Acromegaly

Examine this patient's face.

This patient complains of excessive sweating; examine him. Examine this patient's hands.

. On shaking hands there is excessive sweating - moist, doughy, enveloping handshake.

· Large hands with broad palms, spatulate fingers; there is an increase in the 'volume' of the hands.

Look for evidence of carpal tunnel syndrome (tap over the flexor retinaculum ).

· Prominent supraorbital ridges.

· Large nose and lips.

· Protrusion of the lower jaw (prognathism); ask the patient to clench his teeth and note the malocclusion and splaying of the teeth (i.e. interdental separation).

Ask the patient to show his tongue and look for macroglossia and for impressions of the teeth on the edges of the tongue .

· For bitemporal hemianopia and optic atrophy.

· Neck for goitre.

· Axillae for skin tags (molluscum fibrosum), acanthosis nigricans (black velvety papillomas).

· Chest for cardiomegaly, gynaecomastia and galactorrhoea.

· Abdomen for hepatosplenomegaly.

· Joints for arthropathy, i.e. osteoarthrosis, chondrocalcinosis.

· Spine for kyphosis.

· Blood pressure for hypertension (present in 15% of cases).

· Tell the examiner that you would like to examine the urine for sugar (impaired glucose tolerance).

This patient has a protruding lower jaw, splaying of teeth and large spade-like hands (lesions) which are features of acromegaly due to a pituitary tumour (aetiology).

Ask about any increase in size of shoes, gloves and hat, and whether the wedding ring is tight to wear.

· Acromegaly.

· Amyloidosis.

· Hypothyroidism.

· Down's syndrome.

One third of patients notice a change in their features, one third are noticed to have a change in their features by their GP, and the remaining third have symptoms such as excessive sweating and visual difficulties.

· Cardiomegaly and heart failure.

· Hypertension.

· Impaired glucose tolerance.

· Hypopituitarism.

· Carpal tunnel syndrome.

· Arthritis of the hip, knee and spine.

· Spinal stenosis resulting in cord compression.

· Visual field defects.

· Symptoms such as headache, increase in size of ring, shoe or dentures.

· Excessive sweating.

· Skin tags.

· Glycosuria.

· Hypertension.

· Increased loss of visual fields.

· Non-suppressibility of growth hormone levels to less than 2 ng/ml after oral administration of 100 g glucose.

· Plasma insulin-like growth factor levels (allow assessment of the efficacy of initial therapy and in the post-therapeutic period).

· Also serum T4, prolactin, testosterone; evaluate pituitary function - static and dynamic tests.

· Calcium levels (to exclude multiple endocrine neoplasia (MEN) type 1 syndrome).

· Pituitary fossa (enlarged sella).

· Sinuses.

· Chest (cardiomegaly).

· Hand (terminal phalangeal 'tufting').

· Foot (terminal phalangeal 'tufting', lateral view shows increased thickness of the heel pad).

· CT scan (MRI scan is more reliable).

· Formal perimetry.

· Obtain old photos.

· New photos of face, torso, hands on the chest.

· ECG.

· Triple stimulation test if hypopituitarism is suspected.

· Neurosurgical intervention - typically trans-sphenoidal - is the primary thera-peutic choice for almost all patients. Diaphoresis and carpal tunnel syndrome often improve within 1 day of surgery. Although growth hormone levels fall immediately, insulin-like growth factor levels fall gradually.

· Radiation therapy is a primary treatment option for a few patients who have acromegaly but are not surgical candidates.

· Octreotide and bromocriptine are valuable as adjunctive therapy.

Remember. The aim of treatment should be symptomatic control and a growth hormone concentration of less than 5 mU/L.

· Cardiac failure.

· Tumour expansion (mass effect and haemorrhages).

· Effects of hypertension.

· Degenerative vascular disease.

· Multiple endocrine neoplasia type 1 (parathyroid hyperplasia, pituitary tumours and gut tumours).

· McCune-Albright syndrome (polyostotic fibrous dysplasia, sexual precocity and caf6-au-lait spots).

· Carney complex, of autosomal dominant aetiology, which consists of multi-centric tumours in many organs (including cardiac myxomas, myxomas in breast, testes, pigmented skin lesions and pigmented nodular hyperplasia).

In 1886, Pierre Marie (1853-1940) used the term 'acromegaly' in describing two patients and reviewed eight previously published papers describing patients with presumed acromegaly (Marie P 1886 Sur deux cas d'acromegalie. Hypertrophie singuliere non-cong~nitale des extremites sup~rieures, inferieures et c~phaliques. Rev Med 6: 297-333). He also described Charcot-Marie-Tooth disease.

In 1887,Minkowski deduced that acromegaly is related to pituitary tumour (Minkowski O 1887 Uber einen Fall von Akromegalie. Berl Kiln Wochenschr 24: 371-4).

In 1891, the New Syndenham Society published a translation of Marie's original paper and a review by Souza-Leite of 48 patients (Marie P, Souza-Leite JD 1891 Essays on Acromegaly. London: New Sydenham Society).

Fuller Albright, Professor of Endocrinology at Massachussetts General Hospital and Harvard Medical School, whose chief interest was calcium metabolism.