Long term outcome of transanal endorectal pull-through for Hirschsprung's disease:A single institute experience

Long term outcome of transanal endorectal pull-through for

Hirschsprung's disease:A single institute  experience

T Gobran,MD; M Khalifa,MD; A Waly,MD

Department of General Surgery, Pediatric Surgery Unit, Zagazig University, Egypt.

Abstract

Background: The transanal endorectal pull-through operation (TEPT) for Hirschsprung's disease (HD) is relatively new and this makes assessment  of the functional outcome difficult. The aim of this study was to assess the long-term.functional outcome ofTEPT for short segment HD (at Faculty ofMedicine-Zagazig University) and to evaluate its effect on the patients' quality of life.

Patients and methods: Fifty-eight patients out of 176 patients who underwent TEPT technique for HD between August 2002 to August 2009 were followed up for at least one year.All patients had aganglionic  segment  that is confined  to the rectosigmoid  area. Long term outcome and quality of life were assessed by interviews with the parents and/or patients using pre-structured questionnaires filled by the attending doctor.

Results: Forty-four patients had  satisfactory results  without  complications. Reported

postoperative complications included soiling in 11 patients, constipation in 3 patients, incontinence in 3 patients and enterocolitis in 7 patients. According to quality of life scoring criteria, 75.9% of patients had good quality of life and 18.9% had fair quality of life.

Conclusion:  TEPT for short segment HD is associated  with gradual  recovery  of stooling pattern and  long  term  normal bowel  function and  good  quality of  life  for  patients.

Key  words:  Hirschsprung's disease, long  term  follow  up, quality  of life, fecal  soiling, constipation, fecal incontinence.

Introduction:

Hirschsprung's disease (HD) is a common neurogenic cause  of functional neonatal intestinal obstruction affecting approximately

1:5000  births.l  Aganglionosis  is confined to rectosigmoid segment in approximately 80%, but proximal extension occurs and up to 15% of patients have total colonic aganglionosis.2

Surgical treatment ofHD has changed inrecent decades, with  attempts made  to reduce extensive surgical dissections to reduce postoperative complications and hospitalization time and ultimately decreasing morbidity.3

Endorectal  pull-through was described  in

1964 by Soave.4 Inthe same year, the technique was modified  by Boley  who performed the colo-anal anastomosis during the pull through.5

Transanal endorectal pull  through  (TEPT) represents the latest development in the concept of the minimally  invasive  surgery for HD.6

Since the description  of TEPT for HD by De Ia Torre Mondragon and Ortega Salgado,7 the approach has become commonly used by pediatric surgeons.8  Compared with  the traditional  approaches  such as the Swenson, Duhamel, and Soave, the most advantage of the operation is that it is minimally  invasive and eliminates the abdominal incision resulting in no abdominal scar and complications of traditional laparotomy. The operating time and hospital delay are also cut short. Furthermore, the anal sphincter is kept in operation so that the morbidity of postoperative incontinence is sharply cut down. It has become increasingly popular to more and more doctors and patients.9

The long term follow-up  of children with HD gives one the best opportunity to critically evaluate the efficacy and results of a particular surgical procedure. In  general, the  most commonly encountered problems include

constipation, incontinence, enterocolitis and the overall impact of the disease on lifestyle of patients and/or their families.IO The aim of this study was to assess the long-term functional outcome ofTEPT for short segment HD (at Faculty ofMedicine-Zagazig University) and to evaluate its effect on the patients' quality of life.

Patients and  methods:

A retrospective review of medical records revealed 176  children  who  had undergone transanal endorectal pull-through (TEPT) for correction of short segment HD in Pediatric Surgery Unit- Faculty of Medicine-Zagazig University,  between August 2002 to August

2009. Of all these children 58 patients were included and 118 were lost.

Exclusion criteria were:

-  Associated Down's syndrome, mental retardation, or cerebral palsy.

-Less than one year follow-up.

-  Patients who  refused to  complete the questionnaire,

All information about clinical,  operative and postoperative data were obtained from

medical records including gender, age at time of surgery, mode of presentation, details  of surgery, results of early postoperative follow up visits and postoperative complications encountered including  anastomotic leakage, stricture and enterocolitis. The next step was to invite families to participate in the study by telephone. The patients and their parents were interviewed by a pre-structured questionnaire. Questions were asked to collect data on stooling pattern (stool frequency, stool consistency, stool  control), fecal  soiling, constipation, incontinence, medication use enterocolitis, and the  effect of  bowel habits on  the  child's activities and social life.

The clinical examination included a digital rectal examination that assessed the presence or absence of rectal prolapse, tone of the anal sphincter, ampulla capacity, amount of feces, and anastomosis.

Long-term outcome  was evaluated by a clinical bowel function scoring system Table(t)ll and quality  of life was assessed according to the Quality of Life Scoring Criteria for children with fecal incontinence Table(2).12

Table (1): Clinical bowel function scoring system.

NOTE: Good, 9 to 12 points; fair, 5 to 8 points; poor, 0 to 4 points.

Table (2): Quality of life scoring criteria for children.

NOTE: Good, 9 to 12 points; fair, 5 to 8 points; poor, 0 to 4 points.

Results:

This  study included 58 patients who underwent TEPT technique during period from August 2002 to August 2009. The mean age at time of surgery was 3.5 years. The youngest age at operation time  was  one month  age, whilst  the  oldest  was  14  years. The  data concerned with bowel function, stooling pattern and their effect on quality of life are shown in Table(3).

1.Stool frequency

Fifty patients had mean stool times 1 to 2 per day (86.2%), only 5 patients (8.6%) had mean stool times 3 to 5 per day and only 3 patients  (5.2%) had mean stool times  I to 2 per week in the exact follow-up.

2.Stool Control

Forty one patients (70.7 %) had long-term normal bowel function and 17 patients (29.3%) had  long-term bowel  dysfunction. Among these, 11158 patients (18.9%) had fecal soiling, particularly at night. Three of total 58 patients (5.2%) suffered from fecal incontinence and

3/58   patients (5.2%) had   constipation.

3.Stool Consistency

Forty-nine patients (84.4%) had a normal stool consistency. Six patients (10.4 %) had muddy foul smelling loose stools and frequent flatus. Three patients had liquid stools (5.2%).

4.Pain or difficulty with defecation

Forty-five patients  (77.6%) had no pain with  defecation. Ten patients (17.2%) had accidental pain with defecation. Three patients (5.2%) had  frequent pain  with  defecation (including 3  patients who  suffered from constipation).

5.Late postoperative complications

5.1.Enterocolitis

Seven cases  (I2%) had postoperative enterocolitis (in the form of offensive diarrhea, fever, toxemia) during long term follow up period, two cases had mild attacks and five cases had moderate attacks and all these cases were hospitalized  and responded  to medical treatment  in the form of intravenous fluids, parentral antibiotics, enemas, and metronidazole.

5.2. Anastomotic stricture

There were 5 (8.6%) cases of anastomotic stricture developed within weeks from surgecy,

4 of them were infants at time of surgery. All these  patients improved on regular anal dilatation during clinic visits and none of them required surgical intervention.

5.3. Constipation

It was found  in 3 patients  (5.2%)  in this study. Barium enema showed colonic dilation and revision of histopathology  was done and detected residual aganglionic segment in two patients. The third patient had redundant colon. Redo transanal  endorectal pull-through was feasible in two patients and the remainder one needed conversion  transanal endorectal pull­ through. Of these  three  cases, two  showed improvement of bowel function during  the long-term follow up and one case showed true incontinence.

5.4.Fecal soiling

Postoperative soiling accidents were observed  inll patients (18.9%). These cases could be classified as having either heavy or light soiling based on frequency of episodes. Seven patients had light (accidental) soiling and four patients had heavy (frequent) soiling. These patients were treated with constipating diet  and  drugs  and  improved with  time.

5.5.Fecal incontinence

Three  patients  (5 .2%)  had  fecal incontinence. They had a nondilated colon on contrast enema and frequent attacks of diarrhea and did not respond to medical treatment. Also these children  were evaluated with revision histopathology and all of the results confirmed the presence  of ganglion  cells. The patients underwent anorectal manometry which revealed decreased basal  resting pressure (BRP),  maximal squeeze pressures (MSP) voluntary sphincter force (VSF) and negative rectoanal relaxation reflex.

5.6.Mortality

No mortality was death recorded during the follow up period.

6.Quality of life

Because  of fecal soiling or incontinence,

14 patients (24.1%) had to restrict their foods. School absence occurred in 5 patients (8.6%) as  patients limited their  physical activity because of soiling or odor.Five patients (8.6%) had problems in  peer relationships. Three patients remained medical therapy dependent and 11 (18.9%)  patients needed  occasional intermittent therapy, such  as  medication, enema, and diapers. Forty-four patients (75.9<'/o) had good quality of life and no limitation  to their social activities. Eleven patients (18.9%) had fair quality oflife and 3 (5.2%) had poor quality of life.

Discussion:

The goal of treating a child with HD should be to achieve anorectal function that is as near to normal as possible. The best approach is to bring ganglionic  bowel down to a point just above the dentate line.13 The transanal endorectal pull-through operation is a relatively new minimally  invasive technique. It leaves no abdominal incision or  scar,  avoids  the potential complications of  laparotomy (adhesions, wound infection), and is associated with shorter operating time and hospital stay. These advantages make it superior to traditional laparotomy in  the immediate term.14  The functional outcome in patients treated for HD is variable. Many reports showed that the results of surgery for HD were satisfactory.11 The majority of  long-term follow-up studies concentrated on the functional  outcome and little  is known  about  the  quality of life of patients after surgical treatment for HD. The aim of this study was to assess the long-term functional outcome ofTEPT for short segment HD (at Faculty of Medicine-Zagazig University) and to evaluate  its effect on the patients' quality of life.

Table (3): Bowel function, stooling pattern and quality of life for study cases.

Stooling patterns remains poorly understood in the most of the reported series after surgical correction of HD. A previous evaluation of stooling patterns in patients after endorectal pull-through (ERPT) clearly showed a return to normal stooling  frequency  over time.15

Although several authors of primary pull­ through series claim that their patients are continent this must be viewed with caution, because follow-up in many of these patients was no longer than 2 or 3 years.16 In this study, the patients were followed up from 1 to 7 years to evaluate  the functional results  of the operation as well as its impact on the quality of patients' life.

Postoperative bowel dysfunction had been

reported to occur in 10% to 30% of patients with HD.ll Incontinence and constipation are

2 common reported problematic sources after surgery for HD.17 In our study, (29.3%) of the patients had long-term bowel dysfunction in the form of soiling, incontinence and constipation. Soiling occurred in 11 patients (18.9%), constipation in3 patients (5.2 %) and incontinence in 3 patients (5.2%).Zhang et ai9 studied 58 patients who underwent transanal pull through operation for HD. The results were soiling in 9 patients (15.5%) and constipation in 5 (8.6%) while incontinence did not complicate any patient.

In this study, there were 5 cases (8.6%) of anastomotic stricture with various degrees of

constipation who  improved on regular dilatation. These complaints did not develop until few weeks to two months after the pull­ through procedure  and improved within one year after operation. One study reported overall incidence  14% anastomotic  stricture of total

84 infants who were managed by TEPT for surgical correction of HD. Ten cases in that study improved by regular  dilatation and 2 cases required surgical intervention.IS So we recommend prophylactic anal bouginage with Hegar probe at 2 weeks after  operation particularly  in   infants  and   neonates.

Inthe current study, persistent constipation was  found  in  3 patients (5.2%), which  is relatively low if compared  to other  studies such as Teitelbaum and colleagues work.l3

They described their experience with 78 infants who were treated with TEPT, and reported constipation  at a rate of28%. Van Leeuwen et ai19 reported constipation  in 22% of cases and El-Sawaf et al,20 found it in (29.3%)  of total41 patients who underwent TEPT for liD.

Inchildren with persistent constipation redo pull-through operation and resection  of the problematic dilated bowel may be required. Indications for a second pull-through  include retained or acquired aganglionosis, severe stricture, dysfunctional bowel segment, marked dilation  of the bowel  as a result of years of constipation, anocutaneous fistula, and intestinal neuronal dysplasia.21 In the present study,  for the 3 patients with   persistent constipation and  who  did  not  respond to conservative management in the form of laxatives and diet modification; barium enema and revision ofhistopathology were done and detected residual aganglionic segment in two patients, while the third patient had redundant dilated colon. Redo transanal endorectal pull­ through was performed  to two patients with resection of the affected segment, while trans­ abdomenal pull-through was performed to the third patient. Two cases showed improvement ofbowel function during the long-term follow up and one case showed fecal incontinence. There  are other  studies  that reported cases required redo  pull-through after  surgical correction ofHD. Aggarwal et a122 performed redo pull-through procedures for four cases. The  frequency of  defecation in their  four

patients following a  second  pull-through operation was high in the early postoperative period. However, the stooling pattern improved considerably over  the next  few months. Teitelbaum and  Coran,23 described an experience  with 26 redo pull-through with a mean follow-up of 14 years. Almost all patients were continent except 2 that had daily leakage of stool. Although one patient required a third pull-through procedure, the authors concluded redo procedures can be performed effectively and   yield  good  to   excellent  results.

Fecal incontinence  after  operative

management of HD is a devastating complication.9 The exact cause of possible incontinence after  TEPT  operation is  still unclear, it may be due to very low transanal dissection which may result in poor sphincter function and poor or absent sensation.24 In our study, 3 patients (5.2%) had fecal incontinence and all of them had nondilated colon on contrast enema and frequent episodes of diarrhea and did not respond to medical treatment and dietary modifications. Zhang et a}l4 and Elhalaby et alB did not report fecal incontinence post TEPT operation in their series. The occurrence of incontinence in our study may be due to the learning curve effect on the results (i.e., poorer results  with  procedures done earlier  in the analysis).

Fecal soiling is one of the common problems occurring after surgical correction ofHD which has significant impact on patient' quality of life and social development especially patients of school age.12 In our study fecal soiling occurred in (18.9%) of patients postoperatively and  (6.9  %) had  heavy  soiling. However, gradual decline could be noted in the rates of soiling seen in the immediate  postoperative period in most patients, gradually  improved with  time. Elhalaby and his colleagues8 reported transient soiling and increases in bowel movements in a significant number of their patients who underwent TEPT, and they felt the cause was the overstretching.However, they  stated  that  this soiling was transient.

Enterocolitis has been considered one of the main problems in patients with HD both before and after defmitive  treatment. It was noticed that incidence of postoperative enterocolitis in this series was relatively low

in comparison to other series.19,20,25 This may be due to our routine use of anal dilatation 2 weeks postoperatively particularly  in infants and neonates, also underestimation in diagnosing early cases of enterocolitis because most  of  our  patients' families are  of  low socioeconomic level and lack awareness  of general condition of their children and reside far away from the hospital so usually were managed by general practitioners in primary health care clinics as a case of gastroenteritis.

There is a strong association between poor continence and negative effects on the child's social  life  and  activities, most  significant between 5 and 15 years of age. These are the formative years when a child begins to develop peer relationships and self-esteem.26 In our study (75.9 %) of patients had good quality of life and no limitation to their social activities. This  is a reflection of the improved bowel function on long term follow-up ofHD cases managed by TEPT.  Eleven patients (18.9%) had fair quality oflife and 3 (5.2%) had poor quality  of life and were not satisfied.  Bai et alll studied forty-five patients who underwent the Swenson  procedure for Hirschsprung's disease and reported eighteen patients (40%) had  good  quality of life  and  , twenty-one patients (46.7%) had fair quality oflife, and 6 (13.3%) had poor quality oflife, according to Quality of- Life Scoring Criteria for children. These results indicate the  importance of addressing and managing  problems  of fecal control  over  long-term follow-up visits  of children with  Hirschsprung's  disease.

Conclusion:

TEPT for short segment HD is associated with gradual recovery of stooling pattern, long term normal bowel function and good quality oflife for patients.

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