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 CASE REPORT
Year : 2010  |  Volume : 5  |  Issue : 2  |  Page : 138-140

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


1 Department of Pediatrics, Government Medical College, Sec 32, Chandigarh - 160 032, India
2 Department of Radiology, Government Medical College, Sec 32, Chandigarh - 160 032, India

Correspondence Address:
Monika Chhajed
H. No.400, Sec 16, Panchkula
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.76113

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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






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