A Child with Skin Lesions, Absent Upper Limb Pulses and Hypertension
A 13-year-old boy presented after an episode of generalized tonic clonic convulsions followed by altered sensorium. On examination, upper limb pulses were not palpable, blood pressure in the right lower limb was 150/110 mm Hg, and left lower limb was 160/120 mm Hg. He had hemihypertrophy of the left half of his face. He had multiple, irregularly shaped, evenly pigmented, brown macules, ≥ 0.5 cm each, all over his body. Slit lamp examination of the iris revealed presence of multiple whitish nodules; the fundi, were normal. There was no abdominal bruit heard. His father was diagnosed with hypertension at age 28 years. He died four years later due to neuro-sarcoma of the mediastinum. His brother age 8 years has also been found to be a hypertensive. The arterial doppler study of both upper limbs revealed chronic total occlusion of bilateral subclavian, axillary arteries with severely dampened patchy flow due to collaterals in the distal brachial artery. Computerized tomography of brain was normal. A contrast enhanced magnetic resonance angiogram of renal arteries was done (Figure 1).
Figure 1.
Magnetic resonance imaging.
What does the contrast enhanced MRA show?
Introduction
A 13-year-old boy presented after an episode of generalized tonic clonic convulsions followed by altered sensorium. On examination, upper limb pulses were not palpable, blood pressure in the right lower limb was 150/110 mm Hg, and left lower limb was 160/120 mm Hg. He had hemihypertrophy of the left half of his face. He had multiple, irregularly shaped, evenly pigmented, brown macules, ≥ 0.5 cm each, all over his body. Slit lamp examination of the iris revealed presence of multiple whitish nodules; the fundi, were normal. There was no abdominal bruit heard. His father was diagnosed with hypertension at age 28 years. He died four years later due to neuro-sarcoma of the mediastinum. His brother age 8 years has also been found to be a hypertensive. The arterial doppler study of both upper limbs revealed chronic total occlusion of bilateral subclavian, axillary arteries with severely dampened patchy flow due to collaterals in the distal brachial artery. Computerized tomography of brain was normal. A contrast enhanced magnetic resonance angiogram of renal arteries was done (Figure 1).
Figure 1.
Magnetic resonance imaging.
What does the contrast enhanced MRA show?
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