Incidence, distribution, and duration of birth-related retinal hemorrhages: a prospective study.

BACKGROUND: Retinal hemorrhages secondary to birth trauma are part of the differential diagnosis of intraocular hemorrhages seen in the setting of Shaken baby syndrome in very young infants. This prospective study aimed to document the morphology, distribution and, most importantly, the natural history of these hemorrhages using digital imaging. SUBJECTS AND METHODS: Infants were recruited as soon after birth as possible and examined by indirect ophthalmoscopy. Retinal hemorrhages were photographed using the RetCam 120. Birth history was documented from the medical notes. Infants were reexamined and photographed until hemorrhages had resolved. RESULTS: Data were analyzed for a total of 53 neonates. The number of infants with retinal hemorrhage was 18 (34%). The incidence in relation to mode of delivery was as follows: vacuum delivery, 77.8%; normal vaginal delivery, 30.4%; cesarean section, 8.3%; forceps delivery, 30.3%. All hemorrhages were intraretinal and in all but two infants hemorrhages had resolved by 16 days. In two subjects hemorrhages were still present at 31 and 58 days, respectively. Both these infants were delivered by vacuum delivery. CONCLUSIONS: The RetCam 120 provides excellent documentation of retinal hemorrhages and their natural history. We have demonstrated hemorrhages still present at 58 days in a child born by vacuum delivery and this may have important implications for consideration in the differential diagnosis of Shaken baby syndrome.

Foreign body episcleral granulomas complicating intravitreal silicone oil tamponade: a clinicopathological study.

PURPOSE: To report two patients with lipid granulomas of the episclera complicating vitrectomy and silicone oil tamponade. DESIGN: Two observational case reports. INTERVENTION: Patient 1, a 41-year-old woman, underwent vitrectomy with silicone oil tamponade for proliferative diabetic retinopathy. Four weeks later, she sought treatment for inflamed episcleral nodules adjacent to one of the sclerostomy sites. The oil was removed and the episcleral nodules were excised. Patient 2, a 33-year-old man, underwent vitrectomy and silicone oil tamponade for tractional retinal detachment. He experienced a painful blind eye with episcleral nodule that required enucleation. MAIN OUTCOME MEASURES: On histopathological analysis, both specimens demonstrated episcleral granulomas caused by silicone oil. CONCLUSIONS: Episcleral nodules adjacent to vitrectomy entry sites with silicone oil tamponade may represent lipid granulomas, probably caused by silicone oil leakage from scleral entry ports.

New classification of peripheral retinal vascular changes in sickle cell disease

The systemic complications of homozygous sickle cell disease (SS) are more severe than in sickle cell haemoglobin C (SC) disease, and yet visual loss due to proliferative retinopathy is more common in the latter. This anomaly is unexplained. It is believed that proliferative disease occurs in response to closure of the peripheral retinal vasculature, yet a systematic longitudinal survey of the peripheral retinal vascular bed has not been undertaken. In the Jamaica Sickle Cohort study all subjects are scheduled to receive annual ocular examination and fluorescein angiography. The results have now been analysed in subjects with SS and SC disease using a new classificaton system based on a comparison of the peripheral retinal vascular bed with that recorded in the cohort with normal haemoglobin (AA) genotype. The vascular patterns could be classified as qualitatively normal (type I) or abnormal (type II). An abnormal vascular pattern was identified more commonly with age, in a significantly larger proportion of subjects with SC than SS disease, and was associated with the development of proliferative disease. In order to establish the dynamics of change, the angiograms were analysed in the 18 subjects (24 eyes) who developed proliferative disease. It is shown that qualitatively normal vascular pattern may be retained despited loss of capillary bed and posterior displacement of the vascular border. A border which is qualitatively abnormal does not revert to normal, and once abnormal, continuous evolution may occur before development of proliferative lesions. The peripheral border of the retinal vasculature was too peripheral to photographed in 13 of the 24 eyes before it becoming qualitatively abnormal. It is concluded that a normal border, if posterior, results from gradual modification of the capillary bed and indicated low risk of proliferative disease. A qualitatively abnormal vascular border occurs as a radical alteration of retinal perfusion in subjects in whom little modification of the vascular bed occurred before the event, and signal risk of proliferative disease. This classification system is useful in identifying the likelihood of threat to vision in young Jamaicans with sickle cell disease, and the higher frequency of proliferative retinopathy in SC can be explained by the higher prevalence of a qualitatively abnormal peripheral retinal vasculture (AU)

Peripheral retinal vasculature in normal Jamaican children

A prospective study of the peripheral retinal vasculature in a Jamaican cohort of subjects with sickle cell disease has been in progress over a period of 12 years using flourescein angiograpphy. Various vascular patterns were identified but their significance was unclear since no comparable records were available in subjects of a similar age with normal (AA) haemoglobin genotype. Fluorescein retinal angioscopy and angiography have been performed in 76 heamoglobin AA controls participating in the cohort study. The peripheral retinal capillary bed could be seen and photographed in a limited portion of the temporal peripheral fundus in a majority of this group, and there was considerable variation in the vascular pattern which could be characterised. These observations allow deviations from normal to be identified in the retinal vasculature in subjects with sickle cell disease (AU)

Sickle cell retinopathy in Jamaican children: further observations from a cohort study

Serial retinal examinations were performed in children aged 5 years and older and fluorescein angiography/angioscopy in children 6 years and older participating in a cohort study of sickle cell disease. There were 1229 patient years of observation among 389 children aged 5 - 13 years. Peripheral retinal vessel closure was present in approximately 50 percent of children with SS and SC genotypes at age 6 years and increased to affect 90 percent of children by age 12 years. A matched pair analysis, comparing groups with minimal and complete closure, indicated that complete closure was associated with significantly lower total haemoglobin and fetal haemoglobin levels and significantly lower weight in SS disease, whereas in SC disease the risk factors appeared to be high mean cell volume and low platelet count. Proliferative retinopathy was rare, occurring only once in an 8-year-old boy with SC disease, despite 592 patient years of observation in children over this age. (AU)

Recurrent visual loss in homozygous sickle cell disease

In sickle cell retinopathy vascular involvement is most frequently recognised at the retinal periphery but obstruction of perimacular arterioles and of major retinal vessels may also occur. This report describes a patient with homozygous sickle cell (SS) disease with recurrent occlusion of major retinal vessels associated with recurring transient impairment of visual function. (Summary)

Retinal changes in sickle cell/hereditary persistence of fetal haemoglobin syndrome

We describe for the first time retinal changes in sickle cell/hereditary persistence of fetal haemoglobin syndrome, which is a rare and benign disorder. The changes are qualitively similar to retinal disease seen with sickle haemoglobin and sickle C haemoglobin, but are mild (AU)

Sickle cell retinopathy in Jamaican children: a search for prognostic factors

Children with homozygous sickle cell (SS) disease and with sickle cell-haemoglobin C (SC) disease, aged 6 1/2 to 8 1/2 years, were examined by fluorescein angiography/angioscopy to determine the presence of retinal nonperfusion. The haematological and clinical features of children with and without nonperfusion were compared. Retinal vessel closure was significantly correlated with low total haemoglobin, and high fetal haemoglobin, reticulocyte, and irreversibly sickled cell counts in SS disease, and with high reticulocyte count in SC disease. No relationships were apparent between vessel closure and other haematologial indices or clinical events in either genotype (AU)

Sickle cell retinopathy in young children in Jamaica

Ophthalmological examinations were performed on 59 of the 74 (80 percent) children with homozygous sickle cell (SS) disease and on 37 of the 54 (69 percent) children with sickle cell-haemoglobin C(SC) disease, aged 5-7.5 years, within the cohort study of sickle cell disease. Arteriolar sheathing was the commonest retinal vessel abnormality, occurring in 30/59 (51 percent) SS children and in 11/37 (30 percent) SC children. Peripheral arteriolar closure was observed in 14 (24 percent) SS children and in 6 (16 percent) SC children. Arteriovenous anastomoses were seen in 3 children, but proliferative retinopathy was not identified. Capillary changes often occurred in patients without confluent closure, suggesting that complex remodelling of the capillary bed may precede retinal nonperfusion. Discrete retinal patches similar to schisis cavities resulting from intraretinal haemorrhages were found in 22 (37 percent) SS children and in 9 (24 percent) SC children, but haemorrhages were observed in only 2 patients (1 SS, 1SC). Vitreous opacities were common and were generally associated with retinal vessel disease. Retinal changes were consistently more common in children with SS disease, though the differences failed to reach statistical significance. The prevalence of peripheral vascular closure and retinal patches showed a significant upward trend with age. These observations contrast with the greater prevalence of proliferative retinopathy characterising SC disease in adults.(AU)