Peculiarities of the Clinical Picture of Hirschsprung’s Disease in Children of the First Year of Life Taking Into Account the Extent of Aganglionosis

Abstract

The aim of the work. To investigate the peculiarities of the clinical course of Hirschsprung’s disease in children of the first year of life and to determine the significance of symptoms in the verification of the disease.
 Research materials and methods. Since 1980 up to 2021, at the pediatric surgery clinic of the National Medical University named after O.O. Bohomolets on the basis of the National Children’s Specialized Hospital “OKHMATDYT” and in the pediatric surgery clinic of the Ivano-Frankivsk National Medical University on the basis of the Ivano-Frankivsk Regional Children’s Clinical Hospital, there were examined and treated 483 children of the first year of life suffering from Hirschsprung’s disease.
 Results of the study. The clinical manifestation and course of aganglionosis varied in length at the time of hospitalization and depended on the time after birth. During the first month of life, 97 (20.08%) patients were hospitalized; of them 39 (8.07%) patients had an atypical clinical picture due to: colonic atresia in 15 (3.10%), colonic atresia + gastroschisis in 3 ( 0.62%), ileal atresia in 9 (1.86%), esophageal atresia in 3 (0.62%), cleft of the hard and soft palate in 9 (1.86%) patients. Depending on the age, there were 280 (57.97%) patients under 6 months, and 203 (42.03%) patients between 6 months and 1 year. The classic typical clinical picture was in 444 (91.93%) patients, which was characterized by the absence of meconium excretion, abdominal distension in 444 (91.93%) patients, delayed physiological weight gain against the background of nutritional insufficiency with the development of hypotrophy in 327 (67.70%) patients, vomiting of stagnant gastric and intestinal contents in 417 (86.34%) patients. On the other hand, there occurred enterocolitis in 315 (65.22%) patients, toxic megacolon in 16 (3.31%) patients, and anemia of various degrees in 241 (49.89%) patients, among the complications that arose during the examination of patients with Hirschsprung’s disease. According to the results of a comprehensive examination, the following extent of aganglionosis was determined: rectal form in 100 (20.70%) patients, rectosigmoid form – in 192 (39.75%), subtotal – in 150 (31.06%) and total in 41 (8.49%) patients. Concomitant malformations were found in 98 (20.29%) patients: renal malformations were diagnosed in 7 (1.45%) patients, concomitant heart malformations in 18 (3.73%) patients. Associated intraoperative findings were: Meckel’s diverticulum in 5 (1.03%) patients, and congenital cyst of the right ovary in 1 (0.21%) patient. The clinical course was affected by the concomitant malformations: incomplete bowel rotation in 10 (2.07%) patients and internal abdominal hernia in 2 (0.42%) patients.
 Conclusions. Clinical manifestations and course of HD primarily depend on the presence of accompanying developmental defects, which may prevail during the examination due to vital disorders. In the clinical course of Hirschsprung’s disease, it is necessary to distinguish between typical and atypical forms. Typical clinical symptoms were in 444 (91.93%), and atypical in 39 (8.07%) patients.