新生儿肝外胆道囊性病变的诊断与治疗

目的了解新生儿肝外胆道囊性病变的不同类型及其临床特点,掌握其鉴别诊断的要点。方法分析9例新生儿肝外胆道囊性病变患儿的临床表现、肝功能、B超、CT、光镜、术中胆道造影的资料。结果本组胆道闭锁和新生儿胆总管囊肿女性多见,以梗阻性黄疸为主要表现,肝功能检查结果提示梗阻性黄疸。二者肝脏的组织学改变相似。但B超和CT检查发现,胆道闭锁组囊肿与胆囊大小均小于胆总管囊肿组。术中胆道造影显示,Ⅰ型胆道闭锁组肝内胆管变形、连续性中断;Ⅲ型胆道闭锁肝内胆管无法显示;胆总管囊肿组肝内胆管形态正常或略有被动扩张。结论多种胆道畸形都可以表现为新生儿肝外胆道囊肿,B超和术中胆道造影可以帮助鉴别这类疾病并且为治疗提供帮助。     

新生儿肝外胆道囊性病变的诊断与治疗

目的了解新生儿肝外胆道囊性病变的不同类型及其临床特点，掌握其鉴别诊断的要点。方法分析9例新生儿肝外胆道囊性病变患儿的临床表现、肝功能、B超、CT、光镜、术中胆道造影的资料。结果本组胆道闭锁和新生儿胆总管囊肿女性多见，以梗阻性黄疸为主要表现，肝功能检查结果提示梗阻性黄疸。二者肝脏的组织学改变相似。但B超和CT检查发现，胆道闭锁组囊肿与胆囊大小均小于胆总管囊肿组。术中胆道造影显示，Ⅰ型胆道闭锁组肝内胆管变形、连续性中断；Ⅲ型胆道闭锁肝内胆管无法显示；胆总管囊肿组肝内胆管形态正常或略有被动扩张。结论多种胆道畸形都可以表现为新生儿肝外胆道囊肿，B超和术中胆道造影可以帮助鉴别这类疾病并且为治疗提供帮助。     

新生儿腹腔囊性肿块108例病理与临床结果

目的分析新生儿期腹腔囊性肿块患儿的病因、病理、预后及转归。方法收集2008年8月至2013年7月我院新生儿外科术前诊断腹腔囊性肿块的病例,分析其性别、入院日龄、病因、治疗效果、病理及随访,总结预后及转归。结果 108例患儿中男女比例为40∶68;入院日龄3 h至28天,平均12天;住院日8～23天,平均11.2天;105例为足月儿,3例为早产儿。产前检查发现腹腔肿块42例（36例择期剖宫产）,新生儿期发现66例（27例剖宫产分娩）。91例患儿在新生儿期完成手术,15例患儿在生后1～3个月实施手术,均痊愈出院,另2例未行手术治疗。术后病理证实：胆总管囊肿51例,卵巢囊肿25例,肠源性囊肿16例,肠系膜淋巴管瘤6例,其他8例。2例开腹胆总管囊肿根治术患儿术后因粘连性肠梗阻再次行肠粘连松解术,恢复良好,其余手术患儿均健康成长。结论新生儿腹腔囊性肿块以女性稍多见,胆总管囊肿和卵巢囊肿占大部分,肠源性囊肿和肠系膜淋巴管瘤也是常见原因。新生儿腹腔囊性肿块患儿绝大多数预后良好,转归满意。     

Kasai术后肝内胆管囊性扩张的诊治与预后

目的 总结胆管闭锁术后肝内胆管囊性扩张的诊治经验,分析其对患儿预后的影响.方法 1998年6月至2008年3月,对胆管闭锁行葛西手术的患儿进行追踪随访.通过超声检查,发现8例患儿存在肝内胆管扩张.其中3例再行MRI检查,6例再行CT平扫加增强检杳以明确诊断.此8例患儿术后均有不同程度胆管炎症状.5例行PTCD显示肝内胆管囊性扩张.3例患儿行囊肿与空肠胆支再吻合术,2例患儿仪行PTCD置管引流,2例患儿暂未予任何处理.结果 3例经手术治疗的患儿,术后黄疸消退或减轻.仅行PTCD的患儿肝内胆管扩张长期存在.结论 对反复发作的胆管炎应定期行超声检查,尽早发现肝内囊肿;对肝内胆管囊性扩张,无论其影像学分型如何,应根据其具体临床表现进行相应的积极治疗;PTCD无法长期放置,仅是暂时性的治疗,但对手术时寻找囊肿有指示作用,使手术时囊肿的定位相对容易;囊肿的大小、位置与门静脉的关系,压迫的时间、治疗是否及时有效都可影响患儿的预后.     

孕期肝门区囊肿：囊肿型胆道闭锁与胆总管囊肿的鉴别

胆道闭锁(biliary atresia, BA)是新生儿期常见梗阻性黄疸的病因。按梗阻的部位及程度分为3型, 其中Ⅰ型囊肿型胆道闭锁(cystic biliary atresia, CBA)是少见但又特殊的一种亚型, 其在临床表现、影像学检查等方面都与先天性胆总管囊肿(choledochal cyst, CC)有着相似的表现, 两者也是产前诊断最常发现的肝门区囊性病变, 但因两者的治疗方案及预后有着显著的差异, 因此, 迄今为止两者的鉴别仍然是临床医生需要破解的难题, 本文将对目前已有的相关研究成果进行综述。     

Notch配体、受体在小儿胆道畸形肝组织中的表达及意义

目的 探讨Notch信号通路在小儿胆道畸形发病机制中的作用.方法 收集23例胆道畸形患儿临床资料(胆道闭锁12例,胆总管扩张11例),术中取肝脏组织样本;9例正常肝脏组织样本作为正常对照.免疫组化方法和实时荧光定量PCR方法检测肝脏组织中的Notch配体、受体的表达分布和相对表达量.结果 Jag1在胆道闭锁增生胆管表达明显增强,Jag2在各组门管区表达为阴性;荧光定量PCR显示:胆道闭锁组及胆总管扩张组Jag1 mRNA的表达明显高于对照组(P＜0.01);Jag2 mRNA的表达在三组问差异无统计学意义(P＞0.05).Notch1、Notch 2在对照组及胆总管扩张组主要表达于肝细胞和成熟胆管细胞,胆道闭锁组胆管细胞无阳性表达.Notch 3在胆道闭锁汇管区新生血管、基质中有较为明显的表达.荧光定量PCR显示:Notch1、Notch 2mRNA的表达在三组间差异无统计学意义(P＞0.05),胆道闭锁组Notch 3 mRNA的表达高于对照组(0.013±0.003比0.009±0.003,P＜0.01).Notch 4表达为阴性.结论 胆道闭锁肝脏组织Notch配体、受体表达异常,增生胆管细胞Jag1的过表达及Notch受体表达缺陷可能参与了胆道闭锁的病理过程.     

临床病理讨论--新生儿黄疸、肝脾肿大、腹水

病史摘要 患儿,男,20天。因皮肤黄染18天入院。患儿生后第3天出现皮肤黄染,呈持续性,无明显加深,同时伴皮肤苍白,无发热、咳嗽、呕吐、腹泻,神态及吃奶均无异常,无腹胀,大便呈黄色,尿色深黄。G_3P_1。孕38周剖宫产(头盆不称),出生体重3.25kg,无窒息抢救史。生后母乳喂养,未添加辅食及鱼肝油、钙粉。未接种任何疫苗。父26岁,体健。母25岁,无疾病史,第1胎孕3月时人工流产,第2胎孕5月时发现死胎。父     

Notch配体、受体在小儿胆道畸形肝组织中的表达及意义

Objective To investigate the expressions of Notch ligands and its receptors in the liver tissues of the pediatric patients with bile duct malformations. Methods Twenty three patients including 12 patients with biliary atresia and 11 with choledochal cyst were enrolled in this study. The patients' liver specimens were harvested during surgery. Immunohistochemistry and real-time fluorescent quantitative RT-PCR were performed to examine the expression of Notch ligands and its receptors in the liver tissues. Nine health liver tissues served as controls. The clinical data of these patients were also collected and analyzed. Results The expression of Jag1 significantly increased in the proliferating ductules of the patients with biliary atresia. Jag2 was negative in the portal area of all patients. The mRNA of Jag1 of the patients with bile duct malformations was higher than that of controls (P＜0. 01). No difference of the mRNA of Jag2 was found between the bile duct malformation patients and the controls (P＞0. 05). The expression of Notch 1 and Notch 2 was mostly found in hepatocytes and bile ductules of the controls' and the choledochal cyst patients' liver tissues. No Notch 1 and Notch 2 expression was found in proliferating ductules of the biliary atresia patients. Notch 3 was expressed in the neovascularization and mesenchyme of the biliary atresia patients. There was no significant difference of Notch1 and Notch 2 mRNA between the biliary atresia patients, choledochal cyst patients and controls (P＞0. 05). Notch 3 mRNA of biliary atresia patients was higher than that of the controls (P＜0. 01). Notch 4 expression was undetectable in all patients. Conclusions The expression patterns of Notch ligands and its receptors are changed in the patients with biliary atresia, which may contribute to the pathogenesis of biliary atresia.     

胆道闭锁Kasai术后肝内胆管囊性扩张的形成特点与病理研究

目的分析胆道闭锁(biliary atresia,BA)患儿Kasai术后肝内胆管囊性扩张(intrahepatic biliary cysts,IBC)的形成特点及病理学表现,探索其与预后之间的关系。方法选取2017年1月至2018年12月天津市儿童医院Kasai术后行肝移植手术的BA患儿179例(男75例,女104例),收集患儿的临床资料和影像学资料,用以判断是否存在IBC,其中36例CT检查结果提示存在肝内胆管扩张,作为IBC(+)组,143例未检测到肝内胆管扩张,作为IBC(-)组,比较两组的自体肝生存状况及肝功能指标。同时收集到上述患儿中50例患儿的肝移植时的病肝组织,其中IBC(+)组18例,IBC(-)组32例,进行HE染色,观察肝脏纤维化程度、胆管增生程度、淤胆以及胆管板畸形的发生情况。结果①IBC(+)组女患儿有27例,占75.0%(27/36),IBC(-)组女患儿有77例,占53.8%(77/143),两组中女患儿占比的差异具有统计学意义(P=0.021),两组患儿在行Kasai术时的日龄及术后胆管炎的发生率的比较中,差异无统计学意义(P>0.05);②IBC(+)组患儿的中位生存期为11.5个月,明显高于IBC(-)组患儿的9.0个月。Log-Rank检测发现,IBC(+)组术后自体肝生存状况优于IBC(-)组患儿,且差异具有统计学意义(P=0.038);③肝功能的比较结果发现IBC(+)组除胆汁酸(total bile acids,TBA)外,均低于IBC(-)组患儿,其中总胆红素(total bilirubin,TBI)、结合胆红素(direct bilirubin,DBI)水平在两组的差异具有统计学意义(P<0.05);④IBC(+)组患儿的肝纤维化程度、胆管增生程度及胆管板畸形发生率均较IBC(-)组略低,差异无统计学意义;但IBC(+)组淤胆程度明显低于IBC(-)组,差异具有统计学意义(P=0.013)。结论BA患儿Kasai术后发生IBC可能是肝脏代偿的积极表现,短期内不会导致胆汁淤积的加重,相反扩张的胆管可以减轻胆汁淤积的程度,改善肝功能及病理分级,从而延长自体肝生存时间。     

新生儿腹腔镜下胆总管囊肿根治术体会

胆总管囊肿切除、肝管空肠Roux—Y吻合术是治疗先天性胆总管囊肿的标准手术方式。1995年Farello等首先将腹腔镜应用于先天性胆总管囊肿的治疗,随后国内也出现腹腔镜手术治疗先天性胆总管囊肿的报道。     

Clinical and pathological characteristics of cystic lesions of extrahepatic bile duct in neonates

Aim: To investigate the differences in clinical and pathological manifestations between biliary atresia with extrahepatic biliary cyst and choledochal cyst in neonates. Methods: Symptoms and clinical signs in 5 neonates with biliary atresia with extrahepatic biliary cyst (4 of type I and 1 of type III) and 17 neonates with choledochal cyst were recorded. The levels of serum alkaline phosphatase, bilirubin, direct bilirubin, transaminase, gamma-glutamyl transpeptidase were analysed. Width and length of gallbladder and choledochal cyst were measured on B-mode ultrasound before surgery. Intrahepatic or extrahepatic biliary ducts were visualized with intraoperative cholangiography. The pathologic features in specimens of the liver were studied with light- and electron transmission microscopy. Results: All malformations occurred more commonly in girls, and obstructive jaundice was the main manifestation in both groups. Laboratory tests showed similar results for all patients in this study. With regard to pathological features, no significant difference was seen in either light microscopy or transmission electron microscopy, but it was shown with ultrasound that the length and width of the cysts and the gallbladder in neonates with biliary atresia were all shorter than the measurements in patients with choledochal cyst. The intrahepatic bile ducts could not be visualized on intraoperative cholangiography in type III biliary atresia. Deformation of the biliary ducts within the liver and stricture of the portal bile duct were the predominant features in type I biliary atresia, while the bile duct within the liver was normal or dilated in neonates with choledochal cyst.

Conclusions: Cystic lesions of the extrahepatic bile duct might be a common manifestation of biliary atresia and choledochal cyst. Intraoperative cholangiography is a precise and effective technique in the differential diagnosis of those lesions and helps decide on the most rational method of treatment.     

Biliary tract malformations of infancy

Biliary malformations in children are rare, but important to recognize. Diagnostic delay compromises outcomes. Some may be diagnosed prenatally, others in the newborn period, usually with clinical presentation of jaundice, with or without abdominal pain and fever. A strong level of suspicion is needed with any patient. Abdominal ultrasound, which is widely available, is an excellent initial imaging tool. Further work up may include magnetic resonance cholangiopancreatography (MRCP) to completely define the biliary tree. Surgical management is the primary treatment for many biliary disorders.     

Does ”Cystic” Biliary Atresia Represent a Distinct Clinical and Etiological Subgroup? A Series of Three Cases

We describe a unique series of 3 cases of biliary atresia (BA) associated with a choledochal cyst. All 3 children presented with jaundice at birth and had no other abnormalities. Although these children had a fetal form of BA, their presentation and outcome differed from those of biliary atresia splenic malformation syndrome (BASM), the well-described form of early onset BA. Unlike those with BASM, these children had no other associated malformations, had a normal birth weight, and did not yet require a liver transplant. We believe that the present series of patients and their associated pathology may represent a distinct phenotype with a common, prenatally acquired etiology that is different from other fetal forms of BA, such as BASM, and from patients who present with the classic perinatal form of BA.     

A case of biliary atresia with cystic dilatation of the extrahepatic bile duct and polysplenia syndrome

A 79-day-old girl presented with jaundice and acholia. Laboratory findings disclosed elevated levels of bilirubin, transaminases, and gamma-glutamyl transferase. A chest X-ray film showed dextrocardia. A computed tomographic scan revealed a cystic mass at the porta hepatis, multiple spleens in the right side of the abdomen, and absence of the inferior vena cava. Under the diagnosis of biliary atresia (BA) (I-cyst) associated with polysplenia syndrome, a hepatic portojejunostomy was performed at 80 days of age. The postoperative course was uneventful, and the jaundice cleared. Although the occurrence of associated anomalies in BA is rare, polysplenia is most commonly seen, and its incidence is reported to be 2% to 10%. Patients with BA and polysplenia usually have a poor prognosis, however, this patient may have a good prognosis due to uneventful recovery from the jaundice. Accepted: 30 August 1999     

Evaluation of prognostic criteria in extrahepatic bile duct atresia

Extrahepatic bile duct atresias must be classified into four histopathological groups according to the characteristic numerical, metrical, and morphological alterations of the interlobular bile ducts. A prospective study based on an observation period of 5 and more years included 43 patients with extrahepatic biliary atresia. It showed that prognosis is generally dependent on three factors: (1) the duration of cholestasis; (2) the structure of the bile ducts in the praehilaeren Versclussplatte; and (3) the histopathologically defined features of the interlobular bile ducts. The total diameter of all bile duct structures in the praehilaeren Verschlussplatte is unmistakably the most significant finding. A total diameter of more than 400 m indicates a favorable prognostic subtype, while a total diameter of less than 400 m is unfavorable. Even if the prognostically favorable type of finding is present, the ultimate prognosis is determined by the histopathological features of the interlobular bile ducts. Therefore, four characteristic groups must be taken into consideration when evaluating prognosis.     

Porcelain gallbladder with extrahepatic bile duct obstruction in a child

An extrahepatic bile duct obstruction was diagnosed in a 13-year-old boy presenting with pruritus, abdominal pain and jaundice. Several weeks after sphincterotomy and biliary stenting via endoscopic retrograde cholangiopancreaticography which relieved the obstruction, the patient was operated on. Severe fibrosis encased the extrahepatic biliary tract, so only cholecystectomy was performed because planned hepaticojejunoanastomosis could jeopardize the vascular supply to the liver. Histopathology showed calcification of the gallbladder wall and chronic fibroproliferative changes in the surrounding tissue. The stricture of extrahepatic biliary duct resolved after 3 years of repeated replacement of stents. The stenting was thereafter terminated. In the following 3 years no dilation of intrahepatic bile ducts and no laboratory signs of cholestasis recurred and the now 19-year-old boy is doing well. Neither a case of porcelain gallbladder with extrahepatic bile duct obstruction in a child nor a successful treatment of the obstruction by long-term stenting has been described in the literature yet.     

Clinical and pathological characteristics of Alagille syndrome in Chinese children

Background  Alagille syndrome (AS) is regarded as the most common cause of chronic cholestasis in childhood associated with specific phenotypic features in western countries. This study was undertaken to investigate the significance of AS in Chinese children with chronic cholestasis and to describe its clinical and histological features. Methods  From October 2004 to January 2007, 157 children who presented with conjugated jaundice from less than 3 months of age were admitted to a tertiary hospital in Shanghai. Investigations of the heart, spine, eyes and kidneys were conducted in 13 children who experienced prolonged cholestasis beyond 1 year of age after exclusion of biliary atresia and familial progressive intrahepatic cholestasis type 1 or 2. In patients with interlobular bile duct paucity, AS was diagnosed if 3 or more of the following 5 major features were present: cardiac murmur, posterior embryotoxon, butterfly-like vertebrae, renal abnormalities and characteristic faces. In patients without interlobular bile duct paucity or who did not receive liver biopsy, 4 or more features were required for the diagnosis. Results  Of the 13 children, 6 were diagnosed with AS at ages ranging from 1 year and 7 months to 3 years and 11 months. Jaundice was noticed in early infancy and then pruritus developed in all the 6 patients, of whom 5 presented with acholic stool and 4 had been misdiagnosed as having presumed biliary atresia by hepatobiliary scintigraphy or laparoscopic cholangiography. Biochemical examinations demonstrated increased concentration of total bile acid and hyperlipidemia. Interlobular bile duct paucity was demonstrated histologically in 5 patients who received liver biopsy. Vertebral abnormalities, heart murmur, characteristic faces and failure to thrive were found in all the 6 patients. Two patients had evidence of renal involvement. Micropenis, empty scrotum, and gall stone were seen in 1 patient. Conclusion  AS is also an important cause of prolonged cholestasis in Chinese children. It is difficult to differentiate AS from biliary atresia. Liver biopsy and spine X-ray may be helpful in the early detection of AS.     

Treatment of extrahepatic bile duct atresia: results and long-term prognosis after hepatic portoenterostomy

Since 1972, 64 infants suffering from extrahepatic bile duct atresia have been examined in our Department. Between 1975 and 1984 conventional or extended hepatic-portoenterostomy was performed on 41 patients. These infants were classified into two groups on the basis of age at the time of surgery in relation to the duration of cholestasis. Of 12 patients operated upon 5 or more years ago, 8 (66%) are still alive with favorable bile flow and 4 (34%) have died from hepatic failure caused by biliary cirrhosis. Of 22 infants above 8 weeks of age who have been treated so far, 7 (31%) are alive with good bile flow and 15 (69%) have died within the past 3 years. In contrast, 12 (63%) of 19 infants operated upon before 8 weeks of age are healthy with good bile flow and only 7 (37%) have died from cirrhosis. The course of the disease was analyzed with regard to the following findings: (1) age at surgery with respect to the duration of cholestasis; (2) extent of liver fibrosis at surgery; (3) cross-section of ductular structures in the porta hepatis; (4) numerical and morphological changes in the interlobular bile ducts; (5) extent of dissection of the porta hepatis; (6) postoperative hepatoportitis. Principles determining the long-term follow-up of this dynamic obliterative process can be deduced from these investigations. Accordingly, a favorable prognosis can be expected if the following conditions are fulfilled: no cholestasis for more than 6 weeks; low-grade fibrosis at the time of surgery, with a mean diameter of the ductular structures in the porta hepatis of more than 450 m; no numerical or morphological alterations in the interlobular bile ducts; no postoperative hepatoportitis. Offprint requests to: P. Schweizer at the above address in Hannover