Investigation of the Distribution of Ganglion Cells in the Appendix According to Location: A Cohort Study

Document Type : Original Article(s)

Authors

1 Associate Professor, Department of Anatomical and Clinical Pathology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

2 Resident of Surgery, Isfahan University of Medical Sciences, Isfahan, Iran

3 Associate Professor, Department of Surgery, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

4 Associate Professor, Department of Obstetrics and Gynecology, Shahrekord University of Medical Sciences, Shahrekord, Iran

5 Anatomical and clinical Pathology, Mazandaran University of Medical Sciences, Mazandaran, Iran

6 Assistant professor of public health, Isfahan University of Medical sciences, Isfahan, Iran

Abstract

Background: Hirschsprung's disease (HD) is a congenital absence of ganglion cells. Due to the lack of consensus about the possibility of relying on the histology of the appendix to diagnose HD, appendectomy and sampling of the appendix are still performed in many centers in Iran and the world to diagnose it. This study aimed to evaluate the possibility of partial appendectomy and prevent total appendectomy to diagnose HD and determine the prevalence of aganglionosis in each part.
Methods: The present study was a prospective cohort study. The study population consisted of patients under 16 years of age in need of appendectomy who were referred to Isfahan teaching hospitals from March 2022 to March 2023. The inclusion criteria were age below 16 years and undergoing appendectomy with the diagnosis of appendicitis based on the Alvarado criteria, and the exclusion criteria were perforated or gangrene appendix and appendix neoplasia. The research variables included age and gender (as background variables) and the number of ganglion cells in each hpf (as dependent variable).
Findings: The average age of the examined patients was 8.84 ± 3.33 years. 58% of the patients were male. The total number of ganglion cells seen in the entire slide containing the samples was the highest in the middle and the lowest in the distal slides, which were significantly different (P = 0.038).
Conclusion: The middle part of the appendix has the highest number of ganglion cells and is the best part for biopsy for the HD diagnosis.

Highlights

Maryam Soltan: Google Scholar, PubMed

Mohammad Javad Yazdani: Google Scholar 

Keywords

Main Subjects


  1. Chen F, Winston 3rd JH, Jain SK, Frankel WL. Hirschsprung's disease in a young adult: report of a case and review of the literature. Ann Diagn Pathol 2006; 10(6): 347-51.
  2. Bhandarkar K, De Coppi P, Cross K, Blackburn S, Curry J. Long-term functional outcomes and multidisciplinary management after ileorectal duhamel pull-through for total colonic aganglionosis-20-year experience in a tertiary surgical center. Eur J Pediatr Surg 2023
  3. Nayak K, Khedkar K, Gattani RG, Shinde RK, Durge S, Patel D. A rare case presentation on total colonic aganglionosis in a female infant of indian origin. Cureus 2023; 15(12): e49847.
  4. Amiel J, Sproat-Emison E, Garcia-Barcelo M, Lantieri F, Burzynski G, Borrego S, et al. Hirschsprung disease, associated syndromes and genetics: a review. J Med Genet 2008; 45(1): 1-14.
  5. Mc Laughlin D, Friedmacher F, Puri P. Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int 2012; 28(8): 773-9.
  6. Eshel Fuhrer A, Govorukhina O, Becker G, Ben-Shahar Y, Moran-Lev H, Sukhotnik I. Extended total colonic aganglionosis and total intestinal aganglionosis: Challenging enemies. Acta Paediatr 2024.
  7. Collins L, Collis B, Trajanovska M, Khanal R, Hutson JM, Teague WJ, et al. Quality of life outcomes in children with Hirschsprung disease. J Pediatr Surg 2017; 52(12): 2006-10.
  8. Wildhaber BE, Teitelbaum DH, Coran AG. Total colonic Hirschsprung's disease: a 28-year experience. J Pediatr Surg 2005; 40(1): 203-6.
  9. Bischoff A, Levitt MA, Pena A. Total colonic aganglionosis: a surgical challenge. How to avoid complications? Pediatr Surg Int 2011; 27(10): 1047-52.
  10. Langer JC, Fitzgerald PG, Winthrop AL, Srinathan SK, Foglia RP, Skinner MA, et al. One-stage versus two-tage Soave pull-through for Hirschsprung's disease in the first year of life. Journal of pediatric surgery 1996; 31(1): 33-7.
  11. Hanani M. Multiple myenteric networks in the human appendix. Auton Neurosci 2004; 110(1): 49-54.
  12. Shayan K, Smith C, Langer JC. Reliability of intraoperative frozen sections in the management of Hirschsprung’s disease. J Pediatr Surg 2004; 39(9): 1345-8.
  13. Kamoshita S, Landing BH. Distribution of lesions in myenteric plexus and gastrointestinal mucosa in lipidoses and other neurologic disorders of children. Am J Clin Pathol 1968; 49(3): 312-8.
  14. O’Donnell A-M, Puri P. Skip segment Hirschsprung’s disease: a systematic review. Pediatr Surg Int 2010; 26(11): 1065-9.
  15. Anderson KD, Chandra R. Segmental aganglionosis of the appendix. J Pediatr Surg 1986; 21(10): 852-4.
  16. Miller SM, Narasimhan RA, Schmalz PF, Soffer EE, Walsh RM, Krishnamurthi V, et al. Distribution of interstitial cells of Cajal and nitrergic neurons in normal and diabetic human appendix. Neurogastroenterol Motil 2008; 20(4): 349-57.
  17. N-Fékété C, Ricour C, Martelli H, Jacob SL, Pellerin D. Total colonic aganglionosis (with or without ileal involvement): a review of 27 cases. J Pediatr Surg 1986; 21(3): 251-4.
  18. Shaw PA. The innervation and neuroendocrine cell population of the appendix in total colonic aganglionosis. Histopathology 1990; 17(2): 117-21.
  19. Lane VA, Levitt MA, Baker P, Minneci P, Deans K. The appendix and aganglionosis. A note of caution—how the histology can mislead the surgeon in total colonic hirschsprung disease. European J Pediatr Surg Rep 2015; 3(1): 3-6.
  20. Shih TY, Chuang JH, Huang CC. Aganglionosis of the appendix: is it reliable for diagnosis of total colonic aganglionosis? J Pediatr Gastroenterol Nutr 1998; 27(3): 353-4.
  21. Singh UR, Malhotra A, Bhatia A. Eosinophils, mast cells, nerves and ganglion cells in appendicitis. Indian J Surg 2008; 70(5): 231-4.
  22. Miyano G, Morita K, Tsuboi K, Kosaka S, Takahashi T, Yamada S, et al. Changes in postoperative quality of life of pediatric total colonic aganglionosis patients: effect of pull-through technique. Pediatr Surg Int 2022; 38(12): 1867-72.
  23. Wood RJ, Garrison AP. Total colonic aganglionosis in hirschsprung disease. Semin Pediatr Surg 2022; 31(2): 151165.
  24. Naiem MEA, Hilal N, Elgurashi MEA, Suliman SH. Total colonic aganglionosis with ileum extension Hirschsprung's disease in an adult: A case report. Int J Surg Case Rep 2022; 92: 106852.
  25. O'Hare T, McDermott M, O'Sullivan M, Dicker P, Antao B. A retrospective cohort study of total colonic aganglionosis: is the appendix a reliable diagnostic tool? J Neonatal Surg 2016; 5(4): 44.
  26. Mohanty S, Kini U, Das K, Puttegowda D, Yadav L, Babu MK, et al. Appendicular biopsy in total colonic aganglionosis: a histologically challenging and inadvisable practice. Pediatr Dev Pathol 2017; 20(4): 277-87.