Development of New Solitary Retinoblastoma Tumors during and after Chemotherapy

Purpose@#To review the occurrence of new solitary tumors during and after intravenous chemotherapy against retinoblastoma. @*Methods@#From 115 eyes of 78 patients with a diagnosis of intraocular retinoblastoma who underwent intravenous chemotherapy and focal treatment without prior treatment, patient demographics, age at diagnosis, laterality, classification (Reese-Ellsworth and International Classification of Retinoblastoma), and treatment options were recorded. In addition, the occurrence of small tumors during and after chemotherapy was documented with a detailed review of medical records and fundus photographs. @*Results@#Of a total of 115 eyes of 78 consecutive patients, new solitary tumors were observed in 50 eyes (50 / 115, 43%) of 40 patients (40 / 78, 51%). Multinominal logistic regression analyses showed that age at diagnosis (before 1 year) and vitreal seeding at diagnosis were linked to the development of isolated and miliary tumors, respectively. Kaplan-Meier analyses demonstrated that all small tumors developed with 20 months from the start of chemotherapy. Twenty-eight eyes (28 / 34, 82%) were salvaged with additional focal treatment in 34 eyes with isolated tumors. @*Conclusions@#Small tumors were observed during and after chemotherapy against retinoblastoma in patients who underwent intravenous chemotherapy and focal treatment. It is necessary to promptly identify and address small tumors for the preservation of eyeball and vision.

Factors Associated with the Prognosis after Operation in Children with Recurrent Intermittent Exotropia

BACKGROUND: To describe factors affecting the prognosis after operation for recurrent intermittent exotropia (X[T]) in children. METHODS: Clinical records of 50 patients who underwent operation for recurrent X(T) by a single surgeon were reviewed. The age at diagnosis of X(T), and first and second operations, deviation angle at distance and near, surgical method, concurrent vertical strabismus, stereoacuity, and Worth's Four Dot (W4D) examination before reoperation were analyzed, along with the postoperative deviation angle. A successful surgical outcome was defined as orthophoria, esodeviation ≤ 5 prism diopters, or exodeviation ≤ 10 prism diopters at distance. RESULTS: Among the 50 recurrent exotropes who underwent surgery and were followed up for more than 1 year postoperatively, 13 showed recurrent exotropia and 1 showed consecutive esotropia. The mean age at reoperation was 8.49 ± 2.19 years, and the mean duration of postoperative follow-up was 27.78 ± 12.02 months. Good near fusion before reoperation was a significant factor in the success of surgery (P = 0.006). Smaller postoperative deviation angle measured immediately and 2 months after surgery were related to smaller final deviation angle (P = 0.027 and P = 0.022, respectively). CONCLUSION: Peripheral suppression lowers the success rate of operation for recurrent X(T) in children. Overcorrection rather than orthotropia should be the target of immediate postoperative deviation angle. Peripheral suppression status and immediate and 2-month postoperative deviation angle may be important clues for predicting the final result of operation for recurrent X(T).

Fluorescein Angiographic Abnormalities in the Contralateral Eye with Normal Fundus in Children with Unilateral Coats' Disease

PURPOSE: We report the detection of peripheral retinal vascular abnormalities in the fellow eye with normal fundus in children with unilateral Coats' disease. METHODS: The clinical records of patients diagnosed with Coats' disease were retrospectively reviewed. We recorded the subjects' characteristics and obtained fundus photography and fluorescein angiography (FA) images. The main outcome measure was peripheral vascular abnormalities in the contralateral eye with normal fundus in children with unilateral Coats' disease, observed with FA. RESULTS: Out of 47 patients with Coats' disease, two (4.3%) were diagnosed with clinically bilateral Coats' disease. Of the 45 patients with presumed unilateral Coats' disease, four (8.9%) had bilateral abnormal peripheral vasculature in FA. The mean age of these four patients was 6.4 ± 5.4 years (range, 1 to 14 years), and three patients were male (75%). All four had peripheral retinal nonperfusion, and two (50%) received laser photocoagulation due to peripheral leakage with telangiectatic vessels. CONCLUSIONS: Coats' disease may more often be a bilateral disease with asymmetry than previously thought. Patients with Coats' disease should undergo careful examination of the fellow eye with FA in order to detect and treat vascular abnormalities that are not visible clinically.

Relationship between Pericytes and Endothelial Cells in Retinal Neovascularization: A Histological and Immunofluorescent Study of Retinal Angiogenesis

PURPOSE: To evaluate the relationship between pericytes and endothelial cells in retinal neovascularization through histological and immunofluorescent studies. METHODS: C57BL/6J mice were exposed to hyperoxia from postnatal day (P) 7 to P12 and were returned to room air at P12 to induce a model of oxygen-induced retinopathy (OIR). The cross sections of enucleated eyes were processed with hematoxylin and eosin. Immunofluorescent staining of pericytes, endothelial cells, and N-cadherin was performed. Microfluidic devices were fabricated out of polydimethylsiloxane using soft lithography and replica molding. Human retinal microvascular endothelial cells, human brain microvascular endothelial cells, human umbilical vein endothelial cells and human placenta pericyte were mixed and co-cultured. RESULTS: Unlike the three-layered vascular plexus found in retinal angiogenesis of a normal mouse, angiogenesis in the OIR model is identified by the neovascular tuft extending into the vitreous. Neovascular tufts and the three-layered vascular plexus were both covered with pericytes in the OIR model. In this pathologic vascularization, N-cadherin, known to be crucial intercellular adhesion molecule, was also present. Further evaluation using the microfluidic in vitro model, successfully developed a microvascular network of endothelial cells covered with pericytes, mimicking normal retinal angiogenesis within 6 days. CONCLUSIONS: Pericytes covering endothelial cells were observed not only in vasculature of normal retina but also pathologic neovascularization of OIR mouse at P17. Factors involved in the endothelial cell-pericyte interaction can be evaluated as an attractive novel treatment target. These future studies can be performed using microfluidic systems, which can shorten the study time and provide three-dimensional structural evaluation.

Effects on Periocular Tissues after Proton Beam Radiation Therapy for Intraocular Tumors

BACKGROUND: To present our experience on orbital and periorbital tissue changes after proton beam radiation therapy (PBRT) in patients with intraocular tumors, apart from treatment outcomes and disease control. METHODS: Medical records of 6 patients with intraocular tumors who had been treated with PBRT and referred to oculoplasty clinics of two medical centers (Seoul National University Hospital and Seoul Metropolitan Government-Seoul National University Boramae Medical Center) from October 2007 to September 2014 were retrospectively reviewed. The types of adverse effects associated with PBRT, their management, and progression were analyzed. In anophthalmic patients who eventually underwent enucleation after PBRT due to disease progression, orbital volume (OV) was assessed from magnetic resonance (MR) images using the Pinnacle3 program. RESULTS: Among the six patients with PBRT history, three had uveal melanoma, and three children had retinoblastoma. Two eyes were treated with PBRT only, while the other four eyes ultimately underwent enucleation. Two eyes with PBRT only suffered from radiation dermatitis and intractable epiphora due to canaliculitis or punctal obstruction. All four anophthalmic patients showed severe enophthalmic features with periorbital hollowness. OV analysis showed that the difference between both orbits was less than 0.1 cm before enucleation, but increased to more than 2 cm3 after enucleation. CONCLUSION: PBRT for intraocular tumors can induce various orbital and periorbital tissue changes. More specifically, when enucleation is performed after PBRT due to disease progression, significant enophthalmos and OV decrease can develop and can cause poor facial cosmesis as treatment sequelae.

Long-term Lens Complications Following Removal of Persistent Pupillary Membrane

PURPOSE: We evaluated the long-term surgical outcome and lens complications in children with persistent pupillary membrane following removal using vitreous scissors. METHODS: Patients diagnosed with persistent pupillary membrane who received surgical treatment from 1987 to 2012 were retrospectively reviewed. The removal was performed using vitreous scissors after instillation of miotics. The minimum follow-up period after surgery was four years. Factors of age, sex, visual acuity, refractive errors, and complications during or after surgery were evaluated. RESULTS: A total of 32 eyes of 26 patients were included. The mean age at the initial visit was 22.6 ± 34.7 (range, 0.9 to 141.2) months, and the mean age at surgery was 43.7 ± 36.0 (range, 1.0 to 142.5) months. There were no intraoperative complications using vitreous scissors, and all lesions were completely removed. After a mean follow-up period of 6.5 ± 3.3 (range, 4.0 to 14.8) years, the best corrected visual acuity at the final visit was 0.6 ± 0.9 logarithm of the minimum angle of resolution, and two eyes (6.3%) presented with lens opacity during follow-up. CONCLUSIONS: In children with persistent pupillary membrane, there were no intraoperative complications, and only two patients presented with lens change during the long-term postoperative follow-up period. Surgical removal should be considered a safe and effective treatment for patients with visually significant persistent pupillary membrane.

Outcomes of Cataract Surgery Following Treatment for Retinoblastoma

PURPOSE: To evaluate the long-term visual outcomes and complications of cataract surgery in eyes previously treated for retinoblastoma. METHODS: We reviewed the medical records of patients who underwent cataract extraction and intraocular lens implantation at Seoul National University Children's Hospital for a secondary cataract that developed after retinoblastoma treatment. RESULTS: During the period between 1990 and 2014, 208 eyes of 147 patients received eye-salvaging treatment (radiotherapy, chemotherapy, and local therapy) for retinoblastoma at Seoul National University Children's Hospital. Among these eyes, a secondary cataract was detected in 17 eyes of 14 patients, and five eyes of five patients underwent cataract surgery. The median age of cataract formation was 97 months (range, 38 to 153 months). The medial interval between the diagnosis of retinoblastoma and cataract formation was 79 months (range, 29 to 140 months). All patients received posterior chamber intraocular lens insertion after irrigation and aspiration of the lens through a scleral tunnel incision. Anterior vitrectomy and posterior capsulotomy were performed in two eyes and a laser capsulotomy was subsequently performed in one eye. No intraoperative and postoperative complications occurred. The median follow-up after surgery was 36 months (range, 14 to 47 months). The final best corrected visual acuities were improved in all five eyes. No intraocular tumor recurrences or metastases occurred. CONCLUSIONS: After retinoblastoma regression, cataract extraction in our series was not associated with tumor recurrence or metastasis. Visual improvement was noted in every patient.

Clinical Presentation and the Treatment of Glaucoma in Patients with a Facial Port-wine Stain

PURPOSE: To characterize the development of glaucoma, age of glaucoma onset, and treatments for patients with a facial port-wine stain (PWS). METHODS: We performed a retrospective analysis of the medical records of 58 patients (116 eyes) with facial PWS between January 2000 and August 2016. We noted patients' age at the initial examination, cup-to-disc ratio, corneal diameter, occurrence of ocular hypertension, development of glaucoma, age of glaucoma onset, and treatments. We compared the clinical features of eyes that developed glaucoma with those that did not develop glaucoma. Among those eyes with glaucoma, we investigated the differences between eyes that underwent surgery and those that did not undergo surgery. RESULTS: Among the 58 patients with a facial PWS (116 eyes), glaucoma was diagnosed in 38 patients (46 eyes; 39.66%). Of these, 26 patients (27 eyes; 58.69%) underwent glaucoma surgery. PWS-associated glaucoma usually developed by the age of 2 years (85.61%). In all patients, glaucoma developed on the same side of the face as the PWS. Of the 58 patients, 19 (32.76%) showed neurological symptoms, including seizures, developmental delays, intellectual disabilities, or hemiplegia, and 32 (55.17%) were diagnosed with Sturge-Weber syndrome. The mean number of glaucoma surgeries was 1.55 ± 0.93. The initial surgery included trabeculectomy (7 eyes), trabeculotomy (5 eyes), combined trabeculotomy/trabeculectomy (13 eyes), and aqueous drainage device insertion (2 eyes). The mean age at the first surgery was 35.14 ± 50.91 months. In 18 of 27 eyes (66.67%), the postoperative intraocular pressure (IOP) was controlled to below 21 mmHg, but 9 eyes (33.33%) showed elevated IOP and required a reoperation. CONCLUSIONS: PWS can be accompanied by ocular hypertension or glaucoma, so patients require regular ophthalmic examinations. When glaucoma occurs, it often does not respond to medication, making it difficult in some cases to control the IOP, so appropriate glaucoma surgery is necessary.

Ophthalmologic Features of Lennox-Gastaut Syndrome

PURPOSE: To describe the characteristics and frequency of ophthalmologic findings in patients with Lennox-Gastaut syndrome (LGS). METHODS: The medical records of patients diagnosed with LGS at Seoul National University Children's Hospital from January 2004 to August 2014 were retrospectively reviewed. The records of 34 patients (mean age ± standard deviation, 2.66 ± 3.51 years; male, 58.8%) were reviewed. The primary measure was the incidence of ophthalmologic manifestations. RESULTS: Of the 34 patients, 88.2% had at least one ocular abnormality. Refractive error (52.9%) was the most frequently observed ophthalmologic manifestation in patients with LGS, followed by strabismus (32.4%), cortical visual impairment (23.5%), and retinopathy of prematurity (8.8%). Among these cases, seven patients had exotropia and three had esotropia. CONCLUSIONS: LGS is a childhood-onset epileptic encephalopathy with variable ophthalmologic manifestations, the most frequent being refractive errors. Patients with suspected LGS should be examined regularly because ophthalmological features can change during their disease course.

Clinical Characteristics of Retinoblastoma Patients whose Diagnosis was Difficult due to Atypical Ocular Manifestation

PURPOSE: To report the clinical characteristics of retinoblastoma patients whose diagnosis was difficult due to atypical ocular manifestations. METHODS: Among retinoblastoma patients who were diagnosed and treated from January 1999 to December 2014 at Seoul National University Children's Hospital, 6 patients whose diagnosis was difficult were retrospectively reviewed. Factors including age, sex, family history, initial findings, time to final diagnosis, histopathologic examination, additional treatment, and survival rate were evaluated. RESULTS: Among 6 patients, 5 were male, and the mean age at the initial visit was 32.9 ± 19.1 months. None of the patients had family history, and all presented with unilateral lesion at the initial visit. The initial diagnoses were Coats' disease and uveitis in 2 patients, respectively, and persistent hyperplastic primary vitreous and traumatic hyphema in 1 patient, respectively. During an intensive short-term follow-up of 8.3 ± 5.3 weeks, 2 patients showed malignant cells after external subretinal fluid drainage procedure, and 4 patients demonstrated increasing ocular size or calcification in imaging. These patients received enucleation under suspicion of malignancy and were finally diagnosed with retinoblastoma after histopathologic examination. There were 2 patients with optic nerve involvement, and 3 patients underwent additional systemic chemotherapy. Five patients were followed-up for 7.6 ± 6.3 years after enucleation, and the mean age at final follow-up was 10.6 ± 7.4 years. CONCLUSIONS: Retinoblastoma is one of the diseases in which early diagnosis and treatment are important. However, some cases are difficult to diagnose, even for experienced clinicians. If there are no typical manifestations such as mass or calcification and early findings show retinal detachment, glaucoma, pseudohypopyon, or hyphema, intensive short-term follow-up to exclude retinoblastoma is needed.

Bilateral Optic Neuropathy in Middle-Aged Woman Associated with Charcot Marie Tooth Disease Type 2A: A Case Report

PURPOSE: Charcot-Marie-Tooth disease type 2A (CMT2A) is caused by mutations in the mitofusin 2 (MFN2) genes associated with variable central nervous system (CNS) involvement. The authors report a case of a middle-aged woman with genetically confirmed CMT type 2 (CMT2), combined with delayed-onset bilateral optic neuropathy. CASE SUMMARY: A 47-year-old woman presented with complaints of subacute decrease of visual acuity in both eyes. Her corrected visual acuity was 20/200 in the right eye and 20/320 in the left eye. Fundus photographs revealed bilateral disc pallor and diffuse retinal nerve fiber layer defects. No papillomacular bundle defect was observed. Goldmann perimetry showed central scotoma in both eyes. She had suffered from muscle wasting of the legs and foot deformities such as high arches and hammer toes since childhood and required a wheelchair for ambulation. A series of CMT gene mutation tests revealed an MFN2 gene mutation, c.617C>T (p.Thr206Ile), and the patient was diagnosed with CMT2A. CONCLUSIONS: Charcot-Marie-Tooth disease is a common inherited neuromuscular disorder and CMT2A, an axonal CMT neuropathy, is associated with bilateral optic neuropathy. Therefore, suspecting CMT and testing for gene mutations as part of the work-up in patients with subacute bilateral optic neuropathy associated with peripheral neuropathy is critical.

Congenital Aniridia: Long-term Clinical Course, Visual Outcome, and Prognostic Factors

PURPOSE: To describe the clinical course of congenital aniridia and to evaluate prognostic factors for visual outcome after long-term follow-up. METHODS: The medical records of 120 eyes from 60 patients with congenital aniridia were retrospectively reviewed. The prevalence and clinical course of ophthalmic characteristics, systemic disease, refractive errors, and visual acuity were assessed. Prognostic factors for final visual outcomes were analyzed. RESULTS: Aniridic keratopathy developed in 82 (69%) of 119 eyes. Macular hypoplasia was observed in 70 eyes of 35 patients (91%). Cataract was observed in 63 of 120 eyes (53%). Nystagmus was present in 41 patients (68% of 60 patients) at the initial visit but decreased in five patients (8% of 60 patients). Ocular hypertension was detected in 19 eyes (20% of 93 eyes), six (32% of 19 eyes) of which developed secondarily after cataract surgery. The mean changes in spherical equivalent and astigmatism during the follow-up period were -1.10 and 1.53 diopter, respectively. The mean final visual acuity was 1.028 logarithm of minimal angle of resolution. Nystagmus and ocular hypertension were identified as prognostic factors for poor visual outcome. CONCLUSIONS: Identification of nystagmus and ocular hypertension was important to predict final visual outcome. Based on the high rate of secondary ocular hypertension after cataract surgery, careful management is needed.

Optimal Timing of the First Screening Examination for Retinopathy of Prematurity

PURPOSE: In the present study, we aimed to evaluate the suitability of the first screening examination for retinopathy of prematurity (ROP) in a tertiary neonatal intensive care unit (NICU). METHODS: A retrospective analysis of ROP screening records of 459 infants admitted to the NICU of Seoul National University Children's Hospital between January 2006 and December 2011 was performed. The first examination was performed at 31-32 weeks of postmenstrual age (PMA) or 4-5 weeks of postnatal age (PNA), whichever was earlier. The infants were divided into subgroups according to the gestational age (GA), and the time of the first examination, time of onset of ROP, and time of laser surgery were assessed with regard to the PMA and PNA. RESULTS: Of the 459 infants, 139 infants developed ROP, with the mean PMA at onset of ROP being 34+2+/-2+3 weeks. Type I ROP developed in 57 infants, with the median PMA at laser surgery being 36+0 weeks. The median PMAs at the time of onset and the time of surgery did not significantly differ between groups divided according to GA. Infants with a GA of or =26 weeks. None of the infants with a GA of <26 weeks were diagnosed with ROP at the first examination, and none of the patients in either group missed treatment. Six infants developed ROP before 31 weeks of PMA, at which time the first screening for ROP is generally performed. CONCLUSION: We suggest that the timing of the initial examination for ROP should be based on PMA or PNA, whichever is earlier, particularly in infants with GA of <26 weeks.

Capillary-free Vascularized Retina in Patients with Aggressive Posterior Retinopathy of Prematurity and Late Retinal Capillary Formation

PURPOSE: To report the clinical features, clinical course, and treatment outcomes after laser photocoagulation in infants with aggressive posterior retinopathy of prematurity (APROP) and capillary-free zones in vascularized retina. METHODS: Six patients (12 eyes) with APROP and capillary-free zones in vascularized retina were retrospectively reviewed. Twelve eyes of six infants were included and were treated with laser photocoagulation for avascular retina and for capillary-free zones in vascularized retina, except for the posterior pole, and fundus findings were photographically-documented in sequence. In addition, anatomic and visual outcomes were evaluated with complications of APROP. RESULTS: Among all of the consecutive infants with APROP, capillary-free zones in vascularized retina were demonstrated in 24% of the infants. All of the infants were >27 weeks of gestation age and had birth weights >1,000 g. After laser treatment, 7 eyes (58.3%) had favorable outcomes, and late capillary filling in capillary-free zones of vascularized retina were noted, however 4 eyes (33.3%) progressed to retinal detachment and 1 eye (8.3%) was complicated by a retinal fold-distorting posterior pole. The visual outcomes were associated with anatomic outcomes. CONCLUSIONS: The anatomic outcomes in infants with APROP who had capillary-free zones were comparable to previously reported infants with APROP. The late capillary filling of capillary-free zones in vascularized retina was noted, and angiogenesis was considered to be involved. This process toward normal capillary formation or neovascularization in APROP, might determine its outcome.

Capillary-free Vascularized Retina in Patients with Aggressive Posterior Retinopathy of Prematurity and Late Retinal Capillary Formation

PURPOSE: To report the clinical features, clinical course, and treatment outcomes after laser photocoagulation in infants with aggressive posterior retinopathy of prematurity (APROP) and capillary-free zones in vascularized retina. METHODS: Six patients (12 eyes) with APROP and capillary-free zones in vascularized retina were retrospectively reviewed. Twelve eyes of six infants were included and were treated with laser photocoagulation for avascular retina and for capillary-free zones in vascularized retina, except for the posterior pole, and fundus findings were photographically-documented in sequence. In addition, anatomic and visual outcomes were evaluated with complications of APROP. RESULTS: Among all of the consecutive infants with APROP, capillary-free zones in vascularized retina were demonstrated in 24% of the infants. All of the infants were >27 weeks of gestation age and had birth weights >1,000 g. After laser treatment, 7 eyes (58.3%) had favorable outcomes, and late capillary filling in capillary-free zones of vascularized retina were noted, however 4 eyes (33.3%) progressed to retinal detachment and 1 eye (8.3%) was complicated by a retinal fold-distorting posterior pole. The visual outcomes were associated with anatomic outcomes. CONCLUSIONS: The anatomic outcomes in infants with APROP who had capillary-free zones were comparable to previously reported infants with APROP. The late capillary filling of capillary-free zones in vascularized retina was noted, and angiogenesis was considered to be involved. This process toward normal capillary formation or neovascularization in APROP, might determine its outcome.

Clinical Outcomes of Penetrating Keratoplasty in Patients Five Years or Younger

PURPOSE: To investigate the clinical outcomes of primary pediatric keratoplasty. METHODS: Records of patients who underwent penetrating keratoplasty at the age of 5 years or younger were retrospectively reviewed. The survival rates of corneal grafts, postoperative complications, and causes of graft failure were evaluated. RESULTS: A total of 31 penetrating keratoplasties were performed in 29 patients, two of which were bilateral. The mean follow-up period was 78.72 +/- 8.94 months. The overall graft survival rate was 51.61%. The graft survival rate was 77.4% at 6 months, 61.3% at 12 months, 57.5% at 2 years, and 49.5% at 5 years after the surgery (the median survival time, 39.2 months). The main surgical indications included sclerocornea (35.5%), followed by Peter's anomaly (25.8%) and congenital glaucoma (9.7%). There were significant differences in graft survival time among the surgical indications, of which sclerocornea was the worst (p = 0.003). The main cause of graft failure was rejection (46.7%), followed by infection (26.7%) and primary endothelial decompensation (20%). When patients were sub-grouped according to age (under 12 months, between 12 to 48 months, and over 48 months), there was significant difference in graft survival time (p = 0.037) but not in overall graft survival rate (p = 0.154). Graft rejection occurred more frequently in patients between 12 to 48 months of age compared to other age groups (p = 0.016). Three out of 13 graft infections occurred in patients under 12 months of age. CONCLUSIONS: The type of disease causing corneal opacity was a significant factor affecting the clinical outcomes of penetrating keratoplasty in children.

Long-term Results of Lens-sparing Vitrectomy for Progressive Posterior-type Stage 4A Retinopathy of Prematurity

PURPOSE: To assess the long-term anatomic and visual outcomes and associated complications of lens-sparing vitrectomy (LSV) in infants with progressive posterior-type tractional retinal detachment (TRD) associated with stage 4A retinopathy of prematurity (ROP). METHODS: In a retrospective case series, the medical records of consecutive patients who presented with progressive posterior-type stage 4A ROP and underwent LSV between 1999 and 2007 were reviewed. Retinal attachment status, visual acuity, and development of postoperative complications were assessed. RESULTS: Eleven eyes of 9 patients were included. The mean follow-up period was 4.6 years. In 8 eyes (73%), plus disease was present at the time of LSV. In 3 eyes (27%), 2 (66%) without plus disease and 1 (13%) with plus disease, the retina remained reattached in the end, while 8 eyes (73%) had TRD on final examination. Two eyes with reattached retinas showed favorable visual acuity. In those eyes with detached retinas, 5 (68%) showed no light perception. When surgery for ROP was unsuccessful, development of cataract, corneal opacity, or glaucoma was common. CONCLUSIONS: The long-term anatomic success rate of LSV for progressive posterior-type stage 4A ROP was low, especially in the presence of plus disease at the time of LSV. Anatomical reattachment is very important for preventing complications and gaining better visual outcomes.

Superior Rectus Muscle Recession for Residual Head Tilt after Inferior Oblique Muscle Weakening in Superior Oblique Palsy

PURPOSE: Residual head tilt has been reported in patients with superior oblique muscle palsy (SOP) after surgery to weaken the inferior oblique (IO) muscle. The treatments for these patients have not received appropriate attention. In this study, we evaluated the superior rectus (SR) muscle recession as a surgical treatment. METHODS: The medical records of 12 patients with SOP were retrospectively reviewed. Each of these patients had unilateral SR muscle recession for residual head tilt after IO muscle weakening due to SOP. The residual torticollis was classified into three groups on the basis of severity: mild, moderate, or severe. Both IO muscle overaction and vertical deviation, features of SOP, were evaluated in all patients. The severity of the preoperative and postoperative torticollis and vertical deviation were compared using a paired t-test and Fisher's exact test. RESULTS: The torticollis improved in nine of 12 (75%) patients after SR muscle recession. The difference between the preoperative and postoperative severity of torticollis was statistically significant (p = 0.0008). After surgery, the mean vertical deviation was significantly reduced from 12.4 prism diopters to 1.3 prism diopters (p = 0.0003). CONCLUSIONS: Unilateral SR muscle recession is an effective method to correct residual head tilt after IO muscle weakening in patients with SOP. This surgical procedure is believed to decrease head tilt by reducing the vertical deviation and thereby the compensatory head tilt.

Tenectomy and Posterior Tenectomy of the Superior Oblique for Superior Oblique Overaction

PURPOSE: The authors of the present study compared the effects of tenectomy and posterior tenectomy of the superior oblique for treating superior oblique overaction (SOOA). METHODS: The records of 30 eyes of 19 patients who underwent tenectomy or posterior tenectomy of the superior oblique alone or in combination with surgery on other horizontal rectus muscles from April 2005 through November 2010 were reviewed. The review was performed in 3 patients who underwent unilateral superior oblique tenectomy, 5 patients who underwent unilateral superior oblique posterior tenectomy, 3 patients who underwent bilateral superior oblique tenectomy, 6 patients who underwent bilateral superior oblique posterior tenectomy, and 2 patients who underwent superior oblique posterior tenectomy and tenectomy. RESULTS: The mean age of the patients was 14.2 years, and the mean follow-up period was 20 months. Through superior oblique tenectomy (11 eyes) and superior oblique posterior tenectomy (19 eyes), the amount of SOOA decreased from +2.27 to +0.09 and from +1.84 to +0.93, respectively. Except for 1 out of 11 eyes with superior oblique tenectomy, SOOA was completely corrected, although SOOA remained in 6 eyes after posterior tenectomy. Therefore, the correctional effects of the 2 methods differed; both procedures showed insignificant correctional effects on vertical strabismus. CONCLUSIONS: Superior oblique tenectomy has a superior long-term weakening effect; however, posterior tenectomy showed a comparable effect in the patient with moderate to severe SOOA. Both of these methods had few complications and are considered to be effective superior oblique weakening procedures.

Clinical Characteristics of Optic Neuritis in Koreans Greater than 50 Years of Age

PURPOSE: To report clinical characteristics of optic neuritis (ON) in Koreans >50 years of age. METHODS: A retrospective chart review was performed on patients with ON between January 2000 and December 2009. We obtained the best-corrected visual acuity (BCVA), Goldmann perimetry, relative afferent pupillary defect (RAPD), and color function tests as well as brain magnetic resonance imaging (MRI) findings in patients who were in the acute stage of the disorder. RESULTS: Nine eyes in eight patients were included. The mean age of patients at presentation was 60.5 years (range, 53 to 71 years). Six patients were female, and two were male. There was one patient with bilateral ON. The mean BCVA at presentation was 20 / 400 (no light perception-20 / 70). Eight eyes (89%) complained of pain with eye movement. Six eyes (66%) had disc edema. Central scotoma was the most common field defect. All eyes had color abnormalities. Five eyes in four patients showed abnormalities of the involved optic nerves on MRI. The patients were followed for a mean of 11.3 months (range, 2 to 34 months). All of the patients recovered to a BCVA of 20 / 40 or better within 2 months. On the last follow-up, the mean BCVA was 20 / 20 (20 / 40 to 20 / 16). Four eyes showed remnant central scotoma. One eye had remnant RAPD, and two eyes had mild color abnormalities. CONCLUSIONS: Although ON is uncommon in elderly patients, it can develop in patients >50 years of age, and clinical features of optic neuritis in elderly patients are similar to those of younger patients.