Hyperparathyroidism

HYPERPARATHYROIDISM REPORT

OF A CASE OF PARATHYROID ADENOMA OF THE BONES SAMUEL KLEINBERG, NEW YORK,

NEW

WITH

CYSTIC

DISEASE

M.D.

YORK

T

etiology of the sciatica. The histvry suggested HE interest of the orthopedic surgeon that the patient’s height had dimrnlshed somcin diseases of the parathyroid gIands what during the preceding few years. A conriiis centered chiefly in the cases of hyperactivity of these organs causing a loss of tion of stooped or round shoulders which had been present for many years was said to have the caIcium content of the bones with rebecome exaggerated. I found a moderate right suItant osteoporosis, deformity of the Iimbs dorsa1 idiopathic kyphoscoliosis known to ha\-c and pathoIogic fractures. StimuIated by the been in existence for many years. An increase work of BaIIin of Detroit, the members of of this deformity would reasonaLl_ explain my staff at the HospitaI for Joint Diseases the reduction in the height, the exact extent and I have been carefuIIy scrutinizing a11 0fxVhich was uncertain. I noted also an cnlargebone Iesions which come under our care for ment of the head, but did not appreciate that evidences of hyperparathyroidism. Cases of this was pathologic, since I did not ha1.e a clear recoIIection of its pre\.ious size and shape. arthritis, IocaIized and genera1 osteoporosis There were no neurologic clisturhances. An and cystic disease of bones have been thors-ra\film of the pel\.is rex.ealed an as~mmctr~ oughIy studied, with particular reference to which I considered as secondary to the scoliosis. the serum calcium and phosphorus and the The bones of the peIvis re\,eaIed no alteration phosphatase. Our attention has been foin their architecture other than the morphocused on the significance of these chemical logic change. Since the sciatica J-ielded to trcatchanges through the work at our hospital ment, the man resumed his work as a printing of Dr. Henry L. Jaffe and his associates, compositor ant1 I dismissed the cast from m\ whose studies are we11 known to man\; in mind. the profession. Yet it was not unti1 recently The patient returned three years later, on February I I, 1938, because of a fracture of his that there came under our care a patient left arm which had occurred three months exhibiting the characteristic manifestations previously during a rather mild effort to go of hyperparathyroidism arising from an through a narrow doorwav I,pulling himself adenoma of one parathyroid gIand, the up bvith his left hand hoI&ng on to the framcfirst case, I believe, in the records of our \\ork of the door. Llore significant \vns the institution. The experiences of this patient presence of symptoms of a sc\.ere systemic disso accurateIy mirror the changes in hyperturbance. He had grown progressively lvcaker parathyroidism and the effects of surgery so that he couIc1 walk only a fe\\, blocks at :t that they are worth reporting in detai1. time, and because of this, he had given up his CASE

REPORT

The patient, a man of 49, had been known to me for fifteen years, but consuIted me professionaIIy for the first time in September 1935 for what appeared to be an idiopathic Ieft sciatic neuralgia. This was readily reIieved by simpIe physiotherapeutic measures. Certain observations were made then which cIid not seem very significant, but which in retrospect have manifestIy an important bearing both on his present condition and perhaps, too, on the

work some time before the fracture incident. As his wife conducted a boarding house he contented himseIf doing odd jobs around tht house. But wiIIing as he was, he had IittIc resistance, and even mild efforts tired him readiIy. He had Iost some weight and sex-era1 inches in height, his lower limbs had become bowed and his head was enlarged, noticeably so now to his wife and friencIs. The examination showed a greatly changed indi\-idual. His stature \vas decidedIy shortened; his skull was enIarged and by contrast his

NEW

KIeinberg-Hyperparathyroidism

SERIES VOL. L, No. 2

face was smaI1; his back presented a severe kyphoscoliosis with teIescoping of the chest into the peIvis. The thighs were bowed outward. He waIked sIowIy, awkwardIy and with a waddling gait. His voice was weak. He appeared asthenic. In the Ieft arm he had an ununited pathoIogic fracture of the shaft of the humerus. (Fig. I.) It was now, of course, apparent that we were deaIing with a lesion that had produced a profound constitutiona disturbance which was very marked in the osseous system. A parathyroid tumor was suspected and the patient was admitted to the HospitaI for Joint Diseases for further study. Roentgen ray fiIms were made of the entire skeIeton. These showed very extensive invoIvement of many of the bones. TABLE

~~~~~~~ of

surgery

359

The Ieft humerus (Fig. I) showed evidence of a pathoIogic fracture through the middIe third of the shaft and a Iarge meduIIary tumor in the

I JFX N.

Preoperative.

American

N.P.N.

Sugar

2.7

4

14/39 Operation.

4W38 Postoperative..

3/S/38........ 3/11/38....... 3/14/38.. 3/n/38....... 3/28/38... 3/30/38... 4/6/38....... 4/S/38....... 4/18/38..... 4/21/38. 4/25/38..... 5/2/38.... 5/3/38.. 5/10/38. 5/16/38....:: 7/28/38. 3/3/39.......

80

8.4

dW38

5.4 5.8 5.8 5.2 .:’

..

3.4 4.2

5.0

5.1

5.6

4.3

5.9 6.2

4.0

6.1 7.0 8.0

4.” 3.8 3.4 3.9 2.9

8.3 10.2

9:

2.8

31

~

33

~

34

~

FIG. I. Pathologic fracture of left humerus. Note cystic condition at site of fracture and lack of cdlus. There is marked demineralizntion. Cortex is very thin. 9’

39 33 34 34

101

4.4

X-ray examination of the skuI1 discIosed extreme thinning of the tabIes of the bones with compIete Ioss of the trabecular markings (Fig. 2) but no definite tumor formations. There was extreme atrophy of the inferior maxiIIa with a large cyst in the symphysis. An extreme kyphoscoIiosis of the dorsa1 spine was accompanied by pronounced wedging of al1 the vertebrae, associated with margina hypertrophic changes. The ribs showed marked osteoporosis. In the scapuIae there was aImost compIete loss of a11 bone detai1 due to resorptive changes. The lumbar spine also discIosed extreme generaIized osteoporosis. No compression fractures were noted and the kidney showed no caIcuIi. In the peIvis there was extreme generalized osteoporosis without tumor formation.

same area. The aIignment of the fragments was good but no caIIus formation was present. In the proxima1 extremity of the humerus extreme cortica1 thinning with granular disintegration was noted. The right humerus had a patchy area of osteoporosis at the junction of the proxima1 and middIe thirds of the shaft, associated with moderate cortica1 thinning. The bones of both hands (Fig. 3) exhibited extreme osteoporosis with marked cortica1 thinning, presenting Iikewise the granuIar disintegration noted in the Ieft humerus. Concentric atrophy was present in the shafts of the intermediate phaIanges of the second, third, fourth and fifth fingers on the right side as we11 as of the shaft of the proxima1 phaIanx of the fifth finger. Extreme cortica1 thinning of the right radius and ulna was more marked in the dista1 extremity of the uIna. A Iarge meduIIary tumor was present in the proxima1 third of the shaft of the right femur and in the proxima1 and middIe thirds of the shaft of the Ieft femur. GeneraIized osteoporo-

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J ournal

or Surgery

Kleinberg-Hyperparathyroidism

so\ EMBER, I

FIG. 2. Extreme thinning of bones of skull. Inferior maxiHa markedIy atrophied with large cyst in the symphysis shown in other views of skuI1.

FIG. 3. Marked

osteoporosis

and cortical thinning. in middle pLaIanges.

Note concentric

atrophy

NEW SERIES VOL. L. No.

2

KIeinberg-Hyperparathyroidism

sis,

but without the extreme corticaI thinning, was seen in these bones aIso. Another Iarge medullary tumor appeared at the junction of

American

the middIe and dista1 thirds of the shaft of the tibia (Fig. 4) while the anterior cortex of the tibia had been practicahy compIeteIy resorbed. In the right tibia a tumor had deveIoped at the proximal third of the shaft. The bones of both feet disclosed marked osteoporosis without any evidence of tumor formation. Studies of the bIood showed that the serum caIcium (Table I) was high and the phosphorus low, whiIe the phosphatase was very high. There was in addition, a moderate secondary anemia. The sedimentation rate was 50. The Wassermann and Kahn tests were negative. There seemed no doubt that we were deaIing with a case of hyperparathyroidism (Drs. Jaffe, Bodansky and Pomeranz concurred). Dr. MiIton Bodenheimer of the genera1 surgica1 staff was invited to examine the patient. He thought that he feIt an unusua1 mass in relation to the Ieft Iobe of the thyroid. The patient stated that he feIt a mass in his neck moving during swalIowing. However, other consultants were not certain of the presence of any tumor. Dr. Bodenheimer operated on this patient under genera1 narcosis on February 26,rg38. He found and removed a tumor of the Ieft inferior para-

ofsurgery 361

thyroid gIand, of the size of a smaI1 lemon. The other parathyroids were found to be normal in size, number, Iocation and appearance.

FIG. 5. Adenoma

FIG. 4. Cystic area in shaft of Ieft tibia. Marked resorption of anterior cortex.

Journal

of parathyroid

gIand.

The tumor measured approximately 3.5 cm. in diameter and weighed 13 Gm. It was encapsuIated and on section appeared very celluIar. The cut surface had a grayish-yeIIow glistening appearance with smaI1, irreguIar areas sIightIy more transIucent. Beneath the covering of dense fibrous tissue, the neopIasm was composed for the most part of a diffuse proIiferation of moderateIy large polyhedra1 ceIIs. The nucIei were moderateIy large, round, possessed a nucIear membrane, and showed a definite chromatin network. Others were more hyperchromatic and tended to be solid. An occasiona nucIeus showed a nucleolus. No mitotic figures were seen. The ceIIs possessed abundant cytoplasm, granuIar and slightly eosinophiIic. Water-clear ceIIs were present but onIy near several areas of degeneration. The tumor contained numerous fine vascuIar channeIs and fibrous septa, but no attempt at gIand formation was noted. The pathoIogic diagnosis was chief ceI1 adenoma of the parathyroid gIand. The patient was somewhat depressed for severa days after the operation, but at no time seemed dangerousIy iI1. DirectIy after the operation he received an intravenous injection of saIine soIution containing caIcium gIuconate and glucose, and thereafter he received caIcium Iactate and viosterol daily. The serum caIcium dropped rapidIy after the operation to 8.4 and

362

A m&can Journalof Surgery

Kleinberg--Hyperparathyroidism

continued to drop more slowly so that at the end of a month, on March 30, 1938, it was 5 mg. per IOO C.C. of blood. The phosphorus in the meantime rose from 2.7 to 5. I.

FIG. 6. Postoperative x-ray fracture of lrft humerus Porosis of bone he&d. Cortex thickened. diminished.

Clinically this patient \zas doing well and the operative wound heaIed by primary union. Hoarseness was noted on the day after operation, due to paraIysis of the Ieft vocal cord, evidently from involvement of the left recurrent IaryngeaI nerve. However, this vocal difflcuIty uItimateIy compIeteIy disappeared. By hlarch 9, 1938, eIeven days after the operation, the patient was well enough to get out of bed. Al1 during this time he had a positive Chvostek sign and at times showed restIessness and twitching of the eyeIids, suggestive of latent tetany. On the night of March I I, thirteen cIays after the operation, his temperature suddenIy rose to 103.5, he became very restIess and noisy and had some diff%xIty in breathing, and became vioIent in his muscular activities. He w-as promptIy given caIcium gIuconate intravenously and parathormone intramuscuIarIv. Thereafter he received parathormone and caGum Iactate daiIy until April I, 1938. He improved a great dea1 and his temperature receded to normaI. Since ApriI I, 1938 the patient has continued to take caIcium Iactate and viostero1. His bIood caIcium graduaIIy rose so that by May 16, 1938

N0vrnrsEr‘. ,<)4,,

it was 8.0; phosphorus was 3.4 and phosphatasc feI1 from 23 to 4.0. On Ala>- 20, 1938 the patient’s general condition \vas good and his strength increasing. Firm union of the fractured humerus had occurred and s-ray pIates sho\ved an increase in bone deposits, especially in the long bones. A year after the operation the patient n-as greatly changed: he was aIert and ambitious, ivalked briskl)- and had acquired considerable physical encIurance as compared with his former listless, weak state. A ne\v set of .x2’-ra\films she\?-ed complete healing of the Ieft humerus (Fig. 6) and a reduction of the cystic areas. The bones stiI1 looked atrophied and “lvashcd out,” but much Iess so than a \-eat before. The gcnerai haziness was cIiminished and thcrc wxs much more definite differentiation brtwccri the medulla and the tortes in all the long bones. The cyst in the shaft of the left tibia had almost completely disappcnred. SirniIarI>- the c)-st in the left femur was greatly reduced and the cortex of this bone had increased in density. The blood chemistry was almost normal. On hlarch 3, 1939 the senm calcium UYIS 10.2, the phosphorus 2.9 and the 4.4. phosphatase DISCUSSION

The original cIinica1 picture, characteristic of hyperparathyroidism, included progressive weakness, enIargement of the skull, deformity of the spine and the lower limbs and a pathologic fracture of the left humerus. The diagnosis was confirmed b>roentgenograms which showed a generalized demineralization and osteoporosis with an occasional cyst-like formation, and 6) the blood cheniistry changes, marked hypercalcemia, hypophosphatemia and increase in phosphatase. The postoperative reaction in this case was a hypoparathyroidism and tetany, including a marked drop in the bIood calcium, a rise in phosphorus, restlessness, hyperactive reflexes and, finall?;, about two weeks after the operation, convulsions and a rise The loss of parathyroid in temperature. secretion was compensated by the administration of parathormone, cal&m gluconate or Iactate and viosterol. The patient is now we11 on the wa>- to :I complete cure.