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31-year-old postpartum patient experiences acute-onset blurry vision
Exam showed blunted foveal reflexes bilaterally with subretinal fluid and retinal folds in the left eye.
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A 31-year-old woman, postop day 1 from cesarean section for dichorionic-diamniotic twins at 35 weeks’ gestational age, was transferred from an outside hospital for evaluation of blurred vision in the right eye that started postoperatively. The patient reported diffuse “fuzzy” vision in the right eye that was stable at distance and near. She denied double vision and eye pain. Systemically, she was feeling well before the visual changes, with no neurologic symptoms, headaches or transient visual obscurations. Neurology was consulted at the outside hospital to rule out neurologic causes of blurry vision. MRI, MRA and MRV were obtained, and all were within normal limits.
The patient denied any ocular history or other medical problems. Her only medications were a prenatal vitamin and iron supplement. She had been given two doses of steroids at 35 weeks’ gestation to promote lung development of her twins. Her only surgical history was the cesarean section 1 day before presentation. She denied allergies, smoking, and drug or alcohol use, and she lived with her husband. Her family history was positive for a twin sister who had also experienced an episode of blurred vision postpartum.
Examination
On presentation, the patient was alert and oriented to person, place and time with normal mood and affect. Visual acuity without correction was 20/20-2 in the right eye and 20/20-3 in the left eye. Both pupils were round and briskly reactive to light, measuring 4 mm in dim light and 3 mm in bright light. There was no evidence of an afferent pupillary defect. IOPs were 14 mm Hg in the right eye and 15 mm Hg in the left eye. Visual fields were full to confrontation bilaterally. Extraocular movements were full bilaterally. Ishihara color plates were slightly diminished in the right eye with +control, 9/10 right eye and +control, 10/10 left eye. The external lids and lacrimal system were within normal limits.
Source: Sarah Adelson, MD, Tara Bryant, MD, and Caroline Baumal, MD
On slit lamp examination, the conjunctiva was white and quiet, cornea was clear, anterior chamber was quiet, iris was round and reactive, and lenses were clear in both eyes. The vitreous was clear bilaterally. The optic nerves were pink and sharp with small cups in both eyes. Retinal examination showed blunted foveal reflexes bilaterally with an area of subretinal fluid with retinal folds in the left eye (Figure 1). OCT of the right eye showed mild subfoveal fibrinous subretinal fluid with a turbid appearance and flat, irregular pigment epithelial detachment. OCT of the left eye showed mild fibrinous subretinal fluid temporal to the fovea with a flat, irregular pigment epithelial detachment (Figure 2). Blood pressure obtained in clinic was 184 mm Hg/112 mm Hg.
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Blurred vision after pregnancy
The differential diagnosis of blurry vision in a postpartum patient includes preeclampsia/HELLP syndrome, which can lead to hypertensive retinopathy, exudative retinal detachments and cortical blindness. Other causes are central serous chorioretinopathy, Purtscher-like retinopathy, arteriolar occlusions, dural venous thrombosis and refractive shift. The differential diagnosis of serous retinal detachments in a postpartum patient includes those secondary to preeclampsia, HELLP syndrome, malignant hypertension or central serous chorioretinopathy.
Hypertensive retinopathy can include exam findings such as arteriolar narrowing (most common), retinal hemorrhages, exudates, cotton wool spots, retinal edema, vitreous hemorrhage and nerve fiber layer defects. Exudative retinal detachment related to hypertensive retinopathy in patients with preeclampsia tends to be bilateral, diagnosed postpartum, more frequent in primiparous women and more common in women who undergo cesarean delivery. Our patient had subretinal fluid in both eyes although she was only symptomatic in one. OCT and fluorescein angiography can help identify subtle subretinal fluid and show choroidal ischemia from intense arteriolar vasospasm, which leads to leakage of fluid into the subretinal space. The retinal pigment epithelium (RPE) layer usually resorbs the subretinal fluid postpartum, and visual acuity typically returns to pre-detachment levels within days to a few weeks.
Central serous chorioretinopathy (CSCR) in postpartum patients typically presents as a unilateral metamorphopsia with moderately reduced visual acuity. CSCR tends to first occur in late pregnancy, and OCT commonly shows unilateral fibrinous subretinal exudates (90% of pregnancy-associated CSCR compared with 20% of general CSCR). This patient had these OCT findings, but it was bilateral. Fluorescein angiography can show an area of leakage that will often resolve within a few months. This patient received two doses of high-dose steroids before the episode of visual loss, which is a known risk factor for CSCR.
Less likely on the differential is Purtscher-like retinopathy, in which patients may experience severe unilateral or bilateral visual loss. Exam shows widespread cotton wool spots with or without intraretinal hemorrhages, indicative of arteriolar obstruction.
Another less likely consideration is cortical blindness in a postpartum patient. This will present as sudden bilateral visual loss with a normal pupillary response and normal fundus examination. Cortical blindness can present in the setting of hypertension and can be seen in up to 15% of preeclamptic and eclamptic patients. Thought to occur from cerebral edema, it is often preceded by headache, hyperreflexia and paresis. Cortical visual loss can recover over a period of 4 hours to 8 days, although bilateral inferior scotomata and visual field defects have been reported to persist for several months postpartum. An MRI scan may reveal focal occipital lobe edema, including bilateral edema of the lateral geniculate nuclei, represented by hyperintense lesions on T2-weighted images. The MRI of our patient was normal at the outside hospital, and she had no other symptoms suggestive of cortical blindness.
One should also consider arteriolar occlusions postpartum, which will present as decreased vision or scotoma from a central retinal artery occlusion or a branch retinal artery occlusion. These can be secondary to amniotic fluid particles, thrombotic thrombocytopenic purpura (TTP) or disseminated intravascular coagulation (DIC), which can occur after delivery. The vision loss in arteriolar occlusions is typically more severe (although not always), and there may be evidence of an occlusion on fundus examination such as retinal whitening and/or a clear embolus in the retinal vessels. A fluorescein angiography should be performed to rule out arteriolar occlusion.
A more serious diagnosis on the differential that should be ruled out is a dural venous sinus thrombosis. This may present as headaches, decrease in consciousness, decreased vision, nausea/vomiting and/or seizures. On examination, one would expect to find evidence of an increase in intracranial pressure, such as papilledema. Our patient did not have any of the other symptoms associated with a venous sinus thrombosis. However, there is a greater risk in pregnancy due to a hypercoagulable state, as well as the risk of TTP or DIC, so it should always be considered. CTV or MRV is necessary to make this diagnosis, which the neurology team performed at the outside hospital. The MRV was negative, essentially ruling out this diagnosis.
Further workup and management
The patient underwent fluorescein angiography, which showed hypofluorescence from blockage in the area of fluid without leakage and nonspecific patchy hyperfluorescence (Figure 3). Of note, there was no petaloid leakage, no leakage into the area of fluid, no vessel leakage and no nerve leakage. There was also no evidence of arteriolar occlusion.
Lab workup was notable for a hemoglobin of 8.3, platelet count of 31,000 and elevated liver function enzymes, AST and ALT, at 103 and 112, respectively. A urine dipstick also showed 2+ protein. The patient was diagnosed with preeclampsia and HELLP syndrome. She was admitted for observation overnight to receive labetalol 200 mg twice daily and a 24-hour course of magnesium.
Our patient was ultimately diagnosed with hypertensive retinopathy complicated by serous retinal detachments in the setting of preeclampsia and HELLP syndrome. While there may be a component of CSCR, the choroid was not thickened and fluorescein angiography did not reveal leakage in a pattern characteristic of CSCR.
Discussion
Preeclampsia is defined by a triad of blood pressure higher than 140 mm Hg/90 mm Hg, edema and proteinuria. Preeclampsia/HELLP can lead to hormonal changes, endothelial damage and hypoperfusion in the eye. This may cause visual disturbances including scotoma, diplopia, diminished vision and photopsias. These symptoms occur in 25% of patients with preeclampsia and 50% of patients with eclampsia (preeclampsia triad plus seizures). Retinal findings include arteriolar narrowing (most common), retinal hemorrhages, exudates, cotton wool spots, retinal edema, vitreous hemorrhage and retinal nerve fiber layer defects. These ocular findings on retinal examination will be seen in more than 50% of preeclamptic patients. Exudative retinal detachments can occur in 1% of preeclamptic patients and 10% of eclamptic patients (preeclampsia plus seizures). HELLP is a complication of preeclampsia and has three main features: hemolysis, elevated liver enzymes and low platelets. Patients may present with any of the ocular findings of preeclampsia/eclampsia but are seven times more likely to develop a serous retinal detachment.
Preeclampsia is caused by abnormal formation and development of the placenta due to inadequate placental cytotrophoblast invasion. This leads to altered immune response to the placenta causing placental injury, endothelial cell injury, oxidative stress, and release of anti-angiogenic proteins and inflammatory mediators into the maternal plasma. This widespread endothelial dysfunction results in hypertension, proteinuria and other systemic manifestations of preeclampsia.
One more diagnosis high on the differential initially was CSCR. CSCR is the accumulation of subretinal fluid that leads to a circumscribed neurosensory retinal detachment in the macula at the level of the RPE. There is a strong association with pregnancy, especially late in pregnancy. This is thought to be due to elevated levels of endogenous cortisol or exogenous steroids leading to increased permeability in the blood-retinal barrier, choriocapillaris and RPE. Visual acuity most commonly returns to normal a few months after onset; however, changes to the central visual field, metamorphopsia and RPE alterations may persist. CSCR also has a strong likelihood of recurring in future pregnancies.
Clinical course continued
Over the course of the patient’s hospital stay, her blood pressure normalized on labetalol. Her labs also normalized, and she was discharged with a hemoglobin and hematocrit of 9.3 and 28.6, respectively. Her platelet count was 141, and her liver enzymes, AST and ALT, were 45 and 80, respectively.
She was seen in retina clinic 1 week after discharge, and her symptoms had resolved. The retinal exam was normal without evidence of subretinal fluid or findings of hypertensive retinopathy (Figure 4). Her OCT showed resolution of subretinal fluid (Figure 5). The rapid resolution of her serous retinal detachments is most consistent with a diagnosis of preeclampsia and HELLP syndrome as CSCR generally takes a few months to fully resolve. She has returned to her regular ophthalmologist for yearly routine eye exams but was educated on the possibility of having visual changes during a future pregnancy and advised to return if she notices any changes to her vision.
- References:
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- Mackensen F, et al. Dtsch Arztebl Int. 2014;doi:10.3238/arztebl.2014.0567.
- Naderan M. J Curr Ophthalmol. 2017;doi:10.1016/j.joco.2017.11.012.
- Rezai S, et al. Obstet Gynecol Int J. 2006;doi:10.15406/ogij.2016.04.00093.
- Sharma S, et al. The Obstetrician & Gynaecologist. 2006;doi:10.1576/toag.8.3.141.27248.
- For more information:
- Sarah Adelson, MD, Tara Bryant, MD, and Caroline Baumal, MD, can be reached at New England Eye Center, Tufts University School of Medicine. 800 Washington Street, Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Adam T. Chin, MD, and Omar Dajani, MD. They can be reached at the New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.