esophagus and trachea, is seen in approximately 1% of all cases.
The five varieties of esophageal atresia and tracheoesophageal fistula. A. Isolated esophageal atresia. B. Esophageal atresia with tracheoesophageal fistula between proximal segment of esophagus and trachea. C. Esophageal atresia with tracheoesophageal fistula between dista l esophag us and trachea. D. Esophageal atresia with fistula between both proximal and distal ends of esophagus and trachea. E. Tracheoesophageal fistula without esophageal atresia (H-type fistula).
Barium esophagram showing H-type tracheoesophageal fistula (arrow).
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