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Difficulty: Very hardPediatric Gastrointestinal Disorders

The neonatal intensive care unit team is called to assess a 36-hour-old full-term male who developed progressive abdominal distension and bilious emesis. He has not produced any meconium since birth. The infant was delivered vaginally at 39 weeks gestation to a 32-year-old mother whose pregnancy was complicated by poorly controlled gestational diabetes mellitus. His birth weight was 4.2 kg (9 lb 4 oz). Vital signs are within normal limits for age. Physical examination reveals a markedly distended and tympanitic abdomen without palpable masses, erythema, or tenderness. The anus is patent. An abdominal radiograph shows multiple dilated bowel loops with air-fluid levels and a paucity of distal gas. A water-soluble contrast enema is performed, revealing an abrupt transition in bowel caliber at the splenic flexure; the colon proximal to the splenic flexure is significantly dilated, while the descending colon and sigmoid colon are of uniformly small caliber. During the procedure, the infant passes a large meconium plug followed by copious amounts of stool and flatus, resulting in immediate relief of his abdominal distension. Which of the following is the most appropriate next step in the management of this patient?

  1. Continued observation and establishment of enteral feedingsAnswer
  2. B
    Performance of a rectal suction biopsy
  3. C
    Evaluation with a sweat chloride test
  4. D
    Emergent exploratory laparotomy and diverting colostomy
  5. E
    Initiation of intravenous broad-spectrum antibiotics

Answer

Continued observation and establishment of enteral feedings
The presentation of failure to pass meconium, bilious emesis, and a transition zone at the splenic flexure in a macrosomic infant of a diabetic mother is classic for Neonatal Small Left Colon Syndrome (NSLCS). This is a transient, functional colonic obstruction. The diagnostic water-soluble contrast enema is also therapeutic, clearing the meconium plug and relieving symptoms. Because the condition is self-limiting following successful decompression, the most appropriate management is observation and gradual initiation of enteral feedings.

Step-by-Step Solution

1
Analyze the maternal history and patient demographics
The patient is a macrosomic infant born to a mother with poorly controlled gestational diabetes.
Maternal diabetes is a major risk factor for Neonatal Small Left Colon Syndrome (NSLCS).
2
Evaluate the clinical presentation and diagnostic imaging
Failure to pass meconium, bilious emesis, and a contrast enema showing a transition zone specifically at the splenic flexure.
This triad of findings distinguishes NSLCS from Hirschsprung disease (which typically has a transition zone at the rectosigmoid junction) and Meconium Ileus (which shows a microcolon and obstruction in the terminal ileum).
3
Assess the response to the contrast enema
The infant passed a large meconium plug with immediate relief of abdominal distension.
Water-soluble contrast enemas are both diagnostic and therapeutic for NSLCS.
4
Determine the definitive management plan
Because NSLCS is a transient functional immaturity of the colon that resolves after decompression, no further invasive testing or surgery is required.
Observation and the introduction of enteral feedings is the standard of care following successful enema decompression in NSLCS.

Key Concept

Diagnosis and management of Neonatal Small Left Colon Syndrome
Estimated Time:1m 30s
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Topics

Pediatric Gastrointestinal Disorders

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