Case Report: Polyneuropathy Pituitary Apoplexy with Normal Perimetry and Initially Normal Neuroimaging.

SIGNIFICANCE: Pituitary apoplexy is a syndrome with a varied appearance, which carries a significant risk of morbidity and mortality. It is important to recognize the potential numerous symptoms and clinical findings, urgently investigate with the proper neuroimaging tests, and coordinate care with the appropriate specialists without delay. PURPOSE: This study aimed to describe a patient with worsening headache and ophthalmoparesis attributable to pituitary apoplexy who initially had reportedly a normal neuroimaging result and were diagnosed with migraine. CASE REPORT: A 39-year-old Hispanic man with a history of migraine developed a new and worsening headache. He presented to a hospital emergency department where he underwent a non-contrast-enhanced computed tomography and MRI, whose results were subsequently interpreted as normal. His headache was attributed to migraine, and he was medicated as such and discharged. Three days later, he developed horizontal and vertical diplopia and sought a second opinion. His visual acuity and visual fields were normal. He manifested a right pupil-sparing, external partial cranial nerve III palsy and concurrent right sixth nerve palsy. He also complained of worsening headache and lethargy. He was immediately referred for contrast-enhanced MRI and magnetic resonance angiography with suspicion of pituitary apoplexy. Subsequent imaging revealed a hemorrhagic pituitary macroadenoma consistent with pituitary apoplexy that was expanding laterally into the right cavernous sinus. He underwent immediate neurosurgical surgical repair. CONCLUSIONS: New or worsening headache with signs and symptoms of hypopituitarism should immediately be investigated for pituitary apoplexy. Other possible findings include involvement of cranial nerves III through VI because of cavernous sinus involvement and visual deficits if the optic chiasm or intracranial portion of the optic nerve is involved. However, growth may be lateral, and no visual deficits may be found, as in this case. Multiple concurrent cranial neuropathies should increase suspicion for cavernous sinus involvement.

Atypical retinal vaso-occlusion with structural and functional resolution.

PURPOSE: The purpose is to report a patient with primary open-angle glaucoma that developed sudden painless unilateral vision loss, a sequential ophthalmoscopic appearance with features of both central retinal artery and later central retinal vein occlusion, and objective visual system dysfunction in the form of a relative afferent pupil defect, who spontaneously recovered vision along with complete resolution of the pupillary defect over several weeks. CASE REPORT: A 50-year-old woman with a long-standing history of glaucoma presented with acute, painless vision loss in one eye, a pallid retina with a cherry red macula, diffuse retinal hemorrhages, and a relative afferent pupil defect. Spectral domain optical coherence tomography and fluorescein angiography were essentially normal with neither retinal edema nor retinal ischemia to account for the visual dysfunction. Over the course of 2 months, the patient regained vision and the relative afferent pupil defect, typically a permanent manifestation of retinal destruction, resolved. CONCLUSIONS: Not all retinal vaso-occlusive phenomena can be completely attributed to a central retinal vein or artery occlusion. In the patient presented, there was no objective diagnostic testing that revealed a cause for the patient's vision loss or relative afferent pupillary defect. This combined with the complete recovery of vision and resolution of the relative afferent pupillary defect underscores a lack of comprehensive understanding of retinal vaso-occlusive disease.

Regression of myelinated retinal nerve fibers in a glaucomatous eye.

PURPOSE: The purpose of this study is to present a case of a patient with primary open angle glaucoma and poorly controlled intraocular pressure, who underwent photodocumented atrophy of myelinated retinal nerve fibers. CASE REPORT: A 48-year-old woman with high myopia, refractive amblyopia, and extensive myelination of retinal nerve fibers underwent profound atrophy and regression of the myelination during an 8-year period. CONCLUSIONS: Myelinated retinal nerve fibers can atrophy and regress because of neurodegenerative conditions such as glaucoma.

The clinical spectrum between posterior polymorphous dystrophy and iridocorneal endothelial syndromes.

BACKGROUND: There are many conditions affecting the corneal endothelium with similar clinical appearances, though with different prognoses, management approaches, and pathophysiologic development. CASE: A 39-year-old black woman with a previous diagnosis of asymmetric corneal posterior polymorphous dystrophy (PPMD) presented complaining of irritation in the left eye, worsening over the last week. On examination, her left eye had profuse edema and bullous keratopathy overlying a large placoid gray lesion at the level of the endothelium. Gonioscopy of the left eye found evidence of a membranous development extending from the endothelium to the trabecular meshwork. Examination of her right eye found a perfectly clear cornea and a normal angle. The patient subsequently had Chandler's syndrome diagnosed in the left eye, an iridocorneal endothelial (ICE) syndrome, rather than PPMD. DISCUSSION: PPMD and ICE syndromes present with many similarities; these clinical entities are in fact thought to be variants of the same disease. Unlike PPMD, Chandler's syndrome is unilateral, acquired, and typically symptomatic and progressive. It also characteristically affects middle-age women. CONCLUSION: Understanding the clinical features of PPMD and ICE syndromes is important in proper diagnosis and management.

Bitemporal visual field defects mimicking chiasmal compression in eyes with tilted disc syndrome.

BACKGROUND: Tilted disc syndrome (TDS) is a congenital optic nerve coloboma occurring from embryonic dysgenesis. Several features characterize TDS, including an inferiorly located conus, situs inversus of the major retinal vessels, and an anomalous disc shape. Commensurate with axonal dysgenesis, visual field defects may often occur from TDS, the most common of which involve the temporal and superior temporal visual field. These visual field defects can mimic those seen in chiasmal compression from a mass lesion. CASES: Five patients from New South Wales, Australia, with distinct TDS and bitemporal visual field defects on frequency doubling threshold perimetry seemingly respecting the vertical hemianopic line are presented. Neuroimaging and medical evaluation of each failed to show intracranial chiasmal pathology in any patient. CONCLUSIONS: TDS can present with visual field loss resembling that seen in chiasmal disease. Although most cases of temporal visual field loss from TDS do not respect the vertical hemianopic line and are not true quadrantanopsias, there are instances in which this does occur, likely caused by the testing modality used. It is essential that patients with suspected intracranial pathology undergo immediate neuroimaging, even in the face of TDS.

Optic atrophy after sildenafil use.

BACKGROUND: It has been well-reported that phosphodiesterase-5 (PDE-5) inhibitors, originally investigated for their effect on smooth muscles and now used widely in treatment of erectile dysfunction, can cause mild transient visual disturbances because of their action on inhibiting enzymes involved in retinal transduction. Recently, these medications have been associated with the development of non-arteritic anterior ischemic optic neuropathy (NAAION) with attendant vision loss. CASE REPORT: An older male patient, previously examined and ocularly healthy, presented asymptomatically with an occult optic neuropathy, not characteristic of NAAION. Neuroimaging and serology failed to reveal any other underlying cause. The patient did, however, report the use of sildenafil during the interval between his previously normal examination and the observation of his optic neuropathy. CONCLUSIONS: This case details the development of an optic neuropathy with atrophy seemingly associated with the use of sildenafil, although no cause and effect could be conclusively found. This may indicate that medications used in the treatment of erectile dysfunction may be responsible for optic neuropathies other than NAAION.

Pupil block glaucoma from traumatic vitreous prolapse in a patient with posterior chamber lens implantation.

BACKGROUND: Angle closure secondary to pupil block is an entity known to occur in aphakic and pseudophakic patients. In aphakic patients, typically the cause of the pupil block is vitreous prolapse (aphakic pupil block). In pseudophakic patients, the typical cause of the pupil block is an anterior chamber lens implant, often in the absence of an iridectomy (pseudophakic pupil block). Rarely, a hybrid of these two conditions can occur, in which vitreous prolapse causes a pupil block in a pseudophakic patient with a posterior chamber lens implant. METHODS: The case presented is that of an elderly pseudophakic man with a posterior chamber lens implant who experienced ocular trauma, and who subsequently also experienced a ruptured posterior capsule with resultant vitreous prolapse and pupil block angle closure. Key diagnostic features are presented, as well as patient-specific management for this uncommon condition. CONCLUSIONS: While diagnosis and management of acute primary angle closure are well-known and reported, acute secondary angle closure is not as well reported. It is imperative to understand the mechanism of angle closure, as this properly delineates the management plan. Use of management plans appropriate to acute primary angle closure with pupil block (the use of miotics) could potentially have disastrous consequences in cases of secondary angle closure from vitreous prolapse.