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

Abstract

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.