P29: Right Adrenal Phaeochromocytoma In A Boy With Hypertensive Crisis Initially Suspected To Be Due To An Intracranial Lesion

Introduction : Hypertension is uncommon in children. It is even rare for children to present with hypertensive crisis due to excessive adrenergic stimulation. We report a case of hypertensive crisis secondary to right adrenal phaeochromocytoma and its management. Case : A 14-year old Malay boy presented with worsening symptoms of hot flushes, sweating and palpitation for 3 years with 2 syncopal attacks 3 months prior to presentation. He was hypertensive (BP 180/100 mmHg) and tachycardic (110-120/min), with blurring of vision. Bilateral papiloedema was noted. Right subarachnoid cyst was found on CT brain and later decompressed. However, hypertension persisted and was difficult to control. Further investigations showed marked elevation of urine normetanephrine (91.15 umol/L). Right suprarenal mass (4.1 x 4.3 x 4.8cm) was found on CT scan. MIBG (methyl- iodobenzylguanidine) scan was positive. Short (prazosin) and longacting (phenoxybenzamine) alpha blockers successfully controlled the hypertension. Normal saline infusion was given for five days prior to operation to sustain his intravascular volume. Intra operatively he had a brief hypotensive episode which responded to noradrenaline infusion. He developed heart failure secondary to fluid overload 48 hours post-operation and was successfully treated with diuretics. His blood pressure normalised five days post-operation. Histopathological report confirmed malignant phaeochromocytoma of right adrenal. A repeat 24-hour urine test showed normal levels of catecholamines and its metabolites.