ABSTRACT Sturge-Weber Syndrome is a rare, non-hereditary developmental condition characterized by hamartomatous vascular proliferations affecting the brain and facial tissues. Its main features include corticocerebral angiomatosis, cerebral calcifications, epilepsy, ocular abnormalities, mental impairment, and a facial port-wine stain. This report aims to highlight the main features of Sturge-Weber Syndrome in a patient with unilateral facial and scalp nevus, ipsilateral glaucoma, and imaging alterations. A case study was conducted on a patient treated at an ophthalmology clinic. The diagnosis and follow-up […]

ABSTRACT Sturge-Weber syndrome is a rare sporadic and congenital neurocutaneous disease characterized primarily by the presence of cutaneous and extracutaneous capillary malformations. Our understanding of this syndrome has improved since its first description over the years, particularly with the identification of different clinical presentations and new genetic mutations. Ocular manifestations are frequently observed in this syndrome but often develop insidiously and can threaten visual prognosis. Thus, an appreciation of different ophthalmological manifestations seems crucial. In the same perspective, we report […]

Sturge-Weber Syndrome is a rare neuro-oculocutaneous disorder. The authors describe the case of a 13 years old boy, presented with bilateral Sturge-Weber Syndrome and glaucoma. Surgical treatment with Ahmed valve implantation in both eyes was carried out achieving lower levels of intraocular pressure.

A síndrome de Sturge-Weber trata-se de uma doença neuro-óculocutânea rara. Os autores relatam o caso de um paciente do sexo masculino, de 13 anos de idade, que se apresentou com Síndrome de Sturge-Weber bilateral e glaucoma. Foi realizado o tratamento cirúrgico com implante de válvula de Ahmed em ambos os olhos e alcançado a redução da pressão intraocular bilateral.

The Sturge-Weber syndrome (SWS) is a rare congenital neuro-oculocutaneous disorder. This phacomatosis, also known as encephalotrigeminal angiomatosis, is defined by the classic triad: cutaneous, meningeal and ocular hemangiomas. Despite its typical presentation, incomplete forms are not uncommon. Glaucoma is often present and their pathophysiological mechanisms remain uncertain. Pherhaps this is why the clinical and surgical management of glaucoma associated with Sturge-Weber syndrome (GSSW) is a challenge for the ophthalmic practice, often with disappointing results. We present a literature review with […]