The quantitative link of lung clearance index to bronchial segments affected by bronchiectasis.

In adult patients with cystic fibrosis (CF), the lung clearance index (LCI) derived from the multiple breath washout relates to both acinar and conductive ventilation heterogeneity. The latter component predicts an association between LCI and the number of bronchial segments affected by bronchiectasis. Here, we experimentally demonstrated this association in patients with CF, and also examined an ancillary group of patients with non-CF bronchiectasis. We conclude that lung disease severity in terms of number of bronchial segments results in an associated LCI increase, likely constituting a portion of LCI that cannot be reversed by treatment in patients with CF lung disease.

Lowered dose full-spine radiography in pediatric patients with idiopathic scoliosis.

PURPOSE: To optimize our standard full-spine radiography with respect to diagnostic quality and dose. METHODS: A phantom study was performed to establish an optimal posterior-anterior view (PA) full spine protocol having the lowest dose with non-inferior quality compared to standard. We then applied this protocol in 40 pediatric patients (group B). The radiographs were scored on six criteria by a pediatric radiologist and orthopedist and compared to the scores of 40 PA full spine radiographs performed in 2013 with standard protocol (group A). Radiation dose was assessed by dose area product (DAP) and effective dose (E). Statistical analysis included independent samples t test, Mann-Whitney U test and intra-class correlation coefficient (ICC). RESULTS: An optimized protocol was defined (0.2 mm Cu filter, 0.87 relative exposure, with grid). Mean age was 13.3 ± 1.6 years for group A and 13.4 ± 1.7 years for group B. For group B, the mean DAP was 47.0 µGy m2 with an E of 0.13 mSv. For group A, the mean DAP was 85.3 µGy m2 with an E of 0.24 mSv. This represents a dose reduction of 45%. Mean image quality scores for group A (27.9 ± 2.4) and group B (28.1 ± 2.3) were similar (p = 0.612). Interobserver agreement was observed to be excellent (ICC 0.92). CONCLUSION: This study demonstrates that a low-dose full-spine radiograph can be performed in patients with idiopathic scoliosis without loss of image quality. These slides can be retrieved under Electronic Supplementary Material.

Dedicated sub 0.1 mSv 3DCT using MBIR in children with suspected craniosynostosis: quality assessment.

OBJECTIVE: To retrospectively compare image quality of a lowered dose CT protocol to a standard CT protocol in children with suspicion of craniosynostosis. METHODS: Forty-eight patients (age 0- 35 months), who presented with a cranial deformity underwent cranial 3D CT to assess sutural patency: between 2009 - 2010, 24 patients were imaged with a standard protocol (CTDIvol 32.18 mGy), from 2011-2012, 24 underwent a low dose protocol (0.94 mGy) combined with iterative reconstruction. Image quality was evaluated by both expert reading and objective analysis. Differences were assessed by independent t-test and Mann-Whitney U test, interreader agreement by Cohen's Kappa test. RESULTS: Effective dose of the low dose protocol was 0.08 mSv, corresponding to a reduction of 97 %. Image quality was similar in both groups in terms of overall diagnostic acceptability, objective noise measurements, subjective cranial bone edge sharpness and presence of artefacts. For objective sharpness of cranial bone-brain interface and subjective perception of noise, the images of the low dose protocol were superior. For all evaluated structures, interreader agreement was moderate to almost perfect. CONCLUSION: In the diagnosis of craniosynostosis in children with cranial deformities, a dedicated sub 0.1 mSv cranial 3DCT protocol can be used without loss in image quality. KEY POINTS: 3DCT is used for the diagnosis of craniosynostosis. Imaging protocols should be optimized to minimize radiation exposure to children. Combining 80 kVp with iterative reconstruction can help to reduce dose. A sub 0.1 mSv cranial 3DCT protocol can be used without loss of diagnostic quality.

Pulmonary disease in cystic fibrosis: assessment with chest CT at chest radiography dose levels.

PURPOSE: To investigate a computed tomographic (CT) protocol with iterative reconstruction at conventional radiography dose levels for the assessment of structural lung abnormalities in patients with cystic fibrosis ( CF cystic fibrosis ). MATERIALS AND METHODS: In this institutional review board-approved study, 38 patients with CF cystic fibrosis (age range, 6-58 years; 21 patients <18 years and 17 patients >18 years) underwent investigative CT (at minimal exposure settings combined with iterative reconstruction) as a replacement of yearly follow-up posteroanterior chest radiography. Verbal informed consent was obtained from all patients or their parents. CT images were randomized and rated independently by two radiologists with use of the Bhalla scoring system. In addition, mosaic perfusion was evaluated. As reference, the previous available conventional chest CT scan was used. Differences in Bhalla scores were assessed with the χ(2) test and intraclass correlation coefficients ( ICC intraclass correlation coefficient s). Radiation doses for CT and radiography were assessed for adults (>18 years) and children (<18 years) separately by using technical dose descriptors and estimated effective dose. Differences in dose were assessed with the Mann-Whitney U test. RESULTS: The median effective dose for the investigative protocol was 0.04 mSv (95% confidence interval [ CI confidence interval ]: 0.034 mSv, 0.10 mSv) for children and 0.05 mSv (95% CI confidence interval : 0.04 mSv, 0.08 mSv) for adults. These doses were much lower than those with conventional CT (median: 0.52 mSv [95% CI confidence interval : 0.31 mSv, 3.90 mSv] for children and 1.12 mSv [95% CI confidence interval : 0.57 mSv, 3.15 mSv] for adults) and of the same order of magnitude as those for conventional radiography (median: 0.012 mSv [95% CI confidence interval : 0.006 mSv, 0.022 mSv] for children and 0.012 mSv [95% CI confidence interval : 0.005 mSv, 0.031 mSv] for adults). All images were rated at least as diagnostically acceptable. Very good agreement was found in overall Bhalla score ( ICC intraclass correlation coefficient , 0.96) with regard to the severity of bronchiectasis ( ICC intraclass correlation coefficient , 0.87) and sacculations and abscesses ( ICC intraclass correlation coefficient , 0.84). Interobserver agreement was excellent ( ICC intraclass correlation coefficient , 0.86-1). CONCLUSION: For patients with CF cystic fibrosis , a dedicated chest CT protocol can replace the two yearly follow-up chest radiographic examinations without major dose penalty and with similar diagnostic quality compared with conventional CT.

Myopericytoma in an Infant-Imaging Characteristics of a Rare but Benign Entity.

Teaching point: Myopericytoma is a rare soft tissue tumor but should be considered in the differential diagnosis of infants with a fast-growing perivascular tumor.

Post-Traumatic Pseudoaneurysm of the Superficial Temporal Artery in a Pediatric Patient.

Teaching Point: A pseudoaneurysm of the superficial temporal artery is a rare complication of head injury in a child.

Peripheral precocious puberty in Li-Fraumeni syndrome: a case report and literature review of pure androgen-secreting adrenocortical tumors.

INTRODUCTION: Pure androgen-secreting adrenocortical tumors are a rare but important cause of peripheral precocious puberty. CASE PRESENTATION: Here, we report a pure androgen-secreting adrenocortical tumor in a 2.5-year-old boy presenting with penile enlargement, pubic hair, frequent erections, and rapid linear growth. We confirmed the diagnosis through laboratory tests, medical imaging, and histology. Furthermore, genetic testing detected a pathogenic germline variant in the TP53 gene, molecularly confirming underlying Li-Fraumeni syndrome. DISCUSSION: Only 15 well-documented cases of pure androgen-secreting adrenocortical tumors have been reported so far. No clinical or imaging signs were identified to differentiate adenomas from carcinomas, and no other cases of Li-Fraumeni syndrome were diagnosed in the four patients that underwent genetic testing. However, diagnosing Li-Fraumeni syndrome is important as it implies a need for intensive tumor surveillance and avoidance of ionizing radiation. CONCLUSION: In this article, we emphasize the need to screen for TP53 gene variants in children with androgen-producing adrenal adenomas and report an association with arterial hypertension.

A rare cause of dyspnea due to chylothorax.

We report the case of an 8-year-old boy who presented to the emergency department of another hospital and was referred to our pediatric intensive care unit with dyspnea and tachypnea of recent onset. The diagnosis of massive chylothorax with mediastinal shift was made on chest radiographs and CT scan. Initial investigations revealed no definite cause for the chylothorax. On later radiography and CT imaging with 3D surface rendering, a marked bone loss of the left ribs, clavicle, and shoulder joint was shown. The diagnosis of Gorham-Stout disease associated with chylothorax was suspected and histologically confirmed.

Guidance for computed tomography (CT) imaging of the lungs for patients with cystic fibrosis (CF) in research studies.

Numerous issues must be addressed when developing standard operating procedures for clinical research studies involving chest computed tomography of lung disease in patients with cystic fibrosis (CF). Study success depends on the provision of adequate funding and the identification of personnel with the necessary expertise to conduct the study, along with clear guidelines that detail the CT operating procedure at each site, including breathing maneuvers, and image reconstruction. Close coordination of the quality assurance process between sites and the central review organization is required to maintain protocol adherence. The data transfer process must ensure the integrity and security of the data to comply with patient privacy regulations, and study outcome measures are best assessed with a scoring system or other structured method of imaging data analysis. The recommendations provided are designed to serve as a valuable reference guide for planning clinical research studies of patients with CF involving chest CT.

Isolated tracheoesophageal fistula in a 10-year-old girl.

Isolated tracheoesophageal fistula (H-TOF) is a rare type of tracheoesophageal anomaly and is in most cases diagnosed in the neonatal period because of choking and cyanosis during feeding. Diagnosis may be delayed even until adulthood because of nonspecific and sometimes intermittent symptoms, and because false-negative results of all diagnostic tools are not uncommon. We report a 10-year-old child with H-TOF, whose symptoms had nearly disappeared completely between the ages of 4 and 10 years. Diagnosis was only possible after the recurrence of the symptoms at the time of an episode of bronchitis, profound interrogation of the child's medical history, and questioning of the results of a former diagnostic work-up. In this article, we discuss the potential pitfalls in both clinical diagnosis and diagnostic work-up.

Large wooden foreign body in the hand: recognition of occult fragments with ultrasound.

A 37-year-old man with pain and swelling of the thenar was referred to the emergency department. On ultrasound a 3 x 1 x 0.2 cm large wooden foreign body was depicted in the thenar region. In addition, ultrasonography (US) was able to show multiple smaller fragments adjacent to the larger foreign body. To give a better overview of the position relative to tendons and muscles, CT with soft tissue window settings was performed. CT gave a good anatomic overview but was not able to show the smaller fragments. A total of six additional fragments were depicted at US. Performing US is mandatory in patients with penetrating injuries by foreign bodies because it is very sensitive. Using US in an emergency setting can avoid retained fragments and depict other soft tissue complications.