Long-term results of treatment of Hirschsprung’s disease
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
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Cited by (92)
Anal canal and sphincter function in children with Hirschsprung disease after definitive surgery
2023, Journal of Pediatric SurgeryHirschsprung'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 SurgeryCitation 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 SurgeryCitation 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
Evaluation and treatment of the post pull-through Hirschsprung patient who is not doing well; Update for 2022
2022, Seminars in Pediatric SurgeryPsychosocial factors affecting quality of life in patients with anorectal malformation and Hirschsprung disease-a qualitative systematic review
2022, Journal of Pediatric SurgeryCitation 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].