Open AccessSturge Weber Syndrome
Author(s) -
Kazi Nilufar Moly,
S.M. Abu Ahsan,
Md. Shafiqul lslam
Publication year - 2016
Publication title -
bangabandhu sheikh mujib medical university journal
Language(s) - English
Resource type - Journals
eISSN - 2224-7750
pISSN - 2074-2908
DOI - 10.3329/bsmmuj.v8i1.28924
Subject(s) - leptomeninges , sturge–weber syndrome , port wine stain , medicine , dermatology , birthmark , nevus , pathology , laser , physics , optics , endocrinology , central nervous system , melanoma , cancer research
Sturge weber syndrome is a rare sporadic condition of mesodermal phacomatosis, also called encephalotrigeminal angiomatosis (synonyms : fourth phacomatosis or mother spot), is a neurocutaneous disorder with angiomas that involve the leptomeninges (leptomeningeal angiomas) and the skin of the face (purple colored flat cutaneous haemangiomas ), typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve. The hallmark of sturge weber syndrome is a facial cutaneous venous dilation, also referred to as a nevus flammeus or port wine stain (PWS). Because of the rarity, we report here a one & half year old male child who presented with features of the Sturge Weber Syndrome on both side of face.