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Case Reports
. 2010 Jul;5(2):138-40.
doi: 10.4103/1817-1745.76113.

Klippel-Trenaunay and Sturge-Weber overlap syndrome with phakomatosis pigmentovascularis

Monika Chhajed  1 , Sadbhavna Pandit, Neeraj Dhawan, Amit Jain
Affiliations
Case Reports

Klippel-Trenaunay and Sturge-Weber overlap syndrome with phakomatosis pigmentovascularis

Monika Chhajed et al. J Pediatr Neurosci. 2010 Jul.

Abstract

Klippel-Trenaunay syndrome and Sturge-Weber syndrome are rare disorders with neurologic and cutaneous signs of vascular origin. Phakomatosis pigmentovascularis represents the association of widespread, aberrant, and persistent nevus flammeus and pigmentary abnormalities. We describe a case with features suggestive of overlap between them. A ten-month-old boy presented with seizures, developmental delay, skin lesions on face, trunk and legs, buphthalmos and right lower limb hypertrophy. CT scan of head showed atrophy of brain and calcification. Our case had overlap of Klippel-Trenaunay syndrome and Sturge-Weber syndrome with phakomatosis pigmentovascularis.

Keywords: Klippel–Trenaunay syndrome; Sturge–Weber syndrome; phakomatosis.

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Conflict of interest statement

Conflict of Interest: None declared

Figures

Figure 1
Figure 1
Showing hyperpigmented lesion on right leg and trunk.
Figure 2
Figure 2
CT of head showing atrophy of brain and calcification of left parietal region.
Figure 3
Figure 3
Showing leg hypertrophy
See this image and copyright information in PMC

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