A Study of Total Colonic Aganglionosis.

INTRODUCTION : Intestinal Aganglionosis extending from anus to small bowel is called Total Colonic Aganglionosis (TCA) which may be present with or without small bowel involvement. It is an uncommon form, occurring in 5 to 15 % of all patients with Hirschsprung’s Disease. Though this subgroup has difficulty in diagnosis and treatment, nowadays there is an increase in the reported incidence of Total Colonic Aganglionosis because of greater awareness of this entity, which has led on to improved survival by afforded definitive corrective surgery. Though the Total Colonic Aganglionosis considered to be exceedingly rare, they are important subset because of the associated increased morbidity and mortality. AIM : Aim of the study is to review our experience in the management of 19 patients of Total Colonic Aganglionosis over 7 years (July 1998 to June 2005) MATERIALS AND METHODS : Retrospective review of 302 cases of Hirschsprung’s Disease treated at the Institute of Child Health and Hospital for Children, Egmore, Chennai from July 1998 to June 2005. Showed 19 patients with Total Colonic Aganglionosis. The medical records of the patients were analyzed with emphasis on age & sex incidence, presentation, type of operative procedures, complications of disease and therapy and follow up. CONCLUSION : TCA is a severe form of HD, most commonly recognized in the Neonatal period. Male predominance was seen in this study. Martin’s modification of Duhamel procedure results are better. Functional outcome were good in cases who underwent definitive procedure. Kimura Procedure was not done in this study. Most of the cases in this study presented with bilious vomiting, Abdominal distension, Altered meconium history. Only 4 cases were diagnosed as Total Colonic Aganglionosis by Barium Enema Investigation. Acetyl choline sterase study was not done in any patient in this study. Manometric studies performed in two cases, in which only one case established the diagnosis, and the procedure was not repeated in any of the cases after definitive procedure. According to literature type III colonic atresia is more common than any other type though the presentation is very rare. In this study there was one case of type I atresia seen near the left end of transverse colon, which was associated with Total Colonic Aganglionosis. This was treated by Soave pullthrough and the child is alive and coming for follow up. There were no case of Down syndrome or genitourinary anomalies seen associated with Total Colonic Aganglionosis in this study. In no case Total Parentral Nutrition (TPN) was used in this study. In this study no case presented with a family history of HD or TCA. Child with Total Colonic Aganglionosis admitted at 8 hours after birth was the youngest to present and 15 hours old was the earliest time at which surgery was performed in this study. The best time for pullthrough procedure would be around 6 months of age according to literature. In our Institute we have done pullthrough at the age of 3 months. Almost all the operated TCA children had poor weight gain. Total Colonic Aganglionosis associated with perforation of bowel near the proximal end of aganglionic segment were seen in two cases. As mentioned in the literature child presenting with perforation even at the time of birth was also seen in this study. Constipation- the postoperative complication of Soave pullthrough was relieved rather by posterior sphincterotomy than anal dilatation. The procedure in this study coincides with Elsen hamme (1974) description (he suggested immediate myectomy instead of sphincter dilatation as it causes laceration and fibrosis). Nearly one third of the TCA treated child (Ileostomy) has lost for follow up in this study. Mortality was seen in TCA with associated anomaly and in preterm baby with a complication of ileal perforation and peritonitis, otherwise the survival in TCA is good in operated cases. Morality rate of Total Colonic Aganglionosis in this study is similar to that of literature report