Long-term refractive status of preterm infants from singleton and multiple pregnancies.

OBJECTIVE: To study the effect of plurality on refractive status in former preterm infants at age 8-12 years. METHODS: Refraction was compared in singletons and multiples, in very low birth weight infants (VLBW, <1500 g) at age 6 months and 8-12 years. Preterm infants were compared with a group of term infants. RESULTS: Thirty-seven of 104 (36%) VLBW infants were multiples. Comparison of refraction between singletons and multiples revealed no difference at age 6 months, while at age 8-12 years, multiples had significantly more refractive errors (singletons 28% versus multiples 54% p = 0.01), particularly myopia. In preterms, refractive status at age 6 months and multiple birth were significant predictors of refraction at 8-12 years, while birth weight (BW) and retinopathy of prematurity (ROP) were not predictive. Refractive errors were significantly more common in preterms (37%) than in term-born children (14%) (p = 0.0002). Overall, refraction moved from predominantly hyperopic at 6 months to normal or myopic at age 8-12 years in preterm. CONCLUSIONS: Multiple gestation in preterms is associated with increased risk for refractive errors, particularly myopia in childhood. Refraction in preterms during childhood progresses from hyperopia to myopia. Former preterms have more refractive errors than children born at term-born children.

Ophthalmological findings in Joubert syndrome.

PURPOSE: Joubert syndrome (JS) is an autosomal-recessive inherited complex malformation of the midbrain-hindbrain. It has been associated with ocular and oculomotor abnormalities. The aim of our study was to extend the ophthalmic knowledge in JS and to add new findings. METHODS: In a retrospective study, 10 consecutive patients, who met the revised diagnostic criteria of JS were included. Mutation analysis was carried out in all the cases. Each patient underwent a comprehensive neuro-ophthalmological examination. RESULTS: Bilateral drusen of the optic disc were found in two patients. Four patients showed bilateral morphological and functional signs of retinal dystrophy (CEP290 mutation in two cases and AHI1 mutation in one case). In nine patients performance during smooth pursuit, saccades, and vestibulo-ocular reflex (VOR) cancellation was poor. CONCLUSIONS: To the best of our knowledge, the association of optic disc drusen with JS has not yet been described. In support of the earlier findings, decreased smooth pursuit and VOR cancellation, as well as partial-to-complete oculomotor apraxia seem to be the key oculomotor features of JS. Genotype-phenotype correlations showed the predictive value of CEP290 and AHI1 mutations for retinal involvement.

Selective fovea-related deprived activation in retinotopic and high-order visual cortex of human amblyopes.

Amblyopia is a visual disorder starting at early childhood and characterized by reduced visual acuity not of optical origin or due to any eye disease. One expression of such an anomalous early visual experience is abnormal foveal vision. In a previous fMRI study, faces that were presented to amblyopic eyes evoked little response compared to houses in high-order visual areas. Patients also demonstrated reduced recognition of facial expression, raising the possibility that these face-selective abnormalities are related to foveal vision deficit. Whether this deficit originates in low-level processing or is mediated by compromised activation in high-order visual areas is unresolved. In the present functional magnetic resonance imaging (fMRI) study, we explored the impact of amblyopia on the representation of object images presented in foveally biased central versus peripheral retinotopic eccentricities through manipulation of object size. Small and large pictures were correlated to visual acuities of 6/6 and 6/60, respectively. In low-level visual areas, the amblyopic eye showed significantly reduced activation for centrally placed, small pictures than the sound eye, while activation to large pictures was only slightly reduced. Similarly, in high-order visual areas, the amblyopic eye showed marked reduction in activation in the fusiform gyrus, with normal activation in the collateral sulcus. The center/periphery size-related amblyopic outcomes of this study support a "bottom-up" nature of the center-periphery effect observed in high-order visual areas. Taken together, these findings point to the regional extent and functional selectivity of fovea-related cortical reorganization that is related to abnormal visual development of one eye.

Area-specific amblyopic effects in human occipitotemporal object representations.

The role of early visual experience in the establishment of human high-order visual areas is poorly understood. Here we investigated this issue using human amblyopia--a developmental visual disorder, which manifests a central vision (acuity) deficit. Previous fMRI studies of amblyopes have described abnormal functional activations in early retinotopic areas. Here we report the surprising finding of a selective object-related abnormality in high-order occipitotemporal cortex. Specifically, we found that face-related cortical areas show a severe disconnection from the amblyopic eye, while building-related regions remain essentially normal. The selectivity of the deficit highlights the differential computations performed in the different object-related areas and is compatible with the suggested association of face regions with analysis of fine detail.

Long-term follow-up of occlusion therapy in amblyopia.

OBJECTIVE: To determine whether the results of occlusion therapy for amblyopia are maintained into adulthood. DESIGN: Prospective, observational case series. PARTICIPANTS: Fifty-four patients, who were successfully treated in childhood for unilateral amblyopia by occlusion, were followed up to the age of 9 years, were evaluated in 1984 for long-term results 6.4 years on average after cessation of treatment, and accepted our invitation for reevaluation in 1999. METHODS: All patients were given a complete eye examination. The visual acuity (VA) was measured. RESULTS: The average period of follow-up was 21.5 years (range, 17.2-25.1 years). The mean age at the most recent examination was 29.0 +/- 2.1 years (range, 25.1-34). At this examination, a best-corrected visual acuity (BCVA) of 20/40 or better was achieved by 72.3% of the patients. The mean BCVA was 20/35 (20/25-20/70) at the end of occlusion therapy, 20/45 (20/20-20/300) in 1984, and 20/34 (20/15-20/100) at the present examination. Relative to the results at the end of therapy, BCVA at the present examination was maintained or improved in 66.7% of the patients. Relative to 1984, the BCVA in 1999 was maintained or improved in 87% of the patients. MAIN OUTCOME MEASURE: Visual acuity. CONCLUSION: Comparative evaluation of BCVA at a long-term follow-up examination, performed 21.5 years on average after cessation of occlusion therapy, showed that VA was maintained or improved in two thirds of patients who had been successfully treated by occlusion for unilateral amblyopia in childhood.

Postpartum optic neuritis: etiologic and pathophysiologic considerations.

The clinical course of four patients with visual loss in the postpartum period due to acute optic neuritis is described. Factors that disclosed the underlying etiology and expression of disease are discussed. The clinical records of four women examined and managed for visual loss after uncomplicated pregnancies and term deliveries were reviewed. Neurodiagnostic examination, treatment modalities, and outcomes were assessed. These four women with varied and confounding medical histories, all with optic neuropathy, eventually were demonstrated to harbor demyelinating disease. Although visual loss in the postpartum period evokes differential diagnostic considerations, the authors' experience suggests that puerperal immune-mediated changes are responsible for activation of optic neuritis associated with relapsing multiple sclerosis.

Optic nerve head blood flow measurements in non-arteritic anterior ischaemic optic neuropathy.

PURPOSE: To determine whether a decrease in blood flow, measured by the Heidelberg retiinal flowmeter, can characterise a disc at risk and predict the occurrence of non-arteritic anterior ischaemic optic neuropathy (NAION). METHODS: Blood flow, volume and velocity were measured in the optic nerve heads of 14 unaffected fellow eyes of patients with unilateral NAION, and compared with those of the affected eyes and of the eyes of 7 age-matched healthy controls. RESULTS: The affected eyes were found to differ from the unaffected eyes in all three haemodynamic parameters. Flow (measured in arbitrary units) is the most important parameter to be taken into consideration. Blood flow was significantly lower in affected than in unaffected eyes (upper rim, p < 0.05; lower rim, p < 0.025). It was also significantly lower in the unaffected eyes than in the healthy control eyes (p < 0.005, upper and lower rims), and in the affected eyes than in the control eyes (p < 0.00005, upper and lower rims). CONCLUSION: Decreased blood flow in the optic nerve head may indicate a risk for NAION and be considered a characteristic of the disc at risk.

Poststreptococcal uveitis.

PURPOSE: To report that uveitis may be a manifestation of poststreptococcal syndrome. METHODS: Case report. Documented attacks of bilateral uveitis were clearly associated with streptococcal infection. RESULTS: Group A streptococcal infection was evident in all bilateral uveitis attacks, which were treated with local or systemic corticosteroids and penicillin. The frequency and severity of the attacks were reduced by penicillin prophylaxis and tonsillectomy. CONCLUSIONS: Uveitis should be included as a possible manifestation of poststreptococcal syndrome. If coexisting streptococcal infection is demonstrated, penicillin prophylaxis should be considered.

The course of age-related macular degeneration following bilateral cataract surgery.

BACKGROUND AND OBJECTIVE: Progression of age-related macular degeneration (AMD) following cataract surgery has been described. The aim of this study was to investigate whether an uneventful postoperative maculopathic course in one eye (the first eye) may predict a similar prognosis for the fellow eye (the second eye). PATIENTS AND METHODS: Thirty-three patients with bilateral, similar, early AMD (defined by the presence of drusen and/or pigmentary abnormalities on fundal examinations and by the absence of late leakage as documented by fluorescein angiography) who had undergone unilateral cataract surgery, had had a stable postoperative maculopathic course following the first operation, and were scheduled for cataract surgery in the second eye were prospectively observed for at least 1 year after the second operation. The course of maculopathy of the second eye was compared with that of the first eye during the follow-up period of the second eye. RESULTS: A total of 9 of the second eyes (27.2%) showed progression to wet AMD. Of these, 1 patient (3%) had progression in both eyes and 8 patients (24.2%) had progression in the second eye only. The conditions of 2 of the first operated on eyes (6.1%) deteriorated (P < .05). Hypertension (P < .05), soft drusen (P < .01), and Nd:YAG laser capsulotomy (P < .05) were risk factors for development of wet AMD. CONCLUSION: In this study, 24.2% of the patients with early AMD who underwent bilateral cataract surgery and had an uneventful maculopathic course in the first eye had wet AMD in the second eye. Thus, patients with early AMD and soft drusen undergoing bilateral cataract surgery should be monitored for early detection of progression of maculopathy. Further prospective studies are needed to determine the course of maculopathy following cataract surgery.

Progression of nonischemic central retinal vein occlusion.

Progression of nonischemic central retinal vein occlusion (CRVO) to ischemic CRVO occurs in up to 5-20% of patients with nonischemic CRVO. Eyes presenting with nonischemic CRVO and exhibiting an increase in intraretinal hemorrhages together with angiographic findings of aggravation of venule wall staining were assumed to be showing early signs of progression. Eight eyes of 7 patients were followed prospectively and when they exhibited signs of progression they were assigned to either follow-up or early laser treatment. Four eyes received grid and early panretinal laser photocoagulation. Three of them showed regression of retinopathy and 1 eye progressed to ischemic CRVO. The other 4 eyes did not receive early panretinal photocoagulation; 2 of them received no laser treatment at all and the other 2 received only grid laser photocoagulation. All of these 4 eyes progressed to ischemic CRVO.

Neurosarcoidosis presenting as an intracranial mass in childhood.

A 13-year-old boy presented with bilateral panuveitis and a superior oblique palsy. Exhaustive laboratory workup was unremarkable, but magnetic resonance imaging (MRI) revealed an enhancing pontine mass. The mass was resected, and histopathology revealed a necrotizing granuloma. Although rare, particularly in the pediatric population, the combination of panuveitis and an intracranial mass likely represents sarcoidosis. Necrosis, although also rare, may similarly be seen in neurosarcoidosis.

Prophylactic laser photocoagulation in Stickler syndrome.

The ocular manifestations in a family with Stickler syndrome and the results of laser photocoagulation as preventive treatment for retinal detachment are described. Forty-two family members with Stickler syndrome were retrospectively reviewed; 22 had ocular abnormalities, 22 had myopia and 16 had high myopia. Ten patients had developed retinal detachment and 9 of them were blind in one or both eyes because of irreparable detachment. Only 2 eyes had been operated on successfully. Ten eyes were lasered prophylactically. In eyes with extensive vitreoretinopathy laser burns were applied 360 degrees around the peripheral retina at the border between the pathological and normal retina. Eyes with isolated lesions received focal treatment around the pathological areas. Four eyes received 360 degrees laser photocoagulation and 6 eyes received focal treatment. Of the treated cases, 9 retinas remained attached for a follow-up period ranging from 1 to 15 years. One patient was lost to follow-up, and 5 years later developed retinal detachment in one eye from a new non-lasered lesion. In conclusion, in this particular family the incidence of retinal detachment was significantly higher in non-lasered eyes than in lasered eyes (p < 0.025).

Results of treatment with topical mitomycin C 0.02% following excision of primary pterygium.

AIMS: The effectiveness of instillation of mitomycin C eyedrops on the recurrence rate of pterygium was assessed in patients undergoing primary pterygium surgery. Any side effects were also noted. METHODS: Primary pterygia in 38 consecutive patients were surgically excised during July to December 1992. After surgery, mitomycin C 0.02% eyedrops twice daily for 5 days as well as dexamethasone 0.1% four times tapered for the next 6 weeks were instilled. Postoperative follow up ranged from 6 to 11 months. RESULTS: In one patient the pterygium recurred after 3 months (recurrence rate 2.6%). The side effects encountered were: avascularised sclera in 13 cases between 1-10 months postoperatively; ocular discomfort and lacrimation in five cases; superficial punctate keratitis during the first month in three cases; pyogenic granuloma in two cases. In one patient steroid induced increased intraocular pressure was found 4 weeks after surgery. The adverse side effects were all mild, self limiting, and easily treated. CONCLUSION: This study suggests that postoperative instillation of mitomycin C 0.02% eyedrops twice daily for 5 days following excision of primary pterygium is an effective and safe treatment to obviate pterygium recurrence.

Signaling mechanisms controlled by melanocortins in melanoma, lacrimal, and brain astroglial cells.

Melanocortins appear to be involved as regulators in an ever growing number of physiological processes in cells and tissues of diverse functions. While such trends are apparent also in the case of other peptide hormones, it appears that melanocortin receptors can be regarded as unique among G-protein-linked receptors due to their special need for extracellular Ca2+ which may relate to some, yet undetermined selectivity of their actions. The physiological role that Ca2+ may be playing and the diverse signaling mechanisms regulated, as well as the nature of the cell-specific responses elicited in melanocortin-sensitive cells/tissues, have yet to be elucidated. Likewise, it will be of interest to establish the relationship of melanocortins to processes like growth and differentiation of cells, as well as to higher, more complex processes such as those regulated in the CNS.

Cystoid macular oedema following cataract extraction in patients with diabetes.

The course of cystoid macular oedema (CMO) following extracapsular cataract extraction with posterior chamber intraocular lens implantation was prospectively studied in 44 eyes of 44 consecutive diabetic patients without preoperative CMO. In 50% of eyes CMO was observed 6 weeks after surgery and in 25% was still present at 1 year. The preoperative presence of diabetic retinopathy significantly affected the postoperative onset and persistence of CMO. CMO occurred postoperatively in only 32% of eyes without pre-existing diabetic retinopathy and in 81% of eyes with pre-existing diabetic retinopathy (p < 0.05). CMO persisted at 1 year after surgery in only 7% of eyes without pre-existing diabetic retinopathy and in 56% of eyes in which diabetic retinopathy persisted (p < 0.01). Angiographic CMO (that is, detectable only on fluorescein angiography) was more common than clinical CMO (detectable on ophthalmoscopic examination as well) in eyes with no pre-existing diabetic retinopathy, whereas clinical CMO was seen more often than angiographic CMO when diabetic retinopathy was present preoperatively (p < 0.01). The course and final visual outcome of angiographic CMO were more favourable than in clinical CMO. Final visual acuity of at least 6/12 was achieved in 86% of eyes with angiographic CMO and in only 33% of eyes with clinical CMO. On the basis of the above findings we believe that cataract extraction should not be recommended for eyes with pre-existing diabetic retinopathy until the vision has deteriorated to at least 6/30-6/60.

The course of diabetic retinopathy following cataract surgery in eyes previously treated by laser photocoagulation.

The course of diabetic retinopathy following extracapsular cataract extraction with posterior chamber lens implantation in eyes previously treated by laser photocoagulation for diabetic retinopathy was retrospectively studied in 33 eyes (33 patients). In 20 eyes (61%) there was no change in the retinal status postoperatively. In 13 (39%) there was postoperative progression of diabetic retinopathy compared with the fellow non-operated eye, in which progression occurred in nine eyes (27%). The severity of the preoperative status affected the incidence of progression. Four eyes (12%) developed complications of diabetic retinopathy--that is, rubeosis iridis and vitreous haemorrhage--which regressed after lasering. Cystoid macular oedema developed in 13 eyes (39%) and its incidence varied according to the postoperative course of diabetic retinopathy. The majority of the eyes showed a postoperative improvement in vision.

Subconjunctival injection of 5-fluorouracil following trabeculectomy for congenital and infantile glaucoma.

Trabeculectomy and subsequent subconjunctival injections of 5-fluorouracil (5-FU) were performed in four eyes (two children) with congenital glaucoma. Each of these eyes had previously undergone either goniotomy, trabeculotomy, or both; these procedures, however, had failed to control intraocular pressure (IOP) and progressive optic nerve damage. Sixteen and a half months (+/- 1.5 months) after the trabeculectomy and 5-FU treatments, the IOP in these eyes was in the low teens and there was no evidence of further optic-nerve or visual-field deterioration. Although trabeculectomy has been shown to be unsuccessful in managing congenital glaucoma, when it is done with the adjunct of subconjunctival injections of 5-FU, it may be advisable in these cases after previous surgery has failed.

Variability of Stickler syndrome.

Stickler syndrome is a dominantly inherited disorder characterized by ocular and nonocular manifestations. The phenotype of the affected patients is known to be variable. Our study of 3 families and a review of the literature show that the variability is mostly interfamilial while in each family less variability is present. In one family all the patients had high myopia and most developed a retinal detachment at a young age. In the second family the major symptoms were cleft palate and characteristic facial changes in presence of mild ocular changes. In the third family, all patients had a marfanoid habitus, high myopia, and mental retardation. Interfamilial variability coupled with intrafamilial similarities in clinical manifestation may indicate that the so-called Stickler syndrome represents in fact a phenotype and not a single genetic entity.

Laser treatment of eye disorders in children.

The increasing range of indications for laser treatment and the development of new modalities of lasering have led to a growing tendency to extend this type of treatment to the pediatric population. Problems of compliance in this age group often necessitate the use of general anesthesia. This report describes the operative technique used in 11 children (13 eyes) under the age of 13 years, all of whom underwent laser treatment under general anesthesia for a variety of ocular conditions. Argon or krypton laser photocoagulation was performed in eight children (10 eyes), seven of whom were treated for various retinal pathologies and one for an iris cyst. The other three children underwent Nd:YAG posterior capsulotomy for secondary cataract.