Long-Term Functional Outcomes and Multidisciplinary Management after Ileorectal Duhamel Pull-Through for Total Colonic Aganglionosis—20-Year Experience in a Tertiary Surgical Center

Kailas Bhandarkar; Paolo De Coppi; Kate Cross; Simon Blackburn; Joe Curry

doi:10.1055/a-2181-2065

European Journal of Pediatric Surgery, Table of Contents

Eur J Pediatr Surg 2024; 34(05): 423-429
DOI: 10.1055/a-2181-2065

Original Article

Long-Term Functional Outcomes and Multidisciplinary Management after Ileorectal Duhamel Pull-Through for Total Colonic Aganglionosis—20-Year Experience in a Tertiary Surgical Center

Kailas Bhandarkar

¹Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom

,

Paolo De Coppi

¹Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom

,

Kate Cross

¹Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom

,

Simon Blackburn

¹Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom

,

Joe Curry

¹Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom

› Author Affiliations

Abstract

Introduction Management of patients with total colonic aganglionosis (TCA) is challenging for pediatric surgeons. The purpose of this study was to review our institution's 20-year experience regarding long-term outcomes and multidisciplinary team management of these patients after an ileorectal Duhamel pull-through procedure.

Materials and Methods Retrospective review was conducted for all patients diagnosed with TCA in our tertiary institution. Data were collected on demographics, clinical presentation, complications, need for additional surgery, and long-term effects on bowel function.

Results Of a total of 202 patients with Hirschsprung's disease, 13 were diagnosed with TCA (6.4%). Clinical presentation was variable. Eleven presented in neonatal period with distal bowel obstruction and two presented with constipation in early infancy. Ileorectal Duhamel pull-through was performed in all patients. Median follow-up was 13 years. Eleven are toilet trained, of whom five are fully continent. Six continue to have problems with bowel continence or constipation. One developed recurrent episode of Hirschsprung's associated enterocolitis. Two patients had stoma re-established. Patients experiencing difficulties in bowel function are jointly managed by a multidisciplinary team consisting of surgeons, gastroenterologists, pediatric psychologists, and clinical nurse specialists.

Conclusion TCA can be associated with significant long-term morbidity. Nearly half of the patients in this series have ongoing problems with bowel continence requiring a permanent stoma in some. Diligent follow-up coupled with inputs of a multidisciplinary team has greatly helped manage these complex patients in our institution.

Keywords

total colonic aganglionosis - Hirschsprung's disease - Hirschsprung's associated enterocolitis - ileorectal Duhamel pull-through

Full Text

References

References
1 Stenström P, Brautigam M, Borg H, Graneli C, Lilja HE, Wester T. Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis. J Pediatr Surg 2017; 52 (08) 1302-1307
2 Ieiri S, Suita S, Nakatsuji T, Akiyoshi J, Taguchi T. Total colonic aganglionosis with or without small bowel involvement: a 30-year retrospective nationwide survey in Japan. J Pediatr Surg 2008; 43 (12) 2226-2230
3 Moore SW, Zaahl M. Clinical and genetic differences in total colonic aganglionosis in Hirschsprung's disease. J Pediatr Surg 2009; 44 (10) 1899-1903
4 Cass DT, Myers N. Total colonic aganglionosis: 30 years' experience. Pediatr Surg Int 1987; 2: 68-75
5 Laughlin DM, Friedmacher F, Puri P. Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int 2012; 28 (08) 773-779
6 Hukkinen M, Koivusalo A, Merras-Salmio L, Rintala RJ, Pakarinen MP. Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis. J Pediatr Surg 2015; 50 (11) 1859-1864
7 Menezes M, Pini Prato A, Jasonni V, Puri P. Long-term clinical outcome in patients with total colonic aganglionosis: a 31-year review. J Pediatr Surg 2008; 43 (09) 1696-1699
8 Bischoff A, Levitt MA, Peña A. Total colonic aganglionosis: a surgical challenge. How to avoid complications?. Pediatr Surg Int 2011; 27 (10) 1047-1052
9 Stenström P, Kyrklund K, Bräutigam M. et al. Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood. BJS Open 2020; 4 (05) 943-953
10 Hoehner JC, Ein SH, Shandling B, Kim PC. Long-term morbidity in total colonic aganglionosis. J Pediatr Surg 1998; 33 (07) 961-965 , discussion 965–966
11 Martin LW. Surgical management of total colonic aganglionosis. Ann Surg 1972; 176 (03) 343-346
12 Duhamel B. Retrorectal and transanal pull-through procedure for the treatment of Hirschsprung's disease. Dis Colon Rectum 1964; 7: 455-458
13 Kimura K, Nishijima E, Muraji T, Tsugawa C, Matsumoto Y. A new surgical approach to extensive aganglionosis. J Pediatr Surg 1981; 16 (06) 840-843
14 Coran AG. A personal experience with 100 consecutive total colectomies and straight ileoanal endorectal pull-throughs for benign disease of the colon and rectum in children and adults. Ann Surg 1990; 212 (03) 242-247 , discussion 247–248
15 Rintala RJ, Lindahl HG. Proctocolectomy and J-pouch ileo-anal anastomosis in children. J Pediatr Surg 2002; 37 (01) 66-70
16 Sharif K, Beath SV, Kelly DA. et al. New perspective for the management of near-total or total intestinal aganglionosis in infants. J Pediatr Surg 2003; 38 (01) 25-28 , discussion 25–28
17 Barrena S, Andres AM, Burgos L. et al. Long-term results of the treatment of total colonic aganglionosis with two different techniques. Eur J Pediatr Surg 2008; 18 (06) 375-379
18 Escobar MA, Grosfeld JL, West KW. et al. Long-term outcomes in total colonic aganglionosis: a 32-year experience. J Pediatr Surg 2005; 40 (06) 955-961
19 Rintala RJ, Lindahl H. Is normal bowel function possible after repair of intermediate and high anorectal malformations?. J Pediatr Surg 1995; 30 (03) 491-494
20 Solari V, Piotrowska AP, Puri P. Histopathological differences between recto-sigmoid Hirschsprung's disease and total colonic aganglionosis. Pediatr Surg Int 2003; 19 (05) 349-354
21 Ikeda K, Goto S. Total colonic aganglionosis with or without small bowel involvement: an analysis of 137 patients. J Pediatr Surg 1986; 21 (04) 319-322
22 Moore SW. Total colonic aganglionosis in Hirschsprung disease. Semin Pediatr Surg 2012; 21 (04) 302-309
23 Marquez TT, Acton RD, Hess DJ, Duval S, Saltzman DA. Comprehensive review of procedures for total colonic aganglionosis. J Pediatr Surg 2009; 44 (01) 257-265 , discussion 265
24 Bonnard A, de Lagausie P, Leclair MD. et al. Definitive treatment of extended Hirschsprung's disease or total colonic form. Surg Endosc 2001; 15 (11) 1301-1304
25 Cheung ST, Tam YH, Chong HM. et al. An 18-year experience in total colonic aganglionosis: from staged operations to primary laparoscopic endorectal pull-through. J Pediatr Surg 2009; 44 (12) 2352-2354
26 Wildhaber BE, Teitelbaum DH, Coran AG. Total colonic Hirschsprung's disease: a 28-year experience. J Pediatr Surg 2005; 40 (01) 203-206 , discussion 206–207
27 Ludman L, Spitz L, Tsuji H, Pierro A. Hirschsprung's disease: functional and psychological follow up comparing total colonic and rectosigmoid aganglionosis. Arch Dis Child 2002; 86 (05) 348-351
28 Vilanova-Sanchez A, Halleran DR, Reck-Burneo CA. et al. A descriptive model for a multidisciplinary unit for colorectal and pelvic malformations. J Pediatr Surg 2019; 54 (03) 479-485
29 Rintala RJ, Pakarinen MP. Long-term outcomes of Hirschsprung's disease. Semin Pediatr Surg 2012; 21 (04) 336-343
30 Davidson JR, Kyrklund K, Eaton S. et al. Long-term surgical and patient-reported outcomes of Hirschsprung disease. J Pediatr Surg 2021; 56 (09) 1502-1511
31 Sood S, Lim R, Collins L. et al. The long-term quality of life outcomes in adolescents with Hirschsprung disease. J Pediatr Surg 2018; 53 (12) 2430-2434
32 Menezes M, Puri P. Long-term clinical outcome in patients with Hirschsprung's disease and associated Down's syndrome. J Pediatr Surg 2005; 40 (05) 810-812
33 Davidson JR, Kyrklund K, Eaton S. et al. Outcomes in Hirschsprung's disease with coexisting learning disability. Eur J Pediatr 2021; 180 (12) 3499-3507