H-TYPE ANORECTAL-MALFORMATIONS - INCIDENCE AND CLINICAL CHARACTERISTICS

To establish the incidence of H-type anorectal malformations (ARM), th e hospital records of 629 patients with ARM were studied. Patients who had a congenital rectourogenital connection and an external anal open ing in a normal or ectopic position were considered to have an H-type malformation. Twenty (3.2%; 14 females, 6 males) of the 629 patients s tudied had an H-type ARM. Major associated anomalies were found in 60% of these patients. Thirteen patients (12 females, 1 male) had a norma lly placed anus; the H fistula was low rectovestibular in 10, rectovag inal in 2, and rectourethral in 1. Seven patients had an ectopic anal opening. Three males had a rectourethral H fistula; in two of these th ere was a double fistula. Two males had a rectovesical H fistula. Of t he two females with ectopic anal openings, one had a high rectovaginal H fistula and the other had a low fistula. In only one case was the d iagnosis of H fistula made immediately after birth. Three patients die d of severe cardiac abnormalities during the neonatal period or early infancy. Primary perineal repair or a limited posterior surgical anore ctoplasty (PSARP) was used in 15 cases; formal PSARP was used for the other two. Four patients had between one and 4 recurrences; all but on e of these fistulas were repaired subsequently. Long term (median foll ow-up period, 12 years; range, 4 to 38 years), 12 patients had good bo wel function and no faecal soiling; four of these had constipation tha t was manageable with laxatives. Two patients (aged 10 and 12 years) h ad daily soiling. Two others were too young to evaluate, and one (with severe mental retardation) has a permanent colostomy. (C) 1996 by W.B . Saunders Company