An international comparison of retinopathy of prematurity grading performance within the Benefits of Oxygen Saturation Targeting II trials.

PurposeTo investigate whether the observed international differences in retinopathy of prematurity (ROP) treatment rates within the Benefits of Oxygen Saturation Targeting (BOOST) II trials might have been caused by international variation in ROP disease grading.MethodsGroups of BOOST II trial ophthalmologists in UK, Australia, and New Zealand (ANZ), and an international reference group (INT) used a web based system to grade a selection of RetCam images of ROP acquired during the BOOST II UK trial. Rates of decisions to treat, plus disease grading, ROP stage grading, ROP zone grading, inter-observer variation within groups and intra-observer variation within groups were measured.ResultsForty-two eye examinations were graded. UK ophthalmologists diagnosed treat-requiring ROP more frequently than ANZ ophthalmologists, 13.9 (3.49) compared to 9.4 (4.46) eye examinations, P=0.038. UK ophthalmologists diagnosed plus disease more frequently than ANZ ophthalmologists, 14.1 (6.23) compared to 8.5 (3.24) eye examinations, P=0.021. ANZ ophthalmologists diagnosed stage 2 ROP more frequently than UK ophthalmologists, 20.2 (5.8) compared to 12.7 (7.1) eye examinations, P=0.026. There were no other significant differences in the grading of ROP stage or zone. Inter-observer variation was higher within the UK group than within the ANZ group. Intra-observer variation was low in both groups.ConclusionsWe have found evidence of international variation in the diagnosis of treatment-requiring ROP. Improved standardisation of the diagnosis of treatment-requiring ROP is required. Measures might include improved training in the grading of ROP, using an international approach, and further development of ROP image analysis software.

Clinical characteristics and surgical management of congenital absence of the superior oblique tendon.

We reviewed clinical characteristics and surgical results of nine patients with surgically proved congenital absence of the superior oblique tendon. The following factors indicate absence of the tendon in the setting of superior oblique palsy: (1) an associated horizontal deviation, (2) amblyopia, (3) a large hypertropia in primary position, (4) spread of comitance, and (5) pseudo-overaction of the contralateral superior oblique muscle. The nine patients required a total of 19 operations to correct their vertical and horizontal deviations. Surgical management was based on the preoperative action of the inferior oblique muscle, the amount of hypertropia in primary position, and intraoperative forced ductions. After their operations, eight of nine patients had improvement in or abatement of their symptoms, and seven of seven with preoperative head tilts had improvement of their head position.

Treatment outcome in fellow eyes after laser photocoagulation for retinopathy of prematurity.

PURPOSE: To determine the concordance in structural outcome between fellow eyes in patients undergoing diode laser peripheral retinal photocoagulation for threshold retinopathy of prematurity. METHODS: Records from 103 patients undergoing bilateral diode laser peripheral retinal photocoagulation for retinopathy of prematurity at three academic medical centers were reviewed. Information regarding infant gestational ages, birth weights, characteristics of retinopathy of prematurity, laser treatment variables, complications, and structural outcomes were obtained. The data were analyzed to determine the rate of successful structural outcomes among all eyes as well as the interocular outcome concordance. RESULTS: A successful structural outcome was observed in 182 (88%) of the 206 eyes. Eighty-eight patients (85.4%) had bilateral favorable outcomes. Nine patients (8.7%) had bilateral unfavorable outcomes, and six patients (5.8%) had one favorable and one unfavorable eye. The outcome was concordant between fellow eyes in 94.2% of patients. This rate was higher than predicted if fellow-eye outcomes were truly independent (P < .00001) and did not depend on study center, laser settings, or location of the retinopathy of prematurity. Serious complications related to treatment were uncommon. CONCLUSIONS: Diode laser peripheral retinal photocoagulation is a safe and effective procedure for treating threshold retinopathy of prematurity. The concordance of structural outcomes between fellow eyes is higher than expected by chance and suggests that patient-specific factors play a key role in treatment response.

Persistent hyperplastic primary vitreous involving the anterior eye.

The imaging features of persistent hyperplastic primary vitreous (PHPV) affecting the posterior eye are well known. We recently encountered a patient with the anterior variant of PHPV who had MR imaging of the orbits. We present the clinical and imaging findings of this unusual entity and discuss the therapeutic options available for its management.

Juvenile retinoschisis: imaging findings.

We present the CT and B-scan sonographic findings in an infant with juvenile retinoschisis, a rare hereditary eye disease, which usually follows an X-linked recessive inheritance pattern.

Proton MR spectroscopy in Coats disease.

We describe a case of acute left-sided visual loss in a 4-year-old boy. CT showed hyperdense retinal detachment with a tiny calcification, and MR imaging showed subretinal hyperintensity on both T1- and T2-weighted images. Proton MR spectroscopy showed a large peak between 1 and 1.6 ppm that we believe corresponds mainly to lipids, which are characteristic of the exudate present in Coats disease.

Visual acuity after cycloplegia in children: implications for atropine penalization.

BACKGROUND: Atropinization of the sound eye is an alternative to patching in the treatment of amblyopia. Whether atropine treatment can induce a switch in fixation depends on the refractive error of the sound eye, visual acuity of the amblyopic eye, distance from the fixation target, and presence of any optical correction or penalization. General guidelines are needed on the basis of refractive error and visual acuity in the amblyopic eye to predict which patients may potentially benefit from atropine penalization. METHODS: Refractive error and visual acuity at distance (6 m) and/or at near (33 cm) were recorded in a normal eye of 126 consecutive children (mean age, 8.2 years), 30 to 60 minutes after receiving cyclopentolate 1%. Visual acuity was plotted versus refractive error at distance and at near, and best-fit curves were calculated. RESULTS: There was a consistent, reproducible relationship between refractive error and visual acuity after cycloplegia at both distance and near in healthy children. CONCLUSIONS: The results of this study can be used to quickly determine whether atropine penalization has the potential for success on the basis of a patient's visual acuity in the amblyopic eye and refractive error in the sound eye. When adequate hyperopia is present in the sound eye, one should consider testing for fixation preference or initiating a therapeutic trial of atropine. Those children with insufficient hyperopia in the sound eye relative to visual acuity in the amblyopic eye can be spared the time, expense, and potential side effects of atropine penalization.

Strabismus surgery complicated by "pulled in two syndrome" in a case of breast carcinoma metastatic to the medial rectus muscle.

Metastatic carcinoma to the extraocular muscles is extremely rare; it is reported to occur from breast, lung, and gastric carcinoma as well as skin melanoma. (1-3) Overall, intraocular metastases occur much more frequently than orbital metastases.(4) The most common primary tumors causing orbital metastases are breast and lung carcinomas.(5) Strabismus due to orbital metastases from breast carcinoma usually results from fibrosis of the muscle, which often causes painful ophthalmoplegia and enophthalmos. (6,7) We report a case of presumed metastatic carcinoma to the medial rectus muscle causing restrictive strabismus in which surgery was complicated by the "pulled in two syndrome," or PITS.

Prognostic significance of vascular dilation and tortuosity insufficient for plus disease in retinopathy of prematurity.

BACKGROUND: Plus disease, one of the most important prognostic indicators in retinopathy of prematurity (ROP), is designated as present or absent. A grading system based on comparison with standard, high-quality color photographs may be useful to more accurately describe the spectrum of vascular dilation and tortuosity preceding plus disease, but it is of practical value only if it has prognostic significance. We hypothesized that grading of "pre-plus" vascular changes can identify eyes at risk for progression to vision-threatening ROP. METHODS: Video clips of posterior pole images captured at the examination closest to 33 weeks' postconceptional age of 32 infants screened during an 18-month period were randomized. Two masked examiners viewed and graded the images in comparison with standard photographs representative of 5 distinct grades of retinal vascular dilation and tortuosity. A case-control design was used to compare the incidence of progression to stage 3 ROP, development of plus disease, and requirement of laser treatment between infants with normal posterior poles and those judged to have early dilation and tortuosity insufficient for plus disease. RESULTS: Of the 8 patients with mild vascular dilation and tortuosity insufficient for plus disease, 5 (63%) eventually required laser treatment, 4 (50%) later developed stage 3 ROP, and 5 (63%) progressed to plus disease. Of the 24 patients with normal posterior poles, none required laser treatment, 2 (8%) developed stage 3 ROP, and none progressed to plus disease. The group with mild vascular dilation and tortuosity had a significantly higher incidence of progression to laser treatment (P =.0003), stage 3 ROP (P =.027), and plus disease (P =.0003). CONCLUSIONS: Early vascular dilation and tortuosity judged insufficient for plus disease have prognostic significance in the early course of ROP. A grading system that uses standard, high-quality color photographs representing the spectrum of "pre-plus" vascular changes has potential utility in both the clinical and research settings.

Ophthalmic manifestations of Angelman syndrome.

In 1965, Angelman described 3 cases of what he called "Puppet" children, named for the characteristic signs associated with what is now known as Angelman syndrome, including mental retardation, speech impairment, easy excitability, and frequent spontaneous laughter.(1) Since that report, much progress has been made in defining the syndrome's clinical manifestations and understanding its molecular foundations, including identification of deletions of 15q11-13 in some patients. There are few reports in the ophthalmic literature regarding ocular manifestations of this syndrome. (2,3) We present the case of a child with strabismus associated with Angelman syndrome, and we review the ophthalmic and systemic findings, as well as recent advances in molecular genetics, in these patients.

Significance of isolated neovascular tufts ("popcorn") in retinopathy of prematurity.

BACKGROUND: The significance of isolated neovascular tufts ("popcorn") occurring in association with stage 2 retinopathy of prematurity (ROP) has not been studied. METHODS: We retrospectively reviewed the clinical courses and outcomes of all patients with zone II, stage 2 ROP with popcorn examined over the past 3 years at one institution. Eyes with zone I disease, plus disease, or stage 3 at the initial appearance of popcorn were excluded. The study group was compared with a control group of patients of similar birth weight and gestational age with zone II, stage 2 ROP without popcorn. RESULTS: Popcorn first appeared at a mean age of 36.4 (+/- 2.2) weeks after conception in 26 patients. Of these, 17 patients (65%) progressed to stage 3, 10 (38%) had plus disease, 6 (23%) reached threshold, and 9 (35%) required laser treatment. Of 19 control patients, 4 (21%) progressed to stage 3, 1 (5%) had plus disease, 1 (5%) reached threshold, and 1 (5%) required laser treatment. The popcorn group had a significantly higher incidence of progression to stage 3 (p < 0.005), plus disease (p < 0.025), and laser treatment (p < 0.025). All eyes of both groups had complete regression of disease. CONCLUSIONS: The presence of popcorn significantly increases the risk that an eye with zone II, stage 2 ROP will progress to stage 3, develop plus disease, and require laser treatment. Patients with popcorn and coexistent mild vascular dilation or tortuosity insufficient for plus disease are at particularly high risk for disease progression.

Poor postnatal weight gain: a risk factor for severe retinopathy of prematurity.

PURPOSE: This study aimed to investigate the relationship between rate of postnatal weight gain and severity of retinopathy of prematurity (ROP). METHODS: All infants (n = 111) screened for ROP at a single tertiary intensive care nursery over a 2-year period with an estimated gestational age of 30 weeks or less and follow-up to at least 42 weeks' postconception were included. The authors performed a retrospective review of records and statistical analysis of risk factors for ROP using multivariate analysis. RESULTS: Infants with severe (stage 3 or greater) ROP gained an average 10.9 g/kg per day in the first 6 weeks of life, compared to a mean of 9.6 g/kg per day for those with mild or no ROP (P =.04). By multiple regression, which included birth weight, gestational age, and 9 other reported risk factors, there was an association between rate of postnatal weight gain and severity of ROP (P =.02). By stepwise regression, 4 variables were associated with ROP severity: estimated gestational age at birth (P =.002), rate of postnatal weight gain (P = .0002), volume of transfused erythrocytes (P =.0001), and culture-proven sepsis (P = .02). CONCLUSION: Poor postnatal weight gain is a risk factor for the development of severe (stage 3 or greater) ROP. Ophthalmologists should take note of those infants who gain less than 50% of their birth weight in the first 6 weeks of life.

Ocular medications in children.

Many ocular medications are used by pediatricians or ophthalmologists caring for pediatric patients. Topical antibiotics are commonly prescribed for bacterial conjunctivitis, nasolacrimal duct obstructions, and ophthalmia neonatorum. Many new antiallergy eye drops are now available for the treatment of seasonal (hay fever) conjunctivitis. Dilating eye drops and antiglaucoma medications are generally used or prescribed by ophthalmologists, but pediatricians must be aware of their potentially serious systemic side effects. Before initiating treatment, physicians should evaluate the risks and benefits of ophthalmic medications, establish minimum dosages necessary to achieve a therapeutic benefit, and monitor children for local and systemic side effects.

Corneal diameter in childhood aphakic glaucoma.

BACKGROUND: Glaucoma occurring after successful cataract surgery in infancy and childhood requires persistence to establish timely diagnosis and presents a therapeutic challenge. Previous studies have sought to identify risk factors, which would alert the clinician for the likelihood of developing glaucoma after cataract surgery in the young patient. Some risk factors are early age of surgery, microcornea, poor pupillary dilation, retained lens cortex, coexisting ocular anomalies, and cataract type. Other studies did not find these associations. In our experience, the majority of young aphakic glaucoma patients have microcornea. This study was carried out to investigate this association. METHODS: The records were reviewed of all patients treated for aphakic glaucoma between 1991 and 1995 to determine the age at cataract surgery, age at glaucoma surgery, age at glaucoma diagnosis, corneal diameters, and other clinical characteristics. RESULTS: Forty-eight eyes in 29 patients were identified with aphakic glaucoma. Forty-five of the 48 (94%) eyes were found to have microcornea when compared with the normal corneal diameter for their age. CONCLUSION: All children undergoing cataract surgery should have their corneal diameters recorded. Patients with corneal diameters smaller than normal should be followed closely for the development of glaucoma throughout childhood and beyond.

Preoperative issues in adult strabismus.

Preoperatively, strabismus surgeons need to obtain informed consent, identify any ocular or systemic risk factors, decide on the preferred method of anesthesia, and determine whether the patient is an appropriate candidate for an adjustable suture technique. Three major categories of complications should be listed on the consent form: Loss of vision, double vison, and need for reoperation. If a patient is at particulary high risk to have one of these complications, then the surgeon should emphasize this point during the preoperative discussion. The incidence of visual loss after strabismus surgery due to anterior segment ischemia, retinal detachment, or endophthalmitis is approximately 1 in 7,400 cases. The preferred method of anesthesia depends on the procedure planned, the age and general health status of the patient, and whether or not an adjustable suture technique is to be utilized. At the conclusion of the preoperative discussion, the patient should be well informed and comfortable with the choice or procedure, have reasonable postoperative expectations in terms of diplopia, and be prepared for any unexpected complications.

Amino acid structures of multiple forms of amyloid-related serum protein SAA from a single individual.

Multiple forms of the acute-phase serum protein SAA were isolated from the lipoprotein fraction of plasma from a single individual. These protein forms were purified by size-exclusion, ion-exchange, and reverse-phase high-pressure liquid chromatography, and then the tryptic peptides were subjected to amino acid sequence analysis. A total of three distinct 104-residue proteins were identified. Two of these proteins differed only by having either an arginine or a histidine at position 71 while the third protein had seven amino acid differences. Each of these proteins has a 103-residue companion protein where the amino-terminal arginine has been removed. Two of these protein sequences match the two human SAA cDNA structures reported in the literature. The presence of three unique amino acid sequences in one individual is proof that there must be a minimum of two genes for SAA in humans.

Surgical management of strabismus associated with chronic progressive external ophthalmoplegia.

PURPOSE: The purpose of the study is to describe the clinical characteristics and surgical management of strabismus associated with chronic progressive external ophthalmoplegia. METHODS: The authors present four patients with chronic progressive external ophthalmoplegia and strabismus requiring extraocular muscle surgery, with attention to presenting symptoms, patterns of misalignment, results of surgical and nonsurgical therapies, and associated ocular or systemic conditions or both. RESULTS: Three patients reported diplopia before surgery. One patient presented with an esotropia, one with an exotropia, and two with hypertropia. Three patients required only one strabismus surgery, and one patient required multiple surgeries. CONCLUSIONS: Chronic progressive external ophthalmoplegia may have clinical characteristics similar to those of myasthenia gravis or thyroid ophthalmopathy. Patients with chronic progressive external ophthalmoplegia and strabismus frequently have diplopia in primary position and may benefit from extraocular muscle surgery to improve alignment. In addition, because these patients typically have poor motor fusion, prisms often are useful adjuncts to surgery.