Ano-rectal anomaly associated with Hirschsprung's disease

ABSTRACT

Two hundred and eighty-one patients with ano-rectal malformation [ARM] were treated between September 1983 and August 1994. The criteria for consideration of the patient as a probable case of Hirschsprung's disease [HD] in the absence of anal stenosis were 1] constipation unresponsive to medical therapy; 2] anastomotic leakage following closure of colostomy after ano-rectoplasty. Posterior ano-rectal myectomy [PARM] was carried out as a diagnostic step. If the patient was still symptomatic and the myectomy specimens showed histologic signs of HD, a definitive procedure was performed. Eighteen patients [6.4%] proved to have HD. No instance of HD was observed in high type malformations [28 patients] and cloacal anomalies [3]. However, 13 of 123 patients with low ARM [10.5%] and 5 of 127 patients with intermediate anomalies [3.93%] had HD [P< 0.05]. FARM was therapeutic in the 5 patients with ultrashort HD, all of whom had low ARM. The remaining 13 patients [8 low, 5 intermediate] had to undergo a definitive procedure. In conclusion: 1. association of HD with ARM, especially low types, is not uncommon. 2. biopsy of the rectal wall is recommended at the time of correction of low and intermediate ARM. 3. PARM may be therapeutic in HD, especially if associated with low type anomalies