ABSTRACT

Two patients with no symptoms of hypertension in their medical history before pregnancy were referred to the obstetrics emergency clinic with hypertension and visual complaints. After physical examination and laboratory tests, one of the patients was diagnosed with severe preeclampsia while the other was diagnosed with HELLP syndrome (Hemolysis-Elevated Liver enzymes-Low Platelets). Ocular examinations were performed after delivery due to the patients’ worsening visual complaints. The severe preeclamptic patient showed bilateral serous retinal detachment (SRD) while the patient with HELLP syndrome showed bilateral macular hemorrhage. Systemic blood pressure control was advised. The patients’ ocular findings and visual acuities improved in the follow-up periods. SRD and macular hemorrhage can be observed in patients with preeclampsia and HELLP syndrome as a result of the disruption of retinal and choroidal vasculature.

Introduction

Preeclampsia, the most common medical complication of pregnancy, is characterized by elevated systemic blood pressure (≥140/100 mmHg), proteinuria (≥300 mg/24 hours) and generalized edema, and typically appears after the 20th week of pregnancy. Its occurs in 5-10% of all pregnancies.¹ Severe preeclampsia presents with systemic blood pressure over 160/110 mmHg, proteinuria ≥2 gr/24 hours, serum creatinine >2 mg/dl, oliguria, thrombocytopenia, epigastric pain, cerebral and visual disruptions, headache, pulmonary edema, and elevated liver enzymes. Convulsions before or after birth in addition to the symptoms of preeclampsia indicates progression to eclampsia. HELLP (Hemolysis-Elevated Liver enzymes-Low Platelets) syndrome, which is a more severe form that develops in approximately 4-20% of preeclamptic patients, is characterized by hemolysis, elevated liver enzymes and low thrombocyte numbers, and is associated with high maternal and perinatal morbidity and mortality. HELLP syndrome was first mentioned in 1954 by Prichard and was described clinically in detail in 1982 by Weinstein.² It generally occurs in the third trimester, but more rarely can develop in earlier weeks of pregnancy or in the postpartum period, and may appear without hypertension.

Preeclampsia or HELLP syndrome patients may develop retinal and choroidal circulation dysfunction, and various fundoscopic findings and subsequent vision loss may occur as a result. These patients may have severe hypertensive retinopathy findings such as retinal hemorrhage, subretinal serous fluid accumulation, papilledema, and Elschnig spots.³

In this study we present the concurrent fundus findings of one case of severe preeclampsia and one case of HELLP syndrome.

Discussion

SRD occurs in approximately 1% of preeclampsia and HELLP syndrome cases. SRD may be unilateral or bilateral and may appear before birth or in the postpartum period.^4,5 Other findings such as central retinal vein occlussion, vitreous hemorrhage and cortical blindness have also been reported in HELLP syndrome.^6,7,8 Different theories are proposed for the mechanism of SRD development in disease. One theory is that SRD results from choroidal ischemia; another is that it develops due to subretinal fluid accumulation secondary to increased vascular permeability. SRD in preeclampsia is attributed to damage to choroidal vessels and small-to-medium diameter vessels in the choriocapillaris resulting from severe hypertension.^9,10 Another study from Turkey reported fundus fluorescein angiography findings consistent with choroidal ischemia in a preeclamptic patient with bilateral SRD.¹¹ Lin et al.¹² performed wide-field angiography in a preeclamptic patient with HELLP syndrome and bilateral serous macular detachment and found peripheral retinal leakage, which they attributed to choroidal ischemia as well as blood-retina barrier dysfunction. In our preeclamptic patient, we also observed bilateral serous macular detachment and retinal SRD, especially in the inferior region. Furthermore, widespread ischemic lesions consistent with hypertensive retinopathy at the choroid level were observed on color fundus photographs. However, the patient’s fundus findings completely resolved within 1 month. Similarly, Atış et al.¹³ reported bilateral SRD in a patient with severe preeclampsia, and the patient’s vision completely recovered after 6 weeks.

In the literature, various findings have been identified in the ocular involvement of HELLP syndrome, especially the development of retinal hemorrhage due to lowered thrombocyte count has been reported. In a Turkish study, a case of bilateral concommitant macular hemorrhage was presented.¹⁴ One of our cases also had bilateral macular hemorrhage accompanied by areas of retinal hemorrhage and hypertensive retinopathy findings. A study of ocular findings in HELLP syndrome patients reported hypertensive changes in 16%, SRD in 3.7% and cortical blindness in 2.7% of cases.¹⁵ As stated in the literature, we also believe the retinal hemorrhage in our patient was associated with vascular changes arising in the disease due to other causes. In addition to the vasospasm commonly seen with HELLP, endothelial dysfunction, inflammation and altered chorioretinal circulation result in ischemia, which plays a role in the development of disease signs.⁹

In conclusion, various ocular findings may occur in preeclampsia and HELLP syndrome; in most cases, these findings spontaneously resolve with proper management of the disease. Visual symptoms in these patients should definitely be regarded as serious, and the patients should be referred to an ophthalmologist in a timely manner.