A 51-year-old black female presented complaining of a black spot that appeared in front of her left eye for more than two weeks. Although the spot decreased in size, she said that it still blocked her vision. She said the spot was constant and only disappeared when she closed her left eye. The patient reported no diplopia, headaches, pain, redness, tearing, discharge, itchiness, flashes or floaters. She wore over-the-counter reading glasses.

Her ocular history was remarkable for a corneal abrasion O.D. in 1995. This occurred secondary to glass from a car accident and resolved without sequelae. Three years ago, on her only other visit to our clinic, she had a small peripheral retinal hole with an operculum O.S. but did not require treatment.

The patients medical history was significant for hypertension for the past seven years. She was not taking any medication. Her last medical exam was a year earlier, and she was unaware of her last blood pressure reading.

Her constitutional health that day was fine, she reported. She was not obese, and she appeared mentally intact and alert during the exam. Social history was negative for alcohol or drug use, but the patient said she smoked cigarettes. Pertinent family history included a sister with diabetes.

Diagnostic Data
Best-corrected visual acuity at distance was 20/25 O.D. and 20/60 O.S.; there was no improvement with pinhole. Pupils were equal, round and reactive to light, with no afferent pupillary defect. Confrontation fields were full to paracentral and peripheral finger counting.

Amsler grid testing revealed a large scotoma in the left eye that affected the center of vision. The scotoma was slightly displaced in the superior nasal portion of the eye. The patient reported the area was light black in color.

Extraocular movements were full and smooth. Ishihara color plates yielded 7/7 O.D. and 0/7 O.S. Slit lamp examination revealed healthy anterior segments. Intraocular pressure measured 20mm Hg O.U.

Blood pressure was measured at 260/145 (right arm sitting).

1, 2. Undilated fundus examination (O.D. left, O.S. right) revealed bilateral, severely swollen optic nerves with an obliterated cup.

Undilated fundus exam with a 90.00D lens revealed bilateral, severely swollen optic nerves, each with an obliterated cup (figures 1 and 2). Flame-shaped and pre-retinal hemorrhages and cotton-wool spots surrounded both optic nerves and extended throughout the arcades. There was a severe exudative response present O.U.; exudates spilled from the optic nerve toward the macula (figures 3 and 4) and extended into the macula O.S.

3,4. A severe exudative response was present, with exudates spilling from the optic nerve toward the macula (O.D. left, O.S. right).

We diagnosed this patient with malignant hypertensive retinopathy that was secondary to malignant hypertension.

Treatment and Follow-up
Upon further questioning, the patient admitted that she stopped taking her hypertension medications eight months earlier because they made her heart race, and she felt as though she would have a heart attack. According to our records from three years earlier, she was taking hydrochlorothiazide and diltiazem. She never returned to see her doctor after she discontinued the medications, and she did not check her blood pressure.

She also reported episodes of nausea during the previous week and said she vomited the night before her eye exam. She reported no vertigo, tinnitus, headache or confusion. She had walked several blocks to her appointment that day and said she was feeling fine.

We called an ambulance to take her to the emergency room immediately to bring her blood pressure under control.

At the first follow-up visit 12 days later, she reported that she was admitted to the hospital until her blood pressure was lowered. Her medical history now included a heart murmur and kidney problems, for which she was undergoing testing. She now took metoprolol and Norvasc (amlodipine besylate, Pfizer).

Visual acuity had improved to 20/20 O.D. and 20/30 O.S. Confrontation fields were full to finger counting, but she still reported central haze that was slightly displaced in the superior nasal portion in her field of view. Her color vision had improved slightly to 7/7 O.D. and 3/7 O.S. (The 0/7 color vision O.S. at the initial presentation was probably due to the decreased vision and decline in axoplasmic flow from the swollen optic nerve. As the swelling decreases and her vision improves, so does her color vision). Blood pressure measured 153/100, right arm sitting.

5, 6. Twelve days after initial presentation, dilated fundus examination (O.D. left, O.S. right) revealed bilateral, swollen optic nerves with resolving peripapillary cotton-wool spots and retinal hemorrhages.

Dilated fundus exam revealed bilateral, swollen optic nerves with resolving peripapillary cotton-wool spots and retinal hemorrhages (figures 5 and 6). The exudative response appeared to have worsened. Exudates now leaked from the optic nerve in an arrow-type appearance toward the macula (figures 7 and 8). There were exudates and mild edema in the left macula.

 7, 8. At this same visit, exudates and mild edema were present in the macula O.S.

One month after her initial presentation, she was under the care of her primary-care doctor, a cardiologist and a nephrologist. She now took metoprolol, Norvasc, hydrochlorothiazide and Diovan (valsartan, Novartis).

Visual acuity was now 20/20 O.D. and 20/30 O.S. Blood pressure was 145/75, right arm sitting.

9, 10. One month after her initial presentation, there was still some evidence of cotton-wool spots and the resolving hemorrhages (O.D. left, O.S. right).

Dilated fundus exam revealed bilateral, swollen optic nerves that were slowly improving. Each cup was becoming more distinguishable with time. There was still some evidence of the cotton-wool spots and resolving hemorrhages (figures 9 and 10). The exudative response was still very pronounced, with an arrow-type appearance toward the macula O.U.; this was greater O.S. than O.D. (figures 11 and 12).

11, 12. The exudative response was still very pronounced, with an arrow-type appearance toward both maculae. This was greater O.S. (right) than O.D. (left).

Elevated blood pressure associated with evidence of acute target-organ damageneurological, cardiac, renal and obstetricis considered a hypertensive emergency.1,2 The initial level of function of such target organs as the brain, heart and kidneys is more indicative of an emergency than the actual level of blood pressure.1

The World Health Organization classifies malignant hypertension as severe hypertension (diastolic blood pressure greater than 140mm Hg) with bilateral retinal hemorrhages and/or exudates, with or without papilledema.3-5Papilledema once was necessary for a diagnosis of malignant hypertension, but given the poor prognosis for survival, it is no longer a part of the definition.4,5

Papilledema secondary to severe hypertension may have several etiologies. These include ischemia, elevated intracranial pressure or as a sequela to the hypertensive encephalopathy.6 Fibrinoid necrosis of arterioles in many tissue beds is a hallmark of malignant hypertension but are not seen in benign hypertension.7 Clinical evidence of target end organ damage is part of the malignant hypertension syndrome.8

Malignant hypertension still exists in developed countries but is more prevalent in underdeveloped nations. It most often occurs secondary to poor compliance with hypertensive medications. Malignant hypertension typically affects a relatively young population; the mean age is 50, according to one study.9 These patients tend to be of lower socioeconomic status and may have had inadequate access to health care and/or medications.7 There is a higher incidence of smoking among patients who develop malignant hypertension vs. the general population.10

Hypertensive emergencies occur with an incidence of about one to two per 100,000 people per year.1 Fewer than 1% of hypertensive patients develop the malignant phase.11 Patients who have malignant hypertension often have a recent diagnosis of hypertension, suggesting that the severe spike in blood pressure develops acutely.

Recurrence of malignant hypertension is uncommon but can occur. The most common cause of recurrence: poor drug compliance.12

Symptoms of an insidious increase in blood pressure are numerous, and presentation varies among patients. Visual disturbance is the most frequent symptom on admission, and headache is the second most frequent.3,4 Some important symptoms to ask about include blurred vision, amaurosis fugax, vertigo or transient ischemic attacks.4 Patients may appear agitated or confused, and they may have a change in mental status. Nosebleeds and flushing of the face may occur, and acute abdominal symptoms may be present.4,8 Signs and symptoms of hypertensive encephalopathy include headaches, lethargy, nausea, vomiting, seizures and papilledema.4,13

Ocular manifestations of systemic hypertension include:

Hypertensive retinopathy. Findings include arteriolar narrowing, changes in the arteriolar light reflex, arteriovenous changes, intraretinal hemorrhages, cotton-wool spots, hard exudates and optic disc edema. We classify hypertensive retinopathy using the Keith-Wegener-Barker classification, a grading system developed by physicians Norman Macdonnel Keith, Henry Patrick Wagener and N.W. Barker (see Two Grading Systems for Hypertensive Patients).14

Two Grading Systems for Hypertensive Patients

Hypertensive Retinopathy

Grade     Findings
1 Mild arteriolar narrowing.
2 Focal arteriolar constrictions and arteriovenous nicking.
3 Findings from grades I and II, along with retinal hemorrhages, exudates and cotton-wool spots.
4 A severe presentation of grade III, plus papilledema.

Grade Findings
0 No arteriosclerosis.
1 Mild arteriole narrowing.
2 Obvious arteriole narrowing and an increased light reflex of the vessels.
3 Copper wiring appearance of the arteries, retinal hemorrhages and exudates.
4 Silver wiring appearance and papilledema.4

Hypertensive optic neuropathy. Severe acute retinopathy may be accompanied by hypertensive optic neuropathy with bilateral optic disc edema and the presence of a macular star.15 Optic disc edema and cotton-wool spots often form first, causing a stasis of venous outflow, resulting in retinal hemorrhages. The hemorrhages often begin circumpapillary and may extend throughout the posterior pole.15

Hypertensive choroidopathy. This is caused by ischemia. Fibrinoid necrosis produces occlusion of the choriocapillaris. On fluorescein angiography, the retinal pigment epithelium overlying areas of choriocapillaris occlusion becomes yellow and leaks fluorescein. The leaking ceases as the hypertension resolves, and these spots become pigmented. This is the origin of Elschnig spotsmultiple, round, pigmented lesions that manifest long term after an acute episode of elevated blood pressure. Siegrist spotsmultiple, pigmented and depigmented lesionspresent in a linear fashion and have an etiology similar to that of Elschnig spots.

Additional conditions that may occur secondary to hypertension include retinal vein occlusion, retinal arteriole macroaneurysms, non-arteritic ischemic optic neuropathy, cranial nerve palsies and diabetic retinopathy of increased severity.6

The severity of hypertensive retinopathy does not always correlate with the degree of severity of the systemic hypertension.16-21 For example, severe hypertensive retinopathy may be present even in patients who have mild hypertension.16 Several studies have reported hypertensive-like retinal changes in individuals who had no history of hypertension.16-20 So, retinopathy is not a good indicator of the systemic severity of this disease.

The Blue Mountain Eye Study found that only 42% of all retinopathy, defined as microaneurysms and hemorrhages, could be explained by hypertension in older, nondiabetic subjects.16 The Beaver Dam Eye Study found similar results.18-20 Some 47% to 63% of patients in the Beaver Dam Study who did not have hypertension had arteriolar narrowing, arteriovenous nicking or some degree of retinopathy.16 Another study found arteriovenous crossing changes in 43% of normotensive men ages 55 to 60.15,22 Abnormalities in the retinal vasculature may be the result of arteriosclerosis as well as hypertension.6,15,23 (Scheie developed a system to grade the degree of arteriosclerosis.14)

Another study found that retinal changes are the most frequent expression of target organ damage, yet these changes overestimate the amount of damage done.18 This study found that very few patients actually show advanced retinal vessel abnormalities, that arteriole changes may represent changes from arteriosclerosis rather than hypertension and that there is a significant degree of inter- and intrapractitioner variability.18

The prognosis for patients who have malignant hypertension has improved in recent years.6 Patients who develop papilledema have survival rates similar to those who present with elevated blood pressure without papilledema.10 Blacks have a reduced survival rate vs. whites.12

If untreated, however, malignant hypertension has a mortality rate of more than 90% within one year and a five-year survival rate of 1%.7,13 Those numbers dramatically decrease with the appropriate treatment. Individuals who fail to keep their diastolic blood pressure below 100mm Hg following an episode of malignant hypertension have a significantly reduced survival rate. Many of the retinal manifestations of malignant hypertensive retinopathy resolve within one year, if the blood pressure is lowered to an acceptable level.6

Recognizing and diagnosing malignant hypertension is essential given the dramatic difference in survival rate of those treated vs. those left untreated. Once you diagnose the patient, call an ambulance to transport the patient directly to an urgent care facility to be monitored in-house for at least 24 hours. Notify the attending physician if the patient has papilledema because this is critical in determining how quickly the blood pressure is lowered. While lowering the blood pressure is imperitive, it must be done slowly, especially when papilledema is involved. Do not to dilate these patients because the phenylephrine may have an adverse effect.

The current medical regimen for malignant hypertension is to promptly begin lowering the blood pressure graduallygenerally about 10% in the first hour and another 15% in the next two to three hours.24 Initial testing should also evaluate any target organ damage.1

Prevention and Management of Hypertension
The National High Blood Pressure Education Program presented the Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation and Treatment of High Blood Pressurealso known as JNC 7in August 2004. The report offers these messages about risk of cardiovascular disease and treatment of hypertension:25

Systolic blood pressure (BP) greater than 140mm Hg is a more important risk factor for cardiovascular disease (CVD) than diastolic BP in patients past age 50. Beginning at 115/75 mm Hg, the risk of CVD doubles for each increment of 20/10mm Hg. Patients who are normotensive at age 55 have a 90% lifetime risk of developing hypertension.

Prehypertensive individuals (systolic BP of 120mm Hg to 139mm Hg or diastolic BP of 80mm Hg to 89mm Hg) require lifestyle modifications. This can help prevent the progressive rise in blood pressure and CVD.

A thiazide diuretic used alone or combined with drugs from other classes is warranted for treating uncomplicated hypertension. However, patients who have diabetes and chronic kidney disease require two or more antihypertensive medications to achieve a goal BP (<140/90mm hg or <130/80mm hg). Patients whose BP is more than 20mm Hg above the systolic BP goal or more than 10mm Hg above the diastolic BP goal may require therapy with two agents; one of these usually is a thiazide diuretic. Hypertension can only be controlled if patients are motivated to stay on their treatment plan.

Untreated malignant hypertension can cause serious ocular manifestations and is life threatening. We must recognize this disease and ensure that patients seek medical attention immediately.

Dr. Pukl completed a primary-care residency at Pennsylvania College of Optometry (PCO) and is now in private practice in Warwick, R.I. Dr. Chronister is an associate professor at PCO and chief of the Primary Care Module at the colleges Eye Institute.

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Vol. No: 142:11Issue: 11/15/2005