The usefulness of the Retinomax autorefractor for childhood screening validated against a Danish preterm cohort examined at the age of 4 years.

BACKGROUND AND PURPOSE: Refractometers have gained a foothold in childhood screening for ophthalmic disorders. Given the results of an ophthalmic follow-up of an extremely preterm Danish cohort, the results of the Retinomax autorefractor were further evaluated. MATERIALS AND METHODS: A nationwide cohort of infants born before gestational age 28 weeks (n=178) and 56 term controls were examined at the age of 4 years. Refraction was given as the cycloplegic Retinomax value. For this study, we analysed the equipment's confidence value on the printout and equipment-induced myopization (as the difference between refraction measured before and after topical cyclopentholate 1%), both items hypothetical with a view to having identified factual ophthalmic deviations. RESULTS: Thirty-two of 42 eyes with visual acuity ≤0.4 had high Retinomax confidence values (8-9); the Retinomax values were also high in 10 of 12 children with strabismus and lack of stereopsis. Low values (1-6) were recorded in 11 single eyes, 5 of which were normal (false positives). Three children already known to have low vision were unable to cooperate. The overall mean value for equipment-induced myopization was 1.9 D (range, 0-6.87 D). Myopization showed no correlation with visual acuity and corneal curvature, and a weak positive correlation with refractive value disappeared when the myopic outliers were excluded. CONCLUSIONS: The hand-held Retinomax seemed to be reliable for assessing refraction in 4-year-old children, provided a cycloplegic agent is applied; if used alone, the Retinomax would have missed several cases of ophthalmic deviation during screening. Equipment-induced myopization was not indicative.

Down's syndrome and early cataract.

AIMS: To estimate the occurrence of early cataract among patients with Down's syndrome and to evaluate the clinical characteristics of the cases. METHODS: Cases with Down's syndrome were ascertained from a cohort of all Danish children between 0 and 17 years of age, who were diagnosed with cataract during the period 1977-2001 (n = 1027). Information on the patients was obtained from the medical records. RESULTS: Of the total of 1027 cases with non-traumatic, non-acquired cataract there were 29 cases (13 males, 16 females) with Down's syndrome (2.8%). This corresponds to an occurrence of early cataract among patients with Down's syndrome of 1.4%; 27 had bilateral cataract and two had unilateral cataract. Half of the patients (n = 14) underwent cataract surgery, of whom two had bilateral primary lens implantation. 10 patients had bilateral cataract observed soon after birth, and five of these underwent cataract surgery within the first 6 months of life. CONCLUSION: The frequency of early cataract among children with Down's syndrome is estimated to be 1.4%, with cataracts requiring surgery during childhood being even rarer. In one third of the 29 cases, bilateral cataract was detected in the neonatal period.

Liposarcoma metastatic to the orbit.

PURPOSE: To describe a patient with liposarcoma metastatic to the left orbit. METHODS: A 72-year-old man was admitted with diplopia and proptosis of the left eye. Previously, a retroperitoneal liposarcoma had been surgically removed followed by postoperative radiation. Visual acuity was normal. There was proptosis of the left eye, increased retrobulbar resistance and reduced mobility. Trans-septal orbital biopsies showed liposarcoma. The patient was initially treated with prednisolone and later received radio- and chemotherapy. Despite treatment he lost vision of the left eye and died a few months later. RESULTS: Histological examination of the orbital tumor revealed a spindle-cell liposarcoma similar to the primary tumor of the retroperitoneum. CONCLUSIONS: Liposarcoma metastatic to the orbit is rare, but should be suspected in a patient with proptosis caused by a space-occupying lesion and a history of liposarcoma.

Interferon-alfa treatment of facial infantile haemangiomas: with emphasis on the sight-threatening varieties. A clinical series.

PURPOSE: To report on the efficacy of systemic Interferon-alfa therapy in controlling the endothelial proliferation in expanding facial haemangiomas of infancy threatening the eye. METHODS: The series comprised 9 subjects with facial haemangiomas of rapid growth within the first few months of life. All haemangiomas had relation to an eyelid and affected the visual access to the eye. At least three tumours were also intraorbital, and in one case the glottis was affected. Interferon-alfa was given subcutaneously in daily doses of 3 mio units/m2. Ophthalmic follow-up including ultrasound evaluation was done, initially with intervals of 4-8 weeks. RESULTS: Interferon-alfa was given over 9-41 weeks (median duration 22 weeks) in cumulated doses of 63-287 million units. Progression was arrested in all and with a subsequent tumour regression considered accelerated as judged from earlier clinical giant haemangioma experience. A control series was not possible. One patient had systemic prednisone added over some weeks. Two infants reacted with a flu-like malaise. A premature infant developed a slight spastic diplegia. CONCLUSIONS: In the 9 infants with growing ophthalmic haemangiomas we recorded a good response to systemic Interferon-alfa administered as daily doses by subcutaneous injection. In general the treatment appeared well tolerated. During clinical follow-up diagnostic ultrasound evaluation (the depth dimension) proved helpful; in particular there was a sparing effect on CT and MRI scans which require general anaesthesia.

Foreign body orbital cyst.

PURPOSE: To present the clinical and histopathological characteristics of a retained orbital foreign body. METHODS: A 34-year-old male was hit in his left eye by a flower stake. At first examination there was a massive haematoma of the left orbit and eyelids, reduced movement of the left eye and a small conjunctival lesion beneath the upper lid. There were no signs of deeper orbital or ocular lesions. During the subsequent months the haematoma disappeared, but increased proptosis and impaired eye movements with diplopia were noticed. One year after the injury, ultrasound and CT-scan revealed an ovoid cystic tumour behind the globe. During surgery the content of the cyst appeared purulent and from the centre of the cyst a foreign body measuring 25 x 13 x 1 mm was removed. RESULTS: Microscopy of the foreign body showed the typical structure of a decidual leaf. The superficial cells were empty, whereas the centrally located cells contained remnants of cytoplasm. Confined to the border between the empty and the filled cells, a band of cells containing groups of gram positive cocci was noted. Staining for immunoglobulins revealed traces of IgG in the superficial empty cells only. CONCLUSION: The high degree of preservation of the leaf and the survival of the cocci may be due to a barrier function of the intact plant cell walls with their high content of cellulose.

Neoplasia versus hyperplasia of the retinal pigment epithelium. A comparison of two cases.

PURPOSE: To present the clinical and histopathological characteristics of two different tumor-like lesions of the retinal pigment epithelium (RPE). METHODS: Two cases of tumor-like lesions of the RPE were identified in the files of the Eye Pathology Institute. The clinical characteristics and the light- and electron microscopical morphology of the lesions were compared and the diagnoses were re-evaluated applying modern immunostainings. RESULTS: Clinically, both adenoma and tumor-like hyperplasia of the RPE may present with prominent retinal feeder arterioles. The lesions are hypofluorescent in the filling phases and have multiple hyperfluorescent zones in the late phase in fluorescein angiography. They show high internal reflectivity by A-scan and appear as solid tumors by B-scan ultrasonography. Histologically, the two presented lesions of the RPE are different. The first is an adenoma of the vacuolated subtype. The other lesion is a hyperplasia of the RPE disclosing a tubular morphology. The pathologically active cells in both cases were positive for the reaction with antibodies against: cytokeratin, NSE, vimentin, S-100, HMB-45, desmin and SMA. However, only the adenoma was sporadic melan-A positive. CONCLUSION: Adenomas and tumor-like hyperplastic lesions of the RPE are very rare lesions. They share many morphological and immunohistological characteristics. Of the presented cases only the RPE adenoma is sporadic melan-A positive.

Myopia profile in Copenhagen medical students 1996-98. Refractive stability over a century is suggested.

PURPOSE: To assess the myopia prevalence and profile in today's Danish medical students and to compare the pattern with previous and contemporary investigations. METHODS: As part of the tutoring in ophthalmology 1996-98 Copenhagen medical students of five successive terms (in their 10th semester, n=294) were interviewed and spot-checked about their refraction. Myopia from -0.5 D was recorded and myopia onset age stated. RESULTS: The age range was 22-41 years, median age 26. Myopia ranging from -0.5 to -8 D was encountered in 147 students (50% of all; females 53.9%, males 45%). In seven the myopia was unilateral. Median values for myopia degree and onset age were -2.5 D and 16 years in female myopes (n=89) which differed significantly from the male values (n=58) of -1.5 D and 18 years. Including the age of 18 as lower cut-off for adult-onset myopia 44.9% of the myopes belonged to this category. CONCLUSIONS: The results are in close agreement with recent Norwegian student investigations, and there is no obvious trend of a worsening of the academic myopia issue in our part of the world. This is in marked contrast to student myopia profiles as reported in Taiwan, Singapore, and Hong Kong where myopia is booming, by number and degree.

Primitive neuroectodermal tumor of the orbit in a 5-year-old girl with microphthalmia.

PURPOSE: To report an orbital, intraconal, primitive neuroectodermal tumor (PNET) in a 5-year-old child with microphthalmia since birth. METHODS: Orbitotomy was performed and a large, polycystic, retroscleral, intraconal tumor was removed and subsequent histological, immunohistochemical and electron-microscopic analyses of the excised mass were performed. RESULTS: The tumor showed characteristic features of peripheral primitive neuroectodermal tumor including pseudorosettes, positive immunohistochemical reactions for the MIC2 gene and synaptophysin and ultrastructural finding of neurosecretory granules. CONCLUSION: This case is the first reported intraconal PNET of the orbit, and the first orbital case that expresses the MIC2 gene. In spite of the aggressive malignant features of peripheral PNET, the orbital variety seems to be the least aggressive since most of the reported patients are still alive.

Retinopathy of prematurity, a decrease in frequency and severity. Trends over 16 years in a Danish county.

PURPOSE: To analyse the trends over 16 years (1982-97) in a Danish county regarding the clinical appearance of retinopathy of prematurity (ROP). METHODS: The data for birth years 1993-97 regarding the regular ophthalmic surveillance for ROP in the region have been analysed. With gestational age (GA) 32 weeks and/or a birthweight (BW) 1750 g as attempted screening limits a total of 177 premature infants were surveyed. Comparison was made with studies of a similar set-up from the preceding four 3-year periods comprising a total of 814 subjects. RESULTS: The number of survivors regarded at a higher risk of developing ROP (GA and BW both <32 weeks/1750 g, n=478) showed an even increase over the five investigation periods. The ROP frequency, however, fell from a level of 39% to 10%, there were fewer with severe sequels to ROP, and the children surveyed 'above limits' eventually avoided ROP. CONCLUSIONS: Using the ROP profile as a yardstick for the quality of the neonatal service given to the most immature newborns over the period 1982-97, the risk limits regarding GA and BW have gone down. More and more of the heavier preterm babies now escape ROP. A significant decrease in frequency of ROP in the more immature groups has been observed, and fewer cases have progressed to visual impairment.

Retinopathy of prematurity in a Copenhagen high-risk sample 1997-98. The allover surveillance for ROP appears more and more complete.

PURPOSE: From two recent materials to describe the present clinical status regarding retinopathy of prematurity in Denmark, and to outline trends over time. METHODS: A) Results of regular ophthalmic surveillance of 201 clinically selected (higher risk of ROP than average) pre-term infants of birth year 1997-98 taken care of in the two greater Copenhagen tertiary neonatal units, in an intended prospective design. Gestational age range was 24-32 weeks at delivery; birth weights 490-2200 g. Median values 28 weeks and 1090 g. B) A brief account of the latest ROP-associated registrations of visual impairment in Danish children aged 0-17 years (n=138). RESULTS: A) ROP was observed in 31.3% (n=201). Retinal cryotherapy was given to eleven 'own' cases and to two from elsewhere (n=13, gestational age at delivery 25-31 weeks). Five had cryotherapy twice. Four of the 13 were later registered for visual impairment. B) Comparing the first and the latest third of the registrations, visual impairment has dropped in frequency and severity over the period from 1981 till now. CONCLUSIONS: Compared to previous data the present clinical profile of ROP in Denmark indicates a relatively lower overall frequency of ROP and a decrease in eventual severe visual impairment. Undoubtedly, the continued refinement of neonatal care has been of relevance, but the definite decline in visual impairment further reflects a more complete ophthalmic surveillance, on a national basis. The advanced cases are generally detected in time and retinal ablation therapy offered.

The acoustic lighthouse effect. An ultrasonic response met in eyes after vitreoretinal surgery with silicone oil.

PURPOSE: To discuss and possibly explain the ultrasonic lighthouse effect, a metaphor suggested for an artifact-like phenomenon sometimes found by B-scan in eyes having had intra-ocular perfluorocarbon and/or silicone oil injected as part of vitreoretinal surgery. METHOD: Analysis of the ultrasonic findings with a view to the specific gravities of the above injected substances. Compared to water, perfluorocarbon has a higher and silicone oil a lower gravity; they are heavy and light, respectively. RESULTS: The elicited lighthouse effect may appear localized or 'mobile'. When evoked only from specific positions on the globe, most likely it is explained by a lens-like accumulation of material trapped at the vitreous base just under the transducer. When mobile it depends on head position; there is a gravitational shift in position of the material, as evident in particular when becoming visible in the anterior chamber. CONCLUSIONS: In case reports in literature slit-lamp verified anterior segment perfluorocarbon remnants have caused acoustic disturbances of a similar nature. In this study we found evidence that also silicone oil can underlie the acoustic lighthouse effect.

Irreversible blindness after amyl nitrite inhalation.

PURPOSE: To present a case of blinding bilateral acute optic nerve disease in a 15-year-old male apparently induced by inhalation of amyl nitrite. METHODS: Sequentially over four days he lost his four altitudinal visual hemifields, leaving atrophic discs and only 2/60 and a small field in the better eye. Scannings by CT and MRI and a full laboratory research gave no specific clue to the pathogenesis. CONCLUSIONS: No similar cases have been described in the more than 100-year history of pharmacological use of amyl nitrite for angina pectoris, and pharmacologically it is hard to point out a rationale behind the sequential visual loss As an alternative to frank toxicity, therefore, we have considered a parainfectious mechanism as related to an influenza-like disease immediately prior to the abuse episode.

Unilateral papilloedema after hepatitis B vaccination in a migraine patient. A case report including forensic aspects.

PURPOSE: To report on a unilateral optic nerve reaction appearing 9-10 hours after vaccination against hepatitis B. METHODS: To describe the case and discuss the underlying mechanisms. There was a scintillating scotoma leaving a permanent inferior notch in the visual field, but central vision was left normal and with only a slight affection of colour perception. Shortly the optic nervehead appeared engorged, then slightly ischaemic, but was hardly left with evidence of atrophy. RESULTS: Immune-based reactions could hardly be held responsible, multiple sclerosis was unlikely, and ultrasonography excluded optic nervehead drusen. An optic nerve migraine mechanism is probable, possibly with the vaccination having acted as the trigger.

[Brain damage in relation to occurrence of retinopathy of prematurity. A regional ophthalmologic follow up of premature infants born 1982-1984]. / Cerebrale skader i relation til forekomst af retinopati hos praemature. En regional oftalmologisk efterundersøgelse af proemature født 1982-1984.

Regular ophthalmic surveillance started in 1982 for all surviving prematures in Frederiksborg County considered at risk of developing retinopathy of prematurity (ROP). During 1982-1984 185 infants of gestational age 25-35 weeks at delivery were examined; blindness eventually appeared in four out of the 28 who showed some stage of ROP. All 28 attended ophthalmic reassessment at age 7-10 years and were compared with 60 of the same cohort without evidence of ROP. At follow-up (n = 88) there was evidence of psychomotor disturbances in 45 children, manifesting as major CNS damage in 21, and minor in 24. With correction for drop-outs the regional frequency of severe CNS damage (mainly cerebral palsy and mental retardation) was 29.6% for gestational ages < 32 weeks at delivery and 24% as calculated for birth weights < 1500 g. An analysis of perinatal factors based mainly on matched pairs did not indicate any definite association with subsequent damage to eye or brain, and statistically there was no association between severity of ROP and CNS impairment. We have no explanation for the high frequency of CNS impairment at follow-up.

[Cryotherapy of retinal prematurity in Denmark 1992-1996]. / Retinal frysebehandling for praematuritetsretinopati i Danmark 1992-1996.

Retinopathy of prematurity (ROP) is a vasoproliferative disorder often met in infants of very preterm delivery. Lower stages usually regress spontaneously, but progression to so-called threshold-ROP should lead to anterior retinal ablation therapy, by cryopencil or laser. Fifty-three Danish infants born 1992-96 were evaluated in the University eye clinic of Rigshospitalet for the disease; 65 eyes of 36 infants had cryotherapy; eight infants developed severe bilateral visual handicap (22%). The reason for not giving cryotherapy to the remaining 17 was: too late referral in five, and ROP below threshold in 12; seven of the 17 later appeared in the (mandatory) national register for childhood visual handicap. Perusing these data the other way round, 15 of the registered 18 cases were thus known to us, while three had escaped referral. With reservation for the small numbers included, the national trend at present is an increasing annual number of subjects receiving retinal ablation therapy for ROP, and a decrease in severe visual impairment due to the disorder. Obviously all preterm infants at risk should be under early regular ophthalmic surveillance for ROP.