DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20200678

Profile of Hirschsprung’s disease cases diagnosed in tertiary care teaching hospital: a retrospective observational study

S. Prabakaran, K. Kasthuri Thilagam

Abstract


Background: To date, there are very few studies of Hirschsprung’s disease. Hence, the study was conducted to profile Hirschsprung’s disease in a tertiary care setting.

Methods: This is a retrospective observational study that evaluated the admitted patients with Hirschsprung’s disease in the pediatric surgery department. Biopsy was taken from the patient in the form of the full thickness of intestine, seromuscular biopsy, resection from the colostomy site when doing closure and or appendix was taken and subjected to histopathological study with routine eosin and haematoxylin stain.

Results: Among the study population, 21(28.8%) children were aged less than one month, and only 10(13.7%) were aged 11 years and above. There were 45(61.6%) participants were male and 28(38.4%) female. Most common biopsy site was appendix in 33(46.2%), followed by Ileum full-thickness biopsy in 6(8.2%) , Ileum Seromuscular biopsy in 5(6.8%%) ,Colon full-thickness biopsy in 4(5.6%) subjects. Among the 73 participants, 46(63%) were clinically suspected cases, and the remaining 27(36.9%) were clinically established cases. Among clinically suspected, the majority (50.7%) had ganglionic cells only in proximal segments.

Conclusions: the Hirschsprung's disease diagnosis was established mostly in younger males.


Keywords


Case series, Clinical profiling, Epidemiology, Hirschsprung’s disease

Full Text:

PDF

References


Mabula JB, Kayange NM, Manyama M, Chandika AB, Rambau PF, Chalya PL. Hirschsprung’s disease in children: a five-year experience at a University teaching hospital in northwestern Tanzania. BMC Res Notes. 2014 Dec 1;7(1):410.

Zaafouri H, Mrad S, Mabrouk M, Haoues N, Salah MBH, Noomene R, et al. Hirschsprung’s disease in adults: Clinical and therapeutic features. Int Res J Surg. 2015;2(1):008-17.

Baraket O, Karray O, Ayed K, Baccar A, Moussa M, Triki W, et al. Hirschsprung’s Disease in Adult Treated by Coloprotectomy and Colo-Anal Anastomosis. J Case Rep Stud. 2016;4(2):210.

Ikoma N. Colonic perforation in a case of adult Hirschsprung's disease with acetylcholinesterase-negative neuronal fibers. Am Surg. 2013 Mar 1;79(3):E130.

Adhi M, Khan S, Zafar H, Arshad M. Duhamel's procedure for adult Hirschsprung's disease. J Coll Phys Surg Pakistan. 2012;22(6):395.

Qiu JF, Shi YJ, Hu L, Fang L, Wang HF, Zhang MC. Adult Hirschsprung’s disease: report of four cases. Inter J Clin Exper Pathol. 2013;6(8):1624.

Martins MR, dos Santos CHM, Falcão GR. Late diagnosis of Hirschsprung's disease. J Coloproctol. 2015;35(3):178-81.

Siddiqui K, Choudhary HA, Khan AFA. Adult Hirschsprung’s Disease; a case report of total Colonic Aganglionosis (Zuelzer-Wilson Syndrome). PJMHS. 2015;9(2):785-8.

Moore S. Hirschsprung disease: current perspectives. Open Acc Surg. 2016;9:39-50.

Green HL, Rizzolo D, Austin M. Surgical management for Hirschsprung disease: A review for primary care providers. J Am Acad PAs. 2016 Apr 1;29(4):24-9.

Duess JW, Hofmann AD, Puri P. Prevalence of Hirschsprung’s disease in premature infants: a systematic review. Pediatr Surg Inter. 2014 Aug 1;30(8):791-5.

Bradnock TJ, Knight M, Kenny S, Nair M, Walker GM. Hirschsprung's disease in the UK and Ireland: incidence and anomalies. Archiv Dis Childhood. 2017 Aug 1;102(8):722-7.

Granéli C, Dahlin E, Börjesson A, Arnbjörnsson E, Stenström P. Diagnosis, symptoms, and outcomes of Hirschsprung’s disease from the perspective of gender. Surg Res Pract. 2017;2017.

Anuras S, Hade JE, Soffer E, Lu CC, Loening-Baucke V, Soper RT, et al. Natural history of adult Hirschsprung's disease. J Clini Gastroenterol. 1984 Jun;6(3):205-10.

Wang TY, Lin TC, Hsu H. Hirschsprung's disease manifested with obstructive colitis in adult: a case report. Zhonghua Yi Xue Za Zhi (Taipei). 1996;58(6):444-7.

Croaker GD, Harvey JG, Cass DT. Hirschsprung's disease, colonic atresia, and absent hand: a new triad. J Pediatr Surg. 1997 Sep 1;32(9):1368-70.

Hofmann AD, Duess JW, Puri P. Congenital anomalies of the kidney and urinary tract (CAKUT) associated with Hirschsprung’s disease: a systematic review. Pediatr Surg Inter. 2014;30(8):757-61.

Kyrklund K, Neuvonen MI, Pakarinen MP, Rintala RJ. Social morbidity in relation to bowel functional outcomes and quality of life in anorectal malformations and Hirschsprung's disease. Eur J Pediatr Surg. 2018 Dec;28(06):522-8.

O'donovan AN, Habra G, Somers S, Malone DE, Rees A, Winthrop AL. Diagnosis of Hirschsprung's disease. AJR. Am J Roentgenol. 1996 Aug;167(2):517-20.