Journal of Pediatric Surgery Case Reports (Dec 2023)
Management of a large hemolymphangioma with sirolimus: A case report
Abstract
Background: The treatment of vascular malformations with sirolimus is increasingly reported in the literature with variable results. We report a case of vascular malformation successfully treated with sirolimus in our institution. Case presentation: A full-term neonate born after a well monitored pregnancy was diagnosed prenatally with a slow-flow vascular malformation on the right chest wall and right axillary region. Postnatal physical examination, ultrasound, and MRI confirmed the diagnosis. We initiated our standard treatment protocol which consisted in systemic corticosteroid therapy at 1–2 mg/kg/day for 5 days, combined with propranolol at 2–3 mg/kg/day for 1 month. There was not a favorable response to this treatment. After a multidisciplinary discussion, we decided to treat the patient with sirolimus, initially administered orally at a dose of 0.8 mg per m2 per 12 hours, and gradually increasing it to a maximum dose of 2 mg per m2 per day. We observed a favorable response to this treatment. The treatment duration was 9 months, after which the treatment was interrupted without a taper. Functional and aesthetic results remain acceptable at 3 years of follow-up. Conclusion: Sirolimus seems to be a safe and effective alternative to surgery for vascular slow-flow malformations refractory to other medical therapies.
