Bilateral Femur Research Articles

Preliminary study on the automatic preparation of dental implant socket controlled by micro-robot

Objective: To analyze the quantitative relationship between the number of layers of laser pulses and the amount of step in ultra-short pulse laser cutting of cortical bone, optimize the robot's vertical single stepping parameters, and to explore the feasibility of automatic preparation of dental implant cavity using robot controlling ultra-short pulse laser, in order to lay the foundation for automated dental implant surgery. Methods: Eight pig ribs were segmented into to make 16 specimens. Using the robotic surgical system and path planning software independently developed by our group, circular holes with a diameter of 4 mm were cut two-dimensionally in the rib segments to obtain the quantification relationship of the number of laser pulse layers (n) and the depth of two-dimensional (2D) cutting (d). When conducting the three-dimensional (3D) cutting procedure, the number of pulse layers were set to 5, 10, 15, 20, 25, 30, 35, 40, 45, 50 layers, the vertical single step amount was an integer value corresponding to the results of 2D cutting depth, and the number of pulses (n') corresponding to the minimum difference between the theoretical depth of cut and the actual depth of cut was obtained. The n' was taken as the most suitable single step pulse layer, the rib segment was cut, and the depth of single cut was measured while the integer value was taken as the most appropriate vertical single step amount (d'). The vertical parameters of laser single stepping were set as n' layer pulse and d' μm step size. The 3D cutting produces a cylindrical cavity with a diameter of 4 mm and a height of 2 mm to evaluate the 3D cutting accuracy (the difference between the measured value and the theoretical value of cutting diameter or depth). Ten 4 mm×3 mm implant holes were automatically prepared on the bilateral femurs of 5 Japanese big white rabbits, and ten 4 mm×3 mm implants made by 3D printer were artificially implanted, and the preparation effect of the implant cavities was evaluated. Results: The quantitative relationship curve between the number of laser pulses (n) and 2D depth of cut (d) showed a linear upward trend. The linear fitting obtained the quantitative relation function formula d=9.278 4 n±26.763 0, R(2)=0.988 9. The optimum number of single step pulse layers was 5 layers, and the vertical single step amount was 50 μm, so as to set the vertical parameters of a single step of a 3D cutting, and the 3D cutting diameter accuracy was (3.98±2.87) μm, with a depth accuracy of (15.42±5.44) μm. Automated preparation of 10 implant cavities on the femur of the rabbit were completed. When the implants were placed into the implant cavities, there was resistance, but they were fully seated and primary stability has been achieved after seating implant placement. Conclusions: The method of non-contact automatic preparation of dental implant cavities using robot controlling ultra-short pulse laser is feasible. By optimizing the single cutting process parameters, precise control of laser cutting cortical bone can be realized.

Which factors influence proximal femoral asymmetry?: a 3D CT analysis of 345 femoral pairs.

In patients where the proximal femur shows gross deformity due to degenerative changes or fracture, the contralateral femur is often used to perform preoperative templating for hip arthroplasty. However, femurs may not be symmetrical: the aim of this study was to determine the degree of variation between hips in healthy individuals and to determine whether it is affected by demographic parameters. CT-scan based modelling was used to examine the pelvis and bilateral femurs of 345 patients (211 males, 134 women; mean age 62 years (standard deviation (sd) 17), mean body mass index 27 kg/m2 (sd 5)) representing a range of ethnicities. The femoral neck-shaft angle (NSA), femoral offset (FO), femoral neck version (FNV), femoral length (FL), femoral canal flare index (fCFI), and femoral head radius (FHr) were then determined for each patient. All measurements were constructed using algorithm-calculated landmarks, resulting in reproducible and consistent constructs for each specimen. We then analyzed femoral symmetry based on absolute differences (AD) and percentage asymmetry (%AS) following a previously validated method. We found an asymmetry > 2% for NSA (mean AD 2.9°, mean %AS 2.3; p = 0.03), FO (AD 3.8 mm, %AS 9.1 ; p = 0.01), FNV (AD 5.1°, %AS 46.7 ; p = 0.001) and fCFI (AD 0.2 mm, %AS 5.4 ; p = 0.7). Significant relationships were found for AD regarding NSA and ethnicity (p = 0.037), FL and height (R2 = 0.22), and fCFI and gender (R2 = 0.34). Our data confirm the presence of asymmetry of proximal femurs, which is mostly independent of demographic parameters. In cases where contralateral templating is used, such asymmetry may lead to inaccurate anatomical restoration of the hip if the templated sizes are routinely implanted. However, the clinical impact cannot be determined from our investigation. Cite this article: Bone Joint J 2018;100-B:839-44.

Bruck Syndrome: A Rare Disorder in New-Born with Fractures and Contractures

Bruck syndrome is a very rare autosomal recessive syndrome consisting of bone fragility and congenital joint contractures. It is considered as a combination of arthrogryposis multiplex congenita and osteogenesis imperfecta, while some consider it as the autosomal recessive variant of osteogenesis imperfecta. According to the genotype, it has been classified into types 1 and 2. To our knowledge, only about 28 patients of this syndrome have been reported so far worldwide with none been reported from Nepal. Here, we present a patient with generalized osteopenia, bilateral femur fracture and congenital joint contractures of distal extremities.

Mechanism of melatonin combined with calcium carbonate on improving osteoporosis in aged rats.

The effects of melatonin and calcium carbonate on aged rats with osteoporosis (OP) were assessed. Forty female Sprague-Dawley (SD) rats aged 15 months were randomly divided into a model group (group OP), melatonin group (group M), calcium carbonate group (group Ca) and melatonin combined with calcium carbonate group (group M+Ca), while 10 rats aged 3 months were set as the control group (group NC). The changes of bone density and bone mineral level of lumbar vertebra and bilateral femur in rats of each group were observed. The levels of serum calcium, phosphorus, superoxide dismutase (SOD), malondialdehyde (MDA) and glutathione peroxidase (GSH-Px) in rats of each group were determined. Compared with those in group NC, bone density of lumbar vertebra and bilateral femur and bone mineral level were distinctly reduced, serum calcium and activities of SOD and GSH-Px were obviously decreased, and MDA content was remarkably increased in rats of groups OP, M and Ca; the differences were statistically significant (P<0.05 or P<0.01); compared with that in group OP, bone density of lumbar vertebra and bilateral femur and bone mineral level were remarkably increased, serum calcium and activities of SOD and GSH-Px were obviously increased, and MDA content was remarkably decreased in rats of groups M, Ca and M+Ca; the differences were statistically significant (P<0.05 or P<0.01); compared with those in groups M and Ca, bone density of lumbar vertebra and bilateral femur and bone mineral level were obviously elevated, serum calcium and activities of SOD and GSH-Px were evidently elevated, and MDA content was remarkably decreased in rats of group M+Ca; the differences were statistically significant (P<0.05). Melatonin and calcium carbonate can significantly improve antioxidative ability in rats with osteoporosis, increase bone density, elevate serum calcium level and reduce bone mineral loss, thus preventing and treating osteoporosis, and the combination displays more remarkable effects.

Evaluation of femoral perfusion using dynamic contrast-enhanced MRI after simultaneous initiation of electrical stimulation and steroid treatment in an osteonecrosis model

ABSTRACTThis study aimed to evaluate femoral perfusion using dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) for two weeks after the simultaneous initiation of electrical stimulation (ES) and steroid treatment in a steroid-induced osteonecrosis (ON) model. A single dose of methylprednisolone was injected into 14 rabbits. Seven rabbits underwent ES (ES group), and seven rabbits did not (control group). DCE-MRI was performed before steroid administration and 1, 5, 10, and 14 days after steroid administration. Regions of interest were set in the bilateral proximal femora. The enhancement ratio, initial slope, and area under the curve were analyzed. These parameters were evaluated after steroid administration in each group and between the two groups, and the ratios of ON in both groups were compared. In the control group, the minimum values of all parameters decreased significantly after steroid administration (P < 0.05), but in the ES group, the parameters did not decrease. In the ES group, all parameter values were significantly increased on the 10th and 14th days (P < 0.05). All parameter values in the ES group were significantly higher than those in the control group on the 14th day (P < 0.05). In the control group, ON was detected in three of five rabbits (in three of ten femora). In the ES group, ON was not detected. These results suggest that increased femoral blood flow elicited by ES may be related to ON prevention after steroid administration.

Systematic evaluation of repairing femoral defects by biomechanical measurements in rats

Objective To systematically evaluate the biomechanical recovery of drilled holes in the femur in SD rats. Methods Eighteen female SD rats were randomized into 3 even groups (n=6). Models of 2-mm drilled holes in bilateral femurs were established in groups A and B with 2 holes on each side while no drilling was performed in group C. Samples were harvested in group A at postoperative 4 weeks, in group B at postoperative 8 weeks while at both 4 and 8 weeks in group C. The samples were evaluated in terms of linear elasticity (compression test), viscoelasticity (relaxation and creep tests) and durability (fatigue failure test). Micro-CT scan was performed to measure the bone volume fraction (BV/TV) and bone mineral density (BMD) of new bone. Sirus red staining was performed to measure regeneration of type Ⅰ collagen of new bone. Results The elasticity modulus, maximum load, compression strength and conditional yield limit in groups A were significantly lower than those in group B which were also significantly lower than those in group C (P<0.05). At 7,200 s, the relaxation (14.56±0.69 MPa) and creep variation (11.37%±0.70%) in group A were significantly higher than those in group B (11.06±0.63 MPa and 8.98%±0.40%) which were also significantly higher than those in group C (6.99±0.56 MPa and 5.10%±0.23%) (P<0.05). At the constant amplitude loads from 20 N to 200 N, from 20 N to 300 N and from 20 N to 400 N, the recycling numbers in group A (6,044.3±879.7, 4, 093.3±628.5 and 1,919.3±847.5) were significantly lower than those in group B (10,192.3±1,109.1, 6,750.6±818.0 and 3,376.6±671.3) which were also significantly lower than those in group C (28,068.3±2,702.6, 11,788.3±1,141.6 and 5,296.3±735.0) (P<0.05). By micro-CT scan, the BVT and BMD in group A were significantly lower than those in group B which were also significantly lower than those in group C (P<0.05). The sirus red staining showed the type Ⅰ collagen in the bone defect area was completely regenerated in group B. Conclusion Systematic biomechanical measurements may actually detect the characteristics of biomechanical recovery of bone holes in SD rats, enriching the basic research on the bone damage repairing progress. Key words: Femur; Bone defects; Biomechanics; Rats

Do we need to perform bilateral hip bone mineral density examination?

Background: Bone mineral density (BMD) measurement is one of the method for making osteoporosis diagnosis. World Health Organization (WHO) recommends the measurement of BMD conducted at antero-posterior lumbar vertebrae, unilateral hip (femur), and radius. However, there was a concern about osteoporosis under diagnosis if the measurement is only conducted at unilateral hip. Some studies found significant differences of BMD between both femur and evidence of the importance to examine both femur in making the diagnosis of osteoporosis. This study aims to determine anydifference between right and left femur BMD measurement and to investigate the bone status result with measurement of BMD of bilateral femur in Hasan Sadikin General Hospital. Methods: A retrospective study was conducted from June to November 2015. Patients who received dual-femur BMD testing using General Electrics (GE) Lunar Prodigy dual-energy x-ray absorptiometry at the DXA Facility in Hasan Sadikin General Hospital,Bandung between January 1, 2006 to December 31, 2014 were included. Statistical analysis performed to assess the difference and the correlation between theBMD of two femurs (g/cm2). T-scores of the subject were implemented into bone status according to WHO Diagnostic Criteria for Osteoporosis.Results: From sixty-one patients included in this study, there were difference bone status resulted from BMD of the femoral neck, Ward’s triangle, trochanter, andtotal hip area between right and left femur, although no statistically significance were found. There was a positive correlation between BMD of right and left femurat all areas of femur. There were 16 subjects (26.1%) showed combination level of bone status (normal, osteopenia, or osteoporosis in one femur).Conclusion: BMD results in each area of the right and left femurs are different.Therefore, performing bilateral hip BMD examination as a routine measurement for makingdiagnosis of osteoporosis is important.Keywords: Bone Mineral Density, Dual-energy X-ray Absorptiometry, Femur, Osteoporosis

Steroid-associated osteonecrosis animal model in rats

SummaryObjectiveEstablished preclinical disease models are essential for not only studying aetiology and/or pathophysiology of the relevant diseases but more importantly also for testing prevention and/or treatment concept(s). The present study proposed and established a detailed induction and assessment protocol for a unique and cost-effective preclinical steroid-associated osteonecrosis (SAON) in rats with pulsed injections of lipopolysaccharide (LPS) and methylprednisolone (MPS).MethodsSixteen 24-week-old male Sprague–Dawley rats were used to induce SAON by one intravenous injection of LPS (0.2 mg/kg) and three intraperitoneal injections of MPS (100 mg/kg) with a time interval of 24 hour, and then, MPS (40 mg/kg) was intraperitoneally injected three times a week from week 2 until sacrifice. Additional 12 rats were used as normal controls. Two and six weeks after induction, animals were scanned by metabolic dual energy X-ray absorptiometry for evaluation of tissue composition; serum was collected for bone turnover markers, Microfil perfusion was performed for angiography, the liver was collected for histopathology and bilateral femora and bilateral tibiae were collected for histological examination.ResultsThree rats died after LPS injection, i.e., with 15.8% (3/19) mortality. Histological evaluation showed 100% incidence of SAON at week 2. Dual energy X-ray absorptiometry showed significantly higher fat percent and lower lean mass in SAON group at week 6. Micro-computed tomography (Micro-CT) showed significant bone degradation at proximal tibia 6 weeks after SAON induction. Angiography illustrated significantly less blood vessels in the proximal tibia and significantly more leakage particles in the distal tibia 2 weeks after SAON induction. Serum amino-terminal propeptide of type I collagen and osteocalcin were significantly lower at both 2 and 6 weeks after SAON induction, and serum carboxy-terminal telopeptide was significantly lower at 6 weeks after SAON induction. Histomorphometry revealed significantly lower osteoblast surface and higher marrow fat fraction and oedema area in SAON group. Hepatic oedema appeared 2 weeks after SAON induction, and lipid accumulation appeared in the liver of SAON rats 6 weeks after SAON induction.ConclusionThe present study successfully induced SAON in rats with pulsed injection of LPS and MPS, which was well simulating the clinical feature and pathology. Apart from available large animal models, such as bipedal emus or quadrupedal rabbits, our current SAON small model in rats could be a cost-effective preclinical experimental model to study body metabolism, molecular mechanism of SAON and potential drugs developed for prevention or treatment of SAON.The translational potential of this articleThe present study successfully induced SAON in a small animal model in rats with pulsed injection of LPS and MPS. The evaluation protocols with typical histopathologic ON features and advanced evaluation approaches to identify the metabolic disorders of SAON could be used in future rat SAON studies. The SAON rat model is a suitable and cost-effective animal model to study molecular mechanism of SAON and potential drugs developed for prevention and treatment of SAON.

Is Contralateral Templating Reliable for Establishing Rotational Alignment During Intramedullary Stabilization of Femoral Shaft Fractures? A Study of Individual Bilateral Differences in Femoral Version.

To determine native individual bilateral differences (IBDs) in femoral version in a diverse population. Computed tomography scans with complete imaging of uninjured bilateral femora were used to determine femoral version and IBDs in version. Age, sex, and ethnicity of each subject were also collected. Femoral version and IBDs in version were correlated with demographic variables using univariate and multivariate regression models. One hundred sixty-four subjects were included in the study. The average femoral version was 9.4 degrees (±9.4 degrees). The mean IBD in femoral version was 5.4 degrees (±4.4 degrees, P < 0.001). A total of 17.7% of subjects had a difference in version ≥10 degrees, and 4.3% had a difference in version ≥15 degrees. A femur with anteversion ≥20 degrees or retroversion was associated with a greater mean difference in version from the contralateral side compared with those with midrange anteversion. Bilateral differences in femoral version are common and can result in a difference from native anatomy that may be clinically significant if only the contralateral limb is used to establish rotational alignment during intramedullary stabilization of diaphyseal femur fractures. This is also an important consideration when considering malrotation of femur fractures because most studies define malrotation as a greater than 10-15-degree difference compared with the contralateral side. Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Severe Striae and Steroid Acne as Side Effects of Long-term Systemic Corticosteroid Treatment: A Case Report and Review of The Literature

Background: Systemic corticosteroids are often used in dermatology. The anti-inflammatory and immunosuppressive properties of corticosteroids make this agent as the first-line option in many disorders such as autoimmune and bullous diseases. However, there are also potential side effects and dermatologists who prescribe corticosteroids have to be aware of them. Case Summary: We report a 16-year-old girl with a history of taking oral methylprednisolone in the past 8 months who came with a acne-like eruption on the face, chest, and extremities as well as lines on the skin. Physical examination revealed monomorphic papules on the facial, thorax, and superior and inferior extremities region. Comedones were absent. Striae were found in the bilateral femur, abdomen, and mammary region. The patient was diagnosed with acneiform eruption and striae distensae rubra. Treatment using a combination of topical clindamycin and tretinoin showed clinical improvement of the acneiform eruption after three months of therapy. However, striae did not show improvement despite topical application of tretinoin and Centella asiatica extract. Conclusion: This case demonstrates the importance of understanding the cutaneous side effects that may result from chronic systemic corticosteroids administration as well as the evidence-based management.

Multifocal Osseous Epithelioid Hemangioendothelioma Involving Extremities and Spine with Visceral Metastasis

AbstractEpithelioid hemangioendothelioma (EH) is a rare locally aggressive intermediate grade malignant vasoformative tumor. Bone is a rarer primary site accounting for less than 1% of all malignant bone tumors. Multicentricity and/or multifocality are a relatively frequent occurrence, most commonly clustered in the bones of lower extremities. The axial skeleton is less commonly affected, and concomitant visceral involvement is rarer. We report a unique case of a 20-year-old male who presented with multiple lytic lesions in the skull vault, vertebral bones, bilateral proximal femur, and both radii and humerii with metastasis to the lungs and liver. A final diagnosis of EH was established on biopsy from the right iliac bone. He initially received multiagent chemotherapy and is currently on antiangiogenic therapy. On follow-up positron emission tomography–computerized tomography done 6 months later, marked reduction in fluoro-D-glucose avidity of the hepatic and most skeletal lesions was noted.

Ambulance Nurses' Competence and Perception of Competence in Prehospital Trauma Care.

Introduction We focus on trauma care conducted in the context of a simulated traumatic event. This is in this study defined as a four-meter fall onto a hard surface, resulting in severe injuries to extremities in the form of bilateral open femur fractures, an open tibia fracture, and a closed pelvic fracture, all fractures bleeding extensively. Methods The simulated trauma care competence of 63 ambulance nurses in prehospital emergency care was quantitatively evaluated along with their perception of their sufficiency. Data was collected by means of simulated trauma care and a questionnaire. Results Life-saving interventions were not consistently performed. Time to perform interventions could be considered long due to the life-threatening situation. In comparison, the ambulance nurses' perception of the sufficiency of their theoretical and practical knowledge and skills for trauma care scored high. In contrast, the perception of having sufficient ethical training for trauma care scored low. Discussion This study suggests there is no guarantee that the ambulance nurses' perception of theoretical and practical knowledge and skill level corresponds with their performed knowledge and skill. The ambulance nurses rated themselves having sufficient theoretical and practical knowledge and skills while the score of trauma care can be considered quite low.

Hybrid fixation by retrograde intramedullary nail and locking plate for bilateral periprosthetic supracondylar femur fractures following total knee arthroplasty: A case report

Hybrid fixation by retrograde intramedullary nail and locking plate for bilateral periprosthetic supracondylar femur fractures following total knee arthroplasty: A case report

Effects of combined human parathyroid hormone (1-34) and menaquinone-4 treatment on the interface of hydroxyapatite-coated titanium implants in the femur of osteoporotic rats.

The objective of this study was to investigate the effects of human parathyroid hormone (1-34) (PTH1-34; PTH) plus menaquinone-4 (vitamin K2; MK) on the osseous integration of hydroxyapatite (HA)-coated implants in osteoporotic rats. Ovariectomized female Sprague-Dawley rats were used for the study. Twelve weeks after bilateral ovariectomy, HA-coated titanium implants were inserted bilaterally in the femoral medullary canal of the remaining 40 ovariectomized rats. All animals were then randomly assigned to four groups: Control, MK, PTH and PTH+MK. The rats from groups MK, PTH and PTH+MK received vitamin K2 (30mg/kg/day), PTH1-34 (60μg/kg, three times a week), or both for 12weeks. Thereafter, serum levels of γ-carboxylated osteocalcin (Gla-OC) were quantitated by ELISA and the bilateral femurs of rats were harvested for evaluation. The combination of PTH and MK clearly increased the serum levels of Gla-OC (a specific marker for bone formation) compared to PTH or MK alone. The results of our study indicated that all treated groups had increased new bone formation around the surface of implants and increased push-out force compared to Control. In addition, PTH+MK treatment showed the strongest effects in histological, micro-computed tomography and biomechanical tests. In summary, our results confirm that treatment with PTH1-34 and MK together may have a therapeutic advantage over PTH or MK monotherapy on bone healing around HA-coated implants in osteoporotic rats.

Yenidoğanda Uzun Kemik Kırıklarının Retrospektif Değerlendirilmesi: Tek Merkez Deneyimi

Objective: Long bone fractures are rare in the newborn period. In this study, we aimed to determine the clinical characteristics and the incidence of long bone fractures in neonates. Material and Methods: A retrospective chart review was performed on newborns who were diagnosed with long bone fractures from 2012 to 2016. Results: Fifteen neonates with long bone fractures were identified. There existed eight cases with humerus fracture, four cases with femur fracture, two cases with bilateral femur fracture and one case with intrauterine femur and concomitant postnatal humerus fracture. The incidence of long bone fracture was identified as 0.19 in 1000 live births in general and 0.11 in 1000 live births for humerus fracture and 0.07 in 1000 live births for femur fracture. The probable risk factors were identified as birth trauma in eight cases (53%) and metabolic-genetic diseases in seven cases. Conclusion: Long bone fractures are one of the complications of delivery and can be attributed to maternal and/or fetal causes or maneuvers during delivery. Although long bone fractures are often associated with assisted delivery, it must be kept in mind that caesarian section does not exclude the risk in the presence of co-existing neonatal diseases.

Bilateral Atypical Femur Fracture after Prolonged Use of Alendronate - A Case Report

Bilateral Atypical Femur Fracture after Prolonged Use of Alendronate - A Case Report

Subtrochanteric fractures treated by cerclage cables and long cephalomedullary nails with minimally invasive technique

Objective To explore the surgical methods and evaluate the postoperative outcomes after cerclage cables and long cephalomedullary nails fixation with minimally invasive technique for the treatment of subtrochanteric femoral fractures. Methods From January 2013 to February 2016, 21 patients with subtrochanteric femoral fractures surgically treated were retrospectively analyzed. There were 14 males and 7 females with an average age of 43.5 years old (ranged from 24 to 71 years old). 11 patients suffered by traffic accidents, 8 patients fell down from heights and 2 patients injured by collapses of heavy objectives. Subtrochanteric fractures were classified by Seinsheimer classification with 2 cases of type ⅡB, 5 cases of type ⅡC, 7 cases of ⅢA, 2 cases of ⅢB, 1 case of Ⅳ and 4 cases of Ⅴ. CT scans of bilateral femurs were performed to measure the length, canal diameter and anterior ache before the operation. All the fractures were treated by cerclage cables and long cephalomedullary nails with minimal invasive technique. 7 cases were fixed by one cable and 14 cases by two or more cables. Results All the patients were followed up with an average of 22.5 months (ranged from 12 to 36 months). The healing time of 20 patients was 3-6 months, with an average time of 4.1 months, except 1 was union by re-operation and the healing time was 13 months after initial operation. According to Harris function evaluation, the average score was 85.4 (ranged from 68 to 97). The clinical outcomes were rated with excellent in 13 cases, good in 6 cases, fair in 1 case and poor in 1 case. The excellent and good rate was 90.5%. Complications included 1 case of screw cut-out and 1 case of nonunion. Conclusion For the treatment of subtrochanteric fractures with spiral, oblique pattern or a big butterfly fragment, satisfactory outcome could be achieved by fixation of cerclage cables and long cephalomedullary nails. The key to healing is to protect the blood supply of the fracture fragments with minimally invasive technique. Key words: Surgical procedures, minimally invasive; Fracture fixation, intramedullary; Hip fractures

Complete mimicry: a case of alveolar rhabdomyosarcoma masquerading as acute leukemia

BackgroundA small number of rhabdomyosarcoma (RMS) cases involve the bone marrow. A leukemic presentation of RMS has been reported in a few case series, although almost all cases of leukemic RMS are not completely mimicking leukemia. We encountered a case with RMS cell infiltration of the bone marrow that resembled floating hematological cells.Case presentationWe encountered a rare case of a 15-year-old boy with a 2-week history of left femoral pain. Upon admission, he was afebrile with no other symptoms. No apparent cause of femoral pain was detected on an initial examination. Laboratory findings revealed normal white blood cell (WBC) count and hemoglobin concentration, with a platelet count of 10.3 × 104/μL. WBCs included 2.0% metamyelocytes, 4.5% myelocytes, and 0.5% blasts. Lactate dehydrogenase concentration was 1299 U/L, creatine kinase was 437 U/L, and C-reactive protein was 1.25 mg/dL. Bone marrow aspiration demonstrated hypercellular marrow (nucleated cell count 1.84 × 104/μL) and 89.0% of blast-like cells of all nucleated cells. The proliferating cells were negative for myeloperoxidase and esterase, and strongly positive for CD56. Positron emission tomography exhibited extensive accumulation of 18F–fludeoxyglucose with a SUVmax of 7.09. Magnetic resonance imaging revealed T1-low intensity, gadolinium-enhanced, diffuse, and irregular lesions on his pelvis and bilateral femurs. These laboratory and imaging findings suggested hematological malignancy with diffuse bone involvement, suggestive of acute leukemia. However, the pathological diagnosis of bone marrow and basal penile muscle biopsy was alveolar RMS. Karyotype analysis of bone marrow cells revealed the characteristic translocation of t(2;13)(q35;q14). The final diagnosis was alveolar RMS with massive involvement of the bone marrow and the primary site in the perineal muscles. The tumor cells both of the primary site and bone marrow were positive for myogenin.ConclusionsA literature review found a misdiagnosed case of completely mimicking leukemic RMS as natural-killer (NK)-cell leukemia. Such a misdiagnosis can have critical consequences. We experienced a rare case of alveolar RMS with symmetrical diffuse bone marrow involvement completely masquerading as acute leukemia. The results of a surface marker study showing that the tumor cells had a near NK-cell phenotype were misleading.

Reduction Techniques for Diaphyseal Femur Fractures.

Achieving and maintaining reduction in patients with a diaphyseal femur fracture may be difficult; therefore, thorough preoperative planning is required. To fully prepare for successful surgical management of diaphyseal femur fractures, surgeons must consider appropriate patient positioning and necessary tools, including surgical tables, traction devices, and instruments. Principles of acceptable reduction rely on the restoration of length, alignment, and rotation. Reduction of diaphyseal femur fractures should be attained in the least invasive manner, via percutaneous reduction techniques, if possible, to preserve fracture biology and promote successful fracture healing. Intraoperative assessment of reduction often requires imaging studies of the contralateral extremity as a reference. Intraoperative assessment for associated femoral neck fractures and postoperative clinical examination of the hip and knee are imperative to the successful management of diaphyseal femur fractures. Other reference modalities and clinical examinations are required in patients with bilateral diaphyseal femur fractures.

Erdheim-Chester Disease Presenting as Abdominal Pain

Erdheim-Chester Disease (ECD) is an abnormal malignant proliferation of histiocytes that can affect many systems, most commonly the bone. This is a rare disease with few reported cases and is characterized by an infiltration of foamy histiocytes. Although ECD is classified as an orphan disease, those in the field of gastroenterology should be aware of this disorder because of its ability to produce multisystem symptoms, most notably vague abdominal pain with a similar presentation as gastroesophageal reflux disease (GERD). A 63-year-old male with a past medical history of osteoarthritis, GERD, and myocardial infarction (MI) presented with upper abdominal pain for several months duration, worse with palpation. Patient had lower and upper endoscopy revealing only mild gastritis. Abdominal pain was not relieved by a 30-day trial of pantoprazole. Abdominal and pelvic CT showed abnormal thickening around the kidneys, adrenals, and descending aorta (Image 1). This radiographic image displayed a characteristic “hairy kidney” appearance associated with ECD. Further assesment included a follow up bone scan revealing active regions of uptake in the bilateral distal femurs and bilateral distal tibias (Image 1). The retroperitoneal mass was biopsied for evaluation. Histopathologic findings included lymphocytic infiltrate with foamy cytoplasm, irregular nuclei, and rare Touton type giant cells. These cells stained positive for CD68 and negative for S100 (Image 2). This was all consistent with a diagnosis of ECD. Cells were tested for a BRAF V600 E mutation, but results of this test were inconclusive. Because of the mild symptoms the patient was not started on any chemotherapy for his condition. At one year follow up, the patient developed radiating back pain and diabetes insipidus. Despite worsening symptoms, the mass burden appears to be stable. It is estimated that up to 68% of ECD cases occur with renal and retroperitoneal involvement. Therefore, it is possible for a patient with ECD to present to a gastroenterologist complaining of vague abdominal pain. In this case, the patient's abdominal pain was palpable and not relived with PPI treatment, thus warranting further investigation with CT. Catching ECD early is of particular importance because approximately 54% of ECD cases are positive for the BRAF mutation and can be treated with BRAF inhibitors such as vermurafenib.FigureFigure