Progression of pulmonary cysts in Birt-Hogg-Dubé syndrome: longitudinal thoracic computed tomography study with quantitative assessment.

BACKGROUND: Birt-Hogg-Dubé (BHD) syndrome is a rare autosomal dominant disorder characterized by fibrofolliculomas, renal tumors, pulmonary cysts, and recurrent pneumothorax. Pulmonary cysts are the cause of recurrent pneumothorax, which is one of the most important factors influencing patient quality of life. It is unknown whether pulmonary cysts progress with time or influence pulmonary function in patients with BHD syndrome. This study investigated whether pulmonary cysts progress during long-term follow-up (FU) by using thoracic computed tomography (CT) and whether pulmonary function declines during FU. We also evaluated risk factors for pneumothorax in patients with BHD during FU. METHODS: Our retrospective cohort included 43 patients with BHD (25 women; mean age, 54.2 ± 11.7 years). We evaluated whether cysts progress by visual assessment and quantitative volume analysis using initial and serial thoracic CT. The visual assessment included the size, location, number, shape, distribution, presence of a visible wall, fissural or subpleural cysts, and air-cuff signs. In CT data obtained from a 1-mm section from 17 patients, the quantitative assessment was performed by measuring the volume of the low attenuation area using in-house software. We evaluated whether the pulmonary function declined with time on serial pulmonary function tests (PFT). Risk factors for pneumothorax were analyzed using multiple regression analysis. RESULTS: On visual assessment, the largest cyst in the right lung showed a significant interval increase in size (1.0 mm/year, p = 0.0015; 95% confidence interval [CI], 0.42-1.64) between the initial and final CT, and the largest cyst in the left lung also showed significant interval increase in size (0.8 mm/year, p < 0.001, 95% CI; -0.49-1.09). On quantitative assessment, cysts had a tendency to gradually increase in size. In 33 patients with available PFT data, FEV1pred%, FEV1/FVC, and VCpred% showed a statistically significant decrease with time (p < 0.0001 for each). A family history of pneumothorax was a risk factor for the development of pneumothorax. CONCLUSIONS: The size of pulmonary cysts progressed over time in longitudinal follow-up thoracic CT in patients with BHD, and pulmonary function had slightly deteriorated by longitudinal follow-up PFT.

Assessment of naive indolent lymphoma using whole-body diffusion-weighted imaging and T2-weighted MRI: results of a prospective study in 30 patients.

BACKGROUND: We prospectively evaluated the diagnostic utility of whole-body diffusion-weighted imaging with background body signal suppression and T2-weighted short-tau inversion recovery MRI (WB-DWIBS/STIR) for the pretherapeutic staging of indolent lymphoma in 30 patients. METHODS: This prospective study included 30 treatment-naive patients with indolent lymphomas who underwent WB-DWIBS/STIR and conventional imaging workup plus biopsy. The pretherapeutic staging agreement, sensitivity, and specificity of WB-DWIBS/STIR were investigated with reference to the multimodality and multidisciplinary consensus review for nodal and extranodal lesions excluding bone marrow. RESULTS: In the pretherapeutic staging, WB-DWIBS/STIR showed very good agreement (κ = 0.96; confidence interval [CI], 0.88-1.00), high sensitivity (93.4-95.1%), and high specificity (99.0-99.4%) for the whole-body regions. These results were similar to those of 18F-FDG-PET/CT, except for the sensitivity for extranodal lesions. For extranodal lesions, WB-DWIBS/STIR showed higher sensitivity compared to 18F-FDG-PET/CT for the whole-body regions (94.9-96.8% vs. 79.6-86.3%, P = 0.058). CONCLUSION: WB-DWIBS/STIR is an effective modality for the pretherapeutic staging of indolent lymphoma, and it has benefits when evaluating extranodal lesions, compared with 18F-FDG-PET/CT.

Role of whole-body MRI for treatment response assessment in multiple myeloma: comparison between clinical response and imaging response.

BACKGROUND: Whole-body MRI (WB-MRI) including diffusion-weighted image (DWI) have been widely used in patients with multiple myeloma. However, evidence for the value of WB-MRI in the evaluation of treatment response remains sparse. Therefore, we evaluated the role of WB-MRI in the response assessment. METHODS: In our WB-MRI registry, we searched multiple myeloma patients treated with chemotherapy who underwent both baseline and follow-up WB-MRI scans. Clinical responses were categorized as complete response (CR), partial response (PR), stable disease (SD), or progressive disease (PD), using IMWG criteria. Using RECIST 1.1, MD Anderson (MDA) criteria, and MDA-DWI criteria, imaging responses on WB-MRI were rated as CR, PR, SD, or PD by two radiologists independently. Then, discrepancy cases were resolved by consensus. Weighted Kappa analysis was performed to evaluate agreement between the imaging and clinical responses. The diagnostic accuracy of image responses in the evaluation of clinical CR, objective response (CR and PR), and PD was calculated. RESULTS: Forty-two eligible patients were included. There was moderate agreement between imaging and clinical responses (κ = 0.54 for RECIST 1.1, κ = 0.58 for MDA criteria, κ = 0.69 for MDA-DWI criteria). WB-MRI showed excellent diagnostic accuracy in assessment of clinical PD (sensitivity 88.9%, specificity 94.7%, positive predictive value [PPV] 84.2%, negative predictive value [NPV] 96.4% in all three imaging criteria). By contrast, WB-MRI showed low accuracy in assessment of clinical CR (sensitivity 4.5%, specificity 98.1%, PPV 50.0%, NPV 71.2% in all three imaging criteria). As to the clinical objective response, the diagnostic accuracy was higher in MDA-DWI criteria than RECIST 1.1 and MDA criteria (sensitivity/specificity/PPV/NPV, 84.2%/94.4%/98.0%/65.4, 54.4%/100%/100%/40.9, and 61.4%/94.4%/97.2%/43.6%, respectively). CONCLUSIONS: In the imaging response assessment of multiple myeloma, WB-MRI showed excellent performance in the evaluation of PD, but not in the assessment of CR or objective response. When adding DWI to imaging response criteria, diagnostic accuracy for objective response was improved and agreement between imaging and clinical responses was increased.

Quantitative Assessment of Global and Regional Air Trappings Using Non-Rigid Registration and Regional Specific Volume Change of Inspiratory/Expiratory CT Scans: Studies on Healthy Volunteers and Asthmatics.

OBJECTIVE: The purpose of this study was to compare air trapping in healthy volunteers with asthmatics using pulmonary function test and quantitative data, such as specific volume change from paired inspiratory CT and registered expiratory CT. MATERIALS AND METHODS: Sixteen healthy volunteers and 9 asthmatics underwent paired inspiratory/expiratory CT. ΔSV, which represents the ratio of air fraction released after exhalation, was measured with paired inspiratory and anatomically registered expiratory CT scans. Air trapping indexes, ΔSV0.4 and ΔSV0.5, were defined as volume fraction of lung below 0.4 and 0.5 ΔSV, respectively. To assess the gravity effect of air-trapping, ΔSV values of anterior and posterior lung at three different levels were measured and ΔSV ratio of anterior lung to posterior lung was calculated. Color-coded ΔSV map of the whole lung was generated and visually assessed. Mean ΔSV, ΔSV0.4, and ΔSV0.5 were compared between healthy volunteers and asthmatics. In asthmatics, correlation between air trapping indexes and clinical parameters were assessed. RESULTS: Mean ΔSV, ΔSV0.4, and ΔSV0.5 in asthmatics were significantly higher than those in healthy volunteer group (all p < 0.05). ΔSV values in posterior lung in asthmatics were significantly higher than those in healthy volunteer group (p = 0.049). In asthmatics, air trapping indexes, such as ΔSV0.5 and ΔSV0.4, showed negative strong correlation with FEF25-75, FEV1, and FEV1/FVC. ΔSV map of asthmatics showed abnormal geographic pattern in 5 patients (55.6%) and disappearance of anterior-posterior gradient in 3 patients (33.3%). CONCLUSION: Quantitative assessment of ΔSV (the ratio of air fraction released after exhalation) shows the difference in extent of air trapping between health volunteers and asthmatics.

Collateral ventilation in a canine model with bronchial obstruction: assessment with xenon-enhanced dual-energy CT.

PURPOSE: To determine the feasibility of the use of xenon-enhanced dynamic dual-energy computed tomography (CT) for visualization and quantitative assessment of collateral ventilation in a canine model with bronchial obstruction. MATERIALS AND METHODS: This study was approved by the institutional animal care and use committee. One segmental bronchus was occluded in nine dogs (weight range, 20-25 kg). Dynamic dual-energy CT scanning was performed by using dual-source CT during the wash-in-washout study of xenon inhalation via mechanical ventilation. Imaging parameters were 14 x 1.2-mm collimation, 40 mAs (effective) at 140 kV and 170 mAs (effective) at 80 kV, pitch of 0.45, and 0.33-second rotation time. By using dual-energy software, CT images and xenon maps were reconstructed. CT attenuation values were measured in the airways proximal to obstruction (AW(PROX)) and airways distal to obstruction (AW(DIST)) and at the parenchyma with patent airways (P(PATE)) and parenchyma with obstructed airways (P(OBST)). CT attenuation values on dynamic xenon maps were plotted with exponential function; ventilation parameters, including velocity of ventilation (K value), magnitude of ventilation (A value), and time of arrival (TOA), were calculated on the basis of the Kety model. RESULTS: In all animals, delayed and weaker xenon enhancement was identified at the airway and parenchyma distal to obstruction. For the A value, in the wash-in study, the differences between AW(PROX) and AW(DIST) and between P(PATE) and P(OBST) were significant (71.80 and 57.64, P = .05; 51.86 and 37.52 HU, P = .02). The K value of P(OBST) was lower than that of P(PATE) in the wash-in study (0.006 and 0 .010, P = .06). Mean and standard deviation for TOA were observed in the following increasing order: AW(PROX) ([3.50 +/- 7.70] x 10(-6) sec), P(PATE) (4.58 +/- 2.83), AW(DIST) (9.20 +/- 6.87), and P(OBST) (21.00 +/- 13.44). CONCLUSION: Collateral ventilation in a canine model with bronchial obstruction can be quantitatively assessed by using xenon-enhanced dynamic dual-energy CT.

Dual-energy CT for assessment of the severity of acute pulmonary embolism: pulmonary perfusion defect score compared with CT angiographic obstruction score and right ventricular/left ventricular diameter ratio.

OBJECTIVE: The purpose of this study was to prospectively evaluate the usefulness of scoring perfusion defects on perfusion images at dual-energy CT for assessment of the severity of pulmonary embolism. SUBJECTS AND METHODS: Thirty patients (13 men, 17 women; mean age, 55 +/- 15 [SD] years; range, 26-81 years) with pulmonary thromboembolism underwent dual-source CT at two voltages (140 and 80 kV). The weighted average image of two acquisitions was used for CT angiograms, and a color-coded iodine image was used for perfusion images. Two thoracic radiologists with 15 and 6 years of clinical experience independently assigned perfusion defect scores to perfusion images and both a CT angiographic (CTA) obstruction score and right ventricular-to-left ventricular (RV/LV) diameter ratio to CT angiograms. The CTA obstruction score was based on the Qanadli method. The perfusion defect score was defined as Sigma (n . d) / 40 x 100, where n is the number of segments and d is the degree of perfusion from 0 to 2. Correlations between perfusion defect score, CTA obstruction score, and RV/LV diameter ratio were evaluated. Agreement between perfusion defect score and CTA score was assessed per patient and per segment. Interobserver agreement regarding perfusion defect and CTA obstruction scores was analyzed. RESULTS: Perfusion defect and CTA obstruction scores had good correlation with RV/LV diameter ratio (r = 0.69, r = 0.66; all p < 0.001). Per patient, correlation between perfusion defect score and CTA obstruction score also was good (reader 1, r = 0.87; reader 2, r = 0.85; all p < 0.001). Per segment, moderate agreement was found between perfusion defect score and CTA obstruction score (reader 1, kappa = 0.56; reader 2, kappa = 0.51; all p < 0.05). Both readers were in strong agreement on perfusion defect score and CTA obstruction score. CONCLUSION: The proposed perfusion defect score had good correlation with RV/LV diameter ratio and CTA obstruction score. Therefore, acquisition of perfusion images at dual-energy CT may be helpful for assessing the severity of acute pulmonary embolism.