Ophthalmologic symptoms as the first manifestation of primary complete empty sella syndrome- case report

Background: Empty sella syndrome (ESS) is a rare condition associated with loss of pituitary gland volume. ESS has a heterogeneous clinical manifestation including endocrine, neurological, and visual deficits. The most common symptoms include chronic headaches and hormonal disturbances associated with hypopituitarism.Aim of the study: To emphasize the role of ophthalmologists in the initial management of intracranial hypertension and the significance of a multidisciplinary approach in the management of ESS.Case report: Primary complete ESS was reported in a 32-year-old male presenting nonspecific ophthalmologic symptoms including ophthalmalgia and subjective worsening of the visual acuity. The ophthalmological exam revealed bilateral optic disk elevation. The subsequent MRI identified a suprasellar cistern invaginating within sella turcica with resulting pituitary gland flattening. A complete empty sella syndrome was confirmed. In addition, idiopathic intracranial hypertension was suspected. The patient was proposed hospitalization in the neurology department for additional testing however, he refused due to personal reasons.Conclusions: To date, only a few papers reported ESS with preliminary ophthalmological symptoms. All patients with nonspecific eye symptoms should have a complex ophthalmological examination. Bilateral optic disk elevation requires urgent imaging of the brain and orbits, and the golden-standard approach remains MRI. During the diagnostic process, the secondary causes of intracranial hypertension including tumor, bleeding, abscess, hydrocephalus, or venous sinus thrombosis must be excluded. Due to the heterogeneous clinical course of ESS, patients require multidisciplinary management and follow-up by endocrinologists, neurologists, neurosurgeons, and ophthalmologists.