Abstract

A 4-week-old female infant is referred to the pediatric surgery department with abdominal distention. Antenatal ultrasonography had showed dilated bowel loops and mild polyhydramnios (amniotic fluid index 24) at 22 weeks of gestation. The neonate was born via vaginal delivery at 34 weeks of gestation with a birthweight of 2.5 kg. She had been feeding well since the time of birth, and passed meconium within 24 hours of birth. She developed abdominal distention after 2 weeks of age. She has no history of vomiting, and has passed small amounts of stool every day. On examination, the infant weighs 2.2 kg. She is dehydrated, with mild facial dysmorphism in the form of triangular facies and a prominent forehead. Her vital parameters are stable. The abdomen is distended. In view of the abdominal distention, the infant is given nothing by mouth and a nasogastric tube inserted. Her urine output is 3 to 4 mL/kg per hour. Abdominal radiography shows grossly dilated bowel loops (Figs 1 and 2). Investigations show hypokalemic hypochloremic metabolic alkalosis with severe hypokalemia and hyponatremia (pH 7.56; Pco2 48 mm …

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