A DISORDER OF ENDOCHONDRAL OSSIFICATION INDUCED BY DEXTRAN SULPHATE.
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The transverse area and flattening ratio of the spinal cord were determined with preoperative computed tomographic myelography in 103 patients with cervical compression myelopathy: cervical spondylotic myelopathy (n = 44); ossification of the posterior longitudinal ligament (n = 39); and cervical disc herniation (n = 20). With these values and other clinical items (eg, age, duration of symptoms, preoperative severity), a linear model to predict postoperative recovery was attempted by multiple regression analysis. In cervical spondylotic myelopathy and ossification of the posterior longitudinal ligament, the transverse area of the spinal cord and the duration of symptoms were accepted as effective explanatory variables to predict recovery. In cervical disc herniation, regardless of the transverse area or duration, the recovery was good, and pathologic state was considered essentially different.
OBJECTIVE: To report five children with slit-ventricle syndrome who were found to have increased intracranial pressure despite functioning cerebrospinal fluid shunts. METHODS: Computed tomographic scans demonstrated erosion of the inner table of the cranium and sclerosis of the cranial sutures, particularly the coronal suture. Magnetic resonance imaging scans demonstrated no cerebrospinal fluid over the convexities. The patients were treated with cranial expansion operations that included removal of the sclerotic sutures, which were examined histologically. RESULTS: Postoperatively, symptoms resolved for all children. Sutures were abnormal and contained foci of cartilage and bone within abnormally arranged fibrous tissue. CONCLUSION: We postulate that chronic overdrainage of cerebrospinal fluid via shunts dampens the normal cerebral pressure waves; growth of the calvarium is thus understimulated, and this leads to ossification of the sutures, which become unable to expand to allow normal brain growth. Shunt-induced craniostenosis should be considered for children with symptoms of slit-ventricle syndrome for whom shunts are functional but intracranial pressure is increased. Cranial expansion operations may be more appropriate treatments than subtemporal decompressions for such children, given the diffuseness of the suture pathological features.
Ossifying skeletal muscle angiomas are relatively rare occurrences. The preoperative diagnosis is exceedingly difficult, although ossification may be reflected radiologically as a "Swiss cheese" appearance. Two patients are described whose skeletal muscle angiomas contained osseous tissue. The osseus tissue varied from microscopic foci to an abundant amount which was readily recognizable radiologically. The pathologic and radiological features are discussed and these appearances to hemangioma and lymphangioma are presented.
Recent progress in genetics and mouse genomics enables researchers to unveil the molecular basis for mouse phenotypes that express pathologic calcification in soft tissue and/or articular tissues. A newly identified multipass transmembrane protein, ANK, appears to function as an inorganic pyrophosphate (PPi) transporter or regulator of PPi transport. Abnormal extracellular PPi (ePPi) metabolism has been implicated in abnormal calcification, decreased concentrations predisposing to basic calcium phosphate (BCP) deposition, and increased concentrations promoting calcium pyrophosphate dihydrate (CPPD) crystal deposition in articular tissues. The chromosomal location of human ANK overlaps the locus identified in several kindreds affected with familial chondrocalcinosis. Deficient generation of ePPi by the ectoenzyme nucleoside triphosphate pyrophosphohydrolase also results in excessive ossification and ectopic deposition of BCP crystals in tiptoe-walking mice and PC-1 null mice. Recent studies reinforce the important regulatory role of ePPi in pathologic and physiologic calcification.
Congenital pseudarthrosis of the clavicle is usually situated on the right clavicle. Pathological data on pseudarthrosis are scant. The histological analysis of five cases treated by 'en bloc' surgical resection are reported. Enchondral ossification was observed on both sides of the resected pseudarthrosis in two cases. Less characteristic enchondral features were noted in three cases. These findings confirm that the pseudarthrosis is due to the failure of coalescence of the two primary ossification centers of the clavicle.
Somatometric and radiographic study of bones of the hand and of the distal forearm was performed in 258 healthy children and adolescents (148 boys and 110 girls) aged 11-17 years and in 115 children (84 boys and 31 girl) aged 13-16 years which were under the observation due to growth retardation. It was shown that within the groups of the same age children with various somatometric characteristics possessed different levels of skeletal (and therefore, biological) maturity. In the group of healthy children, different variants of skeletal maturation were established, which corresponded to the constitutionally stipulated rates of somatic development including accelerated, average and retarded. In children with growth retardation, the third (retarded) variant of somatic development rate was prevalent, which was also found in the group of healthy children and was accompanied with its peculiar osteogenesis characteristics. In the other cases, when such abnormalities of skeletal maturation as asymmetry in the appearance of ossification centers and synostoses as well as the presence of pseudoepiphyses, were observed, growth retardation could be caused by some endocrine pathology. Thus, the radiographic study enables the differentiated approach to the evaluation of the morphological status of children and adolescents and permits to avoid the unjustified administration of hormonal treatment.
Growth hormone (GH) and insulin-like growth factor I (IGF-I) play important roles during growth, including effects on enchondral ossification. GH plasma concentration is strongly influenced by the nutritional status of the dog. Fasting induces a non-responsiveness of the liver to GH, leading to a decrease in IGF-I release by the liver and a delay in growth. Overfeeding leads to abnormal skeletal development, i.e., osteochondrosis and hip dysplasia. Subnormal plasma concentrations of sex hormones lead to a delay in physiologic epiphysiodesis, whereas high levels of sex steroids may induce premature physeal closure. Dietary calcium, phosphorus, and vitamin D are important factors in the process of enchondral ossification. If these nutrients are inadequate, imbalanced, or oversupplemented, skeletal development in young growing dogs can be disturbed, leading tot severe pathologic changes. Energy requirements decrease gradually during the first year of life and unrestricted feeding can easily lead to pups being overweight. The protein requirements are strongly influenced by the quality and digestibility of protein sources in the food. In dog foods 12-14 g protein/1000 kJ appears to be adequate for growth. It is likely that the two most commonly made mistakes in dog nutrition in the Netherlands, are oversupplementation with energy and/or calcium. Restricted feeding is advised during growth and no food-additives are required when commercially prepared complete dog foods are used.
In all histopathological studies of the osteochondroses, the one constant finding is a dysplastic change in the cartilaginous epiphysis and apophysis. This gives rise to disturbances of enchondral ossification, diminished mechanical resistance of the abnormal cartilage, and to ischaemic changes in the ossific nucleus. This has been demonstrated histologically only in Perthes disease and Köhler's disease and probably reflects vascular damage provoked by abnormal cartilaginous growth. Cartilage dysplasia could constitute the fundamental pathogenetic background common to all the osteochondroses.
Hypothyroidism was diagnosed in a 5-month-old Thorough-bred colt by clinical and clinico-pathology examinations, thyroid stimulating hormone response test and by microscopic evaluation of the thyroid gland. Skeletal lesions included delayed appearance of ossification centers and delayed development of bone in cartilage models, delayed closure of epiphyseal plates, transverse trabeculation in metaphyses, osteochondrosis dissecans and subchondral cysts.
We studied 3 cases with myelopathy caused by cervical spinal canal stenosis (developmental), who had been suffering from walking difficulty followed by tetraplegia, clinically and pathologically. In all 3 cases, mental retardation and/or cerebral palsy was diagnosed. We hypothesized that the brain damage in the developmental stage might also cause developmental disturbance in the cervical spine. In all cases, pathological investigation showed decreased antero-posterior diameter and degeneration in the gray matter and in the lateral and posterior column in the involved cervical spinal cord. The findings, such as relative preservation of the anterior column and cyst formation in the gray matter, were thought to be in common with cervical spondylotic myelopathy or ossification of the occipital longitudinal ligament (OPLL) which had been reported before. In one case we found aberrant peripheral nerve bundles and peripheral type remyelination in the transverse spinal cord lesion. Compared to the pathological change in the OPLL, our cases showed more severe degenerative change in the spinal segments with a relatively preserved antero-posterior diameter of the spinal cord, which supports the theory that the dynamic factor plays a more important role than the static compression factor. We concluded that the aging process and/or dystonic neck movement added spondylotic change to the narrow canal, and excess movement of the neck and/or falls caused dynamic injury to the spinal cord and secondary circulatory disturbance further worsened spinal lesions. When elderly patients with cerebral palsy develop motor symptoms, we should consider cervical spinal stenosis as a possible cause.
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OBJECTIVE: To study the pathological features of sacroiliitis, aiming at improving the early diagnosis of ankylosing spondylitis (AS). METHODS: CT guided needle biopsy of sacroiliac joint (SIJ) was performed in 96 cases of spondyloarthropathy (SpA) patients with 3 autopsy materials of non-SpA sacroiliac joints serving as control. Pathological features were studied by two independent observers. Clinical data were collected and analyzed. RESULTS: No pathological change was noticed in the control group. Among the 96 cases of SpA, pathological changes were found in 76 cases, including bone marrow inflammation, pannus formation, subchondral bony plate destruction, cartilage degeneration/erosion, synovitis, and enthesitis. The aforementioned first 4 findings were present in 45 cases of grade 0/I CT sacroiliitis. In the synovial specimens obtained, synovitis was seen in some cases of grade I and all of >/= grade II CT sacroiliitis,while no inflammatory change was noticed in all cases of grade 0 CT sacroiliitis. Frequency rate of cartilage erosion and ossification in grade 0/I CT sacroiliitis was the lowest in comparison with the other groups. Moreover, in cases of grade 0/I CT sacroiliitis, cartilage erosion only present at the bony plate side, while the joint cavity side was not affected. The inflammatory index was significantly lower in cases of grade IV CT sacroiliitis than that in the other groups. Enthesitis only presented in some cases of >/= grade II CT sacroiliitis. Among the 65 undifferentiated spondyloarthropathy patients, 45 were diagnosed as AS after SIJ pathological examination. The mean disease duration of these 45 cases was significantly shorter than that in cases of >/= grade II CT sacroiliitis. CONCLUSIONS: Inflammatory changes of SIJ did exist in cases of the < grade II CT sacroiliitis. Sacroiliitis probably initiated with bone marrow inflammation, followed by pannus formation, subchondral bony plate destruction, and cartilage degeneration/erosion, eventually leading to fibrosis, ossification and joint fusion. Synovitis and enthesitis were not the very early changes of sacroiliitis. Pathological examination was beneficial to the early diagnosis and differential diagnosis of ankylosing spondylitis.
In the past few years patients having severe head trauma have survived in growing numbers, and it is likely that they will be more frequently seen on rehabilitation units. They display, in addition to direct structural damage, medical and neurologic problems which may be encountered during their rehabilitative phase. The purpose of this study will be to identify those medical and neurologic problems of consequence and describe their frequency of occurrence within a population of head injured patients. A consecutive series of 180 patients with head trauma undergoing rehabilitation were therefore reviewed, and the type and frequency of medical problems were noted. Neurologic, gastrointestinal, genitourinary, respiratory, cardiovascular, skin, musculoskeletal, and endocrinologic problems were encountered most frequently. Of these, ventricular dilatation, posttraumatic seizures, abnormal liver function tests, hypertension, thrombophlebitis, respiratory infections, periarticular heterotopic ossification, and pituitary-hypothalamic dysfunction are discussed in terms of their morbidity, clinical significance, and therapeutic approach. In many instances, these problems were not identified in the acute care hospital. Awareness of these potential conditions during the rehabilitation period can result in early detection and treatment.
We present a case of achondrogenesis type II with normally developed extremities that was confirmed with postmortem ultrasonographic and radiographic examination. The length of the long bones may vary and the diagnosis of achondrogenesis should not be ruled out with normally developed extremities. Intrauterine sonographic examination of the vertebrae is very important and the absence of vertebral body ossification may be the unique finding of achondrogenesis type II. Axial ultrasonographic images and postmortem plain radiographs are useful to clarify the pathology.
The ossification pathways of both vertebral centra (i.e., vertebral bodies) and neural arches were studied in human embryos and fetuses (CR-length between 38 and 116 mm). A clearing and double-staining method for whole embryo or fetus, using alcian blue and alizarin red S, allowed an easy and precise detection of the morphology of the whole vertebral column and every single vertebra. Both cartilaginous and bony components were clearly visible. Different temporal and topographical patterns of ossification were shown for the centra and arches; the latter were respectively proximal-distal (i.e., bidirectional from a defined starting tract in T10-L1) and cranial-caudal (i.e., monodirectional). The patterns could be related to the morphogenetic processes of other structures (i.e., muscles and nerves). Moreover, the numerical survey of ossification centers provided a possible parameter for the determination of the fetal developmental age. This could be useful in the study of pathological conditions.