Preeclampsia/Eclampsia associated retinopathy

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Hypertensive disorders are seen in 10% of all pregnancies, these disorders includes gestational hypertension, preeclampsia, eclampsia and HELLP syndrome. Preeclampasia/eclampsia and HELLP syndrome can present along with retinal disturbances, serous retinal detachment can be found in 1-2% of the cases presenting preeclampsia/eclampsia.

Disease Entity

Preeclampsia/eclampsia associated retinopathy is a term that defines the retinal pathologies seen in this hypertensive disorders of pregnancy.

Preeclampsia is defined as new-onset hypertension after 20 weeks of gestation and proteinuria and/or evidence of end-organ compromise, including CNS symptoms (headache and/or visual changes), pulmonary edema, thrombocytopenia, renal insufficiency, or liver dysfunction.[1]

HELLP syndrome is a variant of preeclampsia with severe features and is not specifically characterized as a separate entity by the American College of Obstetrics and Gynecologists (ACOG).[1]

Eclampsia is new-onset generalized seizures in a woman with preeclampsia.[1]

Disease

Preeclampsia/eclampsia associated retinopathy is characterized by retinal arteriolar narrowing due to systemic ischemia that generate damage to the retinal and choroidal vasculature and to the retinal pigmentary epithelium.

This ischemic state can manifest as reduced arteriolar caliber and arteriovenous ratio, retinal haemorrhages, oedema and cotton wool spots secondary to arteriolar damage, choroidal dysfunction with secondary RPE damage creating serous retinal detachment, retinal pigment epitheliopathy, vitreous haemorrhage are the most common manifestations.[2][3]

Other alterations as peripheral retinal neovascularization, choroidal neovascularization, macular edema, macular ischemia, a tear of the retinal pigment epithelium, bilateral Purtscher’s like- retinopathy, retinal arterial and venous occlusion and disseminated intravascular coagulopathy (DIC) can also be found.[3][4]

Etiology

Generalized retinal arteriolar narrowing secondary to central retinal artery vasospasm as suggested by increased central retinal artery blood flow velocity.[3]

Epidemiology

The visual system may be affected in 30–100% of patients with pre-eclampsia. The most common ocular finding is severe arteriolar spasm, evidenced by either segmental or generalized constriction of the retinal arterioles reported in 70% of cases of toxaemia. 1–2% of patients who develop severe hypertension, proteinuria, and edema during the third trimester of pregnancy develop loss of vision caused by serous retinal detachment. Retinal pigment epithelium damage and Purtscher like retinopathy have been reported.[2][3][4]

Risk Factors

In a report published by Lupton et al, following normotensive pregnant women with retinal vessel analysis and blood pressure measurements throughout gestation, he found that those who later developed preeclampsia had significant attenuation early in gestation. [5] Li found that in pregnant women every 10-mmHg increase in mean arterial blood pressure was associated with a 1.9-μm reduction in retinal arteriolar caliber.

Risk factors for preeclampsia include primiparity, age 40 years or greater, chronic hypertension, obesity, renal disease, pregestational diabetes, lupus, thrombophilia, antiphospholipid syndrome, multifetal gestation, in vitro fertilization, history of preeclampsia in prior pregnancy, or family history of preeclampsia. Most cases arise in the late preterm and term periods.

General Pathology

Preeclampsia is a systemic syndrome of pregnancy originating in the placenta. It is thought to be caused by inadequate placental cytotrophoblast invasion, followed by widespread maternal endothelial dysfunction.

Research has demonstrated that excess quantities of the antiangiogenic factors soluble fms-like tyro- sine kinase 1 (sFlt1) and soluble endoglin (sEng) are released by the placenta into maternal blood, causing widespread endothelial dysfunction that results in hypertension,proteinuria, and other systemic manifestations of preeclampsia.

The two important antiangiogenic factors implicated in preeclampsia are soluble vascular endothelial growth factor (VEGF) and soluble endoglin. Nitric oxide signaling is involved in vascular relaxation and is reduced in preeclampsia. The molecular basis for placental dysregulation of these pathogenic factors remains unknown.

Diagnosis

The diagnosis is made based on fundoscopy findings and patient history during pregnancy including a review of blood pressure levels along with kidney and liver function test levels.

History

Preeclampsia/eclampsia visual disturbances were first described by Soranus from Ephesus (approx. 120 A.D.); later in 1855 A. von Graefe (1855) first observed serous retinal detachment in patient with this pregnancy hypertensive disorder.

Physical examination

See below

Signs

The commonest ocular finding is severe arteriolar spasm, evidenced by either segmental or generalized constriction of the retinal arterioles reported in 70% of cases of preeclampsia/eclampsia and HELLP syndrome.

Serous retinal detachment usually occurs before or after delivery, the detachment may be confined to the macula or may involve the entire fundus (serous retinal detachment are more common in patients presenting eclampsia) and may rarely be associated with bleeding into the choroid, subretinal space, and vitreous.

A branching pattern of yellow-white patches caused by focal necrosis of the RPE and outer retina may or may not be evident.

These patients may or may not show evidence of cotton-wool patches and hemorrhages in the retina. After delivery and treatment of the hypertension, the detachment rapidly resolves and recovery of visual function is relatively complete in most patients.

An irregular branching pattern of hyperpigmented dots (Elschnig spots), yellowish patches, and interconnecting lines of depigmentation of the RPE often remains in the juxtapapillary and macular area . These changes, caused by infarction of the RPE, are frequently symmetric in both eyes and, if initially detected on a routine eye examination years later, may be incorrectly interpreted as signs of a macular dystrophy.

Symptoms

  • Blurred vision, is the most common symptom.
  • Color vision disturbances
  • Decreased visual field

Clinical diagnosis

Diagnosis is made based on fundoscopy findings along with patient pregnancy history of preeclampsia/eclampsia or HELLP syndrome. Ancillary test are helpful to confirm clinical diagnosis.

  • Fluorescein Angiography
Angiography may demonstrate evidence of delayed perfusion of segments of the choroid as well as multiple pinpoint areas of leakage of dye through the RPE in the region of the subretinal yellow-white patches. The early fluorescein angiogram shows a delay in perfusion of the choriocapillaris from ischemia. Later, a multifocal reticular stain pattern is found over RPE damaged areas in the course of this entity.
  • Fundus Autofluorescence
  • Ultrasonography


The ophthalmic echography mode B ultrasound may be useful for the documentation and follow up of serous retinal detachment.

Example.jpg

  • Visual Fields

Differential diagnosis

  • Pattern Dystrophy
  • Vogt-Koyanagi-Harada (VKH) disease
  • Disseminated intravascular coagulopathy (DIC)

Management

Medical therapy

Usually no treatment is needed after delivery; however, steroids and anti hypertensive drugs have been reported in the treatment of this entity.

Complications

Patients with severe toxemia may develop profound visual loss and blindness secondary to total serous and hemorrhagic retinal detachment and be left following recovery with extensive degenerative changes in the RPE and retina that may simulate a diffuse tapetoretinal dystrophy. Correct diagnosis is important since the changes caused by toxemia are nonprogressive.

Cases with severe preeclampsia may be left with permanent visual loss, despite resolution of the serous retinal detachment due to extensive RPE necrosis.

Prognosis

Most patients with retinal detachment in pregnancy induced hypertension have had full spontaneous resolution within a few weeks, and they did not have any sequelae. Pregnancies complicated by preeclampsia are associated with a worse prognosis for mother and child.

Additional Resources

For information about other ocular alterations during pregnancy, visit the following link:

https://www.aao.org/eyenet/article/ocular-changes-during-pregnancy

References.

  1. 1.0 1.1 1.2 American College of Obstetrics and Gynecologists Task Force on Hypertension in Pregnancy. Hypertension in Pregnancy. Obstetrics & Gynecology. 2013;122(5):1122-1131. doi:10.1097/01.aog.0000437382.03963.88.
  2. 2.0 2.1 Agarwal A, Gass J. Gass' Atlas Of Macular Diseases. Edinburgh: Elsevier Saunders; 2012.
  3. 3.0 3.1 3.2 3.3 Ryan S, Sadda S, Hinton D, Schachat A, Wilkinson C, Wiedemann P. Retina. 6th ed. Elservier; 2018.
  4. 4.0 4.1 Singh K, Jain D, Wallang B. Purtscher’s retinopathy in pre-eclampsia: a blinding combination. Int Ophthalmol. 2013;34(1):103-106. doi:10.1007/s10792-013-9739-1.
  5. Hypertension. 2013 Nov;62(5):899-904. doi: 10.1161/HYPERTENSIONAHA.113.01890. Epub 2013 Sep 9. Changes in retinal microvascular caliber precede the clinical onset of preeclampsia. Lupton SJ1, Chiu CL, Hodgson LA, Tooher J, Ogle R, Wong TY, Hennessy A, Lind JM.