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29МЕДИЦИНСКИЕ НАУКИ
DIAGNOSIS AND SURGICAL TREATMENT OF HIRSCHSPRUNG'S
DISEASE IN CHILDREN
1 2 Shamsieva L.A. , Atakulov J.O.
1Shamsieva Laylo Alisherovna - Student, PEDIATRIC FACULTY;
2Atakulov Jamshid Ostanokulovich - MD, Professor, DEPARTMENT OF PEDIATRIC SURGERY, SAMARKAND STATE MEDICAL INSTITUTE, SAMARKAND, REPUBLIC OF UZBEKISTAN
Abstract: Hirschsprung's disease remains one of the most severe congenital malformations of the abdominal organs in children that require complex reconstructive surgery. The article is devoted to the development of diagnostics and determination of treatment tactics for various clinical forms of Hirschsprung's disease in children. We observed 245 children with various clinical forms of Hirschsprung's disease. The result of the study showed the effectiveness of surgical treatment tactics used for Hirschsprung's disease in children, as well as its diagnosis. Keywords: Hirschsprung's disease, anal dilation, colostomy.
Relevance. Despite the high incidence of the disease and dangerous complications of both the disease itself and the course of the postoperative period, there is no modern single algorithm for diagnosing and treating children with Hirschsprung's disease [2; 4]. Technical features of bowel relegation, residual zone of agangliosis lead to the need to perform repeated operations [1; 3]. Despite the apparent study of Hirschsprung's disease, there are still problems with its diagnosis and treatment in children [5].
The purpose of the research: Development of diagnostics and surgical treatment tactics for various clinical forms of Hirschsprung's disease in children.
Materials and methods of the research. This work is based on the analysis of the results of diagnosis and treatment of 245 children with various clinical forms of Hirschsprung's disease.
All patients were treated in the department of neonatal surgery and the department of general surgery № 2 2-clinic of the Samarkand State Medical Institute for the period from 2008 to 2017, there were more boys - 173 (70.5%), girls - 72 ( 29.5%). Children under 6 months 97 (39.6%), under 1 year 39 (16%), 1-3 years 47 ( 19.2%), 3-5 years 30 ( 12.2%), 5-7 years 13 (5.3%), over 7 years 19 (7.7%). The complex of examinations included a targeted survey of parents, a thorough collection of anamnesis, a finger examination of the rectum, an overview X-ray of the abdominal organs, a passage of the gastrointestinal tract, ultrasound sonography,and irrigation. After a complete clinical and laboratory, instrumental examination, the following anatomical forms of Hirschsprung's disease were identified (table 1, 2).
Table 1. Distribution ofpatients with Hirschsprung's disease by anatomical forms and course of the disease
Age Anatomical forms of the disease In total
rectal rectosigmoid Subtotal total
up to 1 year 102 (41,6%) 27 (11,4%) 5 (2%) 3 (1,2%) 137 (56,0%)
1-3 years 35 (14,2%) 11 (4,4%) 1 (0,4%) 47 (19,1%)
3-5 years 21 (8,6%) 7 (2,8%) - 2 (0,8%) 30 (12,4%)
5-7 years 12 (4,9%) 1 (0,4%) - - 13 (5,3%)
> 7 years 17 (6,9%) 1 (0,4%) - - 18 (7,3%)
In total: 187 (76%) 47 (19,2%) 6 (2,45%) 5 (2,0 %) 245 (100%)
Clinical form Clinical course
compensation decompensation subcompensation In total
Rectal 96 (65,3%) 32 (21,7%) 19 (13%) 147 (60%)
Rectosigmoid 24 (48%) 9 (18%) 17 (34%) 50 (20,4%)
Subtotal 15 (58%) 11 (42%) - 26 (10,6%)
Total 13 (59%) - 9 (41%) 22 (9%)
In total: 148 (60,4%) 52(21,2%) 45(18,4%) 245 (100%)
From table 1 it can be seen that the rectal form was more common, it was diagnosed in 143 (58.3%) cases, rectosigmoid was noted in 89 (36.5%), Subtotal form in 6 (2.4%), total form in 7 (2.8%) patients. Of 245 patients, 14 (5.7%) underwent colostomy during the newborn period.
The results of the research and their discussion: All children reported persistent constipation, lack of independent stool, decreased appetite, increased belly size, and lack of effect from conservative therapy. Young children were admitted in a serious condition with symptoms of fecal intoxication.
Treatment tactics depended on the length of the aganglionic zone and the severity of the disease. In the rectal form, the compensated course of the newborn was performed one or two times devulsion of the anus. In the rectosigmoid form of decompensated flow, subtotal and total form, a double-barrelled colostoma was superimposed above the aganglionic zone. The basis of radical surgical treatment of Hirschsprung's disease is the etiological principle of removing the affected part of the intestine and reducing the normal anatomically and functionally part of the intestine. Radical operations were performed from the age of one year. Surgical interventions were performed in an open manner using the Duhamel Bayirov techniques for rectal forms of u-78 (55.3%), rectosigmoid forms of u-31 (22%) and Soave-Lenyushkin for rectal forms of u-21(11.6%) rectosigmoid forms of u-11 (7.8%) Soave-Lenyushkin for total forms of u-5(3.3%) patients. After Duhamel Bairov's operation, 15 patients had partial fecal incontinence, the cause of which was a posterior rectum spur (sail), which was eliminated by repeated application of the Bairov clamp. After the Soave-Lenyushkin operation, 3 patients had early drop-off of the stump of the reduced intestine and retraction of the intestine with stenosis, which was eliminated by buzhirovaniya.
Conclusions. Thus, in the ultrashort segment of Hirschsprung's disease, a compensated course of conservative treatment was performed, the anus was diverted with decompensated currents, colostomy was applied to young children as preparation for radical surgery, and then radical surgery was performed according to Duhamel Bairov or Soave-Lenyushkin.
References
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3. Wang Y. et al. Three-dimensional versus two-dimensional laparoscopic-assisted transanal pull-through for Hirschsprung's disease in children: Preliminary results of a prospective cohort study in a Tertiary Hospital // Journal of Laparoendoscopic & Advanced Surgical Techniques, 2019. T. 29. № 4. P. 557-563.
4. Dore M. et al. Reliability of the Hirschsprung-associated enterocolitis score in clinical practice // European Journal of Pediatric Surgery, 2019. T. 29. № 01. P. 132-137.
5. Iacusso C. et al. Minimally invasive techniques for Hirschsprung disease // Journal of Laparoendoscopic & Advanced Surgical Techniques, 2019. T. 29. № 12. P. 1605-1608.
