Large, ulcerated infantile hemangioma of the chest wall complicated by life-threatening hemorrhage: Case report and literature review.

While ulceration is one of the most common infantile hemangioma (IH) complications, severe bleeding is a rare consequence, with a paucity of patients reported. We report a 5-month-old girl with a very large, mixed, partial segmental IH of the upper chest wall who, despite medical intervention, developed severe ulceration and multiple episodes of life-threatening bleeding that ultimately led to hemorrhagic shock. Experience in our patient and a review of six previous reports shows that severe bleeding is a risk when ulceration extends directly into an arterial feeding vessel that is often visible clinically. Other potential predictors for severe bleeding include large to very large IH size with extension of the tumor into underlying structures, segmental or partial segmental patterning, mixed and bulky morphology, and white discoloration as a sign of impending or worsening ulceration.

Sporadic inclusion body myositis and primary Sjogren's syndrome: an overlooked diagnosis.

Sporadic inclusion body myositis (sIBM) has been reported to occur in association with autoimmune diseases and in particular, primary Sjogren's syndrome (pSS). This brief report describes patients identified with a positive SSA antibody and diagnosis of sIBM at a large academic medical center over a 13.5-year period. A cohort identification tool was used to identify patients with positive SSA antibody and a diagnosis of sIBM between January 1, 2006 and June 1, 2019. All cases of sIBM had diagnostic confirmation by a neuromuscular specialist. Demographics, clinical features, autoantibodies, MRI and EMG findings, and muscle biopsy features were reviewed for each identified case. Eight patients were found to carry the diagnosis of pSS and sIBM. Two additional sIBM patients were SSA antibody positive without other pSS features. The mean time from initial symptom onset of muscle weakness to diagnosis was 5.4 years (range 1-15 years). All patients had alternative diagnoses offered for their myopathic symptoms prior to sIBM identification. The NT5c1A antibody was positive in 7 of 8 patients tested. No patient had a durable response to immunosuppressive therapy. The diagnosis of sIBM went unrecognized for over 5 years in our cohort of SSA antibody-positive patients with myopathy. The patients in this cohort were treated with a variety of immunosuppressive agents prior to diagnosis without benefit. Recognizing the clinical features of sIBM in patients with pSS is crucial in instituting appropriate therapy and avoiding unnecessary immunosuppression. Key Points • Sporadic inclusion body myositis (sIBM) can be associated with Sjogren's syndrome. • In this case series, prevalence of the NT5c1A antibody was higher among patients with associated Sjogren's syndrome compared to the cited prevalence of the NT5c1A antibody in patients with isolated sIBM. • It is crucial to consider sIBM in patients with Sjogren's syndrome presenting with motor weakness in order to avoid unnecessary immunosuppression and institute appropriate therapy.

A multidisciplinary approach to VA ECMO cannulation in children.

PURPOSE: Extracorporeal membrane oxygenation (ECMO) supports gas exchange and circulation in critically ill patients. This study describes a multidisciplinary approach to ECMO cannulation using the expertise of pediatric surgery (PS) and interventional radiology (IR). MATERIAL AND METHODS: Pediatric patients (<18 years) undergoing percutaneous cannulation for peripheral veno-arterial (VA) ECMO by PS and IR from April 2017 to May 2018 were included. Cardiac patients and children cannulated by PS alone were excluded. RESULTS: Five patients were included in the series. Median age was 16 [12.5-17] years and 3 were female. Median ECMO arterial and venous catheter sizes were 19 [17-22] Fr and 25 [25-28] Fr, respectively. Both catheters were placed in the common femoral vessels. A 6Fr antegrade distal perfusion cannula (DPC) was also placed in the superficial femoral artery by IR at the time of cannulation. The median time from admission to procedure start was 10 [7-50] hours and the children were on ECMO for a median length of 3.2 [2.3-4.8] days. There were two episodes of bleeding. No patients had loss of limb circulation. CONCLUSION: A multidisciplinary approach to peripheral VA ECMO cannulation is feasible and safe. Maintenance of limb perfusion by percutaneous placement and removal of DPC may be an advantage of this collaborative approach. LEVEL OF EVIDENCE: IV. TYPE OF RESEARCH: Case series.

Quantitative and morphologic change associated with breast cancer-related lymphedema. Comparison of 3.0T MRI to external measures.

UNLABELLED: Abstract Introduction: Lymphedema is a chronic disease of increasing importance to cancer survivors. A tape measurement tool used for lymphedema relies on indirect volume calculations based on external circumference, which may not reflect the true extent of abnormal fluid accumulation accurately. Fluid-sensitive MRI sequences may be able to delineate the severity of this condition more precisely and thus also monitor response to therapy. METHODS AND RESULTS: Eight patients being followed by physical therapy for clinically diagnosed breast cancer-related lymphedema were recruited to participate in this study. External measurements and upper extremity MRI were performed on all subjects. Arm circumference, arm volume, and lymphedema volumes were calculated for each method. MR imaging detected lymphedema in all study subjects. Correlation was found between external circumferential measurements and with the 3.0T MRI (r=0.9368). There was poor correlation between lymphedema volumes calculated from clinical measurements and MR imaging (r=0.5539). CONCLUSIONS: External measurements were not found to be an accurate measure of lymphedema volume associated with breast cancer lymphedema. MRI is a reliable means to obtain upper extremity circumferential and volume measurements. MRI is able to evaluate morphologic change associated with breast cancer-related lymphedema. Lymphedema research requires integrated use of tools to further describe the disease process over time, quantitate the distribution of tissue changes, and improve the sensitivity and specificity of the measurements.

Lipid-associated rheumatologic syndromes.

Rheumatologic manifestations of hyperlipidemia and lipid-associated arthritis are rarely seen in the rheumatologist's office. On the other hand, a rheumatologist may be the clinician who identifies and initiates proper therapy for disorders related to hyperlipidemia when the musculoskeletal manifestations of these syndromes are recognized. In this article both the joint and tendon manifestations are reviewed, including the lesser known lipid liquid crystal form of arthritis. The relationship between gout and hyperuricemia is briefly discussed, as are the autoimmune manifestations of lipid-lowering therapy.

A survey of arthrocentesis and soft-tissue injection procedures performed in primary care practice: effect of resident training and using data to shape curriculum.

OBJECTIVE: The purpose of this study is (1) to survey graduates of our internal medicine program for use of musculoskeletal (MSK) procedures in primary care practice and assess the influence of participating in a first-year resident arthrocentesis and soft-tissue injection training course on their MSK procedure comfort/utilization; and (2) to use the results to modify our MSK procedure curriculum. METHODS: A questionnaire designed to assess numbers of, comfort with, and effect of resident training on MSK procedures in the preceding year was sent to 2002-2006 graduates of the internal medicine training program in outpatient primary care (OPC). Graduates practicing hospital medicine (HM) also received the questionnaire and served as a comparison group. RESULTS: There were 52 responses from this group of 84 graduates (64% response rate). OPC graduates (N = 32) were more comfortable doing procedures than those practicing HM exclusively (N = 20), and performed significantly more procedures in the preceding year (32.9 procedures per OPC/year vs 2.2 for HM). The most common procedures performed were knee joint, subacromial bursa, and trochanteric bursa, comprising > 75% of all procedures performed. A structured resident course in MSK procedures had a significant effect on the OPC physicians. Course participants (N = 17) performed almost twice the number of procedures/year as the nonparticipants (N = 15), were more comfortable with the procedures, and were significantly less likely to refer procedures to other clinicians. Written comments by respondents suggest additional MSK procedure training during and after residency is needed. CONCLUSION: Our results suggest a structured resident course in MSK procedures has a longterm influence. A progressively more focused approach to training is needed.

Impact of educational intervention on confidence and competence in the performance of a simple surgical task.

BACKGROUND: Many complex new procedures involve a learning curve, and patients treated by individuals who are new to a procedure may have more complications than those treated by a practitioner who has performed the intervention more frequently. Still, at some point on the learning curve, each individual must decide that he or she is qualified to perform a procedure, presumably on the basis of his or her level of confidence, background, education, and skill. To evaluate the interrelationship of these factors, we designed a study in which we assessed the performance of a simulated knee joint injection. METHODS: Ninety-three practitioners attending a continuing medical education session on knee injection were randomized to receive skills instruction through the use of a printed manual, a video, or hands-on instruction; each performed one injection before and after instruction. The participants completed pre-instruction and post-instruction questionnaires gauging confidence and also provided self-assessments of their performances of injections before and after instruction. Self-assessments were compared with objective performance standards measured by custom-designed knee models with electronic sensors that detected correct needle placement. RESULTS: Before instruction, the participants' confidence was significantly but inversely related to competent performance (r = -0.253, p = 0.02); that is, greater confidence correlated with poorer performance. Both men and physician-practitioners displayed higher pre-instruction confidence (p < 0.01), which was not correlated with better performance. After instruction, performance improved significantly in all three training groups (p < 0.001), with no significant differences in efficacy detected among the three groups (p = 0.99). After instruction, confidence correlated with objective competence in all groups (r = 0.24, p = 0.04); however, this correlation was weaker than the correlation between the participants' confidence and their self-assessment of performance (r = 0.72, p = 0.001). CONCLUSIONS: Even low-intensity forms of instruction improve individuals' confidence, competence, and self-assessment of their skill in performing the fairly straightforward psychomotor task of simulated knee injection. However, men and physicians disproportionately overestimated their skills both before and after training, a finding that worsened as confidence increased. The inverse relationship between confidence and competence that we observed before the educational intervention as well as the demographic differences that we noted should raise questions about how complex new procedures should be introduced and when self-trained practitioners should begin to perform them.