International Journal of Colorectal Disease

, Volume 34, Issue 2, pp 255–259 | Cite as

Optimal time for single-stage pull-through colectomy in infants with short-segment Hirschsprung disease

  • Tianqi Zhu
  • Xiaoyi Sun
  • Mingfa Wei
  • Bin Yi
  • Xiang Zhao
  • Wenjing Wang
  • Jiexiong FengEmail author
Original Article



Short-segment Hirschsprung disease (HSCR) is the predominant type of HSCR that affects approximately 75% of patients. Whether single-stage endorectal pull-through (ERPT) surgery is appropriate for neonatal patients with HSCR has not been definitively determined. This retrospective cohort study concerning infants with short-segment HSCR investigated the optimal age for single-stage ERPT surgery, regardless of the operative approach.


The 198 patients were stratified by operative age ≤ 3 or > 3 months (groups A or B, respectively, n = 62 and 136, respectively). Diagnoses of short-segment HSCR were conducted by preoperative contrast enema and rectal suction biopsy with acetylcholinesterase immunohistochemical staining. The perioperative clinical course for all patients was reviewed and the accuracy rate of the preoperative diagnoses and postoperative short- and midterm outcomes were assessed.


The rates of diagnostic accuracy, according to the results of the preoperative contrast enema or rectal suction biopsy, were lower in group A (67.2 and 93.5%, respectively) than in group B (81.4 and 94.9%, respectively). In groups A and B, 49 (79.1%) and 108 (79.4%) infants, respectively, completed follow-up examinations. The short-term outcomes were postoperative HSCR-associated enterocolitis, adhesive bowel obstruction, anastomosis leakage, and anal stenosis during the first 12 months after surgery. The midterm outcomes were incontinence and constipation at ~24 months after surgery. Compared with group B, group A experienced more incidences of anastomotic leakage in the short-term and more soiling in the midterm. In groups A and B, the rates of constipation recurrence were nil and 1.9%, respectively.


Infants with HSCR ≤3 months old at the time of single-stage ERPT surgery showed lower rates of accurate and conclusive diagnostic results and poorer postoperative outcomes. Waiting to perform this surgery until infants are older might be more beneficial.


Short-segment Hirschsprung disease Infants Colectomy Enterocolitis 


Funding information

This study was supported by Grants from the National Natural Science Foundation of China (No. 81700448).


  1. 1.
    Puri P (2005) Hirschsprung disease and allied disorders. Springer, Berlin, pp 15–18Google Scholar
  2. 2.
    de Lorijn F, Boeckxstaens GE, Benninga MA (2007) Symptomatology, pathophysiology, diagnostic work-up, and treatment of Hirschsprung disease in infancy and childhood. Curr Gastroenterol Rep 9(3):245–253CrossRefGoogle Scholar
  3. 3.
    Zani A, Eaton S, Morini F, Puri P, Rintala R, Heurn EV, Lukac M, Bagolan P, Kuebler JF, Friedmacher F, Wijnen R, Tovar JA, Hoellwarth ME, Pierro A, EUPSA Network Office (2017) European paediatric surgeons’ association survey on the management of Hirschsprung disease. Eur J Pediatr Surg 27(1):96–101CrossRefGoogle Scholar
  4. 4.
    Demehri FR, Halaweish IF, Coran AG, Teitelbaum DH (2013) Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention. Pediatr Surg Int 29(9):873–881CrossRefGoogle Scholar
  5. 5.
    Langer JC (2012) Laparoscopic and transanal pull-through for Hirschsprung disease. Semin Pediatr Surg 21(4):283–290CrossRefGoogle Scholar
  6. 6.
    Kim HY, Oh JT (2009) Stabilization period after 1-stage transanal endorectal pull-through operation for Hirschsprung disease. J Pediatr Surg 44(9):1799–1804CrossRefGoogle Scholar
  7. 7.
    Lu C, Hou G, Liu C, Geng Q, Xu X, Zhang J, Chen H, Tang W (2017) Single-stage transanal endorectal pull-through procedure for correction of Hirschsprung disease in neonates and nonneonates: a multicenter study. J Pediatr Surg 52(7):1102–1107CrossRefGoogle Scholar
  8. 8.
    Chen X, Xiaojuan W, Zhang H, Jiao C, Yu K, Zhu T, Feng J (2017) Diagnostic value of the preoperatively detected radiological transition zone in Hirschsprung’s disease. Pediatr Surg Int 33(5):581–586CrossRefGoogle Scholar
  9. 9.
    Gosain A, Frykman PK, Cowles RA et al (2017) Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int 33(5):517–521CrossRefGoogle Scholar
  10. 10.
    Wang H, Guo XN, Zhu D, Zhu T, Hu LH, Feng JX (2015) Nursing intervention for outpatient rehabilitation in pediatric patients with Hirschsprung disease after colectomy. Eur J Pediatr Surg 25(5):435–440CrossRefGoogle Scholar
  11. 11.
    Zhu T, Feng J, Zhang W, Wei M, Yu D, Zhang X, Yu K, Kuang H (2013) Subtotal colectomy with a single-incision laparoscopic surgery technique in children with long-segment Hirschsprung disease and allied disorders. Pediatr Surg Int 29(2):197–201CrossRefGoogle Scholar
  12. 12.
    Putnam LR, John SD, Greenfield SA, Kellagher CM, Austin MT, Lally KP, Tsao K (2015) The utility of the contrast enema in neonates with suspected Hirschsprung disease. J Pediatr Surg 50(6):963–966CrossRefGoogle Scholar
  13. 13.
    Rouzrokh M, Khaleghnejad AT, Mohejerzadeh L, Heydari A, Molaei H (2010) What is the most common complication after one-stage transanal pull-through in infants with Hirschsprung’s disease? Pediatr Surg Int 26(10):967–970CrossRefGoogle Scholar
  14. 14.
    Gourlay DM (2013) Colorectal considerations in pediatric patients. Surg Clin North Am 93(1):251–272CrossRefGoogle Scholar
  15. 15.
    de Arruda LP, Takegawa BK, Ortolan EV et al (2014) Does calretinin immunohistochemistry reduce inconclusive diagnosis in rectal biopsies for Hirschsprung disease? J Pediatr Gastroenterol Nutr 58(5):603–607CrossRefGoogle Scholar
  16. 16.
    Meinds RJ, Kuiper GA, Parry K, Timmer A, Groen H, Heineman E, Broens PMA (2015) Infant’s age influences the accuracy of rectal suction biopsies for diagnosing of Hirschsprung’s disease. Clin Gastroenterol Hepatol 13(10):1801–1807CrossRefGoogle Scholar
  17. 17.
    Friedmacher F, Puri P (2016) Current practice patterns of rectal suction biopsy in the diagnostic work-up of Hirschsprung’s disease: results from an international survey. Pediatr Surg Int 32(8):717–722CrossRefGoogle Scholar
  18. 18.
    Hayes CE, Kawatu D, Mangray S, LeLeiko NS (2012) Rectal suction biopsy to exclude the diagnosis of Hirschsprung disease. J Pediatr Gastroenterol Nutr 55(3):268–271CrossRefGoogle Scholar
  19. 19.
    Bradnock TJ, Walker GM (2011) Evolution in the management of Hirschsprung’s disease in the UK and Ireland: a national survey of practice revisited. Ann R Coll Surg Engl 93(1):34–38CrossRefGoogle Scholar
  20. 20.
    Langer JC (2013) Hirschsprung disease. Curr Opin Pediatr 25:368–374CrossRefGoogle Scholar
  21. 21.
    Frykman PK, Short SS (2012) Hirschsprung-associated enterocolitis: prevention and therapy. Semin Pediatr Surg 21(4):328–335CrossRefGoogle Scholar
  22. 22.
    Teitelbaum DH, Cilley RE, Sherman NJ, Bliss D, Uitvlugt ND, Renaud EJ, Kirstioglu I, Bengston T, Coran AG (2000) A decade of experience with the primary pull-through for hirschsprung disease in the newborn period: a multicenter analysis of outcomes. Ann Surg 232(3):372–380CrossRefGoogle Scholar
  23. 23.
    Neuvonen MI, Kyrklund K, Lindahl HG, Koivusalo AI, Rintala RJ, Pakarinen MP (2015) A population-based, complete follow-up of 146 consecutive patients after transanal mucosectomy for Hirschsprung disease. J Pediatr Surg 50(10):1653–1658CrossRefGoogle Scholar
  24. 24.
    Levitt MA, Dickie B, Pena A (2012) The Hirschsprungs patient who is soiling after what was considered a “successful” pull-through. Semin Pediatr Surg 21(4):344–353CrossRefGoogle Scholar
  25. 25.
    Langer JC, Rollins MD, Levitt M et al (2017) Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 33(5):523–526CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Tianqi Zhu
    • 1
  • Xiaoyi Sun
    • 1
  • Mingfa Wei
    • 1
  • Bin Yi
    • 1
  • Xiang Zhao
    • 1
  • Wenjing Wang
    • 1
  • Jiexiong Feng
    • 1
    Email author
  1. 1.Department of Pediatric Surgery, Tongji HospitalHuazhong University of Science and TechnologyWuhanChina

Personalised recommendations