RESUMO
PURPOSE: Inflammatory myofibroblastic tumor (IMT) is a rare neoplastic tumor type of intermediate biological potential, only recently distinguished from the non-neoplastic category of inflammatory pseudotumor (IP). The literature describes very few cases of IMTs arising in the central nervous system (CNS), and the distinguishing clinical, pathological, and molecular features of IMT-CNS are not well understood. Our purpose is to publish a case of an IMT-CNS with a novel DCTN1-ALK gene fusion, furthering in the literature's characterization of a rare tumor type. METHODS: Review of the literature included a PubMed Database search of articles found by the following searches: "Inflammatory myofibroblastic tumor;" "Inflammatory myofibroblastic tumor central nervous system;" "ALK gene fusion;" and "DCTN1-ALK gene fusion." Inclusion of articles discovered by these search terms was determined through critical appraisal of article relevance, number of citations, cross-citation within articles of interest, and rare findings with conflicting conclusions in an effort to reduce publication bias. RESULTS: We present a case of IMT-CNS with several distinctive molecular features including a DCTN1-ALK gene fusion, the first of its kind described in an intracranial IMT. CONCLUSION: IMT is an infrequent tumor type and its presentation within the CNS is exceedingly rare. The paucity of cases, along with the ambiguity of terminology in the literature, has stunted accurate clinical, pathological, and molecular characterization of IMT-CNS. Our case report improves the characterization of the recently appreciated category of IMT-CNS so that connections between phenotype and prognosis, and between genotype and treatment, can eventually be made.
Assuntos
Granuloma de Células Plasmáticas , Quinase do Linfoma Anaplásico , Sistema Nervoso Central , Complexo Dinactina , Fusão Gênica , Granuloma de Células Plasmáticas/diagnóstico por imagem , Granuloma de Células Plasmáticas/genética , Granuloma de Células Plasmáticas/cirurgia , Humanos , Receptores Proteína Tirosina Quinases/genéticaRESUMO
Accurate analysis of injuries is paramount when allocating resources for prevention, research, education, and legislation. As burn mortality has improved over recent decades, the societal burden of burn injuries has grown ambiguous to the public while a scarcity of investigational funding for survivors has led to a gap in understanding lifelong sequela. We aim to compare national references reporting the incidence of burn injuries in the United States. The American Burn Association Burn Injury Summary Report (ABA-BISR), American Burn Association Fact Sheet, Centers for Disease Control and Prevention (CDC) Web-based Injury Statistics Query and Reporting (WISQARS) database, the CDC National Center for Health Statistics' National Hospital Ambulatory Medical Care Survey (NHAMCS), National Inpatient Sample (NIS), National Emergency Department Sample (NEDS), and commercially available claims databases were queried for 2020 or the most recent data available. The BISR estimated 30,135 burn admissions in 2022. The 2016 ABA Fact Sheet reported 486,000 burns presented to US emergency departments (ED). In 2020, CDC's WISQARS database reported 3,529 fatal, and 287,926 non-fatal, burn injuries. The 2020 NEDS reported 438,185 ED visits while the 2020 NIS estimated 103,235 inpatients. The NHAMCS reported 359,000 ED visits for burn injuries in the same period, and an analysis of ICD-10 burn codes demonstrated over 698,555 claims. Our study demonstrates a large variability in the reported incidence of burn injury by the ABA, CDC, national samples, and claims databases. Per our analyses, we estimate that 600,000 individuals annually suffer a burn injury which merits emergent care in the United States.
RESUMO
The combination of hemophilia and sickle cell disease poses unique challenges in the perioperative management of patients requiring free tissue transfer (FTT). Extremity FTT requires mitigation of risk factors related to perioperative bleeding and microvascular thrombosis. This case report highlights the nuances of managing an open ankle fracture in a patient with both sickle cell trait and severe hemophilia A. The narrative and discussion highlights the importance of a multidisciplinary team with regard to executing a limb salvage plan in the setting of complex medical decision-making.
RESUMO
Background: Nerve reconstruction techniques for lumbosacral plexus (LSP) injuries vary. There are no clear treatment guidelines available, and summative evaluations of the literature discussing these surgeries are lacking. For these reasons, this investigation aimed to systematically review and consolidate all available literature discussing surgical interventions for LSP injuries and cohesively present patient-reported and objective postoperative outcomes. Methods: The authors conducted a systematic review using PubMed, Embase, Web of Science, ProQuest Dissertations and Theses Global (via Proquest.com), and ClinicalTrials.gov databases according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. After title and abstract screening, identified articles were read in full and selected for inclusion based on prespecified criteria. Results: Our literature search identified 8683 potential citations, and after duplicate removal, abstract screening, and full-text review, 62 studies remained meeting inclusion and exclusion criteria. Outcomes were extracted according to the location of injury and type of surgical repair. Injuries were classified into isolated femoral nerve injuries, isolated obturator nerve injuries, isolated sciatic nerve injuries, and multilevel LSP injuries. Surgical treatment was further classified into exploration with neurolysis, direct repair, nerve grafting, and nerve transfer surgery. Conclusions: Although results vary based on the location of the injury and the surgical technique used, nerve grafts and transfers demonstrated reasonable success in improving functional and pain outcomes. Overall, isolated femoral and obturator nerve injuries had the best outcomes reported with surgical treatment. Furthermore, incomplete sciatic nerve and multilevel LSP injuries had more reported surgical options and better outcomes than complete sciatic nerve injuries.
RESUMO
Background: Cutaneous mucormycosis, while less common than sinonasal or pulmonary infections, can cause widespread tissue necrosis after seemingly innocuous encounters. The most common location of cutaneous mucormycosis is the extremities, and extensive infection has been reported after trauma or orthopedic procedures. Case Report: A 60-year-old female with poorly controlled type 2 diabetes mellitus sustained an open patella fracture after a fall. She underwent washout and internal fixation with cannulated screws and cable tension band wiring. The patient's recovery was complicated by asymptomatic coronavirus disease 2019 (COVID-19) infection and repeated wound dehiscence, with growth of Mucor species initially presumed to be a contaminant. Despite serial washout and debridement, repeat dehiscence and patella exposure were noted. Free tissue transfer to the genicular vessels was selected for coverage of the extensor tendon, patella, and fracture line. In repeat skin cultures, Mucor indicus and Staphylococcus epidermidis grew from the wound. Topical voriconazole and a 6-week course of intravenous isavuconazole and oral doxycycline were started when the Mucor cultures were identified. Conclusion: This case highlights an approach to an indolent mucormycosis infection in the skin over a patella fracture in a patient with poorly controlled diabetes mellitus, including the sequence of surgical care, debridement, and selection of antimicrobials. Major amputation and orthopedic revision were avoided. This patient also underwent successful free tissue transfer after testing positive for COVID-19.
RESUMO
Hand aneurysms in infants are extremely rare and, unlike their adult counterparts, cannot be explained by repetitive trauma to the palm. When they occur, they are most often located in the ulnar artery. Usually there is no history of trauma, and an alternative diagnosis like malignancy must be excluded. Helpful physical findings to aid diagnosis include rapid appearance of a mass over the course of a few days, pulsatile nature, and location along the ulnar side of the hand. These cases can be challenging and excisional biopsy may be required if the lesion is thrombosed and does not have a characteristic appearance on imaging. Aneurysms located in the palmar arch or common digital arteries are usually treated with simple excision, while those located in the ulnar artery often require repair or reconstruction. Here we present a unique case of an infant with a pseudoaneurysm involving the superficial palmar arch on the radial side of the hand with a likely congenital etiology. Diagnostic challenges and treatment options are discussed.