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Inpatient ophthalmology consult for baseline exam in girl with lupus

There was a peripapillary neurosensory retinal detachment extending into the superior macula in the right eye and optic disc hemorrhages in both eyes.

Issue: July 10, 2014

ByShelly T. Lee, MD

ByClaudia E. Bartolini, MD

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A 7-year-old girl presented to the university hospital with a 3-month history of fevers, headaches, fatigue, decreased appetite with associated weight loss, myalgias, oral ulcers, and a rash that started on her face and then spread to her arms and trunk. She was also found to be hypertensive, and a complete blood count ordered by her primary care physician revealed severe anemia. She was admitted for further work-up.

The patient was diagnosed with systemic lupus erythematosus (SLE) after testing positive for antinuclear antibody and double-stranded DNA. She was started on high-dose intravenous methylprednisolone on the day of admission. Ophthalmology was consulted on day 3 of her hospitalization for a baseline exam. She had no visual complaints or eye pain at that time. She also denied flashes or floaters.

The patient’s ocular history was unremarkable. She had no medical or surgical history and did not take medications at home. She was born full term, and the mother reported no problems during pregnancy or delivery. All developmental milestones had been reached. She was in the second grade, doing well in school, and had previously been very active and energetic. She lived with her older sister, mother and father. Both her father and paternal grandmother had SLE.

Examination

On examination, best corrected visual acuity was 20/20 in both eyes. Pupils were equally round and reactive to light, with no afferent pupillary defect in either eye. Extraocular movements were full. IOP was 10 mm Hg in the right eye and 12 mm Hg in the left eye. Ishihara plates were 10/10 in both eyes. Visual fields were full to confrontation.

The anterior segment exam on slit lamp biomicroscopy was unremarkable in both eyes with no signs of inflammation. Dilated funduscopic examination revealed a peripapillary serous retinal detachment extending into the superior macula in the right eye. There were nerve fiber layer hemorrhages superior to the optic nerves in both eyes (Figure 1). A fluorescein angiogram demonstrated an area of hyperfluorescence in the nasal macula consistent with pooling (Figure 2).

Figure 1. Fundus (top) and red-free (bottom) photography showing a large peripapillary serous detachment extending into the macula in the right eye and superior retinal nerve fiber layer hemorrhages in both eyes.

Images: Bartolini CE, Lee ST

Figure 2. Fluorescein angiography demonstrating blockage from superior nerve fiber layer hemorrhages in both eyes. There was normal filling time of the arteries and veins but a focus of pooling in the nasal macula of the right eye.

Spectral-domain optical coherence tomography confirmed the presence of a neurosensory retinal detachment in the right eye (Figure 3).

Figure 3. Spectral domain OCT revealed a right peripapillary neurosensory retinal detachment (top) which extended into the right superior macula (bottom).

What is your diagnosis?

Neurosensory retinal detachment and optic disc hemorrhages

The differential diagnosis for a female child with SLE, bilateral disc hemorrhages and a neurosensory retinal detachment includes lupus choroidopathy, hypertensive choroidopathy, idiopathic central serous chorioretinopathy or, less likely, Vogt-Koyanagi-Harada syndrome.

The patient did not have bilateral granulomatous uveitis or skin findings such as vitiligo, alopecia or poliosis, which are characteristic of Vogt-Koyanagi-Harada syndrome. Idiopathic central serous chorioretinopathy (CSCR) also seemed unlikely because the patient did not fit the common demographic. CSCR usually presents in young white men between the ages of 20 and 50 years and is associated with type A personality. Although stress or steroids may exacerbate CSCR and the patient had recently started intravenous steroids, her underlying systemic disease was the more likely explanation for her eye findings. Although lupus choroidopathy can cause multifocal serous detachments in patients with active lupus, it is rare and not usually associated with disc hemorrhages.

The presumptive diagnosis of hypertensive choroidopathy was made and fit best with the clinical picture of nerve fiber layer hemorrhages with an associated peripapillary neurosensory retinal detachment in the right eye. This diagnosis was supported by a review of the patient’s blood pressure history and echocardiogram findings.

Clinical course

During her hospitalization, our patient’s blood pressures ranged as high as 146/90 mm Hg to 150/98 mm Hg, with a peak of 200/100 mm Hg at the time of MRI of her brain. The upper limit of normal blood pressure for this patient’s age and height is 115/75 mm Hg. A transesophageal echocardiogram showed borderline left ventricular hypertrophy, which supported the chronicity of her hypertension. A MRI of the brain was unremarkable for cerebral vasculitis.

A complete blood count was notable for hemoglobin of 6.2 and hematocrit of 18.7. A peripheral blood smear was positive for spherocytes, and Coombs test was positive, which confirmed that the patient had an autoimmune hemolytic anemia. Her antinuclear antibody level was greater than 1:1250. Double-stranded DNA, histone antibody and erythrocyte sedimentation rate were elevated. Anti-Smith, SSA, SSB, RNP, Scl-70, Jo-1 and RF antibodies were within normal limits. A hypercoagulable panel showed high antithrombin 3, anticardiolipin and von Willebrand factor levels. Urine chemistry showed a low-grade proteinuria and hematuria, despite a normal creatinine and blood urea nitrogen level.

The patient required a blood transfusion for her anemia and was started on amlodipine for blood pressure control and 100 mg daily of hydroxychloroquine. Intravenous steroids were continued for 3 days, and then she was transitioned to oral prednisone 15 mg twice daily. Follow-up at 1 week showed mild improvement in the neurosensory retinal detachment in her right eye. The patient’s systemic signs and symptoms of SLE, including her blood pressure, also improved. Although steroids may have exacerbated her high blood pressure in the short term, they were essential for managing her acute presentation. At the 2-month follow-up, our patient’s neurosensory retinal detachments resolved completely (Figure 4), as did the nerve fiber layer hemorrhages. There was a lingering rash, but the anemia and fatigue had also resolved on the hydroxychloroquine and slow prednisone taper. Hypertension was controlled on the amlodipine, with average readings in the range of 110-118/70s mm Hg.

Figure 4. After antihypertensive treatment for 2 months, spectral-domain OCT showed resolution of the peripapillary serous detachment (top) that previously extended into the macula (middle, bottom).

Discussion

Hypertension in children is rare and usually secondary to an underlying disorder. Hypertensive choroidopathy is an even rarer manifestation in childhood, with only a few case reports in the literature. It has been reported in adults in toxemia of pregnancy, renal disease, pheochromocytoma and malignant hypertension. Our case illustrates a pediatric patient with SLE with severe hypertension and associated hypertensive choroidopathy. Hypertension is prevalent in SLE and is believed to be caused by chronic inflammation and altered renal function.

The definition of hypertension in children is based on blood pressure percentile. Childhood hypertension is defined as average systolic and diastolic blood pressure that is greater than or equal to the 95th percentile for gender, age and height. Hypertension in children is further subdivided into types 1 and 2. Type 2, defined as blood pressure 12 mm Hg or more above the 95th percentile, is the most severe form. The upper limit of normal blood pressure for our patient’s age and height should have been 115/75 mm Hg. Our patient was classified as having type 2 childhood hypertension, according to the Working Group on High Blood Pressure Control in Children and Adolescents 2004 report.

Patients with hypertension are generally visually asymptomatic. In severe cases, however, patients may notice a decrease in vision. On examination, patients with hypertension most commonly show a retinopathy, which in its mildest form consists of generalized arterial narrowing. As the retinopathy progresses, there is a breakdown of the inner blood-retinal barrier with development of cotton wool spots, hemorrhages and hard exudates. When hypertension is especially severe, intracranial pressure can become elevated with ischemia to blood vessels of the optic nerve resulting in optic disc swelling. The choroidal arterioles in the choriocapillaris can also undergo fibrinoid necrosis with subsequent breakdown of the outer blood-retinal barrier. The retinal pigment epithelium can become necrotic, leading to Elschnig spots, Siegrist streaks or serous retinal detachments. Untreated hypertension can cause major visual morbidity, including an increased risk for retinal vein or artery occlusions, macroaneurysms or ischemic optic neuropathy. Systemically, patients with uncontrolled blood pressure are at a higher risk of stroke, heart disease and worsening renal function.

The diagnosis of hypertensive retinopathy or choroidopathy is made by correlating clinical findings in the eye with the patient’s blood pressure history. An echocardiogram may also provide evidence for hypertensive chronicity. Left ventricular hypertrophy is seen in 25% of hypertensive children.

The treatment for hypertensive retinopathy or choroidopathy is treatment of the underlying high blood pressure. There are multiple medications that have been approved by the U.S. Food and Drug Administration for treatment of hypertension in children, including diuretics, angiotensin-converting enzyme inhibitors, angiotensin II receptor blockers, beta blockers, calcium channel blockers and direct vasodilators. Pharmacologic therapy should be initiated with a single drug. The goal of blood pressure treatment in children is reduction of blood pressure to less than the 95th percentile, unless other conditions are present, such as chronic renal disease or diabetes. In those cases, blood pressure should be lowered to less than the 90th percentile.