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Background Lymphatic malformations (LM) can be challenging to treat. Mainstay interventions including surgery and sclerotherapy are invasive and can result in local recurrence and complications. Objective To assess the effect of 20 weeks of oral sildenafil on reducing LM volume and symptoms in children. Methods Seven children (4 boys, 3 girls; ages 13–85 months) with LMs were given oral sildenafil for 20 weeks in this open-label study. The volume of the LM was calculated blindly using magnetic resonance imaging performed before and after 20 weeks of sildenafil. LMs were assessed clinically on weeks 4, 12, 20, and 32. Both the physician and parents evaluated the LM in comparison to baseline. Results Four subjects had a LM volume decrease (1.0–31.7%). In 2 subjects, despite a LM volume increase (1.1–3.7%), clinical improvement was noted while on sildenafil. One subject had a 29.6% increase in LM volume and no therapeutic response. LMs of all 6 subjects who experienced a therapeutic response on sildenafil softened and became easily compressible. Adverse events were minimal. Limitations A randomized-controlled trial will be necessary to verify the effects of sildenafil on LMs. Conclusions Sildenafil can reduce LM volume and symptoms in some children.

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Newborns can present with a host of varying birthmarks. Such nevi can represent a benign condition or a harbinger of a more concerning underlying condition or genetic syndrome. It is important for caretakers to be able to distinguish between the many seemingly similar or unusual entities. This article highlights distinguishing features of the most commonly encountered nevi seen at birth or in early childhood. Furthermore, updated information from the literature about controversies and current understanding of the genetic basis surrounding various nevi is presented.