Nonossifying Fibromas: A Computed Tomography-based Criteria to Predict Fracture Risk.

BACKGROUND: Nonossifying fibroma (NOF) is the most common benign osseous lesion in children; however, our understanding of which lesions progress to a fracture remains unclear. In this study, we seek to formulate a classification system for NOFs to assess for fracture risk and determine what this classification system tells us regarding fracture risk of the distal tibia and distal femur NOFs. METHODS: Charts were retrospectively reviewed for patients with NOFs. A 4-point criteria was created and used to calculate fracture risk for distal tibia and distal femur NOFs. The analysis included incidence, specificity, and sensitivity. RESULTS: One point was given for each of the following findings on computed tomography (CT) scan: (1) >50% width on coronal view; (2) >50% width on sagittal view; (3) any cortical breach; (4) lack of a neocortex. In total, 34 patients with NOFs of the distal tibia had CT scans, of which 14 fractured. Zero with a 0- or 1-point score fractured, 2 with a 2-point score fractured (20%), 4 with a 3-point score fractured (44%), and 8 with a 4-point score fractured (100%). Sensitivities of 1-, 2-, 3-, and 4-point scores were 100%, 100%, 85.7%, and 57.1%, respectively, and specificities were 71.4%, 71.4%, 80%, and 100%, respectively. A total of 41 patients with NOFs of the distal femur had CT scans, of which 5 fractured. Zero with a 0-point score fractured, 1 with a 1-point score fractured (4%), 0 with a 2-point score fractured, 1 with a 3-point score fractured (20%), and 3 with a 4-point score fractured (100%). Sensitivities of 1-, 2-, 3-, and 4-point scores were 100%, 80%, 80%, and 60%, respectively; and specificities were 60%, 87.8%, 90%, and 100%, respectively. CONCLUSIONS: Our 4-point CT criteria is easy to apply and identifies patients at high risk of fracture, helping surgeons make decisions regarding treatment. LEVEL OF EVIDENCE: Level IV-prognostic study.

Transhumeral Anterior Radial Nerve Transposition to Simplify Anticipated Future Humeral Reconstruction.

Anterior transhumeral radial nerve transposition has been described in the treatment of humeral shaft fracture to protect the nerve from implant irritation or callus entrapment. Transposing the radial nerve through a facilitating humeral defect also simplifies revision surgery should nonunion result. Here we describe a surgical technique for anterior transhumeral radial nerve transposition for complex humeral reconstruction when subsequent revision surgery is anticipated. Three cases are used for illustration: (1) revision of a total elbow arthroplasty with periprosthetic fracture; (2) revision of a total elbow arthroplasty after aseptic loosening from an allograft/prosthesis composite; and (3) septic humeral nonunion with substantial bone loss. These 3 patients underwent a total of 16 operations, 6 of which took place after the radial nerve transposition. Transhumeral radial nerve transposition allowed less worrisome dissection during revision surgery despite the multiple previous procedures. We advocate transhumeral radial nerve transposition when the duration and frequency of revisions is uncertain and the anticipated future revision may require a posterior, posterolateral, or anterolateral approach to the humerus.

Nonossifying fibromas of the distal tibia: possible etiologic relationship to the interosseous membrane.

PURPOSE: Nonossifying fibromas (NOFs) present in a characteristic pattern in the distal tibia. Their predilection to this region and etiology remain imprecisely defined. METHODS: We performed a retrospective chart review of patients between January 2003 and March 2014 for distal tibial NOFs. We then reviewed radiographs (XRs), computed tomography (CT), and magnetic resonance imaging (MRI) for specific lesion characteristics. RESULTS: We identified 48 distal tibia NOFs in 47 patients (31 male, 16 female; mean age 12.3 years, range 6.9-17.8). This was the second most common location in our population (30 % of NOFs), behind the distal femur (42 %). Thirty-four lesions had CT and nine had MRI. Thirty-one percent were diagnosed by pathologic fracture. Ninety-six percent of lesions were located characteristically in the distal lateral tibia by plain radiograph, in direct communication with the distal extent of the interosseous membrane on 33 of the 34 (97 %) lesions with CT available for review and all nine (100 %) with MRI. The remaining two lesions occurred directly posterior. CONCLUSIONS: The vast majority of distal tibial NOFs occur in a distinct anatomic location at the distal extent of the interosseous membrane, which may have etiologic implications. LEVEL OF EVIDENCE: IV (case series).

Mobile Device Trends in Orthopedic Surgery: Rapid Change and Future Implications.

Mobile devices are increasingly becoming integral communication and clinical tools. Monitoring the prevalence and utilization characteristics of surgeons and trainees is critical to understanding how these new technologies can be best used in practice. The authors conducted a prospective Internet-based survey over 7 time points from August 2010 to August 2014 at all nationwide American Council for Graduate Medical Education-accredited orthopedic programs. The survey questionnaire was designed to evaluate the use of devices and mobile applications (apps) among trainees and physicians in the clinical setting. Results were analyzed and summarized for orthopedic surgeons and trainees. During the 48-month period, there were 7 time points with 467, 622, 329, 223, 237, 111, and 134 responses. Mobile device use in the clinical setting increased across all fields and levels of training during the study period. Orthopedic trainees increased their use of Smartphone apps in the clinical setting from 60% to 84%, whereas attending use increased from 41% to 61%. During this time frame, use of Apple/Android platforms increased from 45%/13% to 85%/15%, respectively. At all time points, 70% of orthopedic surgeons believed their institution/hospital should support mobile device use. As measured over a 48-month period, mobile devices have become an ubiquitous tool in the clinical setting among orthopedic surgeons and trainees. The authors expect these trends to continue and encourage providers and trainees to be aware of the limitations and risks inherent with new technology.

The effect of intrinsic loading and reconstruction upon grip capacity and finger extension kinematics.

PURPOSE: To compare active and passive reconstructive procedures for tetraplegia and their ability to produce a powerful grip and allow appropriate finger extension in a cadaveric model. METHODS: Seventeen fresh-frozen hands were used, which included 5 in intrinsic minus and intrinsic activation conditions, 6 with Zancolli-lasso tenodeses, and 6 with modified House tenodeses to simulate intrinsic function. To test grip, flexor digitorum profundus tendons were powered with a motor. Polyvinyl chloride cylinders of diameters 43, 51, 57, 70, or 89 mm and masses 250, 400, or 550 g were used. Grip was considered successful if the cylinder was grasped and resisted gravity. Finger extension was tested by powering the extensor tendons in the same hands. RESULTS: No successful grasps were recorded in the intrinsic minus hands for larger diameter cylinders (≥ 70 mm), whereas multiple successes were seen after intrinsic activation and after Zancolli-lasso and House procedures. Whereas active intrinsic and the House reconstruction reached near full extension, this was not true for the Zancolli-lasso group. CONCLUSIONS: These data demonstrated that active and passive intrinsic reconstruction methods improved basic grasp and release kinematics in experimental cadaver hand models. Using our model and based on the more optimal kinematics and full extension of the House procedure, we suggest that this should be the preferred tenodesis-based intrinsic reconstruction method. Nevertheless, both procedures were equally successful at grasping objects of the sizes and masses studied. CLINICAL RELEVANCE: Comparative clinical studies are indicated to corroborate the findings of this cadaveric hand model.

Intrinsic hand muscle function, part 1: creating a functional grasp.

PURPOSE: Regaining hand function has been identified as the highest priority for persons with tetraplegia. In many patients, finger flexion can be restored with a tendon transfer of extensor carpi radialis longus to flexor digitorum profundus (FDP). In the absence of intrinsic function, this results in a roll-up finger movement, which tends to push large objects out of grasp. To enable patients to grasp objects of varying sizes, a functional grasp is required that has a larger excursion of fingertip-to-palm distance than can be supplied without intrinsic function. The aim of this study was to quantify the role of intrinsic muscle force in creating a functional grasp. METHODS: Finger kinematics during grasp were measured on 5 cadaveric hands. To simulate finger flexion, the FDP was activated by a motor and intrinsic muscles were loaded at various levels (0, 125, 250, 375, or 500 g). Finger movement was characterized by the order of metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joint flexion and by the maximal fingertip-to-palm distance during finger closure. RESULTS: Without any intrinsic muscle contribution (0-g load), FDP activation resulted in flexion of all 3 joints, whereby flexion began at the proximal interphalangeal joint, followed by the distal interphalangeal joint, and then the metacarpophalangeal joint. With increasing intrinsic muscle load, finger flexion was initiated at the metacarpophalangeal joint, followed by the proximal interphalangeal and distal interphalangeal joints. This altered joint flexion order resulted in a larger maximal fingertip-to-palm distance during finger flexion. The difference between the 2 extreme conditions (0 g vs 500 g of intrinsic muscle load) was 19 mm. CONCLUSIONS: These findings demonstrate that simultaneous activation of the FDP and the intrinsic muscles results in an apparently more functional hand closing compared with FDP activation alone because of altered kinematics and larger fingertip-to-palm distances. CLINICAL RELEVANCE: These findings suggest that intrinsic muscle balancing during reconstruction of grasp in tetraplegic patients may improve function.

Intrinsic hand muscle function, part 2: kinematic comparison of 2 reconstructive procedures.

PURPOSE: Reconstruction of grasp is a high priority for tetraplegic patients. Restoration of finger flexion by surgical activation of flexor digitorum profundus can result in roll-up finger flexion, interphalangeal (IP) joint before metacarpophalangeal (MCP) joint flexion, which can be improved by restoring intrinsic function. This study compares grasp kinematics between 2 intrinsic balancing procedures-Zancolli-lasso and House. METHODS: The intrinsic muscles of 12 cadaver hands were reconstructed by either the Zancolli-lasso or the House procedure (n = 6 each) and tested by deforming the flexor digitorum profundus (FDP) with a motor to simulate hand closure. Results were compared with 5 control hands. All 17 hands were studied by video analysis. Kinematics were characterized by the order of MCP joint and IP joint flexion. Optimal grasp was defined as the maximal fingertip-to-palm distance during the arc of finger closure. RESULTS: Kinematics differed between the 2 procedures. The Zancolli-lasso reconstructed hands flexed first in the IP joints, and then in MCP joints, resembling an unreconstructed intrinsic-minus hand whereas the House reconstructed hands flexed first in the MCP joints and then in the IP joints, resembling an intrinsic-activated hand. Maximal fingertip-to-palm distance did not differ significantly between the 2 procedures, and both showed improvement over unreconstructed controls. CONCLUSIONS: Both intrinsic balancing techniques improved grasp. Only the House procedure restored hand kinematics approximating those of an intrinsic-activated hand. Improvement in fingertip-to-palm distance in Zancolli-lasso hands resulted primarily from the initial resting MCP joint flexion of 40°. We therefore advocate the more physiological House procedure for restoration of intrinsic function in tetraplegic patients. CLINICAL RELEVANCE: This study provides a rationale for advocacy of 1 reconstructive procedure over another.

Multifocal micronodular pneumocyte hyperplasia: computed tomographic appearance and follow-up in tuberous sclerosis complex.

OBJECTIVE: To characterize pulmonary nodules in patients with tuberous sclerosis complex (TSC) using computed tomography. METHODS: We retrospectively reviewed chest computed tomographic images of 73 patients with TSC (22 males and 51 females; mean ± SD age, 31.5 ± 13.2 years; range, 13.8-63.5 years). RESULTS: Multiple pulmonary nodules were identified in 42 (58%) of 73 patients (mean ± SD size, 6.6 ± 3.0 mm; range, 2-14 mm). Solid nodules were present in 11 (26%) of 42 patients, ground-glass nodules were present in 3 (7%) of 42 patients, and both solid and ground-glass nodules were present in 28 (67%) of 42 patients. The presence of multiple nodules was independent of sex and lymphangioleiomyomatosis. Follow-up images were available for 22 patients with multiple nodules (mean ± SD follow-up, 2.0 ± 1.1 years; range, 0.9-4.9 years), none of whom had change in nodule size or number. CONCLUSIONS: Most men and women with TSC have multiple pulmonary nodules, which likely represent multifocal micronodular pneumocyte hyperplasia in the absence of known predisposing factors.

Efficacy, safety, and tolerability of the low glycemic index treatment in pediatric epilepsy.

PURPOSE: To report the efficacy, safety, and tolerability of the low glycemic index treatment (LGIT) in pediatric epilepsy. METHODS: A retrospective chart review was performed on patients initiating the LGIT at the Massachusetts General Hospital between January 2002 and June 2008. Demographic and clinical information including seizure type, baseline seizure frequency, medications, blood chemistries, side effects, and anthropometrics were collected. Initiation of the LGIT was done in an outpatient setting. Patients were educated by a dietitian to restrict foods with high glycemic index and to limit total daily carbohydrates to 40-60 g. Change in seizure frequency was assessed at 1-, 3-, 6-, 9-, and 12-month follow-up intervals. RESULTS: Seventy-six children were included in the study. Eighty-nine percent had intractable epilepsy (>or=3 antiepileptic drugs). A greater than 50% reduction from baseline seizure frequency was observed in 42%, 50%, 54%, 64%, and 66% of the population with follow-up available at 1, 3, 6, 9, and 12 months, respectively. Increased efficacy was correlated with lower serum glucose levels at some time points, but not with beta-hydroxybutyrate (BOHB) changes or ketosis status at any time point. Only three patients reported side effects (transient lethargy). Blood urea nitrogen (BUN) was elevated in approximately one-third of follow-up laboratory studies. No significant changes were seen in body mass index (BMI) or BMI z-score at any follow-up interval. The most cited reason for treatment discontinuation was the restrictiveness of the diet, in 18 patients (24%). CONCLUSION: The LGIT was associated with reduced seizure frequency in a large fraction of patients, with limited side effects.

Infantile spasms in tuberous sclerosis complex: prognostic utility of EEG.

PURPOSE: To assess cognitive and epilepsy outcomes in tuberous sclerosis complex (TSC) patients with a history of infantile spasms (IS), in relation to spasm history, electroencephalography (EEG) characteristics, genetic mutation, and treatment history. METHODS: The authors conducted a retrospective review of 45 children and adults with TSC and a history of IS. EEG reports from the time of spasms were evaluated for all patients, and EEG tracings were accessible and evaluated for 20 patients. RESULTS: Clinical outcome was unfavorable for the majority of patients. However, 33% had experienced at least one year of seizure freedom at follow-up, and 24% of those tested had IQs above 70. Hypsarrhythmia severity scores varied widely, with some EEGs severely hypsarrhythmic and others essentially normal. Lower IQ was significantly associated with higher hypsarrhythmia severity scores on EEG report, the presence of background disorganization on EEG report, the absence of normal sleep patterns on EEG, and a lower degree of treatment success on vigabatrin. A relationship between poor cognitive outcome and poor epilepsy outcome was confirmed. The correlation between poor epilepsy outcome and a greater degree of background disorganization on EEG approached significance, as did the association between subsequent intractable epilepsy and an older age at IS cessation. A greater than expected ratio of TSC2 to TSC1 patients was observed across this IS population. DISCUSSION: Early detection and successful treatment portend a more favorable outcome in TSC patients with IS. Although EEG findings in these patients vary, specific characteristics may serve as clinically useful prognostic markers.

Self-injurious behavior and tuberous sclerosis complex: frequency and possible associations in a population of 257 patients.

Self-injurious behavior (SIB) has been observed in people with tuberous sclerosis complex (TSC), although the frequency of SIB in TSC is largely unknown. SIB is associated with intellectual and developmental disabilities, but there is no single cause of SIB. We retrospectively examined the frequency of SIB in a population of 257 patients with TSC and determined possible associations with SIB. We found a 10% frequency of SIB in our TSC population. When compared with patients without psychiatric symptoms, we identified a significantly higher rate of electroencephalographic interictal spikes in the left frontal lobe and a significantly lower number of tubers in the left occipital, parietal, and posterior temporal lobes. We also found that frequency of TSC2 mutation, history of infantile spasms, history of seizures, mental retardation, and autism are significantly associated with SIB.

Behavior problems in children with tuberous sclerosis complex and parental stress.

Behavioral problems are common in children with tuberous sclerosis complex (TSC) and can be challenging to manage at home. Standardized measures were used to assess behavior in 99 pediatric patients with TSC and to evaluate parenting stress in their parents. About 40% of the pediatric patients presented clinically significant behavioral problems, most frequently involving symptoms of autism spectrum disorder, inattention, and hyperactivity. Higher seizure frequency, mixed seizure disorder, and low intellectual functioning placed the patient at significant risk for behavior problems. Almost 50% of participating parents reported experiencing clinically significant parenting stress, which was associated with specific characteristics of the child, including the presence of current seizures, a history of psychiatric diagnosis, low intelligence, and behavioral problems. Clinicians should be aware that behavioral problems are prominent in children with TSC. Referrals for behavioral intervention and monitoring of parental stress should be included in the medical management of children with TSC.

Psychiatric comorbid conditions in a clinic population of 241 patients with tuberous sclerosis complex.

Psychiatric symptoms were retrospectively assessed in a clinic population of 241 children and adults with tuberous sclerosis complex (TSC). Sixty-six (27%) patients had a history of mood disorder symptoms, 66 (27%) had a history of anxiety disorder symptoms, 73 (30%) had a history of attention-deficit hyperactivity disorder (ADHD) symptoms, and 68 (28%) had a history of aggressive/disruptive behavior disorder symptoms. Significant relationships were found between these symptoms and patient age, gender, genetic mutation, seizure history, surgical history, cognitive impairment, features of autism or pervasive developmental disorder, and neurological manifestations of TSC. In 43 patients seen by at least one of two affiliated psychiatrists, the most common formal diagnoses were anxiety disorders (28%), mood disorders (26%), adjustment disorders (21%), ADHD (21%), and mental disorders not otherwise specified due to general medical condition (42%). Citalopram demonstrated efficacy in treating anxiety and depression, and risperidone, in treating problematic behaviors.