O.Yu. KARPUKHIN^1,2, M.N. NASYBULLIN³, E.R. KHASANOV¹, B.Sh. BIKBOV¹

¹Kazan State Medical University, 49 Butlerov Str., Kazan, Russian Federation, 420012

²Republican Clinical Hospital of the MH of RT, 138 Orenburgskiy Trakt, Kazan, Russian Federation, 420064

³Children’s Republic Clinical Hospital of the MH of RT, 140 Orenburgskiy Trakt, Kazan, Russian Federation, 420064 

Karpukhin O.Yu. ― D. Med. Sc., Professor of the Department of Surgical Diseases №1, curator of Coloproctology Inpatient Unit, tel. (843) 269-00-53, e-mail: oleg_karpukhin@mail.ru

Nasybullin M.N. ― surgeon of Surgical Department №1, tel. (843) 237-30-43, e-mail: marat.doctor@mail.ru

Khasanov E.R. ― student of Pediatrics Faculty, tel. +7-937-528-02-59, e-mail: khasael95@gmail.com

Bikbov B.Sh. ― student of Pediatrics Faculty, tel. +7-953-495-99-63, e-mail: bulat1994bikbov@gmail.com 

The article presents the results of a retrospective analysis of medical records of patients with Hirschsprung’s disease who were treated at the Republic Clinical Hospital and Children’s Republic Clinical Hospital (Tatarstan) over the period from 2005 to 2014. The specificity of distribution of the anatomical forms of lesions, the prevalence of disease by gender and age, and the features of modern diagnostics and treatment of this congenital anomaly are presented. The diversity of anatomical forms of colonic aganglionosis was typical for pediatric patients with the dominance of rectosigmoid (52%) and rectal (32%) forms. In adults the short aganglionic segment in distal part of the rectum dominated (90%). Most often Hirschsprung’s disease was found in males (children ― 82.7%, adults ― 85%) and diagnosed at an early age ― from birth to 7 years of age. Due to diversity of the causes of chronic constipation syndrome in adults, the diagnosis of Hirschsprung’s disease is more difficult to state and requires implementation of the modern diagnostic techniques. The choice of surgery for Hirschsprung’s disease is partly due to the specificity of pathological changes in the gut. In children the most frequently performed operation was Soave in modification of De La Torre-Mondragon (74.7%), in adults ― Duhamel in modification of State Scientific Center of Coloproctology, Moscow (100%). The postoperative period was complicated in 12% of cases of the first group and in 20% of the second group. Postoperative mortality is not recorded in any of the groups.

Key words: Hirschsprung’s disease, different age groups, clinical and statistical parallels.

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