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. 2024 Oct 21;12(10):e9501.
doi: 10.1002/ccr3.9501. eCollection 2024 Oct.

A rare case of a 5-year-old girl with Klippel-Trénaunay syndrome and a bleeding focal vulvar hemangioma in Uganda

Mundeli Simon Peter  1 , Sibali Gidimali Gibu  1 , Mirembe Stephen Kizito  2
Affiliations

A rare case of a 5-year-old girl with Klippel-Trénaunay syndrome and a bleeding focal vulvar hemangioma in Uganda

Mundeli Simon Peter et al. Clin Case Rep. .

Abstract

Key clinical message: Klippel-Trénaunay syndrome can present with atypical manifestations such as a bleeding vulvar hemangioma. This case report, the first documented in Uganda, highlights the need for awareness of such presentations and underscores the importance of continuous follow-up in female patients to manage potential complications throughout adolescence and pregnancy.

Abstract: Klippel-Trénaunay syndrome (KTS) is a rare congenital disorder marked by bone and soft tissue hypertrophy, port-wine stains, and varicosities. Cases involving genital hemangiomas are rare. This report highlights a 5-year-old girl in Uganda with typical KTS features, including hypertrophy and port-wine stains, along with a bleeding vulvar hemangioma, emphasizing its uncommon presentation and potential complications. Treatment involved compression bandaging and timolol 0.2% solution. This case underscores the importance of awareness of atypical manifestations of hemangiomas with KTS and continuous follow-up for female patients, especially through adolescence and pregnancy, due to potential complications such as prepubertal per vaginal bleeding, menorrhagia, and post-partum bleeding.

Keywords: Klippel–Trénaunay syndrome; Mbarara University of Science and Technology; Uganda; bleeding vulvar hemangioma; port‐wine stain.

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Conflict of interest statement

The authors declare no competing interests.

Figures

FIGURE 1
FIGURE 1
Left leg and foot hypertrophy.
FIGURE 2
FIGURE 2
Left fourth and fifth toe macrodactyly.
FIGURE 3
FIGURE 3
Lumbosacral port wine stain with overlying erythematous papules.
FIGURE 4
FIGURE 4
Genital hemangioma.
See this image and copyright information in PMC

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