BackgroundSarcoidosis, a multisystem, granulomatous disorder, sometimes manifests with a neuro-ophthalmic subtype. The latter can pose a diagnostic challenge, especially when ocular symptoms appear before systemic involvement, as the clinical picture then can be non-specific and systemic laboratory and standard imaging investigations can be negative.FindingsA 71-year-old woman presented with a 4-month history of sudden-onset visual loss in the left eye. Slit lamp examination revealed anterior chamber cells, iris, and angle neovascularization. Fundoscopy showed a pale edematous optic nerve head surrounded with intraretinal hemorrhages and yellow retinal infiltrates. The vasculature was very narrow to absent. Indeed, fluorescein angiography filling was limited to the (juxta-)papillary region. An extensive systemic work-up revealed a monoclonal gammopathy and absence of any inflammatory markers. On MRI, a mass infiltration of the intraorbital and the intracranial optic nerve was visible. Additional PET-CT scan revealed hilar lymph nodes. A transbronchial biopsy demonstrating a non-caseating granulomatous lesion led to the diagnosis of sarcoidosis and thus neurosarcoidosis. Treatment with high-dose prednisone and azathioprine was started to avoid progression and subsequent visual loss in the other eye.ConclusionsA patient with neurosarcoidosis presenting with compressive ischemic optic disc edema and neovascular glaucoma is described, increasing the diversity of clinical presentations and confirming the diagnostic challenge of neurosarcoidosis.
Objective:To describe a patient with bilateral multifocal choroidal metastases from an endobronchial carcinoid treated with a somatostatin analogue.Method:A 60-year-old woman presenting with photopsia in the left eye underwent an extensive ophthalmic examination, including fluorescein angiography, OCT and ultrasound.Results:Fundoscopy revealed a small retinal tear in the left eye, for which she received laser treatment. In addition, choroidal masses were detected in both eyes. Her medical history of a pneumectomy for a bronchial carcinoid six years earlier together with recent elevated chromogranin A blood levels prompted a diagnosis of choroidal metastases. Subsequently, a Gallium-68 DOTANOC positron emitting tomography/computer tomography scan revealed a spinal cord metastasis and mediastinal as well as mesenterial lymph node invasion. Systemic treatment with Sandostatin®, a somatostatin analogue was started. Up until two years after the initial presentation and treatment, these choroidal lesions remained stable without any signs of growth.Conclusion:Endobronchial carcinoid tumors have an indolent nature and long-term follow-up is recommended for early detection of metastases. Although treatment with somatostatin analogues rarely induces complete tumor regression, tumor stabilization and prevention of symptoms related to hormone secretion is achieved. This well-tolerated systemic treatment provides a worthy alternative treatment for choroidal metastasis compared to classic radiotherapy without any risk of radiation or laser-related visual loss.
PurposeTo describe a patient who presented with non‐arteritic ischemic optic neuropathy (NA‐AION) as an initial manifestation of neurosarcoidosis.MethodsA case of a 70‐year‐old woman who presented with loss of vision in the left eye and normal neuroimaging, was initially diagnosed as a NA‐AION. An extensive workup included MRI orbita and PET/CT scan.ResultsAt the initial presentation, the left eye showed loss of vision and papilloedema. Further ophthalmological examination was normal, as was a CT scan of the brain. A diagnosis of NA‐AION was entertained. After 4 months, she had no light perception vision. Fundoscopy of the LE showed papilloedema with retinal infiltrates and severe ischaemia. MRI of the brain showed mass infiltration of the intraorbital and the intracranial optic nerve up to the optic chiasm. A complete systemic workup revealed a monoclonal gammopathy with cervical and hilar lymph nodes noted on PET/CT scan. Mediastinoscopy with biopsy of the hilar lymph nodes demonstrated a non‐caseating granulomatous lesion and a diagnosis of sarcoidosis was confirmed.ConclusionsSarcoidosis is a multisystemic disease characterized by granulomatous inflammation. Ocular involvement is seen in approximately 25% of patients with sarcoidosis. Uveitis is the most common ocular manifestation. Orbital and CNS manifestations of sarcoidosis are uncommon. Involvement of the optic nerve, chiasm and visual tract only represent 1–5% of neurosarcoidosis cases. In the latter, sarcoidosis can be initially misdiagnosed as NA‐AION, before involvement of the orbit and the CNS. Systemic involvement should be ruled out in any case of AION with an atypical evolution.
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