Hypertensive crisis in an 11-year-old girl with kidney and inferior vena cava abnormalities and leg thrombosis: Questions

Excerpt

An 11-year-old girl was admitted to the hospital suffering from severe headache, eye pain, diplopia, loss of appetite, and vomiting lasting for 3 days. At the emergency department, the patient was somnolent with blood pressure (BP) of 180/130 mmHg. Electrolyte and biochemical analysis did not reveal any abnormal findings. Fundoscopy showed bilateral papilledema. Therefore, a brain computed tomography (CT) was performed with no signs of any pathologic lesions. Echocardiogram revealed left ventricular hypertrophy (LVH). Kidney ultrasound revealed hypoplastic left kidney (length 5.3 cm, < 5th centile [1]), while the right kidney showed compensatory hypertrophy (length 11.2 cm, > 95th centile [1]). The blood flow to the left renal artery measured by a Doppler ultrasound was not detectable. Therefore, kidney CT angiography was performed, which demonstrated severely stenotic left renal and left polar renal artery without segmental stenosis and an absence of the infrarenal segment of the inferior vena cava (IVC) (Fig. 1) with a collateral drainage through azygous and hemiazygos veins. DMSA kidney scan showed severe hypofunction of the left kidney (14%). The glomerular filtration rate (GFR) calculated by Schwartz equation [2] was 81 ml/min/1.73 m², urinalysis was negative, and 24-h urine protein excretion was 67 mg/m²/day; microalbuminuria was 2.07 mg/mmol.

…