Poster - 224
Treatment of children with vascular intestinal malformations
Olga Shcherbakovа 1, Elmira Alieva 1, Saidkhasan Bataev 2, Ilya Zyabkin 3, Lenara Khabibullina 4, Maksim Tsatsa 1, Oksana Bychkova 1
1 Federal Scientific and Clinical Center for Children and Adolescents of the FMBA of Russia
2 Federal Scientific and Clinical Center for Children and Adolescents of the FMBA of Russia, Burnazyan Scientific Research Center, Pirogov Russian National Research Medical University. Kh. Ibragimov Complex Institute of the Russian Academy of Sciences
3 Federal Scientific and Clinical Center for Children and Adolescents of the FMBA of Russia, Burnazyan Scientific Research Center
4 Federal Scientific and Clinical Center for Children and Adolescents of the FMBA of Russia, Pirogov Russian National Research Medical University. Moscow, Russia.
Introductions. Surgical interventions for angiodysplasia are often accompanied by intraoperative bleeding and intestinal anastomotic failure. To date, no single algorithm for treatment tactics has been developed.
The aim of the study is to analyze the results of surgical treatment against the background of Sirolimus therapy.
Method. A retrospective analysis of patients with recurrent intestinal bleeding was performed. Diagnostic methods: endoscopic, radiation, morphological, as well as magnetic resonance imaging and radioisotope scintigraphy. Surgical options are intestinal resections, sclerotherapy and electrocoagulation of malformations. Since 2012, antiproliferative therapy with Sirolimus has been prescribed.
Results. The study included 19 patients (8 boys and 11 girls), from 8 months to 17 years old, the average age was 8 years. Of these, angiodysplasia in the colon (12/63%), small intestine (3/15.8%), combined lesions of the small and large intestine (4/21%), lesions of the rectum and sigmoid colon (10/52.6%). Limited angiodysplasia (lesion of one section of the intestine, up to 5 cm) was diagnosed in 4 children (21%), diffuse angiodysplasia (lesion of one or more sections more than 5 cm) - in 12 (63%), generalized angiodysplasia (systemic angiomatosis with combined lesions of other organs and systems) - in 3 (16%). 12 (63%) underwent surgery, 5 of them once. Two or more operations were undergone by 7 patients. There were no postoperative complications in children who received Sirolimus therapy before surgery.
Conclusion. The use of staged surgical treatment with preventive therapy with Sirolimus allows to influence the risk of unfavorable outcomes and reduce the frequency of complications. The first experience of staged surgical interventions with preoperative therapy with Sirolimus in children with intestinal angiodysplasia shows encouraging results.