ACROMEGALY

Authors


The Middlesex Hospital, London WIN 8AA.

SUMMARY

A personal series of 256 cases of acromegaly/gigantism seen over a 20-year period from 1963 is described. The insidious nature of the condition resulted in delay in diagnosis which was often made by a doctor when seeing the patient for an unrelated problem. Other features which commonly led to the diagnosis being made were headache, change in appearance, carpal tunnel syndrome, amenorrhoea and diabetes. The Hardy system for grading the radiological appearance of the pituitary tumour was used. Widely invasive tumours were not common but tended to occur in patients with younger age of onset and high GH levels. The occurrence of various symptoms and clinical features was noted and the changes resulting from reducing the GH level to normal. The incidence of hypertension, but not of coronary artery disease, is increased and the blood pressure may be reduced following successful treatment. The effects on the upper and lower respiratory tract are reported as well as sleep apnoea and problems associated with anaesthesia. Skin manifestations included sweating, pigmented skin tags, acanthosis nigricans and cutis verticis gyrata. In the skeletal system the incidence of kyphoscoliosis and osteoarthritis especially of the hip is reported: the question of hip replacement is discussed.

Diabetes mellitus disappeared in most cases if the acromegaly was cured. In men but not in women the incidence of colloid nodular goitre was increased as was hyperthyroidism in middle-aged women. In two patients a parathyroid adenoma was present: hypercalcaemia was present in five additional patients, but the cause was not determined. The common occurrence of amenorrhoea in the younger women was noted, it was not always associated with hyperprolactinaemia, and often responded to successful treatment of the acromegaly. The association of acromegaly with hirsutism and galactorrhoea is confirmed. The incidence of impotence and loss of libido in the men is discussed: in a proportion of those in whom the acromegaly was cured, potency returned, but in a number depression occurred and what was believed to be psychogenic impotence persisted. Hyperprolactinaemia was found in 49 out of 151 patients with active acromegaly in whom the prolactin level was measured.

Previous reports have indicated a doubling of death rates in acromegalics. In this series there were 47 deaths observed compared to 37.2 expected. The increased death rate was in women of all ages and in men under the age of 55. The increased deaths in the women were from cardiovascular and cerebrovascular causes and from breast cancer. To demonstrate cure it is emphasized that not only must the GH level be reduced to normal, but that suppression following a glucose load must be demonstrated and disappearance of abnormal responses for example to TRH, that would mark the persistence of adenoma cells, confirmed. Even so, late relapse may occur and GH levels should be checked regularly. Long-term follow up (mean 9.4 years) of 102 patients after transsphenoidal surgery is reported. Eighteen patients had X-ray therapy after transsphenoidal surgery and the GH level had been reduced to normal in 16 of these within 5 years.

Thirteen of the patients in this series died as a direct result of their acromegaly or its treatment. They were mostly young patients in whom the treatment was delayed, This delay is understandable, because this study covers the period when active treatment of acromegaly was being developed. The most important lesson to be learnt is that acromegaly/gigantism in a young person is still a disabling condition with high morbidity and mortality. In these patients early diagnosis and vigorous treatment with surgery and usually irradiation are essential.

Ancillary