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Orthopaedic manifestations of Gaucher disease

Lutsky, Kevin F; Tejwani, Nirmal C
Gaucher disease is a rare, hereditary disease caused by lack of a lysosomal enzyme. This results in the accumulation of glucocerebroside in the cells of the reticuloendothelial system, including the bone marrow. The orthopaedic manifestations of this disease are important for the orthopaedic surgeon to recognize and understand. Patients with Gaucher disease are at risk for pathologic fracture, abnormal bony remodeling and delayed healing, increased intraoperative bleeding, and infection. Osteomyelitis and avascular necrosis, two common sequelae of the disease, can present in very similar fashions and warrant careful and accurate diagnosis to ensure proper treatment. The impact of Gaucher disease on the musculoskeletal system is reviewed with emphasis on the importance of understanding these effects for the treating orthopaedic surgeon.
PMID: 17539760
ISSN: 1936-9719
CID: 73026

Fracture-dislocation of the elbow functional outcome following treatment with a standardized protocol

Egol, Kenneth A; Immerman, Igor; Paksima, Nader; Tejwani, Nirmal; Koval, Kenneth J
Fracture-dislocation of the elbow is a signiicant injury with mixed outcomes. The purpose of the study was to evaluate patient perceived outcome following surgical stabilization of these complex injuries. Twenty-nine available patients (76%) from 37 identiied with 'terrible triad' injury patterns, in- cluding ulnohumeral dislocation, radial head fracture, and coronoid fracture, were available for a minimum 1-year follow-up (mean, 27 months). All patients were evaluated by their treating physician. Radiographic outcome was evaluated at latest follow-up. Functional outcome was based upon DASH, Mayo elbow performance, and Broberg-Mor- rey scores. Complications were recorded. Results included that the average lexion-extension arc of elbow motion was 109 degrees +/- 27 degrees , and the average pronation-supination arc was 128 degrees +/- 44 degrees . Grip strength averaged 72% of the contralateral extremity. The Mayo score was a mean of 81 (range, 45 to 100), the Broberg-Morrey mean was 77 (range, 33 to 100) The mean DASH was 28 (range, 0 to 72). When compared to the age-based normal values, the mean patient's DASH score was 1.4 SD worse than an average person of the same age None of the injury characteristics, patient demographics or treatment modalities was signiicantly associated with a poor outcome at the 95% conidence interval. Conclusions are that the results with terrible triad injuries are often unsatisfactory, but surgical management with the use of a systematic approach may be beneicial. Our approach led to the restoration of elbow joint stability in all patients
PMID: 18081545
ISSN: 1936-9719
CID: 76146

Biomechanics of external fixation: a review of the literature

Moss, David P; Tejwani, Nirmal C
External fixation for the purpose of bony realignment has been in practice since the early 1900s and is widely used today. External fixators are primarily used for trauma but may also be used for deformity correction and arthrodesis, among other applications. The advantages of external fixation over open reduction and internal fixation and intramedullary nailing include simplicity of application, adjustability of the construct, and increased access for wound care and wound monitoring after fixation is achieved. Frame design requires a combination of pins, wires, clamps, rings, and rods to ultimately form a stable construct that can apply compressive, distractive, or neutral forces on bone
PMID: 18081548
ISSN: 1936-9719
CID: 76148

Tourniquet Cuff Pressure: The Gulf Between Science and Practice

Tejwani, Nirmal C; Immerman, Igor; Achan, Pramod; Egol, Kenneth A; McLaurin, Toni
Tourniquet use is effective in producing a bloodless field. It is recommended that the least effective pressures be used to minimize tissue microstructure and biochemical damage from tourniquet application. When applied at the thigh, the minimum effective tourniquet pressure is 90 to 100 mm Hg above systolic BP, and in a normotensive, nonobese patient, pressure of 250 mm Hg is sufficient. Similarly, an arm tourniquet pressure of 200 mm Hg is recommended. The purpose of this survey was to assess the tourniquet pressures used by orthopaedic surgeons, both academic and community based, and their familiarity with associated literature. MATERIALS:: A Website-based survey was distributed to a random sample of academic and community-based surgeons. Respondents were asked the upper and lower-extremity tourniquet pressures they routinely use. They were asked if they were able to cite or were aware of literature to support their answer. They were also asked to specify their practice setting and years in practice. Results were statistically analyzed utilizing Fisher's exact test and Mann-Whitney test. RESULTS:: A total of 199 survey responses were collected. Out of these, 151 (76%) were complete for the lower extremity, and 141 (71%) were complete for upper extremity. The average years in practice were 12.6 years (range, 1-30). The median LE pressure was 300 mm (range, 150-400), and the median UE pressure was 250 mm (range, 150-300). Less than 20% of respondents routinely used pressures of 250 mm or less for the lower extremity. For upper extremity, only 11.3% used pressures at or below 200 mm. Surgeons in academic practice were more likely to use lower tourniquet pressures, and less likely to choose 'don't know' as the option for literature support, but the difference was not statistically significant. Although 60% of respondents thought that they were aware of literature supporting their answers, only 25% of these for the lower extremity and 11% for the upper used the correct pressures. CONCLUSION:: This survey demonstrates the existing gulf between tourniquet use and supporting literature. Tourniquet use is not benign and the correct pressure usage allows the least morbidity. We hope this survey will raise awareness of the correct tourniquet pressures and change practice patterns based on 'that's how we have always done it'
PMID: 16983305
ISSN: 0022-5282
CID: 69350

Helical blade versus sliding hip screw for treatment of unstable intertrochanteric hip fractures: a biomechanical evaluation

Strauss, Eric; Frank, Joshua; Lee, Jason; Kummer, Frederick J; Tejwani, Nirmal
OBJECTIVE: To compare the fixation stability in the femoral head with sliding hip screw versus helical blade designs for unstable, intertrochanteric hip fractures. METHODS: A simulated, unstable intertrochanteric hip fracture was created in six pairs of cadaveric femurs. One of each pair was treated using an intramedullary nail with a sliding hip screw (ITST) for femoral head fixation and the other was treated with a nail with a helical blade (TFN). Each specimen was cyclically loaded with 750N vertical loads applied for 10, 100, 1000 and 10,000 cycles. Measurements for femoral head displacement, fracture fragment opening and sliding were made. Specimens were then loaded to failure. RESULTS: There was significantly more permanent inferior femoral head displacement in the ITST samples compared to the TFN samples after each cyclic loading (all p values<0.05). There was significantly more permanent fracture site opening and inferior displacement in the ITST group compared with the TFN group at 1000 and 10,000 cycles (p<0.05). Final loads to failure were not significantly different (p=0.51) between the two treatment groups. Nine specimens demonstrated fracture extension into the anteromedial cortex and subtrochanteric region and three specimens, which had an ITST implant, demonstrated a splitting fracture of the femoral head. CONCLUSION: This study demonstrated that fixation of the femoral head with a helical blade was biomechanically superior to fixation with a standard sliding hip screw in a cadaveric, unstable intertrochanteric hip fracture model
PMID: 16934256
ISSN: 0020-1383
CID: 73944

Hoffa fragment associated with a femoral shaft fracture. A case report [Case Report]

Miyamoto, Ryan; Fornari, Eric; Tejwani, Nirmal C
PMID: 17015607
ISSN: 0021-9355
CID: 91342

Lisfranc joint injuries: diagnosis and treatment

Hunt, Stephen A; Ropiak, Christopher; Tejwani, Nirmal C
Injuries to the tarsometatarsal or Lisfranc joint, though rare, are often undiagnosed or inadequately treated, resulting in poor long-term outcomes. Clinical and radiographic data are needed to recognize and treat these injuries for optimal outcomes. In this article, we review the anatomy, biomechanics, injury mechanisms, clinical presentation, radiographic evaluation, injury classification, treatment, outcome, and complications of Lisfranc joint injuries
PMID: 16983869
ISSN: 1078-4519
CID: 69593

Preoperative assessment of tibial nail length: accuracy using digital radiography

France, Monet A; Koval, Kenneth J; Hiebert, Rudi; Tejwani, Nirmal; McLaurin, Toni M; Egol, Kenneth A
This study was performed to determine if picture archiving communication systems can provide a more accurate method of determining implant length for intramedullary tibial nailing. Postoperative radiographs of 40 patients who underwent intramedullary nailing of their tibial shaft fractures using picture archiving communication systems were retrieved. In phase one and two of this investigation, tibial nail lengths were measured using 'measuring distance' and 'measure calibration' tools displayed on the respective digital systems. Phase 3 of this study involved 5 tibial Sawbones (Pacific Research Laboratories, Vashon, Wash) radiographically captured on the picture archiving communication systems with a radiograph marker of known length. Using the 'measuring distance' and 'measure calibration' tools in phases one and two did not result in accurate measurements. Of 40 digital radiographic images measured and calibrated with the on-screen ruler and using the digital system tools, 100% of our measurements were inaccurate. An average of 19.4-mm and 10.6-mm difference was noted in uncorrected measurements on anteroposterior (AP) and lateral views, respectively. An average 25.8 mm and 15.7 mm was noted in calibrated (corrected) measurements on AP and lateral views respectively. Digitally measured and calibrated lengths were an average 22 mm and 25 mm greater from the actual known length of the tibial nail, respectively. Phase 3 of our study presented the most accurate results in length determination of tibial nail length
PMID: 16866094
ISSN: 0147-7447
CID: 69354

Predictors of short-term functional outcome following ankle fracture surgery

Egol, Kenneth A; Tejwani, Nirmal C; Walsh, Michael G; Capla, Edward L; Koval, Kenneth J
BACKGROUND: Ankle fractures are among the most common injuries treated by orthopaedic surgeons. However, very few investigators have examined the functional recovery following ankle fracture surgery and, to our knowledge, none have analyzed factors that may predict functional recovery. In this study, we evaluated predictors of short-term functional outcome following surgical stabilization of ankle fractures. METHODS: Over three years, 232 patients who sustained a fracture of the ankle and were treated surgically were followed prospectively, for a minimum of one year. Trained interviewers recorded baseline characteristics, including patient demographics, medical comorbidities, and functional status according to the Short Musculoskeletal Function Assessment (SMFA). Laboratory findings, the American Society of Anesthesiologists (ASA) class, and operative findings were recorded from the chart during hospitalization. Follow-up information included the occurrence of complications or additional surgery, weight-bearing status, functional status according to the SMFA, and the American Orthopaedic Foot and Ankle Society (AOFAS) ankle-hindfoot score. The data were analyzed to determine predictors of functional recovery at three months, six months, and one year postoperatively. RESULTS: Complete follow-up data were available for 198 patients (85%). At one year, 174 (88%) of the patients had either no or mild ankle pain and 178 (90%) had either no limitations or limitations only in recreational activities. According to the AOFAS ankle-hindfoot score, 178 (90%) of the patients had > or = 90% functional recovery. A patient age of less than forty years was a predictor of recovery, as measured with the SMFA subscores, at six months after the ankle fracture. At one year, however, age was no longer a predictor of recovery. Patients who were younger than forty were more likely to recover > or = 90% of function (p = 0.004), and men were more likely than women to recover function (p = 0.02). ASA Class 1 or 2 (p = 0.03) and an absence of diabetes (p = 0.02) were also predictors of better functional recovery at one year. SMFA subscores were below average at baseline, indicating a healthy population. At three and six months postoperatively, all SMFA subscores were significantly higher than the baseline subscores (p < 0.001); however, at one year, the SMFA subscores were almost back to the baseline, normal level. CONCLUSIONS: One year after ankle fracture surgery, patients are generally doing well, with most experiencing little or mild pain and few restrictions in functional activities. They have a significant improvement in function compared with six months after the surgery. Younger age, male sex, absence of diabetes, and a lower ASA class are predictive of functional recovery at one year following ankle fracture surgery. It is important to counsel patients and their families regarding the expected functional recovery after an ankle injury
PMID: 16651571
ISSN: 0021-9355
CID: 64466

Renal osteodystrophy

Tejwani, Nirmal C; Schachter, Aaron K; Immerman, Igor; Achan, Pramod
The incidence of chronic renal disease is increasing, and the pattern of renal osteodystrophy seems to be shifting from the classic hyperparathyroid presentation to one of low bone turnover. Patients with persistent disease also live longer than previously and are more physically active. Thus, patients may experience trauma as a direct result of increased physical activity in a setting of weakened pathologic bone. Patient quality of life is primarily limited by musculoskeletal problems, such as bone pain, muscle weakness, growth retardation, and skeletal deformity. Chronic renal disease also increases the risk of comorbidity, such as infection, bleeding, and anesthesia-related problems. Current treatment strategies include dietary changes, plate-and-screw fixation, and open reduction and internal fixation
PMID: 16675624
ISSN: 1067-151x
CID: 68748