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Salmon patch maculopathy: An amblyogenic complication of pediatric sickle cell retinopathy
Nitya Rao1, Therese McKnight2, Cynthia Norris2
1Scheie Eye Institute, University of Pennsylvania, Philadelphia, PA, USA.
Insights
A pediatric case of sickle cell retinopathy presented with a foveal hemorrhage, leading to amblyopia. Early vision screening is crucial for children with sickle cell disease (SCD) to detect and manage such complications.
Area of Science:
- Ophthalmology
- Pediatrics
- Hematology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder associated with numerous ocular complications.
- Non-proliferative sickle cell retinopathy can manifest in various ways, including retinal hemorrhages and macular changes.
Observation:
- A five-year-old boy with HbSS presented with a large sub-internal limiting membrane hemorrhage obscuring the fovea.
- Peripheral salmon patches and temporal inner retinal macular thinning were also observed via OCT.
- The hemorrhage showed rapid improvement on serial imaging.
Findings:
- The case highlights an unusual amblyogenic presentation of sickle cell retinopathy due to a foveal salmon patch.
- Despite hemorrhage resolution, persistent amblyopia was noted in the pediatric patient.
Implications:
- This case underscores the potential for vision-threatening complications in pediatric SCD patients.
- Emphasizes the critical importance of routine vision screenings in early detection and intervention for sickle cell retinopathy.
Purpose:
To report a case of a large foveal sub-internal limiting membrane hemorrhage from sickle cell retinopathy in a pediatric patient.
Observations:
A five-year-old boy with sickle cell disease (SCD) type SS (HbSS) and numerous complications was referred after a failed vision screening and was found to have a large yellow subacute sub-internal limiting membrane hemorrhage overlying the fovea in his right eye. There were several other peripheral salmon patches noted. Optical coherence tomography (OCT) revealed temporal inner retinal macular thinning in both eyes. Serial imaging showed rapid improvement over time of the hemorrhages, though amblyopia persisted.
Conclusions And Importance:
We describe an unusual amblyogenic presentation of non-proliferative sickle cell retinopathy in five-year-old patient with HbSS due to a foveal salmon patch. Numerous vision-threatening complications are possible in SCD, highlighting the need for early vision screening.
