Abstract

Aim: Modification of myectomy/sphincteromyectomy could be useful in the surgical treatment for short-segment Hirschsprung’s disease (SSHD). This study analyzed the outcome of M/S in SSHD for demographic, clinical findings and results and follow-up after M/S.

Patients and Methods: From 1997 to 2009, 20 children were treated with M/S for SSHD. They were retrospectively screened on the basis of history, examination results, preoperative barium enema (BE) study, anorectal manometry and rectal biopsy findings and postop follow-up.

Results: The study group included 13 boys and 7 girls with a mean age of 36 months (1 month to 10 years). The most common symptom was chronic constipation without soiling (75 %). Others were abdominal distension and vomiting (20 %) and enterocolitis (5 %). Delayed meconium passage (>48 hours) was found in 8 (47 %) patients. Physical examination findings; rectal and/or abdominal fecaloma in 9 (45 %) patients, abdominal distention in 4 (20 %) patients, passage of gas and feces after rectal examination in 3 (15 %) patients. The remaining patients had normal physical examination. Preoperative BE showed rectal dilatation in 19 cases, while narrow segment was only detected in 10 patients (53 %) with low cone position. Barium retention, more than 24 hours, was found in 17 (85 %) patients. An anorectal manometry revealed lack of rectoanal inhibitory reflex in 15 (89 %) patients. Anal hypertonia was only found in 3 (17 %) patients. The diagnosis of SSHD was proven histopathologically (preoperative rectal biopsy in 10 (50 %) patients, others perioperative frozen section biopsy). A simple myectomy was performed in all patients except 3 cases who had anal hypertonia. In these cases were treated by sphincteromyectomy. The length of the rectal muscle strip ranged from 2 to 8 cm (mean: 4.1 cm). Distal end of the strips revealed aganglionosis in all cases but, normal ganglia were seen at the proximal end of the strip only in 13 (65 %) cases. Two of the remaining patients were treated with definitive surgery. Others underwent clinical observation. Average follow-up was 17 months. Laxative treatment was only required in 9 patients during 3 months. Remaining patients are symptom-free.

Conclusion: Myectomy is an effective and minimal invasive treatment for SSHD. If the patient has anal hypertonia, sphincteromyectomy can be performed. If the proximal end of the strip is aganglionic, definitive surgery can be delayed until persistent clinical findings develop.