Case Study: Diagnosing Malignant Hypertensive Retinopathy

Exam findings and a patient’s history of hypertension at a young age helped lead doctors to a diagnosis of malignant hypertensive retinopathy in a teen.

A 19-year-old male college student presented to the ER with a four-day history of left eye pain and redness that began after a sneeze.

“He initially felt a ‘pop’ in his left eye, then experienced pain in the left eye while in the bathroom,” says ophthalmologist Dan Feiler, MD, a resident at Cleveland Clinic’s Cole Eye Institute. “When he came to the ER, he also had tearing, blurry vision and photosensitivity in his left eye.”

The patient had no history of trauma, recent illness, sudden vision loss, flashes or floaters, painful extraocular muscle, diplopia or any relevant family history.

Previous medical history

The patient was born at 27 weeks and had a history of asthma, obstructive sleep apnea and allergic rhinitis. He also had a history of hypertension at age 10 and subsequently developed chronic kidney disease at age 16. His medications included amlodipine, carvedilol, furosemide, hydralazine, isosorbide mononitrate (Imdur®), atorvastatin, albuterol, cetirizine and montelukast (Singulair®).

Base exam findings

On examination, the patient’s visual acuity without refractive correction was 20/25 in the right eye (pin hole 20/20) and 20/400 in the left eye (pin hole 20/100).

“Intraocular pressure via pneumotonometer was16 in the right eye and 13.5 in the left,” Dr. Feiler says. “Visual fields in both eyes were full to confrontation. The patient had full extraocular muscle function in both eyes and orthophoria. Pupils of both eyes were symmetric and equally reactive with no afferent pupillary defect.”

When he first presented to the ER, his blood pressure was 262/160. The patient has a history of a negative workup for secondary causes of hypertension by endocrinology.

Anterior exam findings

Lids, lashes and lacrimation in both eyes were within normal limits.

“On examination of the conjunctiva/sclera, the right eye demonstrated no injection while the left had 2+ injection,” Dr. Feiler says.

Both corneas were within normal limits. The anterior chambers of both eyes were deep and quiet and the iris stroma was within normal limits. Both lenses were clear.

Differential

The differential for a patient with bilateral disc edema and hard exudates with macular star, as seen in this case, includes the following:

• Optic neuritis
• Hypertensive retinopathy
• Elevated intracranial pressure (ICP)
• Bilateral central retinal vein occlusion (CRVO)
• Optic disc vasculitis
• Anterior ischemic optic neuropathy
• Diabetic papillopathy
• Leber’s hereditary optic neuropathy
• Central serous chorioretinopathy
• Compressive lesion

Diagnosis

“The diagnoses that seemed most likely based on the patient’s history and our exam findings were optic neuritis, hypertensive retinopathy, elevated intracranial pressure or bilateral central retinal vein occlusion,” says Dr. Feiler. “Bilateral CRVO was less likely due to a lack of significant intra/preretinal blood, although a hyperviscosity state could present similarly to this. In this case, a workup for optic neuritis or elevated ICP wasn’t necessary because of the patient’s history of hypertension, his blood pressure at the time and our exam findings.”

The patient was diagnosed with malignant hypertensive retinopathy based on the following pertinent findings:

• History of hypertension
• BP: 262/160
• OD: 20/25 ph 20/20
• OS: 20/400 ph 20/100
• No APD
• OD: 4+ edema, exudate, cotton wool spots
• OS: 4+ edema, exudate, cotton wool spots

Management

The patient’s blood pressure was controlled by antihypertensives, although when he returned to the clinic for his six-week follow-up, he had stopped taking his medications one week prior because he ran out. “He was also given extensive counseling on how important it was to take his medications and to follow up with his primary care physician,” Dr. Feiler says.

Treatment course

On initial exam, the patient’s visual acuity without refractive correction was 20/25 in the right eye (ph 20/20) and 20/400 in the left eye (ph 20/100). He had 4+ edema in both eyes with exudate and cotton wool spots, and no APD. When examined two and a half weeks later, the patient’s visual acuity without refractive correction was 20/20 in the right eye and his visual acuity was count fingers at three feet in the left eye. During this exam, the left eye was positive for an APD. He still had 4+ edema in both eyes with exudate and cotton wool spots. Six weeks later, the patient’s visual acuity without refractive correction was 20/20 in the right eye and 20/30- in the left eye and the APD had resolved.

Challenges

The patient did not show up for his most recent primary care physician or eye clinic visits.

“A diagnosis of malignant hypertensive retinopathy can be challenging for this patient population because the patient is young, but an adult by legal standards,” says Dr. Feiler. “Malignant hypertensive retinopathy is also a primarily asymptomatic disease and is treated with antihypertensive drugs, which can have unpleasant side effects.”