Colonic Hypoganglionosis in an Adult Female: A Case Report

Senthil VK; Somanathan Menon; Kumar Hari Rajah; Fitzgerald Henry

doi:10.64387/tjs.2026.276369

Authors

Senthil VK Taylor’s University School of Medicine and Health Science, Selangor
Somanathan Menon Taylor’s University School of Medicine and Health Science, Selangor
Kumar Hari Rajah Taylor’s University School of Medicine and Health Science, Selangor https://orcid.org/0000-0001-8424-2936
Fitzgerald Henry Taylor’s University School of Medicine and Health Science, Selangor

DOI:

https://doi.org/10.64387/tjs.2026.276369

Keywords:

Hypoganglionosis, Constipation, Abdominal distension, Colectomy

Abstract

Hypoganglionosis is a rare cause of intestinal obstruction characterized by a reduced number of ganglionic cells in the colon. Patients with this condition may present with constipation and acute intestinal obstruction. Based on the segment of the intestine affected, it can be classified into type one, in which the pathology is confined to a segment of the colon, and type two, in which the hypoganglionosis is widespread but less severe. In children, the condition can be isolated or associated with Hirschsprung’s disease. Hypoganglionosis is extremely rare in adults and is not easy to diagnose. We present this 39-year-old female patient who presented with symptoms of acute intestinal obstruction and was initially managed medically. Still, she ultimately underwent a total colectomy, and the diagnosis of colonic hypoganglionosis was made on histopathological evaluation. Her recovery was uneventful, and she has been symptom-free.

References

Meier-Ruge W. Epidemiology of congenital innervation defects of the distal colon. Virchows Arch A Pathol Anat Histopathol. 1992;420(2):171-7. doi: 10.1007/BF02358809.

Dingemann J, Puri P. Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect. Pediatr Surg Int. 2010;26(11):1111-5. doi: 10.1007/s00383-010-2693-3.

Lee A, Suhardja TS, Simpson I, et al. Rare case of adult intestinal hypoganglionosis and review of the literature. Clin J Gastroenterol. 2021;14(2):599-607. doi: 10.1007/s12328-021-01342-5.

Knowles CH, De Giorgio R, Kapur RP, et al. Gastrointestinal neuromuscular pathology: guidelines for histological techniques and reporting on behalf of the Gastro 2009 International Working Group. Acta Neuropathol. 2009;118(2):271-301. doi: 10.1007/s00401-009-0527-y.

Do MY, Myung SJ, Park HJ, et al. Novel classification and pathogenetic analysis of hypoganglionosis and adult-onset Hirschsprung's disease. Dig Dis Sci. 2011;56(6):1818-27. doi: 10.1007/s10620-010-1522-9.

Taguchi T, Masumoto K, Ieiri S, et al. New classification of hypoganglionosis: congenital and acquired hypoganglionosis. J Pediatr Surg. 2006;41(12):2046-51. doi: 10.1016/j.jpedsurg.2006.08.004.

Schärli AF, Sossai R. Hypoganglionosis. Semin Pediatr Surg. 1998;7(3):187-91. doi: 10.1016/s1055-8586(98)70016-2.

Kim HJ, Kim AY, Lee CW, et al. Hirschsprung disease and hypoganglionosis in adults: radiologic findings and differentiation. Radiology. 2008;247(2):428-34. doi: 10.1148/radiol.2472070182.

Zhang HY, Feng JX, Huang L, et al. Diagnosis and surgical treatment of isolated hypoganglionosis. World J Pediatr. 2008;4(4):295-300. doi: 10.1007/s12519-008-0053-3.

Kwok AM, Still AB, Hart K. Acquired segmental colonic hypoganglionosis in an adult Caucasian male: A case report. World J Gastrointest Surg. 2019;11(2):101-111. doi: 10.4240/wjgs.v11.i2.101.

Qadir I, Salick MM, Barakzai A, et al. Isolated adult hypoganglionosis presenting as sigmoid volvulus: a case report. J Med Case Rep. 2011;5:445. doi: 10.1186/1752-1947-5-445.

Crocker NL, Messmer JM. Adult Hirschsprung's disease. Clin Radiol. 1991;44(4):257-9. doi: 10.1016/s0009-9260(05)80191-6.

Wheatley MJ, Wesley JR, Coran AG, et al. Hirschsprung's disease in adolescents and adults. Dis Colon Rectum. 1990;33(7):622-9. doi: 10.1007/BF02052222.