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Case Reports
. 2022 Oct 10;14(10):e30128.
doi: 10.7759/cureus.30128. eCollection 2022 Oct.

A Rare Case of Klippel-Trénaunay Syndrome

Akshaya Arasu  1 , Alam Khalil-Khan  2 , Kavin Ilangovan G  3 , Einstein Raju  1 , Lavanya Gunasekaran  1 , Ramprasath Sathiamoorthy  1
Affiliations
Case Reports

A Rare Case of Klippel-Trénaunay Syndrome

Akshaya Arasu et al. Cureus. .

Abstract

The Klippel-Trénaunay syndrome (KTS) is a rare form of a birth disorder that includes capillary malformation, hypertrophy of bones and soft tissues, and tortuous varicosities, as well as hypertrophy of the capillaries resulting in hemangiomas and port wine discoloration. KTS is also known as angio-osteohypertrophy syndrome and dysplastic angiopathy. In this case report, we describe the case of a 13-year-old female with multiple superficial varicosities on the medial aspect of her left leg since birth. Computed tomography angiogram assessed and identified abnormal venous drainage in the lower limb. Klippel-Trénaunay-Weber syndrome (KTWS) differs from KTS in that KTWS involves arteriovenous malformations.

Keywords: bony or soft-tissue hypertrophy; klippel-trénaunay syndrome; painful nevus; portwine stain; venous varicosities.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Clinical photograph showing multiple superficial nodules with overlying prominent port-wine stain in the medial aspect of the left thigh and the proximal part of the leg.
Figure 2
Figure 2. X-ray anteroposterior and lateral view of the left knee joint with femur, tibia, and fibula showing a soft-tissue mass containing phleboliths with underlying bones showing mild osteopenic changes in the medial aspect of the proximal part of the leg.
Figure 3
Figure 3. Ultrasound of the abdomen showing an enlarged spleen with clustered cystic spaces without internal vascularity.
Figure 4
Figure 4. Splenomegaly with multiple geographic hypodense lesions showing progressive enhancement: splenic cystic lymphatic malformation.
Figure 5
Figure 5. Multiple nodular soft-tissue density lesions are seen predominantly involving the posterior, medial intramuscular compartment of the thigh, calf muscles, and medial subcutaneous aspect of the left lower limb. Several similar lesions are also seen in the foot. Several soft-tissue calcific foci are seen around the knee joint and in calf muscles representing phleboliths.
Figure 6
Figure 6. On contrast administration, several of these nodular soft-tissue density lesions are demonstrated to be dilated, tortuous enhancing venous channels.
Figure 7
Figure 7. The underlying bones of the left lower limb appear mildly osteopenic. The muscles of the calf appear atrophied with fatty replacement.
See this image and copyright information in PMC

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