Strabismus developing after unilateral and bilateral cataract surgery in children.

PurposeTo evaluate the prevalence and risk factors of strabismus in children undergoing surgery for unilateral or bilateral cataract with or without intraocular lens implantation.MethodsMedical records of pediatric patients were evaluated from 2000 to 2011. Children undergoing surgery for unilateral or bilateral cataract with at least 1 year of follow-up were included. Children with ocular trauma, prematurity, or co-existing systemic disorders were excluded. The following data were evaluated: strabismus pre- and post-operation; age at surgery; post-operative aphakia or pseudophakia; and visual acuity.ResultsNinety patients were included, 40% had unilateral and 60% had bilateral cataracts. Follow-up was on average 51 months (range: 12-130 months). Strabismus was found preoperatively in 34.4% children, and in 43.3% children at last follow-up. Strabismus developed in 46.2% of children who were orthotropic preoperatively, whereas 32.3% of children who had strabismus before surgery became orthotropic. Strabismus occurred after unilateral or bilateral cataract surgery in 63.9% and 29.6% children, respectively. At the last follow-up, strabismus was found in 46.7% of aphakic and 58.7% of pseudophakic children (P=0.283). Children who developed strabismus were generally operated at a younger age as compared with those without strabismus (mean of 25.9 vs 52.7 months, P<0.001). Final visual acuity was inversely correlated with prevalence of strabismus.ConclusionStrabismus is a frequent complication after cataract surgery in children. Risk factors include unilateral cases and young age at surgery. No correlation was found between prevalence of strabismus and use of intraocular lens. Strabismus was more common in children with poor final visual acuity.

Suppression of inflammatory and fibrotic responses in allergic inflammation by the amniotic membrane stromal matrix.

BACKGROUND: The amniotic membrane (AM), which is the innermost layer of the placenta, was shown to possess anti-inflammatory and anti-fibrotic properties in various in vitro and clinical studies. PURPOSE: To evaluate the anti-fibrotic and anti-inflammatory effects of the AM matrix (AMM) on human conjunctival and lung fibroblasts in an in vitro system that tests fibrotic and inflammatory responses at the effector stages of allergic inflammation. METHODS: Human conjunctival or lung fibroblasts were seeded on plastic or on the stromal aspect of the AM, which was mounted on plastic inserts. Sonicates of human peripheral blood eosinophils activated with lipopolysaccharide (LPS), or human mast cell (HMC-1) leukaemia cell sonicates, were added to sub-confluent fibroblast monolayers. Proliferation of the sub-confluent fibroblasts was assessed using the [3H]-thymidine incorporation assay. The production of transforming growth factor (TGF)-beta1, granulocyte-macrophage colony-stimulating factor (GM-CSF) and IL-8 in conjunctival or lung fibroblasts was measured in conditioned media from these cultures by ELISA. RESULTS: After 4 days in culture, the [3H]-thymidine incorporation assay indicated a reduced proliferation of activated conjunctival and lung fibroblasts when cultured directly on the AMM. The production of both TGF-beta1 and IL-8 was significantly suppressed in activated conjunctival fibroblasts cultured on the AMM compared with those cultured on plastic, while the production of both TGF-beta1 and GM-CSF was decreased in human lung fibroblast cultured on the AMM. CONCLUSIONS: The AMM is capable of suppressing fibrotic responses in an in vitro system of effector stages of ocular allergic inflammation. These data may provide a basis for exploring matrix components in the AM for the treatment of allergic eye disease.

Post-brachytherapy initial tumour regression rate correlates with metastatic spread in posterior uveal melanoma.

AIM: To confirm the prognostic value of post-brachytherapy initial tumour regression rate in posterior uveal melanoma. METHODS: A prospective, comparative, observational cohort study was carried out on 147 eyes (147 patients) with choroidal melanoma (mean age 61 years) treated with Ru-106 brachytherapy. OBSERVATION PROCEDURE: Patients were followed clinically and ultrasonically every 6.7 (SD 0.3) months (1001 examinations). On average each patient was examined 5.8 times (mean follow up 9.6 (3.7) years). The echographic parameters included tumour base size, height, internal reflectivity, regularity, vascularity, and extrascleral extension. The clinical follow up included ocular examination and periodic metastatic screening (liver function tests and liver imaging). Main outcome measures were risk of liver metastasis in correlation with the post-brachytherapy initial tumour regression rate. RESULTS: At brachytherapy the mean tumour height was 5.2 mm (range 2.2-11.8 mm). After brachytherapy 142 tumours (96.6%) responded by a decrease in height. The initial height regression rate was 6.1% (0.8%) per month in patients who later developed metastasis v 4.3% (0.4%) per month in those who did not. Tumours higher than 6 mm, tumours with an internal reflectivity smaller than 50%, and tumours with an initial rate of height regression larger than 0.7 mm/month (10% per month) had higher 5 year melanoma related mortality. Kaplan-Meier survival analysis and the multivariate Cox proportional hazards model showed a significant role for tumour height and initial tumour regression rate on patients' survival. CONCLUSION: This study confirms that post-brachytherapy initial tumour regression rate has a prognostic value.

Orbital growth retardation in retinoblastoma survivors: work in progress.

BACKGROUND: Orbital growth retardation, after enucleation and/or external beam radiation for retinoblastoma (RB), is a serious late effect. We measured orbital volumes of RB survivors treated at Hadassah University Hospital, Jerusalem, between 1980-1998. PROCEDURE: Forty-five orbits of 28 children with RB (17 bilateral, 11 unilateral) were examined. Thirty-six orbits were irradiated, 19 enucleated, and 10 both enucleated and irradiated. The orbital volumes were calculated from a three-dimensional orbital CT reconstruction. The orbits of RB survivors were compared to age-matched controls. RESULTS: The mean age at diagnosis was 13 months, mean follow-up time was 56 months. The mean volume of RB orbits (14.4 cc) was statistically significantly smaller than control orbits (17.8 cc). There was no difference between the mean volume of orbits treated with enucleation, irradiation or both. The orbital volume of children treated before the age of 12 months was statistically significantly smaller than those treated later. There was no difference between mean volume of fellow orbits in unilateral RB and controls. The mean orbital asymmetry index in control children (2.6%) was statistically significantly smaller than in RB survivors (14%). CONCLUSIONS: There was a significant orbital growth retardation after enucleation and/or irradiation for RB. There was no difference between mean orbital volumes after enucleation, radiation or both. Orbital growth retardation was most prominent in children treated in the first year of life. Although small in number, our study suggests that deferring enucleation and/or irradiation until after the age of 12 months may reduce long-term complications.

Ocular manifestations in children born after in vitro fertilization.

OBJECTIVE: To report the ocular abnormalities found in children born after in vitro fertilization. METHODS: Forty-seven children (25 girls and 22 boys) born after an in vitro fertilization pregnancy (mean +/- SD birth weight, 2335 +/- 817 g; range, 924-4300 g) and referred for ophthalmic evaluation were included in the study. All underwent a thorough ocular examination. Obstetric history was gathered following a detailed questionnaire with the mothers. RESULTS: Of 70 eyes among nonverbal children, visual acuity was "normal for age" in 60 (86%), "fair" in 4 (6%), and "poor" in 6 (9%). Visual acuity in 24 eyes in verbal children ranged from 6/6 to no light perception, with 4 (17%) having poor vision. Cycloplegic refraction disclosed an emmetropia in 22 (27%), hypermetropia in 47 (57%), and myopia in 13 (16%) of the eyes. Anisometropia of more than 1.0 diopters was found in 8 children. Major ocular malformations were observed in 12 (26%) of the 47 children. These malformations included Coats disease, congenital cataract, congenital glaucoma, hypoplastic optic nerve head, idiopathic optic atrophy, coloboma with microphthalmos, and retinoblastoma. CONCLUSIONS: Ocular anomalies were frequently observed in this cohort of offspring born after in vitro fertilization. A diligent and prospective prenatal search for such malformations should unveil the real prevalence of ocular malformations in children born after in vitro fertilization.

Traumatic wound dehiscence after cataract extraction.

PURPOSE: To study the visual outcome and factors associated with it in patients with traumatic dehiscence of a cataract surgery wound. SETTING: Department of Ophthalmology and the Brown School of Public Health and Community Medicine, The Hebrew University-Hadassah Medical School, Jerusalem, Israel. METHODS: A retrospective observational study of 37 eyes of 37 consecutive patients with traumatic wound dehiscence of a cataract surgery wound was conducted. A complete ophthalmic evaluation was performed in all patients. Statistical analysis was done to identify factors associated with the best corrected visual acuity at the end of follow-up. RESULTS: Patients had extracapsular cataract extraction (n = 29), intracapsular cataract extraction (n = 4), or lensectomy (n = 4). No patient had phacoemulsification via a small incision. A univariate analysis showed that factors associated with a worse visual outcome included the presence at presentation of hyphema (P = .05), intraocular lens dislocation or loss (P = .006), vitreous hemorrhage (P = .0002), scleral rupture (P = .001), a long interval from surgery to trauma (P < .0001), and fall as the cause of trauma (P < .0001). In a multivariate model, only a surgery-to-trauma interval longer than 8 weeks was associated with a worse visual outcome (P < .0001). Visual acuity immediately after trauma was a poor predictor of final visual acuity. CONCLUSIONS: Visual outcome after traumatic wound dehiscence of a cataract surgery wound was strongly associated with the interval from surgery to trauma. Other factors were less reliable predictors of visual outcome. Traumatic wound dehiscence only moderately affected visual outcome after cataract surgery in most cases.

Retinal and intraventricular cerebral hemorrhages in the preterm infant born at or before 30 weeks' gestation.

PURPOSE: To determine the prevalence of retinal hemorrhages and their association with cerebral intraventricular hemorrhages (IVH) in low-birth-weight preterm neonates born at or before 32 weeks' gestation. METHODS: We prospectively studied a consecutive series of 22 neonates (24-30 weeks' gestation; mean gestational age, 27 weeks; mean weight, 1065 g) admitted to the neonatal intensive care unit. Anterior segment and indirect ophthalmoscopic examination, as well as cranial ultrasonographic examination, were performed on day 1 and day 10 of life. The prevalence of retinal and intraventricular hemorrhage was tested statistically for association with obstetric and neonatal clinical variables. RESULTS: The prevalence of retinal hemorrhage was 9% (2/22; 95% CI, 3%-21%) on day 1 and 2% (1/22) on day 10. The prevalence of IVH was 27% (6/22; 95% CI, 9%-46%): 14% (3/22) on day 1 and 23% (5/22) on day 10. Retinal hemorrhages occurred with greater frequency in neonates born to women who had intrauterine infection (chorioamnionitis, P =.043) and low umbilical cord pH levels (P =.027). No association was found between the presence of retinal hemorrhage and IVH (P = 1.000), mode of delivery (ie, vaginal vs cesarean section, P = 1.000), birth weight (P =.476), or gestational age (P = 1.000). The presence of subconjunctival hemorrhage was associated with IVH (P =.046). CONCLUSIONS: Retinal hemorrhages occur in less than 10% of low-birth-weight neonates, ie, a prevalence one half that observed in term neonates (22%). The hemorrhages tend to resolve without sequelae in the first 10 days of life and occur more commonly in infants born to women with uterine infection. Retinal hemorrhages in very premature neonates are not predictive of IVH-related brain damage.

Retinitis pigmentosa, Coats disease and uveitis.

PURPOSE: To study the anamnestic immune response to retinal specific antigens of two patients suffering from a rare triad of retinitis pigmentosa, Coats disease and uveitis. PATIENTS: 17-year-old girl presented with an acute episode of panuveitis, and her 19-year-old brother suffered from chronic uveitis. On examination, both patients showed retinal vascular changes and subretinal exudations typical of Coats disease, with bone-spicule pigmentary changes as observed in retinitis pigmentosa. RESULTS: All routine examinations were unrevealing. However, the peripheral lymphocytes from these two siblings gave a specific anamnestic response to retinal antigens in vitro. A stimulation index of 4.6 was obtained when the sister's lymphocytes were stimulated with interphotoreceptor binding protein, IRBP--during the acute stage of the uveitis. The brother's lymphocytes showed a stimulation index of 2.7 towards S-Ag during the chronic phase of his uveitic condition. CONCLUSIONS: These results indicate that autoimmunity towards retinal antigens may play some role in specific types of retinitis pigmentosa. Whether these autoimmune reactions are a primary pathological mechanism or are secondary to the extensive destruction of the photoreceptor layer resulting from the retinitis pigmentosa remains debatable.

Ocular and orbital complications following the treatment of retinoblastoma.

PURPOSE: To investigate the ocular and orbital complications observed in children treated for retinoblastoma. SUBJECTS AND METHODS: We retrospectively studied 73 children (39 boys, 34 girls) suffering from retinoblastoma. Thirty-six had bilateral tumor and 37 unilateral disease for a total of 109 eyes affected. The follow-up was 6-180 months (median 36 months). Enucleation was the most common initial treatment approach in the unilateral group, and radiotherapy (by external beam) was the most common initial therapy in the bilateral group. Cryotherapy, photocoagulation, brachytherapy and/or systemic chemotherapy were used as adjuvant treatments when necessary. Ocular complications were recorded at the follow-up examinations. RESULTS: Cataract developed in 20% of the irradiated eyes. The mean time from irradiation until development of cataract was 28.5 months (6-64 months). Radiation retinopathy developed in 12% and was first detected 11-72 months (mean 37 months) after irradiation therapy. Mild transient keratopathy was observed in all eyes undergoing irradiation, and xerophthalmia in one eye. Complications after enucleation included: marked discharge from the socket (11.0%), extrusion of the implant (9.6%), and contraction of the socket (3.0%). No complications were observed after cryotherapy or laser photocoagulation of the tumor. CONCLUSIONS: Ocular complications after treating children with retinoblastoma are common and may seriously affect the quality of life of children surviving the primary malignancy.

The role of preoperative ultrasonography for patients with dense cataract: a retrospective study of 509 cases.

BACKGROUND AND OBJECTIVE: Ocular ultrasonography is widely used for the evaluation of eyes with opaque ocular media. Although preoperative ultrasonography of the globe has been recommended for patients with dense cataracts, the value of such an examination is currently unknown. The purpose of this study was to evaluate the prevalence and nature of intraocular pathologies detected by preoperative ultrasound examination in patients with dense cataract. PATIENTS AND METHODS: The authors retrospectively studied 509 patients referred for routine ultrasound examination of the globe due to dense cataract. Exclusion criteria included known posterior segment pathology, previous ocular surgery or penetrating trauma, and age of younger than 18 years. All the patients underwent ultrasound examination by B-scan and standardized A-scan. RESULTS: In 19.6% of the patients, a posterior segment pathology was revealed by the ultrasound examination. The most frequently disclosed abnormalities were posterior staphyloma (7.2%), retinal detachment (4.5%), and vitreous hemorrhage (2.5%). One patient with a large choroidal malignant melanoma was identified. The prevalence of posterior segment pathologies was slightly higher in patients with a history of ocular trauma, compared with the nontraumatic cataract group (29.6% versus 19.0%, respectively; P = .1). The prevalence of retinal detachment was increased in the traumatic cataract subgroup (14.8% compared with 3.9%), but this difference did not reach statistical significance. CONCLUSION: Preoperative ultrasound examination for patients with dense cataract can be used to detect pathologies that may influence the surgical strategy and the postoperative visual prognosis.

Asymmetric motion visually evoked potentials in infantile strabismus are not an artifact of latent nystagmus.

BACKGROUND: Patients who have infantile strabismus exhibit a directional asymmetry of motion visually evoked potentials (MVEPs) recorded under conditions of monocular viewing. The majority of these patients also have latent nystagmus, raising the possibility that the MVEP asymmetry is an artifact of the nystagmus. To explore this issue, we correlated MVEPs and eye movements under conditions that eliminated or increased latent nystagmus. METHODS: MVEPs and eye movements were recorded under conditions of monocular viewing in three adults who had combined infantile-onset strabismus and latent nystagmus. The subjects viewed vertically oriented grating stimuli that oscillated horizontally at temporal frequencies of 6.6 to 11.0 Hz by use of spatial frequencies of 1 to 3 cycles/degree. Quantitative eye movement recordings of latent nystagmus and horizontal pursuit/optokinetic nystagmus were also obtained. RESULTS: Eye movement recordings showed that the latent nystagmus was absent or markedly diminished when the viewing eye was in a 45-degree adducted position, whereas nystagmus velocity increased 10 to 40 times (to 2.2 to 4.5 degrees/second) when the viewing eye was in an abducted position (p < 0.05). MVEPs were abnormal (asymmetry indices > 0.40) when the viewing eye was in an adducted or abducted position of gaze. No correlation was found between the MVEP asymmetry index and the velocity of latent nystagmus. CONCLUSIONS: MVEP asymmetries in infantile strabismus remain robust under conditions that eliminate or greatly reduce the oscillations of latent nystagmus. MVEP asymmetries and ocular motor abnormalities both characterize infantile strabismus, but the ocular motor defects do not cause the MVEP asymmetries. The nasotemporal asymmetry of MVEPs and the nasotemporal asymmetry of pursuit and latent nystagmus are likely caused by deficits in related but separate binocular visual cortical circuits.

Necrotizing choroiditis-retinitis as presenting symptom of disseminated aspergillosis after lung transplantation.

BACKGROUND: Endogenous endophthalmitis due to Aspergillus is rare affecting the severely immunosuppressed population, in particular recipients of heart and lung transplants. Ocular involvement of aspergillosis has always been observed late in the course of the disease. SUBJECT: A young woman noted blurred vision in one eye three weeks after lung transplantation. At this stage, no systemic manifestations of fungal infection were detected and the ocular findings were attributed to viral infection. RESULTS: Twenty-four hours after the original ocular complaint, an aggressive endophthalmitis developed in the left eye. The possibility of fungal endophthalmitis was raised. Within 48 hours of her first ocular complaint the patient died. Cultures from a vitreous tap and from autopsy ocular specimens were positive for Aspergillus fumigatus. CONCLUSIONS: Aspergillus endopthalmitis may occur in patients undergoing lung transplantation despite antifungal therapy. Increased awareness of this unusual entity may be life and vision saving in these patients.

Variants of congenital ocular motor apraxia: associations with hydrocephalus, pontocerebellar tumor, and a deficit of vertical saccades.

BACKGROUND: Congenital ocular motor apraxia (COMA) is characterized by the inability to generate volitional horizontal saccadic eye movements in the absence of other focal neurologic abnormalities. SUBJECTS: We report on two children (ages 5 months and 3 years) whose COMA did not adhere to these classic criteria. The children were followed up clinically with serial ocular motor examinations and neuroimaging over a period of 3 years. RESULTS: In the first child horizontal COMA was associated initially with neonatal communicating hydrocephalus. Two and one half years after the first signs of COMA, a fourth ventricle medulloblastoma appeared. The second child, who recovered from a periventricular hemorrhage caused by perinatal asphyxia, manifested vertical COMA and compensatory vertical head thrusts. CONCLUSIONS: COMA may be associated with hydrocephalus, pontocerebellar tumor, and periventricular hemorrhage. These rare variants of COMA emphasize that the eye movement deficits may arise from several locations, cerebral as well as pontocerebellar, in the neuronal pathways generating saccades.

Need for confirmation of positioning of ruthenium plaques by postoperative B-scan ultrasonography.

BACKGROUND AND OBJECTIVE: Plaque positioning is of utmost importance for the effective treatment of intraocular lesions. The authors' objective was to verify the position of ruthenium plaques after surgical placement. PATIENTS AND METHODS: The authors studied the positions of ruthenium-106 (Ru-106) plaques in 43 patients treated for choroidal tumors by B-scan echography on the first postoperative day. RESULTS: In 29 of 43 eyes (67.4%) the episcleral plaque was found to be well centered 1 day after surgery. Ten eyes (23.3%) had borderline-placed plaques, and the remaining 4 (9.3%) plaques were malpositioned. All the malpositioned plaques were situated behind posterior tumors whose uncovered edge was adjacent to the optic nerve. CONCLUSION: Ultrasonic verification in the postoperative period is essential to detect inadvertent misplacement or displacement of radioactive plaques. Plaque replacement or additional and modified treatment can be considered for those plaques found to have moved after the surgical placement.

Panuveal malignant mesenchymoma.

Intraocular malignant mesenchymal tumors are very rare, and only a few case reports of such primary and metastatic tumors have been reported. We report a case of a malignant mesenchymoma involving the entire uveal tract. A 21-year-old woman presented with a tumor on the whole iris of the right eye, which caused intractable glaucoma. Upon enucleation of the eye, a very anaplastic tumor was found to occupy the whole uveal tract; its features were compatible with a tumor of mesenchymal origin, including rhabdomyosarcomatous and liposarcomatous characteristics. Choroidal osteoma was a coincidental finding. The histologic findings of the tumor were of two types of malignant mesenchymal tumors, and therefore the diagnosis of malignant mesenchymoma was made. This is to our knowledge the first tumor of its kind to be reported intraocularly.

Visual outcome following traumatic wound dehiscence after cataract surgery.

We reviewed the charts of 21 patients who had cataract surgery and subsequent ocular trauma with wound dehiscence. Eighteen patients were re-examined for this study; the other three were lost to follow-up. A complete examination of both eyes was performed 3 to 84 months after the trauma occurred. Patients were assigned to one of two groups: Group A, minor trauma (n = 14), or Group B, major trauma (n = 7). In Group A, visual acuity was 20/40 or better in four patients, 20/50 to 20/80 in five patients, and 20/300 or worse in three patients. In Group B, visual acuity was 20/60 or better in three patients and 20/300 or worse in three patients. Poor visual acuity was associated with endophthalmitis and bullous keratopathy in Group A and globe rupture or recurrent retinal detachment in Group B. In 14 of the 18 patients, final visual acuity in the traumatized eye was similar to that in the fellow eye, which had the same ocular pathology. This study suggests that pre-existing ocular disease is an important factor in final visual outcome in patients with traumatic wound dehiscence after cataract surgery.

Ocular distribution of the chimeric protein IL2-PE40.

A construct of IL-2 and pseudomonas exotoxin (PE40) has been genetically engineered. An aliquot of 100 microliter of the chimeric protein, radiolabelled with I125, was administered to healthy rats by various routes. At different intervals, ocular and non ocular tissues were removed and the levels of the radiolabelled chimeric protein IL-2-PE40 measured. Systemic administration of IL2-PE40 either intravenously (IV) or intraperitoneally (IP) leads to high levels of the drug in the blood, liver and spleen. Little or no radioactivity is observed within the ocular tissues using this route. On the other hand, local administration of the drug either as subtenon injection or as eye drops resulted in a very high concentration of the drug within the conjunctiva, cornea and sclera, with little radioactivity detected systemically. Subtenon injection induced a significant drug level within the optic nerve. With the drops, the chimeric protein was also detected, in low levels, intraocularly.

Choroidal metastasis of cutaneous malignant melanoma treated by ruthenium-106 plaque irradiation.

Symptomatic dissemination of cutaneous malignant melanoma to the choroid occurs rarely and is a grave prognostic factor. Therefore, choroidal tumors are treated only as a palliative measure. We present a case of cutaneous malignant melanoma with symptomatic metastasis in the choroid OD of a 38-year-old woman. We treated it using brachytherapy with a ruthenium-106 plaque. The tumor totally regressed with restoration of good vision. Twelve months of follow-up showed no systemic spread.