Effect of long-term phosphodiesterase-5 inhibitor use on refractory lymphatic malformations in adult and teen patients.

Abstract Lymphatic malformations (LM) are rare congenital anomalies. LM are often refractory to standard treatments including surgical resection, debulking and sclerotherapy. Use of sildenafil, a phosphodiesterase-5 inhibitor (PDE-5i), for treatment of pediatric LM has been reported with demonstrated benefit to some patients. This case series reports treatment of three patients (ages 14-37 years) suffering from complicated or refractory LM with low dose oral PDE-5i resulting in significant clinical improvement.