Abstract

Chyloperitoneum and chylothorax are rare conditions with high mortality rates whose optimal treatment strategy remains unclear. The aim of the study was to evaluate the results of chyloperitoneum and chylothorax treatment with a synthetic somatostatin analogue (octreotide) and immunosuppressive therapy with sirolimus. The study was approved by the Independent Ethics Committee and the Scientific Council of the National Medical Research Center for Children’s Health of Ministry of Healthсare of Russia. The patients' parents gave their consent to the use of their children's data, including photographs, for research purposes and in publications. We conducted a retrospective study of nine children diagnosed with congenital chyloperitoneum and chylothorax who had been treated from 2018 to 2022. All the children received either abdominal or pleural drainage, parenteral nutrition, and conservative therapy with drugs. The first line of therapy was octreotide for 14–20 days that was then switched to sirolimus if there had been no effect. The effectiveness of conservative therapy with octreotide at a dose of 5–10 µg/kg/hour was observed in 5 cases. If there had been no effect by day 14, the patients were started on sirolimus at a dose of 0.05–0.2 mg/day which proved to be effective in all the patients (n = 4). Our study showed that sirolimus is effective in complex cases of chyloperitoneum and chylothorax in newborns and infants. Because of the rarity of these disorders, our conclusions were based on the analysis of a small cohort. To confirm our results and develop uniform diagnostic and treatment guidelines, further, more targeted multicenter research is needed. Until such guidelines are adopted, decisions on the treatment of chyloperitoneum and chylothorax should be made on an individual basis and approved by the medical committee of a treatment center.

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