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Chondrosarcomas are heterogeneous matrix-producing cartilaginous neoplasms with variable clinical behavior. Subtypes include conventional (75%), dedifferentiated (10%), clear cell (2%), mesenchymal (2%), and periosteal chondrosarcoma (

The objective of the paper was to describe the experience of the authors concerning anexperimental study of the bone tissue and bone fragments, which were exposed to hightemperatures, in order to establish the species in the process of identifying the dead.Material and methods. In the process of performing this work, we used 350 tubular andflat bones of skeletons of people aged 20 to 65 years (long and short tubular bones, ribs) and 120 bones of domestic animals of the same name (cows, pigs, sheep, dogs).Conclusion. A comprehensive osteoscopic, microscopic, microosteometric and micro Xray studies of burned to gray glowing human bones and individual domestic animals ofvarying degrees of destruction (from large fragments to the actual ash) revealed anumber of features of the microscopic structure and mineralization of the bone tissue,which can be used in the process of species differentiation of the burned bone residues.

Distraction osteogenesis has become the most used method for bone lengthening and deformity correction including the bones of the hand and foot. The principle techniques used for hand bone lengthening in posttraumatic and congenital conditions using available external fixators are based on this method. We present the technical specifications and surgical techniques with the use of a mini-fixator that was specially designed for short tubular bones. The surgical techniques of lengthening and web space skin stock creation for future web-pasty are supplied with the diagrams of their application to phalanges and illustrative clinical cases. Between 1999 and 2012, this mini-fixator was used for lengthening of 223 finger segments at our hospital and provided improvement of the hand esthetics and functions.

Background/Purpose The purpose of this study was to evaluate the results of reconstruction of postosteomyelitic defects in short tubular bones in a paediatric population using a universal mini external fixator (UMEX). Methods This was a prospective case series based on 10 consecutive cases who were followed-up for an average period of 3.5 years. Ten patients (mean age: 10.2 years) with an average shortening of 4 cm of the corresponding digit were operated upon using soft tissue distraction by UMEX followed by interposition bone grafting from the iliac crest. The second metacarpal was involved in five patients, the first metacarpal in three patients, and the proximal phalanx of the right big toe was involved in two patients. Results All of the patients achieved union at the graft host site at an average of 12 weeks and an average of 3.8 cm lengthening of the digit was achieved. All patients had improved digit length, and function and appearance of the hand and toe. Apart from a minor pin tract infection and stiffness, there were no major complications. Final average follow-up at 3.5 years did not show any recurrence of infection or loss of correction. Conclusion We believe that UMEX is a versatile method of managing postosteomyelitic defects in short tubular bones, with fewer complications and a high union rate, even in situations in which bone fragments are small and difficult to handle with other methods.

Tuberculous dactylitis is an unusual form of osteoarticular tuberculosis involving the short tubular bones of hands and feet, which is uncommon beyond six years of age. We report the case of a fifteen-year-old adolescent boy who was diagnosed with tuberculous dactylitis, involving contralateral hand and foot. His diagnosis was delayed due to lack of suspicion of this rare entity. The report also examines the diagnostic difficulties faced by clinicians in arriving at an appropriate diagnosis.

Tuberculosis of the metacarpals, metatarsals and phalanges is uncommon after the age of 5 years. It occurs in young children in endemic areas under 5 years of age. Tuberculosis of the short tubular bones like phalanges, metacarpals or metatarsals is quite uncommon beyond 6 years of age, once the epiphyseal centers are well established.

Aneurysmal bone cyst is a rapidly growing and locally aggressive lesion that commonly affects children and young adults. Initially regarded as a reactive process, primary aneurysmal bone cyst is now widely accepted as a neoplasm owing to recent findings of recurrent clonal chromosomal alterations, mostly t(16;17)(q22;p13). However, other infrequent chromosomal rearrangements have also been reported. Giant cell reparative granuloma, previously regarded as a nonneoplastic process and histologically indistinguishable from the solid variant of aneurysmal bone cyst, is frequently seen in the gnathic bones and the short tubular bones of the hands and feet. Here we present such a case of giant cell reparative granuloma (solid aneurysmal bone cyst) in the finger of a 63-year-old white man. Cytogenetic analysis revealed a novel alteration involving a reciprocal translocation between 6q and 13q, with a karyotype of 46,XY,t(6;13)(q15;q34),del(20)(q13.1).

Background: Thanatophoric dysplasia (TD) is the most lethal and most severe type of dysplasia. It has distinct features, the most important of which is short tubular bones and short ribs with platyspondyly, allowing a precise radiologic and prenatal ultrasonographic diagnosis. It has been reported to be caused by mutations in the FGFR3 gene, but exactly how cytogenetic abnormalities might lead to TD is unclear. Case Report: We report a case of TD with different prenatal sonographic features compatible with the classification of type I. In the result of cytogenetic examination, we found de novo CAs in 28% of cells analyzed from the affected infant; 75% of the abnormalities were numerical, and of those, 25% were structural aberrations; 21% of cells revealed predominantly numerical aberrations. Monosomy 18, 21 and 22 was observed in 4% of cells, monosomy 20 in 2%, and monosomy 7, 8, 14, 17 and 19 in 1%. Structural changes were observed in 7% of cells. Conclusions: It appears that these chromosomes may be preferentially involved in and important for TD development. © Am J Case Rep.

Hypertrophic osteoarthropathy (HOA) is characterized by symmetrical periosteal new bone deposition on the long and short tubular bones. Besides its unknown etiology, it has been associated with pulmonary, cardiovascular and gastrointestinal disorders. The aims of this research were to assess the presence of HOA and to analyze its distribution by sex, age at death, bones affected, type of new bone formed, and cause of death. A sample of 329 individuals (non-adults and adults, of both sexes) was selected from the Coimbra Skeletal Identified Collection. The sample comprised 125 individuals diagnosed with tuberculosis, 64 with pulmonary non-tuberculosis, and 140 with an extrapulmonary and non-tuberculous cause of death. Lesions compatible with HOA were found in all three groups, being statistically significantly in the individuals who died from pulmonary diseases (χ2=5.29, df=1, p=0.02). The risk of developing HOA was 3.41 (χ2=7.77, df=1, p=0.005) higher in the individuals with tuberculosis when compared with the other groups. This work, based on individuals who died before the introduction of antibiotics for treatment, agrees with clinical studies that have established a possible correlation between pulmonary conditions and HOA. These data may help in differentially diagnosing the many instances of periosteal new bone formation found in archaeologically derived skeletal samples.

We report on a 9-year-old girl who presented with progressive restriction of spine mobility. Standard spinal radiographs revealed enchondromatous lesions spread out along the anterior end plates of the lumbar vertebrae and MR imaging showed progressive anterior vertebral fusion. The long bones, short tubular bones and flat bones were free of enchondromata, but arcs of chondroid calcifications were observed in the proximal femoral metaphyses. The clinical and the radiographic features encountered in our patient did not fit into any previously reported children with enchondromatosis.

Camurati–Engelmann disease (CED) is a rare diaphyseal dysplasia characterized by progressive cortical sclerosis, both internal and external, of diaphysis of long and short tubular bones and sclerosis of base of skull. We report a case of progressive diaphyseal dysplasia with metaphyseal involvement in a 12-year-old female child, who presented with history of bone pain, deformity of lower limbs, and weight loss, showed significant clinical improvement following steroid therapy and briefly review the literature.

Tuberculous infection of metacarpals, metatarsals and phalanges is known as tuberculous dactylitis. There is a spindle shaped expansion of the short tubular bones due to tuberculous granuloma. Hence it is also known as spina ventosa. In our case, a two year old boy with a swelling in the metacarpal was provisionally diagnosed as enchondromata while the possibility of spina ventosa was kept in mind. He was posted for excision of the metacarpal followed by bone grafting. Histopathological examination report confirmed it as spina ventosa.

Distinguishing enchondroma from low-grade central chondrosarcoma depends on interpreting histologic findings in the light of clinical and radiologic features. In the long bones, enchondromas do not grow, whereas low-grade chondrosarcomas grow slowly. The distinction should be made based on answers to the question, “Is the lesion growing?” Serial radiographs are the best way to answer this question. Other clues are present in the clinical, radiographic, and histologic findings. In the short tubular bones of the hands and feet and in Ollier’s disease, enchondromas do grow. Therefore, the threshold for regarding central cartilage lesions in these settings should be higher. Extreme cortical expansion and/or infiltration of soft tissues should be present to diagnose chondrosarcoma.

A periosteal osteosarcoma arose in the first phalanx of the fourth finger of a 32-year-old woman's right hand. She had no predisposing factors for this cancer. The tumor was initially locally resected, but as recurrences developed, the finger and later the hand were amputated. Despite therapy with doxorubicin and cisplatin, distant metastases occurred and ultimately caused the patient's death. Primary conventional osteosarcomas of the short tubular bones of the hands and feet are rare, but surface lesions are rarer. This case constitutes the first reported periosteal osteosarcoma to arise in a phalanx.

Introduction: Osteosarcoma (Os) is the most common malignant mesenchymal tumor of the bone, and mostly affects the long bones around the knee. Osteosarcoma of short tubular bones such as those of the foot is extremely rare and accounts for less than 1% of all cases. to the best of our knowledge, this is the first reported case of Os of the middle and distal phalanges of the little toe. case report: A 20-year-old Japanese male presented at our hospital due to pain and swelling of his left little toe for over one year. Physical examination revealed a swollen little toe on his

We describe a series of seven male patients from six different families with skeletal dysplasia, characteristic facial features, and developmental delay. Skeletal findings include patellar dislocation, short tubular bones, mild metaphyseal changes, brachymetacarpalia with stub thumbs, short femoral necks, shallow acetabular roofs, and platyspondyly. Facial features include: a flattened midface with broad nasal bridge, cleft palate or bifid uvula and synophrys. All of the patients demonstrated pre-school onset of a cognitive developmental delay with a shortened attention span. Some of the cognitive delay was masked by a warm and engaging personality. We posit that these individuals have a newly recognized syndrome characterized by the described features. There is some phenotypic overlap between these patients and Desbuquois dysplasia; however molecular testing demonstrated that this is a distinct disorder. Given the family information available for each patient, we are suspicious that the constellation of findings reported herein could be an X-linked recessive syndrome.

We analyzed the total hand length (HL) and length of noncarpal bones (NCL) in 50 Japanese patients with Ullrich-Turner syndrome (UTS) and in 443 other patients with short stature used as controls. In each patient group we calculated relative HL (RHL= HL/height) and relative NCL (RNCL= NCL/height). UTS patients had significantly greater RHL than controls. The greater RHL in UTS patients is mainly due to their longer, short tubular bones. The RHL is not affected by ages and karyotypes of UTS patients or growth hormone treatments given to them. We conclude that relatively longer hands are a common manifestation of UTS and that this parameter is useful for the diagnosis of the syndrome among short females, who usually need chromosome analysis. Am. J. Med. Genet. 82:254–256, 1999. © 1999 Wiley-Liss, Inc.

Although osteosarcoma is the most common primary bone malignancy of childhood and adolescence that is not related to marrow cells, involvement of the short tubular bones is uncommon. In contrast to more conventional sites, where the tumor is usually high grade and found in adolescents, osteosarcoma of the small bones is more likely to be low grade, and is often seen in older individuals. We present a case of low-grade primary osteosarcoma of a metatarsal bone in a 25-year-old woman.

Primary osteosarcomas of the short tubular bones of hands and feet are rare. We describe a 46-year-old woman with osteosarcoma of the left metatarsal bone with pulmonary metastases. This is the first reported case of osteosarcoma at this site.

PubMed ID: 10870664, Spondyloepiphyseal dysplasia tarda with progressive arthropathy, described by Wynne-Davies et al., is a rare autosomal recessive disorder. It is characterized by generalized platyspondyly and epiphyseal involvement, with enlargement of both ends of the short tubular bones of the hands. Clinical features include onset in childhood, a disproportionately short stature and premature osteoarthritis. We describe the clinical and radiographic findings of a young woman suffering from spondyloepiphyseal dysplasia tarda with progressive arthropathy.

The case of a 14-year-old girl with rhabdomyosarcoma of the right foot is reported. Plain radiography showed a large nonspecific soft tissue tumor between the metatarsals with bowing of the metatarsals away from the mass. MR imaging showed a large soft tissue mass involving the metatarsals. The findings were conflicting, because the tumor had an infiltrative soft tissue mass and bowing of the metatarsals more suggestive of slow expansive growth. Bowing of short tubular bones may be a process similar to cortical saucerization, which is typically seen in Ewing’s sarcoma, and it can be one of the findings of high grade neoplasms, such as embryonal rhabdomyosarcoma.

Variations of the epiphyseal ossification of short tubular bones were repeatedly mistaken for fractures. The case of an 11–year-old male patient after a minor trauma is presented. An extra ossification center at the epiphysis of the distal phalanx of the first toe was diagnosed as a fracture and treated appropriately. The recovery was very fast, about 4 days. 3 years later the extra ossification center was still visible and a parted epiphysis of the proximal phalanx appeared again like a fracture, but with no clinical symptoms in that region. This time after a minor trauma, there was no treatment and the patient was free of symptoms after 1 week. The variation range of the ossification centers of the first toe is wide. In minor trauma X–ray findings should correlate exactly with the symptoms not to overdiagnose toe fractures.

We report the case of a Japanese boy whose dysmorphic features were consistent with those of Shprintzen-Goldberg syndrome. The radiological features were characterized by late-onset craniosynostosis, arachnodactyly, undermodeling of short tubular bones, mildly undermodeled and slightly bowed long bones, twisted ribs and tall vertebral bodies with elongated neural arches. Apart from the craniosynostosis, these skeletal changes resembled those of frontometaphyseal dysplasia, a well-known craniotubular dysplasia. Shprintzen-Goldberg syndrome also shares many clinical features with frontometaphyseal dysplasia.

Five members of a Japanese family with a new form of spondylo-metaphyseal dysplasia (SMD) are reported. Another member was also probably affected. The disease was characterised by severe coxa vara, moderately severe metaphyseal changes of the long bones of the lower limbs, mild changes in the long bones of the upper limbs and grossly normal short tubular bones. Platyspondyly, present in the boys, was less marked in their father, whereas two affected aunts had normally shaped vertebral bodies.

Garcia-Cruz D, Figuera LE, Feria-Velazco A, Sanchez-Corona J, Garcia-Cruz MO, Ramirez-Duenas RM, Hernandez-Cordova A, Ruiz MX, Bitar-Alatorre WE, Ramirez-Duenas ML, Cantu JM. The Myhre syndrome: report of two cases. Clin Genet 1993: 44: 203–207. © Munksgaard, 1993 Two unrelated patients, aged 19 and 6 years, were studied and diagnosed as having Myhre syndrome (MS). This review, together with three previous cases, permits further delineation of MS. The main features are: short stature, mental retardation, blepharophimosis, muscular hypertrophy, decreased joint mobility, thick calvarium, broad ribs, hypoplastic iliac wings and short tubular bones. Advanced paternal age at the propositi's birth suggests an autosomal dominant mutation as the cause of MS.

Achondroplasia is a physeal dysplasia which leads to dwarfism secondary to a decrease in the proliferation of cartilage in the growth plate. This, coupled with normal persistent bone formation, leads to the development of short tubular bones. Achondroplastic dwarfism is among the more common types of dwarfism and is inherited as an autosomal dominant trait. Its incidence is reported as 1 in 26,000 live births. Most achondroplastic dwarfs have a normal life span. The selection and management of anesthesia for the achondroplastic dwarf must take into account a variety of anatomic deformities. The physiologic and hormonal changes of pregnancy further complicate anesthetic administration. We report the safe use of a continuous lumbar epidural anesthetic in an achondroplastic dwarf who presented for urgent cesarean section.

In the short tubular bones of the feet and hands, a destructive lesion may occur that gives the impression of a malignant tumor growth. Here we are dealing with a benign, non-tumorous and reactive granulation tissue, including many osteoclastic giant cells, that destroys the bone; the lesion is caused by a traumatic bone damage. This lesion is, radiologically and histologically, very similar to an aneurysmal bone cyst.

Using the femur and its proximal growth plate as an example, we will try to expose as dynamically as possible the changes which take place during bone growth. Basic to this realization is the fact that the entire bone is preformed as a cartilaginous anlage. The first evidence of bone formation is seen at the midpart of the diaphysis where mesenchymal cells lay down a periosteal sleeve of bone (Fig. 2.1). This process is followed by a cellular and vascular invasion of the cartilage model at this level where the chondrocytes have reached the stage of degeneration (Streeter phase 5, Fig. 2.2). This invasion denotes the beginning of enchondral bone formation; in long tubular bones it will move in opposite directions (Fig. 2.3) to reach eventually the metaphyseoepiphyseal region at both ends of the bone where, through a spatial rearrangement of the chondrocytes, the physis or growth plate will be formed. In many short tubular bones only one physis is formed; at the end opposite to the physis, enchondral ossification will progress into the epiphyseal area where, through spatial rearrangement of chondrocytes, a growth plate will be formed under the articular cartilage. It is often referred to as a mini growth plate. The mechanisms of modeling which permit the bone to retain its original shape during growth in length and in width need reemphasizing.

Spondylo-epi-metaphyseal dysplasia is a rare skeletal dysplasia showing radiographic abnormalities in the epiphyses and metaphyses of the long and short tubular bones, and in the spine. There are very few reports of a case followed throughout the entire growth period. Here we report the case of a Japanese girl followed from 1 month old to skeletal maturity at 16 years old. She developed progressive kyphoscoliosis at 7 years old, and underwent a surgical correction of the spine at 14 years old. The diagnosis of spondylo-epi-metaphyseal dysplasia was made from examining all the follow-up radiographs showing abnormalities in the spine, long bones and hands involving epiphyses and metaphyses. She had normal stature--a rare finding in this condition.

Eight cases of giant-cell reparative granuloma were encountered in the short tubular bones of the hands and feet. This entity was originally described by Jaffe as a jaw lesion. Subsequently the lesion was found in the skull and facial bones. The name "giant cell reaction" was applied to similar lesions found in the short tubular bones of hands and feet. Thirteen prior cases have been reported and none recurred. Of the eight new cases described herein, four have recurred one or more times, requiring further treatment. No association with hyperparathyroidism was found. We prefer to designate these lesions as giant cell reparative granulomas of extragnathic sites. Because of overlapping ultrastructural and light-microscopic features, as well as similar biologic behavior, we feel that aneurysmal bone cyst and these giant-cell reparative lesions represent related responses to intraosseous hemorrhage.

Giant-cell reparative granulomas are observed not infrequently in the mandible as sharply demarcated osteolytic lesions. On the other hand, these tumour-like lesions occurring in short tubular bones are rarely reported in the literature and, so far, only 27 cases have been described. We have records of five cases in the Westphalian Bone Tumour Register. The actual occurrence is probably greater if one assumes that the lesion is caused by an intra-osseous bleeding. This pathogenic mechanism is supported by histological evidence.

Giant cell reactions in the short tubular bones comprise mainly enchondromas, aneurysmal bone cysts and giant cell tumours. In both cases a history of trauma can be related. We therefore agree with Aegerter and Lorenzo that an intra-osseous haematoma due to minor trauma is likely to be of pathogenic significance.

Skeletal changes seen in 12 patients with mannosidosis included thickened calvaria, ovoid configuration, flattening and hook-shaped deformity of the vertebral bodies, hypoplasia of the inferior portions of the ilia, and mild expansion of the short tubular bones of the hands. The pattern of skeletal changes is that of mild to moderate dysostosis multiplex with considerable intrafamilial variation. The skeletal abnormalities may decrease with age. Correlation of the skeletal abnormalities with clinical and biochemical findings is necessary for a specific diagnosis.

The clinical and roentgenographic manifestations of six cases suffering from spondyloepiphyseal dysplasia tarda with progressive arthropathy are presented. The roentgenographic features consist of generalized platyspondyly and varying degrees of epiphyseal involvement with conspicuous enlargement of both ends of the short tubular bones of the hands. This disorder appears at an earlier age, and is more crippling than the usual form of spondyloepiphyseal dysplasia tarda. It is suggested that orthopaedic measures are essential for all patients with this disorder, as profound disability may be expected by progressive involvement of the major joints.

Since the presentation of Osteoid Osteoma as an entity by Jaffe (1935) and the report on 62 cases by the same author (Jaffe, 1945), it has been found in almost all bones. However, it seems according to the review of the cases reported in the literature that the location of the lesion in flat bones is far less common than in the long and short tubular bones or in the short spongy bones. The first case in a flat bone (innominate) was reported by Lewis (1944) and another case was seen by Jaffe (1945). The number of cases reported since then in flat bones is relatively small in comparison with that found in tubular or short spongy bones. Sherman (1947) reviewed 158 cases reported in the literature including 30 of her own. Out of these, three were located in the ilium, none in the scapula. Golding (1954) reviewed 198 cases reported in the literature, out of these 20 of his own. Only four were situated in the pelvic bones, one in the scapula. Flaherty, Pugh and Dockerty (1956) reported 52 cases, estimating the ...

The manifestations of generalised enchondromatosis are multiple enchondromas in the metaphyses of long and short tubular bones, and in flat bones. The skull and vertebrae are relatively spared. The changes are usually bilateral but asymmetrical. The association of enchondromatosis with haemangiomas in Maffucci's syndrome is well known, but unusual manifestations of enchondromatosis are increasingly described. Spranger et al (1978) described two types, one with multiple enchondromas of the long, tubular bones and flat bones, with generalised irregular dysplasia of the vertebral bodies, but only minimal involvement of the phalanges; the second type consisting of generalised evenly distributed enchondromas with severe involvement of the hands and feet, mild platyspondyly and skull deformity. Van Creveld et al (1971) described metaphyseal chondrodysplasia calcificans, which is now accepted as a variant of generalised enchondromatosis. Kaibara et al (1982) described generalised enchondromatosis with haemangiom...

Two cases of Ollier’s disease, one with extensive bilateral involvement of the metaphysis of most of the long bones of the extremities, and the other with monomelic distribution affecting most of the short tubular bones of the left hand, are described.

Microradiographic examination of metaphyses in long and short tubular growing bones allowed detection of a repetitive, clearly defined pattern of three adjacent zones; the latter are successively formed by the mineralization of cartilaginous longitudinal intercolumnar septa and by the subsequent apposition of other mineralized tissues concurrently with resorption. Consequently, each zone of the metaphysis includes mineralized tissues of various compositions and ages, identifiable by their different mineralization densities. Microradiography of pieces of the growing skeleton in several animal species shows that the same organization is not only present in long and short tubular bones but also in many other such as the pelvis and scapula, cuboid bones like the calcaneum and talus, and cartilaginous bones at the base of the skull. This suggests that there is no difference between the osteogenesis pattern of these bones and tubular ones. The problem of identifying the factors generating such metaphyseal organization is raised.

A review of the roentgenograms of 126 children with sickle-cell disease seen over a five-year period (1956–61) at Kings County Hospital Center (Brooklyn, N. Y.) disclosed several unusual skeletal changes which are the subject of this report. The changes to be emphasized here have been included in the excellent discussions of sickle-cell disease by Middlemiss (1) and by Moseley (2). In general, three types of bony abnormalities occur. Marrow hyperplasia, involving the long and short tubular bones and the axial skeleton, frequently results in the roentgen appearance of widened medullary cavities and intertrabecular spaces, with cortical thinning. A second type of osseous lesion results from infarction. The roentgen appearance due to this cause varies according to (a) the site and extent of infarction, (b) the degree of surrounding hyperemia, (c) the state of osteoclastic resorption and osteoblastic repair, and (d) the effect of weight-bearing on compromised bone. A third complication is osteomyelitis, parti...

The principal bone abnormalities in glycogen storage disease (GSD), Type 1, are delayed skeletal maturation, osteoporosis, and gouty changes. Other occasional changes were concavity of the anterior border of the upper lumbar vertebrae, reversal of lumbar lordosis, and notching of the upper medial humeri. The distal ends of the metacarpals may be widened in childhood or adulthood and a generalized broadening of the short tubular bones ofthe hands may be seen in infancy.

Benign tumors of the short tubular bones of the hands and feet are relatively frequent. Malignant tumors occur rarely, and adequate information is not available to permit a full discussion of the nature of such lesions, or to draw conclusions concerning their clinical course and prognosis. We have observed a tumor of the fourth metacarpal bone that seems to be of interest clinically, roentgenologically and pathologically. REPORT OF A CASE The patient (C. Z.) was a girl, 15 years of age when she first came under our care. She had noticed a swelling in the region of the fourth metacarpal bone of the left hand in March, 1927, when she was 12 years of age. At first the enlargement was not great, but pain on pressure was present. Roentgen examination at that time showed expansion of the diaphysis of the fourth metacarpal bone by a tumor extending from the base