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The radiographic changes of healed extremity fractures in eight children with spinal cord lesions included cortical thinning and metaphyseal splaying. A history of a spinal cord lesion such as meningomyelocele should alert the radiologist to the possibility of a healed fracture causing metaphyseal flaring.
Generalized enchondromatosis is a newly delineated type of enchondromatosis. Radiographically there are multiple enchondromata in almost all metaphyses of the long and short tubular bones and the lesions are in almost the same stage of development with mild platyspondyly and skull deformity. The pelvic changes are characteristic and, together with the metaphyseal changes of the long and short tubular bones, are probably diagnostic of this disorder. The present case demonstrates advanced radiographic features of this disorder associated with unusual soft tissue calcifications and hemangiomas. The presence of hemangiomas in our case as well as three cases in the literature suggests this feature is more than a coincidence.
The effects of cyclophosphamide were studied 2, 5 and 9 days after terminating a two-week every-second-day intraperitoneal medication in young, male rats. The cytostatic effect was assessed by counting white blood cells (WBC) in arterial blood. WBC counts were reduced by 72% of control values at 2 days and regained normal values at 9 days after an overshooting leucocytosis at 5 days. Compared to control animals, the treated rats had significantly reduced body weights, metaphyseal bending strength and longitudinal growth of the femur at all time intervals observed. The torsional strength of the femur diaphysis and the strength of wounded skin were not affected at 2 days, but significantly reduced at 5 and 9 days. The tensile strength of intact skin was not found to be affected by the drug. From the 5th to the 9th day after ending medication, the curves for control and treated animals were assuming parallel slopes regarding metaphyseal bone strength, longitudinal bone growth and tensile strength of wounds. This may indicate reversion of the drug effects approximately one week after terminating medication.
Pathologic, ultrastructural and radiologic studies are described on 3 infants with the rhizomelic form of chondrodysplasia punctata. Radiologic criteria in the young infant include radiolucent coronal clefts dividing all or most of the thoracic and lumbar vertebral bodies, short humeri with flared metaphyses and punctate calcifications commonly present adjacent to the ossified ischial and pubic bones and less commonly in other locations. In late infancy and childhood the radiologic criteria include demineralization in all bones with slow maturation, flat vertebral bodies, short humeri and femora, metaphyseal flaring, especially in the distal humerus, proximal femur and proximal tibia, immature shape of pelvis, and disappearance of the punctate calcifications with advancing age. The histologic changes of the resting cartilage include areas of degenerating cartilage which had become partially calcified, cystic changes with severe disturbance of the maturation of the cartilage at the physial plate, and the formation of cancellous bone directly on resting cartilage. Ultrastructural changes are characterized by degeneration of chondrocytes, delicate collagenous fibrils without visible periodicity, and the presence of flocculent material within greatly distended endoplasmic reticulum.
Skeletal survey in an 8-year-old girl with primary hyperoxaluria and in chronic renal failure revealed wide translucent metaphyseal bands and defects at the ends of the long bones. Similar disordered bone growth was seen as rims of rarefaction around the large epiphyses, the patella and the tarsal bones. Bone density was generally increased. The translucent metaphyseal bands were separated from the diaphyses by an irregular line of increased bone density. Biopsy of a bone defect showed massive deposition of oxalate crystals indicative of advanced oxalosis of bone.
Radiographic analysis of 24 cases of hypophosphatasia (H) from 9 Paediatric Centres was performed. 3 cases were of neonatal (lethal), 18 cases of infantile (severe) and 3 cases of late (benign) type. Some of the patients were in reality borderline cases between these groups. In the authors' material all the patients showed radiographic signs of the disease. These were divided into diagnostic, characteristic and suggestive features. All of the patients had in common generalised (usually irregular) osteoporosis, generalised (usually irregular) metaphyseal changes, craniostenosis (13 of 18 infantile cases) or widened cranial sutures and ofter bowing of the long bones. Besides the well know radiographic features of hypophosphatasia some less well known, rare or 'new' ones such as, 1. spurs of the long bones (Bowdler sign), 2. distal femoral central metaphyseal defects and epiphyseal defects, 3. S-like deformities of the tibiae, 4. abnormal shape of the distal phalanges of the fingers, 5. multiple rib fractures and slender bones, 6. wedging of the lower thoracic and upper lumbar vertebrae, 7. partial premature fusion of the epiphyses, 8. nephrocalcinosis, 9. loss of lamina dura around the teeth, 10. variation in radiographic appearances of a pair of siblings with lethal form, and, 11. rapid changes in roentgen appearances. are discussed. In two of our patients (siblings) phosphoethanolamine was undetectable in the urine. The authors doubt if a normal skeletal survey may be present at any stage in any of the three major types of hypophosphatasia.
Five babies, aged between one to three months presented with clinical and serological evidence of syphilis. In addition to the usual periosteal and metaphyseal bone lesions of lues, areas of focal lucencies and sequestra were present. The association of sequestra in focal diaphyseal erosions may represent a non-dystrophic manifestation of the disease. The lesions were attributed to the syphilitic process. Trivial trauma to disorganised metaphyses, dystrophic changes and possibly focal osteomyelitis account for the spectrum of bone lesions in congenital syphilis. No tubular bone appears immune to these processes.
Reconstruction surgery in posttraumatic deformities in the lower leg and the foot is indicated under different aspects: restoration of function, improvement of prognosis, improvement of function and melioration of physical appearance. Technically effective surgical means are available to date, such as: metaphyseal closing and opening wedge osteotomies, diaphyseal step-cut osteotomies, diaphyseal and metaphyseal displacement osteotomies, lengthening osteotomy, external fixation, revision of infections, joint revisions, scar revisions, nerve revisions, plastic skin operations.
Silicon is known to ensure an essential role in the formation of cross-links between collagen and proteoglycans during bone growth. In this study, we have evaluated the short-term effects of a preventive treatment with silanol, a soluble organic silicon (Si), on trabecular bone in mature ovariectomized rats. Three-month-old rats were sham-operated (sham) or were ovariectomized (OVX) and treated with 10 micrograms/kg/day of 17 beta estradiol (E2), or with 0.1 mg Si/kg/day or 1.0 mg Si/kg/day of silanol for 1 month. Plasma alkaline phosphatase and osteocalcin levels were increased by 50% in OVX rats compared with sham rats and were corrected by E2 but not by silanol treatment. The trabecular bone volume measured at the tibial metaphysis was decreased by 48%, and histomorphometric indices of bone resorption and formation were increased in OVX rats compared with sham, and these parameters were corrected by E2 treatment. Treatment of OVX rats with silanol decreased the osteoclast surface by 31% and the number of osteoclasts by 20%. The mineral apposition rate, the bone formation rate, and the osteoblast surface at the tibia metaphyseal area were increased by 30% at the higher dose of silanol compared with OVX rats. In contrast, silanol treatment had no effect on the periosteal apposition rate. The reduction of the metaphyseal bone resorption and the increased bone formation induced by silanol resulted in a slight improvement of the trabecular bone volume (+14%) compared with controls.(ABSTRACT TRUNCATED AT 250 WORDS)
Microradiographically detectable alterations of the bone structure in the femur of young rats induced by monomeric 241-Am(III) (i.v., 30 muCi/kg) were studied. The morphometric and dosimetric measurements were carried out by means of an electronic image analyzer. 8 weeks after injection of 241-Am a characteristic alteration of the frequency distribution of the chord lengths over the trabeculae in the epiphysis and over the metaphyseal marrow spaces was found. The structure of the spongiosa is irregular with both large, coarse and small fragmented trabeculae. The complexity of the bone architecture and the area of the endosteal surfaces is reduced. The surface/volume ratio in control animals varies between 36 mm-1 in the epiphysis and 64 mm-1 in the region of the epiphyseal cartilage plate. From the specific surface burden (pCi/mm2) the average dose rates were determined. There is no significant difference between the calcified tissue fraction in controls and animals with 241-Am, with the exception of the metaphyseal band where the locally high dose rates cause a devitalization of the tissue with inhibition of bone resorption as well as an abnormal trabeculation in the metaphysis. Treatment by Ca-DTPA reduces the 241-Am deposition nonuniformly and the pathological manifestations are markedly less pronounced. The mean trabecular width is about 100 mum in the epiphysis and has a minimum of 40 mum in the central part of the epiphyseal plate. The mean chord length over the marrow spaces varies between 90 and 210 mum.
Epiphyseal slipping in uraemia differs strikingly from juvenile epiphyseal slipping with respect to pathology and therapy. Based on our own experience with the treatment of 8 uraemic children with epiphyseal slipping, an effort was made to establish the respective indications for conservative and surgical treatment. Mechanical stabilization of slipped epiphyses was achieved within a few weeks without any surgery and usually without parathyreoidectomy by vitamin D3 alone. The initial dose was 10,000 to 30,000 I.U./day, the total curative dose 1.8 to 5.6 millions I.U. Prolonged immobilization was unnecessary. Rising urinary calcium excretion was a valuable indicator of vitamin D intoxication even in advanced renal failure. In one case, pronounced metaphyseal deformations (distal femur, distal tibia) required surgical correction before the ability to walk normally was restored. - The following therapeutical approach is recommended: metabolic bone disease must be cured by vitamin D therapy with or without parathyreoidectomy. Osteotomy to correct metaphyseal deformities or coxa vara epiphysaria never should be performed before metabolic bone disease is healed.
A male infant with "classical" Seckel syndrome and a girl with osteodysplastic primordial dwarfism type II are described. The boy with classical Seckel syndrome had severe brain dysplasia, a finding hitherto unreported in patients with this syndrome. The patient with osteodysplastic dwarfism type II had skeletal abnormalities including lumbar scoliosis, a small and high pelvis, metaphyseal flaring of the distal radii and ulnae, V-shaped metaphyseal flaring of the distal femorae, and short metacarpals and phalanges. The mother of this girl was short, microcephalic, and had disproportionately short forearms and legs. In view of this, dominant inheritance of the disease was suggested.
Among children with recessive metaphyseal dysplasia, cartilage hair hypoplasia, as described by McKusick is often recognized only during the 2nd year or later. The early radiological changes observed in six children with cartilage hair hypoplasia demonstrate the misleading aspect of this chondrodysplasia: micromelia, massive appearance of the long bones and round inferior femoral epiphyses, without distinct metaphyseal involvement. Early diagnosis permits the organisation of clinical, immunological and orthopaedic follow up and allows for correct genetic counselling.
Six children with Shwachman-Diamond syndrome have been diagnosed and treated in our hospital since 1986. We describe the radiological and sonographic findings of this rare disease which is characterized by metaphyseal chondrodysplasia, neutropenia and pancreatic exocrine insufficiency. It presents with variable extremity shortening, "cup" deformation of the ribs, metaphyseal widening and hypoplasia of the iliac bones, and increased echogenicity of the pancreas without change in size. We discuss the differential diagnosis and review the literature.
We report two cases of a platyspondylic lethal osteochondrodysplasia in siblings. The condition affects predominantly the metaphyses of the axial skeleton associated with limb shortening and mild platyspondyly. The radiological and pathological features correlate very closely with Sedaghatian's lethal metaphyseal chondrodysplasia, which has previously been described only in Iranian Nationals. Both the radiological and pathological appearances will be described, together with a discussion of the differential diagnosis, the genetical implications and a review of literature.
Bone changes in thalassemic patients receiving deferoxamine therapy for iron chelation include metaphyseal and growth plate irregularities. We present a case of an 8-year-old female with thalassemia major, who had magnetic resonance imaging after plain radiographs had shown metaphyseal changes in the distal femur. The signal characteristics of these abnormalities were consistent with hyaline cartilage; the surrounding marrow showed no evidence of iron overload.
Six children with Shwachman-Diamond syndrome have been diagnosed and treated in our hospital since 1986. We describe the radiological and sonographic findings of this rare disease, which is characterized by metaphyseal chondrodysplasia, neutropenia and exocrine pancreatic insufficiency. It presents with varying extremity shortening, "cup" deformation of the ribs, metaphyseal widening and hypoplasia of the iliac bones, as well as increased echogenicity of the normalized pancreas. We discuss the differential diagnosis and review the literature.
Analysis of 330 exostoses in 18 patients affected by hereditary multiple exostoses disease suggested a new classification of exostoses as eccentric or full-thickness. Radiographically arrest of metaphyseal remodeling with failure of coning and persistence of the primary metaphyseal trabeculae was evident in full-thickness exostoses. Similar bone lesions can be obtained experimentally with inhibitors of bone turn-over. A localized, peripheral defect in remodeling over a limited time can give a satisfactory explanation also for the origin of eccentric exostoses. The thesis that this is the basic mechanism of exostosis formation is presented.