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Correlation between the number of Interstitial cells of Cajal (ICC) and defecation pattern in patients with Hirschsprung's disease after Duhamel's surgery at General Hospital Dr. Soetomo

  • Yohanes Santosa Honggowarsito ,
  • Adria Hariastawa ,
  • Fendy Matulatan ,
  • Alphania Rahniayu ,


Background: Hirschsprung is a congenital disorder of the distal bowel caused by the absence of a ganglion in the Aurbach and Meissner plexuses. Definitive treatment of the disorder is operative, namely by resection of the aganglionic segment of the intestine and performing a pull-through on the ganglionic segment. It cannot be denied that there are still disturbances in the pattern of defecation after definitive surgery, even though the pull-through segment has enough ganglion cells. Interstitial cells of Cajal (ICC) are the pacemaker in smooth muscle contraction in the intestine. In several studies, the number of these cells decreased in Hirschsprung's disease (HD), and it is not known with certainty the effect on the occurrence of defecation pattern disturbances.

Methods: Data were collected retrospectively from all patients with Hirschsprung’s Disease (HD) who underwent Duhamel Procedure in Dr. Soetomo General Hospital, starting January 2016 – December 2021. Correlation analytic research with a cross sectional design was performed to analyze the correlation between the number of ICC and the defecation pattern of patients at Dr. Soetomo after Duhamel surgery.

Results: From this study, it was found a decrease in the number of ICC in the ganglionic segments of the resected specimens from pull-through surgery. From the results of the correlation test, it was found that the p-value was 0.49 for the defecation pattern of postoperative Duhamel patients at Dr. Soetomo for the period January 2016 – December 2021.

Conclusion: In this study, a significant correlation was found between the number of ICC and definitive pull-through postoperative defecation pattern.


  1. Jacob CL. Hirschsprung Disease. Holcomb and Ashcraft’s Pediatric Surgery, 7th Edition. Elsevier. 2020.
  2. Taghavi K, Goddard L, Evans SM, et al. Ethnic variations in the childhood prevalence of Hirschsprung disease in New Zealand. ANZ J Surg. 2019;89(10):1246-1249. doi:10.1111/ans.14857.
  3. Khazdouz M, Sezavar M, Imani B, Akhavan H, Babapour A, Khademi G. Clinical outcome and bowel function after surgical treatment in Hirschsprung's disease. Afr J Paediatr Surg. 2015;12(2):143-147. doi:10.4103/0189-6725.160403.
  4. Rolle U, Piotrowska AP, Nemeth L, Puri P. Altered distribution of interstitial cells of Cajal in Hirschsprung disease. Arch Pathol Lab Med. 2002;126(8):928-933. doi:10.5858/2002-126-0928-ADOICO.
  5. Mostafa RM, Moustafa YM, Hamdy H. Interstitial cells of Cajal, the Maestro in health and disease. World J Gastroenterol. 2010;16(26):3239-3248. doi:10.3748/wjg.v16.i26.3239.
  6. Chen X, Zhang H, Li N, Feng J. Pathological changes of interstitial cells of Cajal and ganglion cells in the segment of resected bowel in Hirschsprung's disease. Pediatr Surg Int. 2016 Nov;32(11). p1019-1024. doi:10.1007/s00383-016-3961-7.
  7. Henna N. Expression of Interstitial Cells of Cajal in Colorectum of Patients with Hirschsprung’s Disease. Biomedica. 2017;33(2). p124-127.
  8. Gfroerer S, Rolle U. Interstitial cells of Cajal in the normal human gut and in Hirschsprung disease. Pediatr Surg Int. 2013;29(9):889-897. doi:10.1007/s00383-013-3364-y.
  9. Menezes M, Corbally M, Puri P. Long-term results of bowel function after treatment for Hirschsprung's disease: a 29-year review. Pediatr Surg Int. 2006;22(12):987-990. doi:10.1007/s00383-006-1783-8.
  10. Aravind KL, Nisha N, Sushmitha R, and Madiwal C. Duhamel’s procedure for Hirschsprung’s disease and the functional outcome in a tertiary care center. Indian Journal of Child Health. 2021;8(1). p51–55.
  11. Rolle U, Piaseczna-Piotrowska A, Puri P. Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood. Pediatr Surg Int. 2007;23(12):1139-1152. doi:10.1007/s00383-007-2022-7.
  12. van den Berg MM, Di Lorenzo C, Mousa HM, Benninga MA, Boeckxstaens GE, Luquette M. Morphological changes of the enteric nervous system, interstitial cells of cajal, and smooth muscle in children with colonic motility disorders. J Pediatr Gastroenterol Nutr. 2009;48(1):22-29. doi:10.1097/MPG.0b013e318173293b.
  13. Rintala RJ, Lindahl H. Is normal bowel function possible after repair of intermediate and high anorectal malformations?. J Pediatr Surg. 1995;30(3):491-494. doi:10.1016/0022-3468(95)90064-0.
  14. Taguchi T, Suita S, Masumoto K, Nagasaki A. An abnormal distribution of C-kit positive cells in the normoganglionic segment can predict a poor clinical outcome in patients with Hirschsprung's disease. Eur J Pediatr Surg. 2005;15(3):153-158. doi:10.1055/s-2005-837612.

How to Cite

Honggowarsito, Y. S., Hariastawa, A., Matulatan, F., & Rahniayu, A. (2023). Correlation between the number of Interstitial cells of Cajal (ICC) and defecation pattern in patients with Hirschsprung’s disease after Duhamel’s surgery at General Hospital Dr. Soetomo. Bali Medical Journal, 12(1), 675–677.




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Yohanes Santosa Honggowarsito
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Adria Hariastawa
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Fendy Matulatan
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Alphania Rahniayu
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