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Case Reports
. 2020 Dec 18:38:e2020091.
doi: 10.1590/1984-0462/2020/38/2020091. eCollection 2020.

INVERSE KLIPPEL-TRENAUNAY SYNDROME

[Article in English, Portuguese]
Mariana Franco Ferraz Santino  1 , Maria João Paiva Lopes  2
Affiliations
Case Reports

INVERSE KLIPPEL-TRENAUNAY SYNDROME

[Article in English, Portuguese]
Mariana Franco Ferraz Santino et al. Rev Paul Pediatr. .

Abstract
in English, Portuguese

Objective: To report a rare case of inverse Kipplel-Trenaunay.

Case description: A 16-year-old girl with a grayish-depressed plaque on her left thigh. Angioresonance showed a vascular malformation affecting the skin and subcutaneous tissue.

Comments: Inverse Klippel-Trenaunay is a Klippel-Trenaunay syndrome variation in which there are capillary and venous malformations associated to hypotrophy or shortening of the affected limb. Modifications on the limb's length or width result from alterations in bones, muscles, or subcutaneous tissues. It has few described cases. Further clinical and molecular studies must be performed for a proper understanding.

Objetivo:: Relatar um caso raro de Klippel-Trenaunay inverso.

Descrição do caso:: Menina de 16 anos com placa deprimida acinzentada na coxa esquerda, evidenciando-se, por meio de angioressonância, uma malformação vascular, acometendo a pele e tecidos subcutâneos.

Comentários:: Klippel-Trenaunay inverso é uma variante da síndrome de Klippel-Trenaunay em que há malformação capilar e venosa associada à hipotrofia ou encurtamento do membro afetado. Pode envolver acometimento ósseo, muscular ou subcutâneo, modificando o comprimento ou a circunferência do membro. Há poucos casos descritos, e mais estudos clínicos e moleculares precisam ser realizados para seu correto entendimento.

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Conflict of interest statement

The authors declare there is no conflict of interests.

Figures

Figure 1
Figure 1. (A) Depressed plaque on the lower lateral face of the left thigh with visible vein reaching it; (B) grayish-depressed plaque, interspersed by hypochromic areas and with telangiectatic vessels located peripherally.
Figure 2
Figure 2. Vascular malformation on skin and subcutaneous tissue, supplied by non-dilated intermuscular branches tributary from popliteal artery, with corresponding thinning of the subcutaneous tissue thickness, but without extension to the musculature. (A) MIP reconstruction in coronal plane; (B) post-gadolinium sequence in coronal plane.
See this image and copyright information in PMC

References

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