ABSTRACT
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that typically presents in children and young adults. Occurrence outside of the extremities and the head and neck region is exceedingly rare. We report the case of a 9-year-old boy who presented with recurrent retroperitoneal hemorrhage initially thought to be a manifestation of an underlying bleeding disorder. After comprehensive diagnostic work-up, including multiple negative biopsies, the patient underwent surgical resection of an extensively hemorrhagic intramuscular mass and to date remains well. Pathologic examination confirmed AFH with EWSR1 gene rearrangement. This first documented report of an AFH in a retroperitoneal location in a child highlights the diagnostic difficulties and clinical challenges of AFH arising in an atypical location.
Subject(s)
Gene Rearrangement , Hemorrhage , Histiocytoma, Benign Fibrous , RNA-Binding Protein EWS/genetics , Retroperitoneal Neoplasms , Child , Hemorrhage/genetics , Hemorrhage/pathology , Hemorrhage/surgery , Histiocytoma, Benign Fibrous/genetics , Histiocytoma, Benign Fibrous/pathology , Histiocytoma, Benign Fibrous/surgery , Humans , Male , Retroperitoneal Neoplasms/genetics , Retroperitoneal Neoplasms/pathology , Retroperitoneal Neoplasms/surgeryABSTRACT
A term neonate was cannulated for venoarterial extracorporeal life support (ECLS) via the right neck for non-postoperative junctional ectopic tachycardia. Initial echocardiogram demonstrated an echogenic strand in the transverse arch. Computed tomography angiogram confirmed arterial dissection of the right common carotid artery that extended into the proximal transverse arch. Dissection flap was repaired at the time of ECLS decannulation without cardiopulmonary bypass. Follow-up computed tomography angiogram revealed a segment of narrowing of approximately 50% of the right common carotid artery without false lumen or aneurysm.
Subject(s)
Aortic Dissection/etiology , Carotid Artery, Common , Catheterization, Central Venous/adverse effects , Extracorporeal Membrane Oxygenation/adverse effects , Aortic Dissection/diagnosis , Echocardiography , Female , Humans , Infant, Newborn , Tomography, X-Ray ComputedSubject(s)
Actinomycosis/diagnosis , Actinomycosis/microbiology , Thoracic Diseases/diagnosis , Thoracic Diseases/microbiology , Actinomycosis/drug therapy , Adolescent , Anti-Bacterial Agents/therapeutic use , Cough/microbiology , Diagnosis, Differential , Female , Humans , Thoracic Diseases/drug therapy , Thoracic Wall/microbiology , Weight LossABSTRACT
BACKGROUND: Extracorporeal life support (ECLS) is a life-saving technology for the critically ill child. Our objective was to evaluate the outcomes of an educational curriculum designed to introduce an ECLS program to a noncardiac pediatric surgical center. METHODS: An interdisciplinary curriculum was developed consisting of didactic courses, animal labs, simulations, and debrief sessions. We reviewed all patients requiring ECLS between October 2011 and December 2013. All health care practitioners involved in the ECLS training curriculum were surveyed to evaluate their perception of the educational program. Primary outcomes include successful cannulation and 30-day survival. RESULTS: The knowledge and confidence improved with statistical significance (p<0.0001-0.0003) for all of the components of the training curriculum. The highest score was given to the simulations. Twenty-one patients underwent cannulation. All patients were successfully cannulated to bypass, including six (28.6%) ECPR. Median time from activation to cutting was 52min (IQR 40-72), and from cutting to bypass 40min (IQR 30-45). Sixteen patients (76.2%) were decannulated to a sustainable cardiac rhythm and survived 30-days. CONCLUSION: An ECLS curriculum incorporating simulation and dedicated practice seems to have eliminated the potential learning curve associated with the introduction of a complex technology to a novice environment.