Jože Maučec
Klinični oddelek za otroško kirurgijo in intenzivno terapijo, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Julija Pavčnik
Klinični oddelek za otroško kirurgijo in intenzivno terapijo, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Diana Gvardijančič
Klinični oddelek za otroško kirurgijo in intenzivno terapijo, Univerzitetni klinični center Ljubljana, Slovenija

Abstract

Hirschsprung’s disease is a congenital disruption of bowel motility, which occurs in approximately 1 / 5000 births. Timely recognition and treatment of the disease are of utmost importance in enabling the affected children to have good functional outcomes with low morbidity and mortality. The treatment is surgical, with resection of the affected part of the bowel. In the literature, there are also individual descriptions of studies connected with treatment with stem cells. The article will present the basics of Hirschsprung’s disease, its recognition and diagnostic work-up as well as treatment options and early and late postoperative complications. Possible new approaches to treatment, which are still at the level of laboratory tests, will also be presented. Our experience in treating the disease in UKC Ljubljana will also be presented. Data were retrospectively collected in the period 2010−2018, when 32 children from throughout Slovenia were treated. All children were treated according to the newest treatment principles. Five children had significant postoperative complications that were successfully treated. The data collected are comparable with the data obtained from the literature. The treatment success is also comparable, which confirms the importance of a good interdisciplinary approach with early detection of the disease, appropriate surgical treatment and quality postoperative care and follow-up.

Key words: Hirschsprung's disease, aganglionic megacolon, endorectal pull-through approach, Swenson, Duhamel, Rehbein, Soave, De la Torre-Mondragon, Botox