Polysplenia syndrome, defined as the presence of multiple spleens of almost equal volume, is a rare condition involving congenital anomalies in multiple organ systems. We report this anomaly in a 41-year-old female wh...Polysplenia syndrome, defined as the presence of multiple spleens of almost equal volume, is a rare condition involving congenital anomalies in multiple organ systems. We report this anomaly in a 41-year-old female who underwent a left lateral sectionectomy due to recurrent cholangitis and impacted left lateral duct stones. Polysplenia syndrome with preduodenal vein was diagnosed preoperatively by computed to-mography (CT) and surgery was done safely. Although the polysplenia syndrome with preduodenal portal vein (PDPV) in adult is rarely encountered, surgeons need to understand the course of the portal vein and exercise caution in approaching the biliary tract.展开更多
Biliary atresia (BA) is the result of a process of unknown etiology which can occur as an isolated lesion or in association with several congenital abnormalities and remains the main indications of liver transplantati...Biliary atresia (BA) is the result of a process of unknown etiology which can occur as an isolated lesion or in association with several congenital abnormalities and remains the main indications of liver transplantation among infants. One of the malformations associated with biliary atresia is Situs inversus totalis, which is a rare congenital abnormality found in 10% - 20% of infants with biliary atresia and presents an incidence varying from 1:5000 to 1:20,000. This case report aims to present an extremely rare case of a patient with Situs inversus associated with biliary atresia, polysplenia and abscence of inferior vena cava that underwent liver transplantation. Case Report: A 10-month-old boy was referred to our hospital with the diagnosis of cirrhosis, due to biliary atresia. Abdominal ultrasound and Doppler scan showed the liver in the left upper quadrant as well as signs of chronic liver disease. In addition, we could notice that the spleen had several lobes, suggesting polysplenia, and was positioned in the right upper quadrant. The patient underwent living donor liver transplant. His mother was the donor and the graft, segment III, was positioned in the left upper quadrant associated with a Y en Roux reconstruction of the biliary tract. During the first post-operatory day, the patient presented signs of disseminated sepsis. Laboratory and imaging diagnostic tests were performed presenting a small intestinal malrotation associated with diffuse ischemia. The patient died at the third day of PO due to complications related to this condition. Conclusion: Any patient with Situs inversus with acute abdomen should be aggressively investigated for other structural and biochemical abnormalities. If liver transplant is indicated, the procedure can be successfully performed.展开更多
Aims:Evidence is emerging that,in the setting of isomerism,the atrial and bronchial arrangement are not always concordant,nor are these patterns always harmonious with the arrangement of the abdominal organs.We aimed ...Aims:Evidence is emerging that,in the setting of isomerism,the atrial and bronchial arrangement are not always concordant,nor are these patterns always harmonious with the arrangement of the abdominal organs.We aimed to evaluate the concordance between these features in a cohort of patients with cardiac malformations in the setting of known isomerism,seeking to determine whether it was feasible to assess complexity on this basis,in this regard taking note of the potential value of bronchial as opposed to appendage morphology.Methods and Results:We studied 78 patients known to have isomerism of the bronchuses,43 with right and 35 with left isomerism.Appendage anatomy could be determined in 49 cases(63%),all but one of these being concordant with bronchial anatomy.When assessing abdominal features,in only 59 cases(76%)was splenic morphology in keeping with the thoracic findings.As expected,right isomerism was associated with greater complexity of cardiac malformations,with an odds ratio of 6.53,with confidence intervals from 2.2–19.3(p<0.001).The odds were slightly decreased with thoraco-abdominal disharmony,when lesions shown to carry higher risk were then found in the setting of left isomerism.Conclusion:Harmony is excellent between bronchial and appendage isomerism,but less so with the arrangement of the abdominal organs.Right isomerism in our cohort,was indicative of a sixfold increase in intracardiac complexity.When discordance was found between the systems,however,the cardiac anomalies were less typical of the anticipated findings for right vs.left isomerism of the appendages.展开更多
Congenital duodenal obstruction (CDO) is one of the most common anomalies presented in the neonatal period of intestinal obstruction, with an incidence of 1:10,000, and Preduodenal Portal Vein (PDPV) is a rare extrins...Congenital duodenal obstruction (CDO) is one of the most common anomalies presented in the neonatal period of intestinal obstruction, with an incidence of 1:10,000, and Preduodenal Portal Vein (PDPV) is a rare extrinsic cause of CDO. PDPV leads to CDO with a persistent vomiting, impairment of the oral feeding and weight loss, but other publications state an asymptomatic disease that may requires late surgical correction. Prenatal diagnosis of CDO, including PDPV, is evidenced by association of polyhydramnios with a dilated fluid-filled gastric and duodenum and a double-bobble image in the ultrasonography and permits a proper management of associated malformation, delivery, surgery and allows family counseling. PDPV is commonly associated to other intestinal obstruction such as extrinsic (annular pancreas, adhesive bands, malrotation, peritoneal bands, intestinal duplication), and intrinsic (atresia, web and stenosis), and also associated with cardiovascular anomalies, situs inversus, Down’s syndrome and polysplenia. This is full term 5 days old newborn, 2865 g girl, without prenatal diagnosis of CDO. It was not still established PDPV until surgery procedure such as Duodenoduodenostomy (Diamond-Shape) and can guide Paediatric Surgeons that face high intestinal obstruction in the neonate with or without prenatal diagnosis. In the ambulatory follow up after 4 months, baby has developed very well, in breast-feeding associated with formula-feeding with a normal weight gain (average of 550 g/month).展开更多
文摘Polysplenia syndrome, defined as the presence of multiple spleens of almost equal volume, is a rare condition involving congenital anomalies in multiple organ systems. We report this anomaly in a 41-year-old female who underwent a left lateral sectionectomy due to recurrent cholangitis and impacted left lateral duct stones. Polysplenia syndrome with preduodenal vein was diagnosed preoperatively by computed to-mography (CT) and surgery was done safely. Although the polysplenia syndrome with preduodenal portal vein (PDPV) in adult is rarely encountered, surgeons need to understand the course of the portal vein and exercise caution in approaching the biliary tract.
文摘Biliary atresia (BA) is the result of a process of unknown etiology which can occur as an isolated lesion or in association with several congenital abnormalities and remains the main indications of liver transplantation among infants. One of the malformations associated with biliary atresia is Situs inversus totalis, which is a rare congenital abnormality found in 10% - 20% of infants with biliary atresia and presents an incidence varying from 1:5000 to 1:20,000. This case report aims to present an extremely rare case of a patient with Situs inversus associated with biliary atresia, polysplenia and abscence of inferior vena cava that underwent liver transplantation. Case Report: A 10-month-old boy was referred to our hospital with the diagnosis of cirrhosis, due to biliary atresia. Abdominal ultrasound and Doppler scan showed the liver in the left upper quadrant as well as signs of chronic liver disease. In addition, we could notice that the spleen had several lobes, suggesting polysplenia, and was positioned in the right upper quadrant. The patient underwent living donor liver transplant. His mother was the donor and the graft, segment III, was positioned in the left upper quadrant associated with a Y en Roux reconstruction of the biliary tract. During the first post-operatory day, the patient presented signs of disseminated sepsis. Laboratory and imaging diagnostic tests were performed presenting a small intestinal malrotation associated with diffuse ischemia. The patient died at the third day of PO due to complications related to this condition. Conclusion: Any patient with Situs inversus with acute abdomen should be aggressively investigated for other structural and biochemical abnormalities. If liver transplant is indicated, the procedure can be successfully performed.
文摘Aims:Evidence is emerging that,in the setting of isomerism,the atrial and bronchial arrangement are not always concordant,nor are these patterns always harmonious with the arrangement of the abdominal organs.We aimed to evaluate the concordance between these features in a cohort of patients with cardiac malformations in the setting of known isomerism,seeking to determine whether it was feasible to assess complexity on this basis,in this regard taking note of the potential value of bronchial as opposed to appendage morphology.Methods and Results:We studied 78 patients known to have isomerism of the bronchuses,43 with right and 35 with left isomerism.Appendage anatomy could be determined in 49 cases(63%),all but one of these being concordant with bronchial anatomy.When assessing abdominal features,in only 59 cases(76%)was splenic morphology in keeping with the thoracic findings.As expected,right isomerism was associated with greater complexity of cardiac malformations,with an odds ratio of 6.53,with confidence intervals from 2.2–19.3(p<0.001).The odds were slightly decreased with thoraco-abdominal disharmony,when lesions shown to carry higher risk were then found in the setting of left isomerism.Conclusion:Harmony is excellent between bronchial and appendage isomerism,but less so with the arrangement of the abdominal organs.Right isomerism in our cohort,was indicative of a sixfold increase in intracardiac complexity.When discordance was found between the systems,however,the cardiac anomalies were less typical of the anticipated findings for right vs.left isomerism of the appendages.
文摘Congenital duodenal obstruction (CDO) is one of the most common anomalies presented in the neonatal period of intestinal obstruction, with an incidence of 1:10,000, and Preduodenal Portal Vein (PDPV) is a rare extrinsic cause of CDO. PDPV leads to CDO with a persistent vomiting, impairment of the oral feeding and weight loss, but other publications state an asymptomatic disease that may requires late surgical correction. Prenatal diagnosis of CDO, including PDPV, is evidenced by association of polyhydramnios with a dilated fluid-filled gastric and duodenum and a double-bobble image in the ultrasonography and permits a proper management of associated malformation, delivery, surgery and allows family counseling. PDPV is commonly associated to other intestinal obstruction such as extrinsic (annular pancreas, adhesive bands, malrotation, peritoneal bands, intestinal duplication), and intrinsic (atresia, web and stenosis), and also associated with cardiovascular anomalies, situs inversus, Down’s syndrome and polysplenia. This is full term 5 days old newborn, 2865 g girl, without prenatal diagnosis of CDO. It was not still established PDPV until surgery procedure such as Duodenoduodenostomy (Diamond-Shape) and can guide Paediatric Surgeons that face high intestinal obstruction in the neonate with or without prenatal diagnosis. In the ambulatory follow up after 4 months, baby has developed very well, in breast-feeding associated with formula-feeding with a normal weight gain (average of 550 g/month).
