Early Clinical Signs of Acromegaly
Acromegaly is a chronic disease caused by hypersecretion of growth hormone. It is characterized by enlargement of many parts of the skeleton, including the nose, ears, jaws, fingers, and toes. In 1772, French physician Nicolas Saucerotte (1741-1814) presented a case before the Académie de Chirurgie regarding what is now known as acromegaly. He published “Accroissement singular en grosseur des os d’un homme agé de 39 ans” in 1801 in Mélanges de chirgurie. In 1886, neurologist Pierre Marie (1853-1940), a student of Jean-Martin Charcot (1825-1893) at the Salpêtrière Hospital, provided the first complete clinical description of the disease. He included five cases in his final study, one of which was Saucerotte’s.
The following year, Oscar Minkowski (1858-1931) was the first to recognize pituitary enlargement in acromegaly. In 1893, English surgeons Richard Caton (1842-1926) and Frank Thomas Paul (1851-1941) attempted to treat acromegaly by surgical decompression—they were unsuccessful. In 1912, neurosurgeon Harvey Cushing (1869-1939) published The Pituitary Body and Its Disorders. Cushing’s innovative operative techniques led to today’s modern surgical procedures.
More than 90% of patients with acromegaly have a pituitary adenoma, and surgery is the therapy of choice, curing about 50% of patients. Radiation and hormone therapy can be used as adjunctive therapies. An elegant, minimally invasive, microsurgical procedure known as endoscopic endonasal transsphenoidal surgery is the most successful treatment and involves the surgeon reaching the pituitary through an incision in the nasal cavity wall. As with all types of surgery, the patient’s hormone levels must be monitored for years afterward.
In 1921, E. B. Krumbhaar, MD, from the laboratory of the Postmortem Pathology Department at the Philadelphia General Hospital, published “Pituitary Disorders in Their Relation to Acromegaly; With Suggestions for the Use of a More Precise Terminology” in the Medical Clinics of North America. Dr. Krumbhaar illustrated his paper with photographs documenting some of the common signs of acromegaly, such as pituitary disease in the absence of overt neurologic symptoms and changes in facial structure. This photograph illustrates separation of the teeth of the lower jaw and clubbing of the fingers. The patient, a 43-year-old janitor, was admitted to Philadelphia General Hospital dyspneic and spitting up blood. He died from pneumonia and lung abscess. The patient’s pulmonary condition had precluded diagnostic radiologic studies and other pituitary tests. His pulmonary condition began when he contracted influenza. At autopsy, a hyperplastic pituitary gland due to acidophil cells was identified.
© Stanley B. Burns, MD, FACS