Diseases of bile duct in children

Several diseases originate from bile duct pathology.Despite studies on these diseases,certain etiologies of some of them still cannot be concluded.The most common disease of the bile duct in newborns is biliary atresia,whose prognosis varies according to the age of surgical correction.Other diseases such as Alagille syndrome,inspissated bile duct syndrome,and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction.The majority of these diseases present with cholestatic jaundice in the newborn or infant period,which is quite difficult to differentiate regarding clinical acumen and initial investigations.Intraoperative cholangiography is potentially necessary to make an accurate diagnosis,and further treatment will be performed synchronously or planned as findings suggest.This article provides a concise review of bile duct diseases,with interesting cases.

Bile duct cyst in adults:Interventional treatment,resection,or transplantation?

Cystic dilatations of the bile ducts may be found along the extrahepatic biliary tree,within the liver,or in both of these locations simultaneously.Presentation in adults is often associated with complications.The therapeutic possibilities have changed considerably over the last few decades.If possible,complete resection of the cyst(s)can cure the symptoms and avoid the risk of malignancy.According to the type of bile duct cyst,surgical procedures include the Roux-en-Y hepaticojejunostomy and variable types of hepatic resection.However,the diffuse forms of Todani type Ⅴ cysts(Caroli disease and Caroli syndrome)in particular remain a therapeutic problem,and liver transplantation has become an important option.The mainstay of interventional treatment for Todani typeⅢbile duct cysts is via endoscopic retrograde cholangiopancreatography.The diagnostic term"bile duct cyst"comprises quite different pathological and clinical entities.Interventional therapy,hepatic resection,and liver transplantation all have their place in the treatment of this heterogeneous disease group.They should not be seen as competitive treatment modalities,but as complementary options.Each patient should receive individualized treatment after all of the clinical findings have been considered by an interdisciplinary team.

A rare case of bile duct cyst

Choledochal cyst is an uncommon disease usually seen in young women and can be divided into five types. We report a 66-year-old woman who was diagnosed with types and bile duct cyst simultaneously after surgery, which is a rare type of bile duct cyst.

A case of peribiliary cysts accompanying bile duct carcinoma

A rare case of peribiliary cysts accompaying bile duct carcinoma is presented. A 54-year-old man was diagnosed as having lower bile duct carcinoma and peribiliary cysts by diagnostic imaging. He underwent pylorus preserving pancreatoduodenectomy. As for the peribiliary cysts, a course of observation was taken. Over surgery due to misdiagnosis of patients with biliary malignancy accompanied by peribiliary cysts should be avoided.

Familial occurrence of congenital bile duct dilatation

Congenital bile duct dilatation (CBD) that developed in a parent and son is presented. Familial occurrence of CBD is rare, with only a few male cases having been reported. Since the initial report of CBD occurring in siblings in 1981, a total of 20 cases (10 pairs) have been published as of 2007. Clinical and genetic features of CBD are discussed.

Impact of previous cyst-enterostomy on patients' outcome following resection of bile duct cysts

AIM:To analyze the impact of previous cyst-enterostomy of patients underwent congenital bile duct cysts(BDC)resection.METHODS:A multicenter European retrospective study between 1974 and 2011 were conducted by the French Surgical Association.Only Todani subtypes I and IVb were included.Diagnostic imaging studies and operative and pathology reports underwent central revision.Patients with and without a previous history of cystenterostomy(CE)were compared.RESULTS:Among 243 patients with Todani types I and IVb BDC,16 had undergone previous CE(6.5%).Patients with a prior history of CE experienced a greater incidence of preoperative cholangitis(75%vs 22.9%,P<0.0001),had more complicated presentations(75%vs 40.5%,P=0.007),and were more likely to have synchronous biliary cancer(31.3%vs 6.2%,P=0.004)than patients without a prior CE.Overall morbidity(75%vs 33.5%;P<0.0008),severe complications(43.8%vs 11.9%;P=0.0026)and reoperation rates(37.5%vs 8.8%;P=0.0032)were also significantly greater in patients with previous CE,and their Mayo Risk Score,during a median follow-up of 37.5 mo(range:4-372 mo)indicated significantly more patients with fair and poor results(46.1%vs 15.6%;P=0.0136).CONCLUSION:This is the large series to show that previous CE is associated with poorer short-and longterm results after Todani types I and IVb BDC resection.

Common Bile Duct Cysts Leading to Cholestasis and Jaundice: A Rare Case Report

Cystic dilatation of the common bile duct constitutes a rare congenital malformation prevalent in people of Asian origin with a female predominance. Its classification follows that of Todani. The commonest form, found in 80% of cases, is type I. Common bile duct cysts are often diagnosed in 2 out of 3 cases, during childhood with adult forms often rare. We hereby report the case of a cystic dilatation of the bile duct in a 75-year-old patient without significant history of disease.

Bile duct hamartomas-the von Meyenburg complex

: Hamartomas of the bile duct (von Meyenburg complex) are benign neoplasms of the liver, constituted histologically cystic dilatations of the bile duct, encompassed by fibrous stroma. We report a 42-year-old female patient with symptomatic cholecystitis, whose gross and ultrasonic appearance suggestive of multiple liver metastases. Magnetic resonance imaging and liver biopsy are the gold standards for diagnosis of this rare hepatobiliary condition.

Cholangiographic characteristics of common bile duct dilatation in children

AIM: To investigate whether children with congenital common bile duct dilatation(CBDD) differ from children with obstructive CBDD in cholangiographic characteristics.METHODS: In this retrospective cohort study, the baseline data and the results of imaging analyses were reviewed among children who had endoscopic retrograde cholangiopancreatography(ERCP) due to CBDD. ERCP was performed on all pediatric patients by experienced pediatric endoscopists. The maximal transverse diameter of the common bile duct(CBD) was measured on ERCP. To assess whether ageadjusted CBDD could be used for differential diagnosis, a CBDD severity index(SI) was calculated by dividing the measured CBD diameter by the age-corrected maximal diameter of a normal CBD. RESULTS: A retrospective medical chart review revealed that 85 consecutive children under 16 years of age with hepatobiliary disease and CBDD were referred to Seoul Asan Medical Center. Fiftyfive(64.7%) children had congenital CBDD and 30(35.3%) had obstructive CBDD. The two groups did not differ significantly in terms of clinical characteristics except for sex. The congenital and obstructive CBDD groups did not differ significantly in terms of mean CBD diameter(19.3 ± 9.6 mm vs 12.2 ± 4.1 mm, P > 0.05). However, congenital CBDD cases had a significantly higher mean SI than obstructive CBDD cases(3.62 ± 1.64 vs 1.98 ± 0.71, P = 0.01). In multivariate analysis, an SI value ≥ 2.32 and comorbidity with anomalous union of pancreaticobiliary duct(APBDU) in ERCP independently predicted congenital CBDD.CONCLUSION: Measuring the CBD may aid the differential diagnosis of both CBDD and APBDU in children.

直线切割吻合器在儿童腹腔镜胆总管囊肿根治术Roux-en-Y吻合中的应用

目的探讨直线切割吻合器应用于儿童腹腔镜胆总管囊肿根治术Roux-en-Y吻合中的有效性、安全性及可行性。方法本研究为前瞻性研究,选取2020年1月至2023年1月湖南省儿童医院接受腹腔镜胆总管囊肿根治术Roux-en-Y吻合的34例患儿作为研究对象,按照随机数字表法进行分组,采用直线切割吻合器实施Roux-en-Y吻合术的患儿纳入观察组(n=17),采用传统缝合法实施Roux-en-Y吻合术的患儿纳入对照组(n=17)。记录两组患儿手术时长、术中出血量、术后肠道功能恢复时间、首次进食流质时间、拔除引流管时间、术后住院时间、总住院费用和术后并发症发生率。结果34例均顺利完成手术,无一例中转开放手术。观察组与对照组手术时长[(130.43±31.32)min比(141.51±30.39)min]、术中出血量[(55.45±20.73)mL比(58.62±22.13)mL]差异均无统计学意义(P>0.05);观察组与对照组患儿术后肠道功能恢复时间[(4.03±0.42)min比(4.91±1.13)min]、首次进食流质时间[(3.95±0.61)d比(4.88±1.09)d]、拔除引流管时间[(5.95±0.68)d比(6.65±1.28)d]、术后住院时间[(8.29±2.17)d比(10.33±2.18)d]均短于对照组,差异均有统计学意义(P<0.05);观察组与对照组患儿总住院费用[(34948.17±1019.57)元比(35151.91±1151.15)元]、并发症发生率(1/17比2/17)差异无统计学意义(P>0.05)。结论直线切割吻合器在儿童腹腔镜胆总管囊肿根治术Roux-en-Y吻合中与传统缝合吻合技术的有效性和安全性无明显差异,可促进患儿术后恢复,值得临床推广应用。

内镜逆行胰胆管造影治疗先天性胆总管囊肿切除术后胆总管残端结石

目的 评估内镜逆行胰胆管造影术(ERCP)治疗胆总管残端结石的有效性及安全性。方法 收集2019年1月至2023年12月山东省立第三医院8例因腹痛入院诊断为先天性胆总管囊肿切除术后胆总管残端结石、再行内镜治疗的患者临床资料。分析ERCP操作成功率、结石取净率、并发症发生率、腹痛缓解率、结石复发率、癌变率。结果 8例患者结石直径(1.12±0.35)cm,结石数为(1.03±0.25)个,总操作成功率100%(8/8),一次结石取净率87.5%(7/8),总结石取净率100%(8/8)。术后1例患者出现轻度ERCP术后出血,8例患者腹痛均有不同程度缓解。术后随访6个月~2年,期间1例患者复发结石;1例患者复查发现早期壶腹肿瘤,行外科手术治疗。结论 ERCP治疗先天性胆总管囊肿切除术后胆总管残端结石是安全有效的。

全覆膜金属支架在胆总管囊肿切除术后胆总管残端结石治疗中的应用

目的 评估全覆膜金属支架(FCMS)在胆总管囊肿切除术后胆总管残端结石治疗中的应用价值。方法 回顾性分析2019年1月至2022年12月同济大学附属东方医院采取内镜逆行胰胆管造影(ERCP)置入FCMS治疗的22例胆总管囊肿切除术后胆总管残端结石患者的临床资料,观察FCMS首次置入成功率、FCMS二次置入成功率、FCMS留置时间、并发症发生率、胆总管狭窄解除率、FCMS取出成功率,胆总管残端结石消失率、溶解率及清除率,以及结石复发率。结果 FCMS首次置入成功率为72.7%(16/22)、二次置入成功率为100.0%(6/6),FCMS留置92~205 d,中位时间176 d,术后未发生急性胰腺炎、出血、支架移位等并发症,狭窄解除率为90.9%(20/22),FCMS取出成功率为100%(22/22),胰管结石消失率为68.2%(15/22),胰管结石溶解率为85.7%(6/7),胰管结石清除率为100%(7/7)。随访16~50个月,胆总管残端结石复发率为4.5%(1/22),再次行ERCP成功取出结石。结论 FCMS治胆总管囊肿切除术后胆总管残端结石安全、有效,在解除胆总管狭窄的同时可引起结石的溶解、消失,进而提高结石清除率。

经皮肝穿刺胆管引流联合经皮空肠穿刺引流治疗胆总管囊肿切除术后肝内胆管结石

目的 评估经皮肝穿刺胆管引流(PTCD)联合经皮空肠穿刺引流(PJD)治疗胆总管囊肿切除术后肝内胆管结石的临床价值。方法 回顾性分析2022年1月至2023年12月同济大学附属东方医院采取PTCD联合PJD治疗的16例胆总管囊肿切除术后肝内胆管结石患者的临床资料,总结治疗体会。结果 16例患者中,诊断为肝内胆管结石6例,肝内胆管结石合并胆肠吻合口狭窄7例,肝内胆管结石合并左肝管狭窄3例;7例因梗阻性黄疸于外院行PTCD,7例未能找到确切的胆肠吻合的空肠襻,2例引流管穿刺进十二指肠。在我院的9例患者均成功实施PTCD,其中左肝管3例,右肝管6例,未发生出血、胆漏、气胸等并发症。16例患者在PTCD引导下成功实施PJD并完成瘘道扩张术,未发生出血、肠漏等并发症。经皮空肠胆道镜(PJCS)下球囊扩张解除胆肠吻合口及左肝管狭窄,经过3~6次胆道镜操作取净肝内胆管结石。5例胆肠吻合口狭窄患者及3例左肝管狭窄经PTCD瘘道置入全覆膜金属支架(FCMS),FCMS留置6~9个月无移位,经PTCD瘘道取出FCMS后,行经皮经肝胆道镜(PTCS)或PJCS检查见吻合口通畅、吻合口黏膜移行良好。随访5~16个月未发现狭窄复发病例。结论 PJD为胆总管囊肿切除术后肝内胆管结石的胆道镜治疗提供了一条便捷路径,PTCD引导不仅能增加PJD成功率,还能降低FCMS的移位率。

Biliary cystadenoma

The diagnosis of cystadenoma is rare, even more so when located in the extrahepatic bile duct. Unspecific clinical signs may lead this pathology to be misdiagnosed. The need for pathological anatomy in order to distinguish cystadenomas from simple biliary cysts is crucial. The most usual treatment nowadays is resection of the bile duct, together with cholecystectomy and Roux-en-Y reconstruction.

Computed tomography virtual endoscopy with angiographic imaging for the treatment of type Ⅳ-A choledochal cyst

Type Ⅳ-A choledochal cysts (CCs) are a congenital biliary anomaly which involve dilatation of the extrahepatic and intrahepatic bile ducts. We present the case of a 30-year-old woman with type Ⅳ-A CC, on whom threedimensional computed tomography (3D CT) and virtual endoscopy were performed. 3D CT revealed partial dilatation in the posterior branch of the intrahepatic bile duct and a relative stricture between it and the extrahepatic bile duct. Virtual endoscopy showed that this stricture was membrane-like and separated from the surrounding blood vessels. Based on these image findings, complete cyst resection, bile duct plasty for the stricture, and hepaticojejunostomy were safely performed. To the best of our knowledge, there are no reports of imaging by virtual endoscopy of the biliary tract which show the surrounding blood vessels running along the bile duct.

FTS护理干预联合个性化饮食护理干预在小儿胆总管囊肿手术中的应用

目的:探讨加速康复外科护理(FTS)联合个性化饮食护理干预在小儿胆总管囊肿手术中的应用效果。方法:将2019年6月1日~2021年6月1日80例胆总管囊肿手术患儿采用随机数字表法分为研究组和对照组各40例;对照组给予常规护理方案,研究组给予FTS联合个性化饮食护理干预。比较两组患儿手术相关指标,术后不良事件发生率,生活质量[采用简明健康状况调查问卷(SF-36)]。结果:研究组术中出血量、胃肠功能恢复时间、引流管拔除时间、住院时间、术后首次饮水时间、术后出现浮肿、腹腔积液>2 cm、达全量场内营养时间、住院费用情况均优于对照组(P<0.01,P<0.05);研究组不良事件发生率低于对照组(P<0.01);术后1年,研究组生活质量各维度评分均高于对照组(P<0.01,P<0.05)。结论:FTS联合个性化饮食护理用于小儿胆总管囊肿手术效果满意,对降低患儿术后不良事件发生风险具有积极作用,有助于提高患儿生活质量。

先天性胆管囊肿癌变与首次手术术式的关系探讨

目的 探讨先天性胆管囊肿癌变与原手术术式的关系。方法 对近 30年来收治的 2 1例先天性胆管囊肿癌变患者的资料进行回顾性分析。结果 本组先天性胆管囊肿总癌变率 14 8% ;原手术为囊肿内引流术者的癌变率显著高于囊肿切除术 (P <0 .0 0 1) ;囊肿内引流术后患者癌变年龄显著小于囊肿切除术 (P <0 .0 1)和未手术者 (P <0 .0 1) ;囊肿内引流术后发生癌变的年限显著短于囊肿切除术 (P <0 .0 1) ;囊肿内引流术后癌变者的发病年龄比未手术者早 15 4年。结论 囊肿内引流术能加速和促进癌变发生 ,应废用 ;囊肿切除术应列为首选术式 ;囊肿应尽可能切净 ,不能切净者应剥除内膜或破坏其粘膜。

先天性胆管囊肿内引流术后胆管癌:附25例报告

目的 探讨先天性胆管囊肿内引流术后癌变及其手术处理原则。方法 对 1970～ 1996年间收治的先天性胆管囊肿行内引流术的 82例患者进行随访 ,并对其中术后 2 5例癌变患者的临床资料进行分析。结果 本组先天性胆管囊肿内引流术后癌变率为 3 0 .49%( 2 5 /82 ) ,其中囊肿十二指肠吻合术后癌变率为 3 5 .2 9%( 14 /5 1) ,囊肿空肠Roux en Y吻合术后癌变率为 2 2 .5 8%( 11/3 1)。 2 5例中 3例行胰十二指肠切除术 ,4例行肿瘤切除胆道重建术 ,4例行局部病灶切除外引流术 ,14例肿瘤广泛转移无法切除仅行外引流术。结论 囊肿内引流术后癌变率高 ,应该摒弃该术式。

成人先天性胆总管囊肿的二次手术分析

目的探讨成人先天性胆总管囊肿二次手术的原因及治疗对策。方法回顾性分析2010年9月-2015年12月我科7例先天性胆总管囊肿接受二次手术的临床资料。3例第1次手术囊肿残留未处理,4例第1次手术行囊肿切除及胆肠吻合（其中1例囊肿部分残余伴结石,其余3例吻合口狭窄伴结石）。二次手术以囊肿完全切除、胆肠吻合口重建为首选手术方式,1例Ⅳ型囊肿加做左半肝切除。二次手术中1例选择腹腔镜手术。结果 7例手术顺利,手术时间203-466min,平均338.7 min;术中出血量50-500 ml,平均178.6 ml;术后住院时间7-20 d,平均10.7 d。1例二次手术后34个月复发肝内胆管结石,局麻下行盲襻切开、胆道镜取石;其余6例术后恢复顺利,无结石及胆管炎复发。结论成人先天性胆总管囊肿二次手术以首次手术术式选择不当[单纯改道和（或）囊肿切除不全]、胆管结石、吻合口狭窄等为常见原因,再次治疗以囊肿完整切除和（或）肝脏部分切除、胆管（肝管）-空肠Roux-en-Y吻合手术为首选;对于合并肝内胆管结石复发患者,二次手术可皮下埋置空肠盲襻,便于再次处理复发的胆道结石。

磁共振胰胆管成像诊断胰胆管合流异常的价值

目的 探讨磁共振胰胆管成像（MRCP）诊断胰胆管合流异常（APBDU）的价值。资料与方法 分析11例患者的MRCP、轴面T2WI及T1WI。测量共通管长度和胆总管宽度，观察APBDU的形式，以及胆管内有无结石和肿瘤等相关疾病。将观察结果与B超及内镜逆行性胰胆管造影（ERCP）检查和／或外科手术所见对照。结果 10例胆总管囊肿型APBDU包括胆管囊状扩张7例，梭形扩张3例；1例非囊肿型APBDU表现为胆总管轻度扩张。胆总管最大直径范围是12～90mm，平均34mm。APBDU的合流形式包括A型7例，B型4例，在MRCP图像测量的共通管长度范围是18～30mm，平均23mm。轴面T2WI显示胆总管或胰管突然蝌蚪或逗点样增粗5例。APBDU相关疾病包括胆囊／胆管结石6例，胆管肿瘤1例，胰腺炎5例。对7例切除的病变胆管和胆囊标本进行病理组织学检查，均有慢性炎症改变。结论 MRCP检查有助于发现和诊断APBDU，轴面T2WI和B超检查阴性结果不能完全排除本病。