Clinical features of the course of Hirschsprung’s disease in newborns and infants with consideration of the extent of colonic aganglionosis

Abstract

The clinical course of Hirschsprung’s disease (HD) in newborns and infants depends on the extent of сolonic aganglionosis, the age of patient, the presence of associated malformations and complications. The aim - to study the peculiarities of the clinical course of HD in newborns and infants. Materials and methods. An analysis of the clinical course of HD in 483 in newborns and infants. There were 100 (20.71%) patients with the rectal, 192 (39.75%) with rectosigmoid, 150 (31.05%) with subtotal, and 41 (8.49%) with total form of HD. 98 (20.29%) patients were found to have associated malformations. Results. 64 (13.25%) patients with a rectal form, 72 (14.91%) - with rectosigmoid, 150 (31.05%) - with subtotal, and 41 (8.49%) with total form had an acute clinical course and subacute clinical course was observed in 36 (7.45%) patients with rectal and 120 (24.85%) with rectosigmoid forms of HD. 327 (67.70%) infants with HD were diagnosed with varying degrees of hypotrophy, 315 (65.22%) had HD-associated enterocolitis, 16 (3.31%) had toxic megacolon, and 241 (49.89%) patients had varying degrees of anemia. Conclusions. In the presence of associated malformations in newborns and infants, in 8.07% of cases, clinical symptoms may prevail over the classic signs of HD. The severity of the course and late diagnosis are the main reasons for the appearance of severe complications of HD in newborns and infants - hypotrophy (67.70%), HD-associated enterocolitis (65.22%), toxic megacolon (3.31%) and anemia (49.89%). The research was carried out in accordance with the principles of the Declaration of Helsinki. The research protocol was approved by the Local Ethics Committee of all institutions mentioned in the work. Informed consent of the children’s parents was obtained for the research. No conflict of interests was declared by the authors.