Management of Chronic Osteomyelitis by an Orthoplastic Team: 7-Year Experience of a University Hospital.

BACKGROUND: Treating chronic osteomyelitis of the lower extremities is challenging. The treatment of acute lower limb trauma by orthoplastic teams has shown good results over the past few decades. This study aimed to characterize surgical outcomes of leg and heel chronic osteomyelitis by an orthoplastic team. METHODS: The cases of 113 consecutive leg and heel chronic osteomyelitis patients undergoing soft-tissue reconstruction with an orthopedic procedure were reviewed in this retrospective single-center observational study. The main objective was to assess surgical outcomes of skin healing and gait recovery at the 1-year follow-up. The secondary objective was to evaluate the global success rate at the last follow-up. RESULTS: The median follow-up was 19.7 months. A free flap was performed for 33 patients (29.2 percent) and a locoregional flap was used in 79 patients (69.9 percent). Seventy-two patients (63.7 percent) had chronic osteomyelitis on continuous bone. The others had a septic pseudarthrosis with a mean bone defect length of 42.9 mm. Forty-four patients (38.9 percent) underwent curettage only, eight (7.1 percent) underwent curettage and cement, 20 (17.7 percent) underwent curettage and bone fixation, and 39 (34.5 percent) underwent the Masquelet technique. At the 1-year follow-up, 72 patients (63.7 percent) had achieved skin healing and had recovered their gait. The success rate at all follow-up time points was 82.3 percent. The median time to achieve skin healing was 6.5 months and that to bone union in cases of septic pseudarthrosis was 7.9 months. CONCLUSION: Orthoplastic management of leg and heel chronic osteomyelitis patients with combined soft-tissue reconstruction using an orthopedic procedure was a viable strategy that offered good results even though the time to complete healing was long. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Outcome of conservative and surgical treatment of enchondromas and atypical cartilaginous tumors of the long bones: retrospective analysis of 228 patients.

BACKGROUND: Sufficient data on outcome of patients with clinically and radiologically aggressive enchondromas and atypical cartilaginous tumors (ACT) is lacking. We therefore analyzed both conservatively and surgically treated patients with lesions, which were not distinguishable between benign enchondroma and low-grade malignant ACT based upon clinical and radiologic appearance. METHODS: The series included 228 consecutive cases with a follow-up > 24 months to assess radiological, histological, and clinical outcome including recurrences and complications. Pain, satisfaction, functional limitations, and the musculoskeletal tumor society (MSTS) score were evaluated to judge both function and emotional acceptance at final follow-up. RESULTS: Follow-up took place at a mean of 82 (median 75) months. The 228 patients all had comparable clinical and radiological findings. Of these, 153 patients were treated conservatively, while the other 75 patients underwent intralesional curettage. Besides clinical and radiological aggressiveness, most lesions were histologically judged as benign enchondromas. 9 cases were determined to be ACT, while the remaining 7 cases had indeterminate histology. After surgery, three patients developed a recurrence, and a further seven had complications of which six were related to osteosynthesis. Both groups had excellent and almost equal MSTS scores of 96 and 97%, respectively, but significantly less functional limitations were found in the non-surgery group. Further sub-analyses were performed to reduce selection bias. Sub-analysis of histologically diagnosed enchondromas in the surgery group found more pain, less function, and worse MSTS score compared to the non-surgery group. Sub-analysis of smaller lesions (< 4.4 cm) did not show significant differences. In contrast, larger lesions displayed significantly worse results after surgery compared to conservative treatment (enchondromas > 4.4 cm: MSTS score: 94.0% versus 97.3%, p = 0.007; pain 2.3 versus 0.8, p = 0.001). The majority of lesions treated surgically was filled with polymethylmethacrylate bone-cement, while the remainder was filled with cancellous-bone, without significant difference in clinical outcome. CONCLUSION: Feasibility of intralesional curettage strategies for symptomatic benign to low-grade malignant chondrogenic tumors was supported. Surgery, however, did not prove superior compared to conservative clinical and radiological observation. Due to the low risk of transformation into higher-grade tumors and better functional results, more lesions might just be observed if continuous follow-up is assured.

How Accurate is the Multiplier Method in Predicting the Timing of Angular Correction After Hemiepiphysiodesis?

PURPOSE: The purpose of the study was to validate the accuracy of the Multiplier Method (MM) in predicting the timing of angular correction after hemiepiphysiodesis and to determine the role of using skeletal age when calculating those predictions. METHODS: This retrospective study included 131 physes in 77 patients treated with hemiepiphysiodesis to gradually correct a coronal plane deformity before skeletal maturity. To compare the MM's predictions to the actual treatment duration, the "desired angular correction" was considered the actual achieved angular correction determined from the "endpoint x-ray" (last x-ray before implant removal). We measured the bone length and width of the growth plate from the preoperative x-ray and calculated the MM's prediction of the duration of treatment based on the MM formula. We compared the predicted duration to the observed duration of treatment for each case. The difference was calculated by subtracting the observed duration from the predicted duration. The result was the "absolute difference," which is the number of months over or under predicted by the MM. RESULTS: The mean absolute difference between the MM's predicted duration and the observed duration was 2.31 months, which was highly significant (P≤0.001). The MM's prediction agreed with the observed duration of treatment (ie, zero absolute difference) in 15% of the predictions, 69% were under predicted, and 16% were over predicted. Sixty-eight percent of the absolute differences were within 3 months regardless of the direction of error. The mean difference was relatively less in genu varum cases and was statistically significant (P=0.047). Comparing the mean difference using chronological age and skeletal age in the formula showed no statistically significant difference. CONCLUSIONS: The MM has a tendency to under predict. Therefore, doing a guided growth right before skeletal maturity should be started 2 to 4 months earlier than suggested by the MM. Moreover, our data did not show that the bone age gave more accurate predictions than chronological age. LEVEL OF EVIDENCE: Level IV.

Outcomes after extensive manual curettage and limited burring for atypical cartilaginous tumour of long bone.

AIMS: Adjuvant treatment after intralesional curettage for atypical cartilaginous tumours (ACTs) of long bones is widely accepted for extending surgical margins. However, evaluating the isolated effect of adjuvant treatment is difficult, and it is unclear whether not using such adjuvants provides poor oncological outcomes. Hence, we analyzed whether intralesional curettage without cryosurgery or chemical adjuvants provides poor oncological outcomes in patients with an ACT. PATIENTS AND METHODS: A total of 24 patients (nine men, 15 women) (mean age 45 years; 18 to 62) were treated for ACTs of long bones and followed up for a median of 66 months (interquartile range 50 to 84). All patients were treated with extensive manual curettage and limited burring. Bone cement and grafts were used to fill bone defects in 16 and eight patients, respectively. No chemical adjuvants or cryosurgery were used. RESULTS: No local recurrence was detectable on plain radiographs and MRI or CT images. At the last follow-up, there were no distant metastases or disease-specific deaths. No procedure-related complications or postoperative fractures developed. CONCLUSION: Intralesional curettage without cryosurgery or chemical adjuvants may provide excellent oncological outcomes for patients with ACTs of long bones, without the risk of complications related to adjuvant use. Our investigation suggests thorough curettage alone is a reasonable treatment option for ACT. However, we acknowledge the limited size of our investigation warrants a multicentre collaborative study to confirm our findings. Cite this article: Bone Joint J 2018;100-B:256-61.

Does a less intensive surveillance protocol affect the survival of patients after treatment of a sarcoma of the limb? updated results of the randomized TOSS study.

AIMS: A single-centre prospective randomized trial was conducted to investigate whether a less intensive follow-up protocol would not be inferior to a conventional follow-up protocol, in terms of overall survival, in patients who have undergone surgery for sarcoma of the limb. Initial short-term results were published in 2014. PATIENTS AND METHODS: The primary objective was to show non-inferiority of a chest radiograph (CXR) group compared with a CT scan group, and of a less frequent (six-monthly) group than a more frequent (three-monthly) group, in two-by-two comparison. The primary outcome was overall survival and the secondary outcome was a recurrence-free survival. Five-year survival was compared between the CXR and CT scan groups and between the three-monthly and six-monthly groups. Of 500 patients who were enrolled, 476 were available for follow-up. Survival analyses were performed on a per-protocol basis (n = 412). RESULTS: The updated results recorded 12 (2.4%) local recurrences, 182 (36.8%) metastases, and 56 (11.3%) combined (local + metastases) recurrence at a median follow-up of 81 months (60 to 118). Of 68 local recurrences, 60 (88%) were identified by the patients themselves. The six-monthly regime (overall survival (OS) 54%, recurrence-free survival (RFS) 46%) did not lead to a worse survival and was not inferior to the three-monthly regime (OS 55%, RFS 47%) in terms of detecting recurrence. Although CT scans (OS 53%, RFS 54%) detected pulmonary metastasis earlier, it did not lead to a better survival compared with CXR (OS 56%, RFS 59%). CONCLUSION: The overall survival of patients who are treated for a sarcoma of the limb is not inferior to those followed up with a less intensive regimen than a more intensive protocol, in terms of frequency of visits and mode of imaging. CXR at six-monthly intervals and patient education about examination of the site of the surgery will detect most recurrences without deleterious effects on the eventual outcome. Cite this article: Bone Joint J 2018;100-B:262-8.

The Efficacy of Percutaneous Lateral Hemiepiphysiodesis on Angular Correction in Idiopathic Adolescent Genu Varum.

BACKGROUND: Percutaneous lateral hemiepiphysiodesis of the lower extremity is a simple and excellent method to correct the angular and length problems cosmetically. However, the efficacy of percutaneous lateral hemiepiphysiodesis is not well established in the literature. The purpose of this study was to evaluate the efficacy of percutaneous lateral hemiepiphysiodesis for angular corrections in adolescent idiopathic genu varum patients with proximal tibia vara and identify the factors affecting the amount of deformity correction of the lower limb in the coronal plane. METHODS: We retrospectively reviewed 20 patients (40 lower limbs) who had percutaneous lateral hemiepiphysiodesis on the proximal lateral tibia between 1997 and 2010. Radiographic evaluations were made using (1) the hip-knee-ankle angle and (2) the length of the tibia. Furthermore, the intercondylar distance was evaluated at the level of the knee joint. Preoperative factors (gender, age, body mass index, intercondylar distance, preoperative hip-knee-ankle angle, remaining growth of tibia, and calculated correctable angle) were analyzed, as well as their correlation with the degree of the actual correction angle. RESULTS: The amount of coronal deformity of the lower limb was improved from its preoperative state. The median average of hip-knee-ankle angle improved from 8.0° (interquartile range [IQR], 7.0° to 10.0°) preoperatively to 3.0° (IQR, 2.5° to 4.0°) at the final follow-up (p < 0.001). The median percent ratio of the angular correction was 60% (IQR, 50% to 71.3%). The correlation coefficients were -0.537, 0.832, 0.791, and 0.685 for the bone age, preoperative hip-knee-ankle angle, the remaining growth of tibia, and calculated correctable angle, respectively. CONCLUSIONS: Despite the excellent cosmetic outcome of percutaneous lateral hemiepiphysiodesis on the proximal lateral tibia in adolescent idiopathic genu varum, the effect was limited in most cases. For optimum results, surgery a few months earlier is recommended, rather than at the calculated operation time.

Re-evaluation of bone pain in patients with type 1 Gaucher disease suggests that bone crises occur in small bones as well as long bones.

Bone crises in type 1 Gaucher disease are reported in long bones and occasionally in weight bearing bones and other bones, but rarely in small bones of the hands and feet. We retrospectively examined the incidence of bone pain in patients followed at the Rabin Medical Center, Israel, before and following the initiation of enzyme replacement therapy (ERT) and evaluated them for bone crises. Of 100 type I Gaucher disease patients, 30 (30%) experienced one or more bone crises. Small bone crises represented 31.5% of all bone crises and were always preceded by crises in other bones. While the incidence of long bone crises reduced after the initiation of ERT, small bone crises increased. Almost 60% of patients with bone crises were of the N370S/84GG genotype suggesting a greater susceptibility of N370S/84GG patients to severe bone complications. These patients also underwent the greatest number of splenectomies (70.6% of splenectomised patients). Splenectomised patients showed a trend towards increased long and small bone crises after surgery. Active investigation of acute pain in the hands and feet in patients in our cohort has revealed a high incidence of small bone crises. Physicians should consider imaging studies to investigate unexplained pain in these areas.

Do Surgical Margins Affect Local Recurrence and Survival in Extremity, Nonmetastatic, High-grade Osteosarcoma?

BACKGROUND: Long-term survival for all patients with osteosarcoma using current aggressive adjuvant chemotherapy and surgical resection is between 60% and 70%. In patients who present with nonmetastatic, high-grade extremity osteosarcoma of bone, limb salvage surgery is favored, when appropriate, over amputation to preserve the limb, because limb salvage may lead to a superior quality of life compared with amputation. However, concern remains that in the attempt to preserve the limb, close or microscopically positive surgical margins may have an adverse effect on event-free survival. QUESTIONS/PURPOSES: (1) Does a positive or close surgical margin increase the likelihood of a local recurrence? (2) Does a positive or close surgical margin adversely affect the development of metastatic disease? (3) What is the relationship of surgical margin on overall survival? METHODS: With institutional review board approval, we retrospectively evaluated 241 patients treated at our institution between 1999 and 2011. Exclusion criteria included nonextremity locations, metastatic disease at initial presentation, low- or intermediate-grade osteosarcoma, treatment regimens that did not follow National Comprehensive Cancer Network (NCCN) guidelines, incomplete medical records, and any part of treatment performed outside of Moffitt Cancer Center or All Children's Hospital. Fifty-one patients were included in the final analysis, of whom 31 (61%) had followup data at a minimum of 2 years or whose clinical status was known but had died before 2 years of followup. Margin status was defined as (1) microscopically positive; (2) negative ≤ 1 mm; and (3) negative > 1 mm. Margin status, histologic response (tumor percent necrosis), type of osteosarcoma, type of surgery, presence of local recurrence, metastatic disease, and overall survival were recorded for each patient. The mean age was 22 years (range, 12-74 years) and the mean followup was 3 years (range, 0.1-14 years). Margin status was positive in 10% (five of 51), negative ≤ 1 mm 26% (13 of 51), and negative > 1 mm 65% (33 of 51). RESULTS: Local recurrence was noted to be 14% (seven of 51) at 3.4 years. After controlling for relevant confounding variables, the presence of a positive margin compared with a negative margin > 1 mm was the only independent predictor of local recurrence (hazard ratio [HR], 8.006; 95% confidence interval [CI], 1.314-48.781; p = 0.0241). At a mean of 3.4 years, 29% (15 of 51) of the patients developed metastatic disease with no difference with the numbers available in the probability of developing metastatic disease among the three margin groups (p = 0.614). Overall survival at 3.8 years was 75% (38 of 51). After controlling for relevant confounding variables, we found that patients with positive margins were more likely to die from disease than those with negative margins (HR, 6.26; 95% CI, 1.50-26.14; p = 0.0119); no other independent predictors of survival were identified. CONCLUSIONS: With the numbers of patients we had, we observed that patients with extremity, nonmetastatic, high-grade osteosarcoma who had positive margins showed a higher probability of local recurrence in comparison to those with negative surgical margins. Given that positive margins appear to be associated with poorer survival in patients with high-grade osteosarcoma of the extremities, surgeons should strive to achieve negative margins, but larger studies are needed to confirm these findings. LEVEL OF EVIDENCE: Level III, therapeutic study.

Evaluation of outcome of surgical excision of the nidus of osteoid osteoma of long bone.

Osteoid osteoma is a benign bone tumour usually found in the lower extremities of children and young adults. This tiny bone tumour causes pain out of all proportion to its size and hinders the daily activities. This Quasi-experimental study conducted in the department of Orthopaedic surgery of BSMMU from January 2008 to December 2009. Twenty one patients were included in the study where purposive sampling technique was used on the basis of inclusion and exclusion criteria and all the ethical conditions were fulfilled. Diagnosis was almost obtained by taking history, clinical examination, and relevant investigations. Clinical variables were age, sex, site, pain, swelling, deformity and outcome variables were painless active life, removal of swelling, prevention of deformity, rate of recurrence. After localization of the tumour with the help of C arm, the nidus was excised in a small block of bone. The outcome is categorized by consensus, as clinically successful, only if the patient was free of pain and was taking no medication. The treatment was considered to have failed if a subsequent procedure had been performed to remove tumour. Among 21 cases, 14(66.7%) were male and 7(33.7%) were female. Maximum number of patients 15(71.4%) was between 10 years to 20 years. Most of the patients (76.2%) affected by osteoid osteoma were young students and most of the patients (95.2%) experienced moderate aching pain, usually aggravating at night which was typically relieved by aspirin or other NSAIDs (71.4%). Lower limbs (76.2%) particularly femur and tibia were commonly affected. Out of 21 patients, 19(90.5%) patients have got immediate pain relief or required no medication. In only 2 patients (9.5%), subsequent procedure has been performed to relief pain. So, successful outcome (in 19 out of 21) was significantly (p<0.001) higher in comparison to failed. Surgical excision of the nidus is a simple and easy procedure and does not require extensive resection of bone. If localization is done properly success rate is high and patients can return to normal daily activities.

Dosimetric comparison between VMAT with different dose calculation algorithms and protons for soft-tissue sarcoma radiotherapy.

BACKGROUND: To appraise the potential of volumetric modulated arc therapy (VMAT, RapidArc) and proton beams to simultaneously achieve target coverage and enhanced sparing of bone tissue in the treatment of soft-tissue sarcoma with adequate target coverage. MATERIAL AND METHODS: Ten patients presenting with soft-tissue sarcoma of the leg were collected for the study. Dose was prescribed to 66.5 Gy in 25 fractions to the planning target volume (PTV) while significant maximum dose to the bone was constrained to 50 Gy. Plans were optimised according to the RapidArc technique with 6 MV photon beams or for intensity modulated protons. RapidArc photon plans were computed with: 1) AAA; 2) Acuros XB as dose to medium; and 3) Acuros XB as dose to water. RESULTS: All plans acceptably met the criteria of target coverage (V95% >90-95%) and bone sparing (D(1 cm3) <50 Gy). Significantly higher PTV dose homogeneity was found for proton plans. Near-to-maximum dose to bone was similar for RapidArc and protons, while volume receiving medium/low dose levels was minimised with protons. Similar results were obtained for the remaining normal tissue. Dose distributions calculated with the dose to water option resulted ~5% higher than corresponding ones computed as dose to medium. CONCLUSION: High plan quality was demonstrated for both VMAT and proton techniques when applied to soft-tissue sarcoma.

Low-grade chondrosarcoma of long bones treated with intralesional curettage followed by application of phenol, ethanol, and bone-grafting.

BACKGROUND: A common treatment of low-grade cartilaginous lesions of bone is intralesional curettage with local adjuvant therapy. Because of the wide variety of different diagnoses and treatments, there is still a lack of knowledge about the effectiveness of the use of phenol as local adjuvant therapy in patients with grade-I central chondrosarcoma of a long bone. METHODS: A retrospective study was done to assess the clinical and oncological outcomes after intralesional curettage, application of phenol and ethanol, and bone-grafting in eighty-five patients treated between 1994 and 2005. Inclusion criteria were histologically proven grade-I central chondrosarcoma and location of the lesion in a long bone. The average age at surgery was 47.5 years (range, 15.6 to 72.3 years). The average duration of follow-up was 6.8 years (range, 0.2 to 14.1 years). Patients were evaluated periodically with conventional radiographs and gadolinium-enhanced magnetic resonance imaging (Gd-MRI) scans. When a lesion was suspected on the basis of the MRI, the patient underwent repeat intervention. Depending on the size of the recurrent lesion, biopsy followed by radiofrequency ablation (for lesions of <10 mm) or repeat curettage (for those of ≥10 mm) was performed. RESULTS: Of the eighty-five patients, eleven underwent repeat surgery because a lesion was suspected on the basis of the Gd-MRI studies during follow-up. Of these eleven, five had a histologically proven local recurrence (a recurrence rate of 5.9% [95% confidence interval, 0.9% to 10.9%]), and all were grade-I chondrosarcomas. General complications consisted of one superficial infection, and two femoral fractures within six weeks after surgery. CONCLUSIONS: This retrospective case series without controls has limitations, but the use of phenol as an adjuvant after intralesional curettage of low-grade chondrosarcoma of a long bone was safe and effective, with a recurrence rate of <6% at a mean of 6.8 years after treatment.

The outcome of surgical treatment for recurrent giant cell tumor in the appendicular skeleton.

BACKGROUND: The treatment for recurrent giant cell tumor (GCT) remains controversial. In this study, we evaluated the outcome of surgical intervention for recurrent GCT. METHOD: Twenty-seven patients (14 males and 13 females) with recurrent GCT were recruited. Their primary GCTs were all treated with intralesional surgery. Among these recurrent GCTs, 9 grade III and 1 grade II tumors were treated with en bloc resection and endoprosthetic replacement, whereas 16 grade II and 1 grade III tumors were treated with intralesional curettage and PMMA bone cement filling. RESULTS: The mean interval between initial surgery and first recurrence was 28.8 months (range 7-97 months). About 70 % of first recurrences affected bones around the knee, 44 % in the proximal tibia and 26 % in the distal femur. Of 27 patients, 3 women treated with intralesional procedures suffered second recurrences in the proximal tibia. No second recurrence was found in patients with en bloc resection. Two grade III re-recurrence GCTs were treated with en bloc resection, and 1 grade II was treated with an intralesional procedure. One patient with en bloc resection developed tumor metastasis in both lungs. Compared to patients with intralesional treatment, the functional score was significantly decreased in patients with en bloc resection (p < 0.01). CONCLUSION: The re-recurrence risk of grade III GCTs can be significantly decreased by wide en bloc resection and endoprosthetic replacement. However, intralesional treatment is a good option for less aggressive (

Stress fractures in children.

BACKGROUND: Presentation of own experiences, observations and results of treatment of patients with stress fractures treated in the Department of Paediatric Orthopaedics. The authors present an algorithm of diagnosing and treatment of stress fractures. MATERIAL AND METHODS: Over the period 1998 - 2010, 38 patients were hospitalised (40 fractures) with the final diagnosis of stress fracture. Personal patients' data, case histories and management preceding the diagnosis of the disease, diagnostic and therapeutic methods, as well as the results of treatment were collected on the basis of medical documentation. Standard diagnostic procedure consisted of: clinical and radiological examination, laboratory tests as well as computed tomography (CT). In 11 cases, the procedure included also scintigraphy and in two children MRI scan was performed. RESULTS: Complete healing of fractures was finally achieved in all patients CONCLUSIONS: In case of suspected stress fracture, it is necessary to perform a series of radiograms, which should present the picture of bone rebuilding and incorporation of the periosteal callus. If plain radiograph is inconclusive we should consider MRI , CT or scintigraphy scan. The results of laboratory tests should not show any abnormalities. In case of confirmed stress fracture nonweight-bearing of the affected extremity is recommended as well as introducing of close clinical-radiological monitoring. The results of MRI examination should be interpreted very cautiously, be cause it can be misleading. The observation strategy ("wait and see") should be implemented. However, the lack of the regression of pain sensation, bone rebuilding features and incorporation of periosteal callus or the appearance of visible bone destruction (lysis) should raise questions and present an indication for taking biopsy of the laesion.

Survival from high-grade localised extremity osteosarcoma: combined results and prognostic factors from three European Osteosarcoma Intergroup randomised controlled trials.

BACKGROUND: Neoadjuvant chemotherapy improves outcome in osteosarcoma. Determination of optimum regimens for survival, toxicity and prognostic factors requires randomised controlled trials to be conducted. PATIENTS AND METHODS: Between 1983 and 2002, the European Osteosarcoma Intergroup recruited 1067 patients with localised extremity osteosarcoma to three randomised controlled trials. Standard treatment in each was doxorubicin 75 mg/m(2) and cisplatin 100 mg/m(2). Comparators were addition of methotrexate (BO02/80831), a multidrug regimen (BO03/80861) and a dose-intense schedule (BO06/80931). Standard survival analysis methods were used to identify prognostic factors, temporal and other influences on outcome. RESULTS: Five- and 10-year survival were 56% (95% confidence interval 53% to 59%) and 52%, respectively (49% to 55%), with no difference between trials or treatment arms. Median follow-up was 9.4 years. Age range was 3-40 years (median 15). Limb salvage was achieved in 69%. Five hundred and thirty-three patients received the standard arm, 79% completing treatment. Good histological response to preoperative chemotherapy, distal tumour location (all sites other than proximal humerus/femur) and female gender were associated with improved survival. CONCLUSIONS: Localised osteosarcoma will be cured in 50% of patients with cisplatin and doxorubicin. Large randomised trials can be conducted in this rare cancer. Failure to improve survival over 20 years argues for concerted collaborative international efforts to identify and rapidly test new treatments.

Multicystic bone disease (Gorham-Stout syndrome) in a spider monkey (Ateles geoffroyi ).

BACKGROUND: In April 2000, a 2.5-year-old pet female Geoffroyi's spider monkey presented for reduced activity, a subdued demeanor, and boney enlargement involving both radii. METHODS: On further examination, polyostotic bone cysts were identified involving many of the tubular bones and were identified radiographically. Microscopic examination of a bone biopsy revealed hemorrhage and other characteristics typical of an aneurysmal bone cyst. In addition, excessive osteoclasia was noted, in association with fibrotic areas rather than with Howship's lacunae as expected from a growing animal. RESULTS: These findings were consistent with Gorham-Stout syndrome, a rare condition reported previously in ∼175 human cases and in a dog at necropsy. The diet history and further testing suggested a negative calcium balance. Treatment included the administration of bis-phosphonates, which appeared to bring about marked improvement. Almost 8 years later (November 2008), radiographs were again taken and suggested some resolution of bone cysts, primarily those in the legs. CONCLUSIONS: This represents the first reported case and a potential therapy for this rare condition in a non-human primate.

Radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis in nine patients.

OBJECTIVE: The purpose of this article was to evaluate the radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis (LCH), emphasizing the CT and MR findings. MATERIALS AND METHODS: The images of nine patients with pathologically proven LCH (five men and four women; mean age, 37.11 years) were retrospectively reviewed. Imaging analysis was confined to the long and flat bones. CT scans were performed in five patients and MR imaging was performed in eight. Images were assessed for the following features on CT and MRI: the location and number of lesions; the presence of cortical destruction, endosteal scalloping, and a periosteal reaction on CT or MRI; the margin of soft tissue masses, the presence of bone marrow edema, and a "budding" appearance on MRI; and the presence of sclerotic margins or septations on CT. RESULTS: The involved skeletal sites were the pelvis (seven), femurs (five), humeri (two), tibias (two), fibula (one), clavicle (one), scapula (one), and sternum (one). Endosteal scalloping, a periosteal reaction, and a budding appearance were common on MRI or CT images. Although cortical destruction and the soft tissue lesion formation were rare, soft tissue masses had well-defined margins. CONCLUSIONS: Endosteal scalloping and a budding appearance with a periosteal reaction on CT and MRI may be helpful signs for differentiation of LCH from malignant tumors in adults.

Possible beneficial effect of bisphosphonates in osteonecrosis of the knee.

UNLABELLED: Osteonecrosis (ON) in the knee occurs as a localized inflammatory disease in relation to spontaneous or non-traumatic ON. Conservative treatment possibilities are limited, and prognosis appears to be poor; in most cases, ON results in knee arthroplasty. Bisphosphonates are suggested to prevent bone resorption and collapse of necrotic bone. In this observational, prospective study we investigated the effect of bisphosphonate treatment in patients with spontaneous or arthroscopy-induced ON of the knee. Twenty-eight patients with osteonecrotic lesions and bone marrow oedema in the knee were included. In 22 patients (80%), ON was identified after arthroscopic surgery of the knee; six patients were diagnosed with spontaneous ON. Patients were initially given pamidronate 120 mg i.v. divided in 3-4 perfusions over 2 weeks, followed by oral bisphosphonate treatment with alendronate 70 mg weekly for 4-6 months. Bisphosphonate treatment resulted in a rapid pain relief, VAS decreasing from 8.2 ± 1.2 at baseline to 5.02 ± 0.6 after 4-6 weeks (p < 0.001). After 6 months, the VAS decreased by 80% (p < 0.001). At the 6-month follow-up, symptoms had resolved completely in 15 patients out of 28; in 6 patients, minimal symptoms (VAS 1-2) remained. In two patients, treatment effect was unsatisfactory, and surgical intervention was needed (arthroplasty). Bone marrow oedema on MRI resolved completely in 18 patients out of 28 with substantial reduction in the remaining. Furthermore, osteonecrotic area resolved completely or demarcation with sclerotic changes of the necrotic area could be observed. Bisphosphonate treatment in patients with osteonecrosis of the knee was associated with a rapid improvement in pain score and radiological consolidation of the area of osteonecrosis. Further randomized, controlled trials are warranted to confirm the potential beneficial role of bisphosphonates in the treatment of osteonecrosis of the knee. LEVEL OF EVIDENCE: observational study, level IV.

[Infiltrative basal cell carcinoma presenting as chronic leg ulcer]. / Carcinome basocellulaire infiltrant se présentant comme un ulcère de jambe chronique.

BACKGROUND: Basal cell carcinoma is the most common carcinoma of the skin and is usually found on the head and neck. We report an unusual case of basal cell carcinoma presenting as a chronic leg ulcer, with underlying bone involvement. CASE REPORT: A 70-year-old woman presented with a 15-year history of leg ulcer refractory to treatment. Because of the exuberant granulation tissue on the base and the indurated edges of this circumferential leg ulcer, several biopsies were taken from the edge and the base of the ulcer. Histological examination revealed infiltrative basal cell carcinoma. Treatment consisted of surgical excision of 80% of the primary lesion and coverage with a split-thickness skin graft. Examination of the surgical piece revealed invasion of bone by the carcinoma. The remainder of the lesion not accessible to surgery was irradiated. DISCUSSION: There is a need for awareness among all doctors of the clinical signs evocative of malignant transformation of a leg ulcer so that a skin biopsy may be performed for suspicious ulcers. Our case is distinguished by the underlying invasion of bone by basal cell carcinoma, as attested by imaging and histology.

TGF-beta type II receptor phosphorylates PTH receptor to integrate bone remodelling signalling.

Parathyroid hormone (PTH) regulates calcium homeostasis and bone metabolism by activating PTH type I receptor (PTH1R). Here we show that transforming growth factor (TGF)-beta type II receptor (TbetaRII) forms an endocytic complex with PTH1R in response to PTH and regulates signalling by PTH and TGF-beta. TbetaRII directly phosphorylates the PTH1R cytoplasmic domain, which modulates PTH-induced endocytosis of the PTH1R-TbetaRII complex. Deletion of TbetaRII in osteoblasts increases the cell-surface expression of PTH1R and augments PTH signalling. Conditional knockout of TbetaRII in osteoblasts in mice results in a high bone mass with increased trabecular bone and decreased cortical bone, similar to the bone phenotype in mice expressing a constitutively active PTH1R. Disruption of PTH signalling by injection of PTH(7-34) or ablation of PTH1R rescues the bone phenotype of TbetaRII knockout mice. These studies reveal a previously unrecognized function for TbetaRII and a mechanism for integration of PTH and local growth factor at the membrane receptor level.