Osteitis Fibrosa Cystica Due to Hyperparathyroidism

Abstract

In recent years the rôle of the parathyroid gland in relation to certain diseases of the bones has assumed considerable proportions. This subject is of extreme importance to the roentgenologist because of the bony changes observed by the X-ray. In 1904, Askanazy (1) first reported a case of generalized osteitis fibrosa, described by von Recklinghausen (2) in 1891, associated with a parathyroid tumor. In 1907, Erdheim (3) described three cases of osteomalacia in which there was hypertrophy or tumor formation of the parathyroids. This raised a question as to whether the hyperplastic condition of the parathyroid was primary and the skeletal changes secondary or vice versa. Erdheim contended that the enlarged glands were a compensatory change due to the increased drain of calcium metabolism resulting from the bony condition. As late as 1926, Kerl (4) in describing a case of osteomalacia and osteoporosis, believed this possibility. However, most authorities are of the opinion that the enlarged parathyroid is the primary condition because usually one gland is adenomatous and because later work shows absolute cures in these cases when a parathyroidectomy has been performed. In 1915, Schlagenhaufer (5) suggested the possibility of removing the parathyroid gland but it was not until 1925 that the first parathyroidectomy was done for generalized osteitis fibrosa cystica by Mandl (6). The patient was cured of all symptoms. The typical history of hyperparathyroidism is best described in the case reports of Snapper (7), Barr, Bulger, and Dixon (8), and others. The patient complains of severe pain over various parts of the body. There is a hypotonia of the skeletal muscles, pathologic fracture with delayed healing, high serum calcium, and cystic condition of the bones as shown by X-ray examination. There may be other calcium changes, such as urinary calculi, arteriosclerosis, etc. Hubbard and Wentworthy (9) described a case which they called metastatic calcification associated with chronic nephritis and hyperplasia of the parathyroids. There were numerous soft tumor masses over the body and aspiration of the fluid revealed 30 per cent calcium phosphate. There were beady nodules on the peripheral blood vessels and an excised piece showed that the media had been replaced by calcium. A range of from 11.9 mg. to 13.4 mg. showed in the serum calcium. Other blood studies were indicative of a high degree of nephritis. Autopsy revealed a severe interstitial nephritis with a right hydronephrosis. There were calcium deposits on the wall of the left auricle and of the various blood vessels excepting those of the liver and spleen. There was an osteitis fibrosa of the skull, ribs, and vertebræ, and two large parathyroids showed hyperplasia, one being adenomatous, Hunter, in the Goulstonian lectures given in 1930 (10), states that the term generalized osteitis fibrosa indicates a disease entity distinctly different from focal osteitis fibrosa.