Adrenal crisis caused by inhaled fluticasone in an adolescent with cystic fibrosis and advanced hepatopathy: a case report.

Inhaled corticosteroids are widely accepted in the treatment of cystic fibrosis. Long-term use may cause systemic complications, especially high-dose fluticasone. We report about a young man who presented with encephalopathy after excessive physical activity caused by secondary adrenal insufficiency. He recovered quickly after systemic corticosteroid replacement therapy. This problem is considered to be underdiagnosed in clinical practice.

Measurement of the Optic Nerve Sheath Diameter in Children: Comparison Between Transbulbar Sonography and Magnetic Resonance Imaging.

PURPOSE: To evaluate the accuracy of measurements of the optic nerve sheath diameter (ONSD) in children by comparing transbulbar sonography with magnetic resonance imaging (MRI). MATERIALS AND METHODS: 65 children (age: 3 mo- 17y; mean age: 11.3y) underwent MR imaging of the brain including a heavily T 2-w sequence of the orbit and transbulbar sonography using a 17 MHz linear array transducer. Measurements of the ONSD were performed retrospectively by two experienced readers and all images were evaluated regarding the image quality. Bland-Altman Plots were produced to assess the accuracy of measurements. The correlation between readers and between MR imaging and transbulbar sonography was calculated by the concordance correlation coefficient (CCC). RESULTS: Overall the mean values of the ONSD for MRI (5.86 ±â€Š0.66 mm) and transbulbar sonography (5.86 ±â€Š0.71 mm) were identical. There was a high correlation between readers for measurements of the ONSD both for transbulbar sonography (CCC = 0.93) as well as for MRI (CCC = 0.9). Comparing the measurement values between transbulbar sonography and MRI, the correlation of ONSD values was good to moderate with a high dependency on image quality (CCC [0.31, 0.68]). CONCLUSION: We were able to demonstrate that the ONSD values of transbulbar sonography in children correlate well with MRI if the relevant anatomic structures are depicted and the measuring points are set correctly. Based on the findings of our study, it seems necessary to define normal and cut-off values for the ONSD in children again to finally assess the clinical relevance of the ONSD as a noninvasive parameter for the evaluation of intracranial pressure.

[Uncommon presentation of tuberous sclerosis in an infant]. / Présentation inhabituelle de la sclérose tubéreuse de Bourneville de chez un nourrisson : à propos d'un cas.

We report on an infant with an unusual presentation of tuberous sclerosis. After uncomplicated birth, a routine ultrasound was performed because the patient's brother had undergone nephrectomy at the age of four months due to multicystic renal dysplasia. All other family members were healthy. Multiple renal cysts were found in the boy's left kidney. The right kidney, which was normal initially, showed cysts after a few months. In a follow-up sonography at the age of 10 months, we found an aortic aneurysm measuring 4 × 7 cm. A brain NMR showed typical signs of tuberous sclerosis. Aortic aneurysm is very rarely associated with tuberous sclerosis. As a TSC2/PKD1 contiguous gene syndrome was excluded, in this case the child probably has two different diseases, i.e. tuberous sclerosis in addition to phenotypically unusual multicystic renal dysplasia.

High resolution ultrasound and magnetic resonance imaging of the optic nerve and the optic nerve sheath: anatomic correlation and clinical importance.

PURPOSE: We performed a cadaver study to evaluate the accuracy of measurements of the optic nerve and the optic nerve sheath for high resolution US (HRUS) and magnetic resonance imaging (MRI). MATERIALS AND METHODS: Five Thiel-fixated cadaver specimens of the optic nerve were examined with HRUS and MRI. Measurements of the optic nerve and the ONSD were performed before and after the filling of the optic nerve sheath with saline solution. Statistical analysis included the calculation of the agreement of measurements and the evaluation of the intraobserver and interobserver variation. RESULTS: Overall a good correlation of measurement values between HRUS and MRI can be found (mean difference: 0.02-0.97 mm). The repeatability coefficient (RC) and concordance correlation coefficient (CCC) values were good to excellent for most acquisitions (RC 0.2-1.11 mm; CCC 0.684-0.949). The highest variation of measurement values was found for transbulbar sonography (RC 0.58-1.83 mm; CCC 0.615/0.608). CONCLUSION: If decisive anatomic structures are clearly depicted and the measuring points are set correctly, there is a good correlation between HRUS and MRI measurements of the optic nerve and the ONSD even on transbulbar sonography. As most of the standard and cut-off values that have been published for ultrasound are significantly lower than the results obtained with MRI, a reevaluation of sonographic ONSD measurement with correlation to MRI is necessary.