Lymphangioma circumscriptum and Whimster's hypothesis revisited

An 11-year-old boy was referred to our department for vesicular lesions of the buttocks of 7 years' duration (Figure 1). He also complained of intermittent oozing of serosanguineous fluid, which caused social impairment. On clinical examination, clusters of flesh-colored to translucent papules were present over the buttocks, perianal area, and left calf. The lesions were excised 4 years ago, after confirming the diagnosis of lymphangioma circumscriptum on histopathology. They started recurring on and around the surgical site within 6 months of surgery. Magnetic resonance imaging (MRI) was performed to evaluate the deeper extension of the lesion. The lymphangioma previously identified by cutaneous examination manifested as an altered T2 signal, spreading to subcutaneous tissues and major and minor muscles and infiltrating the sacral vertebrae and presacral area (Figure 2). Although the muscles were infiltrated, the boy was asymptomatic. He did not have pain, tenderness, or difficulty in the movement of the back and hip muscles. Renal function was also normal. Options of surgical resection and sclerotherapy were rejected by the parents because of the extensive spread of lymphangioma and fear of disfigurement. Carbon dioxide laser ablation was performed, and the parents were asked to follow up regularly for control of any symptoms, if they appear in the future.