STUDY OF OUTCOME AND COMPLICATIONS OF ANORECTAL MYECTOMY IN CHILDREN WITH ULTRASHORT SEGMENT HIRSCHSPRUNG’S DISEASE

摘要

The term ultra short is not clearly defined in ultrashort-segment Hirschsprung’s disease. The limited extent of the ultrashort-segment Hirschsprung’s disease allows for treatment with extended sphincteromyectomy. In anal sphincter achalasia, anal sphincter dilatation under general anesthesia may be sufficient to treat the condition; in cases with persistent constipation, sphincteromyectomy is indicated. Some investigators believe that the term ultrashort-segment Hirschsprung’s disease and anorectal achalasia are the same. Our study was performed to define the efficacy of transanal anorectal myectomy and digital dilation under general anesthesia in children with ultra short-segment Hirschsprung’s disease and internal anal sphincter achalasia. A total of 87 patients were included in our study. Among these, 15 cases (17.24%) were female and 72 (82.76%) were male. In 12 patients (13.79%), the muscle strip had normal ganglion cells in both distal and proximal ends (group A). In 10 patients (11.49%), there was not any ganglion cell in both distal and proximal ends of muscle strip (group B). In 65 patients (74.71%), there were normal ganglion cells in proximal end with no ganglion cell in distal end of the muscle strip (group C). There was no meaningful differences between group A, B and C in their outcome and partially or complete response to anorectal myectomy. We recommend the term “sluggish rectum” for these patients instead of ultrashort-segment Hirschsprung’s disease or internal anal sphincter achalasia that causes ambiguity in diagnosis and treatment of these cases.