Primary Empty Sella Syndrome and Central Retinal Vein Occlusion

Abstract

Empty sella syndrome (ESS) is a peculiar disease that may involve systemic, neurological or ophthalmological symptoms. Our report presents a case of primary ESS complicated with central retinal vein occlusion. In an attempt to explain the pathogenesis of the retinal venous stasis, it may be hypothesized that the compression of the optic nerve may produce a slowing of axoplasmic flow, with a consequent optic nerve head swelling. Thus the central retinal vein gets compressed with the clinical result of a retinal venous stasis.

A Hypertensive Early-Middle-Aged Man with Loss of Vision: a Rare Case of Primary Empty Sella Syndrome and Central Retinal Vein Occlusion

“…Empty sella, frequently an incidental radiological finding, is a misnomer and, a more appropriate term is 'intra-sellar arachnoidocele', as because here, the sella turcica is filled either fully or partially with CSF, due to herniation of the arachnoid through diaphragm sella. 1,2,3 It is incidentally revealed in 8-38% during routine MRI of the brain and 5-10% at autopsy. 7 ESS is the clinical condition arising from this pathology.…”

“…Galactorrhoea, hirsutism, sterility, and, gynecomastia are other endocrine problems encountered. 3,4 In the index case, a male person in his late thirties had no clinical evidence of endocrinopathy, but he could not undergo a thorough biochemical evaluation of endocrine function owing to time constraints from early and rapid referral. However, he had normal thyroid hormones on a routine investigation.…”

“…The most wellknown are the weak diaphragm sella and raised intracranial pressure. 3 In the majority of the cases with primary empty sella (PES), patients remain asymptomatic, the condition being revealed only incidentally on radiology. 4 However, on rare occasions, PES can produce hormonal dysfunctions due to compression of the pituitary gland and, ocular manifestations due to traction and/or compression on the optic nerve.…”

“…5 Common ocular features include loss of visual acuity, oculomotor nerve dysfunction, and glaucoma-like tunnel vision. 3,4 Central retinal vein occlusion (CRVO) is per se a rare ocular condition with a prevalence of 0.11% ± 0.05% per subject among Indian people and 0.1-0.7 % according to other population-based studies, more rarely it can be a complication of PES. 5,6 This article is about a hypertensive man developing loss of vision in one eye due to CRVO in the presence of PES.…”