CLINICAL FEATURES OF THE COURSE OF HIRSCHSPRING'S DISEASE INCHILDREN OF THE FIRST YEAR.

The purpose 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. From 1980 to 2021, at the pediatric surgery clinic of the National Medical University named after O.O. Bogomolets 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, 483 children of the first year of life suffering from Hirschsprung's disease were examined and treated. 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 which 39 (8.07%) hadatypical 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%), clefts of the hard and soft palate in 9 (1.86%). 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%), delayed physiological weight gain against the background of nutritional insufficiency with the development of hypotrophy in 327 (67.70%) , vomiting of stagnant gastric and intestinal contents in 417 (86.34%). On the other hand, enterocolitis in 315 (65.22%), toxic megacolon in 16 (3.31%), and anemia of various degrees occurred 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 established: rectal in 100 (20.70%), rectosigmoid 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%) and congenital cyst of the right ovary in 1 (0.21%) patient. The clinical course was affected by concomitant malformations: incomplete bowel rotation in 10 (2.07%) and internal abdominal hernia in 2 (0.42%). The clinical manifestations and course of Hirschsprung's disease 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%).

RECONSTRUCTIVE FUNCTIONAL RESERVOIRS IN TREATMENT OF CHILDREN WITH AGANGLIONOSIS AFTER TOTAL COLECTOMY.

The object of the study is to develop optimal reconstructive functional reservoirs for the treatment of children with aganglionosis after total colectomy. Since 1980 till 2020 we have been treating 53 children up to 3 years with aganglionosis after total colectomy using optimal reconstructive surgery technique lying in the formation of a functional intestinal reservoir. Colostomy was performed as the first stage of surgical treatment of all children. Effective ways of restoring the integrity of the intestinal tract after a total colostomy with creating of functionally advantageous circumstances for reservoirs formation were presented as "J" - reservoirs (n=2); ileotransplant with lateral ileo-ileoanastomosis (n=34); ileotransplant with lateral ileo-colonoastomosis (n=7); ileorectal primary anastomosis (n=1) or entero-enteroanastomosis with an invagination valve (n=8) or implantation of an ileocecal valve (n=1). The colostomy was closed after 3-4 months. There were no results in the postoperative period. After 3 months the frequency of defecation often is 10-15 times a day, and after 1 year it changes to 2-4 times a day. All the children survived. The results of functional tests are good. Bowl function is tolerable. The radiographs show a formed rectum with a sufficient reservoir and normal colonization. Restoration of integrity of the intestinal tract with the formation of functionally advantageous reservoir in the form of double ileo-colotransplant and ileo-ileotransplant "side-to-side" is the best option for reconstructive surgery performing in children with agangliosis after total colectomy. Formation of functionally advantageous reservoir prevents impaired water-electrolyte balance, improves normobiosis, improves formation and accumulation of feces, slows down passage through the digestive tract and prevents the development of other metabolic disorders. A modernized surgical approach, which lies in creation of various options for functionally advantageous reservoir in order to eliminate effects of colon absence in children, contributes to facilitating the postoperative period, accelerating rehabilitation, improving social adaptation and patients quality of life.