2017, Number 3
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Rev Méd Electrón 2017; 39 (3)
Encephalotrigeminal angiomatosis or Sturge-Weber syndrome. A propos of a case
Morales QMC, Sierra BEM, León PMQ, Márquez OJA, Robles LL, López ÁTC
Language: Spanish
References: 11
Page: 592-601
PDF size: 410.88 Kb.
ABSTRACT
The Sturge-Weber syndrome is, in its complete form, the association of ocular,
cutaneous and cerebral anomalies. From the clinical point of view, it is
characterized by a wine-color spot on the face, epilepsy, mental retardation, other
neurological deficit manifestations and glaucoma. We presented the case of a male,
black patient, aged 9 years, who was born by eutocic delivery, with 4 200 g weight,
and perinatal symptoms of cyanosis, finding several facial hemangioma from the
moment of birth. At the physical examination, it was confirmed on the face and
crania, evasive forehead with facial hemangioma or choroidal hemangioma, left
unilateral glaucoma, micrognathia. Dental malocclusion was stated and clinodactyly
of the fifth finger. Besides that, he has an asymmetric trunk, severe mental
retardation and tonic-clonic convulsive episodes frequently repeated. Taking into
account all these elements we began to suspect the possible diagnosis of a Sturge-
Weber syndrome.
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