Collaborative Case #006: Red Tied
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Case Presentation
An 11-year-old Black female presented to our office for evaluation of traction of the macula OS. Her medical history was remarkable for sickle cell at birth, and both parents had sickle cell trait. The patient’s BCVA was 20/20 OD, 20/30+ OS. Preliminaries, IOP and slit-lamp examination were all unremarkable, as was a dilated fundus examination OD. However, dilated fundus examination OS revealed the presence of a thick, fibrotic tissue in the macula that radiated towards the optic nerve (Figure 1).
The supero-temporal peripheral view OS also revealed neovascularization associated with a preretinal hemorrhage and tortuous arteriovenous anastomosis (Figure 2). Regressed fibrovascular areas were also denoted OS.
Spectral domain OCT of the patient’s right eye revealed mild temporal retinal thinning (Figure 3). Left eye SD-OCT revealed increased retinal thickness with associated traction, correlating to the fibrotic macular tissue (Figure 3). Ultra-widefield fluorescein angiography (UWFA) of her right eye revealed areas of nonperfusion temporally and inferotemporal (Figure 4).
UWFA of the left eye reveled superior temporal peripheral leakage as well as areas of nonperfusion temporally and inferotemporally (Figure 4).
A decision was made to closely observe the patient for signs of potential regression. She was also asked to continue care with her pediatrician. At the 2-month follow-up visit, the neovascularization OS, as well as the macular fibrotic tissue had shown extensive regression with VA improvement to 20/20 (Figure 5), as well as retinal OCT showing normalization (Figure 6).
A Crash Course on Sickle Cell Disease
In the United States, sickle cell disease (SCD) has a predilection for individuals of African American descent. It is a hereditary group of hemoglobinopathies characterized by sickling hemoglobin, which leads to a decrease in oxygen and blood flow, as well as a disruption in the normal blood flow. Under certain conditions, such as acidosis or hypoxia, the anomalous hemoglobin results in hemolysis and vaso-occlusive events, causing a host of ocular and systemic manifestations linked to specific genetic variants. SCD is comprised of four genetic variants: sickle cell anemia (SS), which has the highest systemic complications; sickle cell C disease (SC), which carries the highest risk towards ocular complications; sickle trait (AS), which is often not associated with ocular manifestations, and sickle cell Thalassemia (S-Thal), which although the rarest variant, can lead to ocular complications.
Sickle cell retinopathy (SCR), like diabetes, has nonproliferative and proliferative stages. The nonproliferative (nonPSCR) stage is characterized by intraretinal hemorrhage known as salmon-patch. Over time, the hemorrhage reabsorbs, leaving refractile deposits along with migration of the retinal pigment epithelium (Black Sunburst lesion). Proliferative SCR (PSCR) is divided into five stages (Goldberg classification): peripheral arteriolar occlusion, peripheral arteriovenous anastomosis, sea-fan neovascularization, vitreous hemorrhage, and tractional retinal detachment. Other posterior segment signs may include, but are not limited to, retinal vascular occlusive diseases, angioid streaks, and dark without pressure.
Diagnostic Modalities
Common diagnostic modalities used in the assessment of SCR include fundus photography, SD-OCT, and UWFA. SD-OCT is particularly useful in assessing inner retinal thinning temporal to the fovea, representing an area of ischemia, such as in our case. On the other hand, UWFA is useful in revealing areas of nonperfusion along with active neovascularization, especially in the periphery. The peripheral retina yields the highest propensity for the manifestation of SCR.
Management
Various management approaches exist for SCR. The nonproliferative stage of the disease is often monitored closely for progression toward PSCR. Treatment options are usually reserved for PSCR, implemented for the active stage of PSCR (stages 3-5). Laser photocoagulation and anti-VEGF injections have proven to be an effective treatment option for PSCR, although vitreoretinal surgery is often reserved for unresolving vitreous hemorrhage and/or tractional RD. Treatment is often initiated in PSCR, however monitoring closely for peripheral nonvisually threatening neovascularization may be a management option in some cases. Neovascularization associated with PSCR has shown spontaneous regression, such as the in the case presented. Yet, reports have also revealed several untreated cases that have progressed, resulting in vision loss. Regardless, close follow-up is crucial.
IMPORTANT TAKEAWAYS
SCR is a common ocular condition in patients with known SCD. Thus, dilated fundus examination is recommended for children ages 9 through 13 with known SCD. Ultra-wide imaging, including UWFA, may be especially valuable in this disease. Of note, although dark without pressure is a common benign retinal finding, it may be associated with SCD in Black or Caribbean patients. Any patient who is suspected of having SCD should be screened with CBC), Sickledex, peripheral blood smear, and plasma hemoglobin electrophoresis.
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ABOUT THIS SERIES
On the path from initial diagnosis to severe disease management, patients may encounter a number of eye care providers. The Collaborative Care Case Series was developed, with guidance from William Trattler, MD, and Diana Shechtman, OD, FAAO, to challenge clinicians' understanding of diagnostic and treatment conventions and advance knowledge of all eye care providers along the continuum.
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