[Severe bilateral visual loss as the presenting sign of cerebral venous sinus thrombosis: case report]. / Perda visual bilateral grave como sinal de apresentação de trombose de seio venoso cerebral: relato de caso.

Our purpose is to report a case of cranial venous thrombosis causing pseudotumor syndrome, where the principal clinical manifestation was severe bilateral vision loss. A 24-year-old man presented with severe bilateral vision loss, transient obscuration of vision, headache, bilateral papilledema and constricted visual fields. Computed tomography as well as neurologic examination was unrevealing. A lumbar puncture disclosed an increased cerebrospinal fluid opening pressure and complete blood count (CBC) showed increased numbers of platelets. A brain magnetic resonance imaging disclosed characteristic sings of chronic papilledema and suggestive signs of superior sagittal sinus thrombosis. A cranial magnetic resonance venography disclosed thrombosis of superior sagittal, transverse and sigmoid sinuses. The patient was treated with an optic nerve sheath decompression in the left eye, acetazolamide and anticoagulation. His ocular evaluation showed reduction of the papilledema, but severe vision loss remained. Pseudotumor cerebri with severe vision loss can be the presenting sign of cranial venous thrombosis secondary to a potentially life-threatening hematologic disorder.

Optic nerve sheath meningioma in the first decade of life: case report and review of the literature.

BACKGROUND/AIM: Tumors of the optic nerve are mostly either optic nerve gliomas or optic nerve sheath meningiomas (ONSMs). While gliomas occur in children, most meningiomas are slow-growing tumors affecting middle-aged individuals with progressive visual loss, proptosis, disc edema and optociliary veins on fundus examination. ONSMs are extremely rare in children, with only 14 cases reported in children under the age of 10 years. The purpose of this study is to describe an additional case and review clinical, radiologic and histopathological findings that can help differentiate such tumors in children and allow an early and accurate diagnosis. CASE REPORT: An 8-year-old girl had a 2-year history of progressive proptosis and total visual loss on the right side. A computed tomography scan revealed a well-defined intraconal mass with perioptic calcification. Magnetic resonance imaging showed a tumor surrounding the optic nerve and extending intracranially, with enhancement following gadolinium injection. The patient was submitted to fronto-orbital craniotomy for complete tumor excision. Histological studies identified the neoplasm as ONSM. CONCLUSIONS: Though unusual, ONSMs may occur in children and, in these cases, present a much more aggressive behavior than in adults. ONSM should therefore be differentiated from optic glioma in children because of its aggressive behavior and need for different treatment modalities.

Perda visual bilateral grave como sinal de apresentação de trombose de seio venoso cerebral: relato de caso / Severe bilateral visual loss as the presenting sign of cerebral venous sinus thrombosis: case report

O objetivo é relatar um caso de trombose de seio venoso cerebral causando síndrome de pseudotumor, onde a principal manifestação clínica foi uma perda visual bilateral grave. Homem de 24 anos apresentando perda visual bilateral grave, obscurecimentos transitórios da visão, cefaléia, papiledema bilateral e campos visuais constrictos. Tomografia computadorizada, bem como exame neurológico, apresentavam-se normais. Punção lombar mostrou um aumento da pressão liquórica inicial e o hemograma mostrou aumento do número de plaquetas. A ressonância magnética de crânio apresentava sinais característicos de papiledema crônico e sinais sugestivos de trombose de seio sagital superior. A venorressonância magnética de crânio mostrou trombose de seios sagital superior, transversos e sigmóides. Paciente foi tratado com descompressão de bainha de nervo óptico do olho esquerdo, acetazolamida e anticoagulação. O exame oftalmológico após o tratamento mostrou redução do papiledema, mas com persistência de perda visual acentuada. O pseudotumor cerebral com perda visual severa pode ser um sinal de apresentação de trombose venosa cerebral secundária a doença hematológica grave.