Long-term results of treatment of Hirschsprung’s disease

https://doi.org/10.1053/j.sempedsurg.2004.10.015Get rights and content

Many children with Hirschsprung’s disease (HD) have a good outcome following surgical treatment, but long-term follow-up studies have identified a number of concerns. Analysis of long-term function in children after surgical management is difficult. The most commonly encountered problems include constipation, incontinence, enterocolitis and the overall impact of the disease on lifestyle (quality of life). Other complications are less frequent. Each of these problems will be discussed.

Section snippets

Definitive procedures

Currently, the one-stage, Swenson, Duhamel, and Soave pull-through procedures have been accomplished by laparotomy1, 2, 3 or with laparoscopic assistance.4, 5, 6, 7, 8 The results are as good or better than those classically completed in two- or three-stage procedures. The one-stage procedure avoids the need for a colostomy (and potential complications) and reduces the cost when compared with multiple-stage procedures.9, 10, 11, 12 The one-stage Soave pull-through is one of the more commonly

Constipation and stooling frequency

Constipation is probably the most common complaint after a pull-through procedure for Hirschsprung’s disease. Typically, these complaints do not develop until a few weeks to months after the pull-through procedure.22 Rescorla and coworkers23 found that 88% of patients had difficulty stooling with a follow-up less than 5 years. Although some patients with incontinence were in this group, symptoms improved with longer follow-up, with 100% having satisfactory stooling when followed for 15 years or

Continence

Few long-term complications have a greater impact on the quality of a patient’s life than incontinence. An accurate assessment of continence has been difficult to determine and many studies either do not include this information or fail to make a distinction between occasional soiling and significant incontinence. Incontinence rates range from none in several series to as high as 74% in one series.25, 29, 30 In general, with increasing appreciation of the importance of preserving the bulk of

Enterocolitis

Enterocolitis represents one of the most significant causes of morbidity and mortality in HD.26, 50, 51, 52, 53, 54 Enterocolitis can occur both before and after operative intervention. This serious complication is reported to occur at a frequency of up to 40% after definitive pull-through procedure,55, 56, 57 with an associated mortality rate of 3% to 30%.23, 58, 59 Rates of occurrence vary widely and this is likely the result of how various authors’ define this complication.60

Hackam and

Transitional zone pull-through

The outcome of transitional zone pull-through (TZPT) has not been well documented in the literature. Ghose and coworkers74 conducted a retrospective evaluation of 13 children and found the incidence to be 14.8%, however, their technique did not involve frozen-section analysis. No child with a TZPT had normal bowel control with most noting fecal leakage and soiling. Ultimately 54% of patients underwent repeat pull-through procedures.

A recent evaluation of 96 patients that underwent a Duhamel

Anastomotic leak

The incidence of anastomotic leak varies from 1.5% to 8%.26, 47, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78, 79, 80, 81, 82, 83, 84, 85, 86 Most, but not all strictures are associated with an early leak after the initial pull-through procedure (75%).87 In a review of 880 patients with the Swenson procedure (a combination of two- or three-stage procedures), Sherman and coworkers reported an anastomotic leak rate of 5.6%.88

Cuff abscess

Stricture formation

The incidence of anal anastomotic stricture varies from 3% to 35% and most can be managed with repeated dilations.26, 47, 54, 84, 85, 86 The etiology of most strictures is probably anastomotic ischemia or dehiscence, circular rather than oblique anastomosis, and failure to adhere to a dilation program. Fistulae and intraabdominal sepsis also may accompany strictures or may result from attempts at dilation.89

Teitelbaum and coworkers62 reported a 15% stricture rate following a primary endorectal

Anorectal achalasia

The use of anal manometry is helpful in evaluating disorders of the internal anal sphincter. Manometry may detect absence of anal relaxation with anorectal distension which is consistent with internal anal sphincter achalasia (IASA) and ultrashort segment HD.91 The results of anorectal manometry are not always entirely clear because many infants and children with HD that have normal stooling function may never regain a relaxation reflex following a pull-through procedure,71 while many22

Redo pull-through

Despite numerous reports describing excellent results following surgical correction of HD, a small subset of patients will inevitably have complications after a pull-through procedure.23, 26, 30, 84, 95, 96 In this select group of patients, these complications can occasionally be insurmountable without resorting to a redo pull-through operation. A second pull-through procedure is a formidable challenge. Indications for a second pull-through include: retained or acquired aganglionosis, severe

Total colonic aganglionosis

TCA accounts for approximately 3% to 12% of infants with HD and the incidence of total colonic aganglionsis with ileal involvement is estimated at 1 case in 50,000 live births.100 These patients are a unique subset because of the associated increased morbidity and mortality.101, 102, 103, 104 The long-term outcome in patients with TCA is significantly worse than the general HD patient population and the mortality rate ranges from 8% to 30%.101, 105

Survival for TCA has improved significantly

Intestinal neuronal dysplasia

The association of IND with HD was first described by Lassmann and Wurnig in 1973.116 In two studies by Kobayashi117 and Schmittenbecher118 30 to 40% of cases documented IND in association with HD. There may be a relationship between the incidence of postoperative complications and the presence of IND in the pull-through segment, but it is not clear whether this dysganglionosis alone is responsible for the complications.

Postoperatively, there is a delay of regular defecation in the patients

Down syndrome

Previous reports have shown that the presence of Down syndrome (DS) portends a worse outcome for patients with HD. This is evidenced by an increased incidence of postoperative enterocolitis,69, 126 anastomotic leak,127 incontinence,128 and mortality.23, 88, 128 In view of these findings, more conservative treatment strategies have been advocated, however, most of these studies are based on relatively small numbers of patients and reflect a period of time when treatment of Down syndrome was more

Impotence and urinary function

An operation in the pelvis puts the patient at risk for injury to nerves affecting bladder and sexual function. The surgical modifications of the Swenson procedure (Duhamel, Soave) were designed to reduce the risk of injury to delicate pelvic structures. Despite the incidence of these complications being quite rare, diligence in surgical technique is mandatory.

Pelvic and perineal surgery may cause damage to the pelvic splanchnic nerves, the hypogastric nerves, or the pelvic nerve plexus,

Quality of life outcomes

HD involves a more complex abnormality of the autonomic and nervous system than initially perceived.142, 143 This may explain the wide variability of outcomes that are observed due to the fact the surgical procedure involves an anatomic reconstruction of what is essentially a neurogenic form of intestinal obstruction.

The postoperative results of surgically treated HD generally are highly satisfactory.23, 88, 144, 145, 146 However, some patients still have problems and the majority of long-term

References (149)

  • Y. Gao et al.

    Primary transanal rectosigmoidectomy for Hirschsprung’s diseasePreliminary results in the initial 33 cases

    J Pediatr Surg

    (2001)
  • D.H. Teitelbaum et al.

    Reoperative surgery for Hirschsprung’s disease

    Semin Pediatr Surg

    (2003)
  • T. Marty et al.

    Gastrointestinal function after surgical correction of Hirschsprung’s diseaseLong-term follow-up in 135 patients

    J Pediatr Surg

    (1995)
  • D. Drossman et al.

    Bowel patterns among subjects not seeking health care

    Gastroenterology

    (1982)
  • H.A. Heij et al.

    Long-term anorectal function after Duhamel operation for Hirschsprung’s disease

    J Pediatr Surg

    (1995)
  • A. Nagasaki et al.

    Postoperative sequential anorectal manometric study of children with Hirschsprung’s disease

    J Pediatr Surg

    (1980)
  • D.C. Liu et al.

    Transanal mucosectomy in the treatment of Hirschsprung’s disease

    J Pediatr Surg

    (2000)
  • M. Kuwahara et al.

    Endosonographic study of anal sphincters in patients after surgery for Hirschsprung’s disease

    J Pediatr Surg

    (1999)
  • A. Livaditis

    Hirschsprung’s diseaseLong-term results of the original Duhamel operation

    J Pediatr Surg

    (1981)
  • H.G. Mishalany et al.

    Postoperative functional and manometric evaluation of patients with Hirschsprung’s disease

    J Pediatr Surg

    (1987)
  • M. Heikkinen et al.

    Long-term anal sphincter performance after surgery for Hirschsprung’s disease

    J Pediatr Surg

    (1997)
  • I.M.C. MacIntyre et al.

    Results of the Lord non-operative treatment of haemorrhoids

    Lancet

    (1972)
  • A.G. Catto-Smith et al.

    Fecal incontinence after the surgical treatment of Hirschsprung’s disease

    J Pediatr

    (1995)
  • R. Squire et al.

    The clinical application of the Malone antegrade colonic enema

    J Pediatr Surg

    (1993)
  • G.C. Fraser et al.

    Mortality in neonatal Hirschsprung’s disease with particular reference to enterocolitis

    J Pediatr Surg

    (1967)
  • S.W. Moore et al.

    Clinical outcome and long-term quality of life after surgical correction of Hirschsprung’s disease

    J Pediatr Surg

    (1996)
  • S. Kleinhaus et al.

    Hirschsprung’s diseaseA survey of the members of the Surgical Section of the American Academy of Pediatrics

    J Pediatr Surg

    (1979)
  • S. Brearly et al.

    Pseudomembranous colitisA lethal complication of Hirschsprung’s disease unrelated to antibiotic usage

    J Pediatr Surg

    (1987)
  • D.J. Hackam et al.

    Enterocolitis after the surgical treatment of Hirschsprung’s diseaseRisk factors and financial impact

    J Pediatr Surg

    (1998)
  • A.H. Bill et al.

    Then enterocolitis of Hirschsprung’s diseaseIts natural history and treatment

    Am J Surg

    (1962)
  • C.L. Berry et al.

    The experimental production of colitis in the rabbit with particular reference to Hirschsprung’s disease

    J Pediatr Surg

    (1968)
  • A. Hadidi

    Transanal endorectal pull-through for Hirschsprung’s diseaseExperience with 68 patients

    J Pediatr Surg

    (2003)
  • H. Kobayashi et al.

    Intestinal neuronal dysplasia is a possible cause of persistent bowel symptoms after pull-through operation for Hirschsprung’s disease

    J Pediatr Surg

    (1995)
  • S.W. Moore et al.

    Long-term clinical, manometric, and histological evaluation of obstructive symptoms in the postoperative Hirschsprung’s patient

    J Pediatr Surg

    (1994)
  • K. Kimura et al.

    Posterior sagittal rectal myectomy for persistent rectal achalasia after the Soave procedure for Hirschsprung’s disease

    J Pediatr Surg

    (1993)
  • G.K. Blair et al.

    Internal sphincterotomy in post-pull-through Hirschsprung’s disease

    J Pediatr Surg

    (1996)
  • S.I. Ghose et al.

    Hirschsprung’s diseaseProblems with transitional zone pull-through

    J Pediatr Surg

    (2000)
  • M.K. Farrugia et al.

    Does transitional zone pull-through in Hirschsprung’s disease imply a poor prognosis?

    J Pediatr Surg

    (2003)
  • P.K.H. Tam et al.

    Origin, course and endings of abnormal enteric nerve fibers in Hirschsprung’s disease defined by whole mount immunohistochemistry

    J Pediatr Surg

    (1990)
  • H. Miura et al.

    The structure of the transitional and aganglionic zones of Auerbach’s plexus in patients with Hirschsprung’s diseaseA computer-assisted three-dimensional reconstruction study

    J Pediatr Surg

    (1996)
  • A. Yamataka et al.

    Laparoscopy-assisted suction colonic biopsy and intraoperative rapid acetylcholinesterase staining during transanal pull-through for Hirschsprung’s disease

    J Pediatr Surg

    (2002)
  • D.T. Wilcox et al.

    Repeat pull-through for Hirschsprung’s disease

    J Pediatr Surg

    (1998)
  • G.M. Tariq et al.

    Complications of endorectal pull-through for Hirschsprung’s disease

    J Pediatr Surg

    (1991)
  • K. van Leeuwen et al.

    Long-term follow-up of redo pull-through procedures for Hirschsprung’s diseaseEfficacy of the endorectal pull-through

    J Pediatr Surg

    (2000)
  • J.O. Sherman et al.

    A 40-year multinational retrospective study of 880 Swenson procedures

    J Pediatr Surg

    (1989)
  • H. Kobayashi et al.

    Abnormal internal anal sphincter innervation in patients with Hirschsprung’s disease and allied disorders

    J Pediatr Surg

    (1996)
  • R. Minkes et al.

    A prospective study of botulinum toxin for internal anal sphincter hypertonicity in children with Hirschsprung’s disease

    J Pediatr Surg

    (2000)
  • H.B. So et al.

    Eighteen years’ experience with neonatal Hirschsprung’s disease treated by endorectal pull-through without colostomy

    J Pediatr Surg

    (1998)
  • J. Langer

    Repeat pull-through surgery for complicated Hirschsprung’s diseaseIndications, techniques, and results

    J Pediatr Surg

    (1999)
  • S.K. Aggarwal et al.

    Combined abdominal and posterior sagittal approach for redo pull-through operation in Hirschsprung’s disease

    J Pediatr Surg

    (2002)
  • Cited by (92)

    • Hirschsprung's disease

      2022, Surgery (United Kingdom)
    • Routine botulinum toxin injection one month after a Swenson pull-through does not change the incidence of Hirschsprung associated enterocolitis

      2022, Journal of Pediatric Surgery
      Citation Excerpt :

      Hirschsprung associated enterocolitis (HAEC) is a condition of unknown etiology which can occur both before and after pull-through surgery. The broad definition of HAEC makes it challenging to determine its actual incidence, which ranges in the published literature between 5% and 42% [1–4]. The timing of surgery does not seem to be associated with a higher risk of developing postoperative HAEC, however, older age patients at diagnosis have less postoperative HAEC, which may point to a phenotypic difference [4,5].

    • What parents need to know about Hirschsprung disease

      2022, Seminars in Pediatric Surgery
      Citation Excerpt :

      If the surgeon plans on having the parents dilate the anastomosis in the postoperative period to prevent stricture formation, or if they perform dilations contingent upon assessment at a postoperative evaluation, this should then be explained. If there are no plans for postoperative dilations, it is prudent to discuss stricture as a complication that may require treatment, as it is reported in approximately 5% of cases but has been described at varying rates up to 33% in one series.7,8 Additionally, postoperative stricture has relevance for long term bowel function as well as risk of developing HAEC.9

    • Psychosocial factors affecting quality of life in patients with anorectal malformation and Hirschsprung disease-a qualitative systematic review

      2022, Journal of Pediatric Surgery
      Citation Excerpt :

      Positive parental responses led to overall improved quality of life in children with HSCR and ARM [4,20–32] and helped patients cope and adapt to chronic medical conditions [33]. Familial response influenced emotional and psychosocial development despite stooling difficulties or enterocolitis episodes in children with HSCR [19]. Similarly, other studies found that children with ARM developed more robust relationships with siblings than controls [34,35] and had no concerns related to self-expression or peer relationships [28].

    View all citing articles on Scopus
    View full text