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Watchful waiting
2%
1/47
Daily stool softeners and laxatives
4%
2/47
Colectomy
40%
19/47
Ileostomy
9%
4/47
Ileostomy with appendectomy
43%
20/47
Select Answer to see Preferred Response
The child in this vignette most likely suffers from Hirschsprung disease. When the entire colon is is involved, the treatment of choice is a diverting ileostomy with appendectomy for pathologic confirmation of absent neurons in the myenteric plexus. Embryologically the enteric neurons arise from neural crest cells and must travel from the caudal hindbrain to the gut. In Hirschsprung disease, these ganglion cells fail to migrate to the bowel, especially the colon. Important risk factors for the disease include Down syndrome, Chagas disease, Waardenburg's syndrome, and MEN 2. This condition should be suspected when newborns fail to pass meconium. On physical examination, there is an absence of stool in the rectal vault. Enterocolitis is the most common and feared side effect. Treatment is purely surgical and involves resection of the affected bowel segments and there is no medical management for the disease. McKeown et al. discuss Hirschsprung disease, which is a developmental disorder of the enteric nervous system characterized by an absence of the enteric neurons in the distal bowel. Because of a lack of colonic peristalsis, infants with this disorder suffer from severe constipation. The only treatment is surgical removal of the affected bowel. Males are affected 4 times as much as females, but the disease is still rare (incidence of 1 in 5,000 births). Kessmann discusses the diagnosis and management of Hirschsprung disease. Symptoms may include poor feeding, poor weight gain, and progressive abdominal distention. Complications of the disease include enterocolitis and colonic rupture. The disease most commonly affects the rectosigmoid region of the colon, but may affect the entire colon and rarely the small bowel. If the entire colon is not involved (as in the vignette), diagnosis involves rectal suction biopsy showing an absence of ganglion cells in the colonic submucosa. Incorrect Answers: Answers 1-3: These are not appropriate treatments for the patient at this stage. Answer 4: While Ileostomy is required, the appendix should be removed for pathologic confirmation.
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