Loeys-Dietz syndrome (LDS) is a recently described genetic connective tissue disorder with a wide spectrum of multisystem involvement. LDS is characterized by rapidly progressive aortic and peripheral arterial aneurysmal disease. LDS and the other inherited aortopathies such as Marfan syndrome have overlapping phenotypic features. However, LDS is characterized by a more aggressive vascular course; patient morbidity and mortality occur at an early age, with complications developing at relatively smaller aortic dimensions. In addition, there is more diffuse arterial involvement in LDS, with a large proportion of patients developing aneurysms of the iliac, mesenteric, and intracranial arteries. Early diagnosis and careful follow-up are essential for ensuring timely intervention in patients with arterial disease. Cross-sectional angiography has an important role in the baseline assessment, follow-up, and evaluation of acute complications of LDS, the thresholds and considerations of which differ from those of other inherited aortopathies. In this article, LDS is compared with other genetic vascular connective tissue disorders. In addition, the genetic, histopathologic, and cardiovascular manifestations of this disease process are reviewed, with a focus on computed tomographic and magnetic resonance imaging findings. Online DICOM image stacks and supplemental material are available for this article. RSNA, 2018.
CTA regularly overestimates thrombus length as the distal end of the thrombus is not delineated. This can be overcome through the use of a CECT acquisition which can reliably be used to estimate thrombus length.
BackgroundEndoscopic ultrasound is seldom available at paediatric centres; therefore drainage of pancreatic pseudocysts in children has traditionally been achieved by surgery.ObjectiveThis study assessed the feasibility and safety of performing image-guided internal drainage of pancreatic pseudocysts with a flanged self-expanding covered nitinol pancreatic pseudocyst drainage stent.Materials and methodsWe conducted a retrospective case note review of children undergoing image-guided cystogastrostomy at two paediatric hospitals. Percutaneous access to the stomach was achieved via an existing gastrostomy tract or image-guided formation of a new tract. Under combined ultrasound, fluoroscopic or cone-beam CT guidance the pancreatic pseudocysts were punctured through the posterior wall of the stomach. A self-expanding covered nitinol stent was deployed to create a cystogastrostomy opening.ResultsImage-guided cystogastrostomy was performed in 6 children (4 male; median age 6 years, range 46 months to 15 years; median weight 18 kg, range 13.8–47 kg). Two children had prior failed attempts at surgical or endoscopic drainage. Median maximum cyst diameter was 11.5 cm (range 4.7–15.5 cm) pre-procedure. Technical success was 100%. There were no complications. There was complete pseudocyst resolution in five children and a small (2.1-cm) residual pseudocyst in one. Pseudocyst-related symptoms resolved in all children.ConclusionPancreatic pseudocyst drainage can be successfully performed in children by image-guided placement of a cystogastrostomy stent. In this cohort of six children there were no complications.
To determine interobserver agreement between paediatric radiologists interpreting tuberculosis (TB) screening chest radiographs (CXR) in children in the UK, and the TB detection rate.MATERIALS AND METHODS: A retrospective review was undertaken of electronic request, notes, and CXRs (>7 years) in children exposed to an infectious case of TB and new entrants to the UK, at a tertiary children's hospital. Included were those with positive Mantoux/interferon gamma release assay (IGRA), positive culture, or high clinical suspicion of TB. CXR reports were categorised as normal, abnormal without features of TB, or abnormal with features of pulmonary TB. Three paediatric radiologists from a dedicated paediatric radiology department evaluated available CXRs, aware of the TB screening indication, using a published CXR reporting tool and blinded to the initial CXR report and to each other. A majority decision was collated, and considered lymphadenopathy and miliary nodules as diagnostic of primary TB. Interobserver agreement was calculated using Cohen's kappa.RESULTS: One hundred and forty-eight children underwent TB screening with a CXR. One hundred and twenty-five had available CXR reports and case notes, which indicated 20/125 (16%) had CXR features of TB. One hundred and twenty-one of the 125 had CXRs available to for panel review. Twenty of these 121 (17%) yielded a majority decision of pulmonary TB. Interreader agreement was moderate in all aspects (kappa 0.4e0.6).CONCLUSION: The high percentage of pulmonary TB on CXR (16% original reports; 17% by panel review) suggests that it is worthwhile investigating childhood TB contacts; however, the
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