DIAGNOSING HIRSCHSPRUNG DISEASE IN CHILDREN: A MULTICENTER RESEARCH TRIAL
Authors:   I.M. Sechenov First Moscow State Medical University, Moscow, Russia, Morozov D.A. ,   G.N. Speransky City Children’s Hospital No. 9, Moscow, Russia, Pimenova E.S. ,   I.M. Sechenov First Moscow State Medical University, Moscow, Russia,   G.N. Speransky City Children’s Hospital No. 9, Moscow, Russia, Poddoubnyi I.V. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia,   Moscow State University of Medicine and Dentistry named after A.I. Evdokimov, Moscow, Russia, Kozlov M.Y. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia, Sharkov S.M. ,   I.M. Sechenov First Moscow State Medical University, Moscow, Russia,   Morozov Children’s City Clinical Hospital, Moscow, Russia, Talalaev A.G. ,   Morozov Children’s City Clinical Hospital, Moscow, Russia,   Pirogov Russian National Research Medical University, Moscow, Russia, Okulov E.A. ,   National Medical Research Center for Children’s Health, Moscow, Russia, Fomenko O.Y. ,   Ryzhikh National Medical Research Centre for Coloproctology of the Ministry of Health of Russia, Moscow, Russia, Lokhmatov M.M. ,   I.M. Sechenov First Moscow State Medical University, Moscow, Russia,   Morozov Children’s City Clinical Hospital, Moscow, Russia, Korchagina N.S. ,   G.N. Speransky City Children’s Hospital No. 9, Moscow, Russia,   Pirogov Russian National Research Medical University, Moscow, Russia,   N.F. Filatov Moscow City Children's Clinical Hospital of the Moscow Department of Healthcare, Moscow, Russia, Kezhenbaeva K.M. ,   G.N. Speransky City Children’s Hospital No. 9, Moscow, Russia, Eremin D.B. ,   G.N. Speransky City Children’s Hospital No. 9, Moscow, Russia, Klementyev M.V. ,   G.N. Speransky City Children’s Hospital No. 9, Moscow, Russia, Tarasova D.S. ,   I.M. Sechenov First Moscow State Medical University, Moscow, Russia,   G.N. Speransky City Children’s Hospital No. 9, Moscow, Russia, Zagrebina A.A. ,   I.M. Sechenov First Moscow State Medical University, Moscow, Russia
Published: March 2021

Hirschsprung disease (HD) is a congenital absence of nerve cells (ganglions) in a segment of the intestinal wall, leading to its obstruction. Diagnosis of BG in children is a labor-intensive process, on which surgical treatment tactics depend. The concentration of patients in large surgical centers with equipped diagnostic departments makes it possible to develop the most adequate examination plan while minimizing the risk of diagnostic errors. The aim of the study was to analyze methods for diagnosis of HD in children; a retrospective analysis of the records of patients hospitalized in three medical organizations in Moscow (n=201) in 2017–2019 was carried out. The first group – children with confirmed diagnosis (n=152), the second group – patients with suspicion of BG with unsatisfactory results of previous operative treatment, postoperative complications (n=49). Results: in the 1st group, irrigography was performed before hospitalization in 118 patients (77,6%). In the hospitals, irrigography was performed (repeated) for 109 (71,7%) children. 25 (16,4%) patients showed discrepancies in interpretation of the results. Thus, irrigography was applied to all patients. Intestine biopsy before hospitalization was performed in 79 patients (52%), in the hospitals – performed/repeated in 50 patients (32,9%). There were discrepancies in the interpretation of histological findings in 8 patients (18,6 per cent). Thus, in the diagnosis of HD in children, biopsies were used (before hospitalization and/or during hospitalization) in 89 patients (58,6%). Anorectal manometry was performed to 3 (2%) children. In the 2nd group a histological examination was performed (before and during hospitalization) in 41 patients (84%), anorectal manometry – in 15 patients (31%), irrigography – in all children. Correlation analysis did not reveal any relationship between the HD variant and manifestation symptoms (Spearman's correlation coefficient was –0,232 at p<0,05. Conclusion: all medical teams began examining children with suspected HD with irrigography. Various intestinal biopsies were performed in 58,6% of cases. Anorectal manometry is currently practically not used in the diagnosis of HD in children, although it is a promising non-invasive method with high sensitivity and specificity.