A 50 year old lady undergoing elective surgery for pyloric stenosis secondary to chronic ulceration, was diagnosed with primary hyperparathyroidism (calcium 3 mmol/l), elevated parathyroid hormone (PTH). Following exploratory parathyroidectomy, left and right superior parathyroid adenomas were removed. 2 years later she re-presented with hypercalcaemia (Calcium 2.74 mmol/l, PTH 26.5 pmol/l (1.16.8 pmol/l)) and appeared Cushingoid. Investigations confirmed Cushings disease (LDDST 48 hr cortisol failed to suppress, suppression greater than 50% on HDDST) and Acromegaly (GH 16.6 mU/l, failed to suppress on OGTT). MRI pituitary revealed a right-sided adenoma. The diagnosis of MEN1 was established. The patient proceeded to trans-sphenoidal surgery followed by radiotherapy with subsequent remission of Acromegaly and Cushings. Parathyroid scinitgraphy and venous sampling results were consistent with a parathyroid adenoma on the left but second neck dissection found no abnormal parathyroid tissue. Post-operative calcium remained elevated requiring intermittent bisphosphonate infusions. Abdominal radiograph demonstrated nephrocalcinosis. Follow-up imaging and venous sampling confirmed a right lower parathyroid adenoma (elevation of PTH in right superior thyroid vein) removed at third parathyroidectomy. Post-operative calcium was low (1.84 mmol/l) (PTH 0.4 pmol/L), requiring short-term calcichew and alphacalcidol. Six months later calcium 2.48 mmol/l, PTH 3.0 pmol/l. One parathyroid gland remains in-situ. Primary hyperparathyroidism in MEN1 involves hyperplasia of all 4 glands. Surgery to remove all four glands renders the patient permanently hypoparathyroid and dependent on alphacalcidol and calcium supplements for life, which can cause hypercalciuria and renal stones. An alternative is the removal of 3 or 3.5 glands (with tagging of the remaining parathyroid, or reimplantation to a more accessible site). The patient then remains at risk of recurrence, as with our patient, and further surgery may be complicated by scar tissue. Our practise is to give the patient informed choice regarding the surgical options. All patients require life long follow up.
06 - 07 Nov 2006
Society for Endocrinology