Intravitreal and subconjunctival melphalan for retinoblastoma in transgenic mice.

Purpose. To measure the chemotherapeutic effects of focal melphalan (intravitreal and subconjunctival) on tumor burden, hypoxia, and vasculature in LHBETATAG murine retinoblastoma model. Methods. LHBETATAG transgenic mice were treated with a single 1 mcg intravitreal injection of melphalan, 100 mcg subconjunctival injection, or semiweekly 10 mcg subconjunctival injections for 3 weeks. At 1 or 3 weeks, eyes were enucleated, serially sectioned, and processed with haematoxylin and eosin (H&E) for tumor burden measurements and probed with immunofluorescence to analyze tumor hypoxia and vasculature. Results. Focal melphalan significantly reduced retinal tumor size (P < 0.02) when given intravitreally or subconjunctivally. Eyes treated with a one-time intravitreal injection of 1 mcg melphalan had significantly smaller tumors at both 1 week (P = 0.017) and at 3 weeks after injection (P = 0.005). Intratumoral hypoxia showed a significant decline in hypoxia at 1 week following intravitreal injection and after maximum dosage of subconjunctival melphalan. Total vasculature was not significantly affected following intravitreal administration. Conclusion. Focal delivery of melphalan via intravitreal or subconjunctival injection has a significant effect on reducing tumor burden, hypoxia, and vasculature, in the treatment of murine retinoblastoma tumors.

Macular spectral-domain optical coherence tomography in patients with X linked retinoschisis.

AIM: To evaluate macular anatomy in patients with X linked retinoschisis (XLRS) using spectral-domain optical coherence tomography (SD-OCT). METHODS: Consecutive observational case series. Clinical features were obtained through retrospective chart review. Only eyes without prior surgical interventions and those scanned with SD-OCT were included. The OCT images were analysed. RESULTS: Fourteen eyes of seven males with XLRS scanned with SD-OCT, age 5 to 45 years, were identified. On clinical examination, stellate spoke-like cystic maculopathy was present in nine eyes, and an atrophic foveal lesion in five eyes. SD-OCT revealed cystoid spaces accounting for retinal splitting in the inner nuclear layer in 12 eyes, and outer plexiform layer in two eyes of one patient. A few small cysts, not accounting for the foveal splitting, were seen in the outer nuclear layer in four eyes and in the ganglion cell layer and/or nerve fibre layer in six eyes. CONCLUSIONS: SD-OCT localised the foveomacular retinoschisis in XLRS to the retinal layers deeper than the nerve fibre layer. In the present study, the foveomacular schisis was seen most frequently in the inner nuclear layer.

Lack of effect of SU1498, an inhibitor of vascular endothelial growth factor receptor-2, in a transgenic murine model of retinoblastoma.

SU1498, a tyrosine kinase inhibitor of vascular endothelial growth factor receptor 2 (VEGFR-2), has activity against retinal neovascular diseases. To determine if this drug might have clinical utility against retinoblastoma, we evaluated the effects of SU1498, as well as the expression of VEGFR-2, in a transgenic animal model of retinoblastoma. Optical coherence tomography (OCT) was evaluated as a technology to measure retinal tumors in vivo, in response to treatment. Immunofluorescence analysis was performed to evaluate the distribution and expression of VEGFR-2 in enucleated eyes from LHbetaTag transgenic mice and controls at 4, 8, 12, and 16 weeks of age. VEGFR-2 and phosphorylated (p)VEGFR-2 levels were quantitated by Western blot. OCT was used to pair 10-week-old animals based on tumor volume (n=10), and these animals were treated with 6 periocular injections of SU1498 (50mg/kg, given twice weekly) or vehicle for 3 weeks. Tumor burden was determined by histology and in vivo imaging by OCT. VEGFR-2 and pVEGFR-2 expression levels were upregulated during tumorigenesis. However, SU1498 did not significantly reduce tumor burden compared to vehicle (p=0.29). OCT imaging of one matched pair demonstrated equivalent, linear tumor growth despite treatment with SU1498. Retinal tumors can be followed non-invasively and quantitatively measured with OCT. VEGFR-2 is strongly upregulated during tumorigenesis in transgenic retinoblastoma; however, SU1498 does not decrease tumor volume in transgenic murine RB at the studied dose and route of administration.

Corneal graft survival and intraocular pressure control after penetrating keratoplasty and glaucoma drainage device implantation.

OBJECTIVE: To investigate corneal graft survival rates and intraocular pressure (IOP) control in eyes after penetrating keratoplasty (PK) and glaucoma drainage device (GDD) implantation. DESIGN: Retrospective, comparative, consecutive case series. PARTICIPANTS: All patients who underwent PK and GDD implantation at the Bascom Palmer Eye Institute between January 1, 1993 and October 31, 1998. MAIN OUTCOME MEASURES: Graft clarity and IOP control. RESULTS: Of the 72 eyes in 72 patients identified, 47 (65%) underwent combined PK and GDD implantation, and 25 (35%) underwent GDD placement after PK (2-30 months after PK; median, 13 months). The GDD type was Baerveldt 350 mm(2) in 57 eyes, Ahmed in 9, Krupin in 2, and other in 4 eyes. The GDD was placed in the anterior chamber in 54 eyes (75%) and in the vitreous cavity in 18 eyes (25%). Preoperative IOP was 11 to 53 mmHg with or without antiglaucoma medications in 16 eyes (30%) with the GDD implanted in the anterior chamber and in 4 eyes (22%) with the GDD placed in the vitreous cavity (P = 0.76). At 1 year after GDD implantation, the graft was clear in 26 eyes (48%) with the GDD in the anterior chamber compared with 15 eyes (83%) with the GDD in the vitreous cavity (P = 0.013). Forty-eight eyes (89%) with the GDD in the anterior chamber and 18 eyes (100%) with the GDD in the vitreous cavity had IOP between 5 and 21 mmHg with or without antiglaucoma medications (P = 0.33). The mean reduction in IOP, 1 year after surgery, was 12 mmHg among eyes with the GDD in the anterior chamber, compared with 17 mmHg among eyes with the GDD in the vitreous cavity (P = 0.13) CONCLUSIONS: Corneal graft survival at 1 year is significantly higher among eyes with the GDD implanted in the vitreous cavity compared with those in which the GDD is implanted in the anterior chamber. The IOP was significantly lower at 1 year after surgery compared with before surgery in both groups, and there was no significant difference between the groups in IOP control and amount of IOP reduction. There was no significant difference in corneal graft survival or IOP control between eyes with the GDD implanted concurrently with the PK versus after the PK.

Surgical removal of submacular choroidal neovascularization: a clinicopathologic study and factors influencing visual outcomes.

BACKGROUND AND OBJECTIVE: Clinical and histopathologic characteristics of submacular choroidal neovascular membranes (CNV) are investigated and features predictive of postoperative complications or poorer visual outcome following CNV removal are identified. MATERIALS AND METHODS: Records of patients who underwent submacular CNV removal at Bascom Palmer Eye Institute between 1991 and 1998 are reviewed. RESULTS: At 6 months postoperatively, vision was stable or improved in 19/26 (73%) eyes, with 15/26 (58%) having 20/200 to 20/400 vision. Postoperative retinal detachment occurred more commonly in age-related macular degeneration eyes (AMD) (P <0.05), and CNV recurrence occurred most frequently in presumed ocular histoplasmosis (POHS) eyes (P <0.05). The one eye with an extrafoveal CNV in-growth site had the largest improvement in vision postoperatively. Choroid in the specimen was associated with worse postoperative vision (P = 0.05). CONCLUSION: Submacular CNV removal achieves visual stabilization at the 20/200 to 20/400 level in most eyes. Potential risk factors for postoperative complications or poorer visual outcome include the underlying disease process, subfoveal in-growth site of CNV, and presence of choroid in the specimen.

Echography of retinoblastoma: histopathologic correlation and serial evaluation after globe-conserving radiotherapy or chemotherapy.

PURPOSE: To assess the sensitivity of echography in detecting retinoblastoma, compare tumor features observed by echography with histopathology data, and assess the usefulness of echography in serially following retinoblastoma tumors after globe-conserving treatments. METHODS: The medical and echography records of all patients treated for retinoblastoma at the Bascom Palmer Eye Institute between 1991 and 1997 were reviewed. All eyes underwent pretreatment echographic evaluation, and eyes treated with external beam radiotherapy, brachytherapy, or chemotherapy underwent serial follow-up echography. RESULTS: Sixty-nine eyes of 48 patients were identified. Echography demonstrated evidence of retinoblastoma in 69 of 69 (100%) eyes and calcification in 63 (91.3%) eyes. Histopathology was superior to echography in detecting optic nerve invasion, extraocular extension, and presence of calcification. CONCLUSION: Echography is a useful adjunct to indirect ophthalmoscopy in establishing the diagnosis of retinoblastoma. While not as specific as histopathology, echographic evaluation before and after treatment of retinoblastoma permits monitoring of treatment response and may aid in detecting recurrent tumor growth or failure to respond to treatment.

Traumatic macular hole: observations, pathogenesis, and results of vitrectomy surgery.

PURPOSE: To review our experience with vitrectomy surgery techniques for the treatment of traumatic macular holes and the biomicroscopic and surgical findings. DESIGN: Retrospective noncomparative, multicenter, case series. PARTICIPANTS AND INTERVENTION: Twenty-five patients with traumatic macular hole underwent surgical repair. INTERVENTION: Vitrectomy with membrane peeling and gas injection followed by prone positioning for 7 to 14 days. MAIN OUTCOME MEASURES: Postoperative evaluation included visual acuity testing, closure of the macular hole, and ocular complications. RESULTS: The macular hole was successfully closed in 24 of 25 cases (96%). The visual acuity improved two or more lines in 21 (84%) cases, and 16 (64%) achieved 20/50 or better vision. CONCLUSIONS: Vitrectomy surgery can successfully close macular holes associated with trauma and improve vision.

Evaluation of chemoprophylaxis in patients with unilateral retinoblastoma with high-risk features on histopathologic examination.

OBJECTIVES: To identify risk factors for metastatic disease on histopathologic specimens of enucleated eyes from patients with unilateral retinoblastoma, and to evaluate the value of chemoprophylaxis in preventing disease dissemination. METHODS: Medical records from patients with unilateral retinoblastoma who underwent primary enucleation were reviewed at the University of California, San Francisco (1977-1998) and Bascom Palmer Eye Institute, University of Miami, Miami, Fla (1991-1998). All routine histopathologic specimens were reexamined. The extent of tumor invasion into the optic nerve or ocular coats and the prescribed chemoprophylactic regimen were recorded. RESULTS: This retrospective study included 129 patients followed for a median of 54 months. Three patients had tumor invading the sclera. The optic nerve was involved to some extent in 82 patients, 11 of whom had tumor extension beyond the lamina cribrosa. The surgical margin of the optic nerve was involved in an additional 4 patients. The choroid was involved in 43 patients, and was considered massively affected in 12 patients. Anterior segment involvement was observed in 10 patients. Postenucleation chemoprophylaxis was administered to 4 of 4 patients who had tumor cells at the surgical margin of the optic nerve and to 7 of 11 patients with postlaminar disease, all of whom had at least 1 mm of postlaminar tumor extension. External beam radiotherapy was administered to 3/4 and 1/11 of these patients, respectively. Chemoprophylaxis was not administered to patients with choroidal or anterior chamber involvement unless the optic nerve was also involved beyond the lamina cribrosa. One patient with tumor extending to the surgical margin of the optic nerve died of metastatic disease. CONCLUSIONS: Chemoprophylaxis is necessary for patients with tumor extending to the surgical margin of the optic nerve and is likely to be beneficial in preventing metastases in patients with tumor extending beyond the lamina cribrosa. We did not offer chemoprophylaxis to patients with prelaminar optic nerve disease or isolated choroidal involvement, and these patients remained free of disseminated disease.

Outcomes of vitreoretinal surgery for complications of branch retinal vein occlusion.

OBJECTIVE: To investigate outcomes of vitreoretinal surgery for complications of branch retinal vein occlusion (BRVO). DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: The medical records of all patients who underwent vitreoretinal surgery for complications of BRVO at Bascom Palmer Eye Institute between January 1, 1991 and December 31, 1998 were reviewed. Thirty-six eyes from 36 consecutive patients were identified. MAIN OUTCOME MEASURES: Visual acuity outcomes include preservation of preoperative visual acuity and visual acuity greater than or equal to 20/40, 20/200 and 5/200. When preoperative retinal detachment was present, the anatomic outcome assessed was complete retinal attachment. Postoperative event rates of retinal detachment, vitreous hemorrhage, epiretinal membrane (ERM), and cataract were tabulated. All outcomes were assessed at 6 months. RESULTS: Surgical indications included nonclearing vitreous hemorrhage (17 patients), traction retinal detachment involving the macula (15), and ERM (4). Mean follow-up was 19 months. Preoperatively, best-corrected vision was greater than or equal to 20/200 in 19/36 (53%) eyes. Six months postoperatively, best-corrected vision was greater than or equal to 20/40 in 12/36 (33%) eyes, greater than or equal to 20/200 in 27/36 (75%) eyes, and greater than or equal to 5/200 in 31/36 (86%) eyes. Postoperative complications included retinal detachment (2/36; 6% eyes), ERM (3; 8%), vitreous hemorrhage (2; 6%), suprachoroidal hemorrhage (1; 3%), central retinal vein occlusion (1; 3%), and central retinal artery occlusion (1; 3%). Clinical features associated with better visual outcome include better preoperative visual acuity (P: = 0.05), absence of preoperative afferent pupillary defect (P: = 0.01), and absence of preoperative macular edema (P: = 0.08). CONCLUSIONS: Following surgery, retinal attachment and improved visual acuity were achieved in the majority of patients. Pre-existing pathology and postoperative complications may limit final vision in eyes with BRVO.

Subconjunctival carboplatin in retinoblastoma: impact of tumor burden and dose schedule.

OBJECTIVE: To evaluate the impact of tumor burden and chemotherapy dose scheduling on the response to subconjunctival carboplatin treatment in a murine transgenic retinoblastoma model. METHODS: Eighty simian virus 40 T antigen-positive mice were treated at age 5 or 10 weeks. Six control animals received placebo treatment. Twenty-four 5-week-old mice received 6 subconjunctival carboplatin injections at doses of 30 to 300 microg delivered at 72-hour intervals. Fifty 10-week-old mice received either 6 or 12 subconjunctival carboplatin injections at doses of 30 to 300 microg delivered at 72-hour intervals. All eyes were obtained at age 16 weeks for histopathologic examination. Eyes were graded as positive if any tumor was present. RESULTS: All simian virus 40 T antigen-positive control eyes contained large tumor foci throughout the retina. Subconjunctival carboplatin injections controlled tumors in a dose-dependent manner. Tumor control was observed in 50% of treated eyes at 138.3 microg for the 10-week-old 6-injection group, 94.3 microg for the 5-week-old 6-injection group, and 85.9 microg for the 10-week-old 12-injection group. CONCLUSION: Increased tumor burden requires an increase in subconjunctival carboplatin dose scheduling to maintain local tumor control in this murine model of retinoblastoma. CLINICAL RELEVANCE: This study documents the efficacy of subconjunctival carboplatin in the treatment of an animal model of retinoblastoma. These data establish a framework for further human clinical trials. Arch Ophthalmol. 2000;118:1549-1554

Bilateral diffuse choroidal hemangiomas with unilateral facial nevus flammeus in Sturge-Weber syndrome.

PURPOSE: To report bilateral choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge-Weber syndrome. METHODS: Case report. RESULTS: A 6-year-old male with a left facial nevus flammeus and a history of a left cerebral angioma had been followed 2 years for increasing esotropia. Examination demonstrated bilateral diffuse choroidal hemangiomas with overlying exudative retinal detachments. After bilateral external beam radiotherapy, the retinal detachments resolved and vision improved. CONCLUSION: Patients with Sturge-Weber syndrome and unilateral facial nevus flammeus may harbor bilateral choroidal hemangiomas. Clinical manifestations of the Sturge-Weber syndrome are characteristically unilateral and ipsilateral to the facial nevus flammeus. Bilateral choroidal hemangiomas associated with bilateral facial nevus flammeus are rare. 1,2 We report bilateral diffuse choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge-Weber syndrome.

Echographic features of medulloepithelioma.

PURPOSE: To evaluate the echographic features of medulloepithelioma that may assist in establishing the diagnosis. METHODS: Retrospective review identified four eyes with medulloepithelioma studied with echography. Clinical records, echographic findings, histopathologic tumor features, and the clinical course were reviewed. RESULTS: The initial preoperative diagnosis of medulloepithelioma was uncertain, based on clinical findings alone in three cases but was accurate when echographic findings were combined with clinical findings in all four cases. Cysts posterior to the iris were detected on clinical examination in only two cases, but were revealed on echography in all four cases. Additional echographic findings included irregular high internal reflectivity (n = 4), irregular tumor surface (n = 3), molding around intraocular structures (n = 3), and internal vascularity (n = 2). CONCLUSIONS: The diagnosis of medulloepithelioma is not always apparent on clinical examination alone. Echographic findings of a highly reflective, irregularly structured tumor with associated cystic changes involving the ciliary body region may help establish a presumed diagnosis of medulloepithelioma.

Design of a magnetically integrated microporous implant.

OBJECTIVE: To determine the orbital tolerance of a microporous implant fitted with an integrated stainless steel post and the enhanced motility associated with magnetic coupling of the prosthetic and the implant in a rabbit model. METHODS: Six New Zealand white rabbits underwent primary enucleation with implantation of a 12-mm microporous polyethylene implant with a 2 x 3-mm stainless steel post embedded flush with the anterior surface. At 1 month, the rabbits were fitted with an external prosthesis containing two 1-mm circular rare earth dental magnets embedded at 0. 5 mm off the midline (right and left of center at the horizon). Magnetic coupling forces were determined with a hanging block technique. RESULTS: No evidence of toxicity was observed in association with this integrated ocular implant. Magnetic coupling forces were noted maximally at 0.47 N. Clinical grading of motility documented enhancement in lateral excursion when compared with nonintegrated controls. CONCLUSION: Magnetically integrated microporous implants achieve excellent enhancement of motility without evidence of complications in this rabbit model. CLINICAL RELEVANCE: This study establishes a framework for the clinical evaluation of a magnetically integrated implant that may enhance prosthetic motility without requiring direct mechanical coupling of the implant to the prosthesis. Arch Ophthalmol. 2000;118:1259-1262

Rhegmatogenous retinal detachment in retinoblastoma patients undergoing chemoreduction and cryotherapy.

PURPOSE: To report the development of rhegmatogenous retinal detachment, with the retinal break adjacent to a cryotherapy scar, in three patients undergoing systemic chemotherapy for intraocular retinoblastoma. METHOD: Case series. RESULTS: Three patients with intraocular retinoblastoma were referred after poor response to systemic chemotherapy and local cryotherapy; three eyes of these three patients were noted to have rhegmatogenous retinal detachment and active retinoblastoma, with retinal breaks adjacent to cryotherapy scars. CONCLUSION: Rhegmatogenous retinal detachment may result from retinal necrosis associated with cryotherapy in the setting of intraocular retinoblastoma treated with systemic chemotherapy and local cryotherapy.

Development and characterization of a rodent model of methanol-induced retinal and optic nerve toxicity.

Methanol is an important public health and environmental concern because of the selective actions of its neurotoxic metabolite, formic acid, on the retina, optic nerve and central nervous system. Humans and non-human primates are uniquely sensitive to methanol-induced neurotoxicity as a consequence of the limited capacity of primate species to oxidize and thus detoxify formic acid. The toxic syndrome in primates is characterized by formic acidemia, metabolic acidosis and blindness or serious visual impairment. Nonprimate species are normally resistant to the accumulation of formate and associated metabolic and visual toxicity. We have characterized retinal and optic nerve toxicity in a nonprimate model of methanol toxicity using rats in which folate-dependent formate oxidation has been selectively inhibited, allowing formate to accumulate to toxic concentrations following methanol administration. Methanol-intoxicated rats developed formic acidemia, metabolic acidosis and visual toxicity analogous to the human methanol poisoning syndrome. Visual dysfunction was manifested as reductions in the electroretinogram and the flash-evoked cortical potential which occurred coincident with blood formate accumulation. Histological studies revealed mitochondrial disruption and vacuolation in the retinal pigment epithelium, photoreceptor inner segments and optic nerve. The temporal relationship between methanol administration and the onset and development of ocular toxicity, as well as, the degree of metabolic acidosis and extent of formic acidemia in this rodent model are remarkably similar to that documented in human methanol intoxication. Moreover, the functional and morphologic findings in methanol-intoxicated rats are consistent with the hypothesis that formate acts as a mitochondrial toxin in the retina and optic nerve. The establishment and characterization of this nonprimate animal model of methanol intoxication will facilitate research into the mechanistic aspects of methanol toxicity and the development and testing of treatments for human methanol poisoning.

Effect of scleral shortening on axial length.

BACKGROUND: Partial thickness sclerectomy is the most commonly employed scleral shortening technique used in conjunction with pars plana vitrectomy in the repair of myopia-associated macular holes in patients with staphyloma. Recently, scleral shortening induced through scleral invagination has been advocated as an adjunct in retinal translocation surgery. OBJECTIVE: To determine whether a correlation exists between the amount of sclera infolding and the posttreatment reduction in axial length (AL) as a result of lamellar scleral resectioning or full-thickness scleral invagination. METHODS: Three groups of 10 eyes each underwent lamellar scleral resection with dissection of 6, 8, and 10 mm in height, and 1 group of 10 eyes underwent a 10-mm invagination. Presurgical and postsurgical external AL of globes with stabilized intraocular pressure was measured to +/-0.022-mm precision. RESULTS: Average (+/-SD) AL shortening following lamellar resections for 6-, 8-, and 10-mm groups were 1.50+/-0.24, 2.10+/-0.13, and 2.65+/-0.24 mm, respectively, and 2.50+/-0.23 mm for the 10-mm invagination group. Differences in AL before and after scleral shortening were found to be significantly different between dissections of different heights (P<.05), and not significantly different between the 10-mm resection and invagination groups (P>.17). The AL of each group was shortened by approximately 25% of the resection-invagination height. The relation was quasilinear. CONCLUSIONS: Lamellar scleral resection and nonresected scleral invagination reduce the ocular AL. The extent of the reduction significantly correlates to the amount of removed or invaginated sclera. CLINICAL RELEVANCE: Surgical shortening of the sclera is useful in the management of several retinal disorders, but causes significant changes in AL. Arch Ophthalmol. 2000;118:965-968

Combined cataract surgery and vitrectomy for recurrent retinal detachment.

PURPOSE: To report our experience with combined cataract surgery, posterior chamber intraocular lens implantation, and pars plana vitrectomy in the management of recurrent retinal detachment (RD) and visually significant cataract. METHODS: Retrospective chart review of patients with cataract and recurrent RD who underwent combined cataract extraction, posterior chamber intraocular lens implantation, and pars plana vitrectomy between January 1991 and September 1998 at the Bascom Palmer Eye Institute. Sixteen eyes were included. All eyes had visually significant cataract and had undergone primary repair of the RD with encircling scleral buckle; eight eyes also had undergone pars plana vitrectomy during the primary repair. The technique of cataract extraction included phacoemulsification (10 eyes), extracapsular cataract extraction (5 eyes), and pars plana lensectomy (1 eye). All eyes underwent pars plana vitrectomy, membrane peeling, fluid-air exchange, endolaser treatment, and placement of a retinal tamponade. Perfluoropropane (C3F8) gas was used in 14 eyes, and silicone oil was placed in two eyes. RESULTS: The postoperative follow-up interval ranged from 4 to 64 months (mean, 16 months). Preoperative visual acuity ranged from 20/60 to hand motions and was better than 20/200 in 3 (19%) eyes. Postoperatively, 9 (56%) eyes improved to better than 20/200. Anatomic success was achieved after the initial reoperation in 13 (81 %) eyes. With further surgery, the overall success rate was 94%. CONCLUSIONS: Combined cataract surgery, posterior chamber intraocular lens implantation, and pars plana vitrectomy in selected patients with cataract and recurrent RD was successful in improving visual acuity and achieving retinal reattachment in most of these reoperated patients.

Visual acuity outcomes with and without surgery in patients with persistent fetal vasculature.

PURPOSE: To investigate visual acuity outcomes in patients with persistent fetal vasculature (PFV) left untreated or treated with vitreoretinal surgical techniques and to investigate clinical features associated with prognosis. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: All patients with PFV examined at the Bascom Palmer Eye Institute from January 1, 1983 through December 31, 1998. INTERVENTION: All patients in the study had unilateral PFV. Of 42 PFV patients identified, 30 patients underwent vitreoretinal surgery. Indications for surgery included media opacity (e.g., cataract), vitreoretinal traction, and retinal detachment. MAIN OUTCOME MEASURES: Final best postoperative visual acuity, prognostic ocular clinical features, and surgical complications. RESULTS: In the surgical group of patients, median age at diagnosis was 8 weeks, and median length of follow-up was 32 months, with all patients having at least 1 year of follow-up. Two patients had clinical and echographic findings consistent with anterior PFV, 2 patients had strictly posterior PFV, and the remaining 26 patients had components of both anterior and posterior PFV. Fourteen eyes (47%) achieved a final visual acuity of 20/400 or better at last follow-up. Risk factors for a poor visual acuity outcome (<20/400) included microphthalmia (28% of patients with microphthalmia versus 67% of patients with normal axial length achieved a final vision of 20/400 or better; P = 0.061) and preoperative retinal detachment or retinal or optic nerve abnormalities, or both, such as hypoplasia, folds, or indistinct macula with hypopigmentation (25% of patients with any of these anomalies versus 61 % of patients without these findings achieved a final vision of 20/400 or better; P = 0.072). After surgery, retinal detachment developed in three eyes, chronic hypotony in two other eyes, and neovascular glaucoma in one eye. In the nonsurgical group there were 6 male and 6 female patients. Two patients with posterior PFV had minimal disease and were not considered surgical candidates, whereas 10 patients with combined anterior and posterior PFV had advanced pathologic features, and it was believed that surgery would not offer significant visual improvement; median age at diagnosis was 9.5 months, and median length of follow-up was 36 months, with all patients having at least 1 year of follow-up. At last follow-up, 3 eyes (25%) had a final visual acuity of 20/400 or better. During follow-up, retinal detachment developed in 2 eyes and chronic hypotony in an additional 2 eyes. CONCLUSIONS: The current study indicates that approximately 50% of patients undergoing surgery for PFV will achieve useful vision. Visual acuity outcomes in patients with PFV are correlated with the nature and extent of ocular risk factors. Some patients may not be candidates for surgery because of either minimal changes or advanced disease that limit the potential of visual improvement.