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Diseases of bone and cartilage.

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edema, retinal pigmentary changes, and accumulation
of exudative material in the deep retina. One patient developed a necrotizing retinitis with deep retinal exudation and subsequent retinal detachment. Occlusions
of retinal vessels were common. Neuroophthalmologic
manifestations included cranial nerve palsies, a homonymous hemianopic field defect, and papillitis. Hypopyon was a relatively uncommon manifestation in this
In 2 patients with Behcet’s syndrome, neurologic
manifestations antedated the characteristic mucocutaneous lesions by 6 to 32 weeks (K154). Neuroradiologic investigation revealed expanding avascular foci in
the basal ganglia, probably from areas of infarction secondary to vasculitis. [Neurologic manifestations, present
in up to 25% of patients, are highly variable because any
part of the central nervous system may be involved. Ed.]
Several forms of therapy were critically reviewed
(012). It was concluded that most studies were uncontrolled and side effects understressed. Attempts to derive
clues to the etiology of recurrent aphthous ulcerations
and Behcet’s syndrome from analysis of the mode of action of various drugs used in treatment were the basis of
several reports (G204,S17,T1 13). Assuming that potentiation of immune responsiveness is beneficial to patients, it was concluded that clinical trials of levamisole
were justified. However, these experimental observations also suggest that careful monitoring of the drug
dosage is necessary since presumably at higher doses,
the syndrome could be worsened as a consequence of
immunosuppression (S 17).
The results of a double-blind trial of tetracycline
versus placebo in 25 patients with recurrent aphthous
oral ulcerations appeared (G205). Both placebo and tetracycline groups experienced reduction in ulcer incidence during the treatment period, but only the tetracycline group had significant reductions in ulcer
duration, size, and pain. Of 6 Behcet patients with primarily mucocutaneous manifestations treated with
transfer factor, 4 improved and 1 did not, while data on
the other patient were inconclusive because of concomitant drug therapy (W167). [In the absence of major
ocular or neurologic manifestations, trials with either
tetracycline suspension or transfer factor may be warranted. Ed.]
Favorable therapeutic results were reported with
chlorambucil (D 1 19) as well as with cyclophosphamide,
either alone or in combination with colchicine (012).
Oral steroids, when used early and in sufficient dosage,
may be effective for central nervous system involvement
(K154). Suppression of ocular inflammation may be
achieved with topical and oral steroids, as well as with
chlorambucil (C 184). Patients who receive chlorambucil
need close supervision with frequent platelet and leukocyte determinations for potential bone marrow suppression. [Responses to all forms of therapy are variable and
difficult to assess because of the relapsing nature of Behcet’s syndrome. Ed.]
Relapsing polychondritis
While relapsing polychondritis is rarely reported
in Asians, an unusual case was presented of a Chinese
male with airway obstruction (T42). The utility of radiographic study to detect and evaluate upper respiratory tract involvement in relapsing polychondritis was
described; plain lateral neck radiographs, frontal tomographs, and contrast laryngography were demonstrated
Rapidly progressive crescentic glomerulonephritis developed in 3 patients with relapsing polychondritis
(N29). Two patients who were not oligo-anuric responded to aggressive treatment with prednisolone,
azathioprine, and anticoagulants. The multisystem in-
volvement, renal lesions, and response to treatment suggested to the authors a relationship to periarteritis nodosa. Others made similar suggestions (S28 1). Detailed
case studies were also presented (K97).
Ocular involvement has been reported to occur
in 65% of patients with relapsing polychondritis; however, reports of ischemic optic neuropathy continue to
be rare (K87).
Further confirmation of the findings of anticartilage antibodies was presented (C15 1). Antibody
specificity for type I1 collagen and cartilage matrix was
found in 5 patients during the acute phase of disease
0 steolysis
Several case reports with reviews appeared on
osteolysis or Gorham’s disease, but the disorder continued to be enigmatic (H123,Rl41,S6).
Additional case studies were presented of a form
of osteolysis affecting children with a predilection for
carpal and tarsal bones (A85,E62,W108). This disorder
may be familial and may be complicated by glomerulonephritis. Moreover, it may mimic juvenile rheumatoid
arthritis in its clinical presentation. Multiple osteolytic
lesions resulted from traumatic pancreatitis in an
abused 3-year-old child (N36). A 7 1-year-old woman
was described in whom multifocal osteolysis was observed in both axial and appendicular skeleton, but
without the increased bone vascularity usually associated with massive osteolysis (Gorham’s disease) (R63).
Paget’s disease of bone
Reviews of various aspects of Paget’s disease appeared (C74,K159,M235,S92,224,W83).
Study of the segregation of HLA haplotypes and
Paget’s disease in 3 informative families with multiple
cases in 3 generations indicated a likely linkage of HLA
without maintaining significant linkage disequilibrium
Corrugation of the skull was recognized as a
hitherto undescribed deformity and physical sign of Paget’s disease in 3 cases (C75).
An epidemiologic survey utilizing radiologic criteria for Paget’s disease indicated real geographic variation in disease prevalence in British towns, as well as
among British migrants to Australia (B30,G24).
Plasma alkaline phosphatase, plasma nonprotein-bound hydroxyproline concentration, and urinary excretion rate of total hydroxyproline were closely
correlated with each other, but not with fasting plasma
concentrations of calcium or inorganic phosphate in a
group of 83 patients with untreated Paget’s (W26).
Mean whole body retention of 9 9 m Tafter
~ 24
hours was proposed as a sensitive means of detecting increased bone turnover (56.9% retention in 10 patients
with Paget’s disease compared with 19.2% retention in
12 normal controls) (F71).
Nine patients with painful Paget’s disease responded favorably to a drug and diet combination designed to elevate plasma calcium, stimulate endogenous
calcitonin production, and suppress parathyroid hormone production (E77). The low dose regimen consisted
of oral calcium, thiazide diuretic, aluminum hydroxide,
and low phosphorous diet.
Many papers appeared relating favorable, but
uncontrolled, observations regarding treatment of Paget’s disease with calcitonin (synthetic human, porcine,
salmon), diphosphonate (sodium etidronate), and mithramycin, alone or in combination. Subjective responses,
reduced plasma alkaline phosphatase, reduced hydroxyprolinepeptiduria, modified bone biopsies and radionuclide scans, positive calcium balance, and radiologic
regression were all presented as favorable outcomes of
therapy (A165,B 144,H5,K79,L41,M96,041,P 139,R183,
A controlled study of oral sodium etidronate (disodium ethane- 1 hydroxy- 1, 1-disphosphonate) was undertaken for Paget’s disease over a 6-month period with
varying doses of 0, 2.5, 5, 10, or 20 mg/kg/day. No significant changes in either urinary hydroxyproline or
serum alkaline phosphatase occurred in patients receiving placebo; however, both these measurements decreased at all dose levels, with the greatest decline at the
highest dose. High dose had less favorable outcomes
judged subjectively, by disease progression, or by spontaneous fracture appearance. Treatment with 5 mg/kg/
day did not appear to be associated with complications,
but offered sustained remission of disease (C28,K80).
0 steoporosis
Comprehensive reviews of osteoporosis appeared
Osteoporosis was induced in adult rats maintained on low calcium intake for long and short periods
of time (S158). It was found that after a critical period
during the development of osteoporosis, the skeletal
changes either became irreversible or needed an appreciably long period of treatment for their regression,
whether complete or partial.
Total and nondialyzable hydroxyproline excretion did not differ in women with spinal osteoporosis or
postmenopausal women, suggesting that differences in
bone turnover did not exist (A69).
Neutron activation analysis was utilized to determine the effects in rats on skeletal calcium s! age, sex,
genetic background, diet, and corticosteroid or heparin
treatment (A79). It was concluded that age, genetic
background, and sex influence the development of
osteoporosis. This process was accelerated by low calcium diet or prednisolone or heparin treatment. Similarly, rats deprived of adrenal and gonadal sex hormones were more sensitive than normal rats to the
hypercalcemic (osteolytic) effect of parathormone and
toxic doses of vitamin D, (H163).
A histomorphometric analysis of bone from de-
monstrably osteoporotic females compared with a normal group indicated that the percentage of bone tissue
in the spongiosa (trabecular bone volume) gave a better
reflection of osteoporosis than cortical and trabecular
bone (total bone density) (B210).
Prednisone therapy in patients resulted in progressive decrease of bone mineral content which did not
correlate significantly with dose (D58).
Oophorectomized women experienced greatest
bone loss within 3 years of operation (H184).
Loss of dermal collagen was suggested as the
main factor responsible for the decrease in skin fold
thickness and the frequently striking transparency of
skin observed in association with osteoporosis (L76).
Bone biopsy indicated frequent osteomalacia
and/or osteoporosis in the presence of chronic liver disease (L169).
Plasma hormonal concentrations of androstenedione and estrone were significantly reduced in
women with osteoporosis (M86). Similarly immunoreactive calcitonin levels were reduced, but parathyroid
hormone levels were normal (M238).
Bone mass decreased in men and women over a
5-year period, but nonuniformly and without correlation with dietary intake of calcium or vitamin D
(M257). Bone mass increased with body size or increased activity.
Measurements of bone mineral content and total
body calcium, normalized and expressed as ratios, were
compared with radiographic morphometry in 45 women
with spinal osteoporosis (A70). The radiographic indices examined included the femoral score, the femoral
trabecular pattern, the biconcavity and metacarpal indices, and the total peripheral score. Both ratios and all
the radiographic indices except the femoral trabecular
pattern were found to be related to the number of dorsal
spine fractures. Metacarpal cortical area was studied as
an index of bone mass and found to be reliable as compared with direct photon absorptiometry (E78).
Another study indicated that a radiographic index based upon the pattern of trabecular bone at the
proximal femur (Singh index) gave a better separation
of normal subjects and osteoporotics than photon absorptiometry (Cameron-Sorenson) of the radius (W5).
Bone density was determined in subjects by measuring
the intensity of Compton scattered photons; it was suggested that the technique may be applicable to study of
early osteoporosis by virtue of its sensitivity (H95).
Bone mineral content was also measured by using a
method based upon gamma-ray attenuation by pair
production (P175).
Therapy of osteoporosis was the subject of an editorial review (A 164). Exercise, supplemental calcium,
calcitonin, human growth hormone, estrogens, vitamin
D, fluoride, anabolic steroids, alone and/or in combination were found beneficial for therapy of osteoporosis
(A68,7 1,D18,G l63,164,Hl3,183,184,J79,L24,137,139,
l41,193,M87,88,89,P41,R35,133,134, S282,292,V67,
An editorial reflecting the views of international
investigators in osteoporosis urged restraint in the use of
high dose fluoride to treat skeletal disorders because
studies have yet to document efficacy without unacceptable toxicity (M99).
Three patients with osteoporosis were found to
have 2 to 3 times increased serum immunoreactive
parathyroid hormone and inappropriately low serum
1,25-dihydroxy vitamin D (R85). These and other data
suggested that increased serum immunoreactive parathyroid hormone was caused by secondary hyperparathyroidism, probably because of inadequate conversion of 25-OH-D to 1,25 (OH), D.
Studies were undertaken in vitamin D-deficient
adults to compare effects of vitamin D metabolites
(B201). It was concluded that 1,25(OH),D3 is not the
sole biologically active metabolite of vitamin D in humans. It was apparent that either 25(OH)D3 or some
unidentified metabolite of 25(OH)D3 stimulated the
renal tubular resorption of calcium and phosphate, and
that the subsequent rise in serum phosphate concentrations along with the direct actions of la-25(OH),D3,
24,25(OH),D3, and possible 25(OH)D3on bone cells all
participated in the restoration of normal bone formation and bone mineralization in vitamin D-deficient humans.
Regional migratory osteoporosis affecting the hip
was reported in an additional group of patients, with an
association of pregnancy or trauma in some (V3). Another patient with involvement of knee, ankle, and foot
was followed over a 9-year period (M128). Electromyography documented denervation patterns coincident in time and location of each acute attack.
A series of 26 patients with malignant osteoporosis who were seen in a 10-year period was presented
from Costa Rica (L173). Consanguinity, which was felt
to be consistent with an autosomal recessive form of inheritance, was present in 6 of the patients. No therapy,
other than palliative measures, was suggested.
Miscellaneous studies
A family was reported in which the mother and 4
of 8 siblings were affected by primary protrusio aceta-
buli (D3). Double contrast arthrography diagnosed
chondromalacia of the patella with an accuracy of
about 90%, as judged by surgical confirmation of the
disorder (H179).
Nutritional and hormonal effects on bone were
reviewed (G 12,S311). The inhibitory effect of estrogen
on resorption of bone in organ culture was concluded to
be a cellular toxic effect rather than a physiologic one
(L 158). Histomorphometric analysis of iliac crest biopsy
material from 24 patients with acromegaly tended to reflect increased volume density and related findings, but
no consistent changes occurred with therapy (D67). A
vitamin D-dependent, calcium binding protein was detected in spongiosa and epiphyseal plate regions of
chick tibia by utilizing a radioimmunoassay with antisera having specificity for intestinal calcium binding
protein (C119).
A parametric matrix was derived to characterize
stress-generated potentials in Haversian bone (K146). It
was found to agree substantially with experiment,
whereas the commonly accepted C6 hexagonal piezoelectric matrix did not. The specific dependence of stressgenerated potentials on microstructure was shown.
Hence, conventional piezoelectric matrices which are
only applicable to single crystals were suggested to be of
limited applicability to bone. The frequency response of
piezoelectric coefficients of dried bone (mineralized
fibril and ground substance) was described, suggesting
the likelihood that dielectric displacement was proportional to the strain, independent of deformation frequency (P 1 12).
Hypertrophic 0steoarthropa thy
The appearance of a symmetrical rheumatoidlike polyarthritis presenting in a patient with a history
of heavy cigarette smoking should alert one to the possibility of hypertrophic osteoarthropathy (HOA) as well
as rheumatoid arthritis. A patient with carcinoma of the
lung (A166) and 1 with thoracic Hodgkin’s disease
(P64) presented initially with manifestations of symptomatic HOA which mimicked symmetrical polyarthritis.
Symmetrical, painful, and tender articular and perarticular swellings associated with warmth, painful limitation of movement, a history of morning stiffness, a
raised sedimentation rate, and good response to antiinflammatory drugs may prove a trap for the unwary
who believe that they are dealing with early rheumatoid
disease. This is especially so when clubbing is subtle or
absent. Diagnosis is made even more difficult when
periosteal changes are absent, as they may be early in
the development of HOA. Response to anti-
inflammatory medication will not be diagnostic since
HOA is known to respond to these measures.
Delayed presentation of secondary HOA manifested clinically by right hip and lower back pain and
on scintigraphy by increased activity in the clavicle and
metaphyses of the long bones of the elbows, wrists, hips,
knees, ankles, and distal phalanges, associated with
smooth periosteal thickening noted on radiographic
study, was reported (P79). No prior documentation of
such a marked delay of onset of HOA after treatment of
the primary lesion was found by the author.
Six of 18 patients with sarcomatous tumors metastatic to the lung had HOA; in 3 patients in whom tumor regression was achieved by cytotoxic chemotherapy
or surgery, the clinical and radiologic changes of HOA
were reversed. All 6 patients had large mass lesions that
impinged on the pleural surface, and the pathophysiologic mechanism resulting in the clinical syndrome of HOA may be related to the anatomic relationship of the tumor to the pleura with neurovascular
reflex emanating from the tumor-pleura interface. Successful treatment of HOA by selective transection of the
vagus nerve, which provides the major contribution to
the nerve plexus within the pleural surface, lends support to the importance of anatomic position of the metastases and the neuroreflex theory of the production of
HOA. Radionuclide scintigraphy is particularly useful,
and the typical pattern of radionuclide accumulation
along the periosteal surface may precede the changes on
standard roentgenograms (L167).
HOA in childhood is an infrequent occurrence.
Clinical features include clubbing of the fingers and
toes, arthritis, chronic periostitis of the long bones, and
manifestations of the primary disorder. The abnormality has been associated with congenital heart disease,
bronchiectasis, pneumonia, and cystic fibrosis. Conversely, the association with malignancies has rarely
been pointed out and is apparently extremely rare, having been reported only in a few cases of Hodgkin’s disease and other lymphomas. A 9-year-old boy was found
to have metastatic osteogenic sarcoma with lung metastases; he failed to respond to chemotherapy following
thoracotomy with resection of the metastatic lesions
(H198). In addition, an 11-year-old boy reportedly developed undifferentiated epithelial cell carcinoma of the
nasopharynx with neck and lung metastases associated
with HOA (A81).
Finger clubbing was described in association
with leiomyoma of the ileum (F32), cerebral glioma
(W 104), and thalassemia (S232). The available evidence
in the patient with thalassemia suggests that the pres-
ence of the clubbing might be related to the severity of
iron overload and hepatic dysfunction. Clubbing also
occurred in a 24-year-old woman for whom a radial arteriovenous fistula for use in hemodialysis had been
constructed (L50).
When idiopathic HOA, a form occurring predominantly in males and not associated with any known
disease process, is associated with pachyderma, it is
called pachydermoperiostosis. In extremely rare instances, idiopathic HOA may occur without pachyderma. Three cases of idiopathic HOA without pachyderma were reported in blacks, in whom the condition
appears to predominate (B 133). Five additional patients
with pachydermoperiostosis were described; 4 showed
acroosteolysis, a finding not well recognized in this rare
syndrome (G27 1).
Avascular necrosis
Biology and pathogenetic mechanisms. A useful
overview of avascular necrosis provided a skeptical
evaluation of many of the proposed pathogenetic mechanisms and an analysis of the many interacting factors
leading to bone death (G114).
In a review of the epidemiology of traumatic and
nontraumatic osteonecrosis, the major common denominator again appeared to be circulatory compromise
(58). Many factors predisposed to post-hip fracture
osteonecrosis, including the type of fracture and the
quality of fracture reduction and union. Nontraumatic
osteonecrosis was associated with systemic disorders including hyperlipidemia, alcoholism, corticosteroid use,
and other less common problems.
Another review stressed the number of case reports since 1957 implicating excessive corticosteroids as
a cause of nontraumatic osteonecrosis (F56). It was
pointed out that induced hypercorticoidism in rabbits
leads to severe hyperlipidemia, fatty liver, systemic fat
emboli, terminal vascular obstruction in bone, and associated osteocytic death. The clinical features and management of avascular necrosis of the femoral head in
adults were again discussed in detail (S279).
In a study with New Zealand white rabbits, 2 experimental groups received glucocorticoids and 2
groups received semirestricted or ad lib diets. Experimental animals showed osteocyte death, necrotic debris,
and intravascular fat emboli in the distal femur by the
second week. Blood analysis revealed marked elevation
of triglycerides, cholesterol, free fatty acids, and total
lipids (G 128). These workers concluded that osteonecrosis probably develops as a result of the interaction
of several corticosteroid-induced alterations, including
circulating fat emboli and inflammatory free fatty acids
or prostaglandins. It was interesting that prostaglandins PGB and PGF2a were also elevated in the experimental animals.
Thirty-eight hips in 26 patients with alcoholism
associated with ischemic necrosis of the femoral head
were evaluated in detail (H217). The clinical state was
characterized by elevated intraosseous pressure, as well
as poor or absent visualization by intraosseous venography. Core decompression with removal of a bone plug
from the head and neck of the femur was said to relieve
pain in many patients. In advanced disease, core decompression was thought to offer relief of symptoms
without preventing further collapse of the femoral head.
One study in rabbits suggested that clofibrate therapy
might significantly modify steroid-induced changes in
serum lipid levels and protect the microcirculation of
the femoral head (W28).
A careful review of dysbaric osteonecrosis suggested that the bone disease might be independent of
decompression sickness (C 127). The incidence of osteonecrosis was influenced by the number of hyperbaric
exposures, extent of pressure, degree of obesity, and
possibly the rate of compression. It was believed that
while the pathogenesis was unclear, it was probably due
to ischemia, with gas bubbles causing direct or indirect
circulatory impairment. The peculiar vulnerability of
bone was thought to be related to gas supersaturation of
the fatty marrow, sensitivity to extravascular gas pressure because of tissue rigidity, and poor vascularization.
It was also proposed that uranium 238, an isotope present within the body and concentrated principally in the
ends of long bones, might promote nucleation and subsequent gas bubble formation. Principles for avoiding
or preventing osteonecrosis in caisson work were presented (57 1). Suggestions included oxygen decompression under conditions favoring isobaric nitrogen elimination from tissues and residence in compressed air
habitats with pressure adjusted to that of the tunnel.
The management of the osteonecrosis in sicklecell anemia was again reviewed (C 131).
Articular cartilage overlying osteonecrotic femoral heads from patients with longstanding symptoms requiring surgical treatment showed few histologic, biochemical, or metabolic changes, and there was not
evidence of chondrolysis and little evidence of osteoarthritis (M53). [It would have been interesting to have
information concerning some of the measures of proteoglycan aggregation in addition to the analytical data
provided. Ed.]
In a review of the pathologic findings of osteonecrosis occurring in divers, it was suggested that the
histopathologic changes indicated a significant relationship between the occurrence of osteonecrosis and a disturbance of blood supply caused by a platelet aggregation, red cell sludging, and thrombosis associated with
intravascular air bubble formation (K53,54).
Experimental osteonecrosis in the femoral head
of adult rabbits led to a somewhat different picture than
that noted above in humans (K73). The death of the
bone results in the proliferation of capillaries and undifferentiated mesenchymal cells, leading to bone regeneration. New bone formation fails to keep pace with bone
resorption, leading ultimately to loss of subchondral
bone mass. In this model there was penetration by capillaries and resorption of tissue, including cartilage. This
was followed by a cartilaginous response resembling
osteoarthritis with the development of a destructive synovial pannus which spread over and destroyed the cartilage.
Diagnostic procedures. In a roentgenographic
study of 705 alcoholics and 100 controls, it was concluded that the x-ray findings used for the diagnosis of
idiopathic avascular necrosis of the femoral head, as
outlined by the Decompression Sickness Panel of the
British Medical Research Council, were not valid in detecting the early or subtle changes of necrosis of the
femoral head, and that the changes found in those
groups are probably nonspecific (S254). Other workers
apparently found the British Medical Research Council
recommendations for radiographic analysis of patients
with possible osteonecrosis to be more useful (H101).
Scintigraphy using 9 9 m Tdiphosphonate
thought to be useful for diagnosing osteonecrosis before
irreversible changes were visible by routine x-ray techniques. Scintigrams obtained within weeks of an
avascular insult delineate a cold area, whereas examination months after an insult, when reparative processes
are underway, may produce a hot area on scintiscan
(A55,D 1).
In other radionuclide studies, it was suggested
that 9 9 Tsulfur
colloid and 99Tcpyrophosphate might be
valuable in assessing bone vascularity in diseases associated with impaired circulation (W53).
Scans with 99"technetium sulfur colloid were
used as early as 24 hours following injury to permit
early evaluation of circulation in the femoral head and
were thought to facilitate surgical management of these
patients (M2 1 1). Many patients received repeat scans
after 1 to 3 years, and scan accuracy, as corroborated by
histology and clinical results, was said to be 95%. Super-
selective angiography of the hip provided good visualization of the superior capsular arteries (T50). This procedure may have both diagnostic and prognostic value.
Treatment. Transtrochanteric anterior rotational
osteotomy of the femur was proposed as a new approach for the treatment of avascular necrosis of the
femoral head (S407). Results in 41 hip operations suggest that the procedure prevents progression of femoral
head collapse and preserves joint surfaces. Subsequent
prosthetic replacement is not precluded at a later date if
indicated. This procedure may be worthy of consideration in young or middle aged individuals with osteonecrosis localized to the anterosuperior aspect of the
femoral head. Muscle pedicle grafts were thought to be
useful in revascularizing some necrotic bone segments
in osteonecrosis of the hip (M210). Thirteen of 23 patients were described as having had good results from
this procedure.
Surgical problems associated with aseptic necrosis of the femoral head were reviewed, and it was concluded that the treatment of choice was total hip replacement, regardless of underlying pathology (S326).
Total hip replacement was carried out for osteonecrosis
of the femoral head following cardiac transplantation in
2 patients (B301).
The diagnostic features and course of osteonecrosis of the knee were again described (A37,B57).
Characteristically, the condition appears in healthy
older individuals with onset of sudden rest pain in the
medial aspect of the knee, aggravated by weight-bearing. While early clinical and radiographic findings may
be meager, usually a characteristic radiographic lesion
develops within 2 to 6 months. The relationship of medial meniscal tears to spontaneous osteonecrosis of the
knee was emphasized, and the importance of early detection of such tears by arthrography and their prompt
treatment in order to avoid subsequent osteonecrosis
was emphasized (N69).
Several reports described aseptic necrosis following renal transplantation (I 1,L86,N49,P5,U4). Renal
transplantation in children was attended by the development of aseptic necrosis in 6% of 171 recipients of allografts (U4). Another report concluded that avascular
necrosis in transplanted patients was due neither to
parathyroid overactivity nor vitamin D deficiency (P5).
Avascular necrosis was reported in a group of 5
patients with clearcut myxedema, but the mechanism of
the association was unclear (R169). Another patient
with aseptic necrosis as a presenting manifestation of
endogenous Cushing's disease was reported (S 167). The
common single lesion found in 401 stapedectomy speci-
mens removed for otosclerosis proved to be avascular
necrosis in 25% of the specimens (W197). The clinical
and radiographic findings in Legg-Calve-Perthes disease were the subject of several reviews (C147,G51,
Aseptic necrosis was described as a complication
of dislocation of the metacarpophalangeal joint (G92).
Avascular necrosis in a 9-year-old girl treated with cor-
ticosteroids was presented, emphasizing that this complication of steroid therapy is no respecter of ages
(H2 13). Freiberg’s disease (osteochondrosis of the second metatarsal head) was successfully treated in a 12year-old girl with silicone rubber implants (B200). An
additional series of steroid-induced avascular necrosis
of the femoral head was reported (C229).
Benign neoplasms
Synovial chondromatosis. Histopathologic study
of 30 patients with synovial osteochondromatosis revealed that the process appeared to follow a 3-phase
temporal sequence: 1) active intrasynovial disease only,
without loose bodies, 2) transitional phase, with both
active intrasynovial proliferation and loose bodies, and
3) multiple free osteochondral bodies without demonstrable intrasynovial disease (M237). Three patients in
this series had residual extrasynovial cartilaginous lesions which persisted and grew after synovectomy, causing recurrence of clinical symptoms in the absence of
recurrent intrasynovial disease.
A number of case reports of synovial chondromatosis of the temporomandibular joint were presented (A43,M243,R120,127). The initial diagnosis in 1
patient was possible parotid tumor (M243). Patients
were most frequently female, a reversal of the sex incidence found with the more common involvement of
the knee, hip, or shoulder (R127).
Four patients with extraarticular synovial chondromatosis and 1 with combined intra- and extraarticular involvement were described (S2 10). Such lesions were thought to arise in tendon sheaths or bursae,
and the importance of recognizing their synovial origin
in order to avoid an erroneous diagnosis of chondrosarcoma was emphasized. Report of a tenosynovial chondroma in a 73-year-old woman stressed the same point
and noted that these tumors were benign and that,
whereas they occasionally recurred, they did not metastasize (S280).
Pigmented villonodular synovitis. A 2 1-year-old
black woman with a history of intermittent locking,
swelling, and pain in her right knee and a palpable
loose body around the medial aspect of the joint was
found on surgery to have localized villonodular synovitis (W 182). A preoperative contrast arthrogram had
been normal. However, in a 40-year-old man, arthrography demonstrated the characteristic synovial pattern
of diffuse, pigmented villonodular synovitis (PVS) prior
to surgical intervention (H28).
Two patients with multiple joint involvement
with PVS were described (C225, L130). In a 55-year-old
woman, involvement of the right third and left second
distal interphalangeal finger joints was initially thought
to represent Heberden’s node formation until the characteristic pathologic changes were demonstrated in the
excised tissue (C225). A 7-year-old girl underwent synovectomies of the left elbow and the flexon tendon
sheaths of both feet with a pathologic diagnosis of PVS
(L130). However, between ages 8 and 14, she continued
to have swelling of various joints and at age 14 underwent a synovectomy of the right knee and, in the following 18 months, synovectomies of both ankles and the
right elbow. Some pathologists believed that the lesions
might represent synovial hemangioma. The patient also
had pulmonic stenosis, borderline intelligence, and scoliosis, suggesting the possibility of a more generalized
disease process.
Two case reports of PVS mimicking primary
bone neoplasm appeared (J43,K98). Histologic features
of both PVS and synovial chondromatosis were observed in synovial tissue from the temporomandibular
joint of a 62-year-old woman initially suspected of having a parotid tumor (R5).
Eleven patients with giant cell tumors of tendon
sheath (pigmented villonodular tenosynovitis) were examined by light and electron microscopy ((253). The giant cells were found to have many similarities to normal
osteoclasts, while the stromal cells had similarities to
primitive mesenchymal cells, osteoblasts, fibroblasts,
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disease, cartilage, bones
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