Lawrence M. Kaufman

Prevalence of Myopia between 3 Months and 5 '/-- Years in Preterm Infants with and without Retinopathy of Prematurity

Purpose: The purpose of the study was to examine spherical equivalent refractive errors, especially myopia, at six ages between 3 months and 5’/, years post-term in preterm children with birth weights of less than 1251 g. Design: A cohort study. Participants: There were a total of 827 participants in the multicenter study of cryotherapy for retinopathy of prematurity (ROP). Approximately one third of the eyes did not develop ROP, whereas two thirds developed mildto-severe ROP. None of the eyes underwent ctyotherapy. Intervention: Refractive error was measured at 3 months, 1 year, and 5’/2 years after term due date at the five long-term follow-up centers. In most eyes, refractive error also was measured at 2, 3’/*, and 4’/* years. Main Outcome Measure: Myopia was defined as 0.25 diopter (D) or greater with high myopia as 5 D or greater. Results: The proportion of eyes with myopia in this preterm population was increased compared to published data on full-term children and was related to severity of both acute-phase and cicatricial-phase ROP. The percentage of eyes with myopia varied little across ages, ranging from 21.2% at 1 year to 15.7% at 4’1’~ years. The percentage of eyes with high myopia doubled from 1.8% to 3.9% between 3 months and 1 year and remained stable thereafter. The distribution of refractive errors in eyes with mild acute-phase ROP was similar to that of eyes with no ROP. In contrast, eyes with moderate or severe acute-phase ROP showed an increased prevalence of high myopia. The distribution of refractive errors changed between 3 months and 1 year with little change after 1 year. This pattern of refractive development differs from that of full-term infants. Birth weight, severity of ROP, and degree of myopia at 3 months predicted the presence of myopia and high myopia at 5’1, years of age. Conclusions: The distribution of refractive errors in preterm infants from age 3 months to 51/2 years varies with severity of acute-phase ROP and cicatricial disease. Changes in refractive error distribution occur primarily between 3 months and 1 year and involve a decrease in the proportion of eyes with hyperopia and an increase in the proportion with high degrees of myopia. Ophfhalmology 7998; 705:7292- 7300

Pediatric tumors of the eye and orbit

Most ocular and orbital tumors of childhood are distinct from tumors that occur in adults. Many are congenital with early presentations. Most pediatric orbital tumors are benign; developmental cysts comprise half of orbital cases, with capillary hemangioma being the second most common orbital tumor. The most common orbital malignancy is rhabdomyosarcoma. The most common intraocular malignant lesion is retinoblastoma. Choroidal melanoma, which is common in adults, is extremely rare in children. The orbit is the most common location for metastases in children, whereas the choroid is the predominant site in adults. Pediatricians play a vital role in diagnosis of pediatric ocular tumors. They are the first to recognize ocular problems that may not be apparent to parents. It is therefore important to recognize the signs and symptoms of ocular tumors of childhood so that prompt ophthalmologic evaluation and treatment may be undertaken. Whereas the malignant tumors may be life-threatening, both malignant and benign tumors may be vision-threatening.

RETINOBLASTOMA AND SIMULATING LESIONS: Role of CT, MR Imaging and Use of Gd-DTPA Contrast Enhancement

The diagnosis of Rb can usually be made by clinical examination; however, patients should also be studied by CT and MR imaging to help determine the extent of the intraocular tumor, document intralesional calcification, confirm the diagnosis, and delineate orbital or intracranial involvement. These imaging techniques should aid the clinician in differentiating Rb from lesions that simulate Rb.

DIAGNOSTIC IMAGING OF CYSTIC LESIONS IN THE CHILD'S ORBIT

The childs orbit may be involved with a variety of cysts and cyst-like lesions. Such lesions may represent development anomalies of the globe and orbit, acquired lesions of the orbit, or cystic expansions from contiguous structures. CT and MR imaging are important diagnostic tools that aid the ophthalmologist in establishing the correct diagnosis and defining the extent of these cystic lesions.

Mitomycin in the treatment of hypertrophic conjunctival scars after strabismus surgery.

A recognized complication of strabismus surgery is the development of hypertrophic conjunctival scars over the operated-on muscle. Our standard treatment of these scars has been excision followed by corticosteroid eye drops, which results in a scar recurrence rate of 33%. Antimetabolic chemotherapeutic agents, such as mitomycin, can reduce posttraumatic scarring and vascularization of the ocular surface. We studied the use of mitomycin eye drops as an adjunct to standard treatment of hypertrophic conjunctival scars after strabismus surgery. Four patients were treated with adjunct mitomycin eye drops (0.2 to 0.4 mg/ml). Postoperative follow up ranged from 16 to 56 weeks. None of these cases showed significant scar recurrence. Mitomycin in the higher dosage caused mild superficial punctate keratitis after 8 days of use. Despite a lamellar sclerectomy in a patient treated with 0.2 mg/ml of mitomycin, there was no further clinically apparent loss of scleral tissue. The results of this pilot study suggest that topical mitomycin is a safe and effective adjunct to excision and corticosteroid eyedrops in the treatment of hypertrophic conjunctival scars after strabismus surgery.

Optic glioma warranting treatment in children

PurposeTo describe cases of optic pathway glioma (OPG) warranting treatment in children.MethodsThis is a retrospective review of pediatric patients treated for OPG. The clinical data and imaging studies were obtained from the medical records and radiology files of patients seen at the Pediatric Neuro-Ophthalmology Clinic at the University of Illinois, Chicago and the private office of the author (LMK).ResultsA total of seven cases with an age range of 3–48 months at presentation were reviewed. Three of the patients were also ultimately diagnosed with neurofibromatosis type 1. Presenting symptoms included proptosis, decreased vision, gaze deficit, and nystagmus. Four patients underwent biopsies that confirmed OPG. Six of the patients were treated with intravenous chemotherapy, with three patients requiring a second chemotherapy cycle. One patient was successfully treated with an en-bloc optic nerve excision. Two patients underwent unilateral enucleation owing to globe complications.ConclusionAlthough benign tumours, OPG can behave very aggressively in young children. Both chemotherapy and en-bloc excision can be employed for treatment.

Anatomy, development, and physiology of the visual system ☆

This article serves to familiarize the reader with the normal anatomy, embryology, and physiology of the visual system. Other articles in this issue introduce examination techniques and the pathology of the eyes of pediatric patients.

The effectiveness of occluder contact lenses in improving occlusion compliance in patients that have failed traditional occlusion therapy

Purpose. Management of infants and young children with dense amblyopia, including that which results from unilateral congenital cataracts, is challenging because of noncompliance with occlusion therapy. Occluder contact lenses (OCLs) have been described to successfully improve visual acuity in patients with amblyopia. The purpose of this study was to evaluate the effectiveness of OCLs in improving occlusion compliance in infants and young children with dense amblyopia who had failed traditional occlusion therapy. Methods. Thirteen patients were fit in their nonamblyopic eye with OCLs provided by Wesley Jessen (Des Plaines, IL). All patients had dense amblyopia as diagnosed by a pediatric ophthalmologist. Nine of the 13 patients had deprivational amblyopia associated with unilateral aphakia, 2 patients had strabismic amblyopia, and the other 2 had mixed mechanism amblyopia. All patients had failed traditional occlusion methods. Occlusion was prescribed on a part-time basis to prevent occlusion amblyopia to the better-seeing eye. Compliance with the OCLs was determined by questioning the parents as to whether the prescribed occlusion time was accomplished. Results. Five of the 13 patients (38.4%) did not succeed in the initial 1-mo trial period because of parental difficulties with lens handling. Four patients (30.7%) achieved partial success, as they were able to wear the lens between 4 to 14 mo, at which time they learned to remove the occluder lens. The four remaining patients (30.7%) successfully wore the occluder lenses until the conclusion of the study, a time period ranging from 26 to 60 mo. No complications resulted from OCLs. Conclusions. OCLs can provide an alternative to achieving occlusion compliance after the failure of traditional occlusion therapy in infants and young children with dense amblyopia.

Peripheral Nonperfusion and Tractional Retinal Detachment Associated with Congenital Optic Nerve Anomalies

PURPOSE To report an association of congenital optic nerve anomalies with peripheral retina nonperfusion and to describe the clinical manifestations and treatment. DESIGN Retrospective, observational case series. PARTICIPANTS Fifteen patients with congenital optic nerve anomalies referred for pediatric retina consultation were studied. Sixteen eyes of 9 patients with optic nerve hypoplasia and 8 eyes of 6 patients with other congenital optic nerve anomalies, including optic nerve coloboma, morning glory disc, and peripapillary staphyloma, were included. METHODS All patients underwent examinations under anesthesia. Wide-angle retina photographs and fluorescein angiograms were reviewed. The severity of nonperfusion was graded. The presence of fibrovascular proliferation (FP), vitreous hemorrhage (VH), and tractional retinal detachment (TRD) were documented. Anatomic outcome after treatment was recorded. MAIN OUTCOME MEASURES Severity of nonperfusion, occurrence of secondary complications, and the anatomic outcome of patients who underwent laser treatment. RESULTS In patients with optic nerve hypoplasia, 12 of 16 eyes (75%) had severe peripheral nonperfusion, 12 of 16 eyes (75%) had FP, 3 of 16 eyes (19%) had VH, and 10 of 16 eyes (63%) had TRD. Six of these eyes with severe nonperfusion received laser photocoagulation to the nonperfused retina; laser-treated retinas remained attached in all 6 eyes. In patients with the other optic nerve anomalies, 7 of 8 eyes (88%) had mild to moderate nonperfusion, 2 of 8 eyes (25%) had FP, 1 of 8 eyes (12%) had VH, and 2 of 8 eyes (25%) had TRD. Six of 9 patients (67%) with optic nerve hypoplasia and 1 of 6 patients (17%) with other anomalies had a coexisting congenital brain disease. CONCLUSIONS Congenital optic nerve anomalies may be associated with peripheral retina nonperfusion and the secondary complications of FP, VH, and TRD. In this select group of patients, the nonperfusion associated with optic nerve hypoplasia seemed to be more severe and associated more frequently with secondary complications. Peripheral retina examination in eyes with optic nerve anomalies may identify nonperfusion or FP. Laser treatment of the avascular retina may have helped prevent complications from proliferative retinopathy in eyes clinically observed to have progressed or considered at risk for progression to proliferative retinopathy.

Apnea and bradycardia in two premature infants during routine outpatient retinopathy of prematurity screening

Two unrelated premature infants at risk for retinopathy of prematurity (ROP) were discharged home from a neonatal intensive care unit. Appropriately timed ROP examinations were performed in an outpatient setting in a free-standing pediatric ophthalmologists private office remote from any hospital. The infants developed severe apnea and bradycardia at the end of the ROP examinations. As a result, a new protocol for outpatient ROP screening was developed.