Gregory S. Kosmorsky

Low-dose Methotrexate Therapy for Ocular Inflammatory Disease

BACKGROUND Methotrexate is a second-line anti-inflammatory agent used in the treatment of rheumatic diseases. At low doses (12.5 mg/week), it is associated with few serious side effects. METHODS Twenty-two patients (5 men, 17 women) with chronic noninfectious ocular inflammatory disease, who had not responded to or who had become intolerant of corticosteroid or alternate cytotoxic agents, were treated weekly with oral low-dose, pulse methotrexate. Treated diseases included chronic uveitis-vitreitis (9), scleritis (4), inflammatory pseudotumor (3), orbital myositis (3), and retinal vasculitis (3). RESULTS Follow-up ranged from 2 to 39 months (mean, 11 months). Response time ranged from 3 to 9 weeks (mean, 5 weeks) after implementation of methotrexate therapy. Sixteen of 22 patients had reduction of inflammatory activity. Fourteen of these 16 patients were able to taper or discontinue corticosteroid therapy. Five patients had complete remission of their disease; six patients did not respond to methotrexate. CONCLUSION Treatment with low-dose methotrexate appears to be effective therapy for steroid-resistant ocular inflammatory disease.

Operative Complications of Optic Nerve Sheath Decompression

BACKGROUND Optic nerve sheath decompression (ONSD) is a procedure that is advocated for the treatment of certain types of optic nerve dysfunction associated with progressive decline in visual function. The reported postoperative complications attributed to ONSD surgery are relatively few and predominantly transient and benign. Few postoperative vascular complications are reported. METHODS The authors performed a retrospective review of the operative and postoperative course of 31 consecutive patients (38 eyes) undergoing ONSD surgery for various types of optic nerve dysfunction associated with progressive decline in visual acuity, visual fields, or both. The authors evaluated the reports of other investigators to ascertain the spectrum and diversity of postoperative complications associated with ONSD. RESULTS Fifteen (40%) of the 38 eyes undergoing ONSD had postoperative complications, including temporary motility disorders (29%) and pupillary dysfunction (11%). Additionally, four eyes (11%) had postoperative vascular complications, including two central retinal artery occlusions (CRAOs), one superotemporal branch retinal artery occlusion (BRAO), and one episode of transient outer retinal ischemia. Both eyes with postoperative CRAOs had poor visual outcome. Eyes that had undergone prior ONSD were significantly more likely to have vascular complications than those without a previous operation (Fishers exact test, P = 0.025). CONCLUSIONS Optic nerve sheath decompression can be associated with a variety of postoperative complications, the majority of which are minor and resolve without sequelae. Other complications, such as a CRAO, can be associated with significant visual loss. The physician and patients should be aware of the potential risks of ONSD, including significant visual morbidity, when considering this form of treatment.

Surgical management of clinoidal meningiomas.

OBJECTIVE Surgical outcome has been less than desirable in the management of patients with clinoidal meningiomas in the past, and little attention has been directed at improving their visual function. The purpose of this article is to advocate an available cranial base technique for removing these difficult tumors and to delineate the technique’s advantages that aid in achieving an improved extent of tumor resection and enhancing the patients’ overall outcome, particularly their visual outcome. METHODS A retrospective analysis was performed on 15 consecutive patients with clinoidal meningiomas (including a patient with hemangiopericytoma) who underwent surgical resection at the Cleveland Clinic Foundation between June 1995 and January 2000. A cranial base technique consisting of extradural anterior clinoidectomy, coupled with optic canal unroofing and optic sheath opening, was used in 13 patients, and standard pterional craniotomy was used in 2. Eight of 15 patients had significant visual deficits preoperatively. All patients had thorough preoperative and postoperative ophthalmological evaluations. The follow-up period ranged from 6 to 60 months (mean, 37.2 mo). RESULTS Total resection was achieved in 13 (86.7%) of the 15 patients in this series, and the majority of the patients with preoperative visual impairment experienced significant improvement (6 of 8 patients; 75%). CONCLUSION In the majority of patients with clinoidal meningiomas, total resection may be achieved with minimal complications. For large tumors encasing the optic nerve and internal carotid artery, or for those tumors causing preoperative visual impairment, use of the cranial base technique delineated in this study may lead to significant improvement in the patients’ visual and overall outcomes.

Crossover trial of gabapentin and memantine as treatment for acquired nystagmus

We conducted a masked, crossover, therapeutic trial of gabapentin (1,200mg/day) versus memantine (40mg/day) for acquired nystagmus in 10 patients (aged 28–61 years; 7 female; 3 multiple sclerosis [MS]; 6 post‐stroke; 1 post‐traumatic). Nystagmus was pendular in 6 patients (4 oculopalatal tremor; 2 MS) and jerk upbeat, hemi‐seesaw, torsional, or upbeat‐diagonal in each of the others. For the group, both drugs reduced median eye speed (p < 0.001), gabapentin by 32.8% and memantine by 27.8%, and improved visual acuity (p < 0.05). Each patient improved with 1 or both drugs. Side effects included unsteadiness with gabapentin and lethargy with memantine. Both drugs should be considered as treatment for acquired forms of nystagmus. ANN NEUROL 2010;67:676–680

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.

Ischaemic optic neuropathy after spinal fusion.

PurposeWe report a case of ischaemic optic neuropathy which occurred after prolonged spine surgery in the prone position in an obese, diabetic patient.Clinical featuresThe 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.ConclusionThis 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.ObjectifNous rapportons un cas de neuropathie optique ischémique consécutive à une chirurgie rachidienne prolongée en décubitus ventral chez un obèse diabétique.Éléments cliniquesUn patient de 44 ans, pesant 123 kg et mesurant 183 cm était opéré pour une laminectomie décompressive avec ostéosynthèse instrumentale de la colonne lombaire. Le thiopental associé à du fentanyl et de la succinylcholine a servi à induire l’anesthésie, qui a été entretenue avec du protoxyde d’azote, de l’oxygène, de l’isoflurane et une perfusion de fentanyl. Il était immobilisé en décubitus ventral sur un cadre de Relton-Hall et l’opération s’est déroulée sans incident. On a estimé la perte sanguine à 3000 ml. Il a été transféré à l’unité des soins intensifs (USI) et extubé 3,5 h plus tard. Il ne présentait aucun problème respiratoire et hémodynamique et on l’a ramené dans son unité de soins le lendemain matin. Il était libéré le cinquième jour. Par téléphone, il avisait son chirurgien le septième jour que sa vision était embrouillée depuis l’intervention. Son acuité visuelle avait diminué et à l’examen, il présentait une lésion papillaire bilatérale, de l’oedème du nerf optique et une hémorragie linéaire de l’oeil droit. L’imagerie par résonance magnétique de la tête et des orbites ne montrait aucune autre anomalie. Cet examen suggérait une neuropathie optique ischémique bilatérale qui fut traitée de façon conservatrice. Le 47e jour postopératoire, son acuité visuelle s’était améliorée considérablement et était presque redevenue normale. Parmi les facteurs contributoires possibles cette neuropathie ischémique, il faut retenir l’engorgement veineux.ConclusionÀ la suite d’une immobilisation prolongée en décubitus ventral sur un cadre de Relton-Hall, ce patient a présenté une neuropathie optique par ischémie causée vraisemblablement par engorgement veineux.

Ocular Ethambutol Toxicity

Ethambutol is an antimicrobial agent used frequently to treat tuberculosis. The most commonly recognized toxic effect of ethambutol is optic neuropathy, which generally is considered uncommon and reversible in medical literature. We describe a 43-year-old man who developed signs and symptoms of bilateral optic neuropathy during treatment with ethambutol. This case and a review of the literature show the severe and unpredictable nature of ethambutol toxicity and its potential for irreversible vision loss despite careful ophthalmologic monitoring.

Optic atrophy and sensorineural hearing loss in a family caused by an R445H OPA1 mutation

Autosomal dominant optic atrophy (ADOA) is the most common form of inherited optic atrophy. Four genetic loci have been associated with ADOA: OPA1, OPA2, OPA3, and OPA4. Out of these four loci, only one gene has been identified, OPA1. We previously described a unique syndrome of optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia in two unrelated families associated with an R445H mutation in OPA1. The R445H mutation is the only OPA1 mutation that has been associated with this syndrome. In this manuscript, we clinically characterize an unrelated family with four members affected by optic atrophy and hearing loss without extraocular motility abnormalities or ptosis. This family also harbors the R445H mutation. These cases help illustrate the intra‐ and inter‐family variability in phenotype associated with this mutation. As we continue to learn more about OPA1 and the function of its protein product, we will begin to understand the pathophysiology of optic atrophy. This understanding will ultimately lead to novel treatments directed toward preventing the visual loss and disability associated with this inherited 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.

Neuro‐ophthalmologic complications of cardiac catheterization

We examined ten patients who, from 1981 to 1986, sustained neuro-ophthalmologic events during cardiac catheterization. Eight patients, most of whom recovered, were believed to have sustained embolic phenomena. Two patients experienced a typical migraine during the catheterization and likewise did well. We conclude that the likelihood of sustaining a neuro-ophthalmic complication during cardiac catheterization is low and that the prognosis after having sustained such a complication is generally favorable. Evidence suggests that artery-to-artery emboli is the dominant pathogenic factor.