From 1978 to Dec. 1991, 50 cases of dilatation of the extrahepatic biliary duct in children were treated. They were classified as: cystic dilatation in 34 cases, arid fusiform dilatation in 16 cases. Types of reconstruction of the extrahepatic biliary duct included: excision of cystic dilatation and Rorx-en-Y hepatoductojejunostomy in 25 cases, and interposition of jejunum and hepatoductoduodenostomy in 9 eases. for those cases having fusiform dilatation, interposition of jejunum and hepatoductoduodenostomy，cases and Rorx-en-Y-hepatoductojejunostmy 5 cases.The follow-up period averajed 6.5 years. Forty nine patients were recoverwd from the teatment and 1 patient died.
Since October 1975, 35 patients with congenital constriction bands were treated. Follow-up for 10 months to 14 years showed that the results of correction were satisfactory except 1 patient died from other disease. The etiolology, the concurrent deformities and the method of treatment were discussed.
For the evaluation of the result from McKay operation in the correction of rigid clubfoot, 71 patients (103 feet) were followed up postoperatively for 2 to 9 years. According to the criteria concerning the functional recovery of the foot, the external appearance of the foot after correction and the roentgenographic findings, the result was evaluated as excellent in 55 patients (78 feet), good in 11 patients (16 feet), fair in 5 patients (9 feet) and with no recurrence. It was considered that the favorable time for operation was when the child being 6 to 18 months old. In 7 patients (12 feet), there were morphological changes of the calcaneal and navicular bones in 12 feet, limitation of ankle movement in 9 patients and resultant flatfoot deformity in 5 patients (7 feet). The factors influencing the functional recovery of the foot and the relationship between the corrective result and the age of patient when the operation being given were discussed in details.
Objective
To discuss the imaging characteristics and clinical treatment methods of congenital biliary dilation.
Methods Clinical data of 70 cases of congenital biliary dilation who treated in The Third Affiliated Hospital of Henan University of TCM and Henan Provincial People’s Hospital from Jan. 2010 to Jan. 2015 was collected and analyzed.
Methods
Along the bile duct region (all cases received ultrasound), the ultrasound of 57 cases (81.4%) showed irregular spherical, spindle, or prismatic area without echo connected to the proximal part of the bile duct, the intracavity wall of it was skin pass rolling, and part of it had the spotty detailed or slightly strong stones sound shadow, 1 case combined spindle or capsular area without echo connected to the intrahepatic bile duct partly and along with it. CT of 45 cases (64.3%, 43 cases were diagnosed as congenital biliary dilation) without and with enhancement scanning showed low-density and irregular oblate, cystiform, columniform or fusiform expansion shadow, slight mass effect, no difference before and after the enhancement of partial intrahepatic bile duct and choledoch, and that the structure of surrounding tissues were compressed, lapsed, and deformed. The MRI and magnetic resonance cholangiopancreatography (MRCP) inspection of 65 cases (92.8%, all of 65 cases were diagnosed as congenital biliary dilation) showed tadpole-shaped, irregular cystiform, columniform or fusiform expansion with long T2 and high MRCP signal shadow image of partial intrahepatic bile duct and choledoch, the dilated bile duct also being connected to biliary tree. Of the 70 cases, there were 66 cases (94.3%) of type Ⅰ, 1 case (1.4%) of type Ⅱ, 1 case (1.4%) of type Ⅳa, 2 cases (2.9%) of type Ⅳb. Sixty eight cases (65 cases of type Ⅰ, 1 case of type Ⅱ, 2 cases of type Ⅳb) underwent cholecystectomy+dilated bile ducts resection+common hepatic duct jejunum anastomosis (Roux-en-Y), 1 case underwent cholecystectomy+dilated bile ducts resection+pancreatic duodenal resection, another 1 case underwent cholecystectomy+dilated bile ducts resection+common hepatic duct jejunum anastomosis (Roux-en-Y)+resection of left hepatic lobe. All the cases were successfully recovered without severe complications and had no dead case, but 13 cases (18.6%) suffered from minor complications, including 6 cases of short-term abdominal pain and abdominal distension, 1 case of bile leak, 2 cases of incision infection, 3 cases of pulmonary infection, and 1 case of alteration of intestinal flora. All of the 70 cases were followed-up for 6-56 months (average of 36 months). During the follow up period, 2 cases died in reason of other incidence, 4 cases suffered from simple cholangitis, 3 cases suffered from cholangitis combined with intrahepatic bile duct stone, 2 cases suffered from cholangitis combined with intrahepatic bile duct stone, slight anastomotic stoma stenosis, and mild jaundice, 2 cases suffered from cholangitis.
Conclusion
Congenital biliary dilation has no typical clinical feature, but it has identifiable imaging manifestation, which can provide a theoretical foundation for congenital biliary dilation in diagnosing, preoperative evaluation, and chosing operative methods. Ultrasound is the first choice, MRI and MRCP are propitious to diagnose, locate, and classfy. The treatment of congenital biliary dilation is resecting the dilated bile ducts fully and performing the common hepatic duct jejunum anastomosis.
Objective To investigate the effect of prostaglandin E1 (PGE1) on serum vascular endothelial growth factor(VEGF) in patient with pulmonary hypertension secondary to congenital heart disease and its relation to different pathologic gradings of pulmonary arterioles. Methods Fifty three patients suffering from pulmonary hypertension secondary to congenital heart disease were chosen at random to undergo active tissue test of lung, including 6 patients suffering from severe cyanosis. All of them were intravenously dripped with PGE 1 for 15 days at the speed of 10 15 ng /kg·min, 12 hours a day. Venous blood was taken for study in the morning on the day before infusion, on the 5th day, the 10th day, and the 15th day after infusion. Then the concentration of VEGF was measured by enzyme linked immunosorbent assay (ELISA). Lung biopsy was taken from each patient and pathologic grading performed according to Heath and Edwards pathologic grading. Results Fifty three patients were classified into Grade Ⅴ:9 of them belonged to Grade Ⅰ, 14 to Grade Ⅱ, 19 to Grade Ⅲ, 5 to Grade Ⅳ, the other 6 with severe cyanosis belonged to Grade Ⅴ or even severe than Grade Ⅴ. Before administration of PGE 1, serum VEGF reached the peak while the pathologic grading of pulmonary arteriole was Grade Ⅲ, VEGF level markedly decreased in Grade Ⅳ and Ⅴ. After administration of PGE 1 serum VEGF in Grade Ⅰ showed no difference with that before administration of PGE 1( P gt;0.05), VEGF decreased in GradeⅡ and Ⅲ ( P lt;0.01), slightly decreased in Grade Ⅳ ( P lt; 0.05), while patients greater or equivalent to Grade Ⅴ showed no VEGF change during the course of PGE 1 administration ( P gt;0.05). Conclusions PGE 1 can lower the VEGF level, but the extent closely relates to the degree of pathologic change in pulmonary arteriole. It might be a pre operative parameter for pathologic grading of pulmonary arteriole.
ObjectiveTo summarize the minimally invasive experiences and medium-long-term results of perventricular device closure of ventricular septal defects (VSD) under transesophageal echocardiography (TEE) guidance.MethodsWe retrospectively analyzed the clinical data and medium-long-term follow-up results of 783 patients who undertook perventricular device closure under TEE guidance in Dalian Children’s Hospital from July 2011 to January 2020, in which perimembrane VSD were found in 598 patients, VSD with aortic valve prolapse in 135 patients and muscular VSD in 2 patients. There were 463 males and 320 females at age of 5 months to 13 years with average age of 3.3±1.2 years, and body weight of 5.9-51.0 (15.9±8.3) kg. The left ventricular defect diameter of the VSD ranged from 5.0 to 11.0 mm, with an average of 6.3±1.2 mm. The right ventricular defect diameter of the VSD ranged from 2.3 to 8.0 mm, with an average of 4.3±0.9 mm.ResultsThe procedures were completed successfully in 753 patients. The device of 1 patient (0.1%) fell off and embedded in the right pulmonary artery after the operation, and the occluder was taken out and the VSD was closed with cardiopulmonary bypass (CPB) in the secondary operation. One patient (0.1%) appeared Ⅲ degree atrioventricular block in 2 years after operation. The device was taken out and VSD was closed with CPB in the secondary operation, and the patient gradually reached to sinus rhythm in post-operation. Eight patients (1.1%) presented delayed pericardial effusion in 1 week after operation, and were cured by pericardiocentesis with ultrasound-guided. Symmetric occluders were used in 580 patients, eccentric occleders were used in 171 patients and muscular occluders were used in 2 patients. The follow-up time was 9 months to 9 years. The rate of loss to follow-up was 96.7% (704/728). No residual shunt, occlude-loss or arrhythmia was found during follow-up. Conclusion The minimally invasive penventricular device closure of VSD guided by TEE is safe and availabe. Medium-long-term follow-up results are satisfactory, it is worthy of clinical promotion, and longer term follow-up is still needed.
Abstract： Objective?To evaluate clinical experiences and long-term outcome of morphologic left ventricle （mLV） retraining for congenitally corrected transposition of the great arteries （cCTGA）. Methods From May 2005 to May 2011, 24 patients with cCTGA anomaly underwent left ventricle retraining by means of pulmonary artery banding in Fu Wai Hospital. There were 13 males and 11 females with their age of 0.17-22.00 （3.73±4.35） years and body weight of 5.10-61.00（15.71±10.95）kg. Major concomitant malformations included tricuspid valve insufficiency （TR）in 23 patients （mild in 11 patients, moderate in 7 patients, severe in 5 patients）, restrictive ventricular septal defect in 18 patients, atrial septal defect in 5 patients, patent foramen ovale in 5 patients, patent ductus arteriosus in 4 patients, mild pulmonary stenosis in 5 patients, and aortic coarctation in 1 patient. All the patients were preoperatively diagnosed by echocardiography, cardiovascular angiography or cardiac catheterization. The mLV end diastolic diameter （mLVEDD） was 8-32（21.56±6.60）mm, posterior wall thickness of mLV was 2-7 （4.29±1.52）mm , mLV to morphologic right ventricle （mRV） pressure ratio （mLV/mRV） was 0.12-0.65 （0.41±0.12）. Pulmonary artery banding operation was performed through upper partial sternotomy or median sternotomy without circulatory arrest. Results The mLV/mRV pressure ratio reached to 0.57-0.93 （0.76±0.10） under direct pressure monitoring after surgery. There was no in-hospital death in this group. Echocardiography before discharge showed that the structure and function of the two ventricles were good, the interventricular septum moved partially towards mRV, mLVEDD was increased slightly, and there was a tendency of reduced TR. Postoperative follows-up was from 1 to 35 months, and there was no late death during follow-up. All the patients were in good general condition with stable vital signs and New York Heart Association （NYHA） classⅠ-Ⅱ. The mLVEDD was 14-40 （26.17±7.11） mm, posterior wall thickness of mLV was 4-9 （4.95±1.44）mm, mLV/mRV pressure ratio was 0.52-0.98 （0.72±0.16） , and TR was significantly decreased. Fourteen patients successfully underwent staged complete double-switch procedure. Conclusion Left ventricle retraining is a safe and effective method to train mLV for cCTGA patients. Pressure load and posterior wall thickness of mLV are increased, mLV cavity is dilated, and TR is significantly reduced after the surgery.
ObjectiveTo review the current development in therapy of congenital funnel chest. MethodsRecent literature concerning the development of the treatment method for congenital funnel chest was extensively reviewed and summarized. ResultsThe main therapies for congenital funnel chest are thoracoplasty (Ravitch sternum elevation procedure and minimal invasive Nuss procedure) and prosthesis implantation. The magnetic mini-mover procedure and the vacuum bell are still in the research phase. ConclusionBesides the improvement in function, the requirement in appearance after surgery is also improved in the treatment of congenital funnel chest. The minimally invasive surgery and non-invasive procedures could be expected in the future.
Twenty five children with congenital biliary dilatation were treated with hepatico-jejuno-duodenostomy following excision of choledochal cysts between 1983 and 1985. The age ranged from two months to eleven years. The last follow-up ranged from 6-9 years (mean 7.5 years). All patients were free of jaundice with normal growth and development and none had peptic ulcer. The results from the last follow-up was better than that of the first one. This procedure was safe, effective and physiologically appealing.
In order to observe the absorption of the fat solube vitamine afer operative treatment of the congenital biliary dilation(CBD),the plasma fat soluble vitamin A,D and E were determined in 57 cases of CBD at the postoperative stage and 51 cases of normal children as control.The normal values of vitamin A,D and E was 576.25±170.93ng/ml,13.21±2.20ng/ml and 7.34±1.96ng/ml respectively in control group versus 501.59±120.64ng/ml, 11.66±1.81ng/ml and 6.16±1.18ng/ml respectively in the postoperative group of CBD.The differences were significant for a long period of about 10 years,and gradually disappear after that to approach or near the normal level.Therefor,the radical operation of CBD may affect the absorption of the fat soluble vitamins.
