BASICS

DESCRIPTION

• Various ophthalmic conditions are associated with or exacerbated by pregnancy.

– Refractive changes: transient loss of accommodation; increased corneal thickness, edema, and curvature; decreased corneal sensitivity

– Preeclampsia or pregnancy-induced hypertension (PIH): marked by hypertension, proteinuria, peripheral edema. Eclampsia also includes seizures. Symptoms include headache and blurred vision. Hypertension can lead to cortical blindness.

– HELLP syndrome: hemolysis, elevated liver enzymes, and low platelet counts. Occurs in 10% of patients with severe PIH.

– Occlusive vascular disease: due to the hypercoagulable state of pregnancy. Can lead to artery or vein occlusions, disseminated intravascular coagulopathy (DIC), and thrombotic thrombocytopenia purpura (TTP; thrombus formation, hemolytic anemia, thrombocytopenia, neurologic changes, fever, renal dysfunction). Ocular DIC has widespread small vessel thrombosis, particularly in the choroid.

– Amniotic fluid embolism: can occur during labor, delivery, or the immediate postpartum period from particulate matter from the amniotic fluid entering the maternal circulation. It can cause cardiopulmonary failure, as well as central retinal artery occlusion, and is associated with 80% mortality.

– Purtscher-like retinopathy: within 24 hours of birth, usually associated with late complications such as preeclampsia, pancreatitis, TTP.

– Meningioma of pregnancy: meningiomas can grow aggressively during pregnancy. May regress postpartum.

– Diabetic retinopathy and diabetic macular edema: tends to progress during pregnancy usually with varying degrees regression postpartum. No risk of diabetic retinopathy with gestational diabetes. Nonproliferative diabetic retinopathy (NPDR) progresses in up to 50%. Proliferative diabetic retinopathy (PDR) progresses rapidly.

– Central serious chorioretinopathy (CSCR): associated with pregnancy, as well as increased endogenous or exogenous cortisol

– Pituitary apoplexy: preexisting pituitary adenomas can enlarge during pregnancy. Apoplexy is the acute expansion of a pituitary adenoma due to infarction or hemorrhage. Leads to acute headache, nausea, visual field loss (bitemporal hemianopia), limitation of extraocular motility (due to involvement of cranial nerves III, IV, and VI, in the cavernous sinus), and/or Horner syndrome. Sheehan syndrome is pituitary apoplexy of a nontumorous pituitary gland, presumably due to postpartum arterial spasm of arterioles supplying the anterior pituitary and its stalk.

EPIDEMIOLOGY

Prevalence

Preeclampsia occurs in 5% of pregnancies, typically after 20 weeks of gestation and in primigravidas (first pregnancy).

RISK FACTORS

• PIH risk factors: first and multifetal pregnancies, extremes of maternal age, mothers with vascular disease

• DIC risk factors: complicated abortions, abruptio placenta, severe preeclampsia, and retained dead fetus.

• Diabetic retinopathy: increased risk of progression if longer duration of preexisting diabetes, poorer glycemic control before and during pregnancy, rapid normalization of glucose levels during pregnancy, severity of retinopathy at conception, and presence of concomitant hypertension.

GENERAL PREVENTION

Prenatal care to monitor for hypertension, hyperglycemia, and proteinuria

PATHOPHYSIOLOGY

• Serous retinal detachments in PIH is felt due to choroidal ischemia.

• Cortical blindness in PIH is due to cerebral arteriolar vasospasm and cerebral edema.

• Purtscher-like retinopathy is due to complement-induced granulocyte aggregation and vascular occlusion.

• It is unclear why diabetic retinopathy accelerates during pregnancy.

DIAGNOSIS

HISTORY

• History of hypertension or diabetes?

• Blurred vision, distortion, micropsia, photopsias, floaters, visual field loss, headache, fluctuating vision?

• Seizures, increased urinary frequency, peripheral edema?

• History of corticosteroid use?

• Nausea or ocular paresis?

PHYSICAL EXAM

• Complete ophthalmic examination, including vital signs (particularly blood pressure), pupils, visual fields, color plates, gonioscopy (if PDR), optic nerve assessment, and dilated fundus examination.

– Preeclampsia or PIH: findings are related to HTN, including retinal hemorrhages, nerve fiber layer infarcts, exudation, serous retinal detachment (in 1% of preeclamptic and 10% of eclamptic patients), focal arteriolar narrowing and spasm, optic nerve edema, nonarteritic ischemic optic neuropathy, and bilateral occipital lobe infarcts (preeclampsia/eclampsia hypertensive posterior encephalopathy syndrome [PEHPES])

– HELLP: bilateral serous retinal detachment, subretinal yellow–white opacities, vitreous hemorrhage

– Occlusive vascular disorders: retinal artery and vein occlusions, nerve fiber layer infarcts, retinal hemorrhage and exudation, Purtscher-like retinopathy with multiple nerve fiber layer infarcts around the optic nerve associated with TTP

– Ocular DIC: hemorrhage, tissue necrosis, serous retinal detachments, underlying retinal pigment epithelium (RPE) alterations

– Purtscher-like retinopathy: widespread nerve fiber layer infarcts with or without hemorrhage

– Diabetic macular edema: cystic macular edema in foveal region often associated with microaneurysms and hard exudates

– NPDR: dot-blot retinal hemorrhages, microaneurysm, venous beading, exudation, macular edema, intraretinal microvascular abnormalities

– PDR: NPDR plus neovascularization of optic nerve, elsewhere in the retina, iris, or angle

– CSCR: serious retinal detachment without hemorrhage, subretinal exudation is more common (90%) than in nonpregnant females (<20%), RPE irregularities

DIAGNOSTIC TESTS & INTERPRETATION

Lab

Initial lab tests

• Urinalysis for proteinuria

• Electrolytes

• CBC

• PT/PTT

Follow-up & special considerations

• Ophthalmic examination warranted if patient experiences visual symptoms

• Gestational diabetes: no risk of retinopathy during pregnancy; routine ophthalmic examination not needed

• None or minimal NPDR: minimal to no progression during pregnancy; ophthalmic examination first and third trimester

• Mild to moderate NPDR: progression in up to 50% during pregnancy; ophthalmic examination every trimester

• High-risk NPDR: progression in up to 50% during pregnancy; monthly ophthalmic examination

• PDR: tends to progress rapidly during pregnancy; monthly ophthalmic examination (1)[A]

Imaging

• If PEHPES is suspected, check brain MRI. Findings include bilateral occipital lobe lesions (identical to nonobstetric hypertensive encephalopathy)

• If pituitary apoplexy suspected, emergently check brain MRI. Hemorrhage within the sella is isointense or hypointense on T1-weighted images within first 3–5 days. In nonhemorrhagic (ischemic) pituitary apoplexy, MRI reveals an enlarged pituitary gland bulging under the optic chiasm with peripheral enhancement surrounding a hypointense gland (pituitary ring sign).

• If meningioma is suspected, check MRI of brain and/or orbits.

Diagnostic Procedures/Other

• OCT to evaluate for CSCR or macular edema

• Intravenous fluorescein dye crosses the placenta and is present in breast milk for at least 76 h. No additional adverse effects have been noted in pregnant women nor teratogenic or embryogenic effects in animal studies. Most vitreoretinal specialists would avoid fluorescein angiograms in pregnant women unless necessary for a sight threatening condition (e.g., choroidal neovascularization).

• Indocyanine green (ICG) dye does not cross the placenta and it is unknown if it’s found in breast milk. ICG has been used for nanophthalmic conditions in pregnant woman without adverse effect on mother or fetus. Most vitreoretinal specialists would avoid ICG angiography in pregnant women unless necessary.

DIFFERENTIAL DIAGNOSIS

For PEHPES: posterior circulation stroke, intracranial venous thrombosis due to coagulation disorder, migraine, intracranial hemorrhage, infectious cerebritis or meningitis, tumor with hemorrhage, atypical seizure, demyelination.

TREATMENT

MEDICATION

• Avoid prescribing new glasses until several weeks postpartum given the shifting refraction during pregnancy

• For preeclampsia, eclampsia, or PIH, treat electrolyte imbalances and hypertension, and deliver baby

ADDITIONAL TREATMENT

General Measures

• Treat PDR with panretinal photocoagulation (PRP) and follow monthly. Consider earlier PRP for severe pre-PDR.

• Observe diabetic macular edema; it often resolves postpartum.

• Consider caesarian section in PDR to prevent risk of vitreous hemorrhage during Valsalva.

• CSCR usually resolves spontaneously.

Issues for Referral

• Internal medicine for hypertension and hyperglycemic control

• Endocrinology for hyperglycemic management and for pituitary apoplexy

• Neurology for seizures with eclampsia, for neurologic findings with meningiomas, and for PEHPES.

• Neurosurgery for pituitary apoplexy

• Ophthalmologist or vitreoretinal specialist for diabetic retinopathy, diabetic macular edema, CSCR, Purtscher’s retinopathy.

SURGERY/OTHER PROCEDURES

Neurosurgical transsphenoidal surgical decompression of pituitary apoplexy.

IN-PATIENT CONSIDERATIONS

Initial Stabilization

• Control blood pressure in PIH

• Medically stabilize patients with pituitary apoplexy, administer high-dose corticosteroids, evaluate pituitary hormones, electrolytes, and glucose, administer appropriate endocrinologic replacement therapy, consider neurosurgical transsphenoidal surgical decompression.

ONGOING CARE

DIET

Healthy, well-balanced diabetic diet for patients with diabetes.

PATIENT EDUCATION

Contact your obstetrician or seek emergency care if you experience visual changes, blurred vision, visual field loss, double vision, headache, nausea/vomiting, or seizures.

PROGNOSIS

• Perinatal mortality rate with PIH is 13–30%. Causes of death include pulmonary edema, CNS hemorrhage, or cardiac, liver, or renal failure.

• Vascular changes seen in PIH resolve postpartum. Permanent visual change is unusual.

• Serous retinal detachment seen in PIH usually resolves postpartum. Visual acuity may be affected by RPE changes.

• CSCR usually resolves but may recur postpartum or during a subsequent pregnancy.

• High likelihood of postpartum regression in diabetic retinopathy changes that occur during pregnancy

COMPLICATIONS

• Decreased visual acuity

• Visual field loss

• Ophthalmoplegia

• Vitreous hemorrhage

• Tractional retinal detachment

• Neovascular glaucoma

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