Bloom where you are planted: Hemangioma or malignancy?

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.

Perforated Meckel Diverticulum: A Rare Cause of Acute Abdomen in Children.

Meckel diverticulum (MD) is the most common congenital anomaly of the gastrointestinal tract and the most common cause of gastrointestinal bleeding in children. Although it usually follows the rule of 2's, exceptions to this rule are reported in the literature. Often asymptomatic, MD is commonly an incidental finding during surgical interventions. When symptomatic, the most common presentation of this condition is painless rectal bleeding. A myriad of other nonspecific symptoms are however possible, especially in adults, thus making this diagnosis difficult. Meckel diverticulum has been reported to mimic other abdominal pathologies like appendicitis, inflammatory bowel disease, and pancreatitis to name a few.We report a patient with acute abdomen in whom the more common causes of acute abdomen were ruled out and a diagnosis of MD was established on exploratory laparoscopy, only after he developed perforation. This report emphasizes the need for maintaining a high index of suspicion towards a possibility of a complicated MD in patients presenting with an acute abdomen, once other causes of acute abdomen are ruled out.

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.

Toward Establishing a Standardized Magnetic Resonance Imaging Scoring System for Temporomandibular Joints in Juvenile Idiopathic Arthritis.

OBJECTIVE: The temporomandibular joints (TMJs) are frequently affected in children with juvenile idiopathic arthritis (JIA). Early detection is challenging, as major variation is present in scoring TMJ pathology on magnetic resonance imaging (MRI). Consensus-driven development and validation of an MRI scoring system for TMJs has important clinical utility in timely improvement of diagnosis and serving as an outcome measure. We report on a multi-institutional collaboration toward developing a TMJ MRI scoring system for JIA. METHODS: Seven readers independently assessed MRI scans from 21 patients (42 TMJs, from patients ages 6-16 years) using 3 existing MRI scoring systems from American, German, and Swiss institutions. Reliability scores, scoring system definitions, and items were discussed among 10 JIA experts through 2 rounds of Delphi surveys, nominal group voting, and subsequent consensus meetings to create a novel TMJ MRI scoring system. RESULTS: Average-measure absolute agreement intraclass correlation coefficients (avICCs) for the total scores of all 3 scoring systems were highly reliable at 0.96 each. Osteochondral items showed higher reliability than inflammatory items. An additive system was deemed preferable for assessing minor joint changes over time. Eight items were considered sufficiently reliable and/or important for integration into the consensus scoring system: bone marrow edema and enhancement (avICC 0.57-0.61, smallest detectable difference [SDD] ± 45-63% prior to redefining), condylar flattening (avICC 0.95-0.96, SDD ± 23-28%), effusions (avICC 0.85-0.88, SDD ± 25-26%), erosions (avICC 0.94, SDD ± 20%), synovial enhancement and thickening (previously combined, avICC 0.90-0.91, SDD ± 33%), and disk abnormalities (avICC 0.90, SDD ± 19%). CONCLUSION: A novel TMJ MRI scoring system was developed by consensus. Further iterative refinements and reliability testing are warranted in upcoming studies.

Magnetic Resonance Imaging Findings following Intraarticular Infliximab Therapy for Refractory Temporomandibular Joint Arthritis among Children with Juvenile Idiopathic Arthritis.

OBJECTIVE: To evaluate the involvement of intraarticular (IA) infliximab (IFX) in the management of temporomandibular joint (TMJ) arthritis associated with juvenile idiopathic arthritis (JIA) that is refractory to systemic treatment and IA corticosteroid therapy. METHODS: Ours was a retrospective study of children with JIA who received IA IFX into the TMJ. The effectiveness of treatment on the progression of acute and chronic changes was assessed by a quantitative magnetic resonance imaging scoring system. RESULTS: Median acute and chronic scores worsened by 0.25 and 0.75, respectively. In multivariate analysis, worsening acute scores and passage of time predicted worsening of the chronic scores. CONCLUSION: IA IFX allowed for progression of refractory TMJ arthritis in most but not all children with JIA.

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.

Complete response to carboplatin, gemcitabine, and paclitaxel in a patient with advanced metastatic renal medullary carcinoma.

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.

Failure in the nonoperative management of pediatric ruptured appendicitis: predictors and consequences.

INTRODUCTION: The initial nonoperative management of perforated appendicitis fails in 15% to 25% of children. These children have complications and increased hospitalization. The purpose of this study was to identify predictors of failure. METHODS: Children with perforated appendicitis treated with antibiotics and intent for nonoperative management over a 4-year period were reviewed. Seventy-five children were identified and included in the study. Failure was defined as undergoing appendectomy before the initially planned interval. RESULTS: Nine (12%) of the patients required appendectomy sooner than initially planned. Age, presenting symptoms, physical examination findings, and white blood cell (WBC) count were similar in both success and failure groups. Absence of abscess and presence of appendicolith were both predictors of failure in a multivariate analysis, which included the presence of small bowel obstruction. The failed group had a longer median total length of stay (18 days [range, 4-67] vs 8 days [range, 4-31]; P = .002) and underwent 3 times as many computed tomography scans as successes (3 [range, 2-7] vs 1 [range, 0-5]; P < .001). CONCLUSION: Lack of abscess and presence of an appendicolith predict failure of nonoperative management of perforated appendicitis in children even when the effect of small bowel obstruction is accounted for. Children with these characteristics may benefit from alternative management strategies.