Modified Duhamel Procedure in Hirschsprung’s Disease : AK Suarma! SK Kotuarr’ C SHAR Ten Years’ Experience V Cuaturventt The clinical experience of modified Duhamel procedure as a definitive treatment of Hirschsprung’s Disease (HD) over 10 years was analyzed, Forty eight patients of HD were treated with modified Duhamel procedure. Forty six patients had short segment and 2 had long segment disease. Spur remnant was seen in 8 patients. Persistent constipation (n=6), soiling (n=2) and incontinence (n=2) were seen in long term follow up. The procedure is technically easy and gives satisfactory long term results for definitive treatment of HD. Key Words : Hitschsprung’s Disease, modified Duhamel procedure. Hirschsprung’s Disease (HD) is a relatively common cause of intestinal obstruction in The classical description of the discase was reported by Harald Hirschsprung in 1886. Since the first reconstructive operation described by Swenson and Bill! in 1948, much progress has been made in the heonates surgical treatment of this disorder The other procedures are modifications of Duhamel’ and Soave? procedures. Each proced appears to be equally effective in correcting the disease. The functional outcome of surgery for Department of Pediatric Surgery SMS Medical College © Associated SPM Child Health Institute, Jaipur, India ' Ex Professor & Head Senior Research Associate Associate Professor * Assistant Professor ei Dr AK Sharma K-51, Income Tax Colony Tonk Road, Durga Pura Jaipur, India variable. The continuing the optimum surgical management needs to be supported by carefully conducted long term outcome studies. The purpose of this study was to determine the morbidity, mortality and functional outcome in a large series of patients treated by a single operation, the Duhamel procedure (Lester Martin modified Duhamel procedure). Hirschsprung’s disease is debate about Materials and Methods Patients of Hirschsprung’s Disease treated with modified Duhamel operation over a 10- far period in one unit were included in the study. The study was done by retrospective analysis of hospital records and routine follow up. Results Demographics Forty eight patients of HD were treated by Duhamel procedure over a period of 10 years (1989-1999). The age at diagnosis rangedMODIFIED DUHAMEL PROCEDURE IN HIRSCHSPRUNG’S DIS! 63 from birth to 11 years (Table 1). The sex ratio was 11:1 with males predominating. The distribution of the disease was localized to rectosigmoid in 46 (96%) and extending upto transverse colon in 2 cases. Table | : Age at presentation Neonatal Period 31% (n=15) upto 1 year 15% {n=7) 1-5 years 44% (n=21) > 5 years 10% (n=5) Management The diagnosis of the disease was established by a full thickness rectal biopsy demonstrating absence of ganglion cells. The level of transition zone was determined by laparatomy and multiple biopsy, and in older patients by barium enema if possible. All the patients irrespective of age were subjected to a three stage procedure A right transverse loop colostomy was done at the diagnosis of the disease. A modified Duhamel pull-through procedure was done after 6 months to 1 year. This was followed by colostomy closure after 6 weeks. The septum crushed with Duhamel clamp in 36 patients and staplers were used in 12 patients. colorectal was In patients with long segment disease the colostomy was mobilized and an ileostomy done. Tleostomy was closed after definitive procedure. was Mortality Three patients died in the postoperative period after modified Duhamel procedure. The cause of death in these patients were fulminant septicemia, hyperpyrexia and aspiration pneumonia. Surgical Complications Repairs of burst abdomen (n=4) and laparotomy and adhesiolysis were done in 2 patients. Division of spur remnants were required in 8 patients. Spur remnant was detected by per rectal examination and was completely removed prior to colostomy clost e. Residual aganglionosis was present in 3 patients. In patients with residual aganglionosis posterior sagittal myectomy was done. The myectomy strip was subjected to histopathological examination for evidence of ganglion cells in the proximal end. Long term follow up revealed persistent constipation in 6 patients. These patients were treated by diet modification, toilet training, laxatives and enema. All patients responded well. Soiling (n=2) incontinence (n=2) were also present in patients treated with myectomy. and Constipation was defined as less than three spontaneous bowel movements per week, rectal impaction and/or abdominal fecal mass, Soiling was defined as inadvertent loss of small amount of feces and staining the underwear, Incontinence was defined as regular loss of solid feces. Discussion Hirschsprung’s disease is one of the most common congenital anomalies that pediatric surgeons manage. Inspite of — various modifications of the pull through procedures! available the long term functional results are less then ideal. There are various surgical procedures with modifications for definitive treatment of64 J INDIAN ASSOC PEDIATR SURG VOL 9 (APR-JUNE 2004) Hirschsprung’s disease. The basic aim of all these procedures is to remove the aganglionic segment and to pull-through the normal bowel thus removing the obstructing part. The three most commonly used procedures are modification of Swenson! Duhamel? and Soave? The procedure depends on the choice of surgeon's preference. The diagnosis of the disease is done by the sence of ganglion cells in a rectal biopsy.! We prefer a full thickness rectal biopsy in all patients suspected of Hirschsprung’s Disease. Anorectal manometry though preferred in neonatal period! was not done in any of our patients as this facility was not available. Laparotomy and multiple colonic biopsies are done to know the level of aganglionosis. However, in older children barium enema casily delineates a transition zone and thus eliminates the need of laparotomy.* A 3-stage procedure was done in all the patients. A right transverse colostomy diverts the fecal stream. The dilated and hypertrophied colon thus comes to near normal caliber in 6 months to 1 year It leaves enough colon for subsequent pull- through and also protects the Martin's modification and pull through anastomosis. Incomplete crushing of | spur isa complication of Duhamel — procedure. Presence of spur lead to fecal impaction in the rectal pouch and soiling The spur remnant was seen in both the group of Table Il : Summary of studies analysing outcome of the duhamel procedure Report No a1 Patients Outcome Follow-up Period | Percent Followed up Duhamel 270 3.7% constipation Not mentioned 100 100% continence Hung” 198 100% “good” < 9 years 100 outcome, five repull- throughs Jung" 107 91.6% “normal” 1-2 years 100 Rescorla et al’? 103 Normal 65% overall, Mean, 6yrs 56 94% normal at 15 yrs| Marty et al’ ot Fecal soiling, 12.1% Not mentioned 78 Hejj et al 63 Good, 204% > 4 yrs} Mean, 6 yrs 84 Sieber'® 44 Good, 93% > 5 yrs, 100 Foster et al 43 “Asymptomatic” 100%| > 7 yrs. 700 Baillie et al’ 89 “Satisfactory” Median, 8.4 yrs 100 : outcome 4-8 yrs 27%! > 14 yrs, 79%; 10.1%} failure rate Current study 48 “Satisfactory” failure rate 14% Routine 1-2 yrs 92MODIFIED DUHAMEL PROCEDURE IN HIRSCHSPRUNG'S DISEASE 65 patients using crushing clamps as well as with staplers. We used a policy to remove the spur completely prior to colostomy closure thus avoiding fecal impaction. Residual aganglionosis was seen in 3 patients in the present series. These were the patients who were older and diagnosed on barium enema. The pull-through segment was aganglionic. (Frozen Section facilities are not available.) The treatment for such cases is redo pull-through. Repeat pull-through operation is a major procedure and is not casy to perform. A simpler procedure like anorectal myectomy** through a posterior sagittal approach is easier and a long strip can be removed. This is suitable for short segment residual aganglionosis. For long segment residual aganglionosis, a repeat pull- through procedure is required.” Long term functional results vary in different series*© (Table II). The result may range from ‘fair’ to ‘satisfactory’. Constipation was seen as a major problem during long term follow up. The incidence was comparable to other series. The cause of constipation is multifactorial. Poor toilet training, spur remnant, residual aganglionosis_ and spasticity of internal sphincter are most important factors. Toilet training, dict modification, laxatives and enemas are helpful in few patients. In present series most of the patient responded to above modes of treatment. However residual spasticity may need anal stretching or myectomy. Soiling and incontinence were seen in patients with myectomy. No other patient had this problem. The near normal defecation is achieved by 2 years after pull through operation." However mild symptoms may persist and improve with age. Although the length of aganglionic segment has been considered as a determinant of functional outcome? no significant difference was seen in the present series. We conclude that modified Duhamel. procedure is technically easy, avoids anterior pelvic dissection, thus avoiding damage to the nerves. 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