Clinical, tomographic and functional comparison of sporadic and tuberous sclerosis complex-associated forms of lymphangioleiomyomatosis: a retrospective cohort study.

Background: Lymphangioleiomyomatosis (LAM) is a rare disease that can occur sporadically (S-LAM) or associated with the tuberous sclerosis complex (TSC-LAM). The natural history of LAM is not completely understood, including whether there is a difference between the clinical courses of the two forms. This study aimed to compare the clinical, functional and tomographic features between S-LAM and TSC-LAM, and evaluate the annual rates of change in lung function. Methods: This retrospective cohort study included patients with LAM followed up between 1994 and 2019. Clinical, functional and imaging variables were evaluated, and the lung cysts were automatically quantified. Quality of life and predictors of lung function impairment were accessed, and the annual rate of lung function decline was compared between S-LAM and TSC-LAM. Results: Of the 107 patients included, 77 had S-LAM and 30 had TSC-LAM. Although patients with TSC-LAM had a higher prevalence of renal angiomyolipomas and neurological and dermatological manifestations, pulmonary function tests were similar. Patients with S-LAM had a greater rate of forced expiratory volume in 1 s decline and a higher extent of cysts. Pneumothorax, desaturation in the 6-minute walking test and a higher extent of lung cysts were predictors of functional impairment. A greater impact on vitality and emotional health was observed in the TSC-LAM. Conclusion: Greater functional decline and a higher cystic extension were found in patients with S-LAM. Our study provides a broad clinical, functional and tomographic characterisation of patients with LAM, adding valuable information to the existing evidence to better understand the two forms of the disease.

Parafoveal chondral lesion of the femoral head in patients with femoroacetabular impingement.

Objective: To describe cases of parafoveal chondral lesion of the femoral head in patients with femoroacetabular impingement, correlating the clinical and imaging data. Materials and Methods: This was a retrospective descriptive case series of parafoveal chondral lesion of the femoral head in 21 patients who underwent computed tomography and magnetic resonance arthrography scans of the hip, having then received an imaging-based diagnosis of femoroacetabular impingement. Results: Of the 21 patients evaluated, 15 (71%) had cam-type femoroacetabular impingement, whereas five (24%) had mixed-type impingement, and one (5%) had pincer-type impingement. Twelve patients (57%) had a low frequency of physical activity, which was significantly associated with the presence of cam-type impingement (p = 0.015). Although the extent of the lesion correlated significantly with the acetabular coverage angle (p = 0.04), it did not correlate significantly with the alpha angle or femoral head-neck offset value (p = 0.08 and p = 0.06, respectively). We also found no correlation between the extent of the lesion and the other main parameters that define the femoroacetabular impingement types. Conclusion: This was one of the largest case series of parafoveal chondral lesion of the femoral head in patients with imaging findings of femoroacetabular impingement. The extent of such lesions does not appear to correlate with the parameters of femoroacetabular impingement, with the exception of the acetabular coverage angle.

Objetivo: Descrever casos de lesão condral parafoveal da cabeça femoral em pacientes com impacto femoroacetabular, correlacionando dados clínicos e de imagem. Materiais e Métodos: Esta foi uma série de casos descritiva retrospectiva de lesão condral parafoveal da cabeça femoral em 21 pacientes submetidos a tomografia computadorizada e artrorressonância magnética do quadril e que receberam diagnóstico por imagem de impacto femoroacetabular. Resultados: Dos 21 pacientes avaliados, 15 (71%) tiveram impacto femoroacetabular do tipo cam, enquanto cinco (24%) tiveram impacto do tipo misto e um (5%) teve impacto do tipo pincer. Doze pacientes (57%) apresentaram baixa frequência de atividade física, sendo esta significativamente associada a impacto do tipo cam (p = 0,015). Houve correlação significativa entre a extensão da lesão e o ângulo de cobertura acetabular (p = 0,04), porém, não se correlacionou significativamente com o ângulo alfa ou com o valor do deslocamento cabeça-colo femoral (p = 0,08 e p = 0,06, respectivamente). Também não encontramos correlação entre a extensão da lesão e os outros principais parâmetros que definem os tipos de impacto femoroacetabular. Conclusão: Esta foi uma das maiores casuísticas de lesão condral parafoveal da cabeça femoral em pacientes com achados de imagem de impacto femoroacetabular. A extensão dessas lesões não parece se correlacionar com os parâmetros do impacto femoroacetabular, com exceção do ângulo de cobertura acetabular.

Tomographic pleuropulmonary manifestations in rheumatoid arthritis: a pictorial essay.

Rheumatoid arthritis (RA) is an autoimmune inflammatory and heterogeneous disease that affects several systems, especially the joints. Among the extra-articular manifestations of RA, pleuropulmonary involvement occurs frequently, with different presentations, potentially in all anatomic thoracic compartments, and may determine high morbidity and mortality. The most common pleuropulmonary manifestations in patients with RA include interstitial lung disease (ILD), pleural disease, pulmonary arterial hypertension, rheumatoid lung nodules, airway disease (bronchiectasis and bronchiolitis), and lymphadenopathy. Pulmonary hypertension and ILD are the manifestations with the greatest negative impact in prognosis. HRCT of the chest is essential in the evaluation of patients with RA with respiratory symptoms, especially those with higher risk factors for ILD, such as male gender, smoking, older age, high levels of rheumatoid factor, or positive anti-cyclic citrullinated peptide antibody results. Additionally, other etiologies that may determine tomographic pleuropulmonary manifestations in patients with RA are infections, neoplasms, and drug-induced lung disease. In these scenarios, clinical presentation is heterogeneous, varying from being asymptomatic to having progressive respiratory failure. Knowledge on the potential etiologies causing tomographic pleuropulmonary manifestations in patients with RA coupled with proper clinical reasoning is crucial to diagnose and treat these patients.

Quantitative analysis of pulmonary perfusion with dual-energy CT angiography: comparison of two quantification methods in patients with pulmonary embolism.

The study aimed to evaluate a quantification method of pulmonary perfusion with Dual-Energy CT Angiography (DE-CTA) normalized by lung density in the prediction of outcome in acute pulmonary embolism (PE). In this prospective study with CTA scans acquired with different breathing protocols, two perfusion parameters were calculated: %PBV (relative value of PBV, expressed per unit volume) and PBVm (PBV normalized by lung density, expressed per unit mass). DE-CTA parameters were correlated with simplified pulmonary embolism severity index (sPESI) and with outcome groups, alone and in combinationwith tomographic right-to-left ventricular ratios (RV/LV). PBVm showed significant correlation with sPESI. PBVm presented higher accuracy than %PBV In the prediction of ICU admission or death in patients with PE, with the best performance when combined with RV/LV volumetric ratio.

Tomographic pleuropulmonary manifestations in rheumatoid arthritis: a pictorial essay / Manifestações pleuropulmonares tomográficas na artrite reumatoide: ensaio pictórico

ABSTRACT Rheumatoid arthritis (RA) is an autoimmune inflammatory and heterogeneous disease that affects several systems, especially the joints. Among the extra-articular manifestations of RA, pleuropulmonary involvement occurs frequently, with different presentations, potentially in all anatomic thoracic compartments, and may determine high morbidity and mortality. The most common pleuropulmonary manifestations in patients with RA include interstitial lung disease (ILD), pleural disease, pulmonary arterial hypertension, rheumatoid lung nodules, airway disease (bronchiectasis and bronchiolitis), and lymphadenopathy. Pulmonary hypertension and ILD are the manifestations with the greatest negative impact in prognosis. HRCT of the chest is essential in the evaluation of patients with RA with respiratory symptoms, especially those with higher risk factors for ILD, such as male gender, smoking, older age, high levels of rheumatoid factor, or positive anti-cyclic citrullinated peptide antibody results. Additionally, other etiologies that may determine tomographic pleuropulmonary manifestations in patients with RA are infections, neoplasms, and drug-induced lung disease. In these scenarios, clinical presentation is heterogeneous, varying from being asymptomatic to having progressive respiratory failure. Knowledge on the potential etiologies causing tomographic pleuropulmonary manifestations in patients with RA coupled with proper clinical reasoning is crucial to diagnose and treat these patients.

RESUMO A artrite reumatoide (AR) é uma doença inflamatória autoimune e heterogênea que afeta vários sistemas, principalmente as articulações. Dentre as manifestações extra-articulares da AR, o acometimento pleuropulmonar ocorre com frequência, com diferentes apresentações, potencialmente em todos os compartimentos anatômicos do tórax e pode determinar alta morbidade e mortalidade. As manifestações pleuropulmonares mais comuns em pacientes com AR incluem doença pulmonar intersticial (DPI), doença pleural, hipertensão arterial pulmonar, nódulos pulmonares reumatoides, doença das vias aéreas (bronquiectasia e bronquiolite) e linfadenopatia. A hipertensão pulmonar e a DPI são as manifestações com maior impacto negativo no prognóstico. A TCAR de tórax é essencial na avaliação de pacientes com AR sintomáticos respiratórios, principalmente aqueles com fatores de risco maiores para DPI, como sexo masculino, tabagismo, idade mais avançada, níveis elevados de fator reumatoide ou anticorpos antipeptídeos citrulinados cíclicos positivos. Além disso, outras etiologias que podem determinar manifestações pleuropulmonares tomográficas em pacientes com AR são infecções, neoplasias e doença pulmonar induzida por drogas. Nesses cenários, a apresentação clínica é heterogênea, variando de ausência de sintomas a insuficiência respiratória progressiva. O conhecimento das possíveis etiologias causadoras de manifestações pleuropulmonares tomográficas em pacientes com AR, aliado a um raciocínio clínico adequado, é fundamental para o diagnóstico e tratamento desses pacientes.

Parafoveal chondral lesion of the femoral head in patients with femoroacetabular impingement / Lesão condral parafoveal da cabeça femoral em pacientes com impacto femoroacetabular

Abstract Objective: To describe cases of parafoveal chondral lesion of the femoral head in patients with femoroacetabular impingement, correlating the clinical and imaging data. Materials and Methods: This was a retrospective descriptive case series of parafoveal chondral lesion of the femoral head in 21 patients who underwent computed tomography and magnetic resonance arthrography scans of the hip, having then received an imaging-based diagnosis of femoroacetabular impingement. Results: Of the 21 patients evaluated, 15 (71%) had cam-type femoroacetabular impingement, whereas five (24%) had mixed-type impingement, and one (5%) had pincer-type impingement. Twelve patients (57%) had a low frequency of physical activity, which was significantly associated with the presence of cam-type impingement (p = 0.015). Although the extent of the lesion correlated significantly with the acetabular coverage angle (p = 0.04), it did not correlate significantly with the alpha angle or femoral head-neck offset value (p = 0.08 and p = 0.06, respectively). We also found no correlation between the extent of the lesion and the other main parameters that define the femoroacetabular impingement types. Conclusion: This was one of the largest case series of parafoveal chondral lesion of the femoral head in patients with imaging findings of femoroacetabular impingement. The extent of such lesions does not appear to correlate with the parameters of femoroacetabular impingement, with the exception of the acetabular coverage angle.

Resumo Objetivo: Descrever casos de lesão condral parafoveal da cabeça femoral em pacientes com impacto femoroacetabular, correlacionando dados clínicos e de imagem. Materiais e Métodos: Esta foi uma série de casos descritiva retrospectiva de lesão condral parafoveal da cabeça femoral em 21 pacientes submetidos a tomografia computadorizada e artrorressonância magnética do quadril e que receberam diagnóstico por imagem de impacto femoroacetabular. Resultados: Dos 21 pacientes avaliados, 15 (71%) tiveram impacto femoroacetabular do tipo cam, enquanto cinco (24%) tiveram impacto do tipo misto e um (5%) teve impacto do tipo pincer. Doze pacientes (57%) apresentaram baixa frequência de atividade física, sendo esta significativamente associada a impacto do tipo cam (p = 0,015). Houve correlação significativa entre a extensão da lesão e o ângulo de cobertura acetabular (p = 0,04), porém, não se correlacionou significativamente com o ângulo alfa ou com o valor do deslocamento cabeça-colo femoral (p = 0,08 e p = 0,06, respectivamente). Também não encontramos correlação entre a extensão da lesão e os outros principais parâmetros que definem os tipos de impacto femoroacetabular. Conclusão: Esta foi uma das maiores casuísticas de lesão condral parafoveal da cabeça femoral em pacientes com achados de imagem de impacto femoroacetabular. A extensão dessas lesões não parece se correlacionar com os parâmetros do impacto femoroacetabular, com exceção do ângulo de cobertura acetabular.

Lobar pulmonary perfusion quantification with dual-energy CT angiography: Interlobar variability and relationship with regional clot burden in pulmonary embolism.

Purpose: Semi-automated lobar segmentation tools enable an anatomical assessment of regional pulmonary perfusion with Dual-Energy CTA (DE-CTA). We aimed to quantify lobar pulmonary perfusion with DE-CTA, analyze the perfusion distribution among the pulmonary lobes in subjects without cardiopulmonary diseases and assess the correlation between lobar perfusion and regional endoluminal clots in patients with acute pulmonary embolism (PE). Methods: We evaluated 151 consecutive subjects with suspected PE and without cardiopulmonary comorbidities. DE-CTA derived perfused blood volume (PBV) of each pulmonary lobe was measured applying a semi-automated lobar segmentation technique. In patients with PE, blood clot location was assessed, and CT-based vascular obstruction index of each lobe (CTOIlobe) was calculated and classified into three groups: CTOIlobe= 0, low CTOIlobe (1-50%) and high CTOIlobe (>50%). Results: Among patients without PE (103/151, 68.2%), median lobar PBV was 13.7% (IQR 10.2-18.0%); the right middle lobe presented lower PBV when compared to all the other lobes (p < .001). In patients with PE (48/151, 31.8%), lobar PBV was 12.6% (IQR 9.6-15.7%), 13.7% (IQR 10.1-16.7%) and 6.5% (IQR 5.1-10.2%) in the lobes with CTOIlobe= 0, low CTOIlobe and high CTOIlobe scores, respectively, with a significantly decreased PBV in the lobes with high CTOIlobe score (p < .001). ROC analysis of lobar PBV for prediction of high CTOIlobe score revealed AUC of 0.847 (95%CI 0.785-0.908). Conclusion: Pulmonary perfusion was heterogeneously distributed along the pulmonary lobes in patients without cardiopulmonary diseases. In patients with PE, the lobes with high vascular obstruction score (CTOIlobe> 50%) presented a decreased lobar perfusion.

Natural history of incidental sporadic and tuberous sclerosis complex associated lymphangioleiomyomatosis.

Lymphangioleiomyiomatosis (LAM) is a rare disease affecting women in childbearing age. A sporadic form (S-LAM) affecting previously healthy women, and a form associated with Tuberous Sclerosis Complex (TSC-LAM) are described. Some data suggested that TSC-LAM could be a milder disease compared to S-LAM. To investigate whether the different disease behavior is real or due to overdiagnosis of screened TSC women, we compared the natural history of S-LAM and TSC-LAM in patients with incidental diagnosis. Clinical, and functional data from 52 patients (23 with S-LAM and 29 with TSC-LAM) were analysed. At diagnosis functional impairment was mild without differences between groups [FEV1 % pred was 97% (88-105) and 94% (82-106) in TSC-LAM and S-LAM, respectively, p = 0.125]. Patients with S-LAM had less renal angiomyolipoma, and lower VEGF-D serum levels than TSC-LAM. There was no difference in the baseline extent of pulmonary cysts on CT scan and no difference in yearly rate of functional decline between TSC-LAM, and S-LAM patients [e.g. yearly rate of decline of FEV1 % pred was -0.51 (-1.59-2.24) and -0.90 (-1.92--0.42) in TSC-LAM and S-LAM, respectively, p = 0.265]. In conclusion, the natural history of TSC-LAM and S-LAM, when a potential selection bias due to screening in the latter group is balanced, is similar. Our study suggests that the prevalence of S-LAM can be significantly underestimated due to a tendency to diagnosis more frequently patients with more severe impairment, without identifying several ones with asymptomatic disease.

"Pop in a scope": attempt to decrease the rate of unnecessary nontherapeutic laparotomies in hemodynamically stable patients with thoracoabdominal penetrating injuries.

BACKGROUND: Management of patients with thoracoabdominal penetrating injuries is challenging. Thoracoabdominal penetrating trauma may harbor hollow viscus injuries in both thoracic and abdominal cavities and occult diaphragmatic lesions. While radiological tests show poor diagnostic performance in these situations, evaluation by laparoscopy is highly sensitive and specific. Furthermore, minimally invasive surgery may avoid unnecessary laparotomies, despite concerns regarding complication and missed injury rates. The objective of the present study is to evaluate the diagnostic and therapeutic performance of laparoscopy in stable patients with thoracoabdominal penetrating injuries. METHODS: Retrospective analysis of hemodynamically stable patients with thoracoabdominal penetrating wounds was managed by laparoscopy. We collected data regarding the profile of the patients, the presence of diaphragmatic injury, perioperative complications, and the conversion rate. Preoperative imaging tests were compared to laparoscopy in terms of diagnostic accuracy. RESULTS: Thirty-one patients were included, and 26 (84%) were victims of a stab wound. Mean age was 32 years. Ninety-three percent were male. Diaphragmatic lesions were present in 18 patients (58%), and 13 (42%) had associated injuries. There were no missed injuries and no conversions. Radiography and computerized tomography yielded an accuracy of 52% and 75%, respectively. CONCLUSION: Laparoscopy is a safe diagnostic and therapeutic procedure in stable patients with thoracoabdominal penetrating wound, with low complication rate, and may avoid unnecessary laparotomies. The poor diagnostic performance of preoperative imaging exams supports routine laparoscopic evaluation of the diaphragm to exclude injuries in these patients.

The Diagnostic Challenge of an Infrequent Spectrum of Cryptococcus Infection.

Cryptococcal infection results from inhalation of fungal spores and usually is confined to the lungs, but may disseminate systemically. Radiologically, cryptococcal infection has multiple forms of presentation. The diagnosis is usually based on fungal isolation from cultured clinical specimens. Long term antifungal therapy is recommended, but surgical procedures may eventually be necessary when large thoracic symptomatic masses are present. We report a case of a 41-year-old male, immunocompetent, investigating a palpable mass in the left supraclavicular region associated with unintentional weight loss over the last three months. He also reported chest pain in this period. Chest X-ray, ultrasonography, and computed tomography were performed, which diagnosed a mediastinal and left supraclavicular mass, interpreted as lymph node conglomerates of unknown etiology. He also underwent a biopsy of the left supraclavicular mass for etiological determination by histopathology, which confirmed cryptococcosis infection. Although very infrequent, mediastinal cryptococcal infection (simulating masses) is a challenging but important differential diagnosis of benign and malignant lesions, since its treatment is usually clinical.