Multimodal Imaging in Hellp-Related Chorioretinopathy

Abstract

Purpose: To illustrate with multimodal imaging a case of HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelets) complicated by bilateral multifocal serous retinal detachments, subretinal exudation, and papilledema.Methods: Case report. Fundus photography, spectral domain optical coherence tomography (SD-OCT), fluorescein angiography, and indocyanine green angiography were performed at presentation and the day after. We also present the SD-OCT follow-up at 8 days, 1 year, and 4 years.Results: A 25-year-old 5-month-pregnant Guinean woman complained about decreased visual acuity in the right eye. Eye fundus and multimodal imaging were abnormal in both eyes. Spectral domain optical coherence tomography showed the presence of multifocal serous retinal detachments, subretinal deposits, and intraretinal cysts. Indocyanin green angiography revealed an irregular choroidal perfusion and localized choroidal ischemia. Spectral domain optical coherence tomography also provided assessment of retinal changes during the long-term follow-up, showing tissue damage in the outer retina.Conclusion: Serous retinal detachments during pregnancy can be the leading sign of HELLP syndrome-a potentially life-threatening condition. Spectral domain optical coherence tomography is a noninvasive and useful tool for its diagnosis and follow-up. ICG is important to confirm the choroidal ischemia and choroidal vascular abnormalities, underlying conditions leading to main sign of HELLP syndrome in the eye.

Subretinal Drusenoid Deposits in a Patient With HELLP (Hemolysis, Elevated Liver Enzymes, Low Platelet) Syndrome

“…However, several additional factors, including systemic inflammation, endothelial dysfunction, and microthrombi caused by disseminated intravascular coagulation seen in HELLP syndrome, may further exacerbate the choroidal ischemia and subsequent damage to the RPE and Bruch's membrane complex [11]. These alterations may also cause increased vascular permeability and fluid accumulation leading to SRD, which accompanied SDDs in previous case reports with HELLP syndrome, including ours [5,6]. In a crosssectional study investigating the presence of SDD in patients with pre-eclampsia and malignant hypertension, Otero-Marquez et al observed that SRD was present in all eyes with SDD [10].…”

“…So far, hypertensive changes of the retina and choroid, SRD, retinal vein occlusion, and cortical blindness have been shown to be related to HELLP syndrome [ 4 ]. Very recently, a few case reports of HELLP syndrome-related visual loss documented drusenoid deposits after the resolution of serous macular detachment [ 5 , 6 ].…”

“…The prevalence of SDD in pre-eclampsia and MHT patients was 32.7% and 23.4%, respectively, in this study. Multimodal imaging, including fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography angiography (OCTA) documented impaired choroidal circulation in recent case reports diagnosed with HELLP syndrome [ 5 , 6 ].…”

Seltene vaskuläre Erkrankungen der Netzhaut – Teil 1

“…Dabei liegen Störungen der choroidalen Perfusion bis hin zu choroidalen und seltener retinalen Gefäßverschlüssen vor. Sie wird symptomatisch durch eine akute Visusminderung mit variabler Ausprägung aufgrund einer serösen Netzhautabhebung mit subretinalen weißlichen Ablagerungen (selten mit RPE-Einriss) und retinochoroidalen Gefäßverschlüssen bis hin zu einem choroidalen Infarkt, Zentralarterien- oder Zentralvenenverschluss 9 , 31 . Elschnig-Flecken entsprechen nicht perfundierter Choriokapillaris.…”

“…Klinisch ist eine Verengung der Netzhautarteriolen zu beobachten. Zusätzlich kann eine hypertensive Choroidopathie auftreten, unter anderem bei Gestosen [9]. Durch eine fibrinoide Nekrose der Aderhautgefäße kommt es zu einer fokalen Störung der äußeren Blut-Retina-Schranke im retinalen Pigmentepithel (RPE) mit lokalisierten Netzhautabhebungen.…”