This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell＇s syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell＇s pentalogy ...This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell＇s syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell＇s pentalogy including abdominal ectopia cordis, thoracic-abdominal ectopia cordis and left ventricular diverticulum. Of the three, the 5-month-old boy suffering from complicated congenital heart disease with abdominal ectopia cordis received a successful single stage repair and reconstruction of the abdominal wall. The 33-week-old premature girl with thoracic-abdominal ectopia cordis underwent two stage correction of tetraology of Fallot. The 4-year-old girl underwent ectomy of left ventricular diverticulum and thoracoabdominal wall repair. Twenty-four to thirty-five months follow-up were satisfactory. We hold that two-stage repair are technically feasible for Cantrell＇s syndrome, especially for those with complex congenital heart diseases. Post-operative ventilatory support and multiple post-operative care should be prolonged. Malnutrition, infection and arrhytbmia are central problems in medical care and surgery should be considered if there was progressive heart failure or hemodynamic instability.展开更多
Zollinger-Ellison syndrome(ZES)is characterized by gastric acid hypersecretion causing severe recurrent acid-related peptic disease.Excessive secretion of gastrin can now be effectively controlled with powerful proton...Zollinger-Ellison syndrome(ZES)is characterized by gastric acid hypersecretion causing severe recurrent acid-related peptic disease.Excessive secretion of gastrin can now be effectively controlled with powerful proton pump inhibitors,but surgical management to control gastrinoma itself remains controversial.Based on a thorough literature review,we design a surgical algorithm for ZES and list some significant consensus findings and recommendations:(1)For sporadic ZES,surgery should be routinely undertaken as early as possible not only for patients with a precisely localized diagnosis but also for those with negative imaging findings.The surgical approach for sporadic ZES depends on the lesion location(including the duodenum,pancreas,lymph nodes,hepatobiliary tract,stomach,and some extremely rare sites such as the ovaries,heart,omentum,and jejunum).Intraoperative liver exploration and lymphadenectomy should be routinely performed;(2)For multiple endocrine neoplasia type 1-related ZES(MEN1/ZES),surgery should not be performed routinely except for lesions>2 cm.An attempt to perform radical resection(pancreaticoduodenectomy followed by lymphadenectomy)can be made.The ameliorating effect of parathyroid surgery should be considered,and parathyroidectomy should be performed first before any abdominal surgery for ZES;and(3)For hepatic metastatic disease,hepatic resection should be routinely performed.Currently,liver transplantation is still considered an investigational therapeutic approach for ZES.Well-designed prospective studies are desperately needed to further verify and modify the current considerations.展开更多
Abdominal compartment syndrome(ACS)develops when organ failure arises secondary to an increase in intraabdominal pressure.The abdominal pressure is determined by multiple factors such as blood pressure,abdominal compl...Abdominal compartment syndrome(ACS)develops when organ failure arises secondary to an increase in intraabdominal pressure.The abdominal pressure is determined by multiple factors such as blood pressure,abdominal compliance,and other factors that exert a constant pressure within the abdominal cavity.Several conditions in the critically ill may increase abdominal pressure compromising organ perfusion that may lead to renal and respiratory dysfunction.Among surgical and trauma patients,aggressive fluid resuscitation is the most commonly reported risk factor to develop ACS.Other conditions that have also been identified as risk factors are ascites,hemoperitoneum,bowel distention,and large tumors.All patients with abdominal trauma possess a higher risk of developing intra-abdominal hypertension(IAH).Certain surgical interventions are reported to have a higher risk to develop IAH such as damage control surgery,abdominal aortic aneurysm repair,and liver transplantation among others.Close monitoring of organ function and intra-abdominal pressure(IAP)allows clinicians to diagnose ACS rapidly and intervene with target-specific management to reduce IAP.Surgical decompression followed by temporary abdominal closure should be considered in all patients with signs of organ dysfunction.There is still a great need for more studies to determine the adequate timing for interventions to improve patient outcomes.展开更多
Mirizzi syndrome is a serious complication of gallstone disease.It is caused by the impacted stones in the gallbladder neck or cystic duct.One of the features of Mirizzi syndrome is severe inflammation or dense fibros...Mirizzi syndrome is a serious complication of gallstone disease.It is caused by the impacted stones in the gallbladder neck or cystic duct.One of the features of Mirizzi syndrome is severe inflammation or dense fibrosis at the Calot’s triangle.In our clinical practice,bile duct,branches of right hepatic artery and right portal vein clinging to gallbladder infundibulum are often observed due to gallbladder infundibulum adhered to right hepatic hilum.The intraoperative damage of branches of right hepatic artery occurs more easily than that of bile duct,all of which are hidden pitfalls for surgeons.Magnetic resonance cholangiopancreatography(MRCP)and endoscopic retrograde cholangiopancreatography(ERCP)are the preferable tools for the diagnosis of Mirizzi syndrome.Anterograde cholecystectomy in Mirizzi syndrome is easy to damage branches of right hepatic artery and bile duct due to gallbladder infundibulum adhered to right hepatic hilum.Subtotal cholecystectomy is an easy,safe and definitive approach to Mirizzi syndrome.When combined with the application of ERCP,a laparoscopic management of Mirizzi syndrome by well-trained surgeons is feasible and safe.The objective of this review was to highlight its existing problems:(1)low preoperative diagnostic rate,(2)easy to damage bile duct and branches of right hepatic artery,and(3)high concomitant gallbladder carcinoma.Meanwhile,the review aimed to discuss the possible therapeutic strategies:(1)to enhance its preoperative recognition by imaging findings,and(2)to avoid potential pitfalls during surgery.展开更多
McCune Albright syndrome is a rare genetic disorder which is characterized by café au lait skin pigmentation, precocious puberty and polyostotic fibrous dysplasia. Treating recurring pathological fractures due to...McCune Albright syndrome is a rare genetic disorder which is characterized by café au lait skin pigmentation, precocious puberty and polyostotic fibrous dysplasia. Treating recurring pathological fractures due to Albright syndrome is a very challenging endeavor, and more so when it is accompanied by poor bone quality and deformity. We hereby present the case of a 23-year-old male patient who is treated several times for recurrent pathological fractures of the femur at our center. We analyze the difficulties associated with treating a patient with poor bone quality over several years, discuss our treatment options, review the literature for similar cases and look at what we could have done differently. We weigh in on the difficulties in treating a severely deformed shepherd’s crook, the ways of achieving proper internal fixation and the dangers of using plating instead of an IM nail as suggested in the literature. Our main goal in reporting this case is to bring forth the unusual challenges encountered when treating patients with Albright syndrome and discussing the options of the orthopedic surgeons when treating these types of patients.展开更多
Background Budd-Chiari syndrome （BCS） is characterized by liver sinusoidal congestion,ischemic liver cell damage,and liver portal hypertension caused by hepatic venous outflow constriction.The aim of this research w...Background Budd-Chiari syndrome （BCS） is characterized by liver sinusoidal congestion,ischemic liver cell damage,and liver portal hypertension caused by hepatic venous outflow constriction.The aim of this research was to investigate the clinicopathological features of BCS-associated hepatocellular carcinoma （HCC） and explore its surgical treatment and prognosis.Methods Clinical data from 38 patients with BCS-associated HCC who were surgically treated in our hospital from July 1998 to August 2010 were retrospectively analyzed.The clinicopathological features and prognosis of patients with BCS-associated HCC and surgical treatment for BCS-associated HCC were investigated.Results Compared to the patients with hepatitis B virus （HBV）-associated HCC,the patients with BCS-associated HCC showed a female predominance,and had significantly higher cirrhosis rate,higher incidence of solitary tumors,lower incidence of infiltrative growth,higher proportion of marginal or exogenous growth,lower rate of portal vein invasion,and higher degree of differentiation.Median survival was longer in patients with BCS-associated HCC （76 months） than in those with HBV associated HCC （38 months）.Of 38 patients with BCS-associated HCC,22 patients who received combined surgery mainly by liver resection plus cavoatrial shunts exhibited hepatic venous outflow constriction relief,while the other 16 patients only underwent liver resection.The combined surgery group had significantly longer survival and lower incidences of post-operative lethal complications （P 〈0.05）.Multivariate analysis showed that relief of hepatic venous outflow obstruction was a protective factor for survival of patients with BCS-associated HCC,whereas portal vein invasion was a risk factor.Conclusions BCS-associated HCC has a more favorable biological behavior and prognosis than HBV-associated HCC.For patients with BCS-associated HCC,tumor resection accompanied with relief of hepatic venous outflow obstruction can reduce the incidence of complications and extend survival.展开更多
文摘This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell＇s syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell＇s pentalogy including abdominal ectopia cordis, thoracic-abdominal ectopia cordis and left ventricular diverticulum. Of the three, the 5-month-old boy suffering from complicated congenital heart disease with abdominal ectopia cordis received a successful single stage repair and reconstruction of the abdominal wall. The 33-week-old premature girl with thoracic-abdominal ectopia cordis underwent two stage correction of tetraology of Fallot. The 4-year-old girl underwent ectomy of left ventricular diverticulum and thoracoabdominal wall repair. Twenty-four to thirty-five months follow-up were satisfactory. We hold that two-stage repair are technically feasible for Cantrell＇s syndrome, especially for those with complex congenital heart diseases. Post-operative ventilatory support and multiple post-operative care should be prolonged. Malnutrition, infection and arrhytbmia are central problems in medical care and surgery should be considered if there was progressive heart failure or hemodynamic instability.
基金Supported by the National Natural Science Foundation,No.81773215the General Financial Grant from the China Postdoctoral Science Foundation,No.2017M610813
文摘Zollinger-Ellison syndrome(ZES)is characterized by gastric acid hypersecretion causing severe recurrent acid-related peptic disease.Excessive secretion of gastrin can now be effectively controlled with powerful proton pump inhibitors,but surgical management to control gastrinoma itself remains controversial.Based on a thorough literature review,we design a surgical algorithm for ZES and list some significant consensus findings and recommendations:(1)For sporadic ZES,surgery should be routinely undertaken as early as possible not only for patients with a precisely localized diagnosis but also for those with negative imaging findings.The surgical approach for sporadic ZES depends on the lesion location(including the duodenum,pancreas,lymph nodes,hepatobiliary tract,stomach,and some extremely rare sites such as the ovaries,heart,omentum,and jejunum).Intraoperative liver exploration and lymphadenectomy should be routinely performed;(2)For multiple endocrine neoplasia type 1-related ZES(MEN1/ZES),surgery should not be performed routinely except for lesions>2 cm.An attempt to perform radical resection(pancreaticoduodenectomy followed by lymphadenectomy)can be made.The ameliorating effect of parathyroid surgery should be considered,and parathyroidectomy should be performed first before any abdominal surgery for ZES;and(3)For hepatic metastatic disease,hepatic resection should be routinely performed.Currently,liver transplantation is still considered an investigational therapeutic approach for ZES.Well-designed prospective studies are desperately needed to further verify and modify the current considerations.
文摘Abdominal compartment syndrome(ACS)develops when organ failure arises secondary to an increase in intraabdominal pressure.The abdominal pressure is determined by multiple factors such as blood pressure,abdominal compliance,and other factors that exert a constant pressure within the abdominal cavity.Several conditions in the critically ill may increase abdominal pressure compromising organ perfusion that may lead to renal and respiratory dysfunction.Among surgical and trauma patients,aggressive fluid resuscitation is the most commonly reported risk factor to develop ACS.Other conditions that have also been identified as risk factors are ascites,hemoperitoneum,bowel distention,and large tumors.All patients with abdominal trauma possess a higher risk of developing intra-abdominal hypertension(IAH).Certain surgical interventions are reported to have a higher risk to develop IAH such as damage control surgery,abdominal aortic aneurysm repair,and liver transplantation among others.Close monitoring of organ function and intra-abdominal pressure(IAP)allows clinicians to diagnose ACS rapidly and intervene with target-specific management to reduce IAP.Surgical decompression followed by temporary abdominal closure should be considered in all patients with signs of organ dysfunction.There is still a great need for more studies to determine the adequate timing for interventions to improve patient outcomes.
文摘Mirizzi syndrome is a serious complication of gallstone disease.It is caused by the impacted stones in the gallbladder neck or cystic duct.One of the features of Mirizzi syndrome is severe inflammation or dense fibrosis at the Calot’s triangle.In our clinical practice,bile duct,branches of right hepatic artery and right portal vein clinging to gallbladder infundibulum are often observed due to gallbladder infundibulum adhered to right hepatic hilum.The intraoperative damage of branches of right hepatic artery occurs more easily than that of bile duct,all of which are hidden pitfalls for surgeons.Magnetic resonance cholangiopancreatography(MRCP)and endoscopic retrograde cholangiopancreatography(ERCP)are the preferable tools for the diagnosis of Mirizzi syndrome.Anterograde cholecystectomy in Mirizzi syndrome is easy to damage branches of right hepatic artery and bile duct due to gallbladder infundibulum adhered to right hepatic hilum.Subtotal cholecystectomy is an easy,safe and definitive approach to Mirizzi syndrome.When combined with the application of ERCP,a laparoscopic management of Mirizzi syndrome by well-trained surgeons is feasible and safe.The objective of this review was to highlight its existing problems:(1)low preoperative diagnostic rate,(2)easy to damage bile duct and branches of right hepatic artery,and(3)high concomitant gallbladder carcinoma.Meanwhile,the review aimed to discuss the possible therapeutic strategies:(1)to enhance its preoperative recognition by imaging findings,and(2)to avoid potential pitfalls during surgery.
文摘McCune Albright syndrome is a rare genetic disorder which is characterized by café au lait skin pigmentation, precocious puberty and polyostotic fibrous dysplasia. Treating recurring pathological fractures due to Albright syndrome is a very challenging endeavor, and more so when it is accompanied by poor bone quality and deformity. We hereby present the case of a 23-year-old male patient who is treated several times for recurrent pathological fractures of the femur at our center. We analyze the difficulties associated with treating a patient with poor bone quality over several years, discuss our treatment options, review the literature for similar cases and look at what we could have done differently. We weigh in on the difficulties in treating a severely deformed shepherd’s crook, the ways of achieving proper internal fixation and the dangers of using plating instead of an IM nail as suggested in the literature. Our main goal in reporting this case is to bring forth the unusual challenges encountered when treating patients with Albright syndrome and discussing the options of the orthopedic surgeons when treating these types of patients.
文摘Background Budd-Chiari syndrome （BCS） is characterized by liver sinusoidal congestion,ischemic liver cell damage,and liver portal hypertension caused by hepatic venous outflow constriction.The aim of this research was to investigate the clinicopathological features of BCS-associated hepatocellular carcinoma （HCC） and explore its surgical treatment and prognosis.Methods Clinical data from 38 patients with BCS-associated HCC who were surgically treated in our hospital from July 1998 to August 2010 were retrospectively analyzed.The clinicopathological features and prognosis of patients with BCS-associated HCC and surgical treatment for BCS-associated HCC were investigated.Results Compared to the patients with hepatitis B virus （HBV）-associated HCC,the patients with BCS-associated HCC showed a female predominance,and had significantly higher cirrhosis rate,higher incidence of solitary tumors,lower incidence of infiltrative growth,higher proportion of marginal or exogenous growth,lower rate of portal vein invasion,and higher degree of differentiation.Median survival was longer in patients with BCS-associated HCC （76 months） than in those with HBV associated HCC （38 months）.Of 38 patients with BCS-associated HCC,22 patients who received combined surgery mainly by liver resection plus cavoatrial shunts exhibited hepatic venous outflow constriction relief,while the other 16 patients only underwent liver resection.The combined surgery group had significantly longer survival and lower incidences of post-operative lethal complications （P 〈0.05）.Multivariate analysis showed that relief of hepatic venous outflow obstruction was a protective factor for survival of patients with BCS-associated HCC,whereas portal vein invasion was a risk factor.Conclusions BCS-associated HCC has a more favorable biological behavior and prognosis than HBV-associated HCC.For patients with BCS-associated HCC,tumor resection accompanied with relief of hepatic venous outflow obstruction can reduce the incidence of complications and extend survival.
文摘阴道松弛综合征(vaginal laxity syndrome,VLS)属于盆底功能障碍性疾病,VLS是由于妊娠、分娩、长期腹压增高、细胞老化等多种原因引起盆底支持结构松弛、盆底结构缺陷,导致阴道口和阴道壁松弛的病症,严重者可以发展为盆腔器官脱垂(pelvic organ prolapsed,POP),为妇产科常见病和多发病,影响女性的生活质量。本文通过查阅古籍及临床文献,对该病古代中医和现代中医治疗方式,CO_(2)激光治疗、铒(Er):YAG激光治疗、射频、盆底肌训练(PFMT)和局部雌激素替代疗法等非手术治疗方法进行阐述。
