Case history: A 26 year-old lady was admitted directly from the endocrine clinic with severe hypertension (BP 180/130 mmHg) and bilateral papilloedema. Six weeks prior to admission she had undergone resection of a 24 cm right upper quadrant lesion that was pre-operatively felt to be of hepatic origin. She was normotensive pre-operatively. Histology confirmed this to be an adrenal phaeochromocytoma with deficient SDH immunostaining. Pre-operative biochemical assessment had not been performed but there was no evidence of any blood pressure abnormality or variation pre- or peri-operatively. Two weeks post-operatively she began to experience episodes of palpitations, sweating and hypertension and had attended the emergency department of her local hospital on a number of occasions and been commenced on doxazosin.
Investigations: She was hypokalaemic on admission (K 3.2 mmol/l, Na 19 mmol/l) and given the large size of the original lesion and absence of a crisis during the original surgery, renovascular mediated hypertension was considered. Renin, aldosterone and a renal MRA were requested.
Results and treatment: She was admitted to the High Dependency Unit for invasive blood pressure monitoring and treatment. The MRA confirmed an infarcted right kidney with a patent accessory renal artery. A DMSA demonstrated only a 5% contribution from the right kidney. Hyper-reninaemic hyperaldosteronism was subsequently confirmed (renin 18.3 nmol/l per h NR 0.53.5 nmol/l per h, aldosterone 1,014 pmol/l NR 150550 pmol/l). She was discharged on losartan which was subsequently switched to candesartan. The blood pressure proved difficult to control and she was referred for renal artery embolization. On the day of the procedure she was found to be pregnant and the procedure was postponed. The pregnancy was considered high risk, the candesartan was discontinued and the blood pressure proved difficult to control with a further admission required to treat papilloedema. Genetic analysis subsequently confirmed she carried an underlying SDHB mutation (c.72+1G>T).
Conclusions and points for discussion: This is an unusual and interesting case of acute secondary hyperaldosteronism. We will discuss the risks of renal injury during phaeochromocytoma surgery, the complex medical management of this case outside and during a pregnancy and the results of the renal artery embolization.
08 - 10 Apr 2019
Society for Endocrinology