Anorectal malformations ? results of treatment and long-term follow-up in 208 patients

Abstract

During a 20-year period (1964–1983) 208 patients with anorectal malformations (143 males, 65 females) were treated; 98 had low and 110 had high or intermediate malformations. Associated anomalies were detected in 67.8%. The overall mortality in the series was 14.4%; most deaths were related to associated anomalies and/or prematurity. Four different procedures were used for high and intermediate anomalies: (1) abdominoperineal (18 cases); (2) sacroabdominoperineal (38); (3) sacroperineal (8); and (4) posterior sagittal anorectoplasty (7). Neonatal anoplasty was used to correct male low anomalies. Female low and intermediate anomalies with vestibular fistula were treated with anal transposition. More than one-half of the patients with high or intermediate anomalies had complications related to surgical therapy. Major secondary surgery was performed in 41 cases (12 resections of megarectum, 29 late sphincter reconstructions). Of the living patients, 90% (159/178) were followed up for 5–25 years. A majority (93%) of the patients with low anomalies had a good outcome at the last follow-up examination. In patients with high and intermediate anomalies continence improved with time; the greatest improvement occurred after 10 years of age. At the last follow-up examination 73% of the patients with high or intermediate anomalies had acceptable social continence. Manometric evaluation revealed a rectoanal inhibitory reflex in all assessed patients with a low anomaly, indicating a functioning internal sphincter. In patients with intermediate and high anomalies a rectoanal inhibitory reflex was found only when full-thickness terminal bowel had been used in the reconstruction (by perineal and posterior sagittal repair); presence of the reflex correlated with superior fecal control. Acceptable fecal continence may be achieved in the majority of patients with anorectal anomalies. Operative methods that optimally utilize the voluntary sphincters and preserve the potential internal sphincter in the terminal rectal pouch seem to facilitate earlier development of fecal continence.