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Single Stage Transanal Endorectal Pull-Through for Management of Hirschprung’s Disease

Rakesh Handa¹, Man Mohan Harjai²

¹Department of Pediatric Surgery, Max Healthcare and Kailash Group of Hospitals

²Department of Pediatric Surgery, Surgical Division, Base Hospital Delhi Cantt

New Delhi, India

 

Correspondence:

Rakesh Handa

Senior Consultant Neonatal, Pediatric and Laparoscopic Surgeon

Max Healthcare and Kailash Group of Hospitals,3 Palms Surgical Clinic

A-49 New Friends Colony; New Delhi -110065

Tel: 91-11-26913402, 41626519, 9810125022, E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Introduction: Over the years, the surgical management of recto-sigmoid Hirschsprung’s disease (HD) has evolved radically and at present a single stage transanal pull-through can be done in suitable cases, which obviates the need for multiple surgeries and blood transfusion.

Material and Methods: A retrospective analysis (between January 2003 and November 2005) was carried out on all cases of Hirschsprung’s reporting to a tertiary care hospital that were managed by transanal pull-through as a definitive treatment. All selected patients including neonates had an aganglionic segment confined to the rectosigmoid area, confirmed by preoperative barium enema and postoperative histology. All children had their operation done without construction of prior colostomy. We relied on the level indicated on a barium enema as well as clinical intra-operative assessment since we were lacking the facilities of frozen section.

Results: Transanal pull-through was performed in 14 children including 5 neonates. Mean operating time was 100 minutes (range 64 to 135 minutes). No patients required laparotomy because all patients including neonates had an aganglionic segment confined to the rectosigmoid area. Blood loss ranged between 20 to 85 ml with blood replacement required in 2 cases. Since all children were given an epidural caudal block, the requirement of analgesia in these cases was minimal. Postoperative complications included perianal excoriation in 11 out of 14 (78.5%) patients lasting from 3 weeks to 6 months, which included all neonates (n=5). Complete anorectal continence was noted in 10 of 14 (71.3%) children in follow up of 3 – 5 years, whereas 3 other patients showed a steady improvement in their continence status.

Conclusions: We believe that this procedure can be safely conducted based on the clinical expertise and experience which all pediatric surgeons possess since they regularly perform leveling colostomies in Hirschsprung’s disease. This can be confidently performed even in hospitals that do not have facilities for frozen section.

Key words: Single stage, transanal, pull through procedure, Hirschsprung’s disease, frozen section biopsy, perianal excoriations.

  

Introduction

Hirschsprung’s disease is a fairly common developmental disorder that occurs in newborns as a result of absence of ganglion cells in the distal bowel resulting in functional intestinal obstruction. Traditionally Hirschsprung’s disease has been managed by three staged surgical procedures over a 4 to 6 month period. This included a defunctioning colostomy and multiple biopsies from the colon, a definitive pull-through and finally a colostomy closure [1]. Most patients required a blood transfusion at some stage. Over the years, the surgical management of recto-sigmoid Hirschsprung’s disease (HD) has evolved and at present a single stage transanal pull-through can be done in suitable cases, which obviates the need for multiple surgeries and blood transfusion. The procedure leaves no scars, is associated with less postoperative pain and shorter hospital stay. Return of bowel function post operatively is much earlier and permits initiation of oral feeds. We share our experience with this procedure over a 3-year period.

Material and Methods

A retrospective analysis was carried out on all cases of Hirschsprung’s disease reporting to a tertiary care hospital that were managed by transanal pull-through as a definitive treatment. There were 14 cases, including 5 neonates. The diagnosis in the neonates was based on history of failure to pass meconium in first 24 to 48 hours of life, clinical presentation with abdominal distension and vomiting, plain radiograph which showed absence of gas shadow in the pelvis, barium enema in which there was abnormal retention of contrast in a 24 hour film and confirmed by a full thickness rectal biopsy. Further, the level of the contrast tapering was taken as an indicator of the level of aganglionosis. All selected patients including neonates had an aganglionic segment confined to the rectosigmoid area, confirmed by preoperative barium enema and postoperative histology. All children had their operations done without construction of prior colostomy.

Technique used involved eversion of the anal verge with silk sutures to expose the pectinate line (Fig. 1). Submucosal infiltration with adrenaline saline was done and submucosal dissection was performed up to the level of peritoneal reflection leaving a muscular cuff. The bowel is then pulled out through the anal opening (Fig. 2) and level of resection decided based on the appearance and texture of the bowel. The resected edge is then sutured to the pectinate line (Fig. 3). Following the pull-through, the excised segment was sent for histopathological examination to confirm the diagnosis of Hirschsprung’s disease and also the presence of ganglion cells in the pulled through end. Assessment was done on the following parameters – operation time, blood loss and need for replacement, initiation of feeding postoperatively, requirement of analgesia, duration of hospital stay and perianal skin excoriation.

single stage transanal 1 single stage transanal 2
Figure 1. Per-operative photograph showing eversion of anal verge with silk sutures. Figure 2. Pulled out aganglionic colon through anal opening up to the level of resection

 Results

Transanal pull-through was performed in 14 children including 5 neonates. There were 3 females and 11 males with age ranging from 21 days to 3 years. Mean operating time was 100 minutes (range 64 to 135 minutes). No patients required laparotomy because all patients including neonates had an aganglionic segment confined to the rectosigmoid area. Slight difficulty was experienced in entering the submucosal plane in the initial part of our learning curve. Blood loss ranged between 20 to 85 ml with blood replacement required in 2 cases. Most children were very hungry within 48 hours and feeding was initiated at around 48 to 72 hours. Since all children were given an epidural caudal block, the requirement of analgesia in these cases was minimal. Postoperative complications included perianal excoriation in 11 out of 14 (78.5%) patients lasting from 3 weeks to 6 months, which included all neonates (n = 5). Other complications included enterocolitis (n = 1), recurrent constipation (n = 1) and rectal mucosal prolapse (n = 1). Complete anorectal continence was noted in 10 of 14 (71.3%) children, whereas 3 other patients showed a steady improvement in their continence status. No death occurred in the series. The mean hospital stay was 7 days with a range of 5 to 11 days.

single stage transanal 3

 Figure 3. Completed colo-anal anastomosis. 

Discussion

Hirschsprung’s disease is a common surgical problem in children. Majority of the children have the classical disease in which there is a short aganglionic segment, which may extend up to the rectosigmoid junction. Traditional management of these cases by a 3-stage procedure resulted in significant morbidity associated with a defunctioning colostomy and repeated surgeries.

With the introduction of a single stage trans-anal endorectal pull-through procedure, significant reduction in morbidity is possible as it avoids repeated staged surgeries. The use of a trans-anal endorectal pull-through is feasible in neonates as well as in older children [2]. The mean operating time in our series was 100 minutes ranging from 64 to 135 minutes. The longer operating time occurred in the early cases due to our learning curve. Blood loss varied between 20 and 85 ml with replacement of blood required only in 2 cases (14%).

Perianal excoriation is a troublesome problem associated with trans-anal endorectal pull-through, which results in significant morbidity and more importantly parental anxiety as to whether the operation has been carried out successfully. In our series, 11 out of 14 children had perianal excoriation, which lasted for a period varying from 3 weeks to 6 months. This occurs most probably due to overstretching of the anal sphincters and subsequent increased stool frequency. While Hadidi has also reported increased stool frequency in all cases lasting up to 6 weeks [3], Rintala reported perianal skin rash in 14 out of 26 (54%) cases [4].

Most authors have done the trans-anal pull-through aided by knowledge of the level of aganglionosis on the basis of a frozen section biopsy [5, 6]. In our series, since we did not have the facility of a frozen section biopsy, we relied on the level indicated on a barium enema as well as clinical intra-operative assessment. The intra-operative assess ment consisted of a visual and palpatory examination of the mobilized bowel. The visual examination assisted us by indicating the level of the transition zone and the proximal dilated bowel. Palpation of the mobilized bowel reveals a thicker bowel wall in the transition zone as compared to a thin walled, soft and compliant proximal bowel. To be on the safer side, we resected an extra 5 to 7 cm length of dilated proximal bowel than we would have done with tissue diagnosis. Histopathological confirmation of ganglion cells in the pulled through bowel in all cases justified our clinical assessment in the management of rectosigmoid Hirschsprung’s disease by trans-anal endorectal pull-through. Zhang et al also performed the single stage trans-anal pull-through based on preoperative barium enema and postoperative histopathological confirmation [7].

While many would object to the clinical assessment of the level of aganglionosis as compared to frozen section, our belief in the clinical assessment is strengthened by the fact that most pediatric surgeons used to perform a leveling colostomy at the level of perceived aganglionosis / transition zone. This colostomy was over the years also based on a visual and tactile assessment. The only difference was that the level of aganglionosis was subsequently confirmed histologically prior to the definitive pull-through. However, despite not having the facility of a frozen section, we decided to use our experience and clinical acumen to offer a better surgical option with significantly reduced morbidity to these children. Histological confirmation vindicated our belief in ourselves.

Conclusions

Single stage transanal endorectal pull-through procedure for the management of rectosigmoid Hirschsprung’s disease is now a well-established and preferred approach. Parental satisfaction is immense due to the lack of scars on the abdomen. We believe that this procedure can be safely conducted based on the clinical expertise and experience which all pediatric surgeons possess since they regularly perform leveling colostomies in Hirschsprung’s disease. This can be performed even in hospitals that do not have facilities for frozen section.

 

 

REFERENCES

1. Harjai MM. Hirschsprung’s disease: Revisited. J Postgrad Med 2000; 46:52-4.

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3. Hadidi A. Transanal endorectal pull-through for Hirschsprung’s disease: a comparison with the open technique. Eur J Pediatr Surg 2003; 13:176-80.

4. Rintala RJ. Transanal coloanal pull-through with a short muscular cuff for classic Hirschsprung’s disease. Eur J Pediatr Surg 2003; 13:181-6.

5. Teeraratkul S. Transanal one-stage endorectal pull-through for Hirschsprung’s disease in infants and children. J Pediatr Surg 2003; 38:184-7.

6. Langer JC, Durrant AC, Torre Luis de la, Teitelbaum DH, Minkes RK, Caty MG, Wildhaber BE, Ortega SJ, Hirose S, Albanese CT. One-Stage Transanal Soave Pull-through for Hirschsprung Disease. A Multicenter Experience With 141 Children. Ann Surg 2003; 238: 569–83.

7. Zhang SC, Bai YZ, Wang W, Wang WL. Clinical outcome in children after transanal 1-stage endorectal pull-through operation for Hirschsprung disease. J Pediatr Surg 2005; 40:1307-11.