Case Report

Constipation that needs attention: late Hirschsprung disease

Kristin Agustina , Ni Nyoman Margiani, Pande Putu Yuli Anandasari, Ni Made Mahastuti

Kristin Agustina
Radiology Resident, Faculty of Medicine, Universitas Udayana-Sanglah General Hospital, Bali-Indonesia. Email:

Ni Nyoman Margiani
Radiology Department, Faculty of Medicine, Universitas Udayana-Sanglah General Hospital, Bali-Indonesia

Pande Putu Yuli Anandasari
Radiology Department, Faculty of Medicine, Universitas Udayana-Sanglah General Hospital, Bali-Indonesia

Ni Made Mahastuti
Pathological Anatomy Department, Faculty of Medicine, Universitas Udayana-Sanglah General Hospital, Bali-Indonesia
Online First: April 01, 2021 | Cite this Article
Agustina, K., Margiani, N., Anandasari, P., Mahastuti, N. 2021. Constipation that needs attention: late Hirschsprung disease. Intisari Sains Medis 12(1): 64-67. DOI:10.15562/ism.v12i1.845

Background: Hirschprung disease (HD) or congenital aganglionic megacolon is an intestinal motor disorder that occurs in approximately 1 in 5000 live births. It makes colon fail to relax, mainly producing the symptoms of constipation. Approximately 90% of cases are diagnosed in the first year of life. Most of the remaining 10% are made in early childhood, with less than 1% being made in teenagers or adulthood.

Case report: A 13 years old girl reported to the pediatric surgical outpatient department complaining constipation since birth. There was no history of delayed passage of meconium. A contrast enema study with water-soluble contrast showed high probability of ultrashort segment HD. Histopathological examination revealed neither nerve fibers with ganglion cells nor hypertrophy of nerve fibers were seen confirming the aganglionosis. Surgical treatment was performed with good clinical progression.


Conclusion: Despite of its rarity, the possibility of HD should be considered in teenagers with chronic refractory constipation, especially when there was a history of delayed or non-passage of meconium after birth. An accurate diagnosis is mainly based on collective assessment of medical history, clinical examination, contrast enema study, and rectal biopsy as a gold standard.



Suleiman Lawal, Philip O Ibinaiye, Sefiya A Olarinoye-Akorede, Joseph B Igashi AHU. Case Report Late diagnosis of Hirschsprung ’ s disease. Published online 2017:70-73. doi:10.4103/ssajm.ssajm

Qiu J, Shi Y, Hu L, Fang L, Wang H, Zhang M. Case Report Adult Hirschsprung ’ s disease : report of four cases. 2013;6(8):1624-1630.

Prabhakaran S. Hirschsprung's disease. Int J Acad Med. 2017;3(3):96-100. doi:10.4103/IJAM.IJAM_12_17

Bakari AA, Gali BM, Ibrahim AG, et al. Congenital aganglionic megacolon in Nigerian adults: Two case reports and review of the literature. Niger J Clin Pract. 2011;14(2):249-252. doi:10.4103/1119-3077.84032

Estopiñan Rebollar R, Estopiñan Cánovas R, Pila Peláez R. Hirschsprung’s disease in an adult patient. Rev Colomb Gastroenterol. 2016;31(1):52-55. doi:10.22516/25007440.73

Alehossein M, Roohi A, Pourgholami M, Mollaeian M, Salamati P. Diagnostic Accuracy of Radiologic Scoring System for Evaluation of Suspicious Hirschsprung Disease in Children. Iran J Radiol. 2015;12(2):0-4. doi:10.5812/iranjradiol.12451

Garg SR, Sathe PA, Taware AC, Surve KM. Fatal toxic megacolon in a child of Hirschsprung disease. J Clin Diagnostic Res. 2016;10(12):ED03-ED05. doi:10.7860/JCDR/2016/21075.9083

Indonesian Ministry of Health. National Guide on Hrischprung. Kepmenkes HK0107/MENKES/474/2017. 2017;6:5-9. [Cited March 02nd 2020]. Available at:

Szylberg L, Marsza?ek A. Diagnosis of Hirschsprung’s disease with particular emphasis on histopathology. A systematic review of current literature. Prz Gastroenterol. 2014;9(5):264-269. doi:10.5114/pg.2014.46160

Swenson O, Bill AHJ. Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon; an experimental study. Surgery. 1948;24(2):212-220.

No Supplementary Material available for this article.
Article Views      : 251
PDF Downloads : 157