Sturge-Weber Syndrome with Osteohypertrophy of Maxilla

معرفی دختر مبتلا به سندرم استورج وبر همراه با بدشکلی استخوانی در ناحیه دهان- فک بالا- صورت

Background: Sturge-Weber syndrome is one of the rare neurocutaneous disorders with frequency of approximately 1 per 50,000. Sturge-Weber syndrome consists of a constellation of symptoms and signs including a facial nevus (port wine stain), seizure and hemiparesis. In many cases it may associate with mental retardation. Case presentation: A 7- year old girl with mental retardation and a large fa...

Oromaxillofacial osseous abnormality in Sturge-Weber syndrome: case report and review of the literature.

We report a case of a 17-month-old child affected by Sturge-Weber syndrome who had unusually rapid overgrowth of the left frontal, temporal, orbital, and maxillary regions. CT angiography illustrated osteohypertrophy with periostitis and associated soft tissue hypertrophy directly corresponding to the distribution of the cutaneous port-wine stain. Extended maxillectomy was performed because of ...

Klippel-Trenaunay-Weber syndrome (angio-osteohypertrophy syndrome).

Dental management in an encephalotrigeminal angiomatosis patient: a case report.

Sturge-Weber Syndrome (SWS) is a rare, non-hereditary, congenital neuro cutaneous disorder characterized by vascular hamartomatous proliferations affecting classically, the leptomeninges and the skin of the face and may be associated with glaucoma, seizures and mental retardation. Intraoral findings are variable. A case of Roach type II Sturge Weber syndrome is reported here that did not show a...

Crouzon syndrome - A rare case report

Crouzon syndrome is the most common syndrome among the craniosynostosis group. Crouzon syndrome accounts for about 4.8% of all of them. It commonly has autosomal dominant inheritance with complete penetrance and variable expressivity from subtle to severe forms and characterized by craniosynostosis, exophthalmos, and hypoplastic maxilla with relative mandibular prognathism. Mutation of the fibr...