ABSTRACT
Vascular anomalies comprise a spectrum of disorders characterized by the abnormal development or growth of blood and lymphatic vessels. These growths have unique features and diverse behaviors, mandating a multidisciplinary approach in their evaluation, diagnosis, and management. Here we describe the case of a male toddler presenting with an abdominal mass, originally treated as a metastatic retroperitoneal tumor, but subsequently felt to represent a vascular anomaly.
Subject(s)
Hemangioma , Retroperitoneal Neoplasms , Vascular Malformations , Hemangioma/pathology , Hemangioma/therapy , Humans , Male , Vascular Malformations/therapyABSTRACT
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.
Subject(s)
Arthritis, Juvenile/complications , Magnetic Resonance Imaging/methods , Temporomandibular Joint Disorders/diagnostic imaging , Temporomandibular Joint/diagnostic imaging , Adolescent , Arthritis, Juvenile/diagnostic imaging , Child , Child, Preschool , Contrast Media , Female , Humans , Infant , Male , Prospective Studies , Retrospective Studies , Temporomandibular Joint/pathology , Temporomandibular Joint Disorders/etiologyABSTRACT
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.
Subject(s)
Arthritis, Juvenile/pathology , Magnetic Resonance Imaging , Severity of Illness Index , Temporomandibular Joint/pathology , Adolescent , Child , Child, Preschool , Disease Progression , Humans , Observer Variation , Reproducibility of Results , Retrospective StudiesABSTRACT
Renal medullary carcinoma (RMC) is a rare and aggressive malignancy seen primarily in patients with sickle-cell trait. We report a complete response to carboplatin, paclitaxel, and gemcitabine in a patient with advanced metastatic RMC.
