New severity assessment in cystic fibrosis: signal intensity and lung volume compared to LCI and FEV1: preliminary results.

OBJECTIVES: Magnetic resonance imaging (MRI) aids diagnosis in cystic fibrosis (CF) but its use in quantitative severity assessment is under research. This study aims to assess changes in signal intensity (SI) and lung volumes (Vol) during functional MRI and their use as a severity assessment tool in CF patients. METHODS: The CF intra-hospital standard chest 1.5 T MRI protocol comprises of very short echo-time sequences in submaximal in- and expiration for functional information. Quantitative measurements (Vol/SI at in- and expiration, relative differences (Vol_delta/SI_delta), and cumulative histograms for normalized SI values across the expiratory lung volume) were assessed for correlation to pulmonary function: lung clearance index (LCI) and forced expiratory volume in 1 s (FEV1). RESULTS: In 49 patients (26 male, mean age 17 ± 7 years) significant correlation of Vol_delta and SI_delta (R = 0.86; p < 0.0001) during respiration was observed. Individual cumulated histograms enabled severity disease differentiation (mild, severe) to be visualized (defined by functional parameter: LCI > 10). The expiratory volume at a relative SI of 100% correlated significantly to LCI (R = 0.676 and 0.627; p < 0.0001) and FEV1 (R = - 0.847 and - 0.807; p < 0.0001). Clustering patients according to Vol_SI_100 showed that an amount of ≤ 4% was related to normal, while an amount of > 4% was associated with pathological pulmonary function values. CONCLUSION: Functional pulmonary MRI provides a radiation-free severity assessment tool and can contribute to early detection of lung impairment in CF. Lung volume with SI below 100% of the inspiratory volume represents overinflated tissue; an amount of 4% of the expiratory lung volume was a relevant turning point. KEY POINTS: • Signal intensity and lung volumes are used as potential metric parameters for lung impairment. • Quantification of trapped air impacts on therapy management. • Functional pulmonary MRI can contribute to early detection of lung impairment.

Background parenchymal enhancement in pregnancy-associated breast cancer: a hindrance to diagnosis?

PURPOSE: The purpose of this study is to investigate the detectability of pregnancy-associated breast cancer (PABC) in lactating glandular tissue on magnetic resonance imaging (MRI) by using pre- and post-contrast acquisitions and their derived postprocessed images and compare these results to ultrasound (US) and mammography (MG). MATERIALS AND METHODS: We reviewed the electronic database for women with PABC and existing breast MRI. MR images (T2-weighted short inversion-recovery sequence [STIR], dynamic contrast-enhanced T1-weighted gradient echo sequence and postprocessed subtraction images [early post-contrast minus pre-contrast]) were retrospectively evaluated (image quality, parenchymal/tumour enhancement kintetics, tumour size and additional lesions). Supplemental subtraction images (latest post-contrast minus early post-contrast) to reduce plateau enhancement were additionally calculated and tumour conspicuity and size were measured. Findings were compared to US and MG reports. RESULTS: Nineteen patients (range 27-42 years) were included. Background parenchymal enhancement (BPE) was minimal (n=1), mild (n=3), moderate (n=7) and marked (n=8) with kinetics measured plateau (n=8), continuous (n=10) and not quantifiable (n=1). Tumour kinetics presented wash-out (n=17) and plateau (n=2). Eighteen of nineteen tumours were identified on the supplemental subtraction images. All tumours were visible on US; 12/19 were visible on MG (63.2%). MRI detected additional malignant lesions in two patients. CONCLUSION: Despite high BPE of the lactating breast, MRI securely detects carcinomas and identifies satellite lesions. By using supplemental subtraction images, background enhancement can be eliminated to facilitate diagnosis. US remains a reliable diagnostic tool, but additional MRI is recommended to rule out satellite/contralateral lesions. MG interpretations can be difficult due to high parenchymal density. KEY POINTS: • Despite high background enhancement, MRI of the breast confidently detects carcinomas and identifies further lesions in the lactating breast. • By using supplemental subtraction images, background enhancement in the lactating breast can be eliminated to facilitate diagnosis. • US remains a reliable diagnostic tool. Mammography can be limited due to extremely dense breast tissue related to lactation.

A semiquantitative MRI-Score can predict loss of lung function in patients with cystic fibrosis: Preliminary results.

OBJECTIVES: To evaluate the applicability of a semiquantitative MRI scoring system (MR-CF-S) as a prognostic marker for clinical course of cystic fibrosis (CF) lung disease. METHODS: This observational study of a single-centre CF cohort included a group of 61 patients (mean age 12.9 ± 4.7 years) receiving morphological and functional pulmonary MRI, pulmonary function testing (PFT) and follow-up of 2 years. MRI was analysed by three raters using MR-CF-S. The inter-rater agreement, correlation of score categories with forced expiratory volume in 1 s (FEV1) at baseline, and the predictive value of clinical parameters, and score categories was assessed for the whole cohort and a subgroup of 40 patients with moderately impaired lung function. RESULTS: The inter-rater agreement of MR-CF-S was sufficient (mean intraclass correlation coefficient 0.92). MR-CF-S (-0.62; p < 0.05) and most of the categories significantly correlated with FEV1. Differences between patients with relevant loss of FEV1 (>3%/year) and normal course were only significant for MR-CF-S (p < 0.05) but not for clinical parameters. Centrilobular opacity (CO) was the most promising score category for prediction of a decline of FEV1 (area under curve: whole cohort 0.69; subgroup 0.86). CONCLUSIONS: MR-CF-S is promising to predict a loss of lung function. CO seems to be a particular finding in CF patients with an abnormal course. KEY POINTS: • Lung imaging is essential in the diagnostic work-up of CF patients • MRI serves as a powerful, radiation-free modality in paediatric CF patients • Observational single-centre study showed significant correlation of MR-CF score and FEV 1 • MR-CF score is promising in predicting a loss of lung function.

Magnetic resonance imaging of vaginal support structure before and after Vecchietti procedure in women with Mayer-Rokitansky-Küster-Hauser syndrome.

INTRODUCTION: It is unclear how pelvic floor supporting structures might be affected by the absence of the vagina. It was the aim of this prospective study to analyze the magnetic resonance imaging morphology of pelvic support prior and after a Vecchietti procedure in women suffering Mullerian agenesis (Mayer-Rokitansky-Küster-Hauser syndrome). MATERIAL AND METHODS: 26 women with a diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome associated vaginal agenesis were recruited prospectively prior to the laparoscopic creation of a neovagina according to the Vecchietti procedure. The primary outcome measure was the magnetic resonance imaging morphology of supporting structures. Secondary outcome measures were anatomical and functional vaginal length. Follow up was conducted six months after surgery. RESULTS: Twenty-six women were analyzed. Mean age was 19.8 ± 4.4 years (±SD) and mean body mass index was 23.7 ± 4.3 kg/m2 (±SD). All were Caucasian. Supporting structures consistent with cardinal and uterosacral ligaments were visible on magnetic resonance imaging in all cases (100%). There were no levator ani defects. The vaginal apex could be visualized postoperatively in 12 women (46.2%) reaching up to Level I. The vagina was visible in both Level II and III with normal relations to the pelvic walls in all cases. On gynecological examination, vaginal length was 8.8 ± 2.1 cm (mean ± SD) anatomically and 10.2 ± 2.2 cm (mean ± SD) functionally. CONCLUSIONS: The preoperative presence of pelvic support structures into which the vagina is lengthened by the surgery likely explains the uncommon occurrence of vaginal prolapse in women who had the Vecchietti procedure.

Two patients with intestinal failure requiring home parenteral nutrition, a NOD2 mutation and tuberculous lymphadenitis.

BACKGROUND: Mutations in the NOD2 gene are a significant risk factor to acquire intestinal failure requiring home parenteral nutrition. Tuberculous lymphadenitis is the main manifestation of extrapulmonary tuberculosis. Defects in the innate immunity, including NOD2 mutations, may increase the risk for acquiring infections caused by M. tuberculosis. An association of intestinal failure, mutations in the NOD2 gene and tuberculous lymphadenitis has not been described before. CASE PRESENTATION: We report of two patients with intestinal failure secondary to mesenteric ischemia. Both patients presented with fever and weight loss while receiving long term home parenteral nutrition. Both of them were found to have mutations in the NOD2 gene. Catheter related infections were ruled out. FDG-PET-CT scans initially obtained in search for another infectious focus that would explain the symptoms unexpectedly showed high FDG uptake in mediastinal lymph nodes. Direct or indirect evidence proved or was highly suggestive for tuberculous lymphadenitis. Intravenous tuberculostatic therapy was started and led to a reversal of symptoms and to resolution of the lesions by FDG-PET-CT. CONCLUSION: Mutations in the NOD2 gene may put patients both at an increased risk for acquiring M. tuberculosis infections as well as at an increased risk of intestinal failure after extensive intestinal resection. Thus we suggest to specifically include reactivated and opportunistic infections in the differential diagnosis of suspected catheter related infection in patients with intestinal failure who carry mutations in their NOD2 gene.

Simultaneously acquired MR/PET images compared with sequential MR/PET and PET/CT: alignment quality.

PURPOSE: To compare anatomic alignment between morphologic and positron emission tomography (PET) images acquired in the abdomen and pelvis by using simultaneous magnetic resonance (MR)/PET, PET/computed tomography (CT), and retrospective MR/PET fusion and to compare alignment between MR and PET in simultaneous and sequential thoracic MR/PET by using different breathing, registration, and gating protocols. MATERIALS AND METHODS: Informed consent and institutional review board approval were obtained. The misalignment in 28 abdominal patient datasets was analyzed in simultaneous MR/PET, PET/CT, and retrospective MR/PET fusion. In seven thoracic MR/PET datasets, the effect of different breathing protocols, simultaneous, sequential, and MR-gated data acquisition was evaluated, and the effect of rigid registration versus nonregistered images was evaluated. Analysis of variance and subsequent Tukey test were used for statistical analysis. RESULTS: The misalignment in all abdominal organs was reduced in simultaneous MR/PET compared with retrospectively fused MR and PET images (means, 5.8 mm vs 11.9 mm; P < .001), and in the urinary bladder compared with PET/CT (means, 5.9 mm vs 11.0 mm; P < .007). Thoracic MR/PET with inspiratory breath-hold MR showed the largest misalignment (mean, 24.5 mm; P < .001). None of the other thoracic protocols that were composed of different acquisition, registration, or gating procedures differed significantly from one another. CONCLUSION: The alignment of hybrid datasets acquired in simultaneous whole-body MR/PET was more accurate than retrospective fusion in all investigated abdominal organs, and more accurate than PET/CT in the urinary bladder; the alignment of thoracic MR/PET with expiratory breath hold or free-breathing MR was more exact than with inspiratory MR. However in this preliminary work the clinical importance of these degrees of misalignment was not assessed.

Comparison between high-resolution CT and MRI using a very short echo time in patients with cystic fibrosis with extra focus on mosaic attenuation.

BACKGROUND: It would be beneficial to establish pulmonary MRI as a complementary approach to CT for direct visualization of mosaic perfusion, bullae, and emphysema in patients with cystic fibrosis. OBJECTIVES: The purpose of this study was to compare both modalities, CT and MRI, using the Helbich-Bhalla score with a special focus on reliable detection of a mosaic pattern. METHODS: Out of 51 patients examined by MRI on a 1.5-Tesla system during a period of 2 years, 19 patients were scheduled for additional low-dose CT in a clinical context. The MRI protocol comprised a gradient echo (GRE) sequence with a very short echo time (TE = 0.8 ms) in inspiration and expiration, a 3-D GRE sequence in breath hold, and a fast spin echo sequence with respiration and ECG triggering. MDCT was carried out in inspiration and adapted to body weight using 100 or 120 kV, 30-60 mA, 1- and 3-mm slice thicknesses, as well as low and high kernels. Additionally incremental slices in 3 positions were recorded in expiration for distinct detection of air trapping. CT and MRI analyses were performed by two radiologic readers in consensus unaware of the clinical parameters. The Helbich-Bhalla score of both examinations was correlated. Mean difference and accordance were assessed in each category. RESULTS: There was a strong correlation between CT and MRI (R = 0.87, p < 0.01). The mean Helbich-Bhalla score for CT was 12.2 (range 1-18) and for MRI it was 11.7 (range 2-19). The mean difference was 0.5 points. Besides this strong correlation for findings (bronchiectasis, mucus plugging, peribronchial thickening, and consolidation) with a prolonged T2 TE in MRI, we could also state a qualitative agreement of 95-100% in the categories with short T2 and low signal intensity in MRI as emphysema, bullae, and mosaic perfusion. CONCLUSIONS: These results suggest that in our patient group none of the relevant findings were missed by MR imaging and reading.

Differing Pulmonary Structural Abnormalities Detected on Pulmonary MR Imaging in Cystic Fibrosis Patients with Varying Pancreatic Function.

PURPOSE: In cystic fibrosis (CF) the phenotypic expression of complaints varies widely. Genotypes with sufficient pancreatic function (PS) exhibit milder lung disease compared to CF patients with insufficient pancreatic function (PI). The purpose of this study was to evaluate structural lung disease (SLD) in CF patients with differing pancreatic status but similar results on pulmonary function testing using a pulmonary magnetic resonance imaging score (MR-CF score). MATERIALS AND METHODS: In this retrospective study, 20 patients in our single-center CF database were included: 10 with PS (mean age 12.5 years; six male; BMI 17.4 kg/m2; FeV1 102 %) were matched by gender, age and lung function with 10 PI patients. Experienced observers semi-quantitatively assessed SLD for each lung lobe. The established MR-CF score measures the extent and the severity of bronchiectasis and bronchial wall thickening, mucus plugging, centrilobular opacity, consolidation, sacculation, and air trapping. The total score and sub-score values were compared to the pancreatic status. RESULTS: Patients with CF-PS had overall statistically significant lower MR-CF scores (p = 0.024), and therefore milder SLD, compared to CF-PI. The differences were most significant for bronchiectasis (p = 0.0042) and air trapping (p = 0.0304). SLD was more severe in the upper lobes in all patients. However, differences between CF-PS and CF-PI patients were present in both the upper and lower lung areas (p = 0.0247 and p = 0.0196, respectively). CONCLUSION: Our results demonstrated that CF patients with impaired pancreatic function show more severe lung pathology detected by MRI, especially bronchiectasis and air trapping. KEY POINTS: · Pulmonary MRI offers morphological and functional details without using ionizing radiation. · CF patients with pancreatic insufficiency show more severe pulmonary structural impairment. · Bronchiectasis and air trapping are the most common structural lung changes with predominance in the upper lung lobes.. CITATION FORMAT: · Kraus MS, Teufel M, Esser M et al. Differing Pulmonary Structural Abnormalities Detected on Pulmonary MR Imaging in Cystic Fibrosis Patients with Varying Pancreatic Function. Fortschr Röntgenstr 2020; 192: 567 - 575.

Living-Donor Uterus Transplantation: Pre-, Intra-, and Postoperative Parameters Relevant to Surgical Success, Pregnancy, and Obstetrics with Live Births.

Uterus transplantation (UTx) can provide a route to motherhood for women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), a congenital disorder characterized by uterovaginal aplasia, but with functional ovaries. Based on our four successful living-donor transplantations and two resulting births, this analysis presents parameters relevant to standardizing recipient/donor selection, UTx surgery, and postoperative treatment, and their implementation in routine settings. We descriptively analyzed prospectively collected observational data from our four uterus recipients, all with MRKHS, their living donors, and the two newborns born to two recipients, including 1-year postnatal follow-ups. Analysis included only living-donor/recipient pairs with completed donor/recipient surgery. Two recipients, both requiring ovarian restimulation under immunosuppression after missed pregnancy loss in one case and no pregnancy in the other, each delivered a healthy boy by cesarean section. We conclude that parameters crucial to successful transplantation, pregnancy, and childbirth include careful selection of donor/recipient pairs, donor organ quality, meticulous surgical technique, a multidisciplinary team approach, and comprehensive follow-up. Surgery duration and blood vessel selection await further optimization, as do the choice and duration of immunosuppression, which are crucial to timing the first embryo transfer. Data need to be collected in an international registry due to the low prevalence of MRKHS.