Examine this patient's face.

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



· Ask the patient about old photographs of him/herself for comparison.

· Ask whether the patient has outgrown wedding ring and shoes.

· Hyperhidrosis.

· Headaches, visual field defects (depends on the size of the tumour).

· Paraesthesia and symptoms of carpal tunnel syndrome (p. 212).

· Hypertension.

· Acral and facial changes.

* Oligomenorrhoea/amenorrhoea, galactorrhoea in females.

· Impotence in males.

· Shortness of breath (cardiac failure).

· Arthritis (hands, feet, hips and knees).



. 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.

Proceed as follows:

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).

Proceed as follows:

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).


Would you like to ask the patient a few questions?

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


Mention some causes of macroglossia.

· Acromegaly.

· Amyloidosis.

· Hypothyroidism.

· Down's syndrome.

How can this condition present?

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.

What are the complications of acromegaly?

· 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.

What are the indicators of disease activity?

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

· Excessive sweating.

· Skin tags.

· Glycosuria.

· Hypertension.

· Increased loss of visual fields.

How would you investigate this patient?

Biochemical tests

· 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).

Remember that, unlike in Cushing's disease and prolactinomas, the majority of the patients with acromegaly have macroadenomas.

Other investigations

· Formal perimetry.

· Obtain old photos.

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

· ECG.

· Triple stimulation test if hypopituitarism is suspected.

What therapeutic options are available?

· 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.

Mention four common causes of death in such patients.

· Cardiac failure.

· Tumour expansion (mass effect and haemorrhages).

· Effects of hypertension.

· Degenerative vascular disease.

Mention other conditions in which there is excess growth hormone secretion.

· 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.