Indian Journal of Pediatrics | 2021
Hypertensive Emergency with Medullary and Spinal Hemorrhage in Turner Syndrome
Abstract
An 8-y-female with Turner syndrome (TS) presented with an episode of seizure 7 d back followed byweakness of trunk and lower limbs. At admission, the blood pressure was 184/ 111 mmHg with no difference in all 4 limbs. Neurological examination revealed normal sensorium; lower limb weakness (power of 2/5 Medical Research Council grading), exaggerated deep tendon reflexes, and extensor plantar reflex; saddle anesthesia; and intermittent urinary retention suggesting conus medullaris syndrome (CMS). Magnetic resonance imaging (MRI) showed medullary and spinal hemorrhage (Fig. 1). Hypertensive emergency was managed in pediatric intensive care unit (PICU) with intravenous infusion of sodium nitroprusside and labetalol; and oral labetalol, amlodipine, enalapril, and clonidine. Complete blood counts, renal, liver, and thyroid function tests; serum cortisol; and ultrasonography of kidney, and renal Doppler were normal . Echocardiography revealed left ventricular hypertrophy and fundus examination showed Grade 4 hypertensive retinopathy. Angiography ruled out vascular abnormalities in aorta, cerebral, spinal or renal vessels. Aldosterone/renin ratio was 4.8 (Normal < 20), aldosterone level 26.2 ng/dL (Normal: 3– 16 ng/dL), and plasma renin activity was 5.41 ng/mL/h (Normal: 0.15–2.33 ng/mL/h in supine position). She was discharged after a hospital stay of 36 dwith normal sensorium, lower limb power of 4/5, no urinary retention, and blood pressure at 90th centile. Hypertension is reported in 20%–25% children with Turner syndrome. It may be due to overweight, dyslipidemia, increased insulin resistance, endothelial dysfunction, abnormal sympathetic system, or renin-angiotensinaldosterone (RAAS) activation; or secondary to coarctation of aorta, renal artery stenosis [1]. The most common cause for Pediatric non-traumatic intracerebral and spinal hemorrhages are vascular malformations [2, 3]. Mostly the spinal cord vascular malformations are localized to T8 to L2 spinal cord segments [4]. Yu et al. [5] reported child with TS who presented with spinal hemorrhage due to rupture of cavernous malformation at T11–12 level. Hypertension induced intracranial and spinal hemorrhages are rare in children. Index child had medullary and spinal bleed at D12 level without vascular malformations. Hypertension due to elevated plasma renin activity could be the possible reason for intracranial and spinal hemorrhage in index child.