Bone Infarction Research Articles

Feasibility of MRI in Early Diagnosis of Musculoskeletal Complications of Sickle Cell Disease in Pediatrics

AbstractBackground: Sickle cell disease (SCD) include a group of genetic abnormalities in which there is inheritance of Hb S (sickle hemoglobin) from both parents, or Hb S from one parent and a gene for beta-thalassemia or an abnormal hemo-globin from the other parent. The most common and the most severe form is sickle cell anemia.Aim of Study: To evaluate the feasibility of MRI in early detection of musculoskeletal complications of pediatric sickle cell disease (SCD) aiming to discriminate osteomyelitis from infarction.Patients and Methods: This prospective study included 30 patients (21 boys, 9 girls) with a mean age of 5 years proved to have SCD and suspected to have osteomyelitis. All patients underwent X-ray and different sequences MRI.Results: Persistence of red marrow and multiple bone infarctions were the most common MRI findings followed by osteomyelitis in 8, chondritis in 7, myositis in 4, septic arthritis in 3, subperiosteal and soft tissue abscess each in 2. There was difficulty in discriminating infarction from osteomyelitisConclusion: MRI is helpful in early identification of musculoskeletal abnormalities in SCD in pediatrics. Early detection is crucial to initiate treatment to avoid the compli-cation.

Osteopenia and Osteoporosis Assessed by Dual X Absorptiometry in Sickle Cell Anemia Adults Subjects

Background: Sickle cell anemia is the most common genetic disease in sub-Saharan Africa. It is an inherited autosomal recessive disorder characterized by chronic hemolysis secondary to falciformation of red blood cells, also responsible of ischemia, bone infarction and accompanied by serious infections and organic lesions.Normal for weight at birth, Sickle cell anemia subjects have low pre puberty growth compared to normal children and also have compromised bone remodeling balance which results in decrease of bone mass and increase of bone fragility. Several studies have established that 37% to 50% of SCA patients were osteopenic or osteoporotic. This study aims to confirm the existence of bone remodeling disorders with osteoporotic translation and to compare the values found in Congolese sickle cell adults subjects to the general population.Methods: Spine and hip DXA were conducted on 270 SS homozygotes aged 18 to 50 years (121 men and 149 women) and 359 AA homozygotes as controls (138 men and 221 women), aged from 18 to 50 years old, who agreed to participate in the study, considered as a control group. AS heterozygotes were not included in the study.Results: AA subjects shows higher density (BMD) and Bone mineral content (BMC) values. Both SCA and AA controls showed the characteristic curve with peak bone mass at the fourth decade of life, followed by a decay with age. The difference in BMD and BMC with the control population ranged from 7.94% to 26.34% (average of 16.02%) which means -0.8 to -2.7 standard deviations, whereas, compared to the T -score in the Congolese population, was 11.6% to 22.15% less (average of -17.5%) equivalent of -0.9 to -2 standard deviations.The overall decrease in bone mass rate for -2.5 DS of the T-score was: -28.4% and 33.2% for -2 DS of T-score.Conclusion: SCA subjects shows high rate of osteopenia and osteoporosis and are more likely at risk for fractures.

Positron emission tomography/computed tomography imaging appearance of benign and classic "do not touch" osseous lesions.

BACKGROUNDClassic “do not touch” and benign osseous lesions are sometimes detected on 18-F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) studies. These lesions are often referred for biopsy because the physician interpreting the PET/CT may not be familiar with the spectrum of 18F-FDG uptake patterns that these lesions display.AIMTo show that “do not touch” and benign osseous lesions can have increased 18F-FDG uptake above blood-pool on PET/CT; therefore, the CT appearance of these lesions should dictate management rather than the standardized uptake values (SUV).METHODSThis retrospective study evaluated 287 independent patients with 287 classic “do not touch” (benign cystic lesions, insufficiency fractures, bone islands, bone infarcts) or benign osseous lesions (hemangiomas, enchondromas, osteochondromas, fibrous dysplasia, Paget’s disease, osteomyelitis) who underwent 18F-FDG positron emission tomography/computed tomography (PET/CT) at a tertiary academic healthcare institution between 01/01/2006 and 12/1/2018. The maximum and mean SUV, and the ratio of the maximum SUV to mean blood pool were calculated. Pearson’s correlations between lesion size and maximum SUV were calculated.RESULTSThe ranges of the maximum SUV were as follows: For hemangiomas (0.95-2.99), bone infarcts (0.37-3.44), bone islands (0.26-3.29), enchondromas (0.46-2.69), fibrous dysplasia (0.78-18.63), osteochondromas (1.11-2.56), Paget’s disease of bone (0.93-5.65), insufficiency fractures (1.06-12.97) and for osteomyelitis (2.57-12.64). The range of the maximum SUV was lowest for osteochondromas (maximum SUV 2.56) and was highest for fibrous dysplasia (maximum SUV of 18.63). There was at least one lesion that demonstrated greater 18F-FDG avidity than the blood pool amongst each lesion type, with the highest maximum SUV ranging from 9.34 times blood pool mean (osteomyelitis) to 1.42 times blood pool mean (hemangiomas). There was no correlation between the maximum SUV and the lesion size except for enchondromas. Larger enchondromas had higher maximum SUV (r = 0.36, P = 0.02).CONCLUSIONThe classic “do not touch” lesions and classic benign lesions can be 18F-FDG avid. The CT appearance of these lesions should dictate clinical management rather than the maximum SUV.

PB1857 THE ROLE OF WHOLE‐BODY MAGNETIC RESONANCE IMAGING IN THE DETECTION OF VASCULAR EVENTS IN PATIENTS WITH PAROXYSMAL NOCTURNAL HEMOGLOBINURIA.

Background:Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder characterized by intravascular hemolysis, cytopenias, and recurrent thrombosis. Thrombotic events are just the main cause of morbidity and mortality. Therefore, it is essential to detect them early to treat them and avoid sequels.Aims:To analyze the diagnostic value of the whole‐body magnetic resonance imaging (WB‐MRI) in the detection of symptomatic and silent vascular events.Methods:Retrospective study in which a 1.5 Tesla whole‐body magnetic resonance (Philips Achieva, Philips Medical Systems, Best, the Netherlands) with Gadolinium contrast was performed along 2017 and 2018 to patients diagnosed of classical PNH in Autonomous Community of the Region of Murcia, Spain. The image findings were analyzed.Four patients were included (3 men and 1 woman) with median age of 54 years, diagnosed of either PNH (n = 2) and AA/PNH (n = 2). At the time of investigation, mean size clon in granulocytes measured by FLAER was high (90.7 %, range 75.6‐99.3 %) and mean LDH was 341.7 U/L (range 204‐465 U/L; normal range 135‐214 U/L). Only one patient had prior history of thrombosis (Budd‐Chiari syndrome). All patients were treated according to Spanish PNH guidelines, including the use of complement inhibitor Eculizumab.Results:WB‐MRI findings showed unknown bone infarctions in two (50%) patients (patient 1 and patient 3). In patient 1, symptomatic bone infarctions was diagnosed (both femoral condyle and proximal tibias). Patient 3 showed an asymptomatic area of bone edema in distal left radial bone.Summary/Conclusion:Although the sample from our study is very small, it seems that WB‐MRI could be useful to evaluate vascular events, like bone infarctions, even subclinical ones. Our findings are consistent with the only article published to date (Alaskar et al. Sci Rep. 2018; 8: 13458). Further studies are needed to test the effectiveness of WB‐MRI in the detection of vascular events and their relationship as a thrombotic event in PNH.imageimage

Gaucher Disease in Bone: From Pathophysiology to Practice.

ABSTRACTGaucher disease (GD) is a rare, genetic lysosomal disorder leading to lipid accumulation and dysfunction in multiple organs. Involvement of the skeleton is one of the most prevalent aspects of GD and a major cause of pain, disability, and reduced quality of life. Uniform recommendations for contemporary evaluation and management are needed. To develop practical clinical recommendations, an international group of experienced physicians conducted a comprehensive review of 20 years’ of the literature, defining terms according to pathophysiological understanding and pointing out best practice and unmet needs related to the skeletal features of this disorder. Abnormalities of bone modeling, reduced bone density, bone infarction, and plasma cell dyscrasias accompany the displacement of healthy adipocytes in adult marrow. Exposure to excess bioactive glycosphingolipids appears to affect hematopoiesis and the balance of osteoblast and osteoclast numbers and activity. Imbalance between bone formation and breakdown induces disordered trabecular and cortical bone modeling, cortical bone thinning, fragility fractures, and osteolytic lesions. Regular assessment of bone mineral density, marrow infiltration, the axial skeleton and searching for potential malignancy are recommended. MRI is valuable for monitoring skeletal involvement: It provides semiquantitative assessment of marrow infiltration and the degree of bone infarction. When MRI is not available, monitoring of painful acute bone crises and osteonecrosis by plain X‐ray has limited value. In adult patients, we recommend DXA of the lumbar spine and left and right hips, with careful protocols designed to exclude focal disease; serial follow‐up should be done using the same standardized instrument. Skeletal health may be improved by common measures, including adequate calcium and vitamin D and management of pain and orthopedic complications. Prompt initiation of specific therapy for GD is crucial to optimizing outcomes and preventing irreversible skeletal complications. Investing in safe, clinically useful, and better predictive methods for determining bone integrity and fracture risk remains a need. © 2019 The Authors. Journal of Bone and Mineral Research Published by Wiley Periodicals Inc.

Utility of single-photon emission computed tomography and computed tomography imaging in bone infarction

Utility of single-photon emission computed tomography and computed tomography imaging in bone infarction

Role of Magnetic Resonance Imaging in the evaluation of adult non-traumatic painful hip

Aim of the Work: to assess the role of Magnetic Resonance Imaging as a non-invasive diagnostic modality in adult patients with the non-traumatic painful hip joint. Patients and Methods: This study was conducted on 60 adult patients with non-traumatic hip pain, referred from the outpatient orthopedic clinic, Ain Shams University hospitals. The patients were investigated using magnetic resonance imaging (MRI). All MR imaging examinations were conducted at the Department of Radiodiagnosis of the Ain shams University with the same 1.5-T unit (Philips Healthcare, Best, the Netherlands) with a body coil. Results: avascular necrosis is the commonest cause of bilateral hip pain (25%) of the study patients followed by osteoarthritis (11.7%), while unilateral hip pain is commonly caused by stress fractures (8.3%), isolated joint effusion (10%) and tendinopathies (3.3%). Other causes included Marrow edema with hip effusion, chronic anemic state, migratory osteoporosis, osteoid osteoma, bursitis, bone infarcts, osseous hemangioma, hemosiderosis, sacroiliitis, femoral-acetabular impingement, iliacus abscess, and bone marrow edema. Magnetic resonance imaging doesn’t only demonstrate disorders of hip joint only; it also gives an accurate assessment of other extra-articular causes of referred hip pain as degenerative disc diseases and pelvic conditions. Conclusion: Magnetic resonance is the best imaging modality to assess hip joint in non-traumatic cases. It has a great ability to diagnose disorders of bone, cartilage, ligaments, muscles and soft tissue. MRI can also detect joint effusion and bone marrow edema.

Orbital compression syndrome: a rare complication of sickle cell disease in children

Vasoocclusive crises in sickle cell disease commonly involve bone marrow of the long bones and vertebrae. Involvement of bones with less marrow space, including the bones of the orbit, is reported rarely in the literature. The present case is a 6-year-old boy, a known case of sickle cell disease, who presented with acute onset right upper and lower eyelid swelling, restriction of extraocular movement and pain. USG orbit showed a retroorbital hemorrhage compressing on the orbit, probably due to orbital bone infarction. The child was managed successfully with red blood cell transfusion and intravenous antibiotics, without steroids and surgical intervention. This report highlights the importance of maintaining a high index of suspicion in patients with known sickle cell disease presenting with pain, orbital swelling and restriction of ocular movement. Timely red blood cell transfusion only may be sufficient for uneventful recovery, without steroids or surgical intervention. Expedient diagnosis of orbital compression syndrome in children with sickle cell disease is crucial because this is a potentially sight-threatening entity.

A Comprehensive Study of Bone Manifestations in Adult Gaucher Disease Type 1 Patients in Argentina.

Gaucher disease (GD) is the most prevalent lysosomal storage disease, and bone involvement is the most disabling condition. The aim of the present study was to evaluate bone involvement in adult patients with GD, using an observational cross-sectional study. Patients were evaluated using X-rays, bone densitometry (BMD), trabecular bone score (TBS), magnetic resonance imaging (MRI), and biochemical bone markers. Thirty-two type 1GD patients were included (mean age: 40 ± 16years). Patients had received velaglucerase for 2.7 ± 1.4years; 19/32 had been treated previously with imiglucerase. Ninety-four percent of subjects met therapeutic goals for hematological parameters, and eight were splenectomized (SPX). Nineteen patients had irreversible bone lesions (IL), i.e., avascular necrosis, bone infarction, and/or vertebral fractures. MRI showed marrow infiltration in 71% of patients. Patients with IL had higher bone marrow burden than those without (p = 0.001). All SPX patients had IL, a higher prevalence of bone marrow edema (p = 0.02), and lower TBS (p = 0.03) than non-SPX patients. Only 18.7% of patients had abnormal BMD, with no correlation with fractures (FX). TBS values were < 1350 in 53% of patients and tended to be lower in those with FX (p = 0.06). Patients with P1NP in the lower quartile had lower TBS (p = 0.03) than those with P1NP in the higher quartiles. TBS correlated moderately but not significantly with P1NP (r = 0.32) and BMB (r = - 0.44). A high prevalence of IL was documented. Bone quality was more affected than BMD in fracture patients. Low bone formation, active bone marrow infiltration, and splenectomy might be implicated in IL.

Use of biodegradable materials as local antimicrobial carriers in orthopedic infections

INTRODUCTION: Orthopedic infections have been a major complication; despite numerous advances in prophylactic measures, infections still remain a major complication in orthopedic and trauma surgery. The progressive infection of the bone and the surrounding tissue results in bone infarcts with abscess and sequestrum formation. Adequate treatment can be an implant removal and a radical debridement with a long-term course of systemic antibiotic, but these prolonged therapies can result in side effects or toxicity. The primary reason for using local antibiotic delivery vehicles is its ability to achieve high local concentrations without associated systemic toxicity.MATERIALS AND METHODS: This is a prospective study conducted over 2 years. Thirty-five patients were studies in which 33 patients were managed with calcium sulfate + antibiotic, 1 was managed prophylactically with beads, and one was managed with Abgel + antibiotic as a biodegradable carrier. The aim was to evaluate the efficacy of biodegradable materials as antimicrobial carriers in localized orthopedic infection.OBSERVATIONS: A prospective study consists of 35 cases of orthopedic infections with major number of patients belonged to the age group of 20–40 years, with a mean 37.66 ± 19.9 years. One patient was included with soft tissue infection and rest all the patients had bone infections. It was observed that there is statistically significant improvement in ASEPSIS score at 0 week to ASEPSIS score at the end of 9 weeks.CONCLUSION: In the present study, the patients showed evidence of excellent control of infection and osseous repair, and further, none of the patients required follow-up. In our series, we have used calcium sulfate as a biodegradable antimicrobial carrier with appropriate antibiotics and achieved good-to-excellent results in local orthopedic infections.

Bilateral distal femoral bone infarction revealing polymyositis

Orthopedic manifestations, particularly bone infarctions, remain exceptional and unusual during polymyositis. They are classically related to systemic corticosteroids or other associated thrombotic conditions. We report an original observation of bilateral distal femoral bone infarction revealing polymyositis in a 42-year-old woman. Our observation is, to the best of our knowledge, the first to report bilateral femoral infarction as a possible manifestation of polymyositis apart from any prolonged corticosteroid therapy and any associated anti-phospholipid antibody syndrome.

Acute soft head syndrome and a mini review of bone and neurologic complications in patients with sickle cell disease

Introduction: Acute soft head syndrome is a rare complication in patients with sickle cell disease (SCD) that is characterized by progressive skull pain and swelling, it is usually related to skull bone infarction and full recovery is achieved with conservative management. This case represents a very rare association of sickle cell disease and acute soft head syndrome that necessitates to be differentiated from other bone and neurologic presentations occurring in this entity of patients. Case: We report a rare case of an 11 years old Saudi male patient with sickle cell disease who developed acute soft head syndrome that presented with severe headache. An underlying osteomyelitis of skull and lower limb bones couldn’t be excluded and the patient had full recovery after conservative treatment. Conclusion: Acute soft bone syndrome seldomly occurs in SCD, however, its presentations mimics those of bone and neurologic complications so differentiation is essential in order to set up a clear plan of management.

Orbital Compression Syndrome in a Child with Sickle Cell Anaemia: A Case Report And Review of Literature

Vaso-occlusive crises in sickle cell disease commonly involve bone marrow of the long bones and vertebrae. Involvement of bones with less marrow space, including the bones of the orbit, is reported rarely in the literature. The present case is a six year old boy, a known case of sickle cell disease, who presented with acute onset right upper and lower eyelid swelling, restriction of extraocular movement and pain. USG orbit showed a retro-orbital haemorrhage compressing on the orbit, probably due to orbital bone infarction. The child was managed successfully with red blood cell transfusion and intravenous antibiotics, without steroids and surgical intervention. This report highlights the importance of maintaining a high index of suspicion in patients with known sickle cell disease presenting with pain, orbital swelling and restriction of ocular movement. Timely red blood cell transfusion only may be sufficient for uneventful recovery, without steroids or surgical intervention. Expedient diagnosis of orbital compression syndrome in children with sickle cell disease is crucial because this is a potentially sight-threatening entity.

Relationship between Non-Prescribed Drug Use and Employment Status of Patients with Sickle Cell Disease

Introduction: Patients with sickle cell disease (SCD) suffer from recurrent episodes of acute vaso-occlusive crises that cause acute musculoskeletal pain and chronic pain due to complications such as avascular necrosis of the joints or bone infarcts. Most of these patients are on chronic opioid medications to manage their acute and chronic pain. Generally, patients with chronic pain syndrome are more prone to use illicit drugs more than the general population. In addition, patients with SCD have high rates of unemployment, which may put certain individuals at risk for negative coping behaviors such as illicit drug use. This study aim at investigating the relationship between the use of non-prescribed drug (illicit drug) and employment status of patients with SCD.Patients and Methods: This was a pilot study of SCD patients at UT Physicians Comprehensive Sickle Cell Center from January 2012 to July 2018. We administered surveys during routine clinic visits and we reviewed clinical data in the electronic medical records. We used results of comprehensive urine drug screen (UDS) to evaluate for the use of non-prescribed drugs. UDS was done, on average every 3 to 6 months, on each patient. Descriptive analysis was performed with frequency distributions. Univariate logistic regression was conducted to investigate the association between non-prescribed drug use and employment status.Results: A cohort of 78 SCD patients completed surveys about employment and non-prescribed drug use. In the survey, only ten patients (12.82%) reported using at least one non-prescribed drug. However, after the chart review for urine drug screens, twenty one out of seventy eight (26.92%) were using non-prescribed drugs. Most of the SCD patients using non-prescribed drug (71%) used Marijuana and three patients used multiple non-prescribed drugs. Seven out of twenty one (33.33%) of the non-prescribed drug users and 18 out of 57 (31.58%) of the non-drug users are employed. The univariate logistic regression analysis showed non-prescribed drug use is not statistically associated with employment status among SCD patients (P-value=0.8829).Conclusions: In this sample, we concluded that there was no obvious relationship between non-prescribed drug use and employment status of patients with SCD. In our cohort, marijuana was the most commonly used non-prescribed drug, which might be partly due to its effect on chronic pain management. Non-prescribed drug use was quite common (26.92%), but was under-reported (13%) among users. It is important to note that only 3 out of 78 patients (3.85%) used multiple non-prescribed drugs; hence, polysubstance abuse was rare in our sample. DisclosuresNo relevant conflicts of interest to declare.

Urinary Cross-Linked Carboxyterminal Telopeptide Decreases with Resolution of Painful Vaso-Occlusive Crises in Adults with Sickle Cell Disease - Results from the Sickle Cell Pain Markers (SCPM) Study

IntroductionVaso-occlusive crises (VOCs), the most common acutely painful complication of sickle cell disease (SCD), presumably cause bone infarction. In a preclinical study that exposed sickle cell mice to repeated hypoxia-reperfusion stress-to recapitulate VOCs-the mice over-expressed osteoclast-driven bone resorption markers and suppressed osteoblast-mediated bone formation markers. We hypothesize that bone infarction stems from ongoing hemolysis and inflammation incurred during VOCs, and that the extent of bone degradation can be approximated by increases in concentrations of bone resorption markers in the plasma or urine of adults with SCD. We measured urinary levels of cross-linked carboxyterminal telopeptide (CTX-1, a bone resorption marker) in 52 adults with SCD, both during and after resolution of VOCs. We now report on the association between urinary CTX-1 concentrations and serum markers of hemolysis and inflammation.MethodsIn the Sickle Cell Pain Markers (SCPM) study, adults with SCD were consecutively recruited from Tulane University Sickle Cell Center of Southern Louisiana (2008-2013). Blood and urine specimens were collected for laboratory analyses and symptoms assessed during vaso-occlusive pain crises (visit 1) and at recovery (visit 2). Self-reported symptoms were graded on a 0-10 scale (0=no symptoms, 10=worst symptoms). We restricted our analyses to subjects with complete data obtained at both visits. Three study participants were re-enrolled in SCPM, but only one subject completed data collection and was included in this analysis. We used paired t-tests and non-parametric Wilcoxon sign rank test to assess for between-visit differences in continuous variables and Fisher's exact test to monitor changes in categorical variables. Urinary CTX-1 concentrations, corrected for urine creatinine levels, were measured using enzyme-linked immunosorbent assays performed by the Research Laboratory Services, a core facility of Maine Medical Center Research Institute, Scarborough, ME. We then compared between-visit changes in urinary CTX-1 levels with changes in serum/plasma markers of hemolysis (hemoglobin, total bilirubin, lactate dehydrogenase and reticulocyte counts) and inflammation (white blood cell count, platelets, fibrinogen, C-reactive protein and erythrocyte sedimentation rates). All data were analyzed using R 3.5.1 software, and results presented in standard box plots, with slopes and 95% confidence intervals (CI).ResultsOf the 52 adults enrolled in the SCPM study (62% female, 65% hemoglobin SS/ Sb-thalassemia, 35% hemoglobin SC), we analyzed paired data from 31 subjects (60%). The average duration between visits 1 and 2 was 22 days. Mean concentrations of urinary CTX-1-corrected for urine creatinine-significantly decreased from 3.43±1.95 μg/mmol during vaso-occlusive crises to 2.62±1.34 μg/mmol at recovery (Figure). We found a non-significant positive correlation between changes in urinary CTX-1 levels and days between visits (slope 0.048, 95% CI -0.025, 0.121, p-value 0.184). The correlations between changes in urinary CTX-1 concentration and serum/plasma markers of hemolysis and inflammation were not statistically significant. Secondary analyses of all laboratory markers showed significant increases in serum levels of hemoglobin and platelets between visits 1 and 2 (p<0.05). Pain scores significantly decreased, as expected, between visits; and, shortness of breath, fatigue, nausea and sleep significantly improved with resolution of pain crises.ConclusionsVOCs accelerate bone degradation in adults with SCD. CTX-1 has low intra-individual variability and can reliably approximate the extent of bone resorption that occur during pain crises. The small sample size (n=31 pairs) likely limited our ability to detect a significant association between changes in urinary CTX-1 concentrations and changes in serum/plasma markers of hemolysis and inflammation. Additional studies are required to better understand how CTX-1 concentrations-relative to bone formation markers e.g., bone-specific alkaline phosphatase-can be used to monitor immediate and long-term deleterious effects of recurrent VOCs on the bones of adults with SCD. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Bone manifestations in neuronopathic Gaucher disease while receiving high-dose enzyme replacement therapy

Avascular necrosis (AVN), one type of bone infarction, is a major irreversible complication of Gaucher disease (GD). In this report, two pediatric patients with GD type 3, homozygous for the L483P pathogenic variant (formerly L444P), developed AVN despite treatment on long-term, high-dose enzyme replacement therapy (ERT). ERT was initiated in both patients, who had intact spleens, shortly after diagnosis with an initial dramatic response. However, both patients exhibited AVN after 5.5 and 11 years on high-dose ERT, respectively, despite good compliance and normalized hematological findings and visceral symptoms. This report demonstrates the importance of careful, regular surveillance of the musculoskeletal system in addition to monitoring the neurological symptoms associated with neuronopathic GD. Additionally, it highlights the limitations of ERT in terms of targeting certain sanctuary sites such as bone marrow and suggests the need for new treatment modalities other than ERT monotherapy to address these limitations.

Epidural and Orbital Hemorrhage Accompanying Bone Infarction with Orbital Compression Syndrome in Sickle Cell Anemia: A Case Report

Introduction: Sickle cell anemia is a autosomal recessive genetic disease, which includes microvascular occlusions and infections. Case: We present the case of epidural and orbital hemorrhage accompanying bone infarction with orbital compression syndrome in sickle cell anemia. Discussion: In patients with sickle cell anemia, bone infarctions and epidural hemorrhages should be investigated in the presence of complaints of headache and orbital compression syndrome. Magnetic resonance imaging and computed tomography can be used for diagnosis. Keywords: Sickle cell anemia, orbital compression syndrome, epidural hemorrhage, MRI, patients, genetic disease.

The Role of Whole-Body Magnetic Resonance Imaging (WB-MRI) in Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH)

In PNH thromboembolic events (TEs) represent the leading cause of morbidity and mortality. Between Dec 2013 and Jan 2016 37 PNH patients (pts) (23 PNH, 14 AA/PNH; 51% (19/37) females; median age 44 years, median D-dimer levels 0.22 mg/l) were examined with a whole-body magnetic resonance imaging (WB-MRI) scan at 1.5 T to detect TEs. Pts were treated according to German PNH guidelines, including eculizumab therapy. 64% (24/37) of the pts had no documented TEs prior to observation. Two pts had suspected TEs in their clinical history. 29% of the pts (11/37) had a known history of venous thromboses (deep venous thrombosis (DVT) (5/11), portal venous thrombosis (PVT) (4/11), vena caval thrombosis (VCT) (2/11). A myocardial infarction was reported in one pt, and two had a cerebral venous sinus thrombosis (CVST) or a thalamic infarction. Six pts (16%) had at least two prior TEs. In pts with prior TEs no progression of the existing TEs was observed. In pts on eculizumab and prior TEs as well as treatment-naïve pts silent bone and renal infarctions were detected. Furthermore, a clinically non-critical arterial occlusion was identified. WB-MRI scans present a novel, non-invasive method to assess the complete vascular status of PNH pts and allow the detection of previously undiagnosed vascular complications, affecting treatment indications and regimens.

Extensive Bone Infarctions - an Unexpected Consequence of Corticosteroid Treatment in Idiopatic Polymiositis

Secondary hemochromatosis is a condition generated by defects in the iron metabolism such as elevated intestinal iron absorption and increased iron stores in tissues and organs. The current case report presents a 35-year-old female admitted for inflammatory pain of the left ankle, second and third right MCP joints and right sternoclavicular joint associated with Celsian clinical signs, subcutaneous edema and loss of articular function. The patient is known with beta thalassemia major, secondary hemochromatosis and chronic hepatitis C as a consequence of repeated blood transfusions. We pleaded for the existence of a HC-A associated with microcrystalline calcium pyrophosphate dehydrate crystal deposition (CPPD) disease associated with secondary osteoarthritic changes.

P.095 Holey Spinal Cord - A case of spinal cord stroke secondary to fibrocartilaginous embolism

Background: Fibrocartilaginous embolism (FCE) is a rare reported cause of spinal cord infarction and likely underdiagnosed due to clinical unfamiliarity. FCE can present after a mild trauma and is characterized by back or neck pain along and a rapidly progressive myelopathy. We present a case of FCE and discuss how this clinical entity can break the typical rules of stroke. Methods: Case presentation Results: An otherwise healthy, 56-year-old professional sports coach presented a couple day history of progressive leg paresthesias and mild back pain, followed by unsteady gait and then inability to void. The left leg demonstrated mild weakness, hyperreflexivity, ataxia and an upgoing plantar response. The right leg became spastic and he then developed bilateral impairment of vibration and proprioception at the toes. An initial limited MRI lumbar spine was negative. A repeat MRI spine showed mild diffusion restriction of T10-11 and T11-12 and evidence of a bone infarct L2. He was diagnosed with a spinal cord infarct secondary to FCE. Conclusions: FCE should be considered in the differential for acute myelopathy. It can present with a progressive nature like transverse myelitis and MRI may be non-diagnostic. As more cases are being reported, FCE is becoming better defined and recognized.