Vascular handlebar syndrome with blunt injury of common femoral artery.

Vascular handlebar syndrome with blunt injury of the common femoral artery is a rare vascular trauma mechanism, with high possibility of being missed or delayed. We present two cases of vascular handlebar syndrome treated in our hospital and a systematic review of the literature using MEDLINE and SCOPUS databases. Literature review identified 20 similar cases. The median age of patients was 18 years, and in vast majority males in gender. In most cases, the common femoral artery injury was an intimal flap and lumen occlusion with intramural thrombosis followed by transection and intimal injury without occlusion or thrombosis. The median time between injury and diagnosis/treatment was half an hour. Clinical presentation ranged from asymptomatic to acute limb ischemia. The grade of acute ischemia was mostly Rutherford class I (n=14), while acute IIa (n=4), chronic ischemia (n=3), and no ischemia (n=1) were also noticed. The correct diagnosis was revealed by clinical examination only (n=1), or by the combination of clinical and imaging techniques including computed tomography angiography (n=7) and duplex ultrasonography (n=4) or both (n=10). Management of the handlebar trauma syndrome injuries was surgical in most cases. Outcome was favorable in all patients. Vascular handlebar syndrome is extremely rare and high suspicion is required for early diagnosis and definitive treatment, as the early management is effective and crucial for averting the devastating consequences. An individualized approach to the vascular trauma patient is to be applied with considerations taken to the age of the patient, the mechanism of the injury, the anatomy of the lesion, and symptomatology of the case.

No Difference in Outcomes Between Suspensory (Fixed-Loop Cortical Button) Versus Expandable Anteromedial Femoral Fixation in Anterior Cruciate Ligament Reconstruction With Autologous Hamstring Tendons: A Prospective, Randomized, Controlled Study in Male Patients.

PURPOSE: To compare anterior cruciate ligament (ACL) autograft reconstruction using fixed-looped suspensory versus expandable femoral fixation through the anteromedial portal. METHODS: After we performed prospective power analysis and obtained institutional review board, 70 patients with ACL rupture were block randomized to the expandable or suspensory femoral fixation group (35 in each group). All patients received autologous hamstring autograft through the anteromedial portal and fixed with a sheath-screw system in the tibia. The primary outcome measures were anteroposterior knee stability at 2 years' follow-up measured using the KT-1000 arthrometer and the degree of femoral and tibial tunnels' widening measured by the use of computed tomography imaging performed immediately postoperative and 12 months postsurgery. Secondary outcome measures included pain score on a visual analog scale, the subjective International Knee Documentation Committee 2000 assessment form, the Lysholm score, and the Tegner activity scale at 3, 6, 12, and 24 months. RESULTS: Twenty-four patients were excluded from further analysis, leaving a total of 48 patients (24 in each group) for the final evaluation. The anteroposterior knee stability (KT-1000) showed no difference between groups at 24 months' follow-up (P = .31). The percentile widening (%) of femoral and tibial tunnel at 1-year follow-up showed no difference also, except for greater values at the tibial coronal point T2 (P = .065) and tibial sagittal point T1 (P = .033) in the group of cortical buttons. Secondary clinical outcomes showed no statistical differences between groups at 3, 6, 12, and 24 months' postoperatively. Numerical pain scale (visual analog scale) was similar in both groups except for postoperative day 7, where the AperFix group showed better results (P = .014). There were no major intraoperative and late postoperative complications in any of the groups. CONCLUSIONS: Our results showed no significant differences in knee anteroposterior stability, tunnel enlargement or other clinical outcomes comparing expandable versus cortical button fixation in anteromedial hamstrings ACL reconstruction. LEVEL OF EVIDENCE: Level II, randomized controlled trial.

An MRI study of immune checkpoint inhibitor-induced musculoskeletal manifestations myofasciitis is the prominent imaging finding.

OBJECTIVE: To assess: (i) the prevalence, and clinical and imaging characteristics of immune checkpoint inhibitor (ICI)-induced musculoskeletal immune-related adverse events (ir-AEs) in a prospective manner and (ii) whether serum levels of cytokines associated with the Th1/Th2/Th17 response are differentially expressed in patients with and without musculoskeletal Ir-AEs. METHODS: All patients treated with ICI who developed musculoskeletal manifestations were referred to the Rheumatology Department, and an MRI of the involved area(s) was performed. RESULTS: During the study period, a total of 130 patients were treated with ICIs. Of these, 10 (7.7%) developed ICI-induced Ir-AEs. The median time from ICI treatment since development of symptoms was 2.5 months. Three different patterns of musculoskeletal manifestations were found: (i) prominent joint involvement (n = 3); (ii) prominent 'periarticular' involvement (n = 4). These patients had diffuse swelling of the hands, feet or knees. MRI depicted mild synovitis with more prominent myositis and/or fasciitis in the surrounding tissues in all cases; (iii) myofasciitis (n = 3). Clinically, these patients presented with pain in the knee(s)/thigh(s), whereas MRI depicted myofasciitis of the surrounding muscles. Patients with musculoskeletal ir-AEs had significantly higher oncologic response rates compared with patients not exhibiting musculoskeletal ir-AEs (50% vs 12.5%, respectively, P = 0.0016). Cytokine levels associated with a Th1/Th2/Th17 response were similar between patients with and without musculoskeletal ir-AEs. Overall, symptoms were mild/moderate and responded well to treatment, with no need for ICI discontinuation. CONCLUSION: In our cohort, ICI-induced musculoskeletal manifestations developed in 7.7% of patients. Imaging evidence of myofasciitis was found in most patients, indicating that the muscle/fascia is more frequently involved than the synovium.

An ischemic stroke as the presenting manifestation of rapidly progressive primary angiitis of central nervous system in a 17-year-old boy.

BACKGROUND: Childhood primary angiitis of the central nervous system (cPACNS) is an increasingly recognized inflammatory brain disease in children. CASE PRESENTATION: We present a case of a 17-year-old boy with recurrent ischemic events over a short time period. Diagnosis of angiography positive cPACNS was made based on neuroimaging findings while secondary causes or mimics of CNS vasculitis were meticulously excluded. The patient exhibited rapid deterioration of his condition with poor initial response to immunosuppressive treatment. CONCLUSIONS: Recognition of cPACNS remains a challenge because of rarity of disease, unexplained etiopathogenesis, protean clinical presentation, as well as lack of specific laboratory and neuroimaging markers.

Neck angioedema after anterior cervical discectomy and fusion with coexistent epiglottic cyst.

BACKGROUND: We present a case and reviewed the literature regarding airway obstruction and angioedema after an anterior cervical discectomy and fusion (ACDF). CASE DESCRIPTION: A 60-year-old female with degenerative cervical myelopathy and a previously undiagnosed epiglottic cyst underwent a C5-C6 ACDF; notably, the anesthesiologist found an epiglottic cyst when the patient was first intubated. Two hours postoperatively, the patient acutely developed severe neck swelling with airway obstruction due to angioedema. She was immediately treated with hydrocortisone and required a tracheostomy. The edema decreased markedly in the next 12 h and by the 3rd postoperative day it resolved. Three months later, she had no residual medical sequelae. CONCLUSION: Patients with epiglottic cysts who need cervical spine surgery should either first have the cyst treated or should be closely monitored postoperatively.

Targeting very early systemic sclerosis: a case-based review.

It is unknown whether treatment in very early/early systemic sclerosis (SSc) can affect long-term outcomes. A case-based review was conducted (i) to assess the effect of rituximab (RTX) in very early SSc and (ii) to explore how many clinical trials in SSc targeted early disease and whether treatment of these patients led to better clinical outcomes. We identified cases of very early SSc from our department and performed a search in MEDLINE and Scopus databases for clinical trials in SSc during 2005-2018. Two cases are reported where RTX was administered within 24 months from the appearance of Raynaud's. In the first case, there was an improvement in interstitial lung disease as indicated by the improvement in pulmonary function tests and the regression of changes in high-resolution chest computed tomography. In the second case, a good clinical response in skin fibrosis was observed. The review revealed the following: (i) only one-third of the studies were specifically designed to target early disease, (ii) there is confusion related to disease duration definition across SSc clinical trials but an obvious trend towards improvement was evident during the past years, (iii) the question of whether early implementation of therapy may lead to better clinical outcomes cannot be definitely answered based on existing data and (iv) there is still a very low level of incorporation of the new classification criteria in SSc trials. This review suggests that there may be a window of opportunity in SSc and highlights the need for clinical trials targeting very early/early disease.

Sclerosing Mediastinitis Causing Unilateral Pulmonary Edema Due to Left Atrial and Pulmonary Venous Compression. A Case Report and Literature Review.

Sclerosing mediastinitis (SM), previously named chronic fibrosing mediastinitis, is an inflammatory process that in its end-stage results to sclerosis around the mediastinal structures. SM is quite rare and has been correlated with inflammatory and autoimmune diseases, as well as malignancy. SM may either present in a mild form, with minor symptoms and a benign course or in a more aggressive form with severe pulmonary hypertension and subsequent higher morbidity and mortality. The diagnosis of SM may be difficult and quite challenging, as symptoms depend on the mediastinal structure that is mainly involved; quite often the superior vena cava. However, practically any mediastinal structure may be involved by the fibrotic process, such as the central airways, as well as the pulmonary arteries and veins, leading to obstruction or total occlusion. The latter may be impossible to undergo proper surgical excision of the lesion, and is considered to be a real challenge to the surgeon. We herein report a case of SM that presented with arterial and venous compression. The imaging appearance was that of unilateral pulmonary edema, associated with lung collapse. The case is supplemented by a non-systematic review of the relevant literature.

Sclerosing mediastinitis causing unilateral pulmonary edema due to left atrial and pulmonary venous compression. a case report and literature review

Abstract Sclerosing mediastinitis (SM), previously named chronic fibrosing mediastinitis, is an inflammatory process that in its end-stage results to sclerosis around the mediastinal structures. SM is quite rare and has been correlated with inflammatory and autoimmune diseases, as well as malignancy. SM may either present in a mild form, with minor symptoms and a benign course or in a more aggressive form with severe pulmonary hypertension and subsequent higher morbidity and mortality. The diagnosis of SM may be difficult and quite challenging, as symptoms depend on the mediastinal structure that is mainly involved; quite often the superior vena cava. However, practically any mediastinal structure may be involved by the fibrotic process, such as the central airways, as well as the pulmonary arteries and veins, leading to obstruction or total occlusion. The latter may be impossible to undergo proper surgical excision of the lesion, and is considered to be a real challenge to the surgeon. We herein report a case of SM that presented with arterial and venous compression. The imaging appearance was that of unilateral pulmonary edema, associated with lung collapse. The case is supplemented by a non-systematic review of the relevant literature.

Biologics in SAPHO syndrome: A systematic review.

BACKGROUND: The SAPHO syndrome is a relatively rare clinical entity characterized by a wide range of dermatological and musculoskeletal manifestations. Biologics have been used in cases refractory to conventional treatment. METHODS: We present herein a patient with refractory to treatment SAPHO syndrome who exhibited a dramatic and fast response to IL-17 blockade. Additionally, we performed a systematic review of all cases of patients with SAPHO syndrome treated with biologics to date. RESULTS: We identified 66 cases treated with biologics (45 with TNF blockers, 7 with IL-1 blockers, 13 with biologics targeting the IL-23/IL-17 axis, and 1 with tocilizumab). Data support a positive effect of anti-TNF treatment in SAPHO with a response rate in bone and joint manifestations of 93.3%. Skin disease also improved in 21/29 cases (72.4%). Data related to IL-1 inhibition in SAPHO are encouraging with most patients exhibiting a significant response in musculoskeletal manifestations (6/7, 85.7%). However, IL-1 inhibition is not effective in skin manifestations. Ustekinumab seems to have some efficacy with 2/4 patients responding in skin and 3/5 in bone/joint manifestations. Data related to IL-17 blockade indicate efficacy in skin disease with 4/7 patients responding (57.1%). Joint/bone manifestations improved in 3/8 patients (37.5%). CONCLUSIONS: In SAPHO patients not responding to conventional treatment, TNF blockers appear to be the first choice. In patients failing TNF blockers, IL-1 inhibitors and biologics targeting the IL-17/IL-23 axis could be used.