Capillary malformations in unusual territory: Klippel–Trénaunay syndrome with penoscrotal involvement

TL;DR

A 3-year-old boy had a rare form of Klippel–Trénaunay syndrome affecting his genital area, with no internal organ involvement and a good prognosis.

Contribution

This case highlights the unusual penoscrotal presentation of KTS without systemic involvement, offering insights into its management.

Findings

The child had extensive vascular malformations in the penoscrotal region but no visceral involvement.

The case emphasizes the importance of multidisciplinary care for managing genitourinary KTS.

The absence of systemic disease supports a favorable prognosis in this rare presentation.

Abstract

Klippel–Trénaunay syndrome (KTS) is a rare congenital vascular disorder that typically presents with capillary and venous malformations, limb hypertrophy and, in some cases, lymphatic abnormalities. We report a 3-year-old boy with extensive vascular malformations affecting the left lower limb, predominantly involving the penoscrotal region – an uncommon presentation without systemic involvement. This case illustrates the diagnostic complexity of genitourinary KTS and emphasizes the need for vigilant monitoring, multidisciplinary input and tailored management to reduce complications and improve long-term outcomes.

We present a rare case of Klippel-Trenaunay Syndrome with extensive penoscrotal involvement in a child. Despite diffuse genital and cutaneous disease, there was no visceral extension, supporting a favourable prognosis and emphasizing the need for multidisciplinary follow-up.

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