New Use of Rapamycin Stent in Non-Responding Facial Lymphatic Malformation

Abstract

Introduction: Vascular anomalies represent a diagnostic and therapeutic difficulty. A lymphatic malformation (LM) corresponds to a low-flow vascular malformation. Sclerotherapy is the preferred treatment. Recently, angiogenesis inhibitors such as Rapamycin have been used with promising results. Case Report: A pediatric patient presented with a large facial LM and poor response to Sildenafil and sclerotherapy. After a persistent enlargement of the lesion, with painful ocular occlusion a new sclerotherapy was performed obtaining partial improvement. As a rescue treatment an IRS was installed. The patient achieved a rapid and evident response, with better ocular aperture and pain control. Discussion: LM correspond to vasculogenesis disorders with persistent growth over time. They can present high morbidity. The complications in the facial area are infection, hemorrhage, exophthalmos, and amblyopia. Rapamycin is a potent angiogenesis inhibitor. Rapamycin stents have been safely used in cardiology. They provide local concentration with less adverse effects. There are no reports on their use in lymphatic malformation. The patient had a favorable and persistent response. Conclusion: IRS should be considered as a therapeutic alternative in severe nonresponding lymphatic malformation. The necessity of a multidisciplinary approach is fundamental