A Case of Atypical Klippel-Trenaunay Syndrome

Yujin Han,Sang-Hyun Cho,Se-Hoon Lee,Yu-Ri Woo,Minah Cho,Jeong-Deuk Lee,Hei-Sung Kim

doi:10.3390/dermato1020007

Yujin Han, Sang-Hyun Cho + Show 5 more

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https://doi.org/10.3390/dermato1020007

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Abstract

Klippel-Trenaunay syndrome (KTS) is a rare complex vascular syndrome with limb hypertrophy. KTS is diagnosed if at least two of the three features of capillary malformation, venous malformation, and soft tissue and/or bone overgrowth are present. Of these, capillary malformation (i.e., port-wine stain) is the most commonly observed feature but may be absent in atypical KTS. We herein report a case of atypical KTS with venous malformation and unilateral soft tissue hypertrophy.

Highlights

Klippel-Trenaunay Syndrome (KTS) is a rare congenital disease characterized by a combination of capillary-lymphatic-venous malformation and limb hypertrophy [1]
KTS is a rare disorder of capillary-lymphatic-venous malformation with limb hypertrophy [1]
The clinical manifestation of KTS includes a triad of capillary malformation, atypical varicose vein or venous malformation, and soft tissue or bony hypertrophy of the extremities

Summary

Introduction

Klippel-Trenaunay Syndrome (KTS) is a rare congenital disease characterized by a combination of capillary-lymphatic-venous malformation and limb hypertrophy [1]. Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. The triad includes capillary malformation (i.e., port-wine stain), venous malformation, and hypertrophy of soft tissue and/or bone. Port-wine stain is the most common manifestation of KTS and is observed in about 98% of patients.

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Conclusion