Published papers

© 2020, Springer-Verlag GmbH Austria, part of Springer Nature. Sinking skin flap syndrome is a craniectomy complication characterized by new neurological dysfunction that typically worsens in the upright position and improves after cranioplasty. We present a 33-year-old man who experienced hemiparesis in the upright position after craniectomy. Upright computed tomography (CT) before cranioplasty showed a remarkable shift of the brain compared to supine CT. After cranioplasty, both symptoms and brain shift on CT resolved. Upright CT enables detection and objective evaluation of paradoxical herniation and midline shift that is not obvious on supine imaging modalities. Clinicians need to be aware of positional brain shift in postcraniectomy patients.

© 2020 Elsevier B.V. Purpose: To investigate if the presence and severity of traction bronchiectasis/bronchiolectasis are associated with poorer survival in subjects with ILA. Method: The study included 3,594 subjects (378 subjects with ILA and 3,216 subjects without ILA) in AGES-Reykjavik Study. Chest CT scans of 378 subjects with ILA were evaluated for traction bronchiectasis/bronchiolectasis, defined as dilatation of bronchi/bronchioles within areas demonstrating ILA. Traction bronchiectasis/bronchiolectasis Index (TBI) was assigned as: TBI = 0, ILA without traction bronchiectasis/bronchiolectasis: TBI = 1, ILA with bronchiolectasis but without bronchiectasis or architectural distortion: TBI = 2, ILA with mild to moderate traction bronchiectasis: TBI = 3, ILA and severe traction bronchiectasis and/or honeycombing. Overall survival (OS) was compared among the subjects in different TBI groups and those without ILA. Results: The median OS was 12.93 years (95%CI; 12.67 – 13.43) in the subjects without ILA; 11.95 years (10.03 – not reached) in TBI-0 group; 8.52 years (7.57 – 9.30) in TBI-1 group; 7.63 years (6.09 – 9.10) in TBI-2 group; 5.40 years (1.85 – 5.98) in TBI-3 group. The multivariable Cox models demonstrated significantly shorter OS of TBI-1, TBI-2, and TBI-3 groups compared to subjects without ILA (P < 0.0001), whereas TBI-0 group had no significant OS difference compared to subjects without ILA, after adjusting for age, sex, and smoking status. Conclusions: The presence and severity of traction bronchiectasis/bronchiolectasis are associated with shorter survival. The traction bronchiectasis/bronchiolectasis is an important contributor to increased mortality among subjects with ILA.

Background: Because few have reported the prognostic significance of cardiac magnetic resonance imaging (CMR) for idiopathic pulmonary arterial hypertension (IPAH), in this study we evaluated the value of CMR measurements as a prognostic predictor of IPAH before starting intravenous prostacyclin therapy. Methods and Results: A total of 121 consecutive CMR studies for evaluating right ventricular (RV) function were reviewed. Forty-one patients were diagnosed with IPAH and served as the study group. Factors, such as age, sex, New York Heart Association functional class (NYHAFC), 6-min walk test, plasma brain natriuretic peptide level, serum uric acid level and CMR measurements were analyzed as predictors of first hospitalization and death. The mean follow- up period was 1,350±769 days. Nine patients were hospitalized because of heart failure, and 4 patients died from cardiopulmonary causes. The univariate analyses suggested that the left ventricular (LV) mass index, the left and right ventricular end-diastolic volume indices (LVEDVI, RVEDVI), the LV and RV end-systolic volume indices (LVESVI, RVESVI) and NYHAFC predicted the risk for hospitalization and that RVEDVI, RVESVI and NYHAFC predicted mortality. The multivariate analyses suggested that RVEDVI and NYHAFC are independent predictors of both hospitalization and mortality. The effects of RVEDVI and NYHAFC on hospitalization were not substantially affected by the concomitant medication. Conclusions: In IPAH patients, the RVEDVI predicts both hospitalization for right heart failure and mortality before initiating intravenous prostacyclin therapy.

Purpose: The purpose of this study was to evaluate the effect of the virtual monochromatic spectral images (VMSI) and the model-based iterative reconstruction (MBIR) images, to evaluate the influence of the aperture size (40- and 20-mm beam) on renal pseudoenhancement (PE) compared with the filtered back projection (FBP) images. Methods: The renal compartment-CT phantom was filled with iodinated contrast material diluted to the attenuation of 180 Hounsfield units (HU) at 120 kV. The water-filled spherical structures, which simulate cyst, were inserted into the renal compartment. Those diameters were 7, 15 and 25 mm. These were scanned by conventional mode (helical scan, 120 kV-FBP) and dual energy mode. 70 keV-VMSI were reconstructed from the dual energy mode, and MBIR images were reconstructed from conventional mode at 40- and 20-mm aperture. Additionally, the phantom was scanned using non-helical mode with 20-mm aperture, and FBP images were reconstructed. The CT value of the PE for cyst areas was measured for these images. Results: The CT values of the cysts were 20.0–14.3 HU on the FBP images, 12.8–12.7 HU on the 70 keV-VMSI (PE-inhibition ratio was 36.0–11.2%) and 16.2–14.0 HU on the MBIR images (19.0–2.1%), respectively, at 40-mm aperture. The PE-inhibition ratio scanned by 20-mm aperture was improved by 28.0% with FBP, 32.8% with 70 keV-VMSI and 29.6% with MBIR compared with 40-mm aperture. One of the FBP images with non-helical mode was 11.6 HU. Conclusions: The best CT technique to minimize PE was the combination of 70 keV-VMSI and 20-mm aperture.

There have been a few reports of adenocarcinoma developing in adenomyotic areas without endometrial involvement. We report a case of adenocarcinoma arising in the myometrium, with no involvement of the surface of the endometrium. A 60-year-old woman presented with lower abdominal pain. Pelvic MRI showed a poorly demarcated mass in the myometrium with necrosis or hemorrhage. Histological diagnosis revealed adenocarcinoma. The mass was well circumscribed and localized in the myometrium. The endometrium had normal thickness throughout the cavity. The cervix was normal.

© 2019, Springer Japan KK, part of Springer Nature. Myocardial T1 mapping is clinically valuable for assessing the myocardium, and modified look-locker inversion-recovery (MOLLI) approaches have been commonly used for measuring myocardial T1 values. To date, several other sequences have been developed for measuring myocardial T1 values, and saturation-recovery-based sequences have been shown to be less dependent on various factors, such as T2 times and magnetization transfer, than inversion-recovery techniques. Systematic differences in T1 values between different sequences have been reported; therefore, definition of the normal range of native T1 values is required before clinical usage can begin. The purpose of this study was to evaluate the reference range and sex dependency of native T1 values in the myocardium measured using one such saturation-recovery sequence, i.e., saturation method using adaptive recovery times for cardiac T1 mapping (SMART1Map). Myocardial T1 values were compared between SMART1Map and MOLLI in 24 young healthy volunteers at 1.5 T and 3 T, and differences in the T1 values between the sexes were assessed. The mean native T1 values in the myocardium were significantly longer with SMART1Map than MOLLI [1530.4 ± 49.2 vs 1222.1 ± 48.9 ms at 3 T (p < 0.001) and 1227.3 ± 41.9 ms vs 1014.8 ± 49.4 ms at 1.5 T (p < 0.001)]. A significant difference between the sexes was observed in the T1 values obtained using each sequence, excluding SMART1Map at 3 T. The SMART1Map has a potential advantage to overcome the shortcoming of MOLLI, which underestimates T1 values; however, the sex-dependent difference remains obscure using SMART1Map.

© 2018 American Roentgen Ray Society. OBJECTIVE. Observation of a sinus tract during a barium swallow examination is important for the diagnosis of pyriform sinus fistula; however, to our knowledge, no reports have existed regarding the optimal timing of the examination in relation to the onset of symptoms. The purpose of this study was to compare the timing of the examination, patient age, the number of inflammatory episodes that occurred before the examination, and the barium concentration used for examinations with true-positive results versus those with false-negative results for the diagnosis of pyriform sinus fistula. MATERIALS AND METHODS. Twenty-three children with pyriform sinus fistula were included. The timing of the examination, patient age, the number of the inflammatory episodes that occurred before examination, and the barium concentration used were compared between examinations with true-positive results and examinations with false-negative results, by use of the Mann-Whitney U test. RESULTS. The examination had true-positive results for 60.9% (14/23) of patients and false-negative results for 39.1% (9/23) of patients. The mean (± SD) interval since the onset of symptoms was significantly shorter for patients with false-negative examination results than for those with true-positive examination results (26.33 ± 21.17 days vs 48.57 ± 17.67 days; p = 0.020). By 6 weeks after the onset of symptoms, more than half of the examinations had false-negative results. No significant difference in patient age (p = 0.238) or number of previous inflammatory episodes (p = 0.431) existed between examinations with true-positive and false-negative results; however, a significant difference was noted in the mean barium concentration used (88.57% ± 31.53% vs 52.86% ± 18.68% weight/volume, respectively; p = 0.014). CONCLUSION. Barium swallow examinations with false-negative results were significantly more likely when the examination was performed soon after the onset of symptoms. Therefore, early first examinations would not be recommended for the diagnosis of pyriform sinus fistula, especially in terms of radiation exposure. A higher barium concentration may be useful.

© 2019 International Society for Heart and Lung Transplantation BACKGROUND: A variant of c.14429G>A (p.Arg4810Lys, rs112735431) in the ring finger protein 213 gene (RNF213; NM_001256071.2) has been recently identified as a risk allele for pulmonary arterial hypertension (PAH). PAH can be added as a new member of RNF213-associated vascular diseases, which include Moyamoya disease and peripheral pulmonary stenosis. Our aim was to identify the clinical features and outcomes of PAH patients with this variant. METHODS: Whole-exome sequencing was performed in 139 idiopathic (or possibly heritable) PAH patients. RESULTS: The RNF213 p.Arg4810Lys variant was identified in a heterozygous state in 11 patients (7.9%). Time-course changes in hemodynamics after combination therapy in the patients with the RNF213 p.Arg4810Lys variant were significantly poorer compared with those carrying the bone morphogenic protein receptor type 2 (BMPR2) mutation (n = 36) (comparison of changes in mean pulmonary arterial pressure, p = 0.007). The event-free rate of death or lung transplantation was significantly poorer in RNF213 p.Arg4810Lys variant carriers than in BMPR2 mutation carriers (5-year event-free rate since the introduction of prostaglandin I2 infusion, 0% vs 93%, respectively; p < 0.001). CONCLUSIONS: Idiopathic PAH patients with the RNF213 p.Arg4810Lys variant are associated with poor clinical outcomes even in recent times. Earlier consideration of lung transplantation might be required for RNF213 p.Arg4810Lys variant carriers who are developing PAH. Documentation of the RNF213 p.Arg4810Lys variant, as well as already known pathogenic genes, such as BMPR2, can provide clinically relevant information for therapeutic strategies, leading to a personalized approach for the treatment of PAH.

© 2019 The Authors Congenital diaphragmatic hernia (CDH) is one of the most common major congenital anomalies. The presence of a hernia sac is associated with additional benefits in terms of improved neonatal survival. Here, we report a case of CDH with a hernia sac detected via postnatal ultrasonography. Our literature search did not find other cases where CDH with hernia sac was found by postnatal ultrasound in neonates. In prenatal imaging, the diagnosis of CDH with a hernia sac is challenging. In our case, the meniscus of the thymus was clearly noted, and smooth convexity between the hernia contents and thymus was detected. Although evaluation of the presence of a hernia sac with postnatal ultrasonography might be difficult, our findings suggest that a hernia sac could be evaluated with postnatal ultrasonography.

© 2019 by the American Institute of Ultrasound in Medicine. OBJECTIVES: To compare defect sizes in congenital diaphragmatic hernia (CDH) measured by postnatal ultrasound (US) between neonates who underwent thoracoscopic surgery and neonates who underwent open surgery and between neonates who underwent primary repair and those who underwent patch repair; additionally, to compare the accuracy of US diagnosis with that of surgical diagnosis for the location of the diaphragmatic hernia and the hernial contents. METHODS: We included 8 neonates who underwent preoperative US evaluations of CDH. We compared anterior-to-posterior diaphragm defect sizes between thoracoscopic and open surgery approaches and between primary and patch repair by using the Mann-Whitney U test. The diaphragm was divided into 3 segments: anterior, lateral, and posterior. We evaluated the location of the diaphragmatic hernia and the hernial contents. RESULTS: Four neonates who underwent open surgery had larger diaphragmatic hernias than those who underwent thoracoscopic surgery (mean ± SD, 30.5 ± 5.6 versus 16.3 ± 3.3 mm; P = .030). They were also larger in neonates who underwent patch repair than in those who underwent primary repair (33.0 ± 3.0 versus 17.6 ± 4.2 mm; P = .037). Detection of anterior and lateral diaphragm segments was consistent between US and surgical findings. Three of 4 neonates who underwent open surgery and all 3 neonates who underwent patch repair did not show the lateral segment. The hernial contents were also consistent between US and surgical findings. CONCLUSIONS: Postnatal US examinations of neonates with CDH could provide surgeons with useful information to determine the surgical approach and repair method. However, since our study cohort was small, further studies are needed with a larger number of neonates with CDH.

© 2019 Societatea Romana de Ultrasonografie in Medicina si Biologie. All Rights Reserved. Aims: To evaluate ultrasound findings in order to determine potential predictors of prognosis in pediatric patients with portal venous gas (PVG) detected by ultrasound. Materials and methods: Thirty-nine children were included and divided into two groups: benign PVG (n=24) and life-threatening PVG (n=15; 6 surgical interventions and 9 deaths). Possible predictors, i.e., the location of PVG in the liver, the distribution of intestinal pneumatosis, ascites and free air were compared between the two groups. Results: A significant difference was noted between the two groups in terms of the distribution of intestinal pneumatosis (limited to the large bowel, benign vs life-threatening = 60.9% (14/23):21.4% (3/14), p=0.040), the absence of ascites (benign vs life-threatening = 79.1% (19/24):40.0% (6/15), p=0.019) and patient age (benign vs life-threatening = 52.5±65.3 months vs 19.7±44.0 months, p=0.019). No significant difference was observed in the location of PVG in the liver, the presence of free air, and sex between the two groups. Conclusions: In pediatric patients with PVG, including various ages and underlying diseases, intestinal pneumatosis limited to the large bowel and absence of ascites were predictors of a benign prognosis. However, despite the presence of these predictors, some patients with PVG required surgical intervention, thereby suggesting that the cause of PVG, such as necrotizing enterocolitis, volvulus, or pancreatitis, must be also carefully evaluated.

© 2018 The Authors. Journal of Applied Clinical Medical Physics published by Wiley Periodicals, Inc. on behalf of American Association of Physicists in Medicine. The purpose of this phantom study was to investigate the feasibility of dose reduction with hybrid iterative reconstruction, with and without a noise power spectrum (NPS) model, using both quantitative and qualitative evaluations. Standard dose (SD), three-quarter dose (TQD), and half-dose (HD) of radiation were used. Images were reconstructed with filtered back projection (FBP), adaptive iterative dose reduction 3D (AIDR 3D) (MILD, STR), and AIDR 3D enhanced (eAIDR 3D) (eMILD, eSTR). An NPS analysis, task-based modulation transfer function (MTFtask) analysis, and comparisons of low-contrast detectability and image texture were performed. Although the eAIDR 3D had a higher NPS value in the high-frequency range and improved image texture and resolution as compared with AIDR 3D at the same radiation dose and iteration levels, it yielded higher noise than AIDR 3D. Additionally, although there was no statistically significant difference between SD-FBP and the TQD series in the comparison of the mean area under the curve (AUC), the mean AUC was statistically significantly different between SD-FBP and the HD series. NPS values in the high-frequency range, 10% MTFtask values, low-contrast detectability, and image textures of TQD-eMILD were comparable to those of SD-FBP. Our findings suggested that using eMILD can reduce the radiation dose by 25%, while potentially maintaining diagnostic performance, spatial resolution, and image texture; this could support selecting the appropriate protocol in a clinical setting.

© 2018 Elsevier Ltd Background and objective: No reports exist regarding skeletal muscle involvement in patients with Mycobacterium avium complex lung disease (MAC-LD). The cross-sectional area of the erector spinae muscles (ESMCSA) reflects physical activity and can be assessed by computed tomography (CT). We investigated the relationship between ESMCSA and physiological parameters and prognosis in MAC-LD patients. Material and methods: In this prospective observational study, the ESMCSA was measured on single-slice axial CT images. MAC-LD patients and sex- and age-matched controls (non-MAC-LD participants) were evaluated. We evaluated the relationship between the ESMCSA and physiological parameters and prognosis. Results: A total of 260 patients (209 female; median age, 69 years; 190 with nodular/bronchiectatic disease; 74 with cavitary lesions) were enrolled. The ESMCSA was not different between MAC-LD patients and controls. In MAC-LD patients, the ESMCSA was significantly associated with age, body mass index (BMI), pulmonary function, CT severity, and health-related quality of life (HRQL). Multivariate Cox proportional hazards analyses revealed that an ESMCSA < −1 standard derivation (hazards ratio [HR], 2.76; P = 0.047) was significantly associated with all-cause mortality, along with BMI < 18.5 kg/m2 (HR, 3.67; P = 0.02) and presence of cavitary lesions (HR, 5.84; P = 0.001). However, the ESMCSA was not significantly associated with all-cause mortality when current treatment status, % predicted functional vital capacity, and forced expiratory volume in 1 s were added to the analyses. Conclusions: Although the prognostic impact was limited, ESMCSA was significantly associated with HRQL and prognostic physiological parameters, such as BMI and pulmonary function.

© 2018, The Japanese Society of Nuclear Medicine. Purpose: Quantification of the tracer distribution would add objectivity to the visual assessments of dopamine transporter (DAT) single photon emission computed tomography (SPECT) data. Our study aimed to evaluate the diagnostic utility of fractal dimension (FD) as a quantitative indicator of tracer distribution and compared with the conventional quantitative value: specific binding ratio (SBR). We also evaluated the utility of the combined index SBR/FD (SBR divided by FD). Materials and methods: We conducted both clinical and phantom studies. In the clinical study, 150 patients including 110 patients with Parkinsonian syndrome (PS) and 40 without PS were enrolled. In the phantom study, we used a striatal phantom with the striatum chamber divided into two spaces, representing the caudate nucleus and putamen. The SBR, FD, and SBR/FD were calculated and compared between datasets for evaluating the diagnostic utility. Mann–Whitney test and receiver-operating characteristics (ROC) analysis were used for analysis. Results: ROC analysis revealed that the FD value had high diagnostic performance [the areas under the curve (AUC) = 0.943] and the combined use of SBR and FD (SBR/FD) delivered better results than the SBR alone (AUC, 0.964 vs 0.899; p < 0.001). The sensitivity, specificity, and accuracy, respectively, were 79.1, 85.0, and 80.7% with SBR, 84.5, 97.5, and 88.0% with FD, and 92.7, 87.5, and 91.3% with SBR/FD. Conclusion: Our results confirmed that the FD value is a useful diagnostic index, which reflects the tracer distribution in DAT SPECT images. The combined use of SBR and FD was more useful than either used alone.