Orlo Clark delivered the Historical Lecture entitled “Remarkables: endocrine abnormalities in art” at the recent IAES meeting in Montreal during International Surgical Week and is shortly to publish his book of the same title. It is well known that artists often truly depict anatomical realities associated with disease, including those depicting endocrine disorders. In his lecture Orlo Clark examined the aesthetic, social, cultural, spiritual and historical context in which these depictions take place. Such abnormalities are generally referred to in the art literature as “Remarkables”, “Freakers”, or “Monsters” with the relevant abnormality faithfully portrayed by an often unknowing artist. This has been recognised for many years, indeed there are a number of medical school nowadays which incorporate art appreciation into the curriculum based on evidence that art is an effective technique to train doctors eyes to assess physical features in of the human form. Perhaps the commonest endocrine abnormality depicted in art is that of a goitre. For example many artists have chose models with diffuse goitres to depict the Madonna, perhaps because such a smooth swelling was seen as a thing of beauty. In early days the thyroid was considered to act as a “cushion” smoothing and filling out the contours of the neck between the carotid vessels and trachea. Holbein’s Adam and Eve shows such a diffuse goitre in Eve whilst Adam has a nodular goitre. The difficulty in differentiating between a “fat neck” and a true goitre was achieved by examining various paintings by the same painter using the same model but from different aspects. Of course many other endocrine disorders, such as pituitary disease, can be recognised in art. Dwarfism was commonly portrayed in Renaissance paintings with a number clearly recognisable as due to pituitary deficiency. Such dwarfs were generally in the foreground and associated with animals, giving an idea of their place in society. On the other hand gigantism can be seen in van Oost’s “David Bearing the Head of Goliath” and acromegaly can be seen in Serusier’s “House ou la Seratus Bretone??” Addison’s Disease is apparent in Raphael’s “Fire in the Borgo”, demonstrating obvious weakness and palmar crease pigmentation.
Our understanding of thyroid physiology is largely based upon the outcomes of experimental thyroid surgery in animals. Schiff (1859) first described the striking results of ablation of the thyroid in experimental animals but it was not until 1884 that the matter was put on a firm basis by the same observer. He then also showed that by means of homotransplants of the thyroid, the consequences of thyroidectomy could be avoided, and foreshadowed the possibility of emulsions of the gland having a similar effect. Many of the earlier experiments were invalidated by the coincident removal of the parathyroids so that a complicated picture of thyroid and parathyroid deprivation resulted. Horsley (1886) by his expeiments on monkeys, followed up and confirmed Schiff’s work, although for a time Munk (1887) attributed Horsley’s results to injuries to the sympathetic nerves. Sutherland Simpson (1913) and Basinger (1916) working with sheep and rabbits respectively, obtained effects similar to those of Schiff and Horsley, although on the whole the severity of the symptoms in the herbivora was less than in the carnivora with which Schiff worked. The symptom complex which these animals showed was exactly similar to that of sporadic cretinism, know at the time to be associated with thyroid atrophy. In rabbits for example, the first indications are seen after two weeks. The hair becomes drier and can easily be pulled out. Retardation of growth is noted as early as the third week, reaching a maximum between the eighth and twelfth. The animals remain immobile for hours unless disturbed, and if stimulated, move awkwardly and slowly. The bones are short, the muscles flabby and too weak to support the body weight, while the abdomen is distended, as in the pot belly of the human cretin, and a characteristic posture is assumed.
From “Diseases of the Thyroid Gland”. Cecil A Joll, 1932. William Heinemann, London.
An invaluable adjunct in the pre-operative preparation of patients with Graves’ Disease, Lugol’s iodine markedly reduces both the size and vascularity of the gland – however that was not the intent of its creator. Jean Guillaume Auguste Lugol was born in 1786 at Montauban, France. He graduated MD Paris in 1812 and seven years later was appointed to the staff of the Hopital St Louis. His great interest in life was scrofula, a collective term applied to tuberculous lymph nodes, tuberculous bones , and tuberculous joints before it was known that these conditions were caused by the same organism that caused pulmonary tuberculosis. Scrofula was known for years as the “King’s Evil” because it was believed that anointed monarchs could cure sufferers from the malady by touching them. It is said that between 1662 and 1682, Charles II “touched” over 92,000 sufferers from the King’s Evil. Lugol’s first “Memoir on the Use of Iodine in Scrofulous Diseases” was read before the Royal Academy of Sciences of Paris in 1829. His treatment comprised fresh air, exercise, cold bathing and drugs. Above all, he placed his faith in iodine therapy. His aqueous solution of iodine (liquor iodide aquos) for oral administration consists of 5 per cent of iodine, 10 per cent of potassium iodide, and distilled water to make 100. The first recorded use of Lugol’s iodine in the management of Graves’ disease was by HS Plummer from the Mayo Clinic in 1924, where its routine use transformed surgery for Graves’ Disease into a much safer procedure with an acceptably low morbidity and mortality. Although less used nowadays, good data are available to show an effect with oral administration of Lugol’s iodine in causing a significant decrease in blood flow through the superior thyroid artery, as well as a significant reduction in thyroid hormone secretion.
Adapted from “Notable Names in Medicine and Surgery” H Bailey & WJ Bishop, HK Lewis & Co, London 1959.
Jerome Conn’s belief that adrenal adenomas are a common surgically correctable cause of “essential hypertension” may yet be proven correct. In 1954, two years after the discovery of aldosterone, Jerome Conn from Ann Arbor, Michigan, described the syndrome of primary aldosteronism. The first patient was a woman of 34 years who had intermittent tetany, paraesthesia, periodic muscular weakness and paralysis, polyuria, polydispsia, and mild hypertension, but no oedema. Laboratory tests showed severe hypokalaemic alkalosis, hypernatraemia, im-paired renal tubular absorption of water, and an excess of aldosterone in the utine. Conn considered that total adrenalectomy, followed by substitution therapy, should abolish the metabolic abnormality and William Baum, a urologist, operated on December 14th, 1954. He found a cortical adenoma on the right and removed the whole gland, taking a biopsy from the left. The tumour was round, 4cm in diameter, 14.8 g in weight and deep yellow on its cut surface. Microscopy showed an encapsulated cortical adenoma and atrophy of the zona glomerulosa in the other gland. The tumour contained much aldosterone. All the symptoms were relieved, the metabolic abnormalities were corrected in ten days, and the blood pressure fell to normal in eighteen days. Within six weeks of the first report, four other patients with primary aldosteronism were found elsewhere, and three were cured by removal of adenomas. This led Conn to advise that the electrolytes should be measured in hypertensive patients and, if hypokalaemic alkalosis were present, the adrenal glands should be explored surgically. Conn predicted that up to 20% of hypertensive patients may have adrenal adenomas, and thus be potentially cured of their disorder by surgery. Such a figure was never realised in his lifetime with most series reporting less than a 1% incidence of Conn’s tumours in hypertensive populations. However, in recent years, primary screening of normokalaemic hypertensive pop-ulations with plasma renin-aldosterone ratio, has shown that possibly 10% may have an aldosterone secreting adenoma - Jerome Conn may yet be proven right.
Adapted from “History of Endocrine Surgery”. R Welbourn. Praeger, New York. 1990:174.
Michael Bliss, historian from the University of Toronto, delivered an address at the recent AAES meeting in New York describing the interaction between Harvey Cushing, a surgeon, and the Endocrine Society which had just elected him one of its first presidents. Cushing was scathing about the budding specialty of endocrinology, regarding many of its members as charlatans who practiced “endo-criminology:” In 1920 Cushing was elected president of the Society for the Study of Internal Secretions (later to become the Endocrine Society) without his knowledge at a meeting he did not attend of a society dedicated to a discipline that he believed was mostly “poppycock” and for which he coined the term “endo-criminology”. He was well aware that much of what passed for “glandular therapy” was the old scam of peddling replacements for vital fluids. He only agreed to serve because of his ongoing pituitary interest, out of a sense of duty and to help suppress quacks. His 1921 Presidential address at the society’s Boston meeting room began with a beautiful image of the state of the field: “We find ourselves embarked on the fog-bound and poorly charted sea of endocrinology”. The bulk of his talk, an update on the pituitary, was devoted to outlining how little was known, how little clinical progress had been made, the uselessness of giving patients pituitary extract – “The Lewis Carroll of today would have Alice nibble from a pituitary mushroom in her left hand and a lutein one in her right and presto! She is any height desired”- and how wrong it was for a credulous profession to tolerate “polypharmaceutical charlatanism”. He further stated that “This society must discountenance the exploitation of the few discoveries which have already been made by those who recklessly under full sail plow through a fog bank of therapeutics, their horns tooting” The audience knew he was attacking the Endocrine Society’s founder, first secretary, and greatest enthusiast, a Californian half-quack named Henry Harrower who had a major mail-order business selling every kind of glandular extract.
Extract from “Harvey Cushing – A Life in Surgery”. Bliss M. Oxford University Press, Oxford, 2005.