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The authors report on the incidence of myopia in a large group of premature infants with birth weights of less than 1251 g followed as part of the multicenter study of Cryotherapy for Retinopathy of Prematurity. None of the eyes reported here underwent cryotherapy. Eyes were refracted using cycloplegic retinoscopy at 3 months (n = 2916), 12 months (n = 2626), and 24 months (n = 961 at 5 of the 23 centers) after term. Myopia was observed in approximately 20% of the children at each test age. The percentage of high myopia (greater than or equal to 5 diopters) doubled from 2% to 4.6% between 3 and 12 months and remained stable thereafter. Lower birth weight and increasing severity of retinopathy of prematurity (ROP) were strong predictors of myopia and high myopia. In addition, anisometropia, astigmatism, and the presence of posterior pole residua from ROP also were associated with a higher incidence of myopia and high myopia.

Background and Methods. The use of corticosteroids to treat optic neuritis is controversial. At 15 clinical centers, we randomly assigned 457 patients with acute optic neuritis to receive oral prednisone (1 mg per kilogram of body weight per day) for 14 days; intravenous methylprednisolone (1 g per day) for 3 days, followed by oral prednisone (1 mg per kilogram per day) for 11 days; or oral placebo for 14 days. Visual function was assessed over a six-month follow-up period. Results. Visual function recovered faster in the group receiving intravenous methylprednisolone than in the placebo group; this was particularly true for the reversal of visual-field defects (P = 0.0001). Although the differences between the groups decreased with time, at six months the group that received intravenous methylprednisolone still had slightly better visual fields (P = 0.054), contrast sensitivity (P = 0.026), and color vision (P = 0.033) but not better visual acuity (P = 0.66). The outcome in the oral-prednisone group did ...

We treated three children with optic pathway gliomas who had progressive disease associated with metastatic spread to the leptomeninges. One patient had radiographic resolution of leptomeningeal disease after treatment with intravenous carmustine and oral mercaptopurine but died of progressive pulmonary fibrosis. The second patient was treated with intravenous thiotepa, and the leptomeningeal disease remained stable. The third patient was treated with intravenous vincristine sulfate, cyclophosphamide, cis-platin, and etoposide and had a significant size reduction of the leptomeningeal lesion. Although leptomeningeal dissemination is a seemingly rare event, it is important that all children with optic pathway gliomas be considered for this possibility, particularly after the onset of new, atypical neurologic symptoms.

Botulinum toxin was used to treat 20 patients with strabismus after retinal detachment surgery. Preinjection motility deviations ranged from 10 to 60 prism diopters (D). Postinjection deviations ranged from 0 to 20 prism D, with 75% being 10 D or less. Eighty-five percent achieved fusion that persisted, with 73% requiring only one or two injections. Only muscles in the eye that had undergone retinal reattachment surgery were injected. The average period of follow-up was 12 months. Complications were rare and all resolved spontaneously. Botulinum toxin appears to be useful as a primary treatment modality for persistent strabismus following retinal detachment surgery, possibly obviating the need for complicated strabismus surgery.

To assess the risk of developing multiple sclerosis (MS) after optic neuritis and the factors predictive of high and low risk.Subjects in the Optic Neuritis Treatment Trial, who were enrolled between July 1, 1988, and June 30, 1991, were followed up prospectively for 15 years, with the final examination in 2006.Neurologic and ophthalmologic examinations at 13 clinical sites.Three hundred eighty-nine subjects with acute optic neuritis.Development of MS and neurologic disability assessment.The cumulative probability of developing MS by 15 years after onset of optic neuritis was 50% (95% confidence interval, 44%-56%) and strongly related to presence of lesions on a baseline non-contrast-enhanced magnetic resonance imaging (MRI) of the brain. Twenty-five percent of patients with no lesions on baseline brain MRI developed MS during follow-up compared with 72% of patients with 1 or more lesions. After 10 years, the risk of developing MS was very low for patients without baseline lesions but remained substantial for those with lesions. Among patients without lesions on MRI, baseline factors associated with a substantially lower risk for MS included male sex, optic disc swelling, and certain atypical features of optic neuritis.The presence of brain MRI abnormalities at the time of an optic neuritis attack is a strong predictor of the 15-year risk of MS. In the absence of MRI-detected lesions, male sex, optic disc swelling, and atypical clinical features of optic neuritis are associated with a low likelihood of developing MS. This natural history information is important when considering prophylactic treatment for MS at the time of a first acute onset of optic neuritis.

To evaluate the long-term efficacy, safety, and advisability of using transscleral sutured posterior chamber intraocular lenses (IOLs) in pediatric patients with no capsular support and to determine whether 10-0 polypropylene suture should be used for this purpose.A long-term retrospective interventional case series review of 33 eyes of 26 patients who had a sutured IOL at Duke University Eye Center were evaluated for the intraoperative surgical risks, postoperative visual and refractive outcomes, and the number, type, and severity of the postoperative complications. In addition, a survey of pediatric ophthalmologists' experience with suture breakage was performed.Postoperative visual acuity was significantly improved after surgery (P.001). Predicted vs actual refraction was not significantly different (P = .10) and was within 1.50 diopters of predicted in 66% of patients. A refractive myopic shift occurred over time and was age-dependent. Intraoperative and immediate postoperative complications were minimal and not sight-threatening. Three patients developed subluxation of the IOL secondary to spontaneous 10-0 polypropylene suture breakage at 3.5, 8, and 9 years after surgery. A survey of pediatric ophthalmologists revealed 10 similar cases (mean, 5 years after surgery).Transscleral fixation of an IOL in a child appears to be a safe and effective procedure provided that the suture material used is stable enough to resist significant degradation over time. Caution should be exercised in the use of 10-0 polypropylene suture to fixate an IOL to the sclera in children, and an alternative material or size should be considered.

Purpose To evaluate the long-term efficacy, safety, and advisability of transscleral-sutured posterior chamber intraocular lenses (IOLs) in pediatric patients with no capsular support and to determine whether a 10-0 polypropylene suture should be used for this purpose. Methods A long-term retrospective interventional case series review of 33 eyes of 26 patients who had a sutured IOL at Duke University Eye Center. Cases were evaluated for the intraoperative surgical risks and the number, type, and severity of the postoperative complications. A survey of pediatric ophthalmologists' experience with suture breakage was performed. Results Intraoperative and immediate postoperative complications were minimal and not sight-threatening for the patient. Four patients developed subluxation of the IOL secondary to spontaneous 10-0 polypropylene suture breakage at 3.5, 5, 6, and 8 years after surgery. A survey of pediatric ophthalmologists revealed 13 similar cases (mean, 5 years after surgery). Conclusions Caution should be exercised in the use of 10-0 polypropylene suture to fixate an IOL to the sclera in children, and an alternative material or size (such as 9-0 polypropylene) should be considered.

Duke University and the National University of Singapore (NUS) have partnered to launch a new medical school that brings the American style of postbaccalaureate medical education to Asia. The new institution, called the Duke-NUS Graduate Medical School (GMS) and located in Singapore adjacent to the Singapore General Hospital, admitted its inaugural class of students representing citizens of seven nations in August 2007. The project represents an investment of more than $350 million from three ministries of the Singapore government, and a commitment on Duke's part to provide senior leadership and recruit faculty from Duke, from other international locales, and from within Singapore itself. Graduating students who complete the four-year Duke curriculum will receive an MD degree awarded jointly by Duke and NUS, thereby distinguishing this school from medical education in most Asian institutions that award an MBBS degree after a five-year period of study that follows directly from secondary school. The emphasis of the Duke-NUS GMS is to prepare physician-scientists for academic careers, with plans for 20% of each class to complete a combined MD/PhD degree. This article describes events leading up to this partnership and details of the relationship, including curriculum, organizational structure, milestones, and goals.

Six adult patients with acquired, chronic, complete sixth nerve palsy had vertical rectus muscle transposition to the lateral rectus muscle insertion with intraoperative injection of botulinum toxin into the ipsilateral medial rectus muscle. Five of six patients were orthophoric in primary gaze, and the remaining patient achieved single binocular vision with a small head turn. This method compares favorably with previously described transposition procedures for sixth nerve palsy in terms of amount of correction and size of the window of single binocular vision with a lower risk of anterior segment ischemia.

To provide clinically relevant information regarding the diagnosis, etiology, work-up and treatment of third cranial nerve palsies, while incorporating information from current publications and providing our opinions on these studies.In the past year, an important study focused on the confirmation of current neuro-imaging guidelines for third cranial nerve palsies. Recent case reports have highlighted etiologies such as giant cell arteritis, trauma, neuro-syphilis and demyelination secondary to infliximab and multiple sclerosis. Surgically, newer studies have focused on globe-tethering procedures for correcting strabismus and the use of frontalis suspension for correcting ptosis. Improved imaging technology with high-resolution magnetic resonance imaging (hr-MRI) allows for direct visualization of the entire nerve path and the affected muscles.Management of a third nerve palsy depends upon localization of the causative lesion and determination of the underlying etiology. Once these issues are addressed, strabismus surgery can be both challenging and rewarding in these complex patients.

This chapter highlights the areas of the eye examination that are of significance in assessing pediatric neuro-ophthalmic disorders. Before beginning the formal examination, spend a few moments interacting with the child in a nonthreatening way. Gaining the child’s confidence early in the exam will be rewarded later when cooperation is imperative. A great deal of information can be gathered by simple observation and, therefore, surveillance for subtle findings should be maintained during the exam. After establishing rapport with the child, the order in which information is collected depends on the conditions of the examination and the cooperation of the child. Rigid adherence to a particular regimen may lead to a frustrating experience. It is fruitless to begin taking a long, detailed history if it is obvious that the child is already somewhat fussy and rapidly losing attention. In such circumstances, the exam will have to be goal directed. By prioritizing the examination to fit the “problem,” the maximum amount of information can be obtained in what often turns out to be a very limited amount of time.

To evaluate optical coherence tomography (OCT) measurement of peripapillary retinal nerve fiber layer (RNFL) and macular thickness in children with pseudotumor cerebri.Prospective observational series of children with pseudotumor cerebri compared against controls matched for age and gender. We included 11 pediatric subjects with clinical pseudotumor cerebri and 37 normal controls. Subjects underwent a complete eye examination including stereo disk photographs and OCT. Peripapillary RNFL and macular thickness were evaluated using the Stratus OCT 3000. The peripapillary RNFL was evaluated with two protocols: Fast RNFL Map protocol with values divided in eight sections, made up of four quadrants, each with an inner and outer ring, and Fast RNFL Thickness (3.4) protocol, using values presented for four quadrants. A Fast Macular Thickness Map was also obtained, and results reported similarly included eight sections, made up of four quadrants, each with an inner and outer ring.The Fast RNFL Map protocol showed an increased RNFL thickness (pseudotumor cerebri vs controls) in the temporal and superior quadrants. The Fast RNFL Thickness (3.4) protocol showed a thicker average RNFL for pseudotumor cerebri eyes compared with controls (125.7 vs 106.5 microm, p0.0001). The Fast Macular Thickness Map showed an increased RNFL thickness in the nasal quadrant for the inner ring. The macular volumes for pseudotumor cerebri versus control eyes were 7.21 versus 6.97 mm(3), respectively (p = 0.005).OCT shows increased RNFL and macular thickness in pseudotumor cerebri and may be a useful clinical tool in these children.

PURPOSE: To determine if latanoprost reduces intraocular pressure in eyes with glaucoma associated with Sturge-Weber syndrome. METHODS: We conducted a prospective study in which eyes with uncontrolled intraocular pressure associated with Sturge-Weber syndrome were treated with latanoprost 0.005% once daily. All eyes were already receiving at least two other antiglaucoma medications. Intraocular pressure was measured at baseline and after treatment for at least 1 month. All intraocular pressure measurements were taken within 24 hours of drug instillation. RESULTS: Six eyes of six patients received latanoprost. Two (28%) of the six eyes demonstrated an intraocular pressure decrease that averaged 8.8 mm Hg. These two responders had juvenile onset glaucoma, whereas the four nonresponders had congenital onset glaucoma. CONCLUSIONS: Latanoprost may significantly reduce intraocular pressure in selected patients with glaucoma associated with Sturge-Weber syndrome.

In this study, the authors sought to investigate the response rate and toxicity of carboplatin in patients with progressive low-grade glioma (LGG). Thirty-two patients with progressive LGG were treated with carboplatin at a dosage of 560 mg/m2. Treatment was given at 4-week intervals and continued until the disease progressed, unacceptable toxicity supervened, or for 12 additional courses after achieving maximal response. Patients with stable disease were treated with a total of 12 cycles. All patients were treated as outpatients. Patients were evaluated for response to treatment and toxicity. All patients received a minimum of two cycles of carboplatin, and were examined for response. A partial response was achieved in nine patients (28%) and a minimal response in two (6%), for an overall response rate of 34% (11 of 32 patients). Eighteen patients (56%) had stable disease. A partial response was achieved in the nine patients after a median of six cycles (range 4-11 cycles), a minimal response was achieved in the two patients after five cycles. Glioma progression was noted in three patients after three, five, and five cycles, respectively. The 11 patients in whom some response was achieved had either an optic pathway tumor or a juvenile pilocytic astrocytoma. Twenty-six of the 32 patients had those characteristics, making the response rate in that group 42% (11 of 26 patients). Thirty-two patients received a total of 387 cycles of chemotherapy. Hematological toxicity was moderate. Twenty-one patients developed thrombocytopenia (platelet count < 50,000/μl); three patients required one platelet transfusion each. Nine patients developed neutropenia (absolute neutrophil count < 500/μl); one developed fever and required administration of antibiotic agents. One dose adjustment in each of the patients prevented further thrombocytopenia and neutropenia. Two patients with stable disease died of respiratory complications. One patient developed Grade III ototoxicity after receiving five cycles, one patient developed hypersensitivity to carboplatin, and none developed nephrotoxicity. Carboplatin given at a dosage of 560 mg/m2 every 4 weeks has activity in patients with progressive LGG. This drug regimen is relatively simple and well tolerated. Further investigation and longer follow-up study are warranted.

The diagnosis and etiologies of sixth cranial nerve palsies are reviewed along with nonsurgical and surgical treatment approaches. Surgical options depend on the function of the paretic muscle, the field of greatest symptoms, and the likelihood of inducing diplopia in additional fields by a given procedure.

Introduction: Paradoxical patterns of extraocular muscle, eyelid, or pupillary movements can occur following injury between divisions of the oculomotor nerve, trigeminal and abducens nerves, and trigeminal and oculomotor nerves. We report three cases of unusual ocular motility and eyelid movements that are a result of aberrant connections between the abducens and oculomotor nerves. Methods: Three patients with unusual eye movement abnormalities after trauma were studied. A complete ophthalmic examination plus neuroradiologic evaluation were performed. Results: Each patient manifested an aberrant connection between the 6th and 3rd cranial nerves resulting in third nerve function during sixth nerve stimulation. Two patients demonstrated complete third nerve palsies except for adduction on attempted abduction. The third showed improved bilateral ptosis on abduction. Conclusions: The neuroanatomical abnormalities involve intraorbital structures in one patient and central nervous system pathways in the others. Explanations such as retrograde regeneration, ephaptic transmission, or denervation supersensitivity do not appear to explain these unusual eye movements. The most likely mechanism involves some form of peripheral neuronal misdirection. These rare sixth to third nerve misdirection cases add support to the “neuronal misdirection hypothesis” of aberrant eye movements after trauma.

To compare optotype acuities and re-operation rates in children corrected with a contact lens (CL) compared with an intraocular lens (IOL) following unilateral cataract extraction during infancy in a non-randomised, retrospective case series.25 infants with a unilateral congenital cataract underwent cataract surgery with (IOL group, n = 12) or without (CL group, n = 13) IOL implantation when7 months of age. Optotype acuities were assessed in 19 of these children at a mean age of 4.3 years (range 3.3-5.5 years). The number of re-operations were assessed in 21 children.The visual acuity results were similar in the two treatment groups (p = 0.99); however, two of the four (50%) children in the IOL group compared with two of the seven (28%) children in the CL group undergoing surgery during the first 6 weeks of life had 20/40 or better visual acuity. The children in the IOL group had more re-operations than the children in the CL group (mean 1.1 v 0.36). Most of the re-operations in the IOL group were membranectomies performed during the first year of life (median 8.0 months) whereas all of the re-operations in the CL group were the implantation of a secondary IOL later in childhood (mean 2.2 years).Optotype acuities were similar for the children corrected with a CL compared with IOL, while the children in the IOL group underwent more re-operations .

Purpose To review our experience with optic nerve sheath decompression for pediatric pseudotumor cerebri. Design Retrospective chart review. Participants Seventeen eyes in 12 children younger than 16 years of age. All patients were either unresponsive or intolerant to medication. Intervention, Methods, or Testing An optic nerve sheath fenestration was performed. Main Outcome Measures Optic nerve appearance, visual acuity, color vision, and visual fields. Results The average age at surgery was 10.1 years of age. The average follow-up was 39.6 months. Headache was the most common presenting symptom. All patients showed improvement in optic nerve edema. Visual acuity improved or stayed the same in all surgical eyes ( P = 0.0078). One patient required a neurosurgical lumbar peritoneal shunt, and 2 patients required acetazolamide on the last follow-up appointment. No patient had postoperative infection, loss of vision, or strabismus develop. Five of the patients in this study required sheath decompression on the other eye. Conclusions Optic nerve sheath decompression in children is safe, and the results are similar to those obtained in adults. Close follow-up is required, because 5 of 12 patients in this study required a contralateral optic nerve sheath decompression.

To determine whether a randomized clinical trial, the Infant Aphakia Treatment Study, comparing intraocular lens (IOL) implantation with contact lens (CL) correction for infants with a unilateral congenital cataract (UCC), is feasible by (1) ascertaining whether American Association for Pediatric Ophthalmology and Strabismus (AAPOS) members have equipoise regarding these two treatments and (2) evaluating the willingness of parents to agree to randomization.All AAPOS members were surveyed in August 1997 and again in June 2001 regarding their use of CLs and IOL implants to correct infants vision after unilateral cataract surgery. In addition, a pilot study was begun in March 2002 to evaluate the safety of IOL implantation during infancy and the willingness of parents to randomize their children with a UCC to either IOL implantation or CL correction.In 1997, 89% of the 260 respondents reported that in the previous year they had treated at least one infant with a UCC, but only 4% had implanted an IOL in an infant7 months old. Silsoft (BauschLomb, Rochester, NY) CL correction was the preferred treatment choice for 84% of the respondents. In 2001, 21% of the 279 respondents had implanted an IOL in an infant. On a scale from 1 to 10 with 1 strongly favoring an IOL implant and 10 strongly favoring a CL, the median score was 7.5. Sixty-one percent of the respondents indicated that they would be willing to randomize children with a UCC to one of these two treatments. The main concerns about IOL implantation were poor predictability of power changes, postoperative complications, inflammation, and technical difficulty of surgery. The main concerns about CL correction were poor compliance, high lens loss rate, high cost, and keratitis. In our pilot study, 30 infants7 months of age were evaluated at nine clinical centers for a visually significant UCC. Of 24 infants eligible for randomization, the parents of 17 (71%) agreed to randomization.Although most AAPOS members still favor CL correction after cataract surgery for a UCC, five times as many had implanted an IOL in an infant in 2001 compared with the number in 1997. Parents were almost equally divided in their preference for IOL implant versus CL correction. Given the relative equipoise of AAPOS members regarding these treatments and the willingness of more than two thirds of parents to agree to randomization, it seems likely that a randomized clinical trial comparing these two treatments could indeed be conducted.

PURPOSE To assess visual function more than 10 years after an episode of optic neuritis in patients enrolled in the Optic Neuritis Treatment Trial. DESIGN Longitudinal follow-up of a randomized clinical trial. METHODS Vision testing included measures of visual acuity, contrast sensitivity, and visual field. Quality of life was assessed with the National Eye Institute Visual Function Questionnaire. RESULTS Examinations were completed on 319 patients. In most patients, visual function test results in the eyes that experienced optic neuritis at study entry ("affected eyes") were normal or only slightly abnormal after 9.9 to 13.7 years. Visual acuity in the affected eyes was >or=20/20 in 74%, 20/25 to 20/40 in 18%

Patients with cranial/skeletal defects often exhibit neuroophthalmic abnormalities, which may be caused by specific anomalies within the spectrum of a given condition, or by an associated malformation of the nervous system, or be secondary to mechanical forces such as hydrocephalus. This chapter reviews the ophthalmic abnormalities found in progressive hemifacial atrophy, which are primarily due to structural defects,as well as ophthalmic abnormalities in Arnold-Chiari malformations, meningomyelocele, platybasia, and the Klippel-Feil syndrome,which are related to both structural and secondary neurological mechanisms.

When to recommend surgery for congenital esotropia has long been controversial. In 1939, Chavasse hypothesized that the degree of binocular sensory dysfunction that resulted from a strabismus was directly related to the age of onset and the duration of misalignment. He concluded that the sooner the eyes are aligned, the better. Although this makes sense intuitively, until recently, there was little evidence to support that notion, and, in fact, most studies seemed to suggest that the timing of successful motor alignment, within certain limitations, had very little impact on the patient’s ultimate sensory status. Malcolm Ing performed the most influential study challenging Chavasse’s theory in the early 1980s. He demonstrated that alignment by age 2 could yield gross stereopsis, as measured by the Titmus test, in a high percentage of patients, but earlier alignment did not seem to be more beneficial. That is to say that alignment before 6 months of age appeared to be no better than alignment between 12 and 24 months as long as the eyes were aligned by the magic age of 2. This study had a profound effect on the recommended timing of strabismus surgery for the next 25 years. Surgeons routinely deferred surgery to obtain better motility measurements and thus facilitate a better chance at a good motor outcome. They did so believing that the delay in alignment would not impact the ultimate sensory result. In the early 1990s, reports began to surface demonstrating remarkable sensory outcomes in patients who underwent very early esotropia surgery. Wright et al and later Birch et al demonstrated improved stereopsis and, in some patients, high-grade stereopsis with this more aggressive approach. Last year, Birch et al published results in this Journal on the sensory outcome of congenital esotropic patients who had prompt surgery, some before 6 months, but also others at ages up to 24 months. Those whose eyes were realigned within 12 months of the onset of the esotropia had significantly better stereopsis. The authors concluded that the duration of the strabismus, rather than the age at alignment, was the key factor in determining who would be most likely to achieve a good sensory result from strabismus surgery. Was Ing wrong, or was there something unique or unrecognized about the patients he studied that would explain this contradiction? To answer that question in this issue of the Journal, Ing relooked at his previous data with this new concept in mind. He went back and determined, as best he could, when the esotropia actually began and then, using the same analysis as Birch, compared the sensory outcome with duration of strabismus. Not surprisingly, his reanalysis confirmed Birch’s conclusion that the duration of the strabismus is the controlling factor. Ing’s original study must have included a significant number of patients whose esotropia occurred late, and, therefore, although the surgery was not performed until after 12 months of age, the strabismus had not been present for that entire period. In analyzing the data by age at alignment, the effect of the duration of the strabismus was missed. In retrospect, this makes sense. It has long been known that congenital esotropia is a misnomer and that most are really infantile, possibly having some early period of alignment before the onset of the strabismus. The problem has been to determine just when the eyes became misaligned. Parents and even health professionals are poor observers about the state of alignment of infant eyes. Gathering this type of data is fraught with uncertainty, and thus it is difficult to use it with any reliability. Ing and Birch made acceptable attempts to determine just when the strabismus began and, in doing so, have added a new dimension to our treatment approach to this disorder. The results of these 2 studies indicate that time does matter. The sooner the eyes are aligned after becoming misaligned, the more likely a good sensory outcome will result. When duration is included as a factor in data analysis, those whose eyes were aligned within 12 months of the onset of their strabismus did well, but those whose strabismus persisted beyond 12 months did very poorly. At the same time that this concept is being supported clinically, animal model investigations have demonstrated binocular cortical cell maldevelopment in animals that had disruption of ocular alignment, and the severity appears to be related to age at onset and duration. It is so nice when it all fits together. All of this means that we need to be more aggressive in our treatment of young children with strabismus. Earlier intervention appears to increase the chance of a good sensory outcome, which increases the likelihood of longterm stability of the postsurgical alignment. Pediatricians Corresponding author: Edward G. Buckley, MD, Duke University Eye Center, P. O. Box 3802, Durham, NC 27710. (e-mail: BUCKL002@mc.duke.edu). J AAPOS 2002;6:1-2. Copyright © 2002 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/2002/$35.00 0 75/1/122061 doi:10.1067/mpa.2002.122061

Importance The efficacy and safety of primary intraocular lens (IOL) implantation during early infancy is unknown. Objective To compare the visual outcomes of patients optically corrected with contact lenses vs IOLs following unilateral cataract surgery during early infancy. Design, Setting, and Participants The Infant Aphakia Treatment Study is a randomized clinical trial with 5 years of follow-up that involved 114 infants with unilateral congenital cataracts at 12 sites. A traveling examiner assessed visual acuity at age 4.5 years. Interventions Cataract surgery with or without primary IOL implantation. Contact lenses were used to correct aphakia in patients who did not receive IOLs. Treatment was determined through random assignment. Main Outcomes and Measures HOTV optotype visual acuity at 4.5 years of age. Results The median logMAR visual acuity was not significantly different between the treated eyes in the 2 treatment groups (both, 0.90 [20/159]; P = .54). About 50% of treated eyes in both groups had visual acuity less than or equal to 20/200. Significantly more patients in the IOL group had at least 1 adverse event after cataract surgery (contact lens, 56%; IOL, 81%; P = .02). The most common adverse events in the IOL group were lens reproliferation into the visual axis, pupillary membranes, and corectopia. Glaucoma/glaucoma suspect occurred in 35% of treated eyes in the contact lens group vs 28% of eyes in the IOL group ( P = .55). Since the initial cataract surgery, significantly more patients in the IOL group have had at least 1 additional intraocular surgery (contact lens, 21%; IOL, 72%; P Conclusions and Relevance There was no significant difference between the median visual acuity of operated eyes in children who underwent primary IOL implantation and those left aphakic. However, there were significantly more adverse events and additional intraoperative procedures in the IOL group. When operating on an infant younger than 7 months of age with a unilateral cataract, we recommend leaving the eye aphakic and focusing the eye with a contact lens. Primary IOL implantation should be reserved for those infants where, in the opinion of the surgeon, the cost and handling of a contact lens would be so burdensome as to result in significant periods of uncorrected aphakia. Trial Registration clinicaltrials.gov Identifier:NCT00212134

To evaluate outcomes at 10 years after randomization for eyes undergoing cryotherapy vs eyes serving as controls, for patients enrolled in the Multicenter Trial of Cryotherapy for Retinopathy of Prematurity (CRYO-ROP).The randomized cohort originally consisted of 291 preterm children with birth weights less than 1251 g who developed a defined threshold of ROP severity in one or both eyes. Patients with bilateral threshold ROP (n = 240) were randomly assigned to receive cryotherapy to one eye and no cryotherapy to the other eye. Those with ROP of less severity than threshold in the fellow eye ("asymmetric"; n = 51) were randomly assigned to cryotherapy or no cryotherapy in the eye with threshold ROP. Ten years later, a tester who was masked to treatment status of each eye measured distance and near visual acuity, with "unfavorable" outcome being 20/200 or worse. Patients also were evaluated by study-certified ophthalmologists who assessed ROP residua primarily in the posterior pole of the fundus, with unfavorable outcome being a posterior retinal fold or worse.For the 247 children examined, both functional and structural primary outcomes showed fewer unfavorable outcomes in treated vs control eyes: 44.4% vs 62.1% (P.001) for distance visual acuity and 27.2% vs 47.9% (P.001) for fundus status. Near acuity results were similar to those for distance (42.5% vs 61.6%; P.001). Total retinal detachments had continued to occur in control eyes, increasing from 38.6% at 5(1/2) years to 41.4% at 10 years, while treated eyes remained stable (at 22.0%). A previously disturbing subgroup trend that more control eyes than treated eyes had visual acuity of 20/40 or better (in the 5(1/2)-year report) was no longer present at 10 years; eyes that received cryotherapy were found at least as likely as control eyes to have 20/40 or better visual acuity.At 10 years, eyes that had received cryotherapy were much less likely than control eyes to be blind. A previous trend for a higher proportion of sighted control eyes than sighted treated eyes to show acuity in the normal range was not confirmed. The results show long-term value from cryotherapy in preserving visual acuity in eyes with threshold ROP.

Purpose: The method of correcting aphakia after unilateral cataract extraction during infancy is controversial. Some authorities advocate correction with an intraocular lens (IOL) whereas others advocate correction with a contact lens (CL). We compared grating visual acuity, alignment, and reoperative outcomes in age-matched children treated with these 2 modalities at 5 clinical centers. Methods: Twenty-five infants born in 1997 or 1998 with a dense unilateral congenital cataract who had cataract surgery coupled with (IOL group, n = 12) or without (CL group, n = 13) primary IOL implantation were enrolled in this study. All patients were prescribed half-time occlusion therapy. In July 1999, their grating visual acuities, ocular alignments, and reoperation rates were assessed. Results: The mean grating visual acuity (LogMAR) for the affected eye was 0.70 ± 0.32 for the IOL group and 0.87 ± 0.31 for the CL group (P = .19). The mean interocular difference in grating visual acuity was 0.26 ± 0.30 for the IOL group and 0.50 ± 0.28 for the CL group (P = .048). The incidence of strabismus (>10 PD) was 75% in the IOL group compared with 92% in the CL group (P = .24). The incidence of reoperations was 83% in the IOL group compared with 23% in the CL group (P = .003). Conclusions: Our preliminary data suggest that correcting aphakia after unilateral congenital cataract surgery with primary IOL implantation results in an improved visual outcome but a higher rate of complications requiring reoperation. A randomized clinical trial, the Infant Aphakia Treatment Study, is planned to further study the optimal treatment for aphakia following unilateral cataract extraction during infancy. (J AAPOS 2001;5:70-5)

Purpose: The purpose of this study was to evaluate the effects of oblique muscle surgery on the large-angle incyclotorsion resulting from macular translocation surgery for severe age-related macular degeneration. Methods: Patients undergoing macular translocation (superiorly) at our institution from May 1996 until November 1998 were included. In the Staged Group, strabismus surgery for symptomatic incyclotorsion was performed after the macular translocation, and in the Combined Group, it was performed simultaneous with the macular translocation. Cyclotorsion was quantified using Maddox rod testing. Surgery for incyclotorsion included superior oblique muscle recession combined with inferior oblique muscle advancement and transposition in the affected eyes. The minimum follow-up time was 6 weeks. Results: Fifteen patients (15 eyes) were included (ages 66–89 years). Nine eyes (Baseline Group) had macular translocation surgery before any strabismus surgery; the mean postoperative incylotorsional angle was 33.4 ± 18.3 degrees (range, 20–80 degrees) after a mean follow-up of 6.6 months. Four of these eyes (Staged Group) underwent oblique muscle surgery for symptomatic incyclotorsion, which reduced the mean incyclotorsion from 26.9 ± 6.9 degrees (range, 20–35 degrees) to 9.9 ± 7.9 degrees (range, 2.5–20 degrees)—a mean reduction of 16.9 ± 1.3 degrees (P = .00012), after a mean follow-up of 4.6 months. Six additional eyes (Combined Group) had simultaneous macular translocation and oblique muscle surgery, with a mean postoperative cyclotorsional angle of 14.0 ± 6.7 degrees (range, 4–22.5 degrees), after a mean follow-up of 3.75 months. Conclusion: Oblique muscle surgery is effective at reducing the large degree of incyclotorsion resulting from macular translocation surgery and may be used either following or simultaneous with retinal surgery. (J AAPOS 2000;4:75–83)

Purpose: Open-angle glaucoma may develop after surgery for congenital or developmental cataract with an incidence ranging from 3% to 41%. The pathogenesis of "aphakic" (open-angle) glaucoma remains unknown. Despite numerous reported clinical series (>1000 eyes), we are unaware of any reported case of open-angle glaucoma after primary intraocular lens (IOL) implantation for congenital or developmental cataract. We decided to test the hypothesis that primary posterior chamber IOL implantation might decrease the incidence of open-angle glaucoma in children. Methods: Pseudophakic eyes were collected from surgeons who contributed data to a refractive study and who monitored intraocular pressure on a regular basis. IOL implantation was commonly performed in eyes with a corneal diameter >10 mm. Comparable primary data on aphakic eyes were included from 2 published studies on aphakic glaucoma, which included corneal diameters and the patient's age at surgery. Glaucoma-free survival estimates for each cohort were estimated. Results: Only 1 case of glaucoma was found among 377 eyes with primary pseudophakia (mean age of patient, 5.1 ± 4.7 years; mean follow-up, 3.9 ± 2.7 years). There were 14 eyes (11.3%) with glaucoma among 124 aphakic eyes (mean age of patient, 2.7 ± 2.6 years; mean follow-up time, 7.2 ± 3.9 years). Conclusions: We report a decreased incidence of open-angle glaucoma among eyes rendered primarily pseudophakic compared with those that remained aphakic after cataract surgery. We propose 2 theories on the possible mechanism of reduction in the incidence of glaucoma in pseudophakic eyes. (J AAPOS 2000;4:33-9)

Background: Optical rehabilitation of unilateral aphakia in eyes with no capsular support is problematic in pediatric patients who cannot tolerate contact lenses. Possible options include a unilateral aphakic spectacle, an anterior chamber intraocular lens (IOL), or a scleral fixated posterior chamber IOL. Of these choices the posterior chamber IOL is the most physiologic. Experience in adults shows increased complications with this technique. Objective: The objective of this study was to report the short-term results and complications of unilateral scleral fixated posterior chamber IOLs in the pediatric population. Methods: All patients with scleral fixated lenses younger than 16 years were retrospectively reviewed. Nine patients aged 12 months to 15 years underwent unilateral scleral fixated posterior chamber lens implantation using buried polypropylene fixation sutures. Follow-up averaged 24 months. Results: Postoperative visual acuity improved in all patients. Refractive goals were achieved in all but 1 patient. Complications included elevated intraocular pressure controlled with medications (1 patient), anterior uveitis (1 patient), and mild IOL decentration (1 patient). Conclusions: Although short-term visual results appear encouraging, this procedure is technically more difficult and has an increased incidence of postoperative complications when compared with secondary sulcus-fixated IOLs supported by capsular remnants. Caution should be exercised when recommending this procedure for pediatric patients because long-term risks are unknown. (J AAPOS 1999;3:289-94)

Purpose: The patient with a large esodeviation at near and a minimal if any deviation at distance who is unable to discontinue bifocal wear or demonstrates a decrease in binocular function presents a difficult therapeutic dilemma to the strabismologist. The medial rectus fadenoperation has been proposed as a method of reducing the accommodative convergence/accommodation ( AC A ) ratio and may be a reasonable approach to correct a deviation that is present only at near fixation. We examined our results with this type of operation in these difficult patients. Methods: A review of consecutive patients who underwent a bilateral medial rectus fadenoperation (no recession) for high AC A ratio esotropia with a significant esodeviation only at near was performed. Preoperative and postoperative motility and sensory status were evaluated. Results: Sixteen patients meeting the criteria were identified. The preoperative mean distance deviation was 8.8 PD of esodeviation and the mean near deviation was 33 PD of esodeviation. The mean AC A ratio (by the gradient method) preoperatively was 7.4 PD/D. The average age at surgery was 8 years. Six (38%) had undergone prior strabismus surgery. Average follow-up was 36 months (range 12 to 98 months). Postoperatively the mean distance deviation was 3.2 PD of esodeviation and the mean near deviation was 10.3 PD of esodeviation. The mean decrease in the near deviation was 22.8 PD. The mean postoperative AC A ratio was 2.9. Ninety-five percent of the patients normalized their AC A ratios and 70% attained 400 arc-sec of stereopsis postoperatively (vs 44% preoperatively. The majority of patients (86%) were able to maintain satisfactory near ocular alignment without bifocals. Conclusions: The fadenoperation appears to be useful in this patient population by reducing the distance-near disparity and the AC A ratio, by decreasing the necessity for bifocal wear, and by improving binocular function at near.

Background: The treatment of incomitant strabismus is challenging. Traditional approaches include the use of asymmetric bilateral surgery and the fadenoperation (posterior fixation suture). We report our experience with a different approach: combined resection and recession of a single rectus muscle. Methods: The charts of 12 patients who underwent resection of a single rectus muscle with an equal or greater amount of recession of the same muscle were identified. In 5 patients, the procedure was performed using the adjustable suture technique, and the adjustment was performed later the same day (Group 1). In the remaining 7 patients, permanent sutures were placed at the time of surgery (Group 2). The procedure was performed for horizontal and vertical gaze incomitance, dissociated horizontal deviation, and distance-near disparity. Results: Four of the 5 patients in Group 1 showed stable, long-term correction of their incomitance, both in primary gaze and in gaze in the direction of the muscle operated on. The results for patients in Group 2 showed stable, long-term correction of incomitance in 3 patients; however, these patients also had slight overcorrections in the direction of gaze opposite to the muscle operated on. An additional patient in Group 2 had a shift of her distance-near disparity, shifting from relatively exotropic to relatively esotropic disparity postoperatively. All patients in Group 2 showed at least some decrease in the amount of measured incomitance. We did not encounter complications such as muscle slippage or loss, scleral perforation, or late overcorrection in the field of gaze of the operated muscle. Conclusions: The technique of combined resection and recession of a single rectus muscle shows promise in the treatment of incomitant strabismus. It offers the advantages of posterior fixation combined with the greater technical ease of a standard hangback recession. The muscle may also be placed on an adjustable suture, allowing for postoperative adjustment in selected patients. (J AAPOS 1999;3:263-8)

Purpose Results of a phase II trial of cyclophosphamide (CPM) for children with progressive low-grade astrocytoma are reported. Patients and methods Fifteen patients with a median age of 39 months (range, 2 to 71) were included in this study. The tumors of 11 children were located in the optic pathway, hypothalamus, or thalamus. Four courses of intravenous CPM 1.2 g/m2 were administered every 3 weeks during the upfront window portion of this protocol. Subsequently, chemotherapy was to continue with CPM, vincristine, and carboplatin for 2 years. Results By study design, the first 14 patients were centrally reviewed after completion of the initial 4 CPM courses. Toxicity was primarily hematologic. One patients had a complete response, 8 had stable disease, and 5 had progressive disease (PD). The excessive number of children with PD prompted study closure. Conclusion CPM as used in this protocol showed insufficient activity against astrocytoma to justify further patient accrual.

Purpose: Latanoprost is a prostaglandin F 2α analogue that substantially reduces intraocular pressure (IOP) in adults with open-angle glaucoma or ocular hypertension. The efficacy and safety of latanoprost in children is unknown. We wished to evaluate latanoprost therapy for children with glaucoma. Methods: This was a prospective study of all patients who were given latanoprost at our institution between September 1996 and January 1998. The baseline IOP was compared with the postlatanoprost IOP for each patient, and side effects and any iris color change were noted at each follow-up examination. Responders were defined as those who had at least a 15% IOP reduction on latanoprost, whereas nonresponders showed less than a 15% IOP reduction on latanoprost. Results: Fifty-seven eyes of 48 pediatric patients with a variety of glaucoma diagnoses and an average age of 7.1 years were included in the study. Of these, 31 eyes of 31 patients had interpretable IOP data; the mean IOP reduction for this group after the addition of latanoprost was 0.9% (0.2 mm Hg). Six patients (6 eyes) were responders, with an average IOP reduction of 8.5 mm Hg (34%), whereas the majority of patients (25) were nonresponders. In the responders, there was a good correlation ( r = 0.9) between baseline IOP and the magnitude of IOP reduction. Responders were significantly more likely to have juvenile open-angle glaucoma and to be older than nonresponders. Systemic and ocular side effects were infrequent and mild, and no patient had noticeable iris color changes. Conclusions: When used in a group of pediatric patients with a variety of glaucoma diagnoses and on various therapies, latanoprost is effective in only a minority of cases. In selected patients, however, latanoprost shows an impressive ocular hypotensive effect, comparable with that obtained when this drug is used in adults with open-angle glaucoma. This drug appears to be well tolerated in this short-term study of pediatric patients with glaucoma. (J AAPOS 1999;3:33-9)

PURPOSE: To report refractive changes after cataract surgery and intraocular lens implantation in infants and children. METHODS: In an ongoing prospective study, the refractive errors of all patients younger than 18 years undergoing intraocular lens implantation were determined at 6 weeks, 3 months, 6 months, and 1 year, and at least yearly thereafter. All patients with greater than 6 months of follow-up were included in the study. RESULTS: Eighty-three eyes of 81 patients were identified. Cataracts were traumatic in 32 eyes (38%) and developmental in 42 eyes (50%). At implantation, the mean (±SD) age was 6.3 ± 4.6 years (range, 9 months to 17 years). The mean follow-up was 26.6 months (range, 6 months to 6.6 years). Patients 0 to 2 years old at the time of implantation demonstrated a mean myopic shift of −3.00 diopters during a mean follow-up period of 2.5 years. Patients 2 to 6 years old at the time of implantation demonstrated a mean myopic shift of −1.50 diopters in a similar follow-up period. Children aged 6 to 8 years experienced a mean myopic shift of −1.80 diopters during a mean follow-up period of 3.0 years, while children older than 8 years at the time of intraocular lens implantation experienced a mean myopic shift of −0.38 diopters during a mean follow-up period of 1.8 years. On average, the operated-on eye showed a greater mean myopic shift than the fellow eye. No statistically significant differences in refractive change were found in comparing amblyopic to nonamblyopic eyes, traumatic to nontraumatic cataracts, or primary to secondary intraocular lenses. CONCLUSIONS: Our data demonstrate a trend toward increasing postoperative myopia in pediatric patients undergoing intraocular lens implantation. This myopic shift is greatest in the younger age groups and persists until at least 8 years of age. There is much variability in the postoperative refractive changes, and predicting exactly when and where the refraction will stabilize for an individual patient is difficult.

Purpose To report results of secondary posterior chamber intraocular lens (IOL) implantation in previously aphakic pediatric patients. Methods In 19 pediatric patients, 19 aphakic eyes (11 after infantile and eight after traumatic cataract surgery) received secondary sulcus-fixated posterior chamber IOL implants. Results Visual acuity of 20/40 or better was achieved with IOL implantation and overrefraction in three of 11 infantile (27%) and six of eight traumatic cataract patients (mean follow-ups, 18.1 months [range, 8 to 29 months] and 18.0 months [range, 6 to 28 months]), respectively. Eighteen of 19 patients (95%) demonstrated postoperative vision equal to or better than preoperative levels; 15 of 19 patients (79%) showed improved vision after IOL implantation. The mean ± SD difference between actual and predicted postoperative refraction at 1 month was -0.97 ± 0.96 diopter. Average refractive error at last examination was -0.40 ± 2.43 diopters. Amblyopia therapy was performed in 14 patients. One IOL required repositioning 8 months postoperatively. Strabismus was present in 14 patients before and 13 patients after IOL implantation, requiring surgery in four patients. Conclusions Secondary IOL implantation can be performed successfully in carefully selected pediatric patients. Visual acuity results are better in eyes with a history of traumatic cataract and are influenced by patient compliance. The short-term risks of the procedure appear no greater than those of primary IOL implantation, and complications resemble those seen in adults.

Patients with germinal retinoblastoma (those with bilateral disease or positive family history) have a mutation which puts them at risk for developing new tumors. It is unclear whether the frequency of new tumor development is effected by the type of treatment employed. It may be hypothesized that external beam radiation ‘sterilizes’ the whole retina, and thus decreases the risk of new and recurrent tumors. We reviewed our experience with 66 eyes in 47 patients over the past ten years. We did not find a significant difference in the incidence of new and recurrent retinoblastoma among eyes treated with external beam radiation versus focal modalities.

Background: Retinal astrocytic hamartoma and retinoblastoma may be very similar clinically, and their differentiation in atypical cases can be difficult, even with the use of ancillary methods such as fluorescein angiography, ultrasonography, and computed tomography. To the authors' knowledge, fine-needle aspiration biopsy has not been used to diagnose astrocytic hamartoma in such cases. Patients and Methods: A 7-week-old boy had a minimally calcified retinal mass in the macular area of the left eye associated with an extensive secondary retinal detachment. The differential diagnosis included retinal astrocytic hamartoma and retinoblastoma. A transvitreal fine-needle aspiration biopsy was performed. Results: The cytology of the needle biopsy showed benign spindle and stellate cells, which were compatible with glial cells. The lesion had immunoreactivity for glial fibrillary acidic protein, further supporting the diagnosis of astrocytic tumor. Conclusion: Fine-needle aspiration biopsy is diagnostically useful in unusual cases where the differential diagnosis between retinoblastoma and astrocytic hamartoma is difficult.

HE INCIDENCE of strabismus and diploT pia after retinal detachment surgery has been reported to be as high as 60%.' Fortunately, a large percentage of patients have only transient motility disturbances that have been attributed to postoperative periocular edema, decreased vision, and extraocular muscle swelling and hemorrhage. Recovery is often rapid, with only 5% to 25% of patients experiencing persistent diplopia longer than 3 months after retinal surgery (Table 1).*-Is ETIOLOGY Virtually every known retinal surgical procedure can cause strabismus, including cryopexy alone.4%6J6x17 The cause of strabismus can be divided into four major headings: mechanical, direct muscle injury, redirection of muscle force vectors, and sensory disruption (Fig 1). MECHANICAL Adhesions

Purpose: To assess the effectiveness of vitrectomy for stage V retinopathy of prematurity (ROP) at the authors' institution, 33 patients with ROP who had initially successful total or partial anatomic retinal attachments (51 eyes) were evaluated for visual function and long-term anatomic stability. In addition, this study was an attempt to differentiate levels of visual function in children with very low vision and attendant developmental delays. Methods: Visual function was assessed before retinal examination by an experienced pediatric vision specialist. The behavioral visual assessment was divided into seven segments with two to five tasks in each segment designed to establish a level of functional vision, ranging from light perception to form identification (and ambulation). The status of the retina was classified by the patient's retinal surgeon. Results: The median follow-up was 61 months, and median age at the time of examination was 68 months. Of 51 eyes treated, form identification was achieved in 5, and all five patients had ambulatory vision. Of 51 eyes, 15 had no light perception, 11 had light perception, 6 could localize light, 10 could follow lights, and 4 were able to detect form. Only one eye in ten children with comparable ROP in each eye underwent surgery, and vision improved in six of ten of the surgical eyes. Redetachment was high, with 35 retinas totally or partially detached and only 13 retinas fully attached 61 months after surgery. Conclusion: The visual results are very low and disappointing. Initially successfully attached retinas can detach. There is some evidence that vitrectomized eyes function better than nonvitrectomized eyes. There is also evidence that visual function lower than form identification is useful to these children and that they are able to use their limited vision better than previously thought.