Irene Anteby

Hereditary subluxated lenses: Visual performances and long-term follow-up after surgery☆

PURPOSE To evaluate the visual functions of children with hereditary dislocated lenses after within-the-bag lensectomy surgery and to report the results of 18 eyes with a follow-up interval of 11 to 19 years. DESIGN Prospective, noncomparative, interventional case series. PARTICIPANTS Ninety-seven children (age range, 6 months-16 years) were examined. Fifty-seven patients had poor functional vision. Surgery was performed in 97 eyes. METHODS Fifty-nine eyes (37 patients) were operated on from 1980 through 1994, and 38 eyes (20 patients) underwent surgery from 1994 through 2001. The indications for surgery included a best-corrected visual acuity of less than 20/60, progressive subluxation of the lens with bisection of the pupil, imminent total dislocation, or cataractous changes. Within-the-bag lensectomy combined with a limited anterior vitrectomy was carried out either through the pars plana or the limbus. The resulting aphakia was corrected with contact lenses, glasses, or both and the children were followed up regularly. MAIN OUTCOME MEASURES Best-corrected visual acuity, intraocular pressure, and complications after surgery. RESULTS Preoperative mean visual acuity for all operated eyes was 0.17 +/- 0.1 (standard deviation). The postoperative mean visual acuity of the 59 eyes operated from 1980 through 1994 was 0.60 +/- 0.25. For the group of 38 eyes operated on from 1994 through 2001, the postoperative visual acuity was 0.63 +/- 0.22. In most eyes, vision achieved shortly after the surgery remained stable or improved on a follow-up of up to 19 years after surgery. Repeated refraction of the operated eyes showed a trend toward a decrease of hypermetropia. No major complications occurred during or shortly after surgery. Ten children (18 eyes) followed up regularly for more than 11 years and up to 19 years after surgery demonstrated a stable visual acuity with minor fluctuations and an intraocular pressure within the normal range in all operated eyes. CONCLUSIONS Within-the-bag lensectomy is a safe and rewarding procedure for the handling of dislocated lenses in children.

Treatment options in the management of choroidal metastases.

We performed a retrospective study of 40 consecutive patients (50 eyes) treated for choroidal metastases of solid systemic malignancies in order to evaluate treatment results. Patients received either systemic or local therapy or a combination of both. The most common primary tumor was breast carcinoma (62.5%). Systemic chemotherapy alone was used in 13.3% of eyes, local therapy alone in 44.4%, and a combination of both in 42.2% of eyes. Local treatment modalities included brachytherapy, external beam irradiation, and laser photocoagulation. Complete regression of the choroidal metastases was seen in 57.8% of eyes, partial regression in 15.6 and no response in 4.4%; 22.2% were not available for re-evaluation. We have concluded that the treatment modality in patients with metastatic ocular disease should be individually tailored. When ocular metastases are concurrent with widespread metastatic disease, systemic chemotherapy alone or in combination with local therapy is reasonable. In patients manifesting metastases in the eyes alone, local therapy modalities may be safe, allowing conservation of visual functions with minimal systemic morbidity.

Conjunctival ulcers in Behçet's disease

PURPOSE To describe the occurrence of conjunctival ulcers as a manifestation of Behçets disease. DESIGN Retrospective, noncomparative, interventional case series with histopathologic correlation. METHODS Six patients who fulfilled the diagnostic criteria for Behçets disease and presented with painful conjunctival ulcers were included in the study. Three of these ulcers were biopsied and studied histologically and immunohistochemically. The lesions were treated with topical or subconjunctival injection of corticosteroids and, in one case, with oral indomethacin. RESULTS Although all six patients fulfilled the diagnostic criteria for Behçets disease, two developed uveitis and other signs of Behçets disease only months to years after the appearance of the conjunctival ulcers. The 3- to 5-mm, round to oval ulcers were located in the limbal and/or bulbar conjunctiva. Histopathology revealed disrupted epithelium, infiltration of both acute and chronic inflammatory cells, and high endothelial venules. Immunohistochemical analysis of the infiltrating lymphocytes revealed primarily T-cell populations admixed with several B cells and CD68-positive histiocytes. After treatment, the conjunctival lesions invariably healed without scarring. CONCLUSIONS In addition to the oral and genital ulceration, ulcers can also be found in the conjunctiva of patients with Behçets disease. Although this is a rare clinical sign, when accompanied by uveitis or orogenital ulcers, it may suggest a diagnosis of Behçets disease.

Changes in ultrasound findings in posterior uveal melanoma after ruthenium 106 brachytherapy

PURPOSE To analyze the postbrachytherapy ultrasonographic dynamics of uveal melanoma tumor height and internal reflectivity. DESIGN Prospective, comparative, observational case series. PARTICIPANTS One hundred forty-seven patients (147 eyes) with posterior uveal melanoma having a mean age of 61 years (range, 29-97 years) who were treated with Ruthenium 106 brachytherapy. METHODS Patients were followed-up with ultrasonography using both A and B modes of standardized echography every 6.7 +/- 0.3 months (mean +/- standard error of the mean) for a total of 1001 ultrasound examinations. On average, each patient was examined 5.8 times (range, 3-17 times). The echographic parameters included tumor base size, height, internal reflectivity, regularity, vascularity, and extra-scleral extension. To compare the response of tumors of different sizes, each tumor was standardized to its initial size at brachytherapy. MAIN OUTCOME MEASURES The dynamics of the tumor height and internal reflectivity. RESULTS At the time of brachytherapy, the mean height of the tumors was 5.2 mm (range, 2.2-11.8 mm). After brachytherapy, 142 tumors (96.6%) responded by a decrease in height. The decrease in height was at an initial rate of approximately 3% per month. Most of the tumors did not regress entirely; rather, their height stabilized on a constant value (on average 61% of the initial height) after approximately 18 to 24 months. The decrease in height after brachytherapy was best fitted by the sum of a first order exponential decay and a constant (h = 61 + 35*e(-0.12t), in which t = time in months). The half-life of the decay was 5.8 months. Large tumors (>8 mm) had a faster initial decrease in height, and stabilized on a lower percentage of their initial height (50%) compared with small tumors (70%). Thus, the half-life of the height exponential decay was 5.3 months for small tumors (2-4 mm) and 3.3 months for the large tumors (>8 mm). Internal reflectivity increased from a mean of 40% before therapy to 70% after 2 years. The dynamics of the reflectivity were best fitted with the function: f = 45 + 24(1-e(-0.09t)). Larger tumors, which initially had lower internal reflectivity, presented with a slower increase in internal reflectivity (t() = 8.7 months) compared with smaller tumors (t(1/2) = 5 months). CONCLUSIONS The postbrachytherapy ultrasonographic dynamics of uveal melanomas resemble a function composed of the sum of a constant and a first order exponent, suggesting the possible existence of two components (cell populations), one unaffected by brachytherapy and the other a radiosensitive population that reacts to brachytherapy in an exponential decay. An exponential decay can imply that the postbrachytherapy death of each tumor cell is a stochastic Markovian process that is independent of the death of other tumor cells.

Eyelid Pilomatrixoma : A Description of 16 cases and a Review of the Literature

Pilomatrixoma is an uncommon benign neoplasm that originates from the matrix of the hair root. It occurs more frequently in the head and neck region of children and adolescents, often involving the eyelid or eyebrow. Pilomatrixoma is often misdiagnosed clinically and the correct diagnosis can be established only after excision and histological examination. Pathologic diagnosis of pilomatrixoma is based on the finding of large masses of shadow cells, combined with basophilic cells, inflammation, foreign body giant cells, calcification, and ossification. We report 16 cases of eyelid pilomatrixoma that were treated in our department, and review the relevant literature.

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.

Refractive profile in oculocutaneous albinism and its correlation with final visual outcome

Purpose To evaluate the prevalence of refractive errors in different subtypes of oculocutaneous albinism, and to see if there is any correlation between refractive errors and final visual outcome in this population. Patients/methods This is a retrospective study of 132 albino patients, ranging in age from 0.5 to 35 years. They were divided into four subtypes: OCA1A, OCA1B and OCA1C, and OCA2. Refractive errors were evaluated objectively by cycloplegic refraction and subjectively in cooperative patients. Best corrected visual acuity was assessed binocularly. Refractive errors were divided into three groups—hypermetropia, myopia and astigmatism—to avoid the use of spherical equivalent. Results Refractive errors were mainly astigmatism and hypermetropia. The OCA1A group showed high hypermetropia (≥5 dioptres) in 43.4% of patients, reaching significantly higher levels than in other subgroups (p=0.007). Mean visual acuity in logMAR was: OCA1A=0.81, OCA1B=0.64, OCA1C=0.61 and OCA2=0.48. Astigmatism averaged 2.1 dioptres (consistently with-the-rule), and it was homogeneously distributed between all subgroups (53%). Conclusions The poorest visual acuity was found in those with OCA1A, which was associated with the highest rate of high hypermetropia (statistically significant different from other subgroups). Astigmatism was the most common visually significant refractive error across all subtypes of albinism. These results may help to clarify the prevalence of refractive errors in albino patients and aid the prediction of visual outcome in this heterogeneous population.

Expression and activation of toll-like receptor 3 and toll-like receptor 4 on human corneal epithelial and conjunctival fibroblasts

BackgroundToll-like receptors (TLRs) are recognized as important contributors to the initiation and modulation of the inflammatory response in the eye. This study investigated the precise expression patterns and functionality of TLRs in human corneal epithelial cells (HCE) and in conjunctival fibroblasts (HCF).MethodsThe cell surface expression of TLRs 2-4, TLR7 and TLR9 in HCE and HCF was examined by flow cytometry with or without stimulation with lipopolysaccharide (LPS) or polyinosinic:polycytidylic acid (poly I:C). The mRNA expression of the TLRs was determined by real-time PCR. The protein content levels of interleukin (IL)-6, IL-8, IL-1β and tumor necrosis factor-α (TNF-α) were measured in HCE and HCF using multiplex fluorescent bead immunoassay (FBI).ResultsThe surface expression of TLR3 and TLR4 was detected on both HCE and HCF. Following incubation with LPS, the percentage of HCE cells staining for TLR4 decreased from 10.18% to 0.62% (P < 0.001). Incubation with poly I:C lowered the percentage of HCE cells positive for TLR3 from 10.44% to 2.84% (P < 0.001). The mRNA expression of TLRs2, 4, 7 and 9 was detected in HCE only. Activation of HCE with LPS complex elicited protein secretion up to 4.51 ± 0.85-fold higher levels of IL-6 (P < 0.05), 2.5 ± 0.36-fold IL-8 (P > 0.05), 4.35 ± 1.12-fold IL-1β (P > 0.05) and 29.35 ± 2.3-fold TNFα (P < 0.05) compared to cells incubated in medium.ConclusionsHCF and HCE both express TLRs that respond to specific ligands by increasing cytokine expression. Following activation, the surface expression of TLR3 and TLR4 on HCE is decreased, thus creating a negative feedback loop, mitigating the effect of TLR activation.

Oculoectodermal syndrome with coarctation of the aorta and moyamoya disease: expanding the phenotype to include vascular anomalies.

Oculoectodermal syndrome (OES, OMIM 600268) is characterized by discrete areas of alopecia of the scalp and epibulbar dermoids which are present from birth, with additional variable anomalies. We describe two patients, one of whom has been reported [Lees et al. (2000); Am J Med Genet 91:391–395], who both had coarctation of the descending aorta and moyamoya disease, and developed epilepsy, recurrent transient ischemic attacks, and cerebrovascular accidents during early childhood. We suspect that the vascular findings are an integral part of the syndrome.

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.