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The Generalized Type, However, Has Received THE GENERALIZED TYPE OF OSTEITIS FIBROSA CYSTICA VON RECKLINGHAUSEN'S DISEASE JOHN J. MORTON, M.D. Assistant Professor of Surgery, Yale University School of Medicine NEW HAVEN, CONN. INTRODUCTION The localized form of osteitis fibrosa, especially in its relation to the formation of bone cysts, has been very commonly recognized and com- mented upon during the last twenty years. Bloodgood,1 in numerous publications, and also Barrie,2 Silver,3 and Meyerding,4 have furnished valuable contributions to the American literature; and Elmslie5 has given a good summary of the English cases. The Germans have written at length on the subject. The generalized type, however, has received scant attention, possibly because it is not recognized; and, aside from the article by L\l=o"\tsch,6 no extensive publication has been devoted entirely to this form of the disease, although both Meyerding and Elmslie discuss the generalized cases in their papers. It seemed worth while, therefore, to report the subjoined case: first, because of the rarity of the condition, especially in the United States, and secondly, because of the possibility of correcting some of the deformities arising in this disease. At the same time, it was considered advisable to collect the reports of general types of the disease in order to find what might be learned from them. report of case History.—A man, aged 23, a lathe operator, was referred, Nov. 14, 1919, by Dr. Stanley Cox of Holyoke, Mass., complaining of bowing of the thigh bones. Except for the usual children's diseases, the history was essentially negative. He had never had a serious illness, although he had had a sore I am indebted to Dr. Robert W. Lovett of Boston for permission to use his records of the case reported in this article. 1. Bloodgood, J. C.: Bone Cysts, J. A. M. A. 43:1124-1129 (Oct. 15) 1904; Prog. Med. 5:188-192, 200-204 (Dec.) 1903; ibid. 6:181-194 (Dec.) 1904; ibid. 7:273-280 (Dec.) 1905; Ann. Surg. 12:145-189, 1910. 2. Barrie, G.: Ann. Surg. 61:128-142, 1915; ibid. 67:354-363 (March) 1918. 3. Silver, D.: Am. J. Orthop. Surg. 9:563-588, 1911-1912. 4. Meyerding, H. W.: Am. J. Orthop. Surg. 16:253-276 (Sept.) 1918; ibid. 16:367-382 (Oct.) 1918. 5. Elmslie, R. C.: Brit. J. Surg. 2:17-67, 1914. 6. L\l=o"\tsch,F.: Arch. f. klin. Chir. 107:1-137, 1915. Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 05/31/2015 throat occasionally and frequent colds but no cough. His appetite was good ; the bowel movements regular, and he denied venereal infection. Three years previously, he had an attack of facial erysipelas, lasting about three weeks. At 3 years, he fell and fractured the right femur. Union was good under ordinary treatment. At 4 years, the right arm was hit by a swing and broken Fig. 1.—Three views of patient showing marked bowing of the femurs with shortening of the right leg and equinus position of the right foot. just above the wrist. He had no further trouble in any way until he was 12 years old. At that time he fell, after which he could not walk for two or three days. It was six or seven weeks before the pain and limp disappeared. At 16, he fell over a railroad track and again broke the right femur. He was Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 05/31/2015 in the hospital six weeks ; and the fracture united without complication. He had no trouble during the subsequent five years, until March, 1919, when he began to have pain just above the right knee, which gradually became more severe. Some days he could not get up from his chair because of inability to extend the leg at the knee. This continued until July, 1919. At that time he suffered with pain in the left knee, which extended up the thigh into the hip, and the pain in the right thigh disappeared. The upper portion of the left thigh became bent and a roentgenogram showed a pathologic lesion of the bone. July 21, 1919, while walking, his left foot hit against a clump of grass, tripping him. He fractured the left femur by this fall and was in the hospital eleven weeks. The bowing of the femur was considerably reduced by the Fig. 2.—Defects in the frontal and occipital regions. treatment of this fracture. In October, 1919, there was a return of pain in the right thigh and knee, which had persisted to the time of examination. The pain was not severe, did not keep him awake, but was annoying and "rheumatic¬ like." He walked with a limp on the right side and with the aid of a cane. Examination.—The patient was well developed and well nourished. There was no apparent pain at the time of examination. No abnormalities were noted in the head. The sinuses were not tender on pressure. The pupils were equal ; reacted to light and on accommodation. There was no nystagmus. The von Graefe sign was not elicited. There was no exophthalmos. The extra-ocular move¬ ments and the eye grounds were normal. There was no mastoid tenderness ; no Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 05/31/2015 discharge from the ears and no defect in hearing. Breathing was partially obstructed on both sides of the nose. The mucous membranes of the mouth were of normal color; the tongue protruded in the midline and was clean. The teeth were in bad shape ; many were missing, and there were a number of old snags in the molar region of the upper and lower jaws. The tonsils were embedded and somewhat scarred. There was no general glandular enlarge¬ ment. The epitrochlears were not palpable. The thyroid was not enlarged. There was no tremor and no tachycardia. Fig. 3.—Defects in left frontal region. The chest was not quite symmetrical, being somewhat depressed below the precordial region and the ribs flared slightly laterally. The percussion note and vocal fremitus were normal. There were no râles. The heart was normal in position and size. The sounds were regular and clear. No murmurs were heard. The heart rate was 70. The pulses were equal and of good volume and tension. No tenderness and no masses were detected in the abdomen on palpa¬ tion. No organs were palpable. Rectal examination was negative. The genitalia were normal. The spine showed no scoliosis. Movements of flexion, extension, side bending and rotation were normal. There was no disturbane?- Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 05/31/2015 in the range of motion of any of the joints, except the hip and knee. The reflexes were present and equal in the arms and legs on both sides. Sensation was normal throughout. Both thighs showed a very marked outward bowing, especially the right (Fig. I A). This deformity had shortened the distance between the gluteal fold and the knee joint on the right side to such an extent that the patient could not get his right heel to the floor (Fig. 1 and C). The deformity was mainly of the thigh bones, which palpation demonstrated to be considerably- thickened. The trochanters were high above Nélaton's line and were also Fig. 4.—Beginning changes in the upper humérus and at the junction of the upper and middle thirds. thickened. There was practically no abduction in either hip ; adduction was normal ; flexion to a right angle was possible on both sides ; there was no rotation of either hip. The knee joints flexed to about 75 degrees; extension was complete. Measurements were: the anterior superior spine to the malleolus (left) 35 inches (89 cm.) ; the anterior superior spine to the malleolus (right) 33 inches (83.8 cm.) ; the umbilicus to the malleolus (left) 38 inches (96.5 cm.) ; the umbilicus to the malleolus (right) 36 inches (91.5 cm.). Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 05/31/2015 Tibia measurements were: malleolus to tibia tubercle (left) 14 inches (35.5 cm.) ; malleolus to tibia tubercle (right) 15 inches (38 cm.). Femur measurements were: anterior superior spine to tibia tubercle (left) 21 inches (53.3 cm.) ; anterior superior spine to tibia tubercle (right) 18 inches (45.7 cm.). Circumference measurements were: thigh (left) 18 inches (45.7 cm.); thigh (right) 18Mi inches (47 cm.); calf (left) 13^ inches (34.3 cm.); calf (right) 11% inches (31.3 cm.). Figure 5 Figure 6 Fig. 5.—Changes in the upper end of the right radius and ulna showing areas of rarefaction and thinned cortical bone. Fig. 6.—Right femur showing marked bowing and coxa vara, foamy cystic appearance of bone, no differentiation between cortical and medullary bone and a fracture of the midshaft. Compare with Figure 14, one of von Reckling¬ hausen's original cases. Special Examinations.—Urine : The color was high ; the specific gravity was 1.026. The slightest possible trace of albumin was demonstrated by heat test. Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 05/31/2015 No positive result was obtained with nitric acid ; no sugar was present. No Bence-Jones bodies were found on three examinations. There were many calcium oxalate crystals. Stool : There was no abnormal gas formation and no abnormal odor. The reaction was neutral and smears were microscopically negative. Blood : One c.c. of venous blood in a tube of 9 mm.
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