A male infant was found to have a large congenital hepatic cyst,first noted in late gestation by prenatal ultrasound scan. The cyst communicated with the biliary tree and was eventually removed completely by an extend...A male infant was found to have a large congenital hepatic cyst,first noted in late gestation by prenatal ultrasound scan. The cyst communicated with the biliary tree and was eventually removed completely by an extended right hepatectomy. Histopathologic examination showed a thick-walled, unilocular cyst lined predominantly by ciliated, stratified squamous epithelium with an outer wall composed of smooth muscle cells and fibrous tissue.These features are diagnostic of a ciliated hepatic foregut cyst,a rare congenital malformation with histologic similarities to bronchogenic cysts. The young age of our patient, prenatal detection, large size of the cyst, and a clear communication with the biliary tree have not been previously described with ciliated hepatic foregut cysts.展开更多
Background/Purpose: There is a considerable variation in the use of vascular imaging techniques in the preoperative assessment of children scheduled for liver transplantation. Duplex Doppler ultrasound scan (US), magn...Background/Purpose: There is a considerable variation in the use of vascular imaging techniques in the preoperative assessment of children scheduled for liver transplantation. Duplex Doppler ultrasound scan (US), magnetic resonance angiography (MRA), and conventional angiography are used to varying extents. The authors compared the results of preoperative vascular imaging studies with operative findings to determine their accuracy and usefulness. Methods: Results of preoperative vascular imaging in 37 consecutive children undergoing cadaveric liver transplantation were compared with operative findings. Those undergoing relatively elective transplantations were investigated by US and MRA (group 1), whereas those requiring urgent transplants were assessed only by US (group 2). Results: The median age of the cohort (15 boys; 22 girls) was 4 years (19 days to 16 years) and the median weight was 17 kg (2.9 to 82 kg). In group 1 (n = 26), 20 children had a normalcaliber, patent portal vein at transplant and 6 had a narrow but patent portal vein requiring venous reconstruction in 4. The sensitivity and specificity of MRA in the detection of an abnormally narrow portal vein were 100% (6/6) and 95% (19/20), respectively. If reversed or absent flow in the portal vein on US was taken as an indication of a potentially abnormal vein, the sensitivity and specificity of Doppler US were 83% (5/6) and 95% (19/20), respectively. Magnetic resonance angiography revealed arterial anomalies in 4 children but failed to detect small accessory hepatic arteries in 5. The single patient with an aberrant vena cava was identified by MRA. In group 2 (n = 11), venous findings at operation and on US were concordant in 10 (91% ) cases; one infant with reversed flow in the portal vein on US had a thrombosed vein at surgery. Magnetic resonance angiography was useful in 2 patient groups: those with reversed flow on Doppler US or suspected portal vein thrombosis in whom an abnormal portal vein was present in 86% (6/7) and infants with the biliary atresia splenic malformation syndrome who had multiple venous and arterial anomalies. Conclusions: A detailed Doppler examination of the hepatic vasculature by an experienced sonographer/radiologist provides sufficient vascular imaging for most children scheduled for cadaveric liver transplantation. Routine MRA is recommended in children with the biliary atresia splenic malformation syndrome and in those with abnormal duplex Doppler US findings. Although there are limited data in this study, MRA is also valuable in children with Budd-Chiari syndrome, liver tumors, or a previous portosystemic shunt.展开更多
文摘A male infant was found to have a large congenital hepatic cyst,first noted in late gestation by prenatal ultrasound scan. The cyst communicated with the biliary tree and was eventually removed completely by an extended right hepatectomy. Histopathologic examination showed a thick-walled, unilocular cyst lined predominantly by ciliated, stratified squamous epithelium with an outer wall composed of smooth muscle cells and fibrous tissue.These features are diagnostic of a ciliated hepatic foregut cyst,a rare congenital malformation with histologic similarities to bronchogenic cysts. The young age of our patient, prenatal detection, large size of the cyst, and a clear communication with the biliary tree have not been previously described with ciliated hepatic foregut cysts.
文摘Background/Purpose: There is a considerable variation in the use of vascular imaging techniques in the preoperative assessment of children scheduled for liver transplantation. Duplex Doppler ultrasound scan (US), magnetic resonance angiography (MRA), and conventional angiography are used to varying extents. The authors compared the results of preoperative vascular imaging studies with operative findings to determine their accuracy and usefulness. Methods: Results of preoperative vascular imaging in 37 consecutive children undergoing cadaveric liver transplantation were compared with operative findings. Those undergoing relatively elective transplantations were investigated by US and MRA (group 1), whereas those requiring urgent transplants were assessed only by US (group 2). Results: The median age of the cohort (15 boys; 22 girls) was 4 years (19 days to 16 years) and the median weight was 17 kg (2.9 to 82 kg). In group 1 (n = 26), 20 children had a normalcaliber, patent portal vein at transplant and 6 had a narrow but patent portal vein requiring venous reconstruction in 4. The sensitivity and specificity of MRA in the detection of an abnormally narrow portal vein were 100% (6/6) and 95% (19/20), respectively. If reversed or absent flow in the portal vein on US was taken as an indication of a potentially abnormal vein, the sensitivity and specificity of Doppler US were 83% (5/6) and 95% (19/20), respectively. Magnetic resonance angiography revealed arterial anomalies in 4 children but failed to detect small accessory hepatic arteries in 5. The single patient with an aberrant vena cava was identified by MRA. In group 2 (n = 11), venous findings at operation and on US were concordant in 10 (91% ) cases; one infant with reversed flow in the portal vein on US had a thrombosed vein at surgery. Magnetic resonance angiography was useful in 2 patient groups: those with reversed flow on Doppler US or suspected portal vein thrombosis in whom an abnormal portal vein was present in 86% (6/7) and infants with the biliary atresia splenic malformation syndrome who had multiple venous and arterial anomalies. Conclusions: A detailed Doppler examination of the hepatic vasculature by an experienced sonographer/radiologist provides sufficient vascular imaging for most children scheduled for cadaveric liver transplantation. Routine MRA is recommended in children with the biliary atresia splenic malformation syndrome and in those with abnormal duplex Doppler US findings. Although there are limited data in this study, MRA is also valuable in children with Budd-Chiari syndrome, liver tumors, or a previous portosystemic shunt.
