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Objective@#To investigate the clinical characteristics and risk factors of congenital choledochal cysts (CCC).@*Methods@#This retrospective study recruited 52 cases who were antenatally diagnosed with CCC and underwent surgical treatment after birth in Guangdong Women and Children Hospital from January 2013 to August 2018, with complete clinical data. According to the enlargement of cysts during pregnancy, they were divided into two groups: progressive group (≥15 mm, 22) and stable group (<15 mm, 30). Antenatal and postpartum ultrasound and MRI features of the two groups were analyzed. Clinical manifestations and biochemical examination results before and after operation were compared between the two groups. Other data, including amylase level in cyst fluid during operation, cholangiography findings, liver biopsy results, and post-operation follow-up, were also analyzed. Chi-square test, t (t') test, and Pearson correlations tests were performed for data analysis.@*Results@#(1) The average age of the 52 patients at operation was 46(7-822) d. The cysts of all cases were first detected during 19-21 weeks of gestation. The maximum diameter of the cyst in the progressive group was larger than that in the stable group after 34 weeks of pregnancy [31-34 weeks: (31.1±8.4) vs (23.1±6.6) mm, t=3.911; >34 weeks: (36.1±6.8) vs (27.1±7.3) mm, t=4.557; pre-operation: (51.8±18.0) vs (34.0±15.6) mm, t=3.809; all P<0.01]. (2) In the progressive group, the cysts were irregular in shape and enlarged after birth. The common hepatic duct and intrahepatic bile duct were dilated and gradually distended after birth, while the distal end of the common bile duct was narrowed, thus to form a cone-like duct. Deposits could be seen inside the cysts after delivery. Irregular cysts were also presented in the stable group, and five of them had dilatation of common hepatic duct and intrahepatic bile duct after birth. However, no cone-like formation was seen, the distal end of the common bile duct was visible, and deposits in cysts were occasionally found. (3) Twenty-five patients underwent laparotomy, and seven of them showed increased amylase level in cyst fluid including four with 2-5 times above the upper limit of normal value (one in the progressive group and three in the stable group). The other three cases were all in the stable group and their amylase levels in cyst fluid were more than ten times of the upper limit. The level of direct bilirubin in the progressive group was higher than that in the stable group before the operation [18.40(2.50-113.30) vs 8.70(0.00-16.80) μmol/L, u=2.400, P<0.05]. (4) Among the 52 cases, patients with type Ⅰ, Ⅳ and Ⅴ cyst accounted for 71.1% (37/52), 26.9% (14/52) and 2.0% (1/52), respectively. All cases were followed up regularly six months to one year after the operation. Liver function and bilirubin became normal and the growth and development of the babies were similar to those of the same age. (5) Different degrees of liver fibrosis and inflammation were shown in 46(88.5%) cases and more severe in older babies among those who underwent surgery in the progressive group. The time at operation was not associated with the severity of liver fibrosis and inflammation in the stable group. Hepatic fibrosis and inflammation were more serious in the progressive group than in the stable group (fibrosis grading: χ2=14.260, P=0.006; inflammatory activity grading: χ2=9.904, P=0.019).@*Conclusions@#Larger diameter (≥30 mm) in the initial prenatal examination or a significant increase in cystic diameter (≥15 mm) during pregnancy are risk factors for early stenosis or occlusion in the distal end of common bile duct requiring close follow-up after birth. When jaundice or abnormal liver function occur and stool color becomes light, early surgical treatment (one to two months after birth, generally within three months) for CCC is recommended to rule out the anomalous union of the pancreaticobiliary duct and hepatic disorders, as well as the cystic biliary atresia.
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Objective To investigate the clinical characteristics and risk factors of congenital choledochal cysts (CCC).Methods This retrospective study recruited 52 cases who were antenatally diagnosed with CCC and underwent surgical treatment after birth in Guangdong Women and Children Hospital from January 2013 to August 2018,with complete clinical data.According to the enlargement of cysts during pregnancy,they were divided into two groups:progressive group (≥ 15 mm,22) and stable group (＜15 mm,30).Antenatal and postpartum ultrasound and MRI features of the two groups were analyzed.Clinical manifestations and biochemical examination results before and after operation were compared between the two groups.Other data,including amylase level in cyst fluid during operation,cholangiography findings,liver biopsy results,and post-operation follow-up,were also analyzed.Chi-square test,t (t1) test,and Pearson correlations tests were performed for data analysis.Results (1) The average age of the 52 patients at operation was 46(7-822) d.The cysts of all cases were first detected during 19-21 weeks of gestation.The maximum diameter of the cyst in the progressive group was larger than that in the stable group after 34 weeks of pregnancy [31-34 weeks:(31.1 ±8.4) vs (23.1 ± 6.6) mm,t=3.911;＞34 weeks:(36.1 ± 6.8) vs (27.1 ± 7.3) mm,t=4.557;pre-operation:(51.8± 18.0) vs (34.0± 15.6) mm,t=3.809;all P＜0.01].(2) In the progressive group,the cysts were irregular in shape and enlarged after birth.The common hepatic duct and intrahepatic bile duct were dilated and gradually distended after birth,while the distal end of the common bile duct was narrowed,thus to form a cone-like duct.Deposits could be seen inside the cysts after delivery.Irregular cysts were also presented in the stable group,and five of them had dilatation of common hepatic duct and intrahepatic bile duct after birth.However,no cone-like formation was seen,the distal end of the common bile duct was visible,and deposits in cysts were occasionally found.(3) Twenty-five patients underwent laparotomy,and seven of them showed increased amylase level in cyst fluid including four with 2-5 times above the upper limit of normal value (one in the progressive group and three in the stable group).The other three cases were all in the stable group and their amylase levels in cyst fluid were more than ten times of the upper limit.The level of direct bilirubin in the progressive group was higher than that in the stable group before the operation [18.40(2.50-113.30) vs 8.70(0.00-16.80) μmol/L,u=2.400,P＜0.05].(4) Among the 52 cases,patients with type Ⅰ,Ⅳ and Ⅴ cyst accounted for 71.1％ (37/52),26.9％ (14/52) and 2.0％ (1/52),respectively.All cases were followed up regularly six months to one year after the operation.Liver function and bilirubin became normal and the growth and development of the babies were similar to those of the same age.(5) Different degrees of liver fibrosis and inflammation were shown in 46(88.5％) cases and more severe in older babies among those who underwent surgery in the progressive group.The time at operation was not associated with the severity of liver fibrosis and inflammation in the stable group.Hepatic fibrosis and inflammation were more serious in the progressive group than in the stable group (fibrosis grading:x2=14.260,P=0.006;inflammatory activity grading:x2=9.904,P=0.019).Conclusions Larger diameter (≥ 30 mm) in the initial prenatal examination or a significant increase in cystic diameter (≥ 15 mm) during pregnancy are risk factors for early stenosis or occlusion in the distal end of common bile duct requiring close follow-up after birth.When jaundice or abnormal liver function occur and stool color becomes light,early surgical treatment (one to two months after birth,generally within three months) for CCC is recommended to rule out the anomalous union of the pancreaticobiliary duct and hepatic disorders,as well as the cystic biliary atresia.
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Objective To summarize the etiology and surgical treatment of obstructive infantile cholestasis.Methods Clinical data of 108 cases of obstructive infantile cholestasis was studied retrospectively from April 2009 to April 2014.Results Correct diagnosis was established in all 108 patients by laparoscopic biliary tract exploration and cholangiography.Among those,there were noncorrectable biliary atresia in 81 cases (75.0％),correctable biliary atresia in 5 cases (4.6％),inspissated bile syndrome in 8 cases (7.4％),infantile hepatitis syndrome in 6 cases (5.6％),choledochal cyst in 4 cases (3.7％),biliary hypoplasia in 2 cases (1.9％),1 case (0.9％) suffered from spontaneous bile duct perforation,1 case (0.9％) suffered from oppression of lymph nodes in hepatic portal.Patients of nocorrectable biliary atresia were treated with open Kasai portoenterostomy or laparoscopic Kasai portoenterostomy,correctable biliary atresia and choledochal cyst underwent laparoscopic cyst excision and Roux-Y hepaticojejunostomy,inspissated bile syndrome,infantile hepatitis syndrome and biliary hypoplasia were treated by laparoscopic cholecystostomy and biliary tract irrigation.The patient of spontaneous bile duct perforation was treated with laparoscopic common bile duct exploration and T-tube drainage,the lymph node was excised in patient with oppression of lymph nodes in hepatic portal.All infants were followed-up for 3 months to 48 months,the clearance of jaundice rate varied in patients with Kasai portoenterostomy,patients with non-Kasai portoenterostomy were all in good condition and there were no symptom recurrence.Conclusion Biliary atresia,inspissated bile syndrome,infantile hepatitis syndrome,choledochal cyst and biliary hypoplasia are the most common cause of surgery-related infantile cholestasis.Kasai portoenterostomy,hepaticojejunostomy and cholecystostomy and biliary tract irrigation are the main surgical method for surgery-related infantile cholestasis.
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Objective To investigate the clinical features of pedistric Littre hernia.Methods Clinical data of 11 cases of Littre hermia admitted from January 2002 to December 2010 was studied retrospectively.Results The diagnosis of Littre hernia was all established by laparotomy.All of the 11 cases were boys,the median age was 1.2 years (22 days to 3 years and 7 months).The main clinical features were:painful,irregular and nonresetable mass in the groin area (11/11),vomiting in 6 cases (6/11 ),fever (＞38.5 ℃ ) in 4 cases (4/11 );X-ray showed intestinal obstruction in 9 cases (9/11 ),Ultrasound found mixed mass in the groin area in 11 cases ( 11/11 ),pouch-like structure were found in 6 cases (6/11).Eight cases (8/11) presented with elevated WBC ( ＞ 10000).Palpable lobulated structure were fell in 5 cases (5/11).All cases of Littre hernia were successfully operated on,Meckel diverticulum perforation was found in 2 cases ( 2/11 ),Meckel diverticulum adhered to the hernia sac in 8 eases (8/11).All patients were cured by surgery,and postoperative follow-up (10ms-8y) found no recurrence.Conclusions Pediatric Littre hernia is rare,preoperative diagnosis is difficult.Avoiding preoperative violent reduction and intraoperative injuring Meekel's diverticulum or the small bowel helps improve the cure rate of Littre hernia in children.