Sickle cell disease includes a group of inherited haemoglobinopathies affecting multiple organs including the eyes. Some people with the disease develop ocular manifestations due to vaso-occlusion. Vision-threatening complications of
sickle cell disease are mainly due to proliferative sickle retinopathy which is characterized by proliferation of new blood vessels.
Laser photocoagulation is widely applicable in proliferative retinopathies such as proliferative sickle retinopathy and proliferative
diabetic retinopathy. It is important to evaluate the efficacy and safety of
laser photocoagulation in the treatment of proliferative sickle retinopathy to prevent sight-threatening complications.
OBJECTIVES: Two authors independently assessed trial eligibility, the risk of bias of the included trials and extracted and analysed data. We contacted the trial authors for additional information.
MAIN RESULTS: Two trials (341 eyes of 238 children and adults) were included comparing efficacy and safety of
laser photocoagulation to no
therapy in people with proliferative sickle retinopathy. There were 121 males and 117 females with an age range from 13 to 67 years. The
laser photocoagulation technique used was different in the two trials; one single-centre trial employed sectoral scatter
laser photocoagulation using an
argon laser; and the second, two-centre trial, employed feeder vessel coagulation using
argon laser in one centre and
xenon arc in the second centre. The follow-up period ranged from a mean of 21 to 32 months in one trial and 42 to 47 months in the second. Both trials were at risk of selection bias (random sequence generation) because of the randomisation method employed for participants with bilateral disease. One study was considered to be at risk of reporting bias.Using sectoral scatter
laser photocoagulation, one trial (174 eyes) reported that complete regression of proliferative sickle retinopathy was seen in 30.2% in the
laser group and 22.4% in the control group (no difference between groups). The same trial reported the development of new proliferative sickle retinopathy in 34.3% of
laser-treated eyes and in 41.3% of eyes given no treatment; again, there was no difference between treatment groups. The second trial, using feeder vessel coagulation, did not present full data for either treatment group for these outcomes.There was evidence from both trials (341 eyes) that
laser photocoagulation using scatter
laser or feeder vessel coagulation may prevent the loss of vision in eyes with proliferative sickle retinopathy (at median follow up of 21 to 47 months). Data from both trials indicated that
laser treatment prevented the occurrence of vitreous haemorrhage with both
argon and
xenon laser; with the protective effect being greater with feeder vessel
laser treatment compared to scatter
photocoagulation.Regarding adverse effects, the incidence of
retinal tear was minimal, with only one event reported. Combined data from both trials were available for 341 eyes; there was no difference between the
laser and control arms for
retinal detachment. In relation to
choroidal neovascularization, treatment with
xenon arc was found to be associated with a significantly higher risk, but visual loss related to this complication is uncommon with long-term follow up of three years or more.Data regarding quality of life and other adverse effects were not reported in the included trials.
AUTHORS' CONCLUSIONS: Our conclusions are based on the data from two trials conducted over 20 years ago. In the absence of further evidence,
laser treatment for
sickle cell disease-related retinopathy should be considered as a one of therapeutic options for preventing visual loss and vitreous haemorrhage. However, it does not appear to have a significant different effect on other clinical outcomes such as regression of proliferative sickle retinopathy and development of new ones. No evidence is available assessing efficacy in relation to patient-important outcomes (such as quality of life or the loss of a driving licence). There is limited evidence on safety, overall, scatter
argon laser photocoagulation is superior in terms of adverse effects, although feeder vessel coagulation has a better effect in preventing vitreous haemorrhage. Further research is needed to examine the safety of
laser treatment compared to other interventions such as
intravitreal injection of anti-
vascular endothelial growth factors. In addition, patient-important outcomes as well as cost-effectiveness should be addressed.