Sunken eyes, sagging brain syndrome: bilateral enophthalmos from chronic intracranial hypotension.

PURPOSE: To explain the mechanism for acquired enophthalmos after ventriculoperitoneal shunting (VPS). DESIGN: Case series and a case-control study. PARTICIPANTS AND CONTROLS: Four study patients with bilateral enophthalmos after VPS and 10 control subjects. METHODS: Case description of 4 study patients. Calculated orbital volumes for 2 study patients were compared with controls using the Wilcoxon rank-sum test. MAIN OUTCOME MEASURES: Exophthalmometry measurements and total orbital and fat volumes. RESULTS: Patient 1 is a 25-year-old man who presented with progressive enophthalmos 3 years after VPS for traumatic intracranial bleeding. Imaging demonstrated upward expansion of the orbital roof and evidence of intracranial hypotension. The intracranial pressure (ICP) was 20 mm H2O. The enophthalmos improved after shunt revision. Patient 2 is a 19-year-old man who presented with progressive enophthalmos 18 months after VPS for traumatic intracranial hemorrhage. Patient 3 is a 38-year-old woman who presented with bilateral enophthalmos 15 years after VPS after a ruptured aneurysm. Imaging showed orbital expansion. Patient 4 is a 16-year-old man who presented with severe enophthalmos 5 years after a VPS for aneurysm-related hemorrhage. Imaging demonstrated orbital enlargement and findings of intracranial hypotension. Intracranial pressure ranged between -200 and 0 mm H2O. Shunt revision improved the enophthalmos. Total orbital volumes were significantly greater in the study patients than in the controls. Control subjects (5 male, 5 female, ages 23-45 years) had an average right orbital volume of 24.6 ± 3.3 cm³ (n = 10). In comparison, the right orbital volumes of patients 1 and 3 were 32.6 and 32.1 cm³. Similar results were found for the left orbits (23.9 ± 2.7 cm³ [control average] vs. 35.9 and 32.6 cm³). In patient 1, the post-shunt volumes increased 14% (right) and 23% (left) from pre-shunt volumes. In contrast, orbital fat volume was not statistically significantly different between the control group and enophthalmic patients (right orbit control mean 7.94 ± 3.1 cm³ [n = 10] vs. 7.9 and 9.8 cm³; left orbit control mean 7.88+3.1 cm³ vs. 9.2 and 10.0 cm³). CONCLUSIONS: Enophthalmos after VPS results primarily from chronic intracranial hypotension. Low ICP causes expansion of orbital volume with no fat atrophy. In such patients, shunt revision with a pressure-regulating valve to correct intracranial hypotension should be considered. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Blindness from orbital varices: case report.

A 41-year-old woman presented with a 21-year history of a left orbital mass. She reported 3 distinct episodes of sudden proptosis, periorbital bruising, pain, nausea and vomiting with resulting stepwise deterioration in her vision. Her symptoms resolved spontaneously over several days, with the exception of loss in vision, which persisted. Examination was notable for ipsilateral enophthalmos in primary gaze. With Valsalva she developed proptosis. Magnetic resonance imaging (MRI) demonstrated a left orbital apex malformation consistent with a varix. She had no light perception on the left with end-stage optic atrophy. This case illustrates the severity of visual loss that can occur with orbital varices.

Gaze-evoked amaurosis from vitreopapillary traction.

PURPOSE: We report four patients with gaze-evoked amaurosis attributable to incomplete posterior vitreous detachment and ensuing vitreopapillary traction. We present these cases to illustrate and extend the spectrum of vitreopapillary syndromes and to draw attention to vitreopapillary traction and its expected manifestations in both optic disk appearance and optic nerve and retinal function. DESIGN: This is a retrospective observational case series culled from tertiary neuro-ophthalmology practice. METHODS: Patients were evaluated with direct and indirect ophthalmoscopy, Hruby (precorneal) lens, three-mirror Goldmann contact lens, macular contact lens, formal perimetry, fundus photography, fluorescein angiography, and orbital ultrasound. RESULTS: Four patients with gaze-evoked amaurosis had disk edema associated with a partial posterior vitreous separation. These patients were young and had atypical posterior vitreous detachments characterized by persisting vitreopapillary attachments. CONCLUSIONS: Gaze-evoked amaurosis is a rare visual obscuration precipitated by changes in volitional gaze, usually associated with an underlying orbital mass. We extend its etiologies to implicate the vitreous through traction expressed at the optic disk. In our cases, vitreopapillary traction elevated the nerve head and eye movements precipitated transient visual phosphenes followed by gaze-evoked amaurosis caused by traction transmitted from the vitreous to superficial nerve fibers of the retina and disk.

The pseudopapilledema of neonatal-onset multisystem inflammatory disease.

PURPOSE: To report a rare case of neonatal-onset multisystem inflammatory disease with serial photographs to characterize the optic nerve findings. DESIGN: Observational case report. METHODS: A 6-year-old girl with neonatal-onset multisystem inflammatory disease, who had received systemic corticosteroid therapy for 5 years, had bilateral fibrillar opacities that surrounded the optic disks and extended into the peripapillary nerve fiber layer and vessels. A magnetic resonance imaging examination and lumbar puncture revealed elevated intracranial pressure. RESULTS: The elevated intracranial pressure returned to normal following a corticosteroid taper. Optic disk photographs, taken 4 years earlier, were subsequently obtained. The optic disk appearance had remained unchanged over the 4-year period, consistent with a pseudopapilledema. CONCLUSION: The optic disk appearance is not consistent with papilledema from increased intracranial pressure. The optic disk findings, in conjunction with the underlying inflammatory syndrome, suggest an infiltrative etiology for the atypical optic nerve findings in neonatal-onset multisystem inflammatory disease.

A case of posterior ischemic optic neuropathy in a posterior-draining dural cavernous sinus fistula.

A 79-year-old woman presented with sudden unilateral visual loss after an ocular motor disturbance and pulsatile tinnitus. Neuro-ophthalmologic examination showed a presumed right posterior ischemic optic neuropathy (PION), oculosympathetic, and third, sensory fifth, and sixth cranial nerve pareses. Selective angiography of the right internal and external carotid arteries confirmed a posterior-draining dural carotid cavernous sinus fistula (CCF) fed by the right meningohypophyseal trunk and right middle meningeal artery. Angiography also showed an ophthalmic-middle meningeal arterial anastomosis. We postulate that the PION was caused by an arterial steal, because blood was drawn into the fistula and away from the intraorbital optic nerve.

Papilopatia por irradiaçäo: o seu aspecto oftalmoscópico nas fases aguda e crônica / Papilopathy induced by radiation: its ophthalmoscopic aspect in acute and chronic phases

Fotografias fundoscópicas de sete olhos com papilopatia causada por irradiaçäo revelaram um amplo espectro de alteraçöes no disco óptico. Na fase aguda havia edema pálido de papila, exsudatos algodonosos confluentes na superfície do disco, hemorragias pré-papilares esparsas, estreitamento focal de vasos retinianos de grosso calibre, e exsudatos "duros" intraretinianos de disposiçäo circumpapilar. Nas fases crônicas havia acentuada palidez óptica, excrescências semelhantes à drusas no disco, capilares telangiectásicos, estreitamento e embainhamento de troncos vasculares retinianos e resoluçäo parcial dos exsudatos peripapilares

Papiledema unilateral na síndrome do pseudotumor cerebral / Unilateral papilledema in pseudotumor cerebri

Säo apresentadas as fotografias de fundo de olho de 6 mulheres com papiledema inilateral e a síndrome do pseudotumor cerebral. Achados associados foram: obesidade em 5 e a síndrome de sela vazia parcial em 2. Säo discutidas as possíveis explicaçöes para o desenvolvimento de papiledema unilateral nesses pacientes e que favorecem um mecanismo presente na porçäo distal dos nervos ópticos