Features of the clinic, diagnosis and tactics of surgical treatment of Hirshprung's disease in infants

Abstract

The aim is to present optimal ways of early diagnosis and tactics of Hirschsprung's disease (HD) surgical correction in newborns and young children based on literature data and our own experience.

Materials and methods. Clinical observations on early diagnosis and optimization of the tactics of surgical treatment of HD in 58 infants over the past 9 years have been carried out. All patients underwent a complex of general physical examinations, including the collection of anamnesis and follow-up of patients, clinical examination with rectal examination, laboratory, bacteriological, radiological (survey, polypositional irrigography) and morphological (intraoperatively remote area of the colon agangliosis zone) studies. The features of the clinical course and tactics of surgical treatment of HD in infants, the choice of the surgical method were analyzed, and the risk of complications during the operation was studied. The patients were divided into two groups. The first group included 32 (55.2%) infants with a typical clinical course of HD, radiographically confirmed agangliosis in the rectosigmoid zone, and chronic constipation. The second group included 26 (44.8%) infants with an atypical clinical course of HD with diarrheal syndrome against the background of dysbiosis, enterocolitis, among them there were 2 children with symptoms of Hirschsprung-associated enterocolitis. Research methods: General clinical and biochemical analysis of blood and urine. Scatological examination of feces, sowing on the pathogenic flora of feces and urine. EKG. Ultrasound examination of the abdominal cavity and echocardiography. X-ray examination: an overview roentgenogram of the abdominal and thoracic cavity vertically, irrigoraphy, excretory urography.

Results and conclusions. In newborns with sub- and decompensated typical course of chronic constipation in the absence of the effect of conservative therapy, after the diagnosis of HD is established, an early radical operation or colostomy application above the agangliosis zone is recommended. In infants with a favorable typical clinical course and a left-sided form of HD, transanal endorectal reduction of the large intestine (TENTC) can be performed according to indications, starting from one month of age. In this case, the operation of choice is TENTK without colostomy. In subtotal and rectosigmoidal forms of HD with a long aganglionic zone, laparoscopic assistance is recommended, which makes it possible to release the splenic flexure of the colon from ligaments and constrictions, take a full-thickness biopsy of the transitional zone of aganglionicosis, and this makes it possible to conduct express diagnostics, establish resection levels and freely lower the proximal area colon during TENTC.

The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institution. The informed consent of the patient was obtained for conducting the studies.

No conflict of interest was declared by the authors.