Muthu Krishnan Vallinayagam

Abstract

Ischaemic optic neuropathy is defined as an infarction of the retrolaminar part of optic nerve. This is caused due to hypoperfusion of optic disc leading to short posterior artery occlusion. Giant cell arteritis assumes first and foremost importance as a prominent cause of anterior ischaemic optic neuropathy. Carotid artery dissection is an uncommon aetiological factor. Carotid artery dissection presents with a constellation of ocular features like Horner’s syndrome, amaurosis fugax, hemianopia, sixth cranial nerve palsy and ischaemic optic neuropathy. Ischaemic optic neuropathy has been reported in patients who suffered from dissection of carotid artery and it could be a preliminary manifestation. Suspicion of internal carotid artery (ICA) dissection should be raised in cases of anterior ischaemic optic neuropathy (AION) associated with severe unilateral cephalic pain. Dissection of carotid artery can cause cerebral and retinal ischaemia, especially in young adults, resulting in ischaemic symptoms. An array of investigations were performed to eliminate other triggers of optic neuropathy. Carotid Doppler helps to confirm the diagnosis. This article highlights a patient with a rare presentation of anterior ischaemic optic neuropathy resulting from dissection of internal carotid artery. A high index of suspicion should be entertained by symptoms like severe unilateral headache followed by diminution of vision. Prompt diagnosis and management can prevent visual loss in the fellow eye as well as life threatening complications of ICA dissection such as stroke. Keywords: Anterior ischaemic optic neuropathy, Dissection of internal carotid artery.

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Abstract

Von Hippel Lindau (VHL) syndrome is an autosomal dominant condition caused by mutation of VHL gene. It is characterized by vascular tumors of eye, central nervous system, visceral cysts and tumors. Here we report a case of VHL in a young male patient who presented with phthisis bulbi in the right eye and retinal capillary haemangioma in the left eye. Systemic features of raised intracranial pressure was present along with bilateral abduction deficit and florid disc edema in the left eye. There was a polypoidal reddish mass in the inferotemporal quadrant, with a dilated draining vein, indicating retinal capillary hemangioma. The phthisical right eye was speculated to result from untreated retinal capillary hemangioma, culminating in exudative retinal detachment. Urinary Vanillyl Mandelic Acid suggested pheochromocytoma. Imaging modalities revealed multiple cerebellar hemangioblastomas, pheochromocytoma and pancreatic cysts. Screening of family members revealed no evidence of systemic manifestations, suggesting the possibility of sporadic mutation. Retinal capillary hemangioma is the commonest and earliest manifestation of VHL. The ocular complications include intraretinal exudation and exudative retinal detachment, secondary angiomatosis, epiretinal membrane, vitreous hemorrhage, retinal neovascularization, tractional retinal detachment and macular ectopia. Our case report emphasizes that retinal examination in patients with suspected VHL is not only a screening tool for the presence of capillary hemangioma but also for regular monitoring and prevention of vision threatening complications. Keywords: Von Hippel Lindau (VHL) syndrome, Retinal capillary hemangioma, Cerebellar hemangioblastomas, Pheochromocytoma.

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Abstract

Myasthenia gravis (MG) is an autoimmune disorder with a highly variable clinical expression. Ocular symptoms are the presenting features in 40% patients but MG is restricted to ocular muscles in 15%. Myasthenia gravis is often associated with other autoimmune diseases. Case details of ten myasthenic patients visiting ophthalmology department over two years in a tertiary care hospital were analyzed. Five of them had interesting systemic associations. One patient presented with coexisting thyroid ophthalmopathy and ocular myasthenia. Because of the clinical overlap of ocular manifestations in myasthenia and Graves’ disease, one of the two conditions may be missed. Two patients had thymic mass. Presence of thymoma and prognostic implications are highlighted. One patient had thymic cyst, pemphigus and diabetes mellitus. This assortment of autoimmune conditions has not been reported in literature. One patient had associated Parkinson’s disease. Myasthenia gravis presents with a highly variable clinical spectrum and is associated with a cluster of autoimmune diseases. This unfortunate association is not widely appreciated. This report highlights the importance of detecting systemic associations of MG. The clinical relevance of these associations in diagnosis and management of myasthenia are discussed. Keywords: Myasthenia gravis, Pemphigus, Thyroid ophthalmopathy, Thymoma.

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