When does low mean high? Isolated cerebral ventricular increased intracranial pressure in a patient with a Chiari I malformation.

OBJECTIVE: To present an unusual case of pseudotumor cerebri with increased intracranial pressure isolated to the cerebral ventricles resulting from a Chiari I malformation. MATERIALS AND METHODS: The patient received a complete ophthalmologic examination on initial presentation and subsequent visits, including visual acuity, pupillary examination, intraocular pressures, dilated fundus examination with assessment of degree of papilledema, and visual field testing. Intracranial pressure was measured by lumbar puncture and subsequently by intracranial pressure bolt monitoring. Magnetic resonance imaging (MRI) was used to diagnose the Chiari I malformation. RESULTS: The patient initially presented with bitemporal headaches, elevated opening pressure on lumbar puncture, and mild papilledema with a normal MRI. After lumboperitoneal shunt placement and several revisions, the patient presented with decreased vision OD secondary to Terson syndrome and worsening papilledema. Subsequent evaluation revealed normal lumbar opening pressures and a Chiari I malformation. She underwent ventriculoperitoneal shunt placement with resolution of her symptoms. CONCLUSIONS: Tonsillar herniation is a well-documented complication of lumboperitoneal shunt revision. Obstruction of cerebrospinal flow through the foramina of Magendie and Luschka can result in increased intracranial pressure isolated to the cerebral ventricles. In a patient with signs and symptoms of increased intracranial pressure but normal lumbar opening pressure, a Chiari I malformation should be suspected, particularly with a history of multiple lumboperitoneal shunt revisions.

Arteriovenous communication in the orbit.

Arteriovenous malformations (AVMs) are anomalous communications between arterial and venous systems without interposed capillaries. These lesions are rarely entirely intraorbital. A case of an arteriovenous communication between branches of the internal and external carotid arterial circulations and the ophthalmic veins located within the orbit is reported. Treatment with embolization resulted in a branch retinal artery occlusion. Attempted direct arterial occlusion of a dural-based fistula of the eye is a risky procedure. If embolized, AVMs should probably be approached from the venous side, if at all.

Choroidal folds associated with increased intracranial pressure.

PURPOSE: To determine whether a relationship exists between increased intracranial pressure and the presence of idiopathic choroidal folds. METHODS: A prospective study in which 12 consecutive patients presenting with choroidal folds were evaluated by imaging studies (ultrasonography, magnetic resonance imaging, and/or computed tomography) to rule out known causes of choroidal folds, such as orbital disease, choroidal tumor, posterior scleritis, hypotony, and choroidal neovascular membrane. A lumbar puncture was performed on each of these patients, and measurement of opening pressure of cerebrospinal fluid was obtained. RESULTS: Twelve patients with choroidal folds included nine men and three women. Six patients (50%) presented with papilledema in the eye with choroidal folds. The other six patients (50%) presented with only choroidal folds. In this study, 10 (83%) of 12 patients had an opening pressure greater than 230 mm H(2)O. In patients presenting with only choroidal folds, five (83%) of six patients had an opening pressure greater than 230 mm H(2)O, with an average opening pressure of 290 mm H(2)O. CONCLUSION: Depending on the timing of the evaluation, papilledema may or may not be present, and only choroidal folds may be seen as a reflection of increased intracranial pressure. We believe that increased intracranial pressure from any source (that is, pseudotumor cerebri, sinus thrombosis, or intracranial mass) can present with only choroidal folds; therefore, these patients should have an appropriate work-up that should probably include a lumbar puncture before the title of "idiopathic" is given to their finding.

Ophthalmoplegia associated with the anti-Ri antibody.

Anti-Ri antibodies most often occur in patients with breast cancer and typically are associated with the paraneoplastic syndrome of opsoclonus-myoclonus-ataxia. This study reports a patient with diplopia and ophthalmoplegia. She had anti-Ri antibodies, and despite an exhaustive search for malignancy at presentation, breast cancer was not detected for six months.

Vertical fusional amplitudes in patients wearing vertical anisometropic correction.

OBJECTIVE: To examine the relationship between vertical anisometropic spectacle correction and vertical fusional amplitudes in patients. DESIGN: Comparative observational case series. PARTICIPANTS: Twenty-one patients exposed to greater than 0.5 diopters of vertical anisometropic spectacle correction were compared with 46 patients not exposed to anisometropic correction. METHODS: Vertical fusional amplitudes were recorded in all patients using a prism bar. MAIN OUTCOME MEASURES: Vertical fusional amplitudes and vertical anisometropia. RESULTS: In patients exposed to greater than 0.5 diopters of vertical anisometropic spectacle correction, vertical fusional amplitudes measured 5.2+/-1.4 prism diopters. Patients not exposed to anisometropic correction had vertical fusional amplitudes of 2.7+/-1.2 prism diopters (P < 0.0001). CONCLUSION: Patients with vertical anisometropic correction have increased vertical fusional amplitudes. This finding is relevant when evaluating patients with ocular motility disorders, especially with regard to distinguishing acquired versus longstanding deviations.

Direct orbital manometry in patients with thyroid-associated orbitopathy.

PURPOSE: To determine orbital tissue tension and orbital compartment compliance in patients with and without thyroid-associated orbitopathy (TAO). DESIGN: Prospective case series. PARTICIPANTS: Orbits of patients with TAO (18 orbits) and control patients without TAO (35 orbits) were studied. METHODS: An orbital manometer was designed to directly measure orbital tissue tension in patients undergoing ocular or orbital surgery. MAIN OUTCOME MEASURES: Tissue tension was recorded before, during, and for 5 minutes after a 5-ml retrobulbar injection of anesthetic. Orbital compliance was calculated as change in volume divided by change in tissue tension. RESULTS: Resting orbital tissue tension was 4.4 +/- 2.2 mmHg (mean +/- SD) in normal orbits and 9.7 +/- 4.8 mmHg in orbits of TAO patients (P = 0.0005) Following retrobulbar injection, orbital tissue tension rose to 12.0 +/- 3.6 mmHg (P = 0.0000000000000006 compared with baseline) in the control group and to 36.3 +/- 15.2 mmHg in the TAO group (P = 0.0000007 compared with baseline, and P = 0.000008 TAO group versus control group). Orbital compartment compliance was 0.80 +/- 0.50 ml/mmHg in the control group and 0.27 +/- 0.21 ml/mmHg in the TAO group (P = 0.00001). Resting orbital tissue tension in 8 TAO orbits with compressive optic neuropathy was 12.4 +/- 4.9 mmHg, and was 7.8 +/- 3.5 mmHg in 10 orbits of TAO patients without compressive optic neuropathy (P < 0.05). No adverse events occurred. CONCLUSIONS: Retrobulbar injection causes consistent measurable changes in orbital tissue tension. Orbital manometry safely demonstrated higher orbital tissue tension and lower orbital compartment compliance in the orbits of TAO patients versus those of normal subjects. Resting orbital tissue tension was higher in the orbits of TAO patients with compressive optic neuropathy than in those orbits without. Compressive optic neuropathy may partially result from an orbital compartment syndrome in some patients with TAO. Directly assessing orbital dynamics in vivo may prove useful as an adjunct in the clinical evaluation of patients with TAO and other orbital disorders.

Direct orbital manometry in healthy patients.

PURPOSE: To determine orbital tissue tension and orbital compartment compliance in normal patients. METHODS: An orbital manometer was designed to directly measure orbital tissue tension before, during, and after a 5-ml retrobulbar injection of anesthetic in patients undergoing ocular surgery. Tissue tension was recorded for 5 minutes after the injection. Orbital compliance was calculated as change in volume divided by change in pressure. Data were collected from 18 normal orbits. RESULTS: Resting orbital tissue tension was 4.0 +/- 1.5 mmHg (mean +/- standard deviation). After retrobulbar injection, orbital tissue tension rose to 11.6 +/- 2.6 mmHg (p = 0.00000000009 compared with baseline). After 5 minutes, tissue tension declined to 6.6 +/- 1.9 mmHg (p = 0.00000001 compared with preinjection and p = 0.00002 compared with postinjection). Orbital compartment compliance was 0.74 +/- 0.31 ml/mmHg. No adverse events occurred. CONCLUSIONS: The authors' orbital manometer safely determined orbital tissue tension and orbital compartment compliance in normal orbits. Retrobulbar injection causes consistent measurable changes in orbital tissue tension. Directly assessing orbital dynamics in vivo may prove useful both as an adjunct in the clinical evaluation of patients with disorders resulting in an orbital compartment syndrome as well as in assessing the risk of retrobulbar injection in orbits at greater risk for complications from this procedure.

Sudden painless visual loss: optic nerve and circulatory disturbances.

There are many causes of sudden painless visual loss in older adults. This article reviews the causes that are based on disturbances of the optic nerve and disorders of the circulatory system of the eye. Clinical clues to the recognition of these disorders and treatment (if available) are reviewed.

Corneal opacities in Gaucher disease.

PURPOSE: To describe the corneal findings in a variant of Gaucher disease. METHODS: Case report. In an 18-year-old man, ophthalmic and general clinical evaluation, and enzymatic and molecular genetics studies were performed. RESULTS: Diffuse, well-defined, small, linear, or dotlike corneal opacities were observed through, out the posterior two thirds of the corneal stroma in both eyes. The patient had calcific valvular heart disease. Enzymatic and ultrastructural studies were consistent with Gaucher disease. Analysis of the glucocerebrosidase gene disclosed homozygosity for a D409H mutation. CONCLUSION: Corneal opacities are a distinguishing ocular feature of the variant of Gaucher disease associated with the D409H mutation and with calcific cardiac disease.

Chest computed tomography and mediastinoscopy in the diagnosis of sarcoidosis-associated uveitis.

PURPOSE: To report the usefulness of chest computed tomography and mediastinoscopy in diagnosing sarcoidosis in elderly patients with uveitis. METHODS: Case reports. Two Caucasian women, aged 73 and 70 years, underwent evaluations for uveitis, which included chest computed tomography and mediastinoscopy. RESULTS: A chest computed tomographic scan of each woman disclosed mediastinal lymphadenopathy and warranted mediastinal lymph node biopsy by mediastinoscopy. In both patients, histopathologic and microbiologic studies demonstrated sterile noncaseating granulomas consistent with the diagnosis of sarcoidosis. CONCLUSIONS: The diagnosis of sarcoid-associated uveitis in the elderly may be facilitated by chest computed tomography, which may be more sensitive than conventional chest roentgenography. Laboratory studies of biopsied mediastinal nodes obtained by mediastinoscopy can confirm the diagnosis.

Pseudotumor cerebri sine papilledema with unilateral sixth nerve palsy.

A 17-year-old woman presented with a history of 1-week of headache and 3 days of horizontal diplopia. Examination revealed 20/20 vision in both eyes, no papilledema, and an abduction deficit in her left eye. Lumbar puncture revealed an opening pressure of 440 mm H2O. After treatment with acetazolamide, the headache and abduction deficit resolved. Papilledema never developed. This is a unique case of pseudotumor cerebri sine papilledema with a unilateral abduction deficit. We suggest that young women with headache and unilateral abduction deficits may be unrecognized cases of pseudotumor cerebri.

Ischaemic optic neuropathy after spinal fusion.

PURPOSE: We report a case of ischaemic optic neuropathy which occurred after prolonged spine surgery in the prone position in an obese, diabetic patient. CLINICAL FEATURES: The patient was a 44-yr-old, 123 kg, 183 cm man for decompressive laminectomy and instrumented fusion of the lumbar spine. Anaesthesia was induced with thiopentone, fentanyl and succinylcholine and maintained with nitrous oxide, oxygen, isoflurane and a fentanyl infusion. He was positioned prone on the Relton-Hall frame and had an uneventful intraoperative course. Estimated blood loss was 3,000 ml. He was taken to the surgical intensive care unit (SICU) and the trachea was extubated 3.5 hr later. He had no pulmonary or haemodynamic problems and went to a regular nursing floor in the morning. He was discharged home on postoperative day #5. He telephoned his surgeon on postoperative day #7 to say that his vision had been blurry since surgery. His visual acuity was decreased, and on examination, he had a bilateral papillary defect, optic swelling and a splinter haemorrhage in the right eye. Magnetic resonance imaging (MRI) scan of the head and orbits detected no other abnormality. Based on this examination, he was felt to have bilateral ischaemic optic neuropathy and treated conservatively. By postoperative day #47, his visual acuity was greatly improved and near normal. Careful review of possible contributing factors suggests that the cause of the ischaemic optic neuropathy was venous engorgement. CONCLUSION: This patient developed ischaemic optic neuropathy from a prolonged interval in the prone position of the Relton-Hall frame, which may be related to venous engorgement.

Implications of thrombocytosis in giant cell arteritis.

PURPOSE: To bring attention to the implications of thrombocytosis in giant cell arteritis. METHOD: Case report. Platelet counts were measured before and after corticosteroid therapy in a patient with biopsy-proven giant cell arteritis. RESULT: Platelet counts were increased before corticosteroid therapy was begun and returned to normal levels after corticosteroid therapy was instituted. CONCLUSION: Platelet counts in a patient with giant cell arteritis may have diagnostic, therapeutic, and prognostic value.