The diagnosis and treatment of anorectal malformation associated with Hirschsprung's disease

Abstract

Objective To analyze the clinical features of anorectal malformation (ARM) associated with Hirschsprung's disease(HD) in children. Methods The clinical data of 6 cases with ARM association with HD from January 2004 to April 2008 in this institute were retrospectively analyzed. The age of patients ranged from 8 months to 11 years with the mean age of 4. 1 years, and the gender ratio of males to females was 1:5. All patients still had symptoms of abdominal distention and constipation after anoplasty. Barium enema showed the existence of dilation of rectum in all cases, in which only 2 had a typical sign of spastic and transitional zones. Further anorectal manometries showed absence of anorectal inhibitory reflex in all cases. Four cases underwent the transabdominal Soave procedure, while the other 2 cases received the transanal Soave procedure. All patients were followed up for 2 months to 2 years. Results The aganglionosis was confirmed in the resected distal bowel by pathological diagnosis in all cases. Immunohistochemical staining for protease D in the proximal segments showed positive results while negative in the distal segments. All patients got excellent anal function. Conclusions In patients with ARM, especially in those who still had constipation after anoplasty, the possibility of associated with HD should be taken into consideration. The excellent results can be gained in those patients by Soave procedure. Key words: Anus, abnormalities; Rectum, abnormalities ; Hirschsprung disease