Controversies in neuro-ophthalmology: Steroid therapy for traumatic optic neuropathy.

Background: There is an increase in the incidence of traumatic optic neuropathy (TON) due to increasing urbanization and rapid spurt in the number of motor vehicles on the road. Despite early presentation and ease of diagnosis the visual outcomes in TON are still limited. There is also significant confusion about the timing, dose and efficacy of steroid treatment in its management. Purpose: To provide a clinical update of the pros and cons of steroid therapy for TON. Design: The paper is a retrospective review of the currently available literature in the English language indexed in PubMed. Methods: A PubMed search was conducted by the authors using the following terms: Traumatic optic neuropathy, megadose, steroids, methylprednisolone. Relevant original articles, review articles, and case reports related to the topic of discussion were evaluated and discussed in the paper. Results: There is no prospective randomized control trial evaluating the effect of steroids in TON. There are varying reports on the effect of steroid therapy from significant improvement to no difference compared to observation. Conclusion: The decision to give steroids to patients with TON has to be on an individual case to case basis and must involve informed consent from the patient. There are documented advantages and disadvantages of steroid therapy and a prospective, randomized, controlled trial is necessary comparing steroids, surgery and observation before definitive management can be evolved.

An unusual cause of optic atrophy in a child.

A 13‑year‑old child presenting with gross visual impairment was diagnosed as a case of optic atrophy. However, radiological investigations revealed osteopetrosis, which, though rare, can result in optic atrophy. The aim of this case report is to highlight this possibility while evaluating cases of optic atrophy in young patients.

Bilateral intraocular dirofilariasis.

Ocular dirofilariasis mostly presents as a subconjunctival or eyelid lesion.[1] Intraocular dirofilarial infestation is rare.[2,3] We report a case of a young woman who was accidentally detected to have a live motile worm in the anterior segment in one eye and a cystic lesion on the optic disc in the other eye. To our knowledge, bilateral intraocular dirofilariasis has never been reported.

Clinical profile and short-term outcomes of optic neuritis patients in India.

Purpose: To evaluate the clinical profile and short‑term visual outcome of optic neuritis (ON) patients in India. Materials and Methods: In this prospective study carried out over a period of 3 years, 99 eyes of 83 ON patients were examined and followed up for 10.8 ± 8.2 months for type of presentation, recurrence rate, and visual outcome. Results: Mean age was 27.6 ± 8.8 years. Female preponderance was seen (70% of cases). Papillitis (53.5% of eyes) was more common than retrobulbar neuritis (46.5% of eyes). Bilateral presentation was seen in 19.3% cases. Baseline median logMAR visual acuity (VA) was 1.6 ± 0.8, which improved to 0.2 ± 0.6, with approximately 64% of eyes retaining VA of 20/40 or more. Two patients had previous diagnosis of multiple sclerosis (MS). MS was newly diagnosed in two patients. Recurrence was seen in 16% of eyes and was more common in cases of retrobulbar neuritis. Conclusion: The clinical profile of ON in Indian patients is different from that in the Western population. Unlike reported in the Western literature, papillitis is frequent in the Indian setup, with lower recurrence rates but poorer outcomes.

Evaluation of periosteal fixation of lateral rectus and partial VRT for cases of exotropic Duane retraction syndrome.

Purpose: The purpose of this study is to evaluate the lateral rectus periosteal fixation and partial vertical rectus transpositioning (VRT) as treatment modalities to correct exotropic Duane retraction syndrome (Exo‑DRS). Materials and Methods: Prospective interventional case study of cases of Exo‑DRS with limitation of adduction. A total of 13 patients were subdivided into two groups. Six patients underwent only lateral rectus periosteal fixation (group A) and seven patients also underwent partial VRT (group B). Assessment involved prism bar cover test, abduction and adduction range, extent of binocular single visual field and exophthalmometry. These tests were repeated at 1 week, 1 month and 3 months post‑operatively and data analyzed. Results: The pre‑operative mean values and ranges were 26.2 Δ (22‑35) exotropia for group A and −21.3 Δ (14‑30) exotropia for group B. The post‑operative mean and range was +0.6 Δ esotropia (+20 to −8) for group A and 8 Δ (−2 to −20) exotropia for group B. Mean grade of limitation of abduction changed from −3.8 to −3.6 versus −3.6 to −2.8 and mean grade of limitation of adduction changed from −1.9 to −0.7 versus −1.5 to −0.5 in the groups A and B respectively. Mean binocular single visual field changed from 14.7° to 23.3° in group A and 11.8° to 26.4° in the group B respectively. Conclusion: Lateral rectus periosteal fixation is an effective surgery to correct the exodeviation, anomalous head posture and improving adduction in Exo‑DRS and partial VRT in addition is effective in improving abduction and binocular single visual fields.

Factors predicting recurrence in successfully treated cases of anisometropic amblyopia.

Context: Recurrence after successful treatment of amblyopia is known and understanding the risk factors could help effective management. Aim: To measure incidence of recurrence in successfully treated cases of anisometropic amblyopia and evaluate factors predicting it. Settings and Design: Cohort Study at a tertiary level institution. Materials and Methods: Successfully treated anisometropic amblyopes aged 4−12 years were followed up for 1 year after stopping therapy. Best corrected visual acuity (BCVA), refractive error, stereoacuity and contrast sensitivity were evaluated at baseline and follow‑up. Statistical Analysis: Intergroup analysis with appropriate tests: Chi‑square test, Fisher’s exact test, Wilcoxon rank sum test and paired t‑test. Results: One hundred and two patients with mean age at diagnosis 7.06 ± 1.81 years were followed‑up for a mean duration of 1.0 ± 0.2 years. The mean pre‑treatment BCVA (LogMAR score) at diagnosis was 0.73 ± 0.36 units which improved to 0.20 ± 0.00 with treatment and after 1 year of stopping treatment was 0.22 ± 0.07. Thirteen (12.74%) patients showed amblyopia recurrence during follow‑up. Risk of recurrence was higher with older age of onset of treatment (6.64 ± 1.77 years without recurrence v/s 8.53 ± 1.39 years with recurrence, P = 0.0014). Greater extent of improvement of VA (P = 0.048) and final VA at stopping occlusion (P = 0.03) were associated with higher recurrence. Binocularity status or stereoacuity changes were not associated with risk of recurrence. Conclusions: Significant numbers of children suffer recurrence of amblyopia after stopping therapy. Older age, better BCVA after stopping therapy and greater magnitude of improvement in BCVA are important risk factors for recurrence. Careful follow‑up is essential for early detection and management of recurrence.

Evaluation of changes in retinal nerve fiber layer thickness and visual functions in cases of optic neuritis and multiple sclerosis.

Context: Retinal nerve fiber layer (RNFL) thinning has been demonstrated in cases of optic neuritis (ON) and multiple sclerosis (MS) in Caucasian eyes, but no definite RNFL loss pattern or association with visual functions is known in Indian eyes. Aim: To evaluate RNFL thickness in cases of ON and MS, and to correlate it with visual function changes in Indian patients. Settings and Design: Cross‑sectional case‑control study at a tertiary level institution. Materials and Methods: Cases consisted of patients of (i) typical ON without a recent episode (n = 30:39 ON eyes and 21 fellow eyes), (ii) MS without ON (n = 15;30 eyes) while the controls were age‑matched (n = 15; 30 eyes). RNFL thickness was measured using the Stratus 3°CT. The visual functions tested included the best‑corrected visual acuity (BCVA), contrast sensitivity, stereopsis, visual evoked responses, and visual fields. Statistical analysis used: Intergroup analysis was done using ANOVA and Pearson’s correlation coefficient used for associations. Results: RNFL thickness was reduced significantly in the ON and MS patients compared to the controls (P‑0.001). Maximum loss is in the temporal quadrant. Lower visual function scores are associated with reduced average overall RNFL thickness. In ON group, RNFL thinning is associated with severe visual field defects while contrast sensitivity has strongest correlation with RNFL in the MS group. Conclusions: RNFL thickness is reduced in ON and MS cases in a pattern similar to Caucasians and is associated with the magnitude of impairment of other visual parameters. Contrast sensitivity and stereoacuity are useful tests to identify subclinical optic nerve involvement in multiple sclerosis.

A sphenoid sinus mucocele simulating as retro bulbar optic neuritis.

A 25-year-old male presented with complaints of sudden diminution of vision with pain on eye movement in the left eye which was diagnosed clinically as retro bulbar optic neuritis. However, magnetic resonance imaging (MRI) showed lesion consistent with sphenoid sinus mucocele. Early surgical removal of mucocele led to complete recovery of vision, contrast and visual field. A high index of suspicion is necessary for intracranial lesions in all cases of retro bulbar neuritis, especially those with atypical symptoms.

Management of optic neuritis.

Optic neuritis is an inflammatory condition of the optic nerve characterized by a sudden onset of unilateral visual loss, usually affecting young females. Demyelination associated with multiple sclerosis (MS) is the most common cause in regions where MS is prevalent; while in other places, there are a substantial proportion of cases where infective or autoimmune causes are seen. Optic Neuritis Treatment Trial (ONTT) was the first major study that provided information on the natural history, role of steroids in treatment and risk of development of MS. Subsequently, numerous clinical trials have evaluated different modalities of management of optic neuritis and MS. The Controlled High-Risk Subjects Avonex Multiple Sclerosis Prevention Study (CHAMPS); the Prevention of Relapses and Disability by Interferon β-1a Subcutaneously in Multiple Sclerosis (PRISMS) Trial; and, most recently, the Betaferon in Newly Emerging Multiple Sclerosis for Initial Treatment (BENEFIT) Study have provided large amount of information on the natural history of optic neuritis and management options available. However, due to the low prevalence of MS reported in Asian studies, high cost of therapy and indefinite time period of treatment, it may not be cost effective to start interferon therapy in most cases.

A rare case of orbital apex syndrome with herpes zoster ophthalmicus in a human immunodeficiency virus-positive patient.

We report a rare instance of favorable outcome in orbital apex syndrome secondary to herpes zoster ophthalmicus (HZO) in a human immunodeficiency virus (HIV)-positive patient. The patient complained of pain and decrease in vision in one eye (20/640) for 2 weeks accompanied with swelling, inability to open eye, and rashes around the periocular area and forehead. The presence of complete ophthalmoplegia, ptosis, relative afferent pupillary defect, and anterior uveitis with decreased corneal sensation prompted a diagnosis of HZO with orbital apex syndrome. The enzyme-linked immunosorbent assay test and a low CD4 count confirmed HIV. Highly active antiretroviral therapy (HAART), systemic acyclovir, and systemic steroids were started. Visual acuity and uveitis improved within 10 days. By the end of the fourth week, ocular motility also recovered and the final visual acuity was 20/25. We highlight the role of HAART, used in conjunction with systemic steroid and acyclovir therapy, in improving the outcome.

Evaluation of distance and near stereoacuity and fusional vergence in intermittent exotropia.

Aim: To evaluate the role of distance and near stereoacuity and fusional vergence in patients with intermittent exotropia [X(T)] and their change after surgery. Materials and Methods: This prospective interventional institution-based clinical study included 31 cases of X(T) requiring surgery and 33 age, sex-matched controls. All subjects underwent complete orthoptic assessment including near stereopsis (Randot stereogram) and distance stereopsis by polaroid stereo-projector apparatus using special paired slides and fusional vergence assessment at distance and near prism bar at baseline and one week, one month, three months and six months after surgery in X(T). Results: The successful surgical alignment rate was 74.2%. Preoperatively, cases demonstrated significantly poor distance and near stereoacuity, compared to controls ( P P Conclusion: Early detection of abnormal stereoacuity (near and if possible distance) and near fusional vergence amplitudes may help to decide proper timing of surgery in X(T).

Visual outcome after intravenous dexamethasone therapy for idiopathic optic neuritis in an Indian population: a clinical case series.

PURPOSE: To evaluate the clinical profile, response to dexamethasone treatment and visual function outcome in Indian patients with acute optic neuritis. MATERIALS AND METHODS: We conducted an observational study of patients with acute optic neuritis who were treated with intravenous dexamethasone (100 mg in 250 ml of 5% dextrose over 1-2 hours daily, for three consecutive days) and had completed at least two years of follow-up. Parameters assessed included visual acuity, contrast sensitivity, color vision, visual fields, relative afferent pupillary defect (RAPD) and visually evoked potentials. Out of 40 patients studied, 26 patients (33 eyes) had all visual function parameters assessed. Twenty three patients (28 eyes) had completed two years of follow-up and were included for statistical analysis. RESULTS: Improvement in visual acuity was statistically significant for distance after 24 hours of the first dose (P = < 0.001) and for near vision after 24 hours of the second dose (P = 0.006); improvement in color and contrast sensitivity was statistically significant 24 hours after the third dose (P = < 0.001 for color vision and P = 0.013 for contrast sensitivity). Significant improvement in RAPD and visual fields were seen by 1 month (P = 0.005). Recurrence was seen in 4 eyes of 4 patients. No serious side effects were observed. At two years, 82.14% (23 out of 28) eyes had visual acuity > 20/40. CONCLUSION: Treatment with intravenous pulsed dexamethasone led to rapid recovery of vision in acute optic neuritis, without any serious side effects.

Factors influencing visual rehabilitation after occlusion therapy in unilateral amblyopia in children.

BACKGROUND & OBJECTIVE: Amblyopia is one of the most common causes of visual impairment in adults and children, and visual loss may be permanent if not treated in time. Though many studies have been done on occlusion therapy which is the mainstay in the treatment of unilateral amblyopia, discrepancies exist in literature about quantification of treatment and follow up measures. The present study was undertaken to evaluate the factors responsible for the successful outcome of treatment and the optimum time required for the same in children with unilateral amblyopia. METHODS: Baseline characteristics of 63 verbal patients with unilateral amblyopia (strabismic, anisometropic, mixed) referred to the Strabismus and Amblyopia Clinic at the Dr Rajendra Prasad Centre for Ophthalmic Sciences, New Delhi between September 2001 to December 2002 who improved to the desired level of visual acuity after treatment for amblyopia in the mentioned time period, were analyzed to assess for factors that directly or indirectly influenced the optimum visual rehabilitation and the average duration of therapy required for the same. The evaluation included assessment of the baseline best-corrected visual acuity (BCVA) and refractive status in both eyes, the age at presentation, the type of amblyopia present, fixation pattern in the amblyopic eye, inter-eye visual acuity difference, and evaluation of compliance through a parental diary system. RESULTS: Baseline BCVA in the amblyopic eye was similar in all the three groups. Patients with anisometropic amblyopia showed a quicker response to therapy. Compliance to treatment was the major factor affecting the overall time required for a successful outcome in most cases. The overall time required for the treatment to be successful (including the period of maintenance) was about 1,089 h. INTERPRETATION & CONCLUSION: This hospital-based study showed that the average duration of occlusion therapy to achieve stable isoacuity was 7.2 months with an average occlusion of 6-7 h/day. Compliance to therapy was the most important factor affecting the duration of therapy. With increasing emphasis on paediatric eye diseases, amblyopia is at last getting its due importance as a cause of treatable correctable paediatric visual impairment which can have lifelong repercussions, both in terms of individual disability and financial burden to the society if not treated in time. As the therapy is simple and effective if started early, mass awareness, visual screening, and counselling would go a long way in treating the patients, thus decreasing the prevalence of amblyopia in the country.

Profile of amblyopia in a hospital referral practice.

OBJECTIVE: Evaluation of the clinical profile and distribution of different sub-types of amblyopia in a referral eye hospital in India. METHODS: This was a prospective hospital-based observational study, evaluating the clinical profile of patients with amblyopia presenting to a referral strabismology practice. The examination included assessment of the visual acuity, the refractive status, the fixation pattern, the grade of binocularity, and evaluation of the associated strabismus, if any before treatment was started. RESULTS: The average age of the patients at presentation was 7.97?6.18 years with 81 out of 683 patients (11.8%) presenting above the age of 20 years. The best-corrected visual acuity (BCVA) was less than 6/60 in the amblyopic eye in 121 out of 733 eyes (16.5%). Strabismic amblyopia was the most common sub-type of amblyopia seen (274/733 eyes, 37.38%). Though patients with anisometropic amblyopia presented at a later age (average of 10.03+/-6.92 years), they had better visual acuity, binocular functions, and centricity of fixation at all ages, relative to other sub-types of amblyopia. The BCVA did not show any co-relation with the age of presentation [co-relation co-efficient (CF) of 0.074], refractive status of the amblyopic eye (CF of 0.078), the type of amblyopia (CF of 0.196), or the type of strabismus present (CF of 0.079). However, a very significant co-relation was seen between the BCVA and the fixation pattern of the amblyopic eye (CF of 0.817). CONCLUSIONS: Lack of knowledge and awareness about amblyopia and its appropriate timely management has been the main cause for the late presentations and significant visual impairment associated with the condition.

Ocular and cardiovascular autonomic function in diabetic patients with varying severity of retinopathy.

The study was conducted to assess the ocular and cardiovascular autonomic function in diabetic patients with varying severity of diabetic retinopathy. Ocular and cardiovascular autonomic function tests were performed in 30 patients with type 2 Diabetes Mellitus (10 in each group of proliferative retinopathy, non-proliferative retinopathy and no retinopathy) of more than 5 years duration and 10 normal controls. Ocular autonomic function tests were done by measuring pupil cycle time and denervation hypersensitivity with 0.125% pilocarpine and 0.5% phenylephrine. Cardiovascular autonomic function was measured by a battery of standard tests. Denervation hypersensitivity to 0.125% pilocarpine and to 0.5% phenylephrine and pupil cycle time showed statistically significant differences (P value < 0.001) between controls and patients with proliferative retinopathy (PDR) and also between no retinopathy and PDR (P < 0.001). Systemic autonomic function tests namely expiration--inspiration ratio, difference in heart rate, 30th beat and 15th beat ratio in head up tilt and difference in diastolic blood pressure in head up tilt test also showed significant difference (P < 0.01) between controls and all 3 groups of diabetics. There was statistically significant difference found in para-sympathetic ocular autonomic dysfunction between NPDR and controls. Ocular and systemic autonomic dysfunctions are related to the severity of diabetic retinopathy.

Pontine cavernous hemangioma presenting with horizontal gaze palsy.

Pontine cavernous hemangioma presenting as horizontal gaze palsy is extremely rare. A 32-years-old patient presented with left horizontal gaze palsy with left esotropia. A large pontine mass was present which was removed in-toto using a sub-occipital craniotomy. Post- operatively, the gaze palsy showed recovery and the diplopia decreased. Follow-up MRI showed no residual mass.

Isolated "one and a half syndrome" with brainstem tuberculoma.

A seldom reported causal association of two rare entities, an isolated brainstem tuberculoma and an isolated One and a half syndrome in a 12 year old girl is presented. MRI showed an isolated inflammatory granuloma in the brainstem which on empirical treatment with anti tubercular drugs resulted in complete restoration of ocular motility, along with resolution of the lesion on follow up MRI at 6 months. The diagnosis and management are discussed.