2 -10 (60) 2023 — Gorbatyuk O.M. — HIRSCHSPRUNG’S DISEASE IN CHILDREN OF PUBERTAT TIMING

HIRSCHSPRUNG’S DISEASE IN CHILDREN OF PUBERTAT TIMING

Gorbatyuk O.M.  National University of Health of Ukraine named after P.L. Shupik Ukraine

Resume

Aim. Based on the clincal experience of monitoring and treatment children of pubertat timing with Hirschsprung’s disease (HD), to study clinical aspects of the disease in older children and to establish an adequate diagnostic and treatment policy.

Materials and methods. The paper is based on the analysis of the results of examination and treatment of 26 children with Hirschsprung’s disease, who were first diagnosed with the disease at the age of 10 years and older. There were 19 (73.08%) boys and 7 (26.92%) girls. The following diagnostic methods were used: X-ray examination of the large intestine, ultrasonography of the abdominal organs and the large intestine, rectosigmoidoscopy, morphological examination of biopsy material, immunohistological examination for acetylcholinesterase (AChE). Nonparametric statistical methods were used due to the small sample and the prevalence of the analysis of qualitative rather than quantitative criteria.

Results and discussion. According to anatomical classification, 6 (23.08%) children were diagnosed with the supraanal (short) zone of agangliosis, 15 (57.69%) – with the rectal form of HD, and 5 (19.23%) – with the rectosigmoid form of HD. The clinical picture was dominated by constipation, which occurred in early childhood in 76.92% of patients. Older children had long periods of relative compensation of bowel function and the absence of diarrhea. Diagnosis of HD in older children included: the study of family history, medical history, hereditary predisposition, study of the features of the clinical picture and the course of the disease with the exception of somatic, psycho-neurological and endocrine pathology, assessment of the nature and effectiveness of conservative measures, laboratory examination, sigmoidoscopy, irrigoscopy, colonoscopy, full-thickness rectal biopsy by Swenson, AChE test in the biopsy material of the rectal mucosa. The most common form of HD in teenagers is the rectal form with megarectum – 17.69% of patients. All children underwent surgery for HD in a subcompensated or decompensated state. A one-stage transanal endorectal pull-through with rectal dissection according to the Swenson or Soave technique with laparoscopic vidioassistance was performed in 15 (57.69%) patients. Lynn sphincterectomy was performed in 6 children with supraanal HD. In 4 (15,38%) children, the first stage of surgical intervention was colostomy, the indication for which was the patient’s critical condition, decompensation of the gastrointestinal tract and the inability to adequately prepare the intestine for radical surgery. No postoperative complications were registered. In the long-term period (1 year after the surgery), there was rectal incontinence in 5 (19,23%) cases and anastomotic stenosis in 1 patient. The conservative measures have had a positive effect. All children have recovered.

Conclusions.

1. Hirschsprung’s disease in older children has a latent course with long periods of functional compensation. The rectal form of pathology predominates (57.69%).

2. The main diagnostic methods are anamnesis, features of the clinical course of the pathology, irrigoscopy, morphological examination of full-thickness rectal biopsies and immunohistochemical examination of the rectal mucosa for AChE.

3. 84,62% of teenagers underwent one-stage surgery without postoperative complications; 15,38% of patients had indications for colostomy.

Key words: Hirschsprung’s disease, pubertat timing , diagnostics, treatment.

First page

11

Last page

17

For citation: Gorbatyuk O.M. – HIRSCHSPRUNG’S DISEASE IN CHILDREN OF PUBERTAT TIMING //New Day in Medicine 2023 10(60): 11-17 https://newdaymedicine.com/index.php/2023/10/05/l-390/

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