Objective:To evaluate the safety and efficacy of enhanced recovery after surgery(ERAS) in the perioperative period of congenital choledochal cysts in children.Methods:This is a multicenter prospective randomized controlled study. The clinical data of 273 pediatric congenital choledochal cysts(CCC) patients who underwent surgery at 14 medical centers with complete follow-up data were collected through the medical data analysis platform. Among them, 123 cases in ERAS group were managed perioperatively in strict accordance with ERAS mode, and 150 cases in conventional group were managed according to traditional mode. The length of hospital stay,time to first farting, time to complete feeding, the incidence of complications, cost and readmission rate within 30 days,stress indexes and liver function were compared between the two groups.Results:Compared with the conventional group, median time to start farting (2.0 d vs. 3.0 d, P<0.001), median time to complete feeding (5.0 d vs. 7.0 d, P<0.001), median postoperative hospitalization time (6.0 d vs. 9.0 d, P<0.001),the median total length of stay(13.0 d vs. 15.0 d, P<0.001) were shorter,the median hospitalization cost (37,000 yuan vs.43,000 yuan P<0.001) was lower, and stress indexes recovered quickly. The incidence of postoperative hospital stay and readimission rate within 30 d were not statistically different between the two groups. Conclusion:It is safe and feasible to implement ERAS for children with CCC in the perioperative period, which can reduce stress response, speed up recovery,and save medical costs.

Biliary atresia ( BA) is a pediatric liver disease characterized by progressive inflammation and fi-brosis of both the extrahepatic and intrahepatic bile ducts. Even though Kasai portoenterostomy increases the survival of children with BA, 80% of patients with BA will eventually require liver transplantation, making this condition the leading indication for pediatric liver transplantation worldwide. Currently,the causes of this disease are largely unde-fined and theories of pathogenesis include viral infection,autoimmune-mediated bile duct destruction,and abnormalities in bile duct development.

Biliary atresia is an infant disease characterized by obstruction of the internal and ex-ternal bile duct and progressive fibrosing process of the liver. Its etiology and pathogenesis remains un-clear and most infants would die in one year if thereˊs no medical intervention. In this article,we reviewed recent research progress of liver fibrosis in biliary atresia in infants and animal models,which proposed some unsettled problems and research direction in the future.

Objective To investigate the correlation between the remnants of extrahepatic bilie duct with the prognosis of biliary atresia after Kasai operation.Methods From Sep.2005 to Sep.2011,93 cases of biliary atresia [51 boys and 42 girls,ages at operation:61 days(40-121 days)] in Tongji Hospital Affiliated of Tongji Medical College of Huazhong University of Science and Technology were operated with Kasai procedure,and the diagnosis of type Ⅲ biliary atresia was based on the cholangiography and the intraoperative findings.According to whether there was fibrotic remnant plates of porta hepatis or not,type Ⅲ biliary atresia cases were grouped into 2 subtypes:subtype A,in which there was fibrotic remnant plates of porta hepatis (n =80) ; subtype B,in which there was no fibrotic remnant plates,but solid cords structure like hepatic duct was observed(n =13).Specimens of the remnants were colleted in operation and pathologic section detected the degree of bile ductular proliferation in the remnants.Postoperatively,all of the patients were followed up.The contents included the cholangitis occurrence rate,the jaundice disappearance rate and survival rate during postoperative 2 years.Results The patients had more bile ductular proliferation in subtype A (x2 =18.49,P ＜0.05).There was no significant difference in incidence of cholangitis in postoperative 1 month between the 2 groups (x2 =0.01,P ＞ 0.05).The jaundice disappearance rate in postoperative 6 months of subtype A was higher than the rate of subtype B(x2 =9.19,P ＜ 0.05).The survival rate during postoperative 2 years of subtype A was higher than the rate of subtype B(x2 =4.49,P ＜ 0.05).Conclusions There are higher jaundice disappearance rate and 2 years survival rate in type Ⅲ biliary atresia with the fibrotic remnant plates of porta hepatis,which suggest that there is more bile ductular proliferation in the extrahepatic remnants which is good for biliary drainage in subtype A after Kasai operation,and it can slow down hepatic injury and have a long-term good life quality.

The morbidity of Hirschsprung's disease (HD) is 1/5 000,and HD is the second common congenital digestive tract malformation.The main treatment of HD is surgery.In 1948,Swenson designed a pull-through colorectal resection procedure,which was followed by Duhamel,Soave and Rehbein procedures.All of these procedures are all trans-abdominal approach,severe complications are common.With the development of laparoscopy,it is gradually applied to HD surgery.In 1998,Torre proposed the one-stage endorectal pull-through procedure,which further reduced the operation wound.Subsequently,natural orifice transluminal endoscopic surgery(NOTES) and transumbilical laparoscopy emerged.All the developments are for the purpose of minimal invasion,with the advantages of less pain,shorter hospital stay,a better cosmetic result and at the same time,surgical effect guaranteed.

Intestinal obstruction in children is a common acute abdomen in pediatric surgery.The causes of obstruction are complex.Intestinal obstruction not only impacts on the intestine both in anatomy and function but also causes systemic physiological disorder.So accurate and timely diagnosis of intestinal obstruction is obviously favorable for the treatment.And it is also critical in rescuing serious state and avoiding death.

Objective: To evaluate the omrphological and histochemical properties of cartilage matrix of children with pectus excavatum(PE). Methods: The collagen of cartialge matrix of 19 PE and of 14 age-matched children was examined by transmission electron microscopy(TEM) and immunohistochemical staining examination. Meanwhile, the proteoglycan was studied by PAS and Safranin-O staining examination. All of the results of stanining were also analysed by the GT-2 model of image analysis software. Results: Compared with control group, although the intensity of type II collagen was intact, unequally distribution of collagen had been found by the TEM and immunohistochemical staining examination. The intensity and distribution of proteoglycan of PE group remained normal found by the PAS and Safranin-O staining examination. Conclusion: Although the intensity and distribution of proteoglycan was intact, as well as intensity of collagen, the distribution of collagen in PE was abnormal, and this change may possibly be related to the defectiveness of costal cartialge of PE.

50 mm/h were detected in all the patients. Decreasing C 3 in 6 patients and C 4 in 5 were found. Conclusions SLE is one of the general conditions causing retinal vein occlusion. Visual acuity and barrier of retinal vein and capillary are damaged seriously in patients with retinal vein occlusion caused by SLE, which may be accompanied with other ocular or general lesions. It is suggested that retinal vein occlusion is relative with SLE activity.

Objective To investigate the clinical characteristic of ocular fundus complications in systemic lupus erythematosus (SLE). Methods In 25 cases of SLE with the ocular fundus complications, the ocular fundus, the other ocular tissues, general lesions,and antinuclear antibody (ANA), anti-double-stranded DNA(anti-dsDNA), complement 3 (C 3), complement 4 (C 4)and erythrocyte sedimentation rate(ESR) were analyzed retrospectively. Results In the 25 cases, "classic" SLE retinopathy in 15 (25 eyes), retinal vein occlusion (RVO) in 9 (12 eyes), RVO combined with retinal artery occlusion in 1 (2 eyes), exudative retinal detachment in 1 (2 eyes), vitreous hemorrhage combined with neovascular glaucoma in 1 (1 eye), and optic disc edema except RVO in 3 (6 eyes) were found. Nine cases accompanied with other ocular signs and 21 with general lesions. Positive ANA and anti-dsDNA and elevated ESR in all of the patients, decreasing C 3 in 19, and C 4 in 17 were found. Conclusions SLE can cause serious ocular fundus complications accompanied with other ocular signs. Regular ophthalmic examination should be performed on the patients with SLE to detect and treat the ocular complications promptly.

Objective To evaluate the diagnosis and treatment of intestinal neuronal dysplasia type B (IND) in childhood. MethodsForty-five patients underwent preoperative barium enema examination, 23 patients underwent electromanometry, and mucosal biopsy and immunohistochemical staining for S100 protein were performed in 17 cases. All 45 patients underwent resection of the invalid segment with coloproctostomy. Whole layer was sampled on several sites of the resected segment and examined by two independent pathologists. All patients were followed up from 3 months to 9 years (mean 4.6 years).ResultsTwenty eight patients were complicated with Hirschsprung′s disease, one patient with hypogangliosis, and isolated IND was diagnosed in the other 16 children. The narrowed distal segment with proximal dilatation was merely noted in 4 children with isolated IND. Internal sphincter relaxations were missing in 6 children with isolated IND. The indicative diagnosis might be merely gained in 7 patients by the mucosa biopsy. The correct diagnosis can be established by whole layer biopsy of the resected segment. Three children with enterocolitis after operation were cured by conservative treatment. One patient suffering from postoperative sluice syndrome underwent second resection. Postoperative continence was achived in all patients. ConclusionThe correct diagnosis of IND can be obtained by biopsy of whole layer, and resection of invalid bowel segment with coloproctostomy is the choice of therapy.