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Anterior segment dysgenesis (ASD) encompasses a wide spectrum of developmental abnormalities of the anterior ocular segment, including congenital cataract, iris hypoplasia, aniridia, iridocorneal synechiae, as well as Peters, Axenfeld, and Rieger anomalies. Here, we report a large five-generation Caucasian family exhibiting atypical syndromic ASD segregating with a novel truncating variant of FOXC1. The family history is consistent with highly variable autosomal dominant symptoms including isolated glaucoma, iris hypoplasia, aniridia, cataract, hypothyroidism, congenital heart anomalies, and cystic kidney disease. Whole exome sequencing revealed a novel variant [c.313_314insA; p.(Tyr105*)] in FOXC1 that disrupts the alpha-helical region of the DNA-binding forkhead box domain. In vitro studies using a heterologous cell system revealed aberrant cytoplasmic localization of FOXC1 harboring the Tyr105* variant, likely precluding downstream transcription function. Meta-analysis of the literature highlighted the intrafamilial variability related to FOXC1 truncating alleles. This study highlights the clinical variability in ASD and signifies the importance of combining both clinical and molecular analysis approaches to establish a complete diagnosis.

Anterior segment dysgenesis (ASD) encompasses a wide spectrum of developmental abnormalities of the anterior ocular segment, including congenital cataract, iris hypoplasia, aniridia, iridocorneal synechiae, as well as Peters, Axenfeld, and Rieger anomalies. Here, we report a large five-generation Caucasian family exhibiting atypical syndromic ASD segregating with a novel truncating variant of FOXC1. The family history is consistent with highly variable autosomal dominant symptoms including isolated glaucoma, iris hypoplasia, aniridia, cataract, hypothyroidism, and congenital heart anomalies. Whole-exome sequencing revealed a novel variant [c.313_314insA; p.(Tyr105*)] in FOXC1 that disrupts the α-helical region of the DNA-binding forkhead box domain. In vitro studies using a heterologous cell system revealed aberrant cytoplasmic localization of FOXC1 harboring the Tyr105* variant, likely precluding downstream transcription function. Meta-analysis of the literature highlighted the intrafamilial variability related to FOXC1 truncating alleles. This study highlights the clinical variability in ASD and signifies the importance of combining both clinical and molecular analysis approaches to establish a complete diagnosis.

Importance Intravitreous bevacizumab (0.25 mg to 0.625 mg) is commonly used to treat type 1 retinopathy of prematurity (ROP), but there are concerns about systemic toxicity, particularly the risk of neurodevelopmental delay. A much lower dose may be effective for ROP while reducing systemic risk. Previously, after testing doses of 0.25 mg to 0.031 mg, doses as low as 0.031 mg were found to be effective in small cohorts of infants. Objective To find the lowest dose of intravitreous bevacizumab effective for severe ROP. Design, Setting, and Participants Between April 2017 and May 2019, 59 premature infants with type 1 ROP in 1 or both eyes were enrolled in a masked, multicenter, dose de-escalation study. In cohorts of 10 to 14 infants, 1 eye per infant received 0.016 mg, 0.008 mg, 0.004 mg, or 0.002 mg of intravitreous bevacizumab. Diluted bevacizumab was prepared by individual research pharmacies and delivered using 300-µL syringes with 5/16-inch, 30-guage fixed needles. Analysis began July 2019. Interventions Bevacizumab intravitreous injections at 0.016 mg, 0.008 mg, 0.004 mg, or 0.002 mg. Main Outcomes and Measures Success was defined as improvement by 4 days postinjection and no recurrence of type 1 ROP or severe neovascularization requiring additional treatment within 4 weeks. Results Fifty-five of 59 enrolled infants had 4-week outcomes completed; the mean (SD) birth weight was 664 (258) g, and the mean (SD) gestational age was 24.8 (1.6) weeks. A successful 4-week outcome was achieved for 13 of 13 eyes (100%) receiving 0.016 mg, 9 of 9 eyes (100%) receiving 0.008 mg, 9 of 10 eyes (90%) receiving 0.004 mg, but only 17 of 23 eyes (74%) receiving 0.002 mg. Conclusions and Relevance These data suggest that 0.004 mg may be the lowest dose of bevacizumab effective for ROP. Further investigation is warranted to confirm effectiveness of very low-dose intravitreous bevacizumab and its effect on plasma vascular endothelial growth factor levels and peripheral retinal vascularization.

Purpose To evaluate the development of cataracts or elevated intraocular pressure (IOP) in patients with Duchenne muscular dystrophy (DMD) on long-term glucocorticoid (GC) treatment. Methods The medical records of DMD patients evaluated from 2010 to 2015 at a single center were reviewed retrospectively. The main outcome measures were prevalence of cataracts and elevated IOP, age of first detection of cataract, time from initial steroid use to first detection of cataract, and relative risk of cataract development for deflazacort versus prednisone treatment. Results Of 596 DMD patients, 514 underwent GC therapy; all but one was male. The racial distribution was 82.1% white, 1.0% African American, 5.0% Hispanic, 2.9% Asian, and 8.0% more than one race or “other.” The prevalence of cataracts was 22.4% in patients on GC therapy. The mean age at which cataract formation was first documented was 12.9 ± 4.1 years (IQR, 9.6-14.6). The mean time from initial steroid use to the first detection of cataract was 6.5 ± 3.6 years (IQR, 4.0-8.6). The odds of cataract development were 2.4-fold higher for patients on deflazacort compared with prednisone (95% CI, 1.3-4.5; P = 0.004). Only 7 patients (1.4%) underwent cataract surgery, at a mean age of 16.9 years (range, 10.7-24.6 years); all were on deflazacort. Among patients with available intraocular pressure measurements, elevated IOP occurred in only 1 patient (1.1%), who was on deflazacort. Conclusions In patients undergoing GC therapy for DMD, the rate of cataract formation was slow and well tolerated, with a higher risk among deflazacort patients. The percentage of patients requiring cataract extraction or with elevated IOP was very small. These findings suggest that a schedule of annual eye examinations is appropriate.

Importance The first Postnatal Growth and Retinopathy of Prematurity Study (G-ROP-1) developed new screening criteria with 100% sensitivity for type 1 retinopathy of prematurity (ROP) and 30% reduction of infants requiring examinations in a retrospective development cohort of 7483 infants from 29 North American hospitals in 2006-2012. Infants meeting 1 or more of the following criteria undergo examinations: gestational age less than 28 weeks or birth weight less than 1051 g; weight gain less than 120 g during age 10 to 19 days, weight gain less than 180 g during age 20 to 29 days, or weight gain less than 170 g during age 30 to 39 days; or hydrocephalus. Objective To evaluate the generalizability of the G-ROP screening criteria in a new cohort of at-risk infants. Design, Setting, and Participants This prospective validation cohort study (G-ROP-2) was conducted at 41 hospitals in the United States and Canada (25 G-ROP-1 hospitals and 16 new hospitals) from September 8, 2015, to June 13, 2017, among 3981 premature infants at risk for ROP and with known ROP outcomes. Main Outcomes and Measures Sensitivity for Early Treatment for Retinopathy of Prematurity Study type 1 ROP and potential reduction in infants receiving examinations. Results Among the 3981 infants in the study (1878 girls and 2103 boys; median gestational age, 28 weeks [range, 22-35 weeks]; median birth weight, 1072 g [range, 350-4080 g]; 1966 white; 942 black; 321 Latino; 120 Asian; 22 Native Hawaian or Pacific Islander; and 25 American Indian or Alaskan Native), the G-ROP criteria correctly predicted 219 of 219 cases of type 1 ROP (sensitivity, 100%; 95% CI, 98.3%-100%), while reducing the number of infants undergoing examinations by 35.6% (n = 1418). In a combined G-ROP-1 and G-ROP-2 cohort of 11 463 infants, the G-ROP criteria predicted 677 of 677 cases of type 1 ROP (sensitivity, 100%; 95% CI, 99.4%-100%), reducing the number of infants receiving examinations by 32.5% (n = 3730), while current criteria (birth weight Conclusions and Relevance This study found that the G-ROP screening criteria were generalizable on validation and, if used clinically in the United States and Canada, could reduce the number of infants receiving examinations. The large G-ROP cohorts provide evidence-based screening criteria that have higher sensitivity and higher specificity (fewer infants receiving examinations) for type 1 ROP than currently recommended guidelines.

Purpose To determine the prevalence and characteristics of eyes treated for retinopathy of prematurity (ROP) not meeting currently recommended early treatment (type 1) criteria. Methods This was a secondary analysis of data from the Postnatal Growth and ROP (G-ROP) Study, a retrospective cohort study of 7,483 infants undergoing ROP examinations and treatment at 29 North American hospitals between January 2006 and June 2012. Medical records were reviewed to determine the prevalence and characteristics of eyes treated for ROP less severe than type 1 ROP. Results Of 1,004 eyes that received ROP treatment, 126 eyes of 91 infants (0.8% of all eyes; 12.5% of treated eyes) underwent treatment for ROP less severe than type 1. Mean age at treatment was 38 weeks’ post-menstrual age (range, 32-49 weeks). Reasons for treatment included type 1 ROP in the fellow eye (43%), stage 3 ROP with pre-plus in the treated eye (30%), concerning structural changes in the retina (7%), persistent stage 3 ROP for ≥6 weeks without regression (6%), stage 3 ROP with no plus disease in the treated eye (5%), stage 3, zone III ROP with plus disease (3%), logistical considerations (3%), or stage 2 disease in the treated eye (2%). Conclusions Of all eyes treated for ROP, 1/8 were treated for disease less severe than currently recommended type 1 criteria. Clinician judgment of risk for permanent vision impairment superseded recommended treatment criteria and was usually related to type 1 disease in the fellow eye or pre-plus vascular changes in one or both eyes.

Importance Intravitreous bevacizumab (0.25 to 0.625 mg) is increasingly used to treat type 1 retinopathy of prematurity (ROP), but there remain concerns about systemic toxicity. A much lower dose may be effective while reducing systemic risk. Objective To find a dose of intravitreous bevacizumab that was lower than previously used for severe ROP, was effective in this study, and could be tested in future larger studies. Design, Setting, and Participants Between May 2015 and September 2016, 61 premature infants with type 1 ROP in 1 or both eyes were enrolled in a masked, multicenter, phase 1 dose de-escalation study. One eye of 10 to 14 infants received 0.25 mg of intravitreous bevacizumab. If successful, the dose was reduced for the next group of infants (to 0.125 mg, then 0.063 mg, and finally 0.031 mg). Diluted bevacizumab was delivered using 300 µL syringes with 5/16-inch, 30-gauge fixed needles. Interventions Bevacizumab injections at 0.25 mg, 0.125 mg, 0.063 mg, and 0.031 mg. Main Outcomes and Measures Success was defined as improvement in preinjection plus disease or zone I stage 3 ROP by 5 days after injection or sooner, and no recurrence of type 1 ROP or severe neovascularization requiring additional treatment within 4 weeks. Results Fifty-eight of 61 enrolled infants had 4-week outcomes completed; mean birth weight was 709 g and mean gestational age was 24.9 weeks. Success was achieved in 11 of 11 eyes at 0.25 mg, 14 of 14 eyes at 0.125 mg, 21 of 24 eyes at 0.063 mg, and 9 of 9 eyes at 0.031 mg. Conclusions and Relevance A dose of bevacizumab as low as 0.031 mg was effective in 9 of 9 eyes in this phase 1 study and warrants further investigation. Identifying a lower effective dose of bevacizumab may reduce the risk for neurodevelopmental disability or detrimental effects on other organs.

Our previous study demonstrated that topical ocular instillation can deliver effective concentrations of propranolol in the periocular tissues, and may be superior to oral propranolol in the treatment of periocular capillary hemangiomas. The objective of this study was to investigate the effects of dosing protocol on the distribution of propranolol in the periocular tissues and plasma after topical ocular instillation.Each rabbit received propranolol 0.5% ophthalmic solution using one of the following dosing protocols: three drops of 50 μL, one drop of 50 μL, or one drop of 25 μL. The periocular tissues (e.g. eyelids and extraocular muscles) and blood were collected and assayed for propranolol at 1 h after dosing.Decreasing the concentration of eye drops (1% to 0.5%), decreasing the number of eye drops during dosing (three drops to one drop), or decreasing the instilled volume (50 µL to 25 µL) generally lowered the concentration of propranolol in the periocular tissues. Nevertheless, therapeutic levels of propranolol ( 0.4 μg/g) were delivered to the periocular tissues at 1 h after dosing using any of the three protocols examined. Dose-dependent concentrations in some periocular tissues and plasma were observed over the dose range of 0.125 mg to 1.5 mg. The plasma concentration of propranolol was not measurable when a single 25 μL of propranolol 0.5% was instilled in the eye.The dosing protocol of topical ocular instillation can be tailored to achieve the desired therapeutic concentrations of propranolol in the periocular tissues while minimizing systemic exposure.

Purpose Fetal mice require light exposure in utero during early gestation for normal vascular development in the eye. Because angiogenic abnormalities in retinopathy of prematurity (ROP) are manifested in preterm infants, we investigated whether day length during early gestation was associated with severe ROP (SROP). Design Single-center, retrospective cohort study. Participants We included a total of 343 premature infants (401-1250 g birth weight [BW], from 1998-2002): 684 eyes (1 eye each of 2 patients excluded) with 76 eyes developing SROP, defined as (1) classic threshold ROP in zone I or II, (2) type 1 ROP in zone I, or (3) in a few eyes, type 1 ROP in posterior zone II that was treated. Methods For each infant, average day length (ADL) was calculated during different cumulative time periods and time windows after the estimated date of conception (EDC). Multiple logistic regression analysis (with generalized estimating equations to account for inter-eye correlation) was performed. Main outcome measures Association of ADL during early gestation with SROP. Results In a model evaluating all 684 eyes with 76 eyes developing SROP, BW, gestational age, multiple births, race, per capita income in the mother's residence ZIP code, and ADL during the first 90 days after the EDC were factors associated with the development of SROP. Each additional hour of ADL (90 days) decreased the likelihood of SROP by 28% (P = 0.015; odds ratio [OR], 0.72; 95% confidence interval [CI], 0.55-0.94). In a model evaluating the subset of 146 prethreshold ROP eyes with 76 eyes developing SROP, each additional hour of ADL during the first 105 days after the EDC decreased the likelihood of SROP by 46% (P = 0.001; OR, 0.54; 95% CI, 0.37-0.78). Time windows when ADL was most closely associated with SROP were 31 to 60 days and 61 to 90 days after the EDC for the all eyes and the prethreshold ROP eyes models, respectively. Conclusions Higher ADL during early gestation was associated with a lower risk for SROP and may imply a role for prophylactic light treatment during early gestation to decrease the risk of SROP.

Objective To evaluate the severity of postoperative inflammation, degree of patient discomfort, adequacy of wound closure, and length of operating time when using fibrin glue compared with sutures to close limbal conjunctival incisions after strabismus surgery. Methods Literature searches of the PubMed and Cochrane Library databases were last conducted on January 24, 2013, and resulted in 24 citations, including 2 not in the English language. All citations were reviewed in full text. Five studies compared fibrin glue (68 eyes) with sutures (74 eyes) for closure of limbal conjunctival incisions in patients undergoing strabismus surgery and were included in this assessment; no studies were found that evaluated fornix incisions. A quality rating was assigned to each study using criteria specifically developed for this assessment. Results No level I studies were found, and 5 level II studies were identified. There was significantly less postoperative inflammation and patient discomfort for 1 to 3 weeks after strabismus surgery for eyes treated with fibrin glue compared with sutures. In 3 studies that evaluated wound apposition, 2 of 50 eyes (4%) with conjunctival incisions that were initially closed using fibrin glue subsequently developed a wound gap that required suture repair. In the 2 studies that compared surgical time, fibrin glue required 1 to 5 minutes less time than suturing in 1 study and 55% less time (3.8 vs. 8.4 minutes) in a second study. These 5 studies did not evaluate the cost-effectiveness or risk of viral transmission from fibrin glue. Conclusions Studies in the literature suggest that the off-label use of fibrin glue to close limbal conjunctival incisions in strabismus surgery resulted in less postoperative inflammation and required shorter operating time compared with sutures, but it increased the percentage of wounds requiring subsequent repair with sutures. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Objective To compare intraocular pressure (IOP) measurements in children 18 years of age and younger using rebound tonometry and applanation tonometry and the feasibility of using these techniques in children. Methods Literature searches of the PubMed and the Cochrane Library databases were last conducted in June 2012 and resulted in 43 citations, including citations not in English. Of these 43 citations, 4 studies met the inclusion criterion following full text review. A level of evidence rating was assigned to each study using criteria specifically developed for this assessment. Results No level I study was found in the literature search, and 2 level II and 2 level III studies were identified. Intraocular pressure was 2 to 3 mmHg higher using rebound tonometry compared with Goldmann applanation tonometry in the 2 level II studies performed in a clinic setting and in 1 level III study performed on children under general anesthesia. However, IOP was lower in 1 level III study in which noncontact applanation tonometry was compared with rebound tonometry. Bland-Altman plots showed that the difference in IOP for rebound versus Goldmann applanation tonometry increased as the IOP increased. The success rate for measuring IOP was markedly higher in children 3 years of age and younger using rebound tonometry compared with noncontact tonometry in 1 level III study. Conclusions Rebound tonometry seems to be a reasonably accurate instrument that allows the IOP to be measured in many children without using general anesthesia. More data are required to assess better how the differences between instruments vary with IOP measurement. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Retinopathy of prematurity (ROP) is a proliferative disorder involving the developing vasculature of premature infants that can result in retinal detachment and blindness. Timely screening for ROP and prompt treatment of severe ROP can decrease the prevalence of unfavorable retinal structural and poor visual outcome. The criteria for treatment of ROP has evolved from classic threshold ROP, as defined in the Multicenter Trial of Cryotherapy for ROP, to type 1 prethreshold ROP, as defined in the Early Treatment of ROP randomized trial, with incremental improvement in the overall treatment outcome. The mode of treatment has also transitioned from the use of cryotherapy to laser photocoagulation. The mouse model of oxygen-induced retinopathy has elucidated the pathogenesis of ROP and led to new interventions and screening approaches. Pharmacotherapy using the intravitreal injection of bevacizumab, an antibody to vascular endothelial growth factor, appears to be superior to laser photocoagulation for type 1 ROP in zone I but equivalent to laser therapy for type 1 ROP in zone II. However, the systemic risks associated with bevacizumab are uncertain. New screening algorithms based on the rate of postnatal growth of at risk infants are being developed in order to reduce the percentage of infants screened while maintaining efficacy. Digital fundus photography has a high degree of accuracy for the detection of treatment warranting ROP. Validation studies for the reliability and accuracy of telemedicine for ROP are under way.

Oral propranolol has become a promising treatment of capillary hemangiomas (CHs) despite concerns of side effects associated with systemic beta-blockers. The objective of this study was to investigate the distribution of propranolol in periocular tissues and in plasma after topical application of propranolol as compared with intravenous and oral administration of propranolol.Each rabbit received propranolol as ophthalmic solution (1%) in one eye (1.5 mg dose), intravenous injection (1.5 mg dose), or commercially available propranolol oral solution (5 mg dose). The periocular tissues (e.g., eyelids and extraocular muscles) and blood were collected and assayed for propranolol.After topical instillation of 1.5 mg propranolol, high amounts of propranolol were rapidly delivered to the eyelids and extraocular muscles (4-32 μg/g at 1 h after dosing). The drug in these tissues was slowly cleared, and significant amounts of the drug (0.4 μg/g) were still present at 24 h after the topical application. After oral administration of a clinically relevant dose of 5 mg propranolol, the drug concentrations in the periocular tissues were relatively low (0.4 μg/g) at 1 h after dosing and generally undetectable at 8 h after dosing. After an intravenous injection of 1.5 mg propranolol, the drug concentrations in the eyelids and extraocular muscles were in the range of 0.2-1 μg/g at 1 h after dosing. The plasma concentration of the drug after the intravenous injection was significantly higher than that after topical application of the same dose. The higher drug concentrations in the periocular tissues of the treated eyes as compared with untreated eyes suggest direct penetration of the drug into the periocular tissues from the administration site after topical application.Topical administration can provide increased concentrations of propranolol in the periocular tissues and, thus, is superior to systemic administration for the treatment of periocular CH.

Purpose To determine whether a multivariate risk model can select infants with low-risk eyes for an alternative protocol that reduces retinopathy of prematurity (ROP) screening without loss of effectiveness. Methods This was a retrospective, nonrandomized, comparative study. We assigned 712 eyes of 357 premature infants of 401–1,250 g birth weights as high or low risk for prethreshold or threshold ROP using a risk model with variables of birth weight, gestational age, multiple birth, race, and gender. Using simulations, infants with high-risk eyes ( p ≥ 0.15) were screened conventionally, while those with low-risk eyes were screened with the 35q3 protocol (initial examination at 35 weeks postmenstrual age followed by screening every 3 weeks, with increased or decreased intervals based on ROP severity). The resultant reduction in ROP screening and the delay to detection of severe ROP were calculated. Results The 35q3 protocol reduced the mean number of eye examinations per infant in the overall population by 13.4% ( p = 0.0051). No eyes had a delay to the detection of threshold or type 1 zone 1 ROP. Of a total of 57 type 1 ROP eyes, 2 eyes with type 1 zone 2 ROP were delayed to detection by 1 week. As our study was done prior to the Early Treatment of ROP study, the 2 eyes were observed and regressed without progression to threshold ROP. Conclusions A risk-based alternative screening protocol increased the efficiency of ROP screening for infants of 401–1,250 g birth weight without an apparent loss of effectiveness. This investigational approach requires further validation by multicenter studies.

Background: The aim of this study was to determine the concentrations of propranolol in periocular tissues and plasma after ocular instillation of 0.5% propranolol gel-forming solution (GFS) as compared to 0.5% propranolol non-gelforming solution (non-GFS) for potential use in the treatment of periocular capillary hemangiomas. Methods: A GFS prepared in 1% sodium alginate or a non-GFS in phosphatebuffered saline was instilled into the eyes of rabbits. At predetermined time intervals after dosing, blood was withdrawn, rabbits were euthanized, and periocular tissues were dissected. Results: Ocular instillation of the GFS resulted in higher concentrations of propranolol in the outer layers of both the upper and lower eyelids (in the range of 9.9-36.9 μg/g) and maintained higher levels of propranolol in these tissues for 24 h after dosing, as compared to the ocular instillation of the non-GFS (in the range of 3.4-15.1 μg/g). While the concentrations of propranolol in the other periocular tissues were generally similar for GFS and non-GFS at 1 h after dosing, the concentrations of propranolol in the extraocular muscles and periocular fat were higher for GFS than those for non-GFS between 4-24 h after dosing. Lower level of propranolol in plasma was observed at 1 h with GFS as compared with non-GFS. Conclusion: The use of the propranolol gel-forming solution can prolong drug retention on the ocular surface and increase its distribution to the outer layers of the eyelids while decreasing systemic exposure to the drug.

During retinal vascular development there is simultaneous regression of the hyaloid vasculature and formation of the retinal vasculature; here it is demonstrated that regression of developing vasculature is light dependent and acts via the photoreceptor melanopsin. In the developing eye, as the retinal vasculature forms the blood vessels supplying the transparent hyaloid membrane surrounding the vitreous body regress. This process is clinically important as excessive vascular growth is a major cause of blindness in premature infants. Surprisingly, this study reports that light is involved in this major change in tissue architecture. Richard Lang and colleagues find that light stimulates regression of developing hyaloid vasculature in mice, acting through the photoreceptor melanopsin. In the absence of either light or melanopsin, vascular endothelial growth factor A is upregulated and abnormal retinal angiogenesis results. Whether the same processes are involved in human eye development remains to be determined. Vascular patterning is critical for organ function. In the eye, there is simultaneous regression of embryonic hyaloid vasculature1 (important to clear the optical path) and formation of the retinal vasculature2 (important for the high metabolic demands of retinal neurons). These events occur postnatally in the mouse. Here we have identified a light-response pathway that regulates both processes. We show that when mice are mutated in the gene (Opn4) for the atypical opsin melanopsin3,4,5, or are dark-reared from late gestation, the hyaloid vessels are persistent at 8 days post-partum and the retinal vasculature overgrows. We provide evidence that these vascular anomalies are explained by a light-response pathway that suppresses retinal neuron number, limits hypoxia and, as a consequence, holds local expression of vascular endothelial growth factor (VEGFA) in check. We also show that the light response for this pathway occurs in late gestation at about embryonic day 16 and requires the photopigment in the fetus and not the mother. Measurements show that visceral cavity photon flux is probably sufficient to activate melanopsin-expressing retinal ganglion cells in the mouse fetus. These data thus show that light—the stimulus for function of the mature eye—is also critical in preparing the eye for vision by regulating retinal neuron number and initiating a series of events that ultimately pattern the ocular blood vessels.

Purpose: To assess anatomic outcomes following repair of traumatic retinal detachment (RD) among a cohort of cognitively impaired adolescents with self-injurious behaviors. Methods: Main outcome measures in this retrospective, consecutive, interventional case series were retinal attachment and postoperative complications. Results: Complete retinal reattachment was initially achieved in all 9 eyes of 9 patients. Six (67%) eyes had chronic RD at initial presentation, and 7 (78%) patients had limited vision from inoperable RD in the fellow eye. Proliferative vitreoretinopathy led to recurrent RD in 4 (44%) eyes at a mean of 2.7 months. Final total or partial retinal attachment was achieved in 6 (67%) and 3 (33%) eyes, respectively, with a mean of 1.7 procedures over a mean follow-up of 22.4 months. Silicone oil emulsification led to progressive glaucoma in 4 (50%) of 8 eyes in which it was used, requiring silicone oil exchange (2 eyes) or removal (2 eyes). Recurrent RD developed in both eyes from which silicone oil was removed despite prior retinal anatomic stability for more than 6 months. Conclusion: Cognitively impaired adolescents with self-injurious behaviors typically presented with severe visual impairment from chronic traumatic RD in one or both eyes. Despite initial anatomic success, formation of proliferative vitreoretinopathy and further ocular trauma from continued self-injurious behaviors led to a high rate of recurrent RD. Long-term silicone oil tamponade may prevent recurrent RD in this challenging scenario but is associated with vision loss from progressive glaucoma and corneal decompensation.

Purpose To report on a series of patients who developed orbital myositis following routine strabismus surgery. Methods A retrospective medical record review of patients who developed myositis following routine strabismus surgery. Surgery was performed on all patients between 1998 and 2003 by a single pediatric ophthalmologist (SMA). Results Four patients developed orbital myositis following routine strabismus surgery. All four cases were reoperations. Symptoms of myositis developed at a mean of 21 days after surgery (range, 4-34). In the initial patient, the diagnosis of myositis was based on clinical and computed tomography findings. The clinical course of the three subsequent patients was similar to that of the radiologically confirmed case. In all cases, the diagnosis was supported by rapid resolution after treatment with systemic corticosteroids. Conclusions Orbital myositis is a previously unreported complication of strabismus surgery. Reoperation appears to be a predisposing factor.

Purpose To characterize the results of vertical rectus muscle recession, or recession and resection surgery for vertical abnormal head posture (VAHP) due to null-point nystagmus. Method This is a retrospective, noncomparative, consecutive case series of patients who underwent vertical rectus muscle recession alone, recession then resection, or combined recession/resection surgery for VAHP due to null-point nystagmus. The primary outcome measure was the amount of residual VAHP. Results Twenty patients with VAHP due to null-point nystagmus were studied. The mean follow-up interval after surgery was 49 months (median: 44 months; range 9 to 124 months). Preoperative VAHP ranged from 10° to 45°, and the total amount of bilateral vertical rectus muscle recession, or recession and resection surgery, ranged from 8.5 to 20.0 mm per eye. After recession alone, 5/11 had residual VAHP ≤ 5°. The remaining six underwent subsequent resection or plication for residual VAHP > 10° that resulted in residual VAHP ≤ 5° in each case. After combined recession/resection, 6/9 had residual VAHP ≤ 5° and the remaining three had VAHP Conclusion Large amounts of vertical rectus muscle surgery are indicated to successfully correct the VAHP associated with nystagmus with a vertical null point. Combined recession/resection of the appropriate vertical rectus muscles, instead of recession alone, appears to be a more effective procedure.