A Big Headache: An Unusual Case of Hypertensive Urgency in an Adolescent

A 16-year-old Japanese American female presented to her primary care provider with 1 week of frontal headache, dizziness, and a sensation of “floating while walking.” Medical history was notable only for iron deficiency anemia secondary to menorrhagia and anxiety treated with venlafaxine. Her physical examination in clinic was notable for an upper extremity blood pressure of 148/93 mm Hg and a loud bruit over the left lumbar region. Renal ultrasound ruled out renal artery stenosis but demonstrated weak and delayed blood flow suggestive of proximal obstruction of the abdominal aorta. She was referred to the emergency department. During intake, she had an upper extremity blood pressure of 220/124 mm Hg, with a 40 mm Hg difference in systolic pressure between upper and lower extremities. She did not report fever, abdominal pain, vomiting, or vision changes, but endorsed persistent headache as well as a 6-month history of exertional dyspnea and lower extremity claudication. There was no family history of autoimmune or vascular conditions. She denied any alcohol intake or illicit drug use and was not sexually active. The patient had normal height and weight for age. Vital signs, aside from blood pressure, were within normal limits. She was alert, oriented, and had adequate urine output. Physical examination was notable for epigastric and lumbar bruits. Additionally, radial pulses were 2+ bilaterally, while dorsalis pedis and tibial pulses were barely palpable. The remainder of the physical examination was unremarkable. Initial laboratory testing was notable for a platelet count of 420 × 109/L, a C-reactive protein (CRP) of 0.28 mg/dL (normal ≤0.6 mg/dL) and erythrocyte sedimentation rate of 24 mm/h (normal ≤13 mm/h). Serum creatinine was 0.7 mg/dL. She underwent computed tomography angiogram of the chest, abdomen, and pelvis, which revealed luminal narrowing of the suprarenal descending aorta to 0.6 × 0.6 cm (Figures 1 and 2). After admission to the hospital, magnetic resonance angiogram of the chest, abdomen, and pelvis confirmed severe narrowing of the suprarenal abdominal aorta without additional sites of vascular disease. Pediatric rheumatology was consulted. The differential for large vessel vasculitides included Takayasu arteritis (TA), Behçet syndrome, IgG (immunoglobulin G)4–related disease, and infectious aortitis. She had no other clinical manifestations of Behçet’s syndrome, serum total IgG4 was normal (1170 mg/dL), and she tested negative for Epstein–Barr virus, cytomegalovirus, and tuberculosis.