sildenafil-citrate and Lymphangioma

Lymphatic malformations (LMs) occur in 2.8 to 5 per 100,000 live births. Most involve the head and neck and they are equally common in men and women. They are developmental anomalies of unknown cause, although recent evidence suggests that an upregulation of the mammalian target of rapamycin (mTOR) pathway may be a causal factor leading to the overproduction of abnormal lymph vessels. These vessels are likely dilated lymphatic sacs sequestered from the lymphatic and venous systems. This overproduction results in the accumulation of lymph in dilated cystic spaces, which in turn results in the clinical features of an LM.

Topics: Combined Modality Therapy; Head and Neck Neoplasms; Humans; Lymphangioma; Magnetic Resonance Imaging; Sclerotherapy; Sildenafil Citrate; Sirolimus; Surgical Procedures, Operative; Treatment Outcome

Orbital lymphangiomas, congenital hamartomas of the lymphovascular tissue, are often associated with significant ocular complications and can be challenging to treat. To date, therapeutic approaches have demonstrated variable results and have significant ocular and systemic risks. We present 2 cases of pediatric orbital lymphangioma that responded to treatment with oral sildenafil.. This is a report of a series of 2 patients at the University of California-Davis Medical Center treated between March 2011 and October 2012. The first patient was a 12-month-old male infant whose extensive orbital and facial lymphangioma responded to sildenafil after repeated sclerosing and drainage procedures failed to achieve remission. The second patient was a 12-year-old boy whose orbital lymphangioma and associated ocular pain improved with sildenafil, making enucleation unnecessary.. These reported cases demonstrate promise for sildenafil as a noninvasive therapy for pediatric lymphangioma. Larger clinical trials are needed to clarify the optimal length of treatment, use as monotherapy, and long-term adverse effects.

Topics: Administration, Oral; Antineoplastic Agents; Child; Humans; Infant, Newborn; Lymphangioma; Magnetic Resonance Imaging; Male; Orbital Neoplasms; Phosphodiesterase 5 Inhibitors; Piperazines; Purines; Sildenafil Citrate; Sulfones; Treatment Outcome