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Chapter 25. Pediatrics

A 6-week-old boy has a 6-day history of vomiting, intermittent fussiness, periods of inconsolability, and a 0.3-kg weight loss. On examination his anterior fontanelle is flattened and his mucous membranes are dry. Abdominal examination is unremarkable. Laboratory data are as follows: Na, 130; Ch, 87; CO2, 30. An ultrasound of the abdomen reveals a 3.5 mm × 12 mm pylorus. The next most appropriate step in management of this child would be:

A. Pyloromyotomy

B. Esopahgeal pH monitoring

C. Initiate proton pump inhibitor therapy

D. Order an immediate small bowel follow-through study

E. Begin IV fluid resuscitation

The age of this child’s presentation should exclude diagonoses of duodenal atresia (which typically present earlier) and the clinical presentation, as well as age, may exclude later presenting pathologies, such as intussusception. GERD may be suspected. However, in light of weight loss, dehydration, and electrolyte derangement, other diagnosis must be excluded. This child presents with metabolic alkalosis and subclinical findings on ultrasound of pyloric stenosis (typically 4 mm × 14 mm pylorus effectively clenches the diagnosis). The first goal should be correction of the electrolytes and close monitoring.

A 32-week gestational male presents with persistent cough when feeding and spits up after every feed. An abdominal radiograph reveals gasless stomach. Tracheoesophageal fistula is suspected. Appropriate workup should include:

A. Ultrasound of the back

B. Ultrasound of the kidneys

C. Echocardiogram

D. Rectal biopsy

E. Chromosomal analysis

TEF can be seen in the VACTERL association and additional anomalies should be ruled out.

Vertebral anomalies: Defects of the spinal column usually consist of hypoplastic vertebrae or hemivertebra. 70% of patients with VACTERL association will have vertebral anomalies.

Imperforate Anus: Anal atresia or imperforate anus is seen in about 55% of patients with VACTERL association.

Cardiac defects: Up to 75% of patients with VACTERL association have been reported to have congenital heart disease. The most common heart defects seen with VACTERL association are ventricular septal defect, atrial septal defects, and tetralogy of Fallot.

Tracheo-Esophageal fistula: EA with TEF is seen in about 70% of patients with VACTERL association, although it can frequently occur as an isolated defect.

Renal anomalies: Renal defects are seen in approximately 50% of patients with VACTERL association. In addition, up to 35% of patients with VACTERL association have a single umbilical artery. These defects can be severe ...

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