Review of Various surgical Procedures in the Management of Hirschsprung’s Disease

Ranjith Kumar, S (2010) Review of Various surgical Procedures in the Management of Hirschsprung’s Disease. Masters thesis, Madurai Medical College, Madurai.


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INTRODUCTION: Harold Hirschsprung – Senior paediatrician at Queen Louise children hospital in Copenhagen in 1886 presented his classic description of disease equity that bears his name to the Paediatric congress held at Berlin who described about two children who had classical and anatomical characteristics of the disease and died at the age of 8 and 11 months respectively due to repeated entero colitis. Hirschsprung’s disease of the colon was first reported in 1888 as an “idiopathic dilatation and hypertrophy of large bowel”. It was only in 1948 that Swenson and Bill identified that it was actually the constricted segment of colon which was at fault because of its inability to engage in peristaltic activity due to absence of ganglion cells in the myentric plexuses. AIM OF THE STUDY: To analyze the results by a prospective study of Hirschsprung’s Surgery done in the Department of Paediatric Surgery from August 2007 to July 2010. OBJECTIVES: 1) Analyze the types of procedures done, and their outcome. 2) To study the results and complications. 3) To study the patients who have come for follow up regarding their toilet pattern and recrudescence of the disease. 4) To assess by personal examination minor problems which may not have been noted by their parents. CONCLUSION: Reviewing the cases in the yester years, prior to this study we find that there is a great deviation in the management. Three stage procedures are very occasional. Single stage procedures have come to stay in selected cases. While this should cause increased mortality or morbidity, this has not been the case and actually it has been the other way around. Proper selection of cases and good technique have yielded good results and no mortality. The reduction of staging and good results have reduced the workload of our unit substantially. Most of the cases have been treated conservatively during the Intestinal obstruction episode and taken up electively, after they improved. Frank intestinal Obstruction were submitted to Emergency Colostomy. Transition zone colostomy commits to a two stage procedure, versus Transverse colostomy which commits to a three stage procedure. The has been a definite discernible difference of shift in favour of Transition zone colostomy as seen by Table 2 and the chart below with no discernible increase in mortality or morbidity. Due to down staging most of the definitive procedures have been completed by three years of age as evidenced by the Table: 3. This leaves the child free of his disease before the school going period, Regarding the choice of procedures no strict protocols were observed. Short segment disease was committed to POOP. Long segment disease was evaluated on a patient to patient basis and the appropriate procedure chosen. This was left to the Surgeon’s choice and discretion. But, for minor discrepancies there was not much of difference between the procedures, but the following points were noted. POOP left us with a higher incidence of recrudescence which was managed conservatively by dilatation and myotomies, successfully. Hence we reserved it for the less florid cases. Duhamel is technically the easiest, and was the procedure of choice in complicated cases, Soave gave us very good results as a single stage procedure in the neonates. Swenson was technically more demanding but with practically no recrudescence of symptoms, Voiding dysfunctions were not seen but complications of anastomotic strictures presented which were treated conservatively with dilatation and anoplasty. As with most operations, complications arising even years after surgery can be attributed to problems occurring during the operation itself. The majority of patients with Hirschsprung’s disease have a satisfactory or excellent long-term outcome after their pull-through procedure, provided the procedure is performed by a competent and well-trained paediatric surgeon. However, following even the best operation, problems may persist in many of these children. Therefore, close and long-term follow up of these children is necessary. Often, a conservative, non operative approach can lead to a successful outcome in most patients. As concluded in the last edition of the text by Orvar Swenson: Resection of the aganglionic colon … is a difficult operation. Yet, if a well-trained surgeon has an opportunity to observe the technical details of the operation and then perseveres, good results can be obtained.

Item Type: Thesis (Masters)
Uncontrolled Keywords: 1805 003 10
Subjects: MEDICAL > Paediatric Surgery
Depositing User: Subramani R
Date Deposited: 16 Feb 2020 02:32
Last Modified: 16 Feb 2020 02:32

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