Optic neuropathy and chiasmopathy in the diagnosis of systemic lupus erythematosus.

PURPOSE: To report the clinical presentation of acute visual loss in six patients who were ultimately diagnosed with systemic lupus erythematosus (SLE). METHODS: Retrospective case series. RESULTS: All patients had a positive antinuclear antibody and elevated anti-double stranded DNA titers. Five of six patients demonstrated gadolinium enhancement of the optic nerve and/or chiasm on magnetic resonance imaging (MRI). Most patients showed initial improvement after treatment with high-dose systemic corticosteroids, but five experienced recrudescences during steroid taper, requiring further treatment with immunosuppressive or cytotoxic medications. CONCLUSIONS: Visual loss owing to optic neuropathy or chiasmopathy may be the presenting sign of SLE or the event that leads to this diagnosis. Gadolinium-enhanced MRI is useful for identifying anterior visual pathway lesions in these patients. Corticosteroids are effective in the treatment of this condition; however, relapses requiring further treatment are common.

Long-term visual outcome in patients with anterior visual pathway gliomas.

OBJECTIVES: To investigate the visual outcomes of patients with gliomas of the anterior visual pathway and the clinical features associated with prognosis. MATERIALS AND METHODS: During retrospective review, demographic and clinical data were abstracted from medical records of patients seen at the Bascom Palmer Eye Institute between January 1, 1970 and December 31, 1998. RESULTS: Of the 42 patients identified, 68% were female, and 55% had neurofibromatosis (NF) type I. There was no substantial difference in presenting signs, symptoms, and visual acuity between the NF(+) and NF(-) groups except for nystagmus, which was more common in the NF(-) group (p = 0.014). Throughout follow-up evaluation, vision in the better eye remained stable in both groups, independent of treatment or NF status. Vision in the worse eye often declined, despite treatment. However, binocular visual status, measured as average weighted logMAR (MAR, minimum angle of resolution) vision, did not change significantly over time, regardless of treatment or NF status. CONCLUSIONS: In the NF(+) and NF(-) groups, vision in the better eye remained stable, regardless of treatment, and vision in the worse eye often declined, despite treatment. Binocular visual acuity (measured as weighted logMAR) did not change significantly over time, regardless of NF status or treatment modality.

Neuroma as a cause of oculomotor palsy in infancy and early childhood.

INTRODUCTION: Third nerve palsy in infancy and early childhood is rare. We present 5 children with isolated monocular oculomotor palsy originally believed to be idiopathic but subsequently documented to be secondary to a presumed neuroma of the third nerve, distal to the mesencephalon. METHODS: Retrospective case review of patient charts from 1995 through 1999 was performed. RESULTS: Mean age at initial presentation was 14.4 months (range, 2-36 months). Two patients were male. Initial manifestation was an incomplete paresis in all 5 cases; 2 patients presented initially with exotropia, 2 with ptosis, and 3 with anisocoria. The pupil was ultimately involved in all cases. Progression to total paresis occurred in 4 of 5 patients over a mean of 13 months (range, 4-22 months). Findings of initial neuroimaging were believed to be normal in all cases but further thin-section gadolinium-enhanced magnetic resonance imaging studies, including coronal views, revealed enlargement and/or enhancement of the subarachnoid or intracavernous portion of the oculomotor nerve. Aberrant regeneration did not develop in any case throughout follow-up (mean, 49.4 months). Visual acuity was decreased on the ipsilateral side in all cases, consistent with amblyopia. No patient had neurofibromatosis. CONCLUSIONS: Suspicion for neuroma should be high in infants and young children with acquired, isolated, apparently idiopathic third nerve palsy. Serial gadolinium-enhanced, 1- to 2-mm thin-section magnetic resonance imaging with coronal views is essential for proper diagnosis.

Preschool vision screening using the MTI-Photoscreener.

Amblyopia is a condition that, if detected and treated early, can improve vision for most children. Thus, both pediatric and ophthalmologic groups have acknowledged the need for preschool vision screening. However, vision screening is the exception rather than the rule for preschoolers, since traditional methods of vision screening are often inappropriate for the preschool population and almost impossible for those children who are preverbal or nonverbal, developmentally delayed, and/or have chronic illnesses or disabilities. This study evaluated the use of a photoscreener to detect vision problems in a preschool population. Fifty-one children ages 3 to 5 years were evaluated using the MTI Photoscreener. Results were compared with a complete ophthalmologic examination, including cycloplegia. The sensitivity and specificity calculated for this study was 83% and 68%, respectively. Findings conclude that the MTI Photoscreener detected a broad range of vision problems, seemed to require less time, and seemed more acceptable to preschoolers when compared with the traditional vision screening methods performed by registered nurses. Although the sensitivity and specificity rates for this study were less than desired, it is likely that both could be improved with additional photo interpretation training.

Optic neuritis in children.

PURPOSE: To describe the clinical characteristics of optic neuritis in children, including final visual acuity and development of multiple sclerosis (MS). METHODS: Charts were reviewed of all patients < 15 years of age who presented with optic neuritis to the Bascom Palmer Eye Institute or the Miami Children's Hospital between 1986 and 1998. RESULTS: Fifteen patients were identified. There was a slight female predilection in the study group (60%), with a mean age of 9.8 years at presentation. A preceding febrile illness within 2 weeks of visual symptoms was reported in 66% of patients. Initial visual acuity ranged from 20/15 to no light perception. Involvement was bilateral in 66% of patients, and disc swelling was present in 64% of involved eyes. Of the patients who underwent magnetic resonance imaging, 33% had focal demyelinating lesions in the brain, and 63% of affected nerves were enlarged or enhanced with gadolinium. Eleven patients were treated with intravenous steroids. Final visual acuity was > or = 20/40 in 58.3% of eyes. Thirty percent of the patients had vision of finger counting or worse. Four (26%) patients developed MS. The mean age of patients with MS was 12 years, compared with 9 years in children who did not develop MS. Patients with unilateral involvement had an excellent visual prognosis (100% > 20/40), but a higher rate of development of MS (75%). Two patients had positive serology for Lyme disease. CONCLUSIONS: Optic neuritis presents differently in children than in adults. Children typically have bilateral involvement with papillitis following an antecedent viral illness. Although visual prognosis is poorer in children than adults, the development of MS is less common in children. Children who present with unilateral involvement have a better visual prognosis; however, they also develop MS at a greater frequency than children with bilateral involvement. Patients who developed MS were, on average, older at presentation with optic neuritis than those who did not develop MS.

Postpartum optic neuritis: etiologic and pathophysiologic considerations.

The clinical course of four patients with visual loss in the postpartum period due to acute optic neuritis is described. Factors that disclosed the underlying etiology and expression of disease are discussed. The clinical records of four women examined and managed for visual loss after uncomplicated pregnancies and term deliveries were reviewed. Neurodiagnostic examination, treatment modalities, and outcomes were assessed. These four women with varied and confounding medical histories, all with optic neuropathy, eventually were demonstrated to harbor demyelinating disease. Although visual loss in the postpartum period evokes differential diagnostic considerations, the authors' experience suggests that puerperal immune-mediated changes are responsible for activation of optic neuritis associated with relapsing multiple sclerosis.

Posterior fixation suture augmentation of full-tendon vertical rectus muscle transposition for abducens palsy.

OBJECTIVE: To evaluate the effect of augmenting full-tendon vertical rectus transpositions with posterior fixation sutures in patients with complete or near-complete lateral rectus palsy. METHODS: Transposition of the vertical recti to the lateral rectus muscle was performed in seven patients with unilateral lateral rectus palsy (the mean angle of preoperative horizontal deviation in primary gaze was 36.7 prism diopters (delta); range, 25-62delta of esotropia). A posterior fixation suture of 5.0 Mersilene (Ethicon, Somerville, NJ) was placed in sclera (14-16 mm posterior to the limbus) adjacent to the lateral rectus and incorporated 1/3 belly width of each transposed vertical rectus muscle. RESULTS: The mean angle of postoperative horizontal deviation in primary gaze was 7.1delta (range, 0-20delta). The mean change in primary-position horizontal deviation postoperatively was 41.2delta (range, 37-72delta). Four patients were able to fuse without prism in primary gaze; three patients were orthophoric and one patient had a consecutive intermittent exotropia. The remaining three patients required prism correction to neutralize the postoperative gaze deviation. All patients had improvement in abduction. Mild limitation of adduction was noted in three patients (range, -0.5 to -2.0). CONCLUSIONS: Augmenting full vertical rectus muscle transpositions with posterior fixation sutures improves the abducting effect of surgery without significant limitation of adduction.

Adrenal cortical carcinoma metastatic to the brain in a child.

A 9-year-old girl presented with profoundly decreased vision in both eyes and bilateral optic disc swelling. Magnetic resonance imaging showed a large intraventricular mass. Excisional biopsy diagnosed metastatic adrenal cortical carcinoma (ACC). This is, to the best of our knowledge, the first reported case of ACC metastatic to the brain in a child. It also illustrates the importance of differentiating optic neuritis from papilledema in children.

Bilateral blindness as the initial presentation of lymphoma of the sphenoid sinus.

PURPOSE: To report a patient with large-cell lymphoma of the sphenoid sinus presenting with bilateral blindness and no other signs or symptoms. METHOD: Case report. A previously healthy 5-year-old boy complained of sudden vision loss without other systemic complaints. RESULTS: Ophthalmologic examination revealed no light perception bilaterally. The pupils of the patient were fixed at 8 mm without reaction to the brightest light stimulus. Systemic examination was unremarkable, and neuroimaging revealed a large sphenoid tumor extending intracranially. Biopsy of the tumor proved to be large-cell lymphoma. CONCLUSION: Large-cell lymphoma affecting children may present initially with blindness, without other systemic symptoms.

Blindness from bad bones.

Progressive visual loss is the most common neurologic finding in osteopetrosis. Several mechanisms may explain this phenomenon, including compression of the optic nerves caused by bony overgrowth of the optic canals and retinal degeneration. We report a child with osteopetrosis and progressive visual loss, even though patent optic canals were demonstrated by computed tomography and digital holography. This patient's visual loss was caused by increased intracranial pressure secondary, to obstruction of cerebral venous outflow at the jugular foramen. This case points to the importance of a full evaluation of the skull base foramina in the diagnostic workup of visual loss in patients with osteopetrosis.

Surgical technique, visual outcome, and complications of pediatric intraocular lens implantation.

PURPOSE: To evaluate retrospectively the surgical technique, visual outcome, and complications of pediatric cataract extraction (CE) and intraocular lens (IOL) implantation. METHODS: Forty-three patients ages 2 to 12 underwent CE with IOL implantation with a minimum follow up of 1 month. RESULTS: All IOLs were implanted in the posterior chamber with 17 (40%) in the bag, 25 (58%) sulcus fixated, and one (2%) partially in the bag (one haptic in the bag, one in the sulcus). Primary posterior capsulectomy was performed in 12 (28%) cases. A final visual acuity of at least 20/40 was achieved in 26 (60%) and at least 20/80 in 32 (74%). Posterior capsule opacification developed in 18 (42%) and pupillary capture in 7 (16%). Seventeen (40%) patients had postoperative visual acuity worse than 20/40. Of these, nine (53%) had this visual outcome as a result of presumed amblyopia. CONCLUSIONS: Posterior chamber IOL implantation affords a safe and effective method of visual rehabilitation for cataractous children 2 years of age and older. Amblyopia and antecedent posterior segment trauma, rather than IOL-related or surgical complications, are the limiting factors in final visual outcome.

Pediatric photoscreening for strabismus and refractive errors in a high-risk population.

OBJECTIVE: To determine the accuracy of the MTI Photoscreener in detecting strabismus and refractive errors in children. PARTICIPANTS: One hundred children underwent MTI photoscreening followed by complete ophthalmologic examination. Six observers graded the photographs for strabismus, according to the location of the corneal light reflexes, and for refractive error, according to the size and location of the light crescent. RESULTS: The sensitivity of the MTI Photoscreener in detecting any amblyogenic factor was 80% to 91%, with a specificity of 20% to 67%. The sensitivity and specificity for particular amblyogenic factors varied widely among observers. The ranges were as follows: strabismus, sensitivity = 23% to 50%, specificity = 76% to 96%; myopia, sensitivity = 89%, specificity = 48% to 76%; hyperopia, sensitivity = 20% to 80%, specificity = 88% to 96%; and astigmatism, sensitivity = 46% to 77%, specificity = 79% to 89%. CONCLUSIONS: These results suggest caution in relying on photoscreening to detect strabismus and refractive errors in children.

Agreement between grating acuity at age 1 year and Snellen acuity at age 5.5 years in the preterm child. Cryotherapy for Retinopathy of Prematurity Cooperative Group.

PURPOSE: To examine the relation between grating acuity at age 1 year and Snellen acuity and grating acuity at 5.5 years, in preterm children with birth weights less than 1251 g. METHODS: Subjects were participants in the multicenter study of Cryotherapy for Retinopathy of Prematurity. The Teller acuity card (TAC; Vistech Consultants, Dayton, OH) procedure was used to measure monocular grating acuity in children at ages 1 and 5.5 years. Early-treatment diabetic retinopathy study (ETDRS) charts were used to measure the childrens' monocular recognition (Snellen) acuity at age 5.5 years. Data are presented for 575 eyes with measurable TAC grating acuity at 1 year and 111 eyes that had no measurable acuity at 1 year. RESULTS: Among eyes with normal acuity at 1 year, 86.8% showed normal Snellen acuity, and 94.3% showed normal grating acuity at 5.5 years. Among eyes that were blind (i.e., had no measurable TAC grating acuity) at 1 year, 96.8% showed no quantifiable Snellen acuity, and 89.2% showed no quantifiable grating acuity at 5.5 years. Only 2.4% of eyes had acuity in the range between normal and blind at 1 year (i.e., measurable grating acuity <1.6 cyc/deg); thus, the predictive value of acuity scores in this range could not be determined. Correlation analysis indicated that the relative position within the normal range of an eye's grating acuity score at 1 year was not predictive of the relative position within the normal range of that eye's acuity score at 5.5 years. CONCLUSIONS: Among a large population of low-birth-weight infants, eyes with normal grating acuity at age 1 year generally showed normal Snellen and grating acuity at age 5.5 years, and eyes that had no quantifiable acuity at 1 year remained blind at 5.5 years. Relative position of an eye's acuity score within the normal range was not predictive of the relative position of that eye's later acuity score.

Primary intraocular posttransplantation lymphoproliferative disorder.

We report a case of posttransplantation lymphoproliferative disorder manifesting as an isolated, unilateral iris tumor. A 2-year-old boy who had undergone liver transplantation for biliary atresia at age 4 months was seen with a 2-month history of an enlarging iris nodule. Histopathologic examination of the iris lesion demonstrated a mixed population of lymphoid cells. To our knowledge, this is the youngest patient with posttransplantation lymphoproliferative disorder isolated to the eye.

Outcome of surgical management of superior oblique palsy: a study of 123 cases.

PURPOSE: To determine the outcome of the surgical management of superior oblique palsy at our institution. SUBJECTS AND METHODS: Retrospective review of 123 patients who underwent surgical correction of superior oblique paresis at Bascom Palmer Eye Institute from 1976 to 1996. Subject-Patients: 67% were male and 33% female. The mean age at surgery was 30.5 years (range, 2-78 years). Etiologies of the pareses were trauma (34%), congenital (33%), and acquired/non-traumatic (33%). The mean angle of preoperative vertical deviation in primary gaze was 14.0 delta (range, 0-45 delta). SURGERY: 109/123 (89%) patients underwent single muscle surgery. Of these 109, 57 had single oblique muscle surgery: a superior oblique tuck in 34/57 (60%); an inferior oblique weakening procedure in 22/57 (38%); and a Harada-Ito procedure in 1/57 (2%). The other 14 patients (11%) had bilateral surgery. RESULTS: The final postoperative vertical deviation in primary gaze was < or =3 PD in 60% of patients and < or =7 PD in 80%. The mean change in primary position vertical deviation postoperatively was 10.4 PD for distance and 13.0 PD for near. An "excellent" outcome (final vertical deviation &le3 PD in primary and reading gazes) was achieved most frequently in those patients with congenital pareses and isolated oblique muscle surgery. COMPLICATIONS: Clinically significant Brown's Syndrome occurred in 43/72 (60%) of those cases who had undergone a superior oblique tuck. The incidence of Brown's Syndrome was unrelated to tuck size. Reoperation was three times more likely to be necessary in traumatic cases than in congenital cases (35.0% vs 11.9%, p=0.02). CONCLUSIONS: Based on these results we recommend oblique muscle surgery as the initial procedure to correct superior oblique palsy when appropriate.

The cupped disc. Who needs neuroimaging?

OBJECTIVE: To determine the incidence of positive neuroradiologic studies in consecutive patients with glaucoma associated with normal intraocular pressure and to compare the psychophysical and clinical characteristics of these eyes with eyes with disc cupping associated with intracranial masses. DESIGN: Retrospective case-controlled study. PARTICIPANTS: Fifty-two eyes of 29 patients with glaucoma associated with normal intraocular pressure and 44 eyes of 28 control patients with compressive lesions were reviewed. INTERVENTION: The medical records of consecutive glaucoma patients with normal intraocular pressure who underwent brain magnetic resonance imaging or computed tomography scanning as part of a diagnostic evaluation between January 1, 1985, and July 1, 1995, were reviewed. A masked reading of optic nerve photographs and visual fields was performed by one observer. A similar analysis was performed on a control group of consecutive patients with nonglaucomatous optic nerve cupping with known intracranial mass lesions. MAIN OUTCOME MEASURES: The neuroradiologic findings, clinical characteristics, optic nerve head appearance, and patterns of visual field loss were compared between groups. RESULTS: None of the patients diagnosed with glaucoma had neuroradiologic evidence of a mass lesion involving the anterior visual pathway. Compared to control subjects, patients with glaucoma were older (P = 0.0001), had better visual acuity (P = 0.002), greater vertical loss of neuroretinal rim tissue (P = 0.0001), more frequent optic disc hemorrhages (P = 0.01), less neuroretinal rim pallor (P = 0.0001), and more nerve fiber bundle visual field defects aligned at the horizontal midline (P = 0.0001). Visual acuity less than 20/40, vertically aligned visual field defects, optic nerve pallor in excess of cupping, and age younger than 50 years were 77%, 81%, 90%, and 93% specific for nonglaucomatous cupping associated with compressive lesions, respectively. CONCLUSIONS: Anterior visual pathway compression is an uncommon finding in the neuroradiologic evaluation of patients with a presumptive diagnosis of normal-tension glaucoma. Younger age, lower levels of visual acuity, vertically aligned visual field defects, and neuroretinal rim pallor may increase the likelihood of identifying an intracranial mass lesion.

Foveal cone dysfunction syndrome.

Our objective was to describe and expand the clinical spectrum of a rarely detected, previously reported photoreceptor disorder restricted to the foveal cones. Three patients with bilaterally decreased acuity and hemeralopia were examined to exclude a structural, vascular, inflammatory, or degenerative process. Each patient underwent a full neuroophthalmic examination, including full-field and focal cone electroretinogram (ERG). All three patients had normal-appearing fundi, mild dyschromatopsia, central or paracentral visual field depressions, normal full-field photopic and scotopic ERGs, and markedly reduced focal, foveal cone ERG responses. One patient had a ring scotoma and an asymptomatic family member with abnormal full-field and focal cone ERG. The syndrome of acquired foveal cone dysfunction presents as a bilateral, painless, progressive central visual loss with minimal or absent fundus changes. It eludes diagnosis until focal, foveal cone ERG is performed.

Walker-Warburg syndrome: neurologic features and muscle membrane structure.

Walker-Warburg syndrome is an autosomal-recessive genetic disorder characterized by congenital muscular dystrophy in association with complex developmental abnormalities of the central nervous system and the eyes. Two patients with Walker-Warburg syndrome are presented to demonstrate clinical variability. Previously unreported pathologic findings involving heart, muscle, spinal cord, and gall bladder are described, and the literature is reviewed. Histopathologic studies of the muscle membrane protein network in both Walker-Warburg syndrome patients reveal a decreased immunostaining for laminin alpha2 and beta-dystroglycan. The clinical, histologic, and biochemical variability in Walker-Warburg patients may reflect heterogeneity.

Retinopathy of prematurity in multiple-gestation pregnancies.

PURPOSE: To determine differences in incidence of retinopathy of prematurity between neonates of multiple-gestation and single-gestation pregnancies and to analyze differences in severity of retinopathy of prematurity among siblings of multiple-gestation pregnancies. METHODS: We reviewed the records of 149 neonates of multiple-gestation pregnancies and 691 single-gestation neonates screened for retinopathy of prematurity at one hospital from January 1, 1992, through December 31, 1995. The peak stage of retinopathy of prematurity was recorded for all infants. The multiple-gestation infants were then separated into concordant and discordant retinopathy of prematurity groups, with discordance defined as a difference of at least 2 stages of retinopathy of prematurity between siblings. Between siblings with discordant retinopathy of prematurity, multiple factors were compared. RESULTS: Retinopathy of prematurity was present in 69 (46%) of the multiple-gestation neonates. Retinopathy of prematurity was present in 312 (45%) of single-birth neonates. The percentage of multiple-gestation neonates with stages 1, 2, or 3 (prethreshold) or threshold retinopathy of prematurity was similar to that of single-gestation neonates. Stage 4 or 5 retinopathy of prematurity did not occur in either group. CONCLUSIONS: There was no significant difference in stage of retinopathy of prematurity between infants of single-gestation pregnancies vs those of multiple-gestation pregnancies. The majority (84%) of infants of multiple-gestation pregnancies had concordant retinopathy of prematurity. In those infants with discordant disease, zygosity and postgestational factors other than lowest serum glucose were not related to severity of retinopathy of prematurity.