OUR EXPERIENCE IN TREATMENT OF HIRSCHSPRUNG'S DISEASE IN A 18-YEAR-OLD PATIENT. CLINICAL CASE
https://doi.org/10.38181/2223-2427-2020-4-47-52
Abstract
Introduction: Hirschsprung's disease (HD) is a relatively rare condition, in which the intermuscular (Auerbach) and submucosal (Meissner) plexus are absent in the colon wall at various lengths. In the literature, HD is described mainly in relation to the pediatric surgery area of interests because up to 90% of cases of this disease are detected before the age of 5 years 6. However, in some patients, the symptoms of the disease are not severe, so patients survive to adulthood without an established diagnosis.
Clinical case: we demonstrate our experience of surgical treatment of Hirschsprung's disease in a 18-year-old patient.
Conclusion: Although Hirschsprung's disease is in most cases a disease of childhood, it is necessary to keep in mind its typical symptoms when an adult patient complains of constipation. Proper diagnosis and correctly selected surgical treatment can not only eliminate symptoms and improve quality of life, but also reduce the likelihood of serious complications.
About the Authors
M. A. Agapov
Federal State Budget Educational Institution of Higher Education M.V. Lomonosov Moscow State University (Lomonosov MSU)
Russian Federation
Russian Federation
Mikhail A. Agapov – PhD, Professor of the Department of Surgery
Leninskie Gory St., 1, 119991, Moscow
D. R. Markaryan
Federal State Budget Educational Institution of Higher Education M.V. Lomonosov Moscow State University (Lomonosov MSU)
Russian Federation
Russian Federation
Daniil R. Markaryan – PhD, docent of the Department of Surgery
Leninskie Gory St., 1, 119991, Moscow
V. V. Kakotkin
Federal State Budget Educational Institution of Higher Education M.V. Lomonosov Moscow State University (Lomonosov MSU)
Russian Federation
Russian Federation
Viktor V. Kakotkin – Department of Surgery №1 of the Medical Research Educational Center
Leninskie Gory St., 1, 119991, Moscow
A. M. Lukyanov
Federal State Autonomous Educational Institution of Higher Education I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Aleхander M. Lukyanov – 6th year student of the Filatov Clinical Institute of Children's Health
Bolshaya Pirogovskaya St., 2/4, 119435, Moscow
V. A. Kubyshkin
Federal State Budget Educational Institution of Higher Education M.V. Lomonosov Moscow State University (Lomonosov MSU)
Russian Federation
Russian Federation
Valery A. Kubyshkin – PhD, academician of RSA, Head of the Surgery department
Leninskie Gory St., 1, 119991, Moscow
References
1. Duan, X. L., Zhang, X. S. & Li, G. W. Clinical relationship between EDN-3 gene, EDNRB gene and Hirschsprung’s disease. World J. Gastroenterol. 2003. 9, 2839–2842. doi: 10.3748/wjg.v9.i12.2839.
2. Passarge, E. & Bruder, E. Genetische grundlagen des morbus Hirschsprung. Pathologe. 2007. 28, 113–118. doi: 10.1007/s00292-007-0899-5.
3. Kusafuka, T. & Puri, P. Genetic aspects of Hirschsprung’s disease. Semin. Pediatr. Surg. 1998. 7, 148–155. doi: 10.1016/S1055-8586(98)70010-1.
4. Martucciello, G. Hirschsprung’s disease, one of the most difficult diagnoses in pediatric surgery: A review of the problems from clinical practice to the bench. European Journal of Pediatric Surgery. 2008. 18, 140–149. doi: 10.1055/s-2008-1038625.
5. De Lorijn, F., Boeckxstaens, G. E. & Benninga, M. A. Symptomatology, pathophysiology, diagnostic work-up, and treatment of Hirschsprung disease in infancy and childhood. Current Gastroenterology Reports. 2007. 9, 245–253. doi: 10.1007/s11894-007-0026-z.
6. Reategui, C. O., Spears, C. A. & Allred, G. A. Adults Hirschsprung’s disease, a call for awareness. A Case Report and review of the literature. Int. J. Surg. Case Rep. 2021. 79, 1–7. doi: 10.1016/j.ijscr.2021.01.090.
7. О.М.Бирюков., С.И. Ачкасов. Болезнь Гиршпрунга у взрослых (обзор литературы). Колопроктология, 2010, № 4 (34), 46-54. [Biryukov O.M., Achkasov S.I. Bolezn' Hirshprunga u vzroslyich (obzor literaturyi). Koloproctologya, 2010, № 4 (34), 46-54. (In Russ.)]
8. M J Wheatley, J R Wesley, A G Coran, T Z Polley Jr. Hirschsprung's disease in adolescents and adults. Dis Colon Rectu. 1990 Jul;33(7):622-9. doi: 10.1007/BF02052222
9. Devan Schlund, Sarah B Jochum, Joanne Favuzza, Dana M Hayden, Srikumar B Pillai, Theodore J Saclarides, Anuradha R Bhama. A national analysis of operative treatment of adult patients with Hirschsprung’s disease. Int. J. Colorectal Dis. 2020, 35, 169–172. doi: 10.1007/s00384-019-03442-8
10. Miyamoto, M., Egami, K., Maeda, S., Ohkawa, K., Tanaka, N., Uchida, E., & Tajiri, T. (2005). Hirschsprung’s Disease in Adults: Report of a Case and Review of the Literature. Journal of Nippon Medical School, 72(2), 113–120. doi:10.1272/jnms.72.113
11. Das, K., Mohanty, S. Hirschsprung Disease – Current Diagnosis and Management. Indian J. Pediatr. 2017, 84, 618– 623. doi: 10.1007/s12098-017-2371-8
12. Ahmad, T., Riaz, M., Khan, A. & Khan, M. M. Hirschsprung’s disease: A rare entity in adults. J. Coll. Physicians Surg. Pakistan. 2019, 29, 674–676. doi: 10.29271/jcpsp.2019.07.674
13. Vorobyov, G. I., Achkasov, S. I. & Biryukov, O. M. Clinical features’ diagnostics and treatment of Hirschsprung’s disease in adults. Color. Dis. 2010, 12, 1242–1248. doi: 10.1111/j.1463-1318.2009.02031.x
14. Luukkonen, P., Heikkinen, M., Huikuri, K. & Järvinen, H. Adult Hirschsprung’s disease – Clinical features and functional outcome after surgery. Dis. Colon Rectum. 1990, 33, 65–69. doi: 10.1007/BF02053205
15. R. Nakatake, Y. Hamada, H. Miki, T. Shirai, Y. Nakamura, H. Hamada, M. Ishizaki, M. Kon. A case of Hirschsprung's disease underwent surgery in adulthood, Journal of Pediatric Surgery Case Reports, 2016, Volume 13, 1-5. doi: 10.1016/j.epsc.2016.07.005
Review
For citations:
Agapov M.A., Markaryan D.R., Kakotkin V.V., Lukyanov A.M., Kubyshkin V.A. OUR EXPERIENCE IN TREATMENT OF HIRSCHSPRUNG'S DISEASE IN A 18-YEAR-OLD PATIENT. CLINICAL CASE. Surgical practice (Russia). 2021;(1):47-52. (In Russ.) https://doi.org/10.38181/2223-2427-2020-4-47-52
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