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PubMed2005

Bone involvement in Erdheim-Chester disease: imaging findings including periostitis and partial epiphyseal involvement.

PURPOSE: To retrospectively review the bone findings at radiography, scintigraphy, computed tomography (CT), and magnetic resonance (MR) imaging in 11 patients with immunohistochemical and histologic proof of Erdheim-Chester disease. MATERIALS AND METHODS: This study was designed as a retrospective review; approval of the institutional review board and patient consent were not required for this type of study. Eleven patients (eight men and three women; mean age, 49 years; range, 17-68 years) with Erdheim-Chester disease underwent conventional radiography of the skeleton and bone scintigraphy. Two patients underwent CT of the femora and 10 underwent CT of the skull. Eight patients underwent MR imaging. Conventional radiographs, bone scintigrams, CT scans, and MR images were reviewed in consensus by four musculoskeletal radiologists. RESULTS: All 11 patients had involvement of the long bones and normal axial skeleton, hands, and feet. Bilateral and symmetric osteosclerosis of the diaphysis of the long bones was present in 52 (26 pairs) (98%) of the 53 bone lesions visible on conventional radiographs. Osteosclerosis was heterogeneous in 65% of the patients and homogeneous in 35%. Diaphysis was involved in 100% and metaphysis in 44 (83%) lesions. Partial epiphyseal involvement sparing the subchondral bone was present in 24 (45%) lesions. Periostitis was seen in 35 (66%) and endosteitis in 50 (94%) of the 53 long bones involved. Bone scintigraphy depicted tracer uptake in all bone lesions visible on radiographs. Skull and face bone lesions were present in two patients. MR imaging depicted a replacement of the normal fatty bone marrow by heterogeneous signal intensity on T1- and T2-weighted spin-echo images. Lesion extent, epiphyseal involvement, and periostitis were clearly depicted at MR imaging. CONCLUSION: This series provides a detailed description of bone involvement in Erdheim-Chester disease. Periostitis and partial epiphyseal involvement of the long bones are also features of this disease. (c) RSNA, 2005.

Adolescent↗
PubMed1983

Ultrasonographic identification of fetal lower extremity epiphyseal ossification centers.

The reliability of the sonographic identification of the fetal distal femoral epiphyseal ossification center (DFE) and the proximal tibial epiphyseal ossification center (PTE) in the estimation of gestational age (GA) was assessed in 84 singleton pregnancies. We used the presence of a DFE to indicate a GA greater than or equal to 33 weeks, and this method was 95% sensitive, 95% specific, and accurate overall in 95% of a selected cohort of 61 cases. In the same cohort, the presence of the PTE was highly predictive (accuracy of a positive prediction 95%) of a GA greater than or equal to 35 weeks. Ultrasonographic evaluation of fetal lower extremity epiphyseal ossification centers is a promising technique for the estimation of GA during the last trimester of pregnancy.

Epiphyses↗
PubMed1994

Transient epiphyseal lesions in renal transplant recipients: presumed insufficiency stress fractures.

PURPOSE: To determine epiphyseal abnormalities in renal transplant recipients. MATERIALS AND METHODS: Initial and final findings on serial conventional radiographs and magnetic resonance (MR) images obtained in symptomatic joints of transplant recipients were determined and correlated. RESULTS: In 42 of 47 joints, T1-weighted MR images depicted 106 ill-delimited areas of low signal intensity. At 1-year follow-up, all lesions had disappeared. T1-weighted MR images depicted nine well-delimited regions of normal signal intensity delineated by a rim of low signal intensity in seven joints. No lesion had completely disappeared at follow-up. Initial radiographs either were normal or showed patchy osteoporosis (13 joints). Follow-up radiographs showed epiphyseal collapse of two femoral heads, with well-delimited lesions on MR images, and subtle subchondral sclerosis in 21 noncollapsed epiphyses. CONCLUSION: Initial MR imaging patterns of transient lesions (92% of lesions) are different from those of irreversible lesions. Transient lesions suggest insufficiency stress fractures, while irreversible lesions suggest avascular osteonecrosis.

Adult↗
PubMed2004

Soluble VEGF isoforms are essential for establishing epiphyseal vascularization and regulating chondrocyte development and survival.

VEGF is crucial for metaphyseal bone vascularization. In contrast, the angiogenic factors required for vascularization of epiphyseal cartilage are unknown, although this represents a developmentally and clinically important aspect of bone growth. The VEGF gene is alternatively transcribed into VEGF(120), VEGF(164), and VEGF(188) isoforms that differ in matrix association and receptor binding. Their role in bone development was studied in mice expressing single isoforms. Here we report that expression of only VEGF(164) or only VEGF(188) (in VEGF(188/188) mice) was sufficient for metaphyseal development. VEGF(188/188) mice, however, showed dwarfism, disrupted development of growth plates and secondary ossification centers, and knee joint dysplasia. This phenotype was at least partly due to impaired vascularization surrounding the epiphysis, resulting in ectopically increased hypoxia and massive chondrocyte apoptosis in the interior of the epiphyseal cartilage. In addition to the vascular defect, we provide in vitro evidence that the VEGF(188) isoform alone is also insufficient to regulate chondrocyte proliferation and survival responses to hypoxia. Consistent herewith, chondrocytes in or close to the hypoxic zone in VEGF(188/188) mice showed increased proliferation and decreased differentiation. These findings indicate that the insoluble VEGF(188) isoform is insufficient for establishing epiphyseal vascularization and regulating cartilage development during endochondral bone formation.

Angiography↗
PubMed1992

Somatostatin effects on cultured human fetal epiphyseal chondrocytes.

Somatostatin effects on cultured human fetal epiphyseal chondrocytes were evaluated by studying the effects of somatostatin on DNA synthesis. Cultured epiphyseal chondrocytes from human fetuses (12-40 wk old) were incubated for 48 h in Ham's F-12 serum-free medium. After this, the medium was replaced by MCDB-104 serum-free medium and the cells were incubated for an additional 48 h in the presence or absence of somatostatin 1 pM to 10 microM, with the addition of 3H-thymidine (5 microCi/mL) for the last 24 h of incubation. A significant (p < 0.02) inhibitory effect of somatostatin (1 nM to 10 microM) on 3H-thymidine DNA incorporation was observed in cultured chondrocytes from fetuses of all gestational ages studied (12-40 wk), with no significant differences among fetal ages. In conclusion, our results show that somatostatin exerts a biologic effect on cultured human fetal epiphyseal chondrocytes, as it does in its target cells. These results suggest that somatostatin could regulate human skeletal growth not only by growth hormone secretion regulation, but also by acting directly on chondrocyte metabolism. However, the physiologic significance of the latter remains to be elucidated.

Cells, Cultured↗
PubMed1989

Epiphyseal distraction monitored by strain gauges. Results in seven children.

Ten epiphyses in seven children underwent fixed-rate distraction of 0.25 mm twice daily in an attempt to achieve percutaneous leg lengthening by chondrodiatasis. The forces generated across the growth plate were recorded by means of strain gauges incorporated into the distractors. All epiphyses fractured before 33 days of lengthening. An average gain of 6.75 cm was achieved. Epiphyseal distraction at the lower femur produced many complications, but at the upper tibial epiphysis planned lengthening was achieved, with excellent bone production and few complications.

Adolescent↗
PubMed2003

Primary subacute epiphyseal and metaepiphyseal osteomyelitis in children. diagnosis and treatment guided by MRI.

We present three children with primary subacute epiphyseal and metaepiphyseal osteomyelitis. The diagnosis was delayed because of subtle radiological findings and mild general symptoms. Primary epiphyseal osteomyelitis is extremely rare. We believe that this is the first time that the MRI findings have been presented. In the first case they revealed a perforation into the knee and therefore an intra-articular epiphyseal approach was used for debridement. In the second and third cases the metaepiphyseal lesions showed considerable physical involvement and a metaphyseal approach was chosen. We believe that in this condition MRI is essential both for diagnosis and in the planning of surgical treatment.

Child↗
PubMed1998

Elongation of the epiphyseal trabecular bone in transgenic mice carrying a klotho gene locus mutation that leads to a syndrome resembling aging.

Bone diseases such as osteoporosis and osteoarthritis are regarded as age-associated diseases, and occur in a significantly increasing number of patients, but the underlying mechanisms of these age-associated bone diseases are not yet clear. We have established a transgenic mouse line by an insertion mutation. These mice exhibit many features related to precocious aging. Homozygote mutant mice, which lack expression of the newly identified targeted gene,klotho (kl), exhibit atherosclerosis, emphysema, hypogonadism and calcification of soft tissues, and die within 3-4 months. We describe here the radiological and histological characteristics of the skeletal abnormalities in the bones of the mice with a mutation in the kl gene locus. In heterozygous mice (+/kl), the skeletal patterns and structures remain normal and most features are similar to those in the wild-type, whereas histological examinations of homozygous mice (kl/kl) show abnormal elongation of the trabecular bone(s) in the epiphyses of long bones. As with their long bones, on radiographic examination the mid parts of the vertebral bones of these mice show less radiopacity compared with the wild-type, again resembling human vertebrae of osteoporotic patients. The elongation of the trabecular bones results in high radiopacity on both ends of each of the vertebrae, and in the epiphyses of the long bones. Cancellous bone volume in the epiphyses of the homozygote mice is three times that of the wild-type mice. The kl/kl mice are smaller than the wild-type litter mates and hence the size of their long bones is less than that of the wild-type litter mates. These observations, and the osteopenia in the vertebrae and long bones in these mice, suggest the presence of abnormality in bone metabolism, the elongation of the trabecular bone apparently resulting from the relatively low levels of bone resorption. Therefore, thekl/kl mutant mice could serve as an interesting tool to study the effects of the lack of the product of the new gene,klotho, on bone metabolism.

Aging↗
PubMed1998

Epiphyseal-metaphyseal enchondromatosis. A new clinical entity.

We reviewed the cases of eight unrelated children who had an unusual form of enchondromatosis characterized by unilateral enchondromas arising within the epiphyseal and metaphyseal regions of the long tubular bones of the lower extremity. Unlike previously described enchondromas, the lesions developed extensively within the epiphysis before closure of the growth plate and there was direct extension across the epiphyseal growth plate into the metaphysis. The lesions resulted in severe limb-length discrepancy and angular deformity (which increased in every patient after the time of presentation), asymmetrical premature physeal arrest, and joint incongruity, all of which necessitated numerous operative procedures. Seven patients had limb-lengthening and one had a Boyd amputation without lengthening. Five patients had a second lengthening procedure. Twenty-seven osteotomies (range, one to five procedures per patient) were done; six patients had a repeat osteotomy. Four patients had an epiphyseodesis. We believe that these lesions represent a previously undescribed clinical entity, which we termed epiphyseal-metaphyseal enchondromatosis.

Biopsy↗
PubMed2005

Solitary epiphyseal enchondromas.

BACKGROUND: Enchondromas originating in the epiphyses of long bones are rare. The purpose of the present study was to evaluate the prevalence as well as the radiographic and clinical characteristics of epiphyseal enchondromas among patients who had been referred to the Armed Forces Institute of Pathology and Walter Reed Army Medical Center. METHODS: We performed a retrospective review of 761 patients who had been referred to our two institutions over an approximately fifty-five-year period and who received a final diagnosis of enchondroma. All lesions had been biopsied, and the pathological diagnosis had been confirmed. Lesions of the hands, feet, or axial skeleton (253 patients) as well as lesions that appeared to originate in the metaphysis or diaphysis (475 patients) were excluded. Only enchondromas of the long bones that originated in the epiphysis were analyzed. The study group included thirty-three patients (twenty male patients and thirteen female patients) with a mean age of 26.7 years, including eleven patients with open physes. We performed additional descriptive analyses with regard to patient age, gender, lesion location, clinical presentation, and treatment as well as an extensive radiographic analysis. RESULTS: The most common locations were the proximal part of the humerus (ten lesions; 30%) and the distal part of the femur (six lesions; 18%). The most common presenting symptom was pain (twenty-three patients). Radiographic analysis demonstrated extensive matrix mineralization in association with twenty-three lesions. Twenty-eight of the thirty-three lesions were geographically well defined; of these, twenty-one had sclerotic borders, and seven did not. Although all lesions were centered and were predominantly located within the epiphysis, twenty of the thirty-three lesions demonstrated radiographic evidence of metaphyseal extension, including four of the eleven lesions in patients with open physes. Twenty-four lesions extended into the subchondral bone. The mean size of the thirty-three enchondromas in greatest radiographic dimension was 2.7 cm (range, 1.1 to 4.9 cm). Twenty-six of the thirty-three lesions were amenable to surgical treatment with curettage with or without bone-grafting, with only one recurrence. With the limited follow-up available, no lesion underwent sarcomatous degeneration. CONCLUSIONS: Epiphyseal enchondromas are rare lesions. Although their biologic behavior appears to mirror that of conventional metaphyseal enchondromas, their proximity to the joint space may lead to more frequent painful symptoms, a propensity for physeal involvement, and the need for earlier definitive surgical intervention.

Adolescent↗
PubMed1995

Survival and development of reimplanted fetal epiphyses. An experimental study.

An experiment was undertaken to study the survival and development of fetal epiphyses after excision and reimplantation in rats. The proximal part of fibula in the hindlimb was dissected free from surrounding tissues and then reimplanted again. Of 80 fetuses that were operated on, nine that had been operated on survived to birth. Histological examination on the hindlimbs of these rats at four and six weeks after birth showed that the reimplanted segments survived, and the bony nuclei of the epiphyses were present. Radiographically, at 6 weeks old, secondary centres of ossification at proximal ends of the fibulas could be seen in both operated and normal limbs. These results indicate the clinical possibility of correcting congenital musculoskeletal abnormalities in the future by in utero transplantation of epiphyses.

Animals↗
PubMed1983

Matrix synthesis in high density cultures of bovine epiphyseal plate chondrocytes.

Bovine epiphyseal plate chondrocytes were cultured by a method combining both suspension culture and high density monolayers. The matrix synthesized by the cultured cells was analyzed at fifteen days for glycosaminoglycan, proteoglycan, and collagen content. In the cell culture product glycosaminoglycan distribution was: 65% chondroitin-6-sulfate, 18% chondroitin-4-sulfate, 15% keratan sulfate, and less than 2% dermatan sulfate. Essentially all the radioactive sulfate in labelled specimens was present in high molecular weight aggregates. The collagen which was synthesized co-migrated with Type II collagen standard. Parallel analysis showed the matrix of cultured cells to be similar to that of intact epiphyseal plate tissue. This study demonstrates the ability to grow epiphyseal plate chondrocytes in a cell culture system which allows matrix synthesis similar to that seen in vivo.

Animals↗
PubMed1978

Bone growth alterations resulting from application of CO2 laser beam to the epiphyseal growth plates. An experimental study in rabbits.

The CO2 laser beam was used to create defects 3 mm in depth in the distal growth plate of one femur in a group of 59 rabbits. In most of the 32 rabbits in which the defect was created on the lateral aspect of the epiphyseal plate the procedure resulted in the subsequent development of valgus deformity and shortening of the femur. In most of the 27 rabbits in which defects were produced both medially and laterally to the epiphyseal plate there was a subsequent marked shortening of the bones. Histological studies revealed that the laser-induced defects of the growth cartilage led to epiphysiodesis and premature disappearance of the epiphyseal growth plates.

Animals↗
PubMedDate not supplied

Preossified longitudinal epiphyseal bracket of the foot: treatment by partial bracket excision before ossification.

The longitudinal epiphyseal bracket is a rare ossification defect of tubular bones in the hand and foot. This deformity contains an abnormal secondary ossification center, which may lead to progressive shortening and angular deformity of involved bones. This article describes the results from early treatment of this deformity before the secondary ossification center ossifies. Four patients with seven involved bones (5 metatarsals and 2 phalanges) were treated with surgical excision of the longitudinal epiphyseal brackets, without corrective osteotomy. The average age at surgery was 16 months (range, 6-20 months), and the average follow-up period was 55 months (range, 31-80 months). All five metatarsal patients demonstrated progressive improvement in the deformity. Of the two phalanx patients, one improved and the other did not. Early treatment of the longitudinal epiphyseal bracket before ossification of the secondary center with excision is effective in correcting this deformity. Long-term follow-up assessment until skeletal maturity is necessary to assess the final results of surgery because corrective osteotomy may be necessary for patients who do not have adequate correction.

Bone Diseases, Developmental↗
PubMed2004

[Fractures of the proximal tibial epiphyseal cartilage].

The authors present 9 children who sustained 10 fractures of proximal tibial epiphyseal cartilage (7--left tibia, 3--right tibial), and were treated in Children's University Hospital in Kraków between years 1994-2001. Mean age in the time of trauma was 12.7 years (6-17 years), the mean follow-up time was 17.4 months (8-48 months). According to Salter-Harris classification there was 5 fractures of type I, 4 of type II, 1 of type III. One child was treated by closed reduction and percutaneous Kirschner wires fixation. One child was treated by traction. The rest of the children were treated by casting, in knee flexion about 30 degrees. In one child there was premature closing of epiphyseal cartilage, shortening 1.5 cm and posterior curvature of tibia. In this child we performed flexion tibial osteotomy. In one child few months later we recognized meniscal tears (the patient was operated on). The results in the rest of children were good and there were not complications. The authors discuss the literature connecting to proximal tibial epiphyseal fractures.

Adolescent↗
PubMed1989

The vascular supply of the chondro-epiphyses of the elbow joint in young swine.

The vasculature of the elbow joint was examined in 39 pigs between one day and 15 weeks of age. Each pig was anaesthetised, exsanguinated and the thoracic limbs were perfused with India ink or a silicone rubber injection compound. The humerus, ulna and radius were dissected free, examined, fixed in formalin or ethyl alcohol, cleared by the modified Spalteholz technique and examined mesoscopically. Features of interest were photographed and then a limb from two pigs in each age group was cut into slabs and examined mesoscopically. The vascular supply of the distal part of the humerus was complex. It was supplied by vessels on both the cranial and caudal aspects and locally each aspect had a dual blood supply. Vessels anastomosed and on the cranial aspect formed a vascular ring. The proximal part of the ulna was supplied by vessels that were on its medial and lateral surfaces. The vessel on the lateral surface continued distally and supplied the lateral aspect of the proximal part of the radius. The proximal part of the radius was also supplied by arteries that were on the cranial and medial surfaces. Blood vessels provided branches to numerous cartilage canals of the articular-epiphyseal cartilage complexes, epiphyseal centres of ossification, and growth plates. The patterns of blood vessels in cartilage canals which were in sagittal or transverse planes were best exemplified by those in the distal part of the humerus. Perforating cartilage canals emerged from the epiphyseal centres of ossification. The pattern of cartilage canals was consistent in a general configuration, but individual variation did occur. Although cartilage canals were abundant in the youngest pigs, with increasing age the distribution of cartilage canals changed and the numbers of cartilage canals decreased.

Animals↗
PubMed1988

Traumatic separation of epiphyses. An experimental study in rats.

Four types of physeal fracture-separations, defined by the Salter-Harris classification, were created experimentally in the proximal physis of the right tibia of immature rats. The four types are: Type I, pure epiphyseal separation; Type II, separation of the epiphysis with a metaphyseal fracture; Type III, partial epiphyseal separation with a vertical fracture of the epiphysis; and Type IV, vertical epiphyseal and metaphyseal fractures. A sham operation performed on the left tibia served as the control. The animals were killed at various intervals up to 25 days after the operation. The findings were assessed by roentgenographic, histologic, and vessel injection methods. For Types I and II morphometric analyses were also performed. The influence of various types of lesions on the growth rate and the healing process was documented. For Types I and II lesions there was a transitory growth arrest and an increased thickening of the zone of hypertrophic cells. The alterations regressed after Day 15, and by day 25, a nearly normal plate was seen. For Type III lesions an angular deformity of the tibia occurred that increased with time. For Type IV lesions, a step-off developed on the articular surface that became more severe with time. Early vascular anastomoses between the epiphysis and the metaphysis led to the formation of bone bridges in Type III and Type IV lesions.

Animals↗
PubMed1988

[Periosteal bone resorption in the area of the metaphysis of growing bone as a precursor of epiphyseal injuries. A polarization optical and scanning electron microscopy study].

Macerated epiphyses of the growing infant bone exhibit an irregular surface which can already be observed at the macroscopic level. Polarizing microscopy demonstrates extended zones of bone resorption on the cortical surface, deductable from numerous lacunae of Howship. Ensuing experimentally induced epiphysiolyses, cortical bone fragments adhering to the inner surface of the periosteum are demonstrable by scanning electron microscopy. Having been torn out of the bone together with the periosteum, these cortical fragments leave corresponding defects on the bone surface. Due to the remodelling of the bone, involving the readjustment of the shape of the extremity, the attachment of the periosteum is relatively poor in the metaphyseal region of the growing bone. The influence of pathological forces can therefore easily cause a detaching of the periosteum in this region. The latter results in a significant weakening of the epiphyseal fastening in the zone between the epiphyseal plate and the metaphysis. The patterns of injury in the region of the growth plate are therefore essentially determined by the varying attachment of the periosteum to the metaphysis.

Adolescent↗