Posterior myotomy/myectomy for persistent stooling problems in Hirschsprung's disease

Hirschsprung's disease (HD) patients after pull-through (PT) may have recalcitrant constipation or recurrent enterocolitis (EC). Posterior myotomy/myectomy (POMM) are possible options for these problems. This study analyzed the outcome of POMM in HD patients post-PT.

Records of 348 HD patients were reviewed, and 32 were found to have undergone a POMM post-PT (1981 to 2002). Outcomes after this procedure were assessed. Statistics used linear and logistic regression.

Of the 32 patients, 29 had complete records for analysis. Of those with pure constipation (12), 6 had aganglionosis post-PT. Of those with recurrent EC (17) only 1 had aganglionosis post-PT. POMM was performed at a mean of 3.1 years post-PT (11 myotomy, 18 myectomy). Average follow-up was 8.6 years (range, 0.7 to 21). Type of POMM had no correlation with overall functional outcome (P =.44). Of those with chronic constipation, 60% had good results after POMM; the remainder required a redo-PT or colostomy. Interestingly, most patients with retained aganglionosis and chronic constipation did not respond after POMM (83%). Of those with recurrent EC, 75% became free of symptoms; none of the patients not responding have required redo-PT.

POMM to treat chronic constipation or recurrent EC in patients with HD post-PT is moderately successful. Because of the unsuccessful outcome with POMM in patients with a combination of constipation and aganglionosis, one should defer to a redo-PT in this group.