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Congenital deficiency of the fibula with ipsilateral iliac horn and absence of the kidney.

Congenital deficiency of the fibula is sometimes accompanied by femoral hypoplasia, genu valgum, patellar a/hypoplasia or dislocation, tibial bowing, foot deformity, and toe deficiency in the affected limb. 'Iliac horns' are bony projections extending posterolaterally from the ilium and considered to be pathognomonic of nail-patella syndrome. We report a 5-year-old Japanese girl with congenital complete deficiency of the left fibula, ipsilateral iliac horn and absence of the left kidney.

Female↗

Minimally invasive iliac cancellous bone graft harvesting.

We have described a technique for harvesting cancellous bone from the ilium that is minimally invasive and safe. This technique results in minimal donor-site morbidity, is quick and easy to perform without the need for special equipment or instruments, and allows a two-team operative approach. Sufficient bone graft material can be obtained for wide or bilateral clefts. We now use this technique routinely to harvest cancellous bone for secondary alveolar bone grafting in children with clefts. The procedure could be used in adults, it could also be used to obtain bone graft for treating other conditions, and other donor sites could be approached with the same technique.

Bone Transplantation↗

Osteosarcoma of the pelvis. Oncologic results of 40 patients.

The cases of 40 patients with osteosarcoma of the pelvis treated between 1977 and 1994 were reviewed. The location of the tumor was ilium in 30 patients, ischium in four, pubis in one, and sacrum in five. Most (58%) of the tumors were of the chondroblastic subtype. Thirty patients had surgical excision of the tumors: 10 with hemipelvectomies and 20 with limb sparing procedures. A wide margin was achieved in 16 of 30 (53%) patients, including 12 of 14 who had no sacral tumor involvement. Positive margins occurred at the sacrum in 11 patients, lumbar vertebra in one, perirectal space in one, and contralateral pubic body in one. Macroscopic tumor emboli within the regional large vessels were found in seven patients. The incidence of local recurrence was 32%: 13% in wide excisions, 38% in marginal excisions, and 80% in intralesional excisions. The 1- and 5-year overall patient survivals were 73% and 34%, respectively. Patients who had a surgical excision of the primary tumor had a significantly better survival than did those treated without surgery (5-year survival; 41% and 10%, respectively). Tumor size, surgical excision of the primary tumor, surgical margin, and type of surgical procedure were the prognostic factors for patients with Stage IIB tumors.

Adolescent↗

Hindquarter amputation for pelvic tumors. The importance of patient selection.

Twenty-two patients underwent hindquarter amputation with curative intent for malignant or locally aggressive tumors of the pelvis (21 cases) or proximal thigh (one case). In all cases, the ilium was resected, and in seven cases an osteotomy of the sacrum was done to achieve negative surgical margins. Only six wounds healed without complications. The worst wound problems occurred in patients requiring ligation of the common iliac vessels and in those requiring a sacral osteotomy. Twelve patients have died of disease, two are alive with extensive pulmonary metastases, and one remains well after excision of a local tumor recurrence. Thirteen of 14 patients with high grade sarcoma had metastases develop, compared with only one of eight patients with low grade sarcoma. Eight patients (all with high grade sarcoma) died within 1 year of surgery. Seven patients have remained disease free at a mean of 39 months after surgery. Given the poor prognosis and high morbidity after amputation in high grade sarcoma, the authors think that consideration should be given to pursuing nonoperative management in patients with large, high grade sarcomas and significant medical extension to the sacroiliac joint or sacrum.

Adolescent↗

[An unusual case of chondrosarcoma in a 5-year old boy].

A case of chondrosarcoma in 5 years old boy is presented. This type of neoplasm is extremely rare in this age group usually affecting patients in their forties and fifties. The tumor was located in the acetabular part of the ilium, ischial bone and the capsule what, again, is very rare, especially in children. Radiographs were atypical; there were no cloudy calcifications characteristic for malignant chondral tumors. The boy spent 2.5 years in several hospitals with various diagnoses being treated accordingly. The surgery performed was not radical: focal excision of the tumor was done and the capsule was entirely removed. No signs of avascular necrosis and no signs of neoplastic process recurrence were observed during 3 years follow-up.

Bone Neoplasms↗

Chondromyxoid fibroma of bone presenting as chronic back pain.

OBJECTIVE: To discuss a case of chondromyxoid fibroma presenting with low back pain. CLINICAL FEATURES: A 50-yr-old man had an 8-yr history of low back pain. This was diagnosed and treated as arising from the disc and caused by spinal stenosis. Magnetic resonance imaging findings supported the clinical findings. Years later, a plain radiograph of the pelvis revealed an incidental abnormality of the right ilium, and the patient was subsequently referred to a musculoskeletal tumor center for treatment. INTERVENTION AND OUTCOME: The lesion was surgically removed and the defect was reconstructed via bone allograft. CONCLUSION: Such tumors are a rare cause of back pain. Tumors of the pelvis can at times present as back pain. In cases of refractory back pain, an X-ray of the pelvis can be a useful screening investigation. Chondromyxoid fibromas are rare tumors best treated by excision if they are amenable or by curettage and bone grafting procedures.

Back Pain↗

Oromandibular reconstruction using microvascular composite flaps: report of 210 cases.

OBJECTIVE: To review the experience of 1 microvascular surgeon during an 11-year period in performing 210 vascularized bone-containing free flaps for oromandibular reconstruction. DESIGN: Retrospective medical records review of patients who underwent primary and secondary oromandibular reconstruction with the use of vascularized bone free flaps. SETTING: Academic medical center. PATIENTS: A total of 201 patients underwent 210 composite free-flap reconstructions of the mandible for various disorders and with a range of bony and soft tissue defects. INTERVENTION: All patients underwent the microvascular transfer of vascularized bone flaps from the ilium, fibula, or scapula. In selected cases, 2 simultaneous free flaps were transferred to achieve an optimal bone and soft tissue reconstruction. Endosteal dental implants were used in 81 patients, with a total of 360 fixtures placed during these 11 years. MAIN OUTCOME MEASURES: The success of microvascular free tissue transfer, dental implant extrusion, and short- and long-term complications at the recipient and donor sites. RESULTS: Of the 210 mandibular reconstructions that were performed, 202 were successful in reestablishing mandibular continuity. Reexploration for vascular-related complications was done in 16 patients, 8 of whom were successfully treated, yielding an overall success rate of 96%. The overall success rate for endosteal dental implants was 92%. The implant success rate was 86% when the bone in which the fixtures were placed was irradiated postoperatively. The success rate was 64% in the 14 fixtures that were placed into previously irradiated bone. CONCLUSIONS: The success of the use of vascularized bone free flaps in restoring continuity to the mandible is clearly demonstrated in this series. There was an acceptable incidence of donor- and recipient-site complications that resulted in minimal long-term morbidity. The careful selection of a donor site(s) for oromandibular reconstruction allows for an optimal restoration of bony and soft tissue defects. Dental implants can be safely used in oromandibular reconstruction with a high level of success. Placing these implants during the initial surgery shortens the duration for achieving dental rehabilitation and enhances the success of the implants when postoperative radiotherapy is administered.

Adolescent↗

Sex differences in the sciatic notch of great apes and modern humans.

The sciatic notch has been widely used as a sexing criterion in modern humans. In order to better understand the sex differences of this feature in modern humans and great apes, four measurements of the sciatic notch were taken on samples of modern humans and great apes of known sex. Univariate (ANOVA) analysis and discriminant function analysis were performed on the extant taxa to determine: (1) the discriminating power of each variable in these samples of known group membership; and (2) which of these extant taxa shows the best discrimination between the sexes for the sciatic notch. Of the four extant taxa, the sciatic notch of Homo sapiens is the most sexually dimorphic, followed by Gorilla gorilla, and more weakly by Pongo pygmaeus, while Pan troglodytes is the least dimorphic of these taxa. Since the presence of a well defined sciatic notch is a hominid trait resulting from the dorsal extension of the posterior ilium, the close approximation of the sacrum to the acetabulum, the shortened ischium, and the accentuation of the ischial spine as part of the bipedal adaptation, it seems likely that the configuration of the sciatic notch in hominids was initially related to bipedalism, not reproduction. The development of sex differences in the sciatic notch of modern humans is more likely to have occurred after the transition to bipedality.

Analysis of Variance↗

Measuring the human pelvis: a comparison of direct and radiographic techniques using a modern United States--based sample.

Seven measurements were taken on a sample of 50 human cadaveric pelves, all white Texans born in the 20th century. Two separate methodologies were used to obtain these data: radiographs and direct measurements. These two methodologies were compared and contrasted, with the relative advantages and disadvantages of each explored. Results indicate that significant differences exist between the two methodologies. Pelvic height, breadth of symphysis, sacro-iliac breadth (P = 0.0001) and anterior upper spinal breadth (P = 0.0002) were larger when measured directly. Pelvic breadth, transverse diameter of the pelvic brim, and height of the ilium did not significantly differ between methodologies (P = 0.2037, P = 0.5253, P = 0.1752). Due to secular changes and inherent intrapopulational variation, taking measurements either directly from modern cadaveric specimens or radiographically on living volunteers in a limited geographic or socioeconomic grouping, rather than from skeletal collections or archived radiographs, may be more appropriate for providing data for current anthropometric applications.

Aged↗

Sonographic findings in aneurysmal bone cyst in children: correlation with computed tomography findings.

PURPOSE: This study compares the value of sonography and computed tomography (CT) in assessing fluid-fluid levels (FFLs) in aneurysmal bone cysts (ABCs) in children. METHODS: Five children 4-13 years old with ABC in pubis, humerus, fibula, calcaneus, and ilium-ischium were studied between January 1995 and December 1996. Plain radiography, sonography, and CT were performed. RESULTS: FFLs were visualized equally or better with sonography than with CT. CONCLUSIONS: We propose that sonography be used to complement plain radiography in the prebiopsy assessment of expansile bone lesions with cortical bone thinning because the presence of FFLs is characteristic of ABC. CT and/or magnetic resonance imaging could be used in indeterminate cases and in complex anatomic locations. Such an approach would save time and reduce costs and discomfort to children.

Adolescent↗

Hodgkin's disease presenting as a solitary bone tumor. A report of four cases and review of the literature.

BACKGROUND: Hodgkin's disease (HD) rarely presents as a solitary bone tumor. Fewer than 20 such cases have been reported in the English literature; many of these were reported prior to the development of immunohistologic markers for HD and T- and B-cell lymphomas. In this report, we describe four cases of HD that presented as a localized solitary mass in bone; the diagnosis was confirmed by immunohistochemical studies in all cases. METHODS: The biopsy specimens of four cases identified in our files were studied by conventional histopathology and immunohistochemistry. Clinical data and follow-up information were obtained for all patients. RESULTS: Three cases presented as a localized, solitary mass in the ilium and one case in the vertebra (T12). Three patients were female and one male. The average age was 43 years. Three of the patients presented with lower back pain without constitutional symptoms. All had solitary osteoblastic lesions. All four cases were diagnostic problems, and the diagnosis was confirmed in each case only after finding lymph node involvement. Bone biopsies showed fibrosis and a mixed inflammatory infiltrate with rare atypical cells. All four patients were subsequently found to have nodal involvement by HD. The histology of the associated nodal disease was mixed cellularity in two cases and nodular sclerosis in two. On immunohistochemical staining, the neoplastic cells in all cases expressed CD15 and CD30 and lacked CD45 and other B- and T-cell antigens. Three patients who were treated for HD are alive and well, 1, 6, and 14 years later. CONCLUSIONS: Although rare, HD should be considered in the differential diagnosis of solitary bone lesions. Most patients who present with apparently solitary HD of bone prove to have nodal involvement. Long-term survival is possible with aggressive treatment.

Adult↗

Fine-needle aspiration biopsy in the diagnosis and management of bone lesions: a study of 450 cases.

BACKGROUND: Fine-needle aspiration (FNA) biopsy has proved to be a cost-effective technique, with low complication risks and high diagnostic value in distinguishing neoplastic versus nonneoplastic lesions in many organs. This study was designed to determine the reliability, areas of diagnostic difficulty, and limitations of FNA in the diagnosis of bone lesions encountered in a university-affiliated tertiary care hospital. METHODS: The cytology of 450 FNA biopsies of bone lesions, performed on 427 patients between 1979 and 1996, were reviewed. The results were correlated with the corresponding histology when available, and with clinical follow-up, in an attempt to define the role of FNA in managing patients with bony lesions. RESULTS: The patients ranged in age from 5 to 94 years, with a male-to-female ratio of 1.25:1. The spine was the most frequently aspirated site (49%), followed by the ilium, sacrum, mandible, ribs, and femur. Three hundred and eighty-five aspirates (86%) were adequate for evaluation, with 215 cases diagnosed cytologically as positive for malignancy, 11 cases as suspicious but not diagnostic of malignancy, and 2 cases as inconclusive. One hundred and fifty-seven cases were interpreted as showing no evidence of malignancy. Metastatic carcinoma was present in 175 of the 215 malignant aspirates, and 67% of these were adenocarcinomas. Forty cases were primary malignant bone neoplasms, including myeloma, lymphoma, Ewing's sarcoma, chondrosarcoma, ameloblastoma, chordoma, neurofibrosarcoma, and unclassified high grade sarcoma. False-negative diagnoses were rendered in ten cases; however, on review, material representative of the bone lesion was not present in six cases. Five cases were correctly diagnosed as malignant but were misclassified with regard to the type of malignancy. CONCLUSIONS: FNA biopsy of bone lesions is a reliable and easily performed diagnostic test for metastatic and primary bone tumors. False-positive results have major therapeutic implications, hence the significance of the authors' conservative diagnostic approach, which resulted in a false-positive rate of 0.2%. Areas of difficulty were due to inadequate sampling or misclassification with regard to the exact type of malignancy. The simplicity and accuracy of this procedure, which does not require any surgical incisions (open biopsy or manipulation), supports its important role in triaging and managing bone lesions with minimum risk or morbidity.

Adolescent↗

Epithelioid hemangioendothelioma with multiple site involvement. Literature review and observations.

This report is a case of epithelioid hemangioendothelioma presenting as multiple lytic lesions of the ilium with radiographic findings of diffuse, bilateral lung involvement and biopsy-proven scalp involvement. Histologically, the tumor within bone and skin exhibited cords and nests of plump, epithelioid-appearing cells exhibiting rudimentary vascular differentiation within a myxohyaline stroma. Aggressive histologic features were not present. Immunohistochemical reactivity for Factor VIII-related antigen, Q-bend 10 (CD34), and cytokeratin were demonstrated. Ultrastructural studies revealed abundant intermediate cytoplasmic filaments, pinocytotic vacuoles, and Weibel-Palade bodies. The concurrent bone, skin, and lung involvement, low-grade histologic type, and female sex of the patient aroused speculation about the role of hormones in the development and possible treatment of the tumor, but estrogen and progesterone receptors were not detected. Despite intense combination chemotherapy, the patient died of widely metastatic disease. This report demonstrates the aggressive potential of histologically low-grade epithelioid hemangioendothelioma and the need for a thorough evaluation for metastases.

Adult↗

Significance of overall treatment time for the development of radiation-induced intestinal complications. An experimental study in the rat.

BACKGROUND: Late normal tissue reactions generally are believed to be independent of treatment time. However, previous studies suggest a relationship between acute mucosal injury and development of intestinal obstruction and enterocutaneous fistula formation. Thus, the pathogenesis of late intestinal complications may be complex, and mucosal cell proliferation during treatment may be important. This study assessed the influence of overall radiation treatment time on development of intestinal injury and complications after localized fractionated irradiation of rat ilium. METHODS: Ninety-four male rats underwent orchiectomy, and a loop of small intestine was transposed to the scrotum. Orthovoltage irradiation was administered to the transposed, but functionally intact, intestine using 9 fractions of 5.6 Gy with interfraction intervals of 24, 48, or 72 hours. The animals were observed for complications and killed in groups 2 and 26 weeks after irradiation for assessment of injury. Incidence of intestinal complications and quantitative and semiquantitative histopathologic assessment of injury were used as endpoints. RESULTS: Increasing total treatment time by extending interfraction intervals from 24 to 48 hours significantly reduced radiation injury and the incidence of intestinal complications. Differences in mucosal and fibrotic changes were most prominent. No significant differences were found between groups with interfraction intervals of 48 and 72 hours. CONCLUSIONS: Overall treatment time significantly influenced development of chronic radiation enteropathy. Thus, rapidly proliferating cells, as found in the intestinal mucosa, appear to be involved in the pathogenesis of intestinal complications. This probably represents consequential damage secondary to disruption of mucosal integrity.

Animals↗

Surgical indications for Ewing's sarcoma of the pelvis.

BACKGROUND: Despite advances in adjuvant therapy, Ewing's sarcoma of the pelvis remains an anatomic site with a poor prognosis. This study evaluate the role of surgery in the management of patients with pelvic Ewing's sarcoma who also received conventional radiation therapy and chemotherapy. METHODS: From May 1978 to February 1994, 19 patients with Stage IIB Ewing's sarcoma of the pelvis were treated at the UCLA Medical Center (Los Angeles, CA). There were eight lesions of the ilium, two of the sacrum, and nine involving two adjoining regions of pelvis. All patients received conventional medical management. The 19 patients were divided into two groups according to treatment modality. A group of 12 patients (Group A) had surgical resection, and their results were compared with those of another group of 7 patients (Group B) who did not have surgery. RESULTS: The 5-year cumulative survival (Kaplan-Meier method) was 39% for all patients, 51% for Group A, and 18% for Group B. The 3-year cumulative survival was 59% for all patients, 72% for Group A, and 36% for Group B. Although the survival rate of Group A seemed better than that of Group B, the difference was not statistically significant (P = 0.093, log rank method). This study also suggested that, regardless of treatment modality, the outcome of patients with lesions involving two adjoining pelvic bones was poorer than that of those with a single lesion. In Group A, the 3-year cumulative survival rate for patients with single bone lesions (n = 8) was 86% and for patients with lesions involving two adjoining pelvic bones (n = 4) was 50% (P = 0.045, log rank method). Furthermore, the statistical analysis of the combined data of the single pelvic bone lesions in UCLA and that of Mayo Clinic series (n = 16 for surgery group and n = 15 for nonsurgery group) confirmed the better results for the surgical patients, which was consistent with the results from the Mayo Clinic with an even greater significance (P < 0.002). CONCLUSION: This study demonstrates that surgery plus chemotherapy and radiation therapy is helpful for treating patients with pelvic Ewing's sarcoma so long as the tumor is limited to a single pelvic bone.

Adolescent↗

Quantifying trabecular orientation in the pelvic cancellous bone of modern humans, chimpanzees, and the Kebara 2 Neanderthal.

The adaptive nature of bone lies in its ability to respond to the environment by conforming and reshaping itself constantly to accommodate life-time stresses experienced throughout daily activities. In order to keep strains within the bone as uniform and isotropic as possible, the trabecular orientation is determined by forces acting on the bone through adaptive remodeling. Hence, the preserved structure of bones may contain direct information about the forces they may have undergone. Some authors (Correnti [1952], Atti Acc Naz Lincei 12:518-523, [1955] Riv Antrop 42:289-336; Macchiarelli et al. [1999] J Hum Evol 36:211-232, [2001] Cambridge, UK: Cambridge University Press) have described in detail the trabecular systems of the hip bone in different primate species and have identified a gait-related system above the acetabulum with substantial differences across species (Macchiarelli et al. [1999]; Rook et al. [1999] Proc Natl Acad Sci USA 96:8875-8879). The aim of this study was to quantify trabecular orientation above the acetabulum to test the hypothesis that hominoid biomechanical behavior is recorded in the cancellous bone. The pelvic bones of 23 archaeological adult modern humans (12 females, 11 males), 20 adult Pan troglodytes (10 females, 10 males), and one adult male Neanderthal were radiographed and digitized. Fast Fourier transforms (FFTs) of the regions of interest in the corpus of the ilium were performed, with the angular distribution of the trabeculae quantified. All species displayed a constant and periodic orthogonal arrangement in the trabeculae with differences in the pattern of dominance between the arcades oriented along the 0 degrees or the 90 degrees axes. The variation in the FFT spectrum between species is discussed in the light of distinctive biomechanical features.

Animals↗

Cross-population analysis of the growth of long bones and the os coxae of three Early Medieval Austrian populations.

Inter-population variability in long-bone and pelvic-bone growth during the Early Medieval period is examined. The materials comprise four archaeological populations: two Slavonic (Gars-Thunau, Zwentendorf, Austria, 10th-century AD), one Avar (Zwölfaxing, Austria, 8th-century AD), and one Anglo-Saxon (Raunds, England, 10th-century AD). Bone measurements are analyzed against dental age estimates in order to assess inter-population differences in growth rates for long-bone and os coxae bone dimensions. Growth curves of the upper and lower extremities of additional archaeological populations and a modern North-American population are also assessed. The expectation was that the greatest differences in growth patterns would be found between the Anglo-Saxon and the Austrian samples, due to their distinct genetic and biocultural background. Minimal differences were expected between the two Slavonic populations, as these were approximately contemporaneous, recovered from geographically close locations, and shared relatively similar archaeological contexts. Growth curves were estimated for each bone dimension by fitting least-squares fourth-order polynomials (which allowed testing of population differences by analysis of covariance), and iteratively estimating Gompertz growth curves. The results showed differences between bones in the extent of inter-population variability, with diaphyseal long-bone growth showing equivalent patterns across the four populations, but significant differences between populations in the growth patterns of distal diaphyseal dimensions of the femur and humerus and the dimensions of the ilium. Varying growth patterns are therefore associated with inter-population differences in absolute dimensions in relation to age as well as variations in growth velocities. Inter-population variability in growth curves in the case of femoral and humeral dimensions were most pronounced during infancy (0-2 years). The most consistent differences in bone growth and related dimensions are between Zwölfaxing and the other samples. No significant differences in growth were detected between the Anglo-Saxon and the Austrian populations.

Adolescent↗

Evolution of the sacrum in hominoids.

In order to study the formation of the sacrum during the primate evolution, a new way of numbering mammalian vertebrae is presented; this demonstrates that the thoracolumbosacral complex is fixed at 22 vertebrae in 80% and at 22 +/- 1 in 100% of the cases. The shift of a vertebra from one type to another occurs either at the thoracolumbar or at the lumbosacral junction and not at the cervicothoracic junction. Rarely does the shift take place at the sacrococcygeal junction. Data from 318 primates reveal that the seven original lumbar vertebrae of the Old World monkeys are reduced in the great apes by a caudad "thoracization" of one to two lumbar vertebrae and a cephalad sacralization of one to four lumbar vertebrae. In the apes, sacralization is not total and different stages that are intermediate between lumbar and sacral are described. In Homo sapiens there is a total sacralization of the last two original lumbar vertebrae. In addition, development of the sacral wings (alae) is minimal in apes and reaches its maximum in hominids. The tendency of the hominoid sacrum to incorporate the last lumbar vertebrae and to widen markedly provides for an enhanced articulation of the sacrum with the ilium and offers a firm base of support for the trunk during erect posture. This is necessary for the support of the weight of the trunk above the sacrum and for the stabilization of the body during bipedal posture and locomotion. Encephalization did not play any major role in the widening of the sacrum since the former by far preceded the latter.

Animals↗