What is the initial management for a full‑term newborn with tachypnea and abdominal distention shortly after birth and a contrast enema showing a dilated proximal colon with a narrowed descending colon (transition zone of Hirschsprung disease)?

Initial Management of Suspected Hirschsprung Disease in a Newborn

The correct answer is D: Nasogastric decompression with supportive care is the initial management for this full-term newborn presenting with tachypnea, abdominal distention, and contrast enema findings consistent with Hirschsprung disease. 1, 2

Clinical Presentation and Diagnosis

This clinical scenario describes a classic presentation of Hirschsprung disease in a newborn:

• Full-term infant of diabetic mother presenting shortly after birth with tachypnea and abdominal distention 3
• Contrast enema findings showing proximal colonic dilation with narrowing of the descending colon represent the pathognomonic "transition zone" between the dilated normally innervated proximal bowel and the narrow aganglionic distal segment 1, 2
• The American College of Radiology confirms that contrast enema is the diagnostic imaging procedure of choice for suspected distal obstruction, with approximately 80% sensitivity for detecting the transition zone in Hirschsprung disease 2

Why Nasogastric Decompression with Supportive Care is Correct

Initial conservative management is the standard of care before definitive surgical intervention:

• Bowel decompression via nasogastric tube relieves the proximal obstruction and prevents further distention 1
• Supportive measures include intravenous fluid resuscitation, correction of electrolyte abnormalities, and bowel rest 4
• Stabilization before surgery is essential—the infant must be medically optimized before undergoing the definitive pull-through procedure 3, 5
• Rectal biopsy remains necessary to confirm the diagnosis histologically by demonstrating absence of ganglion cells, even with characteristic imaging findings 2, 3

Why Other Options Are Incorrect

A - Exchange Transfusion

• Not indicated for Hirschsprung disease [@general medical knowledge]
• Exchange transfusion is reserved for severe hyperbilirubinemia or polycythemia, neither of which is suggested by this presentation [@general medical knowledge]

B - Immediate Surgical Intervention

• Premature without stabilization—while surgery is the definitive treatment, immediate operation without initial decompression and stabilization increases perioperative risk 3, 5
• The infant requires nasogastric decompression, fluid resuscitation, and confirmation via rectal biopsy before proceeding to pull-through surgery 2, 3
• Surgical correction (Swenson, Soave, or Duhamel procedures) is performed after the patient is stabilized 3

C - Extended Antibiotics

• Not the primary initial management for uncomplicated Hirschsprung disease 4
• Antibiotics are indicated only if there are signs of enterocolitis (fever, bloody diarrhea, systemic toxicity) or peritonitis, which are not described in this presentation 3, 6
• The focus should be on mechanical decompression rather than antimicrobial therapy 1

Critical Management Pathway

The systematic approach includes:

1. Immediate nasogastric tube placement for gastric and intestinal decompression 1
2. Intravenous fluid resuscitation with isotonic crystalloids to correct dehydration and electrolyte imbalances 4
3. NPO status (bowel rest) until definitive management 4
4. Rectal biopsy to confirm absence of ganglion cells in the myenteric and submucosal plexuses 2, 3
5. Surgical consultation for planned pull-through procedure once the infant is stabilized 3, 5

Common Pitfalls to Avoid

• Do not rush to surgery without adequate decompression and stabilization—this increases the risk of complications including anastomotic leak and enterocolitis 3, 5
• Do not miss associated conditions—infants of diabetic mothers may have other congenital anomalies, and Hirschsprung disease is associated with trisomy 21, Mowat-Wilson syndrome, and other syndromes 3
• Do not delay rectal biopsy—while imaging is suggestive, histologic confirmation is the gold standard and necessary before definitive surgery 2, 3
• Monitor for enterocolitis—the most serious complication in the preoperative period, characterized by fever, bloody diarrhea, and abdominal distention requiring urgent intervention 3, 6