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Hirschsprungʼs Disease as a Cause of Chronic Constipation in Children

Khabibulla AkilovRepublican Research Center of Emergency Medicine, Tashkent, UzbekistanFarhod SaidovRepublican Research Center of Emergency Medicine, Tashkent, UzbekistanNigora HodjimuhamedovaRepublican Research Center of Emergency Medicine, Tashkent, Uzbekistan
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Purpose: The purpose of this study was to investigate the results of treatments for Hirschsprung's Disease (HD) in children. Methods: From 2006 to 2012, we treated 70 children with (HD). The patients included 42 (60%) boys and 28 (40%) girls aged 3 months to 14 years; the majority, 50 (71.4%), of the patients were 3-14 years of age. All the patients experienced chronic constipation. The patients underwent complex diagnostic investigations that included: a detailed anamnesis, general and proctologic examinations, laboratory monitoring, fibrocolonoscopy, irrigography, the passage of a barium meal through the gastrointestinal tract, multislice computed tomography, diagnostic ultrasonography, intestinal flora analysis and an anal muscle contractile function assessment. After these evaluations, there was a suspicion of pathology in the rectosigmoid area. Later, all the children underwent irrigography by Svenson to update their diagnoses. Results: On the basis of the above investigations, HD was confirmed in 13 children with rectosigmoid stenosis and in one child with anal zonestenosis. After the refinement of the diagnosis, these children underwent surgery: Svenson-Khiat-Isakov performed abdominoperineal proctoplasty on 10 patients, Soave performed a Valeo modification in one case of (HD), and one patient underwent anoplasty. All the children underwent control irrigography with barium and large intestine ultrasound sonogram 6 months, 1 year, 2 years and 3 years after the surgery. The following preoperative measurements were recorded. In all the children, the diameter of the upper area of the large intestine was 25-44% larger than normal. In addition, a 16-46% stenosis of the retrorectal area due to the enlarged large intestine diameter was noted. There was a small degree of anal canal length reduction (11% on average) and larger increases in the area of the rectosigmoid and the sigmoid compared with the norm. Six months and 1 year after the surgery, the lower parts of the large intestine were not enlarged. Constipation recidivation andencopresis were observed in one child only. The children who attended follow-up visits two and three years after the operations no longer exhibited increased large intestine diameters, constipation recidivation or encopresis. During the postoperative period, all the patients received conservative therapy every 6 months until the disappearance of clinical symptoms and the establishment of increasingly regular bowel movements. Nine (69.2%) patients required such treatment for 6 months and four (30.8%) for one year after surgery. Conclusion: Specialized consultations with gastroenterologists and pediatric surgeons and complex diagnostic evaluations allow timely diagnoses and improved treatments for this group of patients.

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