Urological Complications of Hirschsprung-Related Crohn’s Disease: A Case Report

Authors

  • Kartika Anastasia Kosasih Department of Internal Medicine, Faculty of Medicine Universitas Indonesia, Jakarta, Indonesia
  • Saut Horas Hatoguan Nababan Division of Hepato-biliary, Department of Internal Medicine, Faculty of Medicine Universitas Indonesia - Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia
  • Andrian Setiabakti Department of Internal Medicine, Faculty of Medicine Universitas Indonesia, Jakarta, Indonesia

Keywords:

Chron’s disease, Hirschsprung disease, hydronephrosis, ureteral obstruction

Abstract

Hirschsprung disease (HSCR) is a rare congenital intestinal disease characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. Individuals with HSCR demonstrate a higher risk for inflammatory bowel disease (IBD), with Chron’s disease (CD) commonly observed. Renal and urinary involvement is reported by between 4 and 23% of IBD patients, which manifests as urinary calculi, fistulas, and ureteral obstruction, which causes hydronephrosis. Those conditions can lead to a predisposition to recurrent urinary tract infections (UTIs) and should be suspected in male patients with IBD. A 26-year-old male with a history of HSCR and multiple surgeries presented with recurrent UTIs over 3 months. Upon further evaluation, he was found to have hydronephrosis in both kidneys. An MRI of the abdomen with contrast showed thickening and fibrosis in contact with the posterior wall of the rectum, causing a narrowing of the bilateral ureter. This clinical case has been reported to raise awareness of urological complications in CD patients with a history of HSCR, with recurrent UTIs as the presenting symptom.

References

Montalva L, Cheng LS, Kapur R, et al. Hirschsprung disease. Nat Rev Dis Primers. 2023;9(1):54.

Löf Granström A, Amin L, Arnell H, Wester T. Increased risk of inflammatory bowel disease in a population‐based cohort study of patients with Hirschsprung disease. J Pediatr Gastroenterol Nutr. 2018;66(3):398–401.

Nakamura H, Lim T, Puri P. Inflammatory bowel disease in patients with Hirschsprung’s disease: a systematic review and meta-analysis. Pediatr Surg Int. 2018;34(2):149–54.

Bernstein CN, Kuenzig ME, Coward S, et al. Increased incidence of inflammatory bowel disease after Hirschsprung disease: a population-based cohort study. J Pediatr. 2021;233:98-104.e2.

Granström AL, Ludvigsson JF, Wester T. Clinical characteristics and validation of diagnosis in individuals with Hirschsprung disease and inflammatory bowel disease. J Pediatr Surg. 2021;56(10):1799–802.

Ambruzs JM, Larsen CP. Renal manifestations of inflammatory bowel disease. Rheum Dis Clin. 2018;44(4):699–714.

Jansen R, Zaslau S. Hydronephrosis and ureteral obstruction in Crohn’s disease. Open J Urol. 2013;03(05):219–21.

Akram W, Shah SK, Sohail M, Rehman U, Rahim M. Recurrent urinary tract infection in a patient with asymptomatic Crohn’s disease. Cureus. 2020;12(8):e9962.

Sutthatarn P, Lapidus-Krol E, Smith C, et al. Hirschsprung-associated inflammatory bowel disease: a multicenter study from the APSA Hirschsprung disease interest group. J Pediatr Surg. 2023;58(5):856–61.

Löf Granström A, Amin L, Arnell H, Wester T. Increased risk of inflammatory bowel disease in a population‐based cohort study of patients with Hirschsprung disease. J Pediatr Gastroenterol Nutr. 2018;66(3):398–401.

Kim HY, Kim TW. Crohn’s disease with ankylosing spondylitis in an adolescent patient who had undergone long ileo-colonic anastomosis for Hirschsprung’s disease as an infant. Intest Res. 2017;15(1):133.

Dray X, Sokol H, Vahedi K, Lavergne-Slove A, Marteau P. Pure ileal Crohnʼs disease without colonic involvement after a long ileo-colonic anastomosis (Lester Martin procedure) for Hirschsprungʼs disease: an argument favoring a specific sensitivity of the ileum in a subset of patients with Crohnʼs disease. Inflamm Bowel Dis. 2007;13(2):243–4.

Verde A, Grammegna A, Petrone E, et al. Crohn-like colitis in a young boy with Hirschsprung disease. Inflamm Bowel Dis. 2021;27(9):e112–3.

Chang JT. Pathophysiology of inflammatory bowel diseases. N Engl J Med. 2020;383(27):2652–64.

McGregor CGC, Tandon R, Simmons A. Pathogenesis of fistulating Crohn’s disease: a review. Cell Mol Gastroenterol Hepatol. 2023;15(1):1–11.

Minordi LM, Bevere A, Papa A, Larosa L, Manfredi R. CT and MRI evaluations in Crohn’s complications: a guide for the radiologist. Acad Radiol. 2022;29(8):1206–27.

Stroie FA, Hasan OM, Houlihan MD, et al. Low diagnostic sensitivity of cystoscopy and cystography of surgically confirmed vesicoenteric fistulae. Int Urol Nephrol. 2020;52(7):1203–8.

Angelberger S, Fink KG, Schima W, et al. Complications in Crohnʼs disease: right-sided ureteric stenosis and hydronephrosis. Inflamm Bowel Dis. 2007;13(8):1056–7.

Elabbassi T, Bachar A, Moufakkir A, El Berni Y, Yaqine K, Lefriyekh MR. Particularities of urological complications in Crohn’s disease: Report of two cases. Annals of Medicine & Surgery. 2022;79:103903.

Aslam S, Albo M, Brubaker L. Recurrent urinary tract infections in adult women. JAMA. 2020;323(7):658.

Murray BO, Flores C, Williams C, et al. Recurrent urinary tract infection: a mystery in search of better model systems. Front Cell Infect Microbiol. 2021;11.

Kar M, Dubey A, Patel S, Siddiqui T, Ghoshal U, Sahu C. Characteristics of bacterial colonization and urinary tract infection after indwelling of the double-J ureteral stent and percutaneous nephrostomy tube. J Glob Infect Dis. 2022;14(2):75.

Mert D, Iskender G, Kolgelier S, Ertek M. Evaluation of risk factors, causative pathogens, and treatment in recurrent percutaneous nephrostomy catheter-related urinary tract infections in cancer patients. Medicine. 2023;102(14):e33002.

Anger J, Lee U, Ackerman AL, et al. Recurrent uncomplicated urinary tract infections in women: AUA/CUA/SUFU guideline. J Urol. 2019;202(2):282–9.

Taxonera C, Barreiro-de-Acosta M, Bastida G, et al. Outcomes of medical and surgical therapy for entero-urinary fistulas in Crohn’s disease. J Crohns Colitis. 2016;10(6):657–62.

Solitano V, Dal Buono A, Gabbiadini R, et al. Fibro-stenosing Crohn’s disease: what is new and what is next? J Clin Med. 2023;12(9):3052.

de Campos-Lobato LF, Kiran RP. Surgical management of complex enteric fistulas in Crohn’s disease. Clin Colon Rectal Surg. 2019;32(04):268–72.

Downloads

Published

2025-04-15

How to Cite

Kosasih, K. A., Nababan, S. H. H., & Setiabakti, A. (2025). Urological Complications of Hirschsprung-Related Crohn’s Disease: A Case Report. Acta Medica Indonesiana, 57(1), 107. Retrieved from https://actamedindones.org/index.php/ijim/article/view/2868