Clinical characteristics and risk factors of congenital choledochal cysts

Q4 Medicine
Jingzi Xiao, Yang Yang, Yingcong Xiang, Peng Li, Chengchao Lyu, Baisha Huang, Long Cen, Penghui Han
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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. \n \n \nResults \n(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). \n \n \nConclusions \nLarger 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. 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引用次数: 0

Abstract

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. Key words: Choledochal cyst; Ultrasonography, prenatal; Risk factors
先天性胆总管囊肿的临床特点及危险因素分析
目的探讨先天性胆总管囊肿(CCC)的临床特点及危险因素。方法回顾性研究2013年1月至2018年8月广东省妇幼医院产前诊断为CCC并在出生后接受手术治疗的52例患者,临床资料完整。根据妊娠期囊肿肿大情况分为进展组(≥15 mm, 22例)和稳定组(34周:(36.1±6.8)vs(27.1±7.3)mm, t=4.557;术前:(51.8±18.0)vs(34.0±15.6)mm, t=3.809;所有P < 0.01)。(2)进展组囊肿形状不规则,出生后囊肿增大。出生后肝总管和肝内胆管扩张并逐渐扩张,而胆总管远端变窄,形成锥形胆管。分娩后,囊肿内可见沉积物。稳定组也出现不规则囊肿,其中5例出生后出现肝总管及肝内胆管扩张。但未见锥形形成,胆总管远端可见,偶见囊肿内沉积。(3) 25例患者剖腹手术,7例患者囊液淀粉酶水平升高,其中4例高于正常值上限2-5倍(进展组1例,稳定组3例)。其余3例均为稳定组,囊肿液淀粉酶水平均高于上限10倍以上。术前进展组直接胆红素水平高于稳定组[18.40(2.50 ~ 113.30)vs 8.70(0.00 ~ 16.80) μmol/L, u=2.400, P<0.05]。(4) 52例中,Ⅰ型、Ⅳ型和Ⅴ型囊肿患者分别占71.1%(37/52)、26.9%(14/52)和2.0%(1/52)。术后6个月至1年定期随访。肝功能和胆红素恢复正常,婴儿的生长发育与同龄儿童相似。(5)进展组46例(88.5%)患儿出现不同程度的肝纤维化和炎症,年龄较大的患儿更为严重。稳定组的手术时间与肝纤维化和炎症的严重程度无关。进展组肝纤维化及炎症较稳定组严重(肝纤维化分级:χ2=14.260, P=0.006;炎症活动度分级:χ2=9.904, P=0.019)。结论产前检查胆囊直径较大(≥30mm)或妊娠期胆囊直径明显增大(≥15mm)是出生后早期胆总管远端狭窄或闭塞的危险因素,需要密切随访。当出现黄疸或肝功能异常,大便颜色变浅时,建议早期手术治疗(出生后1 ~ 2个月,一般在3个月内),排除胰胆管异常连及肝脏疾病,以及胆囊胆道闭锁。关键词:胆总管囊肿;产前超声;风险因素
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来源期刊
中华围产医学杂志
中华围产医学杂志 Medicine-Obstetrics and Gynecology
CiteScore
0.70
自引率
0.00%
发文量
4446
期刊介绍: Chinese Journal of Perinatal Medicine was founded in May 1998. It is one of the journals of the Chinese Medical Association, which is supervised by the China Association for Science and Technology, sponsored by the Chinese Medical Association, and hosted by Peking University First Hospital. Perinatal medicine is a new discipline jointly studied by obstetrics and neonatology. The purpose of this journal is to "prenatal and postnatal care, improve the quality of the newborn population, and ensure the safety and health of mothers and infants". It reflects the new theories, new technologies, and new progress in perinatal medicine in related disciplines such as basic, clinical and preventive medicine, genetics, and sociology. It aims to provide a window and platform for academic exchanges, information transmission, and understanding of the development trends of domestic and foreign perinatal medicine for the majority of perinatal medicine workers in my country.
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