Journal of Indian Association of Pediatric Surgeons Medknow Publications on behalf of the Indian Association of Pediatric Surgeons ISSN: 0971-9261 EISSN: 1998-3891 Vol. 12, Num. 2, 2007, pp. 68-71

Journal of Indian Association of Pediatric Surgeons, Vol. 12, No. 2, April-June, 2007, pp. 68-71

Original Article

Hirschsprung's disease: 8 years experience in a Nigerian teaching hospital

Nasir AA, Adeniran JO, Abdur-Rahman LO

Paediatric Surgical Unit, Dept. of Surgery, University of Ilorin Teaching Hospital, Ilorin, Kwara State
Correspondence Address:P. O. Box 5708, Ilorin, Kwara draanasir@yahoo.com

Code Number: ip07025

Abstract

Keywords: Colostomy, Duhamel, enterocolitis, Hirschsprung's disease, Swenson

Introduction

Hirschsprung's disease (HD) is a congenital malformation characterized by the absence of ganglion cells in the distal bowel beginning at the internal anal sphincter and extending proximally for various lengths. ^[1] Patients may present at birth with delayed passage of meconium or later in life with constipation on and off and failure to thrive. The diagnosis is mainly by radiographic studies, anorectal manometry and histological examination of rectal wall biopsies. ^[2]

Curative surgery consists of total or subtotal resection of the aganglionic segment followed by a pull-through of the normally innervated intestine. Although HD accounts for the 3 ^rd commonest cause of intestinal obstruction in neonates in our unit, ^[3] no previous review had been done of cases seen in this hospital. The aim of this article is to look at the way we have managed these patients since the pediatric surgical unit was started in 1998, analyze the outcome of management including complications and to create a template for future research.

Materials and Methods

The medical records of all children managed for HD between January 1998 and December 2005 were retrospectively reviewed. The data collected included demographic data, clinical presentation, age at diagnosis, types of colostomy and their complications, intervals between colostomy and definitive operations, age at definitive operations, types of definite operations and their complications. Other data retrieved were time of colostomy closure and duration of follow-up. Patients were diagnosed either by limited barium enema or by a full-thickness biopsy of the posterior rectal wall or both.

Biopsy was always taken from the colostomy edge to rule out long-segment aganglionosis. Transverse loop colostomy was commonly done due to lack of facilities for frozen section for a transitional zone colostomy in our centre. One of the definitive operations done was the Swenson's pull through. ^[1] We have recently developed a new technique of hand anastomosis to complete the Duhamel operation without the use of the GI stapler. This consists of the usual retro-rectal pull-through with anastomosis of the native and new rectum above the dentate line in the pelvis. Long-stay sutures were left at 11 and one o'clock. An incision was then made along the new bowel at the antimesenteric border from the pelvic brim.

Another incision was made along the back of the native rectum. A long curved artery forceps was then passed into the incision in the native rectum out of the anal canal. This forceps was now hooked round the anastomosis at 12 o'clock and further pushed in until the point appeared through the lumen in the ganglionic bowel. The two layers of bowel between the forceps were incised. The forceps was now inserted to grab the long sutures at 11 and one o'clock in turn and delivered through the pelvis. Anastomosis between the edges of the native rectum and the neorectum was then done with interrupted polydioxanone sutures (PDS) inside and silk sutures outside.

Results

A total of 43 patients were admitted with clinical suspicion of HD over the eight year period stated above. Fifteen did not complete their investigations to confirm the diagnosis. Seven declined further treatment for fear of colostomy or financial reasons. Twenty-one patients treated for HD had complete notes available for analysis. One patient presented as a neonate with suspected high jejunal atresia. She had jejuno-jejunostomy but died in obstruction one week later. Histology of the resected specimen showed aganglionosis. All the other patients had short segment aganglionosis and were all males. Age at diagnosis ranges between four days and 11 years (mean = 22 months). Thirty-eight percent of the patients were symptomatic at the neonatal age and almost 80% of them were diagnosed before the end of the first year of the life [Table - 1].

Clinical presentation

Ten patients (47.6%) presented with delayed passage of meconium and all 21 had abdominal distension. Seven patients had diarrhea and six had enterocolitis [Table - 2].

Initial management

Twenty patients had diverting colostomy: 16 transverse loop, four sigmoid loop colostomies.

Definitive operation

Fifteen patients (71.4%) underwent definitive operations (nine Swenson's operations and six Duhamel's pull-through [Figure - 1]).

Final outcome

Eleven patients had three-stage procedures, two patients had two-stage Swenson's procedures, one patients had two-stage Duhamel pull-through operations. Two patients are awaiting definitive operations and one awaiting closure of colostomy [Table - 3].

Mean time from diagnosis to definitive surgery was five months (range = two months-one year). Mean age at the time of definitive operations was 30 months (range = five months-11 years) Mean time from definitive surgery to colostomy closure was 2.5 months (range = 2-5 months).

Complications before definitive operation

Colostomy-related complications recorded included sixteen patients with dermatitis, six had colostomy prolapse, one patient each had parastomal hernia, colostomy diarrhea and enterocolitis [Table - 4].

Complications after definitive operation

After definitive operations, three patients had wound infections, one had partial intestinal obstruction, two enterocolitis and one hemorrhage.

Complications after colostomy closure

After colostomy closure, two patients had wound infections, one patient had severe enterocolitis and perianal fistula but he did well on ileostomy which was closed four months later. Another patient had anastomotic stricture following Swenson's operation but he responded well to anal dilatation.

Follow-up

Follow-up period ranged from six weeks to eight years. The overall mortality in this study was 23.8% (five deaths). Two patients died of severe enterocolitis (one after colostomy, one after Swenson's operation). One patient died of sepsis after colostomy, one other died of sepsis and obstruction due to long-segment aganglionosis. The fifth patient died of reaction to analgesia following Duhamel's pull-through.

Discussion

More than 20 reports of congenital megacolon preceded Hirschsprung's classic description of this disease at the 1886 Pediatric Congress in Berlin. ^[4] Tittel and others ^[4] first noted absence of intramural ganglion cells in the distal rectum, suggesting a disturbance of peristalsis, which was correlated with intestinal obstruction in infancy by Robertson and Kernohan in 1938. ^[5] Twenty of our 21 patients were males. This agrees with other reports that this disease is predominant in males. Most of them presented with some major complication especially sepsis.

All the patients (100%) had abdominal distension at presentation. This is high compared to 64% recorded in another study. ^[6] Although more than 80% of patients with HD may be diagnosed in the neonatal period, ^[7] only 38% were diagnosed in the neonatal period in this study and 70% before the end of the first year. This is as a result of cultural practices and reduced awareness in this subregion. Many surgeons ^[4],[7] prefer an initial sigmoid colostomy to defunction the bowel instead of the transverse colostomy. The argument is that the defunctioned bowel is shorter, it produces fewer complications and allows easier washout of the distal segment. That has not been our experience. Children who had transverse colostomy did not fare worse than those who had sigmoid colostomy. This difference may not be apparent in the current study due to fewer numbers of sigmoid colostomies as compared to transverse loop colostomies.

The development of rectosigmoidectomy by Swenson and Bill in 1948 ^[1] made a major contribution to our understanding of HD. Traditionally, the patient undergoes a three-stage operation. An initial diverting colostomy is made and biopsies are taken from colostomy site to exclude long segment disease. At the second stage performed between three months and one year of age, the aganglionic segment is removed and the normally innervated colon is brought down and anastomosed to the anus just above the dentate line. Take down of colostomy completes the operations. Considerably more complications occurred after the Swenson's technique was adopted by other surgeons. ^[8] Their dissatisfaction led to the development of other operative procedures, namely the Duhamel's ^[9] retrorectal anastomosis and the Soave endorectal pull-through. ^[10]

The originators of the various operative procedures for HD claimed to have few, if any, major complications. The incidence of complications increased rather rapidly after the procedures found general acceptance among surgeons. ^[8] These complications varied from centre to centre and surgeon to surgeon. Many surgeons in developed countries practice the Duhamel operation. ^[11] But the GI stapler needed for the final anastomosis is not available in many third world countries. This forces these centers to do either the Swenson's or the Soave operation. The results of this study show that the Swenson's operation gives good results. But there are certain general drawbacks of the Swenson's operation. ^[11] This is why we have developed our own technique of hand anastomosis.

Six patients in this series had this method for definitive operation with good results. Two children had sigmoid colostomies inadvertently placed in the right upper quadrant because they presented late and had very redundant sigmoid colons. Both had the colostomy taken down at the definitive operation and the bowel at the colostomy site used for the pull-through. Tube cecostomies were used as vents. They both did well. Enterocolitis remains a major cause of significant morbidity (28.6%) and mortality (40%). One of the patients with severe enterocolitis after a Swenson's operation did well with initial ileostomy, which was later closed. Some of our patients are still younger than three years and therefore not toilet-trained but still do not soil their perineum. The older ones pass stool 2-3 time a day, are fully continent and do not soil at night. The result of our new Duhamel is as good as the Swenson's procedure.

The overall mortality in our study was 23.8%. This was due to the lower number of patients managed, late presentation of patients with established complications and limited facilities for modern neonatal care. As reported by many authors, ^{[12],[13],[14]} colostomy continues to cause appreciable morbidity. This is because most parents were illiterate, there were no colostomy bags and no stomal nurses. A trend to early diagnosis and single-stage operations will alleviate these complications as is now being practiced in some centers. ^[15],[16] In conclusion, early recognition of symptoms and signs of HD in peripheral hospitals, prompt referral to tertiary health centers, adequate resuscitation, provision of colostomy appliances and improvements in neonatal care are needed to prevent high morbidity and mortality. In our centre, the Swenson and our modified Duhamel operations produce satisfactory results.

References

Copyright 2007 - Journal of Indian Association of Pediatric Surgeons

The following images related to this document are available:

Photo images