Vision on the Brink: How Swept-Source OCTA Uncovered Choroidal Infarcts in HELLP Syndrome

🧠 Clinical Background

• HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelets) is a severe complication of pregnancy, often associated with preeclampsia and eclampsia.
• Ophthalmic manifestations include serous retinal detachments, retinal hemorrhages, cotton-wool spots, and choroidal ischemia.

👁️ Case Presentation

• Patient: 36-year-old pregnant woman at 26 weeks gestation.
• Symptoms: Bilateral blurry vision, abdominal pain, petechiae, and severe hypertension (BP 200/130 mmHg).
• Diagnosis: HELLP syndrome confirmed by lab findings (elevated liver enzymes, low platelets, hemolysis).
• Management: Emergency cesarean section performed.

🖼️ Imaging Findings

• Initial Fundus Exam: Subretinal fluid and Elschnig spots in the right eye.
• OCT Findings: Outer retinal disruption, hyperreflective deposits on Bruch’s membrane, thickened choroid.
• Wide-field SS-OCTA:
  • Revealed choroidal flow voids (subfoveal and peripapillary), consistent with choroidal infarction.
  • Retinal vasculature remained normally perfused.
• Follow-up (6 months):
  • Visual acuity improved.
  • OCTA showed partial resolution of choroidal flow voids.
  • Infrared imaging revealed coalescing RPE changes.

🔬 Pathophysiological Insights

• Choroidal vessels lack autoregulation, making them vulnerable to ischemia during hypertensive crises.
• sFlt-1, a soluble VEGF receptor released during pregnancy, may contribute to endothelial dysfunction.
• Choroidal infarcts can lead to RPE dysfunction and serous retinal detachments due to breakdown of the outer blood-retinal barrier.

🧪 Imaging Modality Significance

• Wide-field SS-OCTA offers a noninvasive, high-resolution alternative to dye-based angiography.
• Especially valuable in pregnant or breastfeeding patients where fluorescein or indocyanine green angiography may be contraindicated.

📝 Conclusion

• HELLP syndrome can cause localized choroidal infarction detectable via SS-OCTA.
• This imaging modality is crucial for diagnosing and monitoring retinal and choroidal vascular complications in pregnancy-related hypertensive disorders.

📸 Figure 1: Multimodal Imaging of Perifoveal Placoid Lesion

• Panels A & B: Color fundus photographs show a polygonal placoid lesion with hyperpigmentation and Elschnig spots near the optic nerve.
• Panel C: OCT reveals ellipsoid zone disruption and subretinal hyperreflective deposits.
• Panel D: Infrared imaging highlights the hyperreflective placoid lesion.
• Panels E & F: Follow-up OCT and infrared images show improvement—condensation of hyperreflective material into discrete deposits and partial resolution of the lesion.

🧠 Figure 2: Wide-Field SS-OCTA Demonstrating Choroidal Infarction

• Panels A & B: En face OCTA shows normal retinal perfusion at baseline and 6-month follow-up.
• Panels E & F: Choriocapillaris OCTA reveals initial flow voids corresponding to the placoid lesion, with improved perfusion at follow-up.
• Panels I & J: Structural en face OCTA of the choroid shows initial flow voids and significant recovery after 6 months.
• Panels C, D, G, H, K, L: B-scans illustrate segmentation used for angiograms in respective panels.

Link: 10.1097/ICB.0000000000001608