Lung disease assessment in primary ciliary dyskinesia: a comparison between chest high-field magnetic resonance imaging and high-resolution computed tomography findings

Abstract Background Primary ciliary dyskinesia (PCD) is associated with pulmonary involvement that requires periodical assessment. Chest high-resolution computed tomography (HRCT) has become the method of choice to evaluate chronic lung disease, but entails exposure to ionizing radiation. Magnetic resonance imaging (MRI) has been proposed as a potential radiation-free technique in several chest disorders. Aim of our study is to evaluate whether high-field MRI is as effective as HRCT in identifying PCD pulmonary abnormalities. We also analyzed the relationships between the severity and extension of lung disease, and functional data. Methods Thirteen PCD patients (8 children/5 adults; median age, 15.2 yrs) underwent chest HRCT and high-field 3T MRI, spirometry, and deep throat or sputum culture. Images were scored using a modified version of the Helbich system. Results HRCT and MRI total scores were 12 (range, 6–20) and 12 (range, 5–17), respectively. Agreement between HRCT and MRI scores was good or excellent (r > 0.8). HRCT and MRI total scores were significantly related to forced vital capacity (r = -0.5, p = 0.05; and r = -0.7, p = 0.009, respectively) and forced expiratory volume at 1 second (r = -0.6, p = 0.03; and r = -0.7, p = 0.009, respectively). Conclusion Chest high-field 3T MRI appears to be as effective as HRCT in assessing the extent and severity of lung abnormalities in PCD. MRI scores might be used for longitudinal assessment and be an outcome surrogate in future studies.</p

Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study

BACKGROUND: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are increasingly compared. There are no chest magnetic resonance imaging (MRI) comparative studies of PCD and CF. We assessed clinical, functional, microbiological and MRI findings in PCD and mild CF patients in order to evaluate different expression of lung disease. METHODS: Twenty PCD (15.1 years) and 20 CF subjects with mild respiratory impairment (16 years, 70% with pancreatic insufficiency) underwent MRI, spirometry, and sputum cultures when clinically stable. MRI was scored using the modified Helbich system. RESULTS: PCD was diagnosed later than CF (9.9 versus 0.6 years, p = 0.03), despite earlier symptoms (0.1 versus 0.6 years, p = 0.02). In the year preceding the study, patients from both groups underwent two systemic antibiotic courses (p = 0.48). MRI total scores were 11.6 ± 0.7 and 9.1 ± 1 in PCD and CF, respectively. FEV1 and FVC Z-scores were -1.75 (range, -4.6-0.7) and -0.6 (-3.9-1.8) in PCD, and -0.9 (range, -5.4-2.3) and -0.3 (-3.4-2.5) in CF, respectively. No difference was found between lung function or structure, despite a higher MRI subscore of collapse/consolidation in PCD versus CF (1.6 ± 0.1 and 0.6 ± 0.2, p < 0.001). These findings were confirmed after data-control for diagnostic delay. Pseudomonas aeruginosa and Staphylococcus aureus were more frequent in CF than in PCD (p = 0.05 and p = 0.003, respectively). CONCLUSIONS: MRI is a valuable radiation-free tool for comparative PCD and CF lung disease assessment. Patients with PCD may exhibit similar MRI and lung function changes as CF subjects with mild pulmonary disease. Delay in PCD diagnosis is unlikely the only determinant of similarities

Diagnostic yield of rapid on-site evaluation transbronchial needle aspiration versus conventional transbronchial needle aspiration: a single center experience

Trans-bronchial needle aspiration allows lymph node sampling in several thoracic conditions; the ability of Rapid On-Site Evaluation (ROSE) to predict the final diagnosis in this setting has not been well characterized. We performed a retrospective study to establish the utility of ROSE in the diagnosis of thoracic diseases with mediastinal lymph node involvement. We retrospectively reviewed 297 patients with hilar-mediastinal lymph node enlargement detected at CT scan from January 2013 to April 2016. 201 patients underwent conventional TBNA; in 96 patients, TBNA procedure was performed by on-site presence of a team of pathologists and research morphologists. Lung neoplasms, sarcoidosis, infections and lymphoma were the most common diseases diagnosed with TBNA samples. TBNA simultaneously performed in combination with ROSE produced an increase in percentage of appropriate samples compared to single cTBNA (adequate samples cTBNA vs ROSE-TBNA: 73% vs 81%; p<0.05). Our observations indicate an increase in adequacy of fine needle aspirations and increased diagnostic yield in the ROSE group. In conclusions, ROSE may serve to reduce procedure time and enhance sample triaging therefore limiting the need for further invasive diagnostic testing

Lymphoepithelial Pancreatic Cyst: An Atypical Benign Pancreatic Mass Presenting with a “Cheerios-Like” Appearance

Context Lymphoepithelial cyst of the pancreas is a rare benign lesion which often presents as an incidental radiological finding, but may cause symptoms, such as abdominal pain and nausea. It can occur at any location in the pancreas. The differential diagnosis includes primary splenic cysts, pseudocysts, mucinous cystic neoplasms of the pancreas, left adrenal cysts, duplication cysts and retroperitoneal cysts. Some of these diagnoses can be excluded by MRI; however, for the final diagnosis, fine needle aspiration or surgical resection may be necessar