Lang CC, Wallace CG**, Browning GGP**, Davie R, Cargill R*, Campbell IWC*
Department of Cardiology, Royal Infirmary of Edinburgh, United Kingdom
*Victoria Hospital, Kirkcaldy, United Kingdom
**St John’s Hospital, Livingston, United Kingdom
Address for correspondence: Christopher Lang Department of Cardiology Royal Infirmary of Edinburgh Little France Crescent Edinburgh
Email: chrislang@katamail.com
SMJ 2006 51(2): 54
Presentation
A 38 year old female with a four year history of palpitations that had increased in severity and frequency in the preceding four months, was referred for out-patient cardiologic assessment by her family practitioner. She described an awareness of her heart beating forcefully, associated with severe nausea, occasional headache and leg weakness during these weekly, twenty-minute attacks that were to some extent relieved by vomiting. Observers had noted the colour draining from her face when symptomatic. She smoked 20 cigarettes per day and worked as a glass blower.
The patient had a past medical history of a stillbirth when aged 24, and a second pregnancy complicated by intra-uterine growth retardation. Five months later she was inconclusively investigated for transient abdominal aches, described as a tight band encircling her central abdomen. When aged 28, she was investigated for paroxysmal peripheral paraesthesia by a neurologist, but no abnormalities were found.
She was noted to be normotensive (mean reading 110/70 mmHg) on several occasions; physical examination and routine blood tests were normal.
Investigations
Twenty-four hour ambulatory electrocardiographic monitoring captured an attack, and was marked on the recording by the patient. Analysis showed a corresponding junctional rhythm at a rate of 50 bpm. The recording was otherwise normal.
Management
It was thought that her symptoms might be attributable to vagal hyperactivity, and she was commenced on regular metoclopramide, with consequent improvement in her nausea but no reduction in the frequency or severity of palpitations. Propranolol 40mg tds was therefore added to her therapy.
Follow-up
At cardiological review three months later she had unfortunately suffered sudden loss of vision in one eye during a recent attack. The attending ophthalmologist attributed this to the concurrent vomiting. The cardiologist suspected that both her retinal haemorrhage and ongoing symptoms could be secondary to hypertensive crises, and instigated a 24h urine collection for metadrenalines. These were highly suggestive of a phaeochromocytoma, with a 24h urinary metadrenaline of 24.5 umol/24h (normal range 0.3 – 1.7umol/24h), and normetadrenaline of 5.9 umol/24h (normal range 0.4 – 3.4umol/24h). A non-contrast abdominal CT scan demonstrated a 5cm diameter mass arising from the left adrenal gland (Figure 1). No metastatic disease was found on CT or abdominal ultrasound scans. The ophthalmologists were contacted to alert them of the diagnosis, but she had already undergone surgery and become markedly hypertensive intra-operatively.
Combined alpha and beta-blockade was commenced in the form of phenoxybenzamine 10mg o.d. and bisoprolol 5 mg o.d., although up-titration of the dose was limited by postural hypotension. Elective open left adrenalectomy was uncomplicated, and histology confirmed a benign phaeochromocytoma (Figures 2 and 3). Multiple endocrine neoplasia (MEN) was discounted by normal urinary parathormone and serum calcitonin levels. The patient made an excellent recovery following surgery.
Discussion
Phaeochromocytoma is a very rare tumour of which every clinician is aware. The Mayo Clinic described 15 phaeochromocytomas from a series of 15,984 post mortem examinations. In a series of 507 cases (1), 64% of patients had persistent and 24% paroxysmal hypertension, the remainder being normotensive. Palpitations and headache are common presenting features.
Our case is unusual in that there were documented brady-arrhythmias during hypertensive crises, although there are occasional case reports (2,3). The bradycardia may be due to a response to stimulation of arterial baroreceptors during crises. The second unusual feature is that the diagnosis was made after the patient re-presented with a retinal haemorrhage. The ophthalmologist attributed this to a peri-emetic surge in arterial and venous pressure, but with hindsight this may well have been secondary to a hypertensive crisis. There were no hypertensive changes visible in either eye at this time. There are no reports in the literature of phaeochromocytoma being suspected and diagnosed based on retinal haemorrhage.
A full history with consideration for a possible phaeochromocytoma is mandatory when assessing patients presenting with peri-emetic retinal haemorrhage, in order to minimise the risk of potentially catastrophic hypertensive crisis during elective ophthalmic surgery.
With
the benefit of hindsight, it might be reasonable to suspect that her past
history of obstetric problems and abdominal and neurological symptoms could
possibly have been early manifestations of the developing phaeochromocytoma.
References
1.
Human Tumours Secreting Catecholamines. Clinical and Physiopathological Study of
the Phaeochromocytomas. Hermann H, Mornex R. Pergamon Press, New York. 1964.
2. Reflex
bradycardia and nodal escape rhythm in pheochromocytoma. Forde TP, Yormak SS,
Killip T. Am Heart J. 1968; 76 (3):388-92.
3.
Twenty-four
hour ambulatory blood pressure and heart rate in a patient with a predominantly
adrenaline secreting phaeochromocytoma. Gallen
IW, Taylor RS, Salzmann
MB, Tooke JE. Postgrad Med J. 1994 Aug;70(826):589-91