Incidentally diagnosed melorheostosis of upper limb: case report.

BACKGROUND: Melorheostosis is quite a rare bone disease with still unclear ethiology. Although multifocal affection is highly debilitating with unfavorable prognosis, there is no clear consensus about therapeutical approach. There is still insufficient evidence in the literature for almost a century after the first description. Affected bone has a typical appearance of melting wax. Diagnosis is usually incidental with pain as a leading symptom. Diagnosis itself is relatively easy, routine X-ray examination is sufficient. Even though it could be easily overlooked and mistaken with other diseases. Melorheostosis is incurable, the therapy is mostly focused on maintaining patient quality of life. Presented case is unique in terms of extent of the affection (index finger, metacarp shaft, carpal bones, forearm, humerus and whole scapula) in combination with osteopoikilotic islands in other 3 regions (vertebrae, manubrium sterni and left collar bone). Currently there is only one such a case published in the literature (Campbell), but without osteopoikilotic islands. CASE PRESENTATION: Melorheostosis was diagnosed in 26-year old female after injury as an incidental finding. This was quite surprising as the patient already suffered by limited movement in the upper limb and pain before the injury. Detailed examination were performed to confirm the diagnosis, no family history was found. Pharmacotherapy with bisphosphonates, non-steroidal antirheumatics and vasodilatans/rheologic drugs seemed to be effective to maintain the relatively good quality of patient life and good performance in daily routine. Questionable is further development of patient performance status and sustainability of conservative treatment in the long term follow up. CONCLUSION: Conservative treatment with bisphopshonates and COX-2 inhibitors in combination with naftidrofuryl can delay surgery solution.

Concave impressio ligamenti costoclavicularis ("rhomboid fossa") and its prevalence and relevance to clinical practice.

PURPOSE: The incidence of fossa rhomboidea (RF) and its relevance to clinical practice were retrospectively studied in 1,017 cases of patients who endured clavicle fractures. The aim of the study was to determine the frequency of different types of insertion areas of costoclavicular ligament (LCC) in our group, as well as gender distribution. Furthermore, we wanted to investigate whether the RF represents a weakened part of clavicle where the fracture occurred. Several of our findings in our control group were quite interesting and worth mentioning. MATERIALS AND METHODS: 1,017 roentgenograms were analyzed from 1,027 clavicle fractures of patients from 2 to 90 years of age who were treated between 2007 and 2011. RESULTS: Using the Allman classification system, 79% of the examined clavicle fractures were identified as type 1, 18.4% as type 2, and 2.6% as type 3. Statistical analysis did not demonstrate a difference in fractures of the clavicle between the right and the left side (47, 53%, respectively; p = 0.0570). There was a statistically significant difference in the incidence of fractures between the genders (males 71%, females 29 %; p < 0.0001). The results for the insertion attachment area were as follows for the given percent of cases: flat (75.2%); concave (21.1%); convex (3.7%). Those 21.1% of concave insertions represent RF. 5.3% of RF cases were between 2 to 10 mm in depth. One of the patients with RF had a deepend insertion of the LCC on the superior surface of the first rib analogous to RF. The bilateral RF was asymmetrical in its appearance. One of the patients suffered from LCC enthesopathy. The difference of the distribution of RF throughout the genders was 10.5% in females and 89.5% in males, marking a difference that is statistically significant (p < 0.0001). Only in 0.3%, the fracture line occurred in RF, which did not prove the RF to be a weakened point of the fracture (p < 0.0001). CONCLUSIONS: RF represents a variety of LCC insertion that is predominantly found in male patients, which can be used in the determination of gender in forensic analysis. RF is not a weak point for fracture formation. Enthesopathy of the LCC together with RF can lead to suspicion concerning the osteolytic process. Biopsy of RF should be avoided. Insertion of LCC similar to RF can be exceptionally found on the first rib.

Superficial soft tissue biphasic synovial sarcoma with apocrine differentiation in the glandular component: a report of two cases.

: The authors present 2 cases of a subcutaneous biphasic synovial sarcoma with marked apocrine differentiation that potentially may be confused with cutaneous epithelial neoplasms, including malignant apocrine mixed tumor or metaplastic carcinoma with an apocrine glandular component. Microscopically, both neoplasms had a biphasic architecture with the epithelial and spindle cell components. The epithelial component was prominent and consisted of simple glands with round lumina and complex glandular structures with intraluminal bridges forming cribriform areas. The glands were lined by cuboidal to columnar cells with eosinophilic or clear cytoplasm manifesting apical apocrine-like and intraluminal eosinophilic secretions. The spindle cell component was less prominent and was composed of relatively uniform or slightly atypical spindle sells surrounding and merging focally with the glandular structures. Immunohistochemically, the tumor cells in both components were positive for vimentin, AE1/AE3, CK7, and epithelial membrane antigen. Desmin, smooth muscle actin, muscle-specific actin, CD34, and S-100 protein were all negative. SYT-SSX1 gene fusion using fluorescence in situ hybridization and RT-PCR methods was detected in both cases.

[Geriatric traumatology - vision or reality?]. / Geriatrická traumatologie - vize nebo skutecnost?

The aim of the paper is to provide information on the establishment of the Geriatric Fracture Centre (GFC) at the Department of Orthopaedics and Traumatology, Faculty Hospital at the Faculty of Medicine, Charles University, Pilsner, whose goals and services are in accordance with the principles of the Rochester GFC, USA, and the AO Foundation at Synthes Inc. The paper presents up-to-date information on the methods of osteosynthesis for the skeletal system affected by osteoporosis, the majority of which was obtained in the AO Geriatric Fracture Course in Davos, December 2007. The authors are concerned with the pre-operative examination of geriatric patients who suffered a fracture.The anaesthesiologist's view on our elderly patients, their pre-operative preparation, anaesthesia application and post-operative care are presented.The novel method of two-phase bone densitometry and its role in the therapy of osteoporosis for elderly patients with fractures are also mentioned.

Crystal-deficient alveolar soft-part sarcoma with cutaneous involvement: a case report.

Alveolar soft-part sarcoma (ASPS) is a rare distinctive sarcoma, in most cases involving deep soft tissues of the extremities. It is associated with a specific unbalanced translocation, der(17)t(X;17)(p11;q25) that results in the formation of an ASPL-TFE3 fusion gene. Microscopically, it is typified by an alveolar growth of large cells containing typical periodic acid-Schiff-positive rod-shaped crystals, often serving as a diagnostic clue. Other distinctive features include nuclear immunoreactivity for transcription factor 3 (TFE3) protein and a typical ultrastructural finding of large crystals with a rectangular or rhomboid shape. The authors present an unusual case of ASPS with cutaneous involvement, which did not exhibit typical large crystals; there were striking round granules. Molecular genetic study revealed fusion transcript ASPL-TFE3, type 2. To the best of our knowledge, cutaneous involvement of a crystal-deficient ASPS has not been reported.

Reduction of venous thromboembolism following prolonged prophylaxis with the low molecular weight heparin Certoparin after endoprothetic joint replacement or osteosynthesis of the lower limb in elderly patients.

The peri- and postsurgical thromboembolic prophylaxis with low molecular weight heparins is a well established therapy regimen, but the optimum duration of prophylaxis after surgery still remains uncertain. A few studies have pointed to the fact that the thromboembolic risk of high-risk patients persists longer than the in-hospital period correlating with respective hypercoagulatory conditions. The aim of the present study was to test if a prolongation of thromboprophylaxis with the low molecular weight heparin Certoparin further reduces the rate of thromboembolism in high-risk patients after orthopedic surgery. The "Long-term Thromboprophylaxis"-Study was a multicenter, randomized, double-blind, placebo-controlled trial. 360 patients who underwent endoprothetic joint replacement or osteosynthesis of the lower limb were initially enrolled, all of them received prophylactically 3000 U anti-Xa of Certoparin once daily for 14 days followed by randomization to prolonged Certoparin application or to placebo up to day 42. Patients were screened for deep vein thrombosis by sonography every week. Coagulation markers (fibrin monomers and D-dimers) were determined during the course of the study. Venous thromboembolism during the prolongation period was observed in 18 patients receiving placebo versus 8 patients of the prolonged Certoparin group (12.1% versus 5.0%, intention-to-treat sample). The analysis revealed a statistically significant difference in favor of Certoparin (p=0.020), which was confirmed by per-protocol analysis (14.2% versus 5.5%, p=0.012). The differences remained significant, if analyses considered only clinically symptomatic thromboembolic events (p=0.040). Patients who developed a thrombosis showed a strong increase of coagulation markers as compared to patients without subsequent thrombosis. The respective differentiation started around 18 days before diagnosis of thrombosis. Only one minor bleeding complication was observed during prolonged Certoparin prophylaxis. The present study shows that patients after joint replacement or osteosynthesis of the lower extremities have a persisting risk to develop thromboembolic complications beyond the routine duration of thromboprophylaxis. Extended prophylaxis with Certoparin resulted in a significantly lower rate of thromboembolism and should be strongly recommended.