Larissa A. Habib

Selective laser trabeculoplasty: Does energy dosage predict response?

Background: Selective laser trabeculoplasty (SLT) is a widely used treatment for open angle glaucoma, producing sustained reductions of intraocular pressure (IOP). The aim of this study was to evaluate the long-term relationship between SLT energy dosage and IOP reduction. Materials and Methods: A retrospective review was performed for patients receiving primary SLT therapy, with inclusion of subjects treated with 360° of SLT. Energy settings were collected upon treatment and IOP was collected at baseline up to 36 months. Pearsons correlation coefficient was used to determine whether there was a significant correlation between SLT energy and IOP reduction at all time points. Kaplan-Meier analysis with log-rank test was performed to determine the differences in IOP reduction ≥20% from baseline among those treated with low (<85 mJ), medium (85-105 mJ), and high (>105 mJ) energy SLT. Results: A total of 104 eyes (75 patients) were included. The mean total SLT energy was 93.73 mJ (standard deviation (SD) = 21.83 mJ, range: 34.4-122 mJ). A significant positive correlation (P ≤ 0.05) between the amount of energy delivered and IOP reduction was found at all time points. Log-rank test showed a significant difference in IOP reduction ≥20% from baseline between the three energy groups, with low energy patients experiencing failure at an earlier time (P = 0.05). Conclusions: Within the range of total energy examined, there is a positive correlation between total energy used and amount of pressure reduction achieved at up to 3 years of follow-up. This may be useful in determining the optimal energy dosage for maximum effect for patients receiving SLT.

Graded Marginal Recession: A Surgical Technique to Correct Small Angle Vertical Deviations.

PURPOSE To describe a novel muscle recession technique to surgically correct small angle vertical deviations in symptomatic adults with fusion potential. METHODS A novel technique involving a graded recession of the medial and lateral poles of a vertical rectus muscle, combined with graded medial and lateral tenotomy of the muscle, was used to treat small vertical deviations. A surgical nomogram was developed based on the configuration of the procedure and its predicted effects. Four patients with small angle hypertropia ranging from 1 to 5 prism diopters (PD) underwent the graded marginal recession procedure and were observed for up to 3 years. RESULTS Three of the four patients had successful correction of their strabismus and resolution of diplopia, with no complications or induced incomitance. One patient was initially orthotropic but showed a 2 PD regression 1 month postoperatively, eventually requiring additional surgery to achieve stable orthotropia. CONCLUSIONS The graded marginal recession can be safely and effectively used to correct very small vertical deviations in adults with fusion potential. A surgical nomogram can be created to guide predicted corrections in increments of less than 0.5 PD.

Spontaneous regression of inflammatory myofibroblastic tumor of the orbit: A case report and review of literature

ABSTRACT Inflammatory myofibroblastic tumor is a rare entity characterized by the presence of myofibroblasts and inflammatory cells within a fibrous stroma. It typically occurs in the pediatric population. The most common site of occurrence is the lung though it has been reported throughout the body. Although rare, it has been reported in the orbit. The clinical course is ill defined in the literature; here we report a case of pediatric IMT with delayed spontaneous regression.

Intravitreal chemotherapy in retinoblastoma: expanded use beyond intravitreal seeds

Background/aims Ophthalmic artery chemosurgery (OAC) has changed the face of retinoblastoma treatment and led to a higher rate of globe salvage. The introduction of intravitreal chemotherapy (IVitC) has further enhanced globe salvage with increased success in treatment of intravitreal seeds. Our group has seen success at treating non-vitreous disease that is refractory to OAC using IVitC. This study was undertaken to quantify and report on this success. Methods A retrospective review was used to identify patients treated with IVitC for indications other than vitreous seeds from two centres. The indication, prior and concurrent treatment, response time and duration of treatment were documented. Kaplan-Meier estimates were used to evaluate ocular and recurrence-free survival. Ocular toxicity was evaluated using the 30 Hz flicker electroretinogram (ERG). Continuous and categorical variables were compared with Student’s t-test and χ2 test, respectively. Results Fifty-six eyes from 52 retinoblastoma patients were identified. There were no disease-related or treatment-related deaths. One patient developed a second primary malignancy (pinealoblastoma) and subsequent leptomeningeal spread. Ninety-eight per cent of the eyes showed clinical regression. Recurrence was seen in 14.3%. Of the recurrences, five occurred in retinal tumours and three in subretinal seeds. The Kaplan-Meier estimated risk of recurrence in all patients treated was 83.5% (95% CI 7.9 to 14.1) at 10 months. The mean change in ERG over treatment course was −17.7 μV. Conclusions Intravitreal chemotherapy is successful for the treatment of subretinal seeds and recurrent retinal tumours and could be considered as adjunctive therapy in globe-sparing treatment of retinoblastoma.

Simultaneous ipsilateral transconjunctival repair of upper and lower eyelid retraction in thyroid-associated ophthalmopathy

ABSTRACT Purpose: To report a simple, highly effective technique of simultaneous transconjunctival repair of upper and lower eyelid retraction in patients with thyroid eye disease (TED). Methods: A retrospective interventional case review was conducted on 22 eyes of 19 TED patients. The lower eyelid was recessed with placement of a tarsoconjunctival spacer graft harvested from the upper eyelid. The upper eyelid was then recessed through the conjunctival incision used to harvest the tarsal graft. A temporary tarsorrhaphy was placed for 5–7 days. The postoperative outcome was assessed by measuring the margin reflex distance of the upper eyelid (MRD1), inferior scleral show (ISS), and lagophthalmos. Results: The absolute change in MRD1 ranged from 0 to 5 mm with an average of 1.86 ± 1.34 mm. The absolute change in ISS ranged from 0 to 2 mm with an average of 1.3 ± 0.49 mm. One patient had postoperative lagophthalmos and 17 of 19 had improvement in their ocular surface exposure symptoms. None of the patients’ grafts were observed to undergo absorption during the postoperative course. Conclusions: This technique of harvesting a free tarsoconjunctival graft from the upper eyelid as a posterior spacer for the lower while simultaneously recessing the upper eyelid through the same incision is an effective and durable method of correcting eyelid retraction in TED.

Two Uveal Melanomas in One Eye: A Choroidal Nevus Giving Rise to a Melanoma in an Eye with a Separate Large Choroidal Melanoma

Multifocal uveal melanomas are extremely rare. In this case report, we describe a patient with 2 independent uveal melanomas in the same eye. A 52-year-old woman presented with a large choroidal melanoma and a smaller ciliary body mass, clinically thought to be a nevus, in her left eye. Enucleated specimen showed 2 primary lesions that were anatomically separate. Lesion 1 was a melanoma and lesion 2 was a melanoma arising centrally from a nevus. Both lesions harbored GNAQ mutations. This patient had no family history of uveal melanomas or signs of ocular melanocytosis and was negative for the BAP1 mutation. This case demonstrates how multifocal uveal melanomas can arise in patients who lack genetic predisposition for the disease. Furthermore, this case is one of the few that have shown, histopathologically, a small focus of malignant cells developing from a benign population within a nevus, which highlights the importance of closely monitoring nevi for signs of malignancy.

Long-Term Follow-Up of 4 Patients with Conjunctival Amyloidosis

Conjunctival amyloidosis is a rare cause of ocular inflammation, mass, and hemorrhage that can be difficult to diagnose and treat. In this case series, we describe 4 patients with a histopathological diagnosis of conjunctival amyloidosis treated at a single institution. All patients underwent surgical excision and biopsy. On histopathological examination, 3 patients had local deposition of either kappa or lambda monoclonal immunoglobulin light chains, favoring localized amyloid light-chain amyloidosis. Systemic workup to exclude rheumatologic disorders (e.g., anti-neutrophil cytoplasmic antibody and rheumatoid factors) and hematological disorders (e.g., imaging, biopsies, and serum protein electrophoresis/urine protein electrophoresis) was negative except for a positive abdominal fat biopsy in 1 patient. Patients were followed for an average of 6.1 years (range 4 months to 15 years) with stable ocular disease.

BRAF, NRAS, and GNAQ Mutations in Conjunctival Melanocytic Nevi

Purpose To evaluate BRAF, NRAS, and GNAQ mutations in surgical specimens of common and blue conjunctival melanocytic nevi. Methods Surgical specimens from 25 conjunctival melanocytic nevi (23 common and 2 blue) of 25 patients were evaluated. All common nevi were analyzed immunohistochemically for the expression of BRAF V600E or NRAS Q61R. One lesion with negative immunoreactivity and for all blue nevi, a hybridization capture-based next-generation sequencing method was employed for mutation analysis. For common nevi, genetic features were compared with clinical and histopathologic findings. Continuous variables (age at excision and largest basal diameter) were compared with a Studentss t-test and all categoric variables were compared with Fishers Exact Test. Results Of common melanocytic nevi, 9 (39.1%) were immunoreactive for NRASQ61R and 13 (56.5%) were immunoreactive for BRAFV600E. One common nevus, which was immunonegative for both BRAFV600E and NRASQ61R was found to harbor an NRASQ61K mutation by sequence analysis. Patients with NRAS-mutated nevi were more likely to report occurrence of the lesion prior to 18-years old and more likely to have intrinsic cysts. The mean largest basal diameter was 6.0 and 3.5 mm for NRAS- and BRAF-immunoreactive lesions, respectively (P = 0.003). GNAQ mutations were identified in each of the two blue nevi of this study. Conclusions These findings document that common conjunctival melanocytic nevi have mutually exclusive mutations in BRAF and NRAS. The two conjunctival blue nevi harbored GNAQ mutations. This suggests the driver mutations of conjunctival nevi are similar to those of nevi of the skin. At the molecular level, conjunctival nevi appear more like cutaneous nevi than choroidal nevi.

Peripheral leptochoroid: clinical and anatomical findings

Background/Aims To investigate the clinical findings and anatomical characteristics of patients with a thinned peripheral choroid (leptochoroid) presenting with bilateral macular geographical hyperpigmented fundus and compare with matched controls. Methods A retrospective, observational case series of 44 eyes (24 patients): 22 study eyes (12 study patients) with clinical findings of leptochoroid (geographical hyperpigmented fundus centred in the macula) matched with 22 control eyes (12 control patients). All eyes received enhanced depth imaging optical coherence tomography at Memorial Sloan Kettering Cancer Center. Two independent observers performed all measurements. Statistical analysis was used to correlate interobserver findings, and compare patient and eye characteristics. Results Study patients had a female predominance and median age of 70 years. Iris colour and refractive error of these eyes were varied: 8 eyes were myopic (one of which was highly myopic with a refractive error of −9.00) and 14 eyes were emmetropic or hyperopic (up to+3.00). The best-corrected visual acuity was 20/40 or better in all study eyes. The geographical macular areas of relative hyperpigmentation correspond to normal choroidal thickness compared with controls (p=0.74). The relative hypopigmented surrounding fundus had significantly thinner choroid compared with controls (p value=0.0001). Choroidal thickness had a strong interobserver correlation (r=0.99, p<0.0001). Conclusion Described here is a clinical entity referred to as peripheral leptochoroid. It appears as geographical hyperpigmented fundus centred in the macula, with adjacent relative hypopigmented fundus. Compared with matched normal subjects, the hyperpigmented and hypopigmented fundus correspond with equivocal and decreased choroidal thickness, respectively.

Second primary malignancies in retinoblastoma patients treated with intra-arterial chemotherapy: the first 10 years

Background/Aims Survivors of retinoblastoma carry a lifetime risk of secondary malignancies. It is well established that external beam radiation increases this risk; however, the risk with ophthalmic artery chemosurgery (OAC) remains unknown. We report on 10 years of experience with OAC and the rate of second primary malignancy (SPM) development. Methods This is a single-centre retrospective review approved by the Memorial Sloan Kettering Cancer Center Institutional Review Board of all patients who received OAC over a 10-year period, from May 2006 to November 2016. The second tumour incidence and survival in patients with germline disease (bilateral and unilateral with family history or confirmed germline mutation) was estimated using the Kaplan-Meier method. Patients who received external beam radiotherapy were excluded from analyses. Results Two hundred and thirty-three patients with heritable retinoblastoma who received OAC were analysed. Nineteen patients were excluded for having received external beam radiation. The Kaplan-Meier estimate of the likelihood for SPM development was 2.7% at 5 years (95% CI 0 to 25). All of the SPMs were pineoblastomas and all patients had bilateral disease in this cohort. Conclusions In our 10-year experience, we have found that SPM development in patients with germline retinoblastoma treated with OAC alone is comparable to previously published rates. In the first 10 years, OAC did not increase the known incidence of SPMs. This cohort will continue to be followed to establish the rate of development with extended follow-up.