After suffering three spontaneous pneumothoraces, Mr A was due to have definitive treatment in the form of video-assisted thoracoscopy and pleurodesis. Soon after induction of anaesthesia he developed severe hypertension; 130/80 →300/220 associated with global high take off and ST elevation on the ECG. Labetol was given i.v. after which the blood pressure became unrecordable and an EMD cardiac arrest ensued. Three cycles of cardiac massage and two boluses of adrenaline were given. An echocardiograph thereafter revealed global systolic dysfunction although an urgent coronary angiogram, performed in theatre, was normal. He was transferred to ITU and requiring an intra-aortic balloon pump to maintain his blood pressure. The following day he had made a full recovery.
Mr A described himself as perfectly fit and well and on direct questioning he denied experiencing any sinister symptoms at all. He maintained an active, unlimited and healthy lifestyle. His mother had died of a cardiac arrest at the age of 50 years.
Mr A had been diagnosed with hypertension at the age of 24. This was picked up incidentally following an admission with a pneumothorax. His blood pressure remained well controlled on bendrofluazide and lisinopril.
Thorough examination following this near-fatal event revealed multiple café-au lait spots, axillary freckling and lisch nodules on the iris.
As expected, 24-hour catecholamines were diagnostic of a phaeochromocytoma.
Mr A was controlled on α and then β-blockers before having his 10 cm in diameter, left adrenal tumour removed. He is now off all antihypertensives and due once again to have his VATS procedure.
In conclusion, a cardiac arrest in theatre unveiled the unifying diagnosis of neurofibromatosis associated with phaeochromocytoma and recurrent pneumothoraces. Both recognised associations of neurofibromatosis Type 1.