title: Ocular manifestations in egyptian children and young adults with sickle cell disease.
abstract: In sickle cell disease (SCD), ocular lesions result from stasis and occlusion of small eye vessels by sickled erythrocytes. Vaso-occlusive disease of the retina can be responsible for nonproliferative (NPR) and proliferative retinopathy (PR). Patients are often asymptomatic until serious complications arise as, vitreous hemorrhage and retinal detachment. This work aimed to study the frequency and pattern of ocular manifestations in Egyptian children and young adults with SCD. In this cross-sectional study, 40 steady state patients (80 eyes) aged 2-28 years (30 children and 10 young adults) with established diagnosis of SCD (26 with homozygous SS and 14 with S/β thalassemia underwent complete ophthalmic examination with dilated fundoscopy. Fluorescein angiography was performed for patients ≥12 years old. The overall frequency of retinal lesions was 47.5 % (46.2 and 50 % of SS and S/β patients respectively). PR and NPR were evident in 32.5 and 27.5 % of all enrolled patients respectively (five patients having both). Peripheral retinal occlusion was a frequent ocular finding in both groups; the youngest patient showing PR was 15 years old. Older age, longer disease duration and splenectomy were significantly more prevalent among patients with PR. Despite lack of visual symptoms, children and young adults are at risk of PR. Frequency of retinal lesions was comparable in SS and S/β patients. Periodic ophthalmologic examination starting at the age of 12 years is recommended for timely-identification of retinal lesions thus minimizing the risk of sight threatening retinopathy.
authors.affiliation: Pediatric Hematology & BMT Unit, Department of Pediatrics, Faculty of Medicine, Cairo University Children Hospital, Cairo, Egypt ; 28 Mourad St., Giza, 12411 Egypt.
authors.forename: Mona Kamal
authors.initials: MK
authors.lastname: El-Ghamrawy
authors.affiliation: Pediatric Department, Institute of Ophthalmologic Research, Cairo, Egypt.
authors.forename: Hanan F
authors.initials: HF
authors.lastname: El Behairy
authors.affiliation: Pediatric Department, Institute of Ophthalmologic Research, Cairo, Egypt.
authors.forename: Amal
authors.initials: A
authors.lastname: El Menshawy
authors.affiliation: Pediatric Department, Institute of Ophthalmologic Research, Cairo, Egypt.
authors.forename: Seham A
authors.initials: SA
authors.lastname: Awad
authors.affiliation: Ophthalmology Department, Institute of Ophthalmologic Research, Cairo, Egypt.
authors.forename: Ahmed
authors.initials: A
authors.lastname: Ismail
authors.affiliation: Department of Community, Environmental and Occupational Medicine, Ain Shams University, Cairo Governorate, Egypt.
authors.forename: Mohamed Salah
authors.initials: MS
authors.lastname: Gabal
keywords: Children; Ocular; Retinopathy; Sickle cell disease