Klippel-Trenaunay Syndrome: A Rare Cause of Vaginal Bleeding in the Prepubescent Girl

Siham Amenzouy,Lamia Kerboubi,Fatima Zohra Oudghiri,Laila Essabar,Badr Sououd Benjelloun Dakhama

doi:10.4236/jbm.2016.411001

Abstract

Klippel-Trenaunay syndrome (KTS) is an uncommon congenital disorder with unknown etiology that consists of mixed vascular malformations. Visceral organs may also be involved leading to a variety of manifestations. Although seemingly uncommon, genital tract involvement can occur and be a source of significant morbidity. We hereby describe a case of KTS of a 7-year-old female patient presenting with genital bleeding, large venous and capillary malformations and soft tissue hypertrophy of right limb. Patient was treated conservatively and asked to follow-up regularly. KTS should be kept on mind as a cause of vaginal bleeding in preteen girls especially those with suggestive clinical features. Management of this complex condition is challenging because of its progressive nature and wide range of disease severity.

Highlights

Klippel-Trénaunay syndrome (KTS) is an uncommon congenital disorder with unknown etiology that consists of mixed vascular malformations
We hereby describe a case of KTS of a 7-year-old female patient presenting with genital bleeding, large venous and capillary malformations and soft tissue hypertrophy of right limb
KTS should be kept on mind as a cause of vaginal bleeding in preteen girls especially those with suggestive clinical features

Summary

Introduction

Klippel-Trénaunay syndrome (KTS), or angioosteohypertrophy syndrome, is a rare congenital malformation syndrome [1] characterized by three clinical features, namely cutaneous capillary malformations (port-wine stain), venous varicosities, and soft tissue and/or bony hypertrophy of the extremity [2]. The etiology of KTS remains unclear many authors believe it to be caused by an embryonic developmental disorder of mesodermal tissues that affects angiogenesis at different stages, possibly following intrauterine insult [3]. It is a sporadic condition with multifactorial inheritance. The purpose of this paper was to present a case of KTS as a rare cause of genital bleeding in a preteen girl, and to highlight the clinical and imaging features of the disease

Case Report

Discussion

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Conclusion