COMBINED HAMARTOMA OF THE RETINA AND RETINAL PIGMENT EPITHELIUM AT PEDIATRIC AGE: Surgical Versus Conservative Approach.

PURPOSE: To report outcomes of pediatric patients with combined hamartoma of the retina and the retina pigment epithelium followed up conservatively or after pars plana vitrectomy. METHODS: This retrospective multicenter study included 62 eyes of 59 pediatric patients with combined hamartoma of the retina and the retina pigment epithelium from 13 different international centers with an average age of 7.7 ± 4.7 (0.3-17) years at the time of the diagnosis and having undergone pars plana vitrectomy or followed conservatively. At baseline and each visit, visual acuity values, optical coherence tomography for features and central foveal thickness, and tumor location were noted. Lesions were called as Zone 1, if it involves the macular and peripapillary areas, and the others were called as Zone 2 lesions. RESULTS: Twenty-one eyes of 20 patients in the intervention group and 41 eyes of 39 patients in the conservative group were followed for a mean of 36.2 ± 40.4 (6-182) months. Best-corrected visual acuity improved in 11 (68.8%) of 16 eyes in the intervention group and 4 (12.9%) of 31 eyes in the conservative group ( P < 0.001). The mean central foveal thickness decreased from 602.0 ± 164.9 µ m to 451.2 ± 184.3 µ m in the intervention group, while it increased from 709.5 ± 344.2 µ m to 791.0 ± 452.1 µ m in Zone 1 eyes of the conservative group. Posterior location of tumor, irregular configuration of the foveal contour and ellipsoid Zone defect in optical coherence tomography, subretinal exudate and prominent vascular tortuosity were associated with poor visual acuity. CONCLUSION: Vitreoretinal surgery is safe and effective in improving vision and reducing retinal distortion in Zone 1 combined hamartoma of the retina and the retina pigment epithelium in children.

A prospective, interventional study comparing the outcomes of macular hole surgery in eyes randomized to C3F8, C2F6, or SF6 gas tamponade.

PURPOSE: Analysis of outcomes of macular hole (MH) surgery using 12% C3F8, 16% C2F6, or 18% SF6 as randomized gas tamponading agent. METHODS: This is a prospective, randomized, interventional study of 159 eyes with idiopathic MH undergoing 23/25G pars-plana vitrectomy with internal limiting membrane peeling and gas tamponade. Eyes were stratified into two groups: Group I (MH < 800 µ) and group II (MH > 800 µ) according to MH apical diameter. Eyes in group I were randomized to receive either 18% SF6, 16% C2F6, or 12% C3F8. Eyes in group II were randomized to either 16% C2F6 or 12% C3F8. Clinical details, MH parameters on optical coherence tomography, surgical details and complications were analyzed. Outcome measures were type of hole closure (I/II), best corrected visual acuity, intraocular pressure, and cataract progression. RESULTS: In Group I (n = 139), type 1 closure was achieved in 107 (77%) eyes. Type 1 closure rates in group I per gas tamponade were as follows: SF6 (70%), C2F6 (80%), C3F8 (78%) (p = 0.503, chi-square test for independence). There was no statistical difference in MH closure rates between SF6 and C2F6 (p = 0.134), SF6 and C3F8 (p = 0.186), and C2F6 and C3F8 (p = 0.373). In Group II (n = 20), type 1 closure was achieved in 12 (60%) eyes. Type 1 closure rates in group II per gas tamponade were as follows: C2F6 (75%), C3F8 (50%) (p = 0.132, Two proportion Z test). Mean follow-up after surgery was 2.66 ± 2.74 months. CONCLUSION: Given the similar outcomes of using 18% SF6, 16% C2F6, or 12% C3F8 in idiopathic macular hole surgery, the advantage of using a shorter acting tamponade translates into earlier recovery and rehabilitation.

Miliary tuberculosis presenting as bilateral pseudo-retinoblastoma.

Objective: To describe an interesting case of miliary tuberculosis mimicking retinoblastoma. Method: A retrospective case report. Result: The twin brother of a known case of retinobastoma presented with headache. On fundus examination, multiple yellowish-white lesions were noted in both eyes. Magnetic resonance imaging of the brain showed multiple enhancing lesions. A diagnosis of miliary tuberculosis was made and anti-tubercular therapy was started. Conclusion: Ocular tuberculosis can mimic retinoblastoma and lead to diagnostic dilemma especially in cases with family history of retinoblastoma.

Vitreous Seed Classification and Regression Patterns in Eyes With Retinoblastoma.

Purpose: This work subclassifies retinoblastoma vitreous seeds and evaluates the efficacy, regression patterns, and adverse effects of combination intravitreal melphalan and topotecan chemotherapy for resistant and recurrent vitreous seeds. Methods: A retrospective review of medical records was conducted of patients with retinoblastoma and resistant or recurrent vitreous seeds who were treated with intravitreal melphalan and topotecan injections from August 2014 to July 2018. Main outcome measures included regression pattern, time for regression, time for recurrence of seeds, treatment outcomes, and ocular toxicity. Results: Nineteen eyes received 138 intravitreal injections over 74 treatment sessions (mean, 7.26 injections per eye); vitreous seeds regressed in 18 eyes. Of cloud vitreous seeds, curvilinear (n = 2) and sphero-linear (n = 2) subtypes were observed. During regression, some sphere seeds showed an intermediary streak-like pattern and took longer to regress (mean, 11.13 ± 14.05 months and 11.67 ± 8.62 injections) than those without the intermediary streak-like pattern (mean, 3.55 ± 2.57 months and 4.2 ± 1.87 injections). Mean follow-up was 34.87 ± 21.09 months (median, 35 months; range, 11-96 months). Anterior segment toxicity was seen in 10 (53%) eyes and posterior segment toxicity in 5 (26%) eyes. Kaplan-Meier survival estimates for globe salvage at 2 years was 94% and 73% at 5 years. Kaplan-Meier survival for vitreous seed-free status was 94% at 2 years and 65% at 5 years. Conclusions: An expanded vitreous seed classification system that further subcategorizes hitherto unrecognized vitreous seed morphology is needed. An intermediate streaking process results in a prolonged regression time for sphere vitreous seeds.

Outcome of sutured scleral fixated intraocular lens in Marfan syndrome in pediatric eyes.

PURPOSE: To report the clinical outcomes and complications in eyes with Marfan syndrome with subluxated/dislocated lens undergoing sutured scleral fixated intraocular lens (SSFIOL) implantation. METHODS: A retrospective review was done for a consecutive series of 73 eyes of 43 patients with Marfan syndrome, with age less than 18 years who underwent SSFIOL from July 2000 to June 2017. Postoperative best corrected visual acuity, intraocular pressure and refractive error, intraoperative and postoperative complications and time for the development of these complications were analyzed. RESULTS: Visual acuity improved significantly to 0.43 ± 0.30 (p = 0.01), 0.40 ± 0.3 (p = 0.00) and 0.39 ± 0.3 (p = 0.00) logMAR units at 6 weeks, 1 year and at last follow-up, respectively. The refractive error reduced significantly (p = 0.056, 0.039 and 0.031) at 6 weeks, 1 year and final follow-up following surgery. Intraoperative complications included iatrogenic retinal break (n = 3) and surgical trauma to the iris (n = 1). Postoperative complications included increased intraocular pressure in 3 eyes (4.1%), intraocular lens dislocation/subluxation in 5 eyes (6.8%), retinal detachment in 3 eyes (4.1%), choroidal detachment in 1 eye (1.3%), pupillary capture in 15 eyes (20.5%), vitreous hemorrhage in 1 eye (1.3%), pre-retinal hemorrhage in 1 eye (1.3%) and hyphema in 1 eye (1.3%). CONCLUSION: SSFIOL provides good visual outcomes in eyes with ectopia lentis associated with Marfan syndrome. Complications can be seen in some cases, and the parents need to be counseled regarding the same before surgical intervention.

Surgical Management of Advanced Coats Disease in 32 Eyes: A 20-Year Study.

Purpose: This work studies outcomes of external subretinal fluid (SRF) drainage in management of eyes with advanced Coats disease. Methods: Patients with advanced-stage Coats disease (≥stage 3B), who were younger than 12 years and underwent external SRF drainage from 1996 to 2016, were included in this retrospective study. Surgical intervention involved external drainage of SRF and cryotherapy. SRF drainage was performed by lamellar scleral dissection or by external needle drainage. Favorable anatomical outcome was defined as retinal reattachment with normal intraocular pressure (IOP). IOP greater than 24 mm Hg was considered raised. Univariate and multivariate analyses were performed to measure the association between preoperative or intraoperative factors and retinal status at final follow-up. Outcome measures evaluated included visual acuity, IOP, retinal status, globe status, and complications of surgery. Kaplan-Meier analysis was performed for globe salvage without pain. Results: Thirty-two eyes of 32 patients were included in the study. Mean age at surgery was 3.8 ± 3 years. The mean duration of follow-up was 7 years (range, 6 months-15.7 years). Improvement in visual acuity was seen in 5 eyes. Retina was attached at final visit in 6 eyes. IOP in the range of 8 to 24 mm Hg was noted in 16 eyes. Favorable anatomical outcome was achieved in 3 (9%) eyes. Globe salvage was achieved in 84% of eyes. Complications included intraoperative vitreous hemorrhage (n = 1) and postoperative inflammation (n = 1). Kaplan-Meier ocular survival rate without pain at 10 years was 76%. Conclusions: SRF drainage and cryotherapy in eyes with advanced Coats disease favorably alter the natural history of the disease and prevent end-stage complications. Visual outcomes remain poor.

Coexisting Optic Disc Melanocytoma and Pituitary Adenoma.

AIM: To report a rare case of optic disc melanocytoma coexistent with pituitary adenoma. METHODS: Ophthalmological examination with perimetry and magnetic resonance imaging (MRI) of the brain and orbits was done. RESULTS: A 42-year-old woman presented with complaints of progressive diminution of vision in the right eye of 6 weeks' duration. Visual acuity in the affected right eye was 6/60 and in the left eye it was 6/6. On examination, a brown-black lesion was noted over the optic nerve head in the right eye. On perimetry, the visual field was constricted in the right eye and a superior altitudinal defect was found in the left eye. MRI of the brain and orbits revealed a pituitary adenoma with suprasellar extension. The patient underwent endoscopic transphenoidal pituitary adenoma excision with uneventful recovery. Three months following surgery, visual acuity improved to 6/6 in both eyes with partial visual field recovery and was maintained at 10 months of follow-up. There was no recurrence or metastasis. CONCLUSION: A high degree of suspicion is required to screen for possible coexistent orbital or central nervous system pathology in cases of optic disc melanocytoma with disproportionate signs or symptoms.

Outcome of Sutured Scleral-Fixated Intraocular Lens in Blunt and Penetrating Trauma in Children.

BACKGROUND AND OBJECTIVE: To determine the anatomical and functional outcomes of sutured scleral-fixated intraocular lens (SSFIOL) implantation in children with blunt and penetrating injuries to the eye. PATIENTS AND METHODS: This is a retrospective, interventional case series study. Case records of children who underwent SSFIOL implantation in a tertiary eye care facility for traumatic aphakia, cataract, or subluxation were screened. Relevant data on demographics, visual acuity (VA) outcomes, and complications and their management were collected. Results for blunt and penetrating trauma were compared. RESULTS: There was stability or improvement of vision in 88.9% of eyes during the follow-up period. Young age at time of trauma (P = .031) and SSFIOL implantation (P = .002), history of retinal detachment (RD) before SSFIOL implantation (P = .019), poor preoperative best-corrected VA (BCVA) (P = .004), and development of RD in the follow-up period (P = .046) were independent risk factors for low final BCVA on univariate regression analysis. RD rate was 6.53% and was comparable in open and closed globe injuries. Intraocular lens (IOL) dislocation rate was 3.9%, and probability of survival was higher for open globe (0.78) as compared to closed globe (0.64) injuries (P = .042). CONCLUSIONS: SSFIOL implantation results in good VA improvement in both open and closed globe injuries. RD remains an important vision-threatening complication. IOL dislocation is more likely to occur in closed globe injuries. A prospective study evaluating the outcomes would better elucidate the role of these IOLs. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:757-764.].