A bstract Rectal atresia (RA) with a normal anus is a rare anomaly mostly described as part of a series of anorectal malformations. Most authors believe it to be an acquired lesion with a vascular genesis. One of the arguments quoted is the lack of other congenital anomalies. Several operative procedures are recommended for this lesion. We describe four patients with RA who had other significant congenital anomalies; two other cases were found in the literature. A lack of other congenital anomalies in patients with RA does not seem to be a strong argument for an acquired lesion. All four patients were treated by a posterior sagittal approach with good functional results.
The ACE reaction in superficial rectal biopsies is a valuable diagnostic procedure for Hirschsprung's disease. Although the literature mentions a reliability as high as 100 percent accuracy, a false negative ACE reaction can occur. So the result of the histochemical examination can never be seen apart from the clinical picture. This paper deals with false-negative ACE reactions in 3 children. A false-negative ACE reaction is suggested to be caused by a developmental disturbance in the 6th or 7th embryonal week. Since this disturbance can be responsible also for an aganglionosis of the total colon and a part of the ileum, the chance of a false negative ACE reaction is postulated to be increased in an extensive aganglionosis in precisely those cases which are already a diagnostic challenge for the clinician.
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