Imaging of biliary disorders in children Rozel, Céline; Garel, Laurent; Rypens, Françoise ...
Pediatric radiology,
02/2011, Volume:
41, Issue:
2
Journal Article
Peer reviewed
Biliary atresia and related disorders of the biliary tree, such as choledochal cyst, must be considered in the differential diagnosis of prolonged conjugated hyperbilirubinemia in infants and ...children. Pediatric biliary tract diseases include a variety of entities with a wide range of clinical presentations. Radiology plays an important role in the diagnosis and management of these pathologies. Unrecognized causes of biliary disease, like biliary atresia, can lead to liver transplantation during the first year of life. The aim of this article is to review the imaging of pediatric biliary disorders, including the implications of interventional radiology in some biliary diseases.
This is a case report of a disseminated fetal rhabdoid tumor discovered at 32 weeks of gestation in a 29-year-old woman on immunosuppressive therapy. The mother consulted for a decrease in fetal ...movement. Fetal ultrasound showed signs of a disseminated tumor affecting the left armpit, liver, spleen, and limbs. A caesarian section was performed because of signs of fetal distress. Immunohistochemical analysis of a fetal biopsy showed deletion of the SMARCB1 gene. Pathological analysis of the placenta showed a rhabdoid tumor invading both fetal and maternal compartments. The mother underwent a whole-body MRI, and no metastasis was found. To the best of our knowledge, this is the first report of a disseminated rhabdoid tumor invading both fetal and maternal compartments.
Objectives
To investigate the contribution of whole-body post-mortem computed tomography (PMCT) in sudden unexpected death in infants and children.
Methods
Forty-seven cases of sudden unexpected ...death in children investigated with radiographic skeletal survey, whole-body PMCT and autopsy were enrolled. For imaging interpretation, non-specific post-mortem modifications and abnormal findings related to the presumed cause of death were considered separately. All findings were correlated with autopsy findings.
Results
There were 31 boys and 16 girls. Of these, 44 children (93.6 %) were younger than 2 years. The cause of death was found at autopsy in 18 cases (38.3 %), with 4 confirmed as child abuse, 12 as infectious diseases, 1 as metabolic disease and 1 as bowel volvulus. PMCT results were in accordance with autopsy in all but three of these 18 cases. Death remains unexplained in 29 cases (61.7 %) and was correlated with no abnormal findings on PMCT in 27 cases. Major discrepancies between PMCT and autopsy findings concerned pulmonary analysis.
Conclusions
Whole-body PMCT may detect relevant findings that can help to explain sudden unexpected death and is essential for detecting non-accidental injuries. We found broad concordance between autopsy and PMCT, except in a few cases of pneumonia. It is a non-invasive technique acceptable to relatives.
Key Points
• Whole-body post-mortem computed tomography (PMCT) is an effective non-invasive method.
• Whole-body PMCT is essential for detecting child abuse in unexpected death.
• There is concordance on cause of death between PMCT and autopsy.
• Whole-body PMCT could improve autopsy through dissection and sampling guidance.
• PMCT shows findings that may be relevant when parents reject autopsy.
Highlights • Early (≤6 days) and later MRIs were both good adverse outcome predictors in HIE. • Both MRIs had 100% sensitivity for adverse outcome. • Early MRI had higher specificity and positive ...predictive value than late MRI. • Early MRI could reliably help parents and neonatologists with intensive care decisions
Background
There is no consensus on the optimum timing of MRI in neonates with hypoxic–ischemic encephalopathy treated with hypothermia. Reliable early imaging assessment might help managing ...treatment.
Objective
To assess non-random differences between early and late MRI that might influence intensive-care decisions.
Materials and methods
This single-center retrospective study included all asphyxiated term neonates eligible for hypothermia treatment November 2009–July 2012. MRI scans were systematically performed at day 4 (early MRI) and day 11 of life as part of routine protocol. Two experienced pediatric radiologists reviewed both scans according to three assessment methods: a pattern classification, a scoring system and a simplified classification. Agreement between early and late imaging findings was assessed using Cohen’s kappa coefficients.
Results
Thirty-three neonates were included. Interobserver agreement was excellent. Early MRI detected all severe injuries. Agreement between early and late MRI was excellent for the simplified classification (κ = 0.82), good for the pattern classification (κ = 0.64), and good to excellent for 3 scores out of 4 in the scoring system (κ = 0.70–0.89).
Conclusion
Early MRI may provide valuable information about brain injury to help parents and neonatologists in intensive-care decisions at the end of hypothermia treatment.
This article provides an overview of the typical appearance of biomechanical and physiologic changes in pregnancy and an update on related pathophysiology. Conditions occurring during the ...childbearing, delivery and postpartum periods will be reported separately.
Pregnancy causes biomechanical and physiologic changes that may be responsible for a wide spectrum of musculoskeletal disorders in the mother.
Abstract Costello syndrome (CS) is a rare multiple congenital disorder caused by activating germline mutations in HRAS gene and is characterized by coarse facial features, severe feeding ...difficulties, failure to thrive, mild to severe intellectual disability, severe postnatal growth retardation, cardiac abnormalities or cancer predisposition. Phenotypic spectrum associated with HRAS mutations is broad, ranging from attenuated CS phenotype to neonatal and lethal forms with limited genotype-phenotype correlations. Congenital myopathy with neuromuscular spindle excess has been rarely described in the literature. We report a new severe fetal case of CS with distal arthrogryposis due to neuromuscular spindle excess, confirmed by the detection of the p.Gly12Val mutation in HRAS gene. This case emphasizes the fact that HRAS is the only gene responsible for neuromuscular spindle excess, underlines a correlation between p.Gly12Val mutation and severe CS phenotype and points out the importance of a muscle biopsy performed according to the suitable procedure in neuromuscular disorders for any fetal arthrogryposis.
The purpose of this study was to evaluate the clinical feasibility of coronary artery imaging during routine preoperative 64-slice MDCT scans of the chest. Ninety-nine consecutive patients in sinus ...rhythm underwent a biphasic multidetector-row spiral CT examination of the chest without the administration of beta-blockers, including an ECG-gated acquisition over the cardiac cavities, followed by a non-gated examination of the upper third of the thorax. Data were reconstructed to evaluate coronary arteries and to obtain presurgical staging of the underlying disease. The percentage of assessable segments ranged from 65.4% (972/1,485) when considering all coronary artery segments to 88% (613/693) for the proximal and mid segments, reaching 98% (387/396) for proximal coronary artery segments. The 387 interpretable proximal segments included 97 (97%) LM, 99 (100%) LAD, 96 (97%) LCX and 95 (96%) RCA with a mean attenuation of 280.70+/-52.93 HU. The mean percentage of assessable segments was significantly higher in patients with a heart rate <or=80 bpm (n=48) than in patients with a heart rate greater than 80 bpm (n=35) (80+/-11% vs. 72+/-13%; P=0.0008). Diagnostic image quality was achieved in all patients for preoperative staging of the underlying disorder. The mean estimated effective dose was 12.06+/-3.25 mSv for ECG-gated scans and 13.88+/-3.49 mSv for complete chest examinations. Proximal and mid-coronary artery segments can be adequately evaluated during presurgical CT examinations of the chest obtained with 64-slice MDCT without the administration of beta-blockers.
To provide an overview of non-specific modifications on whole-body post-mortem computed tomography (PMCT) images of infants and children.
69 infants and children underwent a whole-body PMCT scan at ...our institution following sudden unexpected death. Two paediatric radiologists reviewed the PMCT images, specifically focusing on non-specific postmortem modifications unrelated to the presumed cause of death.
Iatrogenic post-mortem modifications included focal infiltration of the legs (n=15) and hemopericardium (n=2). Vascular postmortem modifications included hypostasis (density in the posterior sagittal sinus was correlated with density in the dependent portion of the heart (p<0.001)), portal vein thrombosis (n=56, 75.3%), hyperattenuating aortic wall and reduced abdominal aortic diameter (n=69, 100%). Intravascular gas was detected in 40 subjects (57.9%). Ligamentum arteriosum calcification was seen in 42 children and was not correlated with age (p=0.68). Umbilical artery calcification was found in 30 children and was correlated with age (p<0.005). Gaseous distension of the stomach (n=45, 65.2%) and bowels (n=44, 63.7%) was a frequent finding. Mean liver density was 49.6±7.5HU and mean spleen density was 43.2±5.9HU. Ground-glass opacity was observed in 63 cases (91.3%) and mild bilateral consolidation in 16 cases (23.1%).
Non-specific post-mortem signs are rare and new to clinical paediatric radiologists. They should be aware of these signs when interpreting whole-body PMCT images in cases of sudden unexpected death in infancy or childhood in order to avoid pitfalls that may have a critical impact.
•Clinical paediatric radiologists should be aware of non-specific post-mortem signs.•Some non-specific post-mortem modifications in children are the same as in adults.•Whole-body PMCT has paediatric specificities.
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