4.4 Article

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

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DOI: 10.1016/j.jvsv.2020.04.001

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Lymphatic malformation; Sildenafil; Phosphodiesterase-5 inhibitor

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

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