Reoperation for Hirschsprung's disease

doi:10.1016/S0022-3468(99)90247-6

Journal of Pediatric Surgery

Volume 34, Issue 1, January 1999, Pages 153-157

https://doi.org/10.1016/S0022-3468(99)90247-6 Get rights and content

Abstract

Background/Purpose: Reoperation for Hirschsprung's disease traditionally has been used for patients with anastomotic leaks or stricture or with severe constipation from retained aganglionic segment or neuronal dysplasia, but there is little information regarding its use for other complications and the long-term outcome in these patients.

Methods: In a 23-year period, 107 infants and children underwent Soave (68 patients) or Duhamel (39 patients) pull-through procedures. The age at operation was newborn to 6 years (mean, 10 months). Eighty percent had aganglionosis limited to the rectosigmoid colon. Follow-up was by office visit or telephone (mean, 8.5 years).

Results: Twenty-three of the 68 patients with Soave pull-through (34%) underwent reoperation for intractable enterocolitis (10 patients, all 10 cured); anastomotic stenosis (four patients, three cured, one continued diversion); anastomotic leak (four patients, four cured); retained aganglionic segment (three patients, three cured); one necrosis of pull-through converted to Duhamel and cured; and one rectal prolapse that was diverted. Fifteen of the 39 patients with Duhamel procedure (38%) underwent reoperation for severe constipation (seven patients, six cured, one diverted); persistent rectal septum (four patients, 4 cured); and intractable enterocolitis (four patients, three cured, one diverted). Overall, 21 of 23 patients (91%) with reoperation after Soave procedures were cured, whereas 13 of 15 patients (87%) who underwent reoperation after Duhamel procedure were cured, and four patients remain diverted.

Conclusions: These data show that aggressive reoperation can result in a high cure rate in Hirschsprung's disease. Although there is no significant difference in the rate of reoperation after Duhamel and Soave procedures, the patients with Soave pull-through required more complex reoperations, with several requiring more than one procedure. An aggressive approach to reoperation in patients with Hirschsprung's disease clearly is justified.

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Cited by (46)

The anal canal is the fine line between “fecal incontinence and colitis” after a pull-through for Hirschsprung disease
2017, Journal of Pediatric Surgery
Citation Excerpt :
This patient is fecal incontinent. When there are residual aganglionosis and the anal canal is preserved, but there is a very short rectum, the Duhamel technique is a suitable method for a reoperation to avoid a low anastomosis over the anal canal and produce fecal incontinence [17–20]. In the group of patients with fecal incontinence, the histopathology in the rectal biopsy of the colon anastomosed in the “anal canal” is aganglionic in 3 patients and transitional zone in 2.
Fecal incontinence after a pull-through is associated with different factors, although damage to the anal canal seems to be the most important. The objective of this article is to identify the variables related to the presence of fecal control and colitis in a homogeneous group of children after pull-through.
A retrospective cross-sectional study was performed in patients with HD for evaluation of post-operative problems from May 2014 to November 2016. The patients (39) had a transanal approach and were divided into two groups: Group 1 patients with fecal continence and Group 2 patients with fecal incontinence.
Group 1 patients (13) had the anastomosis in the rectum, no damage to the anal canal, and a positive history of colitis. Group 2 (26) had the anastomosis at the skin, anoderm, pectinate line, or a combination of these and a negative history of colitis.
We demonstrated that patients with a technical error in the anastomosis have fecal incontinence, but not colitis. Preservation of the anal canal is associated with fecal control and colitis because it is a high-pressure zone. Education for proper identification of the anal canal during a pull-through is an absolute necessity.
Retrospective Comparative Study.
Level III.
Usefulness of posterior sagittal anorectoplasty for redo pull-through in complicated and recurrent Hirschsprung disease: Experience with a single surgical group
2017, Journal of Pediatric Surgery
To retrospectively examine 12 patients with Hirschsprung disease (HD) who underwent posterior sagittal anorectoplasty (PSARP) for various complications.
This study included patients with HD who underwent redo pull-through (PT) via PSARP at our institute between 2005 and 2014. The type of initial procedure, clinical presentations, indications, and functional results were analyzed. Postoperative excretory function was assessed using the Krickenbeck classification.
The study group comprised 9 boys and 3 girls (total, 12). Five patients were diagnosed with rectosigmoid aganglionosis, 5 with long segment aganglionosis, and 2 with total colonic aganglionosis. The primary operations performed on these patients included the Soave, Duhamel, Swenson, Rehbein, and Ikeda–Soper procedures. The interval between the primary operations and reoperation ranged from 5 months to 8 years (median, 3 years). The indications for PSARP were rectocutaneous fistulae (6 cases), frozen pelvis (5 cases), severe anastomotic stricture (3 cases), rectovaginal fistulae (2 cases), and hemorrhagic proctitis with an inflammatory polyp (1 case). All fistulae were repaired using PSARP; only one rectocutaneous fistula recurred and required two additional surgeries. Stricture and hemorrhagic proctitis were cured in all involved cases. Nine patients were followed up for 8 months to 10 years after PSARP surgery (average, 5.1 years). All 9 patients had voluntary bowel movements within 6 months after the last PSARP and stoma closure: 3 had normal bowel movement, while 6 had varying degrees of soiling, depending on the length of residual colon. None complained of constipation.
PSARP is useful for treating severe complications of failed PT in HD. Complex and recurrent rectocutaneous fistulae and frozen pelvis are the main indications for PSARP, while soiling is the most common surgical complication.
Redo pullthrough for Hirschsprung disease: A single surgical group's experience
2014, Journal of Pediatric Surgery
This study presents our surgical experience for redo-pullthrough (RedoPT) for Hirschsprung disease (HD). It reviews the patient's clinical outcomes and assesses stooling patterns after RedoPT.
A retrospective review of our institution's RedoPTs as well as one author's overseas cases was performed. Stooling scores were tabulated using an established survey tool and compared to primary PT matched patients.
Between 1974 and 2012, 46 individuals (52% males) underwent RedoPT, representing 3 percent of all HD pullthroughs. Median age at primary PT and RedoPT was 1 year (range 1 week–18 years) and 3.5 years (range 8 weeks–41 years), respectively. Indications for RedoPT were predominately for aganglionosis/transition zone pathology (71%); followed by stricture or an obstructing Duhamel pouch (19%), tight cuff (8%) and a twisted PT (4%). None were performed for an isolated clinical diagnosis of repeated bouts of enterocolitis. RedoPT surgical approach depended upon the initial pullthrough technique and any previous complications. Stooling scores were significantly (P < 0.05) worse in the RedoPT patients compared to the historically-matched group of children undergoing a primary PT for HD (5.5 ± 1.2 vs. 12.2 ± 1.4, primary PT versus RedoPT, respectively). When breaking down this total score into individual parameters, stooling pattern scores (1.0 ± 0.2 vs. 4.1 ± 0.4, P = 0.001) and enterocolitis scores (2.0 ± 0.4 vs. 4.2 ± 0.4, P = 0.001) were statistically worse in the RedoPT group. Patients in both groups had similar overall continence rates.
Appropriately selected children undergoing a RedoPT can achieve good results, with comparable continence rates to those undergoing a primary PT.
A new technique for redo operation after failed endoanal pull-through procedure for correction of Hirschsprung's disease
2014, Journal of Pediatric Surgery Case Reports
Citation Excerpt :
However, it is important to stress that in the three patients herein described, a huge colonic dilatation was noted in the contrast enema, with clinical intractability, no body weight gain, and frequent necessity of manual fecaloma remotion under general anesthesia. The main post-operative complications of TEPT procedure are due to anastomotic strictures, fistulas and incomplete colon resection in cases of pull-through of the transition zone, as shown by some recent revisions [7,10,11]. Diagnosis confirmation using histological analysis of the pulled-through area is always necessary [15].
A new technique for performing redo operations after transanal endorectal pullthrough in cases of complicated Hirschsprung's disease is presented. Three patients were operated on. At laparotomy, the left colic artery and vein were ligated and sectioned, and the communicating vascular arcade to the medium colic vessels was maintained. For resection of the left colon, all vessel ligations were performed very close to the colon wall to preserve the trunk and most distal branch of the inferior mesenteric vessels. A pouch of the distal previously pulled through colon was vascularized by the preserved branch of the inferior mesenteric vessels. A pull-through of a portion of the colon with an external normal appearance was performed behind the pouch according to Duhamel technique. The anastomosis between the pulled-through colon and the anus was not primarily performed, and a perineal stump of 3–4 cm of exteriorized colon was left to avoid any contamination to the peritoneal cavity. After 3 weeks, this stump was excised. All of the patients had an uneventful recovery with normal fecal continence. This is a new effective technique to be used for redo operation after failed endoanal pull-through procedure.
Hirschsprung-associated enterocolitis: Prevention and therapy
2012, Seminars in Pediatric Surgery
Hirschsprung-associated enterocolitis remains the greatest cause of morbidity and mortality in children with Hirschsprung disease. This chapter details the various approaches used to treat and prevent this disease process. This includes prevention of complications, such as stricture formation, prophylaxis with rectal washouts, and identification of high-risk individuals. The chapter also details approaches to diagnose Hirschsprung-associated enterocolitis as well as to exclude other etiologies.
Redo pull-through in Hirschprung's disease for obstructive symptoms due to residual aganglionosis and transition zone bowel
2011, Journal of Pediatric Surgery
Citation Excerpt :
Intestinal neuronal dysplasia (IND) [27] is frequently reported as responsible for the persistence of obstructive symptoms after an HD pull-through [28,29]. Yet others have found that IND is rarely associated with the problems that occur after the pull-through [1,7,30]. We suspect that many interpretations of IND are actually transition-zone pathology in HD [30] and would advocate for a reanalysis of these specimens with this concept of transition zone in mind.
Reoperations in Hirschsprung disease may be required for residual aganglionosis or transition-zone bowel found at the distal pull-through. We aimed to review the management of patients who had this complication and offer suggestions on how to avoid it.
Ninety-three patients with Hirschsprung disease were referred to our institution with recurrent problems after a pull-through done elsewhere. All required reoperations with a variety of indications, and of these, 25 had residual aganglionosis/transition-zone histology. This was the only indication for redo in 16 children.
Children (range, 2-17 years) presented 6 to 66 months after the initial pull-through. The predominant symptoms were enterocolitis (n = 9 [56%]), constipation (n = 7 [44%]), failure to thrive (n = 5 [31%]), and impaction (n = 4 [25%]). The rectal biopsy performed as part of their post pull-through work up showed hypertrophic nerves (n = 16), absent ganglion cells (n = 6), and normal ganglion cells (n = 10). The original frozen-section biopsy, determining the level of the pull-through, only sampled the seromuscular layer in 3 children, leading to misdiagnosis. Reoperations involved a transanal resection (n = 15) and a posterior sagittal approach (n = 1). In all cases, obstructive symptoms were resolved, and no patient has had recurrent enterocolitis.
Patients' post pull-through with recurrent obstructive symptoms may have residual aganglionosis or transition-zone bowel. Reoperation can result in the resolution of these symptoms. A full-thickness biopsy at the time of the initial pull-through to include the mucosa and submucosa may increase the possibility of identifying hypertrophic nerves.

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^☆: Presented at the 29th Annual Meeting of the American Pediatric Surgical Association, Hilton Head, South Carolina, May 10–13, 1998.

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Reoperation for Hirschsprung's disease☆

Abstract

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