Caroline Ernst

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.

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.

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.

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.

Unilateral sight loss in a 4-year-old girl

For 6 weeks the parents of a 4-year-old girl had noticed a difference between the two eyes of their child. Ophtalmological examination revealed leukocoria. This finding raised clinical suspicion of retinoblastoma. MRI was performed. On T2 weighted images a hypointense mass relative to the vitreous humor was evident. There were several hypointensities compatible with calcifications. An area of retinal detachment was also seen. On T1 weighted images a mildly hyperintense mass relative to vitreous humor was seen. It showed marked contrast enhancement. On diffusion weighted images there was restricted diffusion suggestive for a tumoral lesion. The imaging findings were compatible with a retinoblastoma without transscleral or optic nerve extension. The tumor was complicated by retinal detachment reducing visual potential. The patient was treated with enucleation of the affected eye.