Defining the normal appearance of the temporomandibular joints by magnetic resonance imaging with contrast: a comparative study of children with and without juvenile idiopathic arthritis.

BACKGROUND: Up to 80% of children with juvenile idiopathic arthritis (JIA) develop arthritis involving their temporomandibular joint (TMJ). Recent studies have questioned the sensitivity of an abnormal MRI in the diagnosis of active arthritis. METHODS: 122 children without arthritis undergoing contrast MRI of the head were prospectively consented to undergo a simultaneous contrast MRI of their TMJs. As a comparison point, the initial MRI of the TMJ of 35 newly diagnosed children with JIA were retrospectively scored. The presence and size of effusion and contrast enhancement were measured in the left TMJ in all subjects. RESULTS: 62/122 (51%) controls compared to only 10/35 JIA (29%) patients had an effusion (p = 0.022). Contrast enhancement was present in ≥97% of both groups, although the size of the enhancement was, on average, 0.2 mm larger in controls (1.1 ± 0.24 vs 0.88 ± 0.27 mm, p <  0.001). Among JIA patients, the size of the enhancement correlated inversely with disease duration (r = - 0.475, p = 0.005). Chronic changes were present in none of the controls versus 2/35 (5.5%) of the JIA patients (p = 0.049). CONCLUSION: Findings consistent with minimally active TMJ arthritis appear to be equally likely in children with JIA as compared to non-inflamed controls, while this and other studies confirm that chronic changes are specific to JIA. Thus, small amounts of effusion or contrast enhancement, in the absence of chronic changes, should be interpreted with caution.

Imaging of the temporomandibular joint in juvenile idiopathic arthritis.

OBJECTIVE: Temporomandibular joint (TMJ) arthritis in children with juvenile idiopathic arthritis (JIA) is extremely common but frequently asymptomatic. Magnetic resonance imaging (MRI) with contrast remains the gold standard for identifying TMJ arthritis in JIA. A reliable scoring system with published MRI examples of typical acute and chronic TMJ arthritis changes will be invaluable for future prospective treatment trials of TMJ arthritis in JIA. METHODS: MRIs were collected from routine clinical studies assessing TMJ arthritis in JIA. Representative images were selected for publication to depict acute (synovial fluid, bone marrow edema, and synovial enhancement) and chronic (pannus, disc derangement, and condylar head flattening and erosions) TMJ arthritis findings. A preliminary MRI-based scoring system for assessing degrees of acute and chronic TMJ arthritis was developed and tested for inter- and intrareader reliability. RESULTS: TMJ MRIs representative of acute and chronic TMJ arthritis in JIA were selected from among thousands taken (>500 TMJ MRI studies annually at Children's of Alabama) since September 2007. Moreover, computed tomography scans depicting select bony changes (osteophyte formation, micrognathia) were chosen for publication. A description of the MRI protocol for assessing TMJ arthritis is included. A preliminary scoring system weighted for degree of acute and chronic TMJ arthritis MRI findings was found to have substantial inter- and intrareader reliability. CONCLUSION: A published set of MRIs depicting representative acute and chronic changes will help establish a standardized scoring system to assess TMJ arthritis in children with JIA. Future validation will aid in assessing improvement during treatment trials of TMJ arthritis.

The accuracy of abnormal lumbar sonography findings in detecting occult spinal dysraphism: a comparison with magnetic resonance imaging.

OBJECT: The purpose of this study was to correlate lumbar ultrasound (LUS) and MRI findings in patients suspected of having occult spinal dysraphism (OSD). METHODS: Over a 5-year period, 1273 consecutive infants underwent an LUS study at a major pediatric tertiary referral center. Of these, 106 patients had abnormal LUS findings suggestive of an OSD, and 103 underwent subsequent MRI studies. The anatomical descriptions of the 2 studies were compared for agreement. RESULTS: The average age of the infants was 34 days at the time of the LUS study; OSD was suspected in these patients because of the presence of cutaneous stigmata and congenital defects. The most common anatomical descriptions from the LUS study included a thickened or fatty filum (32 cases), filum cyst (11 cases), and presence of a terminal ventricle or syrinx (9 cases). Using MRI findings as the standard reference, the sensitivity of LUS in detecting a thickened or fatty filum was 20%. The sensitivity of detecting an abnormal conus level at or below L-3 was 76.9%. CONCLUSIONS: In the patient population chosen to undergo LUS studies, abnormal findings had poor sensitivity at detecting anatomical findings consistent with OSD.

Use of lumbar ultrasonography to detect occult spinal dysraphism.

OBJECT: Various cutaneous stigmata and congenital anomalies are accepted as sufficient reasons to perform lumbar ultrasonography as a screening tool to rule out occult spinal dysraphism (OSD). The purpose of this study was to correlate presenting cutaneous findings with lumbar ultrasonography results based on a large number of lumbar ultrasonography tests obtained by regional primary care providers. METHODS: Over the course of 5 years, 1273 infants underwent lumbar ultrasonography screening at a major pediatric tertiary referral center. Of these infants, 1116 had adequate documentation for retrospective chart review. Referral sources included urban academic, urban private practice, and surrounding rural private practitioners. Presence of cutaneous stigmata and/or congenital anomalies and lumbar ultrasonography results were reviewed for all patients. When present, surgical findings were reviewed. RESULTS: A total of 943 infants were referred for presumed cutaneous stigmata, the most common of which was a sacral dimple (638 patients [68%]) followed by hairy patch (96 patients [10%]). Other reported cutaneous findings included hemangioma, deviated gluteal fold, skin tag, and skin discoloration. In comparison, 173 patients presented with congenital anomalies, such as imperforate anus (56 patients [32%]) and tracheoesophageal fistula/esophageal atresia (37 patients [21%]), most of which were detected prenatally by fetal ultrasonography. A total of 17 infants underwent surgical exploration. Occult spinal dysraphism was diagnosed in 7 infants in the cutaneous stigmata group and in 10 infants in the group with congenital abnormalities. None of the cutaneous stigmata as recorded were found to be indicative of the presence of OSD. CONCLUSIONS: Cutaneous markers as currently defined by general practitioners are not useful markers for predicting OSD. The vast majority of findings on lumbar ultrasonography studies performed under these circumstances will be negative.

Severe contrast reaction emergencies high-fidelity simulation training for radiology residents and technologists in a children's hospital.

RATIONALE AND OBJECTIVES: Severe reactions to radiographic contrast agents can be life threatening, and although they are rare, effective recognition and management are essential to improving outcomes. A high-fidelity radiology simulation course for radiology residents and technologists focusing on severe contrast reactions and immediate treatments was designed to test the hypothesis that knowledge would improve with this educational intervention. MATERIALS AND METHODS: A prospective pretest and posttest study design was used. Residents and technologists worked in teams of three to five members. Learning objectives focused on demonstrating when and how to use basic life support skills and epinephrine auto-injectors. Each resident and technologist was administered a pretest prior to the start of the case scenarios and a posttest following the debriefing session. Scores from the pretest and posttest for the residents and technologists were compared using a paired-samples t test. RESULTS: Nineteen radiology residents and 11 radiology technologists participated. The average test scores were higher and improved significantly following the simulation experience for both the radiology residents (57% vs 82%, P < .001) and technologists (47% vs 72%, P = .006). Anonymous evaluations demonstrated that the experience was well received by residents and technologists, with 97% of learners (29 of 30) rating the experience as extremely or very helpful. Important learning themes included the knowledge of epinephrine auto-injector use and basic life support skills. DISCUSSION: High-fidelity simulation for radiology residents and technologists focusing on epinephrine auto-injector use and basic life support skills during the first 5 minutes of a severe contrast reaction can significantly improve recognition and knowledge in treating patients having severe contrast reactions.

Group A streptococcal infection of an intramuscular venous malformation: a case report and review of the literature.

Intramuscular venous malformations are uncommon benign masses of vascular origin that can occur anywhere in the body. They can be small and clinically insignificant or can be large and violate tissue planes and viscera. Presentation is as variable as the extent of the lesions. They can be diagnosed as a result of local pain and swelling or as incidental findings. Rarely, venous malformations can get infected and present with fever or other more severe systemic symptoms. The literature is sparse regarding infection of intramuscular venous malformations. This case describes the presentation, diagnosis, and management of a patient with group A streptococcal infection of a previously undiagnosed intramuscularvenous malformation in a patient who presented to our pediatric emergency department.

Splenogonadal fusion diagnosed by Doppler ultrasonography.

Splenogonadal fusion usually presents as a left scrotal mass but rarely is the diagnosis suspected preoperatively. Herein, we present the first report of a left splenogonadal fusion which was suspected preoperatively by doppler ultrasonography in a 2 year old boy.

Investigations into the association between cervicomedullary neuroschisis and mirror movements in patients with Klippel-Feil syndrome.

BACKGROUND AND PURPOSE: Our purpose was to investigate the association between cervicomedullary neuroschisis and mirror movements in patients with Klippel-Feil syndrome (KFS). METHODS: We conducted a retrospective analysis of 23 patients with KFS who were seen at our institution during a 10-year period. Sixteen of the 23 patients had undergone adequate axial view cross-sectional imaging of the upper cervical spine. The degree of neuroschisis was assessed for each patient, using an objective scoring system. Twelve patients were evaluated for the presence or absence of mirror movements. RESULTS: A high percentage of female patients with KFS was noted (17 [74%] of 23 patients). Adequate cross-sectional images were available for 16 of the 23 patients, six (38%) of whom had some form of cervicomedullary neuroschisis. Five of the six patients had been clinically evaluated, and all were shown to have mirror movements. One patient with Chiari II malformation, which obscured evaluation for neuroschisis, also had mirror movements. Of the remaining nine patients without cervicomedullary neuroschisis, six were evaluated, and none of the six had mirror movements. A review of the theoretical neuroanatomic basis of mirror movements is presented herein, and neurosurgical management concerns for patients with KFS are discussed. CONCLUSION: A strong association exists between cervicomedullary neuroschisis and mirror movements in cases of KFS. Screening of patients with mirror movements may help identify clinically unsuspected KFS and may also help stratify risk within this patient population, identifying patients who might benefit from early neurosurgical intervention.