Abstract
Purpose
Most patients with Hirschsprung’s disease (HD) have a satisfactory outcome after pull-through (PT) operation. However, some children continue to have persistent bowel symptoms after the initial operation and may require redo PT. Redo PT operation in HD is usually indicated for anastomotic strictures or residual aganglionosis (RA). We designed this meta-analysis to determine the incidence and outcome of RA among patients with HD following PT operation.
Methods
A meta-analysis of redo PT operations for HD reported in the literature between 1985 and 2011 was performed. Detailed information was recorded in patients with RA and transition-zone bowel (TZB), including recurrent bowel problems, histological findings on repeat rectal biopsy, type of redo PT operation and outcome.
Results
Twenty-nine articles reported 555 patients with redo PT operations. 193 (34.8%) patients demonstrated abnormal histological findings on rectal biopsy with 144 patients showing RA and 49 patients showing TZB. These 193 patients presented with persistent constipation (n = 135), recurrent enterocolitis (n = 45) and abnormal histology of the pulled-through bowel (n = 13). Mean age at redo PT was 4.4 years (range 4 months–17 years). Redo procedures were Duhamel (n = 57), transanal endorectal PT (n = 40), Soave (n = 35), Swenson (n = 10), posterior sagittal approach (n = 1) and not reported (n = 50). Follow-up information after redo PT was available in 134 (69.4%) patients and not available in 59 patients. Of the 134 patients, 99 (73.9%) patients had normal bowel habits, 19 patients had persistent constipation/soiling and 16 patients had recurrent enterocolitis.
Conclusion
This meta-analysis reveals that RA and TZB are the underlying causes of persistent bowel symptoms in one-third of all patients with HD requiring redo PT operation. Most patients have a satisfactory outcome after redo operation. Rectal biopsy should be performed in all patients with recurrent bowel problems after PT operation.
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Introduction
Pull-through (PT) operations for Hirschsprung’s disease (HD) generally result in a satisfactory outcome [1]. However, some patients after definitive PT operation have disturbances of bowel function such as constipation, enterocolitis and recurrent obstructive symptoms [2–9]. In the majority of these patients, residual bowel problems can be managed by non-surgical treatment such as laxatives, enemas or intrasphincteric botulinum toxin injection [10–12]. In a few patients, persistent abdominal distension, constipation or enterocolitis is due to postoperative stricture or retained aganglionic segment which may require a redo PT operation [13–17]. We designed this meta-analysis to determine the incidence and outcome of residual aganglionosis (RA) in patients with HD following PT operation.
Methods
PubMed® and MEDLINE® databases were searched for all studies that reported cases of patients with HD who had undergone redo PT operations for RA or transition-zone bowel (TZB) after initial operation between 1985 and 2011. The search terms were “residual aganglionosis”, “transition-zone bowel”, “redo and repeat pull-through”, “reoperation Hirschsprung’s disease” and “postoperative follow-up and outcome Hirschsprung’s disease”. The reference lists from retrieved articles were reviewed for additional cases. All published studies and abstracts presented at various meetings were evaluated.
Detailed information was recorded regarding type of study, gender, recurrent bowel symptoms, histological findings on repeat rectal biopsy, patient’s age at initial PT and redo PT operation, type of surgical procedures performed and postoperative follow-up with recurrent symptoms. Only publications containing all the relevant details were included in the literature review. Publications not giving adequate clinical data of patients were excluded.
Results
Between 1985 and 2011, 29 published articles reported 555 patients with HD who underwent redo PT operation. 193 (34.8%) patients demonstrated abnormal histological findings on repeat rectal biopsy. 144 (74.6%) patients revealed RA and 49 (25.4%) patients TZB (Table 1). 24 articles (82.8%) were from single centers, and 5 (17.2%) were from multicenter studies.
Information about patient’s gender was reported in 135 (69.9%) patients. A male-to-female ratio of 3.5:1 was observed. In 143 (74.0%) patients, the initial procedure was documented. 82 had a Soave procedure, 24 had a Duhamel procedure, 15 had a Rehbein procedure, 14 had a Swenson procedure, 7 had a transanal endorectal PT (TERPT), and 1 had a posterior sagittal approach. In 50 patients, the initial type of procedure was not reported.
In 13 (6.7%) patients redo PT operation was performed because of histological evidence of RA in the resected proximal margin of the pulled-through bowel. Of the remaining 180 patients, 135 had persistent abdominal distension/constipation and 45 had recurrent episodes of enterocolitis.
Patient’s age at redo PT operation was documented in 108 (56.0%) patients with a mean of 4.4 years (range 4 months–17 years). Time between initial PT and redo PT operation was reported in 74 (38.3%) patients with a mean of 2.8 years (range 6 months–8 years). In 143 (74.1%) patients, the redo procedure was documented. 57 had a Duhamel procedure, 40 had a TERPT, 35 had a Soave procedure, 10 had a Swenson procedure and 1 had a posterior sagittal approach. In 50 patients, the type of redo procedure was not reported.
Follow-up was reported in 134 (69.4%) patients with a mean follow-up time of 4.1 years (range 3 months–23 years). Of the 134 patients, 99 (73.9%) patients had normal bowel habits after redo PT operation. Nineteen patients had persistent or intermittent constipation with occasional soiling and 16 patients had recurrent enterocolitis with or without perianal excoriation. Except for occasional soiling most of the patients were fecally continent and had normal bowel movements. There was no significant difference in functional outcome between the various redo PT procedures.
Discussion
Over the years, various PT operations have been used to treat patients with HD [18–23]. It has been shown in several studies that there is no statistically significant difference in the functional outcome with respect to bowel function between the various PT procedures [24–27]. For a PT operation to be successful, it is essential that all aganglionic bowel is resected and bowel with normal innervations is anastomosed to the anus. Our meta-analysis reveals that RA and TZB are the underlying causes of persistent bowel symptoms in one-third of all patients requiring redo PT operation.
A redo PT operation for RA or TZB is potentially preventable by accurate identification of the proximal margin of the aganglionic bowel and transition-zone by an experienced histopathologist [28]. During frozen section analysis at the time of the initial PT operation, the pathologist must confirm normal ganglion cells and absence of nerve trunks at the site of the planned anastomosis. One major problem with the intraoperative frozen section biopsies is that it can indicate the presence of ganglion cells without differentiating between hypo- and dysganglionosis [29]. Shayan et al. [30] reported that 3% of 304 children who had intraoperative frozen section analysis during PT operation showed a discrepancy between the frozen section diagnosis and the final pathological diagnosis. The use of rapid technique of acetylcholinesterase staining may help overcome this problem [31, 32]. Another factor which may help prevent pulling-through the transition-zone for anastomosis is resecting several centimetres above the proximal ganglionic bowel identified by the pathologist during frozen sections.
Constipation after PT operation in the vast majority of patients can be managed by non-operative methods such as laxatives and enemas [10, 11]. Postoperative enterocolitis requires rectal irrigation with or without metronidazole prophylaxis [25]. However, if a patient continues to have persistent constipation, abdominal distension or recurrent episodes of enterocolitis, a full thickness rectal biopsy is indicated to rule out RA or TZB [33].
The presence of RA or TZB means that resection of this section of bowel may cure the patients of their recurrent symptoms. Thus, a redo PT is generally recommended for surgical management of RA. However, the choice of which procedure to use is far from being obvious. Any surgical technique may be considered, depending on conditions of each patient’s anatomy. Therefore, type of previous failed procedure, level of anastomosis, rectal blood supply and presence of fibrosis or inflammation in the perirectal pouches must be considered [34]. In the present meta-analysis, most patients with HD had normal bowel function after redo PT operation. The vast majority of patients had either Duhamel procedure, TERPT or Soave procedure for redo PT operation.
References
Fortuna RS, Weber TR, Tracy TF Jr, Silen ML, Cradock TV (1996) Critical analysis of the operative treatment of Hirschsprung’s disease. Arch Surg 131(5):520–524 (discussion 524–525)
Elhalaby EA, Teitelbaum DH, Coran AG, Heidelberger KP (1995) Enterocolitis associated with Hirschsprung’s disease: a clinical histopathological correlative study. J Pediatr Surg 30(7):1023–1026 (discussion 1026–1027)
Heij HA, de Vries X, Bremer I, Ekkelkamp S, Vos A (1995) Long-term anorectal function after Duhamel operation for Hirschsprung’s disease. J Pediatr Surg 30(3):430–432
Kleinhaus S, Boley SJ, Sheran M, Sieber WK (1979) Hirschsprung’s disease—a survey of the members of the Surgical Section of the American Academy of Pediatrics. J Pediatr Surg 14(5):588–597
Marty TL, Seo T, Matlak ME, Sullivan JJ, Black RE, Johnson DG (1995) Gastrointestinal function after surgical correction of Hirschsprung’s disease: long-term follow-up in 135 patients. J Pediatr Surg 30(5):655–658
Moore SW, Laing D, Kaschula RO, Cywes S (1994) A histological grading system for the evaluation of co-existing NID with Hirschsprung’s disease. Eur J Pediatr Surg 4(5):293–297
Polley TZ Jr, Coran AG, Wesley JR (1985) A ten-year experience with ninety-two cases of Hirschsprung’s disease. Including sixty-seven consecutive endorectal pull-through procedures. Ann Surg 202(3):349–355
Tariq GM, Brereton RJ, Wright VM (1991) Complications of endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg 26(10):1202–1206
West KW, Grosfeld JL, Rescorla FJ, Vane DW (1990) Acquired aganglionosis: a rare occurrence following pull-through procedures for Hirschsprung’s disease. J Pediatr Surg 25(1):104–108 (discussion 108–109)
Levitt MA, Martin CA, Olesevich M, Bauer CL, Jackson LE, Pena A (2009) Hirschsprung disease and fecal incontinence: diagnostic and management strategies. J Pediatr Surg 44(1):271–277 (discussion 277)
Menezes M, Corbally M, Puri P (2006) Long-term results of bowel function after treatment for Hirschsprung’s disease: a 29-year review. Pediatr Surg Int 22(12):987–990
Minkes RK, Langer JC (2000) A prospective study of botulinum toxin for internal anal sphincter hypertonicity in children with Hirschsprung’s disease. J Pediatr Surg 35(12):1733–1736
Langer JC (1999) Repeat pull-through surgery for complicated Hirschsprung’s disease: indications, techniques, and results. J Pediatr Surg 34(7):1136–1141
Teitelbaum DH, Coran AG (2003) Reoperative surgery for Hirschsprung’s disease. Semin Pediatr Surg 12(2):124–131
van Leeuwen K, Teitelbaum DH, Elhalaby EA, Coran AG (2000) Long-term follow-up of redo pull-through procedures for Hirschsprung’s disease: efficacy of the endorectal pull-through. J Pediatr Surg 35(6):829–833 (discussion 833–824)
Weber TR, Fortuna RS, Silen ML, Dillon PA (1999) Reoperation for Hirschsprung’s disease. J Pediatr Surg 34(1):153–156 (discussion 156–157)
Wilcox DT, Kiely EM (1998) Repeat pull-through for Hirschsprung’s disease. J Pediatr Surg 33(10):1507–1509
De la Torre-Mondragon L, Ortega-Salgado JA (1998) Transanal endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg 33(8):1283–1286
Duhamel B (1960) A new operation for the treatment of Hirschsprung’s disease. Arch Dis Child 35:38–39
Georgeson KE, Fuenfer MM, Hardin WD (1995) Primary laparoscopic pull-through for Hirschsprung’s disease in infants and children. J Pediatr Surg 30(7):1017–1021 (discussion 1021–1012)
Rehbein F (1958) Intraabdominal resection or rectosigmoidectomy (Swenson’s technic) in Hirschsprung’s disease. Chirurg 29(8):366–369
Soave F (1964) A new surgical technique for treatment of Hirschsprung’s disease. Surgery 56:1007–1014
Swenson O, Bill AH Jr (1948) Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon; an experimental study. Surgery 24(2):212–220
Baillie CT, Kenny SE, Rintala RJ, Booth JM, Lloyd DA (1999) Long-term outcome and colonic motility after the Duhamel procedure for Hirschsprung’s disease. J Pediatr Surg 34(2):325–329
Menezes M, Puri P (2006) Long-term outcome of patients with enterocolitis complicating Hirschsprung’s disease. Pediatr Surg Int 22(4):316–318
Saleh W, Rasheed K, Mohaidly MA, Kfoury H, Tariq M, Rawaf AA (2004) Management of Hirschsprung’s disease: a comparison of Soave’s and Duhamel’s pull-through methods. Pediatr Surg Int 20(8):590–593
Stensrud KJ, Emblem R, Bjornland K (2010) Functional outcome after operation for Hirschsprung disease—transanal vs. transabdominal approach. J Pediatr Surg 45(8):1640–1644
Pena A, Elicevik M, Levitt MA (2007) Reoperations in Hirschsprung disease. J Pediatr Surg 42(6):1008–1013 (discussion 1013–1004)
Ghose SI, Squire BR, Stringer MD, Batcup G, Crabbe DC (2000) Hirschsprung’s disease: problems with transition-zone pull-through. J Pediatr Surg 35(12):1805–1809
Shayan K, Smith C, Langer JC (2004) Reliability of intraoperative frozen sections in the management of Hirschsprung’s disease. J Pediatr Surg 39(9):1345–1348
Kobayashi H, Miyahara K, Kusafuka J, Yamataka A, Lane GJ, Sueyoshi N, Miyano T, Puri P (2007) A new rapid acetylcholinesterase staining kit for diagnosing Hirschsprung’s disease. Pediatr Surg Int 23(5):505–508
Kobayashi H, O’Briain DS, Hirakawa H, Wang Y, Puri P (1994) A rapid technique of acetylcholinesterase staining. Arch Pathol Lab Med 118(11):1127–1129
Levitt MA, Dickie B, Pena A (2010) Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg 19(2):146–153
Pini-Prato A, Mattioli G, Giunta C, Avanzini S, Magillo P, Bisio GM, Jasonni V (2010) Redo surgery in Hirschsprung disease: what did we learn? Unicentric experience on 70 patients. J Pediatr Surg 45(4):747–754
Liem NT, Hau BD, Thu NX (1995) The long-term follow-up result of Swenson’s operation in the treatment of Hirschsprung’s disease in Vietnamese children. Eur J Pediatr Surg 5(2):110–112
Langer JC, Fitzgerald PG, Winthrop AL, Srinathan SK, Foglia RP, Skinner MA, Ternberg JL, Lau GY (1996) One-stage versus two-stage Soave pull-through for Hirschsprung’s disease in the first year of life. J Pediatr Surg 31(1):33–36 (discussion 36–37)
Langer JC, Seifert M, Minkes RK (2000) One-stage Soave pull-through for Hirschsprung’s disease: a comparison of the transanal and open approaches. J Pediatr Surg 35(6):820–822
Aggarwal SK, Yadav S, Goel D, Sengar M (2002) Combined abdominal and posterior sagittal approach for redo pull-through operation in Hirschsprung’s disease. J Pediatr Surg 37(8):1156–1159
Langer JC, Durrant AC, de la Torre L, Teitelbaum DH, Minkes RK, Caty MG, Wildhaber BE, Ortega SJ, Hirose S, Albanese CT (2003) One-stage transanal Soave pullthrough for Hirschsprung disease: a multicenter experience with 141 children. Ann Surg 238(4):569–583 (discussion 583–565)
Keshtgar AS, Ward HC, Clayden GS, de Sousa NM (2003) Investigations for incontinence and constipation after surgery for Hirschsprung’s disease in children. Pediatr Surg Int 19(1–2):4–8
Farrugia MK, Alexander N, Clarke S, Nash R, Nicholls EA, Holmes K (2003) Does transitional zone pull-through in Hirschsprung’s disease imply a poor prognosis? J Pediatr Surg 38(12):1766–1769
Wildhaber BE, Pakarinen M, Rintala RJ, Coran AG, Teitelbaum DH (2004) Posterior myotomy/myectomy for persistent stooling problems in Hirschsprung’s disease. J Pediatr Surg 39(6):920–926 (discussion 920–926)
Langer JC (2004) Persistent obstructive symptoms after surgery for Hirschsprung’s disease: development of a diagnostic and therapeutic algorithm. J Pediatr Surg 39(10):1458–1462
Wester T, Zetterlind L, Fredin K, Olsen L (2006) Postoperative obstructive symptoms are common after Rehbein’s procedure for Hirschsprung’s disease. Eur J Pediatr Surg 16(2):100–103
Gobran TA, Ezzat A, Hassan ME, O’Neill J (2007) Redo transanal endorectal pull-through: a preliminary study. Pediatr Surg Int 23(2):189–193
Schweizer P, Berger S, Schweizer M, Holschneider AM, Beck O (2007) Repeated pull-through surgery for complicated Hirschsprung’s disease—principles derived from clinical experience. J Pediatr Surg 42(3):536–543
Hadidi A, Bartoli F, Waag KL (2007) Role of transanal endorectal pull-through in complicated Hirschsprung’s disease: experience in 18 patients. J Pediatr Surg 42(3):544–548
Obermayr F, Hacker HW, Bornemann A, Stern M, Fuchs J (2008) Redo-endorectal pull through following various pull through procedures in Hirschsprung’s disease. Langenbecks Arch Surg 393(4):493–499
Gad El-Hak NA, El-Hemaly MM, Negm EH, El-Hanafy EA, Abdel Messeh MH, Abdel Bary HH (2010) Functional outcome after Swenson’s operation for Hirshsprung’s disease. Saudi J Gastroenterol 16(1):30–34
Vu PA, Thien HH, Hiep PN (2010) Transanal one-stage endorectal pull-through for Hirschsprung disease: experiences with 51 newborn patients. Pediatr Surg Int 26(6):589–592
Lawal TA, Chatoorgoon K, Collins MH, Coe A, Pena A, Levitt MA (2011) Redo pull-through in Hirschprung’s disease for obstructive symptoms due to residual aganglionosis and transiti on zone bowel. J Pediatr Surg 46(2):342–347
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Friedmacher, F., Puri, P. Residual aganglionosis after pull-through operation for Hirschsprung’s disease: a systematic review and meta-analysis. Pediatr Surg Int 27, 1053–1057 (2011). https://doi.org/10.1007/s00383-011-2958-5
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DOI: https://doi.org/10.1007/s00383-011-2958-5