The efficacy of double-balloon enteroscopy for intrahepatic bile duct stones after Roux-en-Y hepaticojejunostomy for choledochal cysts

Introduction

Intrahepatic bile duct (IHBD) stones are one of the most complicated morbidities that occur after Roux-en-Y hepaticojejunostomy (RYH); however, the optimal therapeutic approach is controversial.

Methods

Double-balloon enteroscopy (DBE) has been widely and frequently performed even in pediatric patients. We herein report the successful management of IHBD stones by biliary lithotripsy using DBE after RYH for a choledochal cyst (CC). DBE has made it possible to perform endoscopic therapeutic intervention, including balloon dilatation of an anastomotic stricture and removal of IHBD stones, without any major complications.

Conclusion

DBE is a less invasive and safe treatment method for IHBD stones in pediatric patients, which is capable of reaching the bilioenteric anastomosis after RYH for CC.     

Experience with choledochal cysts in infants

The purpose of this analysis is to study the clinical features, liver histology and the outcome after surgery in infants with choledochal cysts (CDCs), and to draw conclusions on the management strategy accordingly. The clinical features, liver histology and the outcome after surgery in 14 cases of CDCs treated before 1 year of age (the “infantile group”) were compared with 52 cases that were diagnosed and treated between 1 and 12 years of age (the “classical pediatric group”). The differentiation of the “infantile choledochal cysts” from “biliary atresia with cystic dilatation” was based on the configuration of intrahepatic bile ducts on cholangiogram. Jaundice was the universal presenting feature in 14 patients in the “infantile group”, the clinical presentation in 6 infants was indistinguishable from biliary atresia. On the other hand, pancreatitis as a presenting complication was seen exclusively in those presenting later, i.e. the “classical pediatric group”. Fibrosis, as early as 4 weeks of age, was seen in all nine (100%) infants in whom a liver histology was available. Seven of these nine infants had bridging fibrosis (n = 4) or cirrhosis (n = 3) on liver histology. The outcome was satisfactory in 9 of the 14 (64.3%) infants despite bridging fibrosis (n = 4) or cirrhosis (n = 1) in 5 of these 9 babies. The CDCs presenting in infancy differ from the classical variety of later presenting pediatric CDCs in terms of their presentation, liver histology and outcome. They merit an emergent surgical treatment following which the outcome is satisfactory. Delay results in progression to end stage liver disease.     

Correctable plasma lipoprotein abnormalities in infants with choledochal cysts

Plasma lipoproteins from two female patients--patient A, 4 wk and patient B, 19 months--were examined prior to and at 1 and 5 wk after surgical correction of biliary obstruction due to choledochal cyst. The findings were correlated with standard indices of hepatic function, namely SGPT, GGTP, 5'nucleotidase, serum bile salts, and total and conjugated bilirubin. Prior to surgery in both patients plasma cholesterol, phospholipid, and triglyceride were elevated; cholesterol esters were low; high-density lipoprotein (HDL) cholesterol and apolipoprotein A-I, the major protein constituent of HDL, were subnormal; most of the plasma lipids were contained in the low-density lipoprotein density region; lipoprotein-X was present. Patient B had had a relatively brief obstruction and suffered little secondary hepatic injury. One week after surgery, plasma lipid concentrations returned to normal; apolipoprotein A-I increased in the HDL density region and a concomitant rise in cholesterol esters to near normal, 65%, was observed; plasma lipids were contained predominantly in HDL; hepatic function improved markedly. Patient A had had intrauterine obstruction and suffered major hepatic injury with cirrhosis. One week after surgery, plasma lipid concentrations, cholesterol esters, low-density lipoprotein lipid predominance, and hepatic function remained essentially unchanged. Five weeks after surgery, the lipoprotein levels and composition and hepatic function were near normal.In conclusion, children with biliary obstruction have lipoprotein abnormalities similar to those seen in adult patients. These alterations are rapidly reversible with surgical relief and may be used as prognostic indicators of outcome.     

Wide hilar hepatico-jejunostomy: the optimum method of reconstruction after choledochal cyst excision

Standard reconstruction after choledochal cyst excision is by Roux-en-Y hepaticojejunostomy to the common hepatic duct. Long-term follow up studies have shown a 10% incidence of late complications, including anastomotic stricture. By extending the bilio-enteric anastomosis along the left hepatic duct, a wide hilar bilio-enteric anastomosis is created which may help to minimize late anastomotic complications. Forty-one consecutive patients (24 girls, 18 infants) with a median age of 2.3 years (range 44 days to 15.6 years) and median weight 11.5 kg (range 2.1–59 kg) underwent radical choledochal cyst excision with a wide hilar hepticojejunostomy. Thirty-eight were followed-up both clinically and by ultrasound scan and biochemical liver function tests for a median of 2.7 years (range 0.1–12.5 years). The median width of the hilar hepaticojejunostomy was 8 mm (range 6–25 mm) in 18 infants, and 15 mm (range 10–25 mm) in 22 older children. In one patient it was not measured. Only one surgical complication occurred—a self-limiting bile leak which settled spontaneously. Median postoperative stay was 6 days (range 5–21 days). No patient has had an episode of cholangitis or adhesive small bowel obstruction to date. Postoperative biochemical liver function tests have remained normal in all but one child (with pre-existing biliary cirrhosis). After radical resection of a choledochal cyst, a wide hilar hepaticojejunostomy is a, safe, effective and durable reconstructive technique that can be performed at any age and may help to minimize the long-term risk of complications.     

腹腔镜与开放性肝管空肠吻合术治疗先天性胆总管囊肿的疗效比较

目的比较腹腔镜与开放性肝管空肠吻合术治疗先天性胆总管囊肿的中期疗效,探讨腹腔镜下肝管空肠吻合术治疗先天性胆总管囊肿的可行性及安全性。方法回顾性分析2001年10月至2009年10月作者采取腹腔镜下肝管空肠吻合术治疗的218例先天性胆总管囊肿患儿临床资料,并将1993年9月至2001年9月行开放性肝管空肠吻合术的200例患儿作为对照,对其诊疗情况及术后恢复、随访情况进行比较。结果腹腔镜手术组与开放手术组手术时中位年龄分别为4．16岁（7d至18岁）、4．59岁（13d至17岁）,中位随访时问分别为38个月、146个月,差异均无统计学意义;病例数超过35例时,腹腔镜组平均手术时间较前明显缩短（P〈0．01）,至2009年,与开放手术组相比,平均手术时间无显著差异[（3．04±0．98）hVS（2．95±0．91）h,P=0．557）]。腹腔镜手术组术中平均失血量约（9．08±6．13）mL,而开放手术组约（35．33±33．29）mL,差异有统计学意义（P〈0．001）。腹腔镜手术组术后住院时间、恢复饮食时间及引流时间均较开放手术组明显缩短（P〈0．001）,腹腔镜手术组术后并发症较开放手术组明显减少（P〈0．01）。结论腹腔镜下肝管空肠吻合术治疗先天性胆总管囊肿安全可行,其中期疗效与开放手术相似,甚至优于开放手术。     

Children presenting with hydatid cysts in common bile duct and choledochal cyst

Hydatid cysts developing primarily in the common bile duct are extremely rare. Occasionally, hydatid cysts rupture into the extrahepatic biliary ducts and cause obstruction [1–7]. Two children with intact hydatid cysts in the common bile duct and choledochal cysts were seen during a 2-year period in the Paediatric Surgery Division of the Children's Hospital, Banaras Hindu University, Varanasi. The details of their presentation and operative management are reported.     

改良囊肿切除肝管空肠Roux-Y吻合术治疗先天性胆总管囊肿

目的：探讨治疗小儿先天性胆总管囊肿的手术方法。方法：本组30例,男12例,女18例,年龄14月至6岁,均采用改良囊肿切除、肝管空肠Roux-Y吻合术治疗。结果：30例均痊愈出院。随访3个月至5年,未出现腹痛、黄疸、发热等症状。经B超检查,未见胆管狭窄;6例消化道钡餐检查未见钡剂返流入肝管空肠吻合口。结论：改良囊肿切除、肝管空肠Roux-Y吻合术是治疗先天性胆总管囊肿的较好方法。     

Meckel syndrome with Caroli disease and choledochal cysts

Meckel syndrome is a lethal autosomal recessive disorder characterized by the triad of cystic renal dysplasia, occipital encephalocele, or other anomaly of the central nervous system and post-axial polydactyly. Malformation of the ductal plate is an integral component of Meckel syndrome. Ductal plate malformations include congenital hepatic fibrosis, biliary hamartoma, autosomal dominant polycystic liver disease, Caroli disease, and choledochal cyst. The occurrence of cystic hepatic disease, Caroli disease, and choledochal cyst have not been highlighted. This is a report of a 26-week fetus with features of Meckel syndrome, Caroli disease, and choledochal cyst.     

胆总管囊肿根治术后胆道梗阻的原因分析和治疗策略

目的 本研究旨在分析胆总管囊肿根治术后胆道梗阻的原因, 提出相应的治疗策略.方法 回顾2006年1月至2016年1月间47例在外院接受胆总管囊肿根治术后胆道梗阻,转入我院成功行腹腔镜二次手术的患儿,对其病因进行观察,分析及治疗.结果 出现胆道梗阻症状,即反复胆管炎或持续肝功能异常距离第一次手术的时间为术后2周~10.8年(平均1.7年).在外院接受抗炎保肝利胆PTD治疗时间2周~10年(平均2.7年),未见明显好转.接受二次手术的间隔时间为1个月~11年(平均4.5年).病理显示57.4% (27/47)患儿存在I~IV级肝纤维化.本研究发现术后胆道梗阻的原因如下:①异位肝右动脉前置压迫肝管空肠吻合口近端导致梗阻占27.6% (13/47).术中将异位肝右动脉重置于肝管空肠吻合口后方解除梗阻;②初次手术未解决伴发的单一或多处肝管狭窄占29.8% (14/47).二次手术中行肝管扩大成型+肝管空肠再吻合;③吻合口狭窄占42.6% (20/47).二次手术中行肝管空肠再吻合.中位随访时间为48个月(1~120个月),无1例发生胆道再梗阻或胆管炎.肝功能于二次术后1个月~2年恢复正常.结论 异位前置肝右动脉压迫、合并单一或多处肝管狭窄、吻合技术是胆总管囊肿术后胆道梗阻的原因.应尽早外科手术解除梗阻避免肝功能损伤及肝硬化.     

The management of choledochal cysts in the newborn

Choledochal cysts are now being diagnosed before birth on routine maternal sonography (US). There is no report in the literature outlining the management of newborns with choledochal cysts, many of whom are asymptomatic. Our study details the diagnosis, treatment and outcome of six such children, four girls and two boys. Five had antenatal US revealing cystic abdominal masses. One had intermittent vomiting and US suggested a choledochal cyst. Four of six had normal serum bilirubin levels; two had elevations. In five babies the choledochal cyst was correctly diagnosed from the preoperative studies; in one the preoperative diagnosis was an ovarian cyst. The children underwent an operation at an average of 6 weeks of age (range 5 days to 17 weeks). At exploration, cholangiography showed Alonso-Lej type I cysts in all cases. Treatment consisted of resection of the cyst with Roux-en-Y choledochojejunostomy in five and with a valved jejunal choledochoduodenal conduit in one. In no case was the dissection of the choledochal cyst off the portal vein and hepatic artery difficult. There were no intra- or early postoperative complications. Mean hospital stay was 8 days (range 5 to 9 days). Presently, all 6 patients have normal bilirubin levels at an average length of follow-up of 35 months (range 16 to 70 months) after operation. We conclude that operative treatment of choledochal cysts in early infancy, even in asymptomatic children, is safe and effective and may prevent serious complications later in life.     

Ultrasonographic spectrum of choledochal cysts in children

Four different sonographic patterns which may be encountered in choledochal cyst are described. The most common form is concentric dilatation of the common bile duct (Type I). Rarely eccentric dilatation of the common bile duct, diverticulum, may be seen (Type II). Both forms of the disease may (Types IB and IIB) or may not (Types IA and IIA) be associated with intrahepatic biliary dilatation.     

Robot-assisted resection of choledochal cysts and hepaticojejunostomy in children

Purpose

Choledochal cysts have traditionally been treated as an open procedure. However, recent publications from some large volume centres show that minimally invasive surgery has become their standard approach. Robotic surgical systems facilitate a surgeon’s ability to undertake complex procedures. We present our experience of using robot assistance to treat choledochal cysts.

Methods

Children admitted with a choledochal cyst from March 2009 to Nov 2012 were included. There were 27 patients (20 females). Mean age 5.4 years (range 0.3–15.9). Mean weight 21.9 kg (range 5.9–78.8); 10 weighed <10 kg. The choledochal cysts were types 1c(12), 1f(10) and 4(5). The da vinci Surgical System was used for the robot-assisted resection of the choledochal cyst and hepaticojejunostomy. Roux loop was fashioned extra-corporeally. Mean follow-up is 2.7 years (range 0.9–4.7).

Results

22 cases were completed successfully with robotic assistance. Five open conversions were for anatomical concerns or technical reasons. One child had three complications (omental hernia, anastomotic stricture and subsequent bile leak). Patients were on full feeds by a median of 5 days (range 3–6) and discharged after 6 days (range 4–7).

Conclusion

Robotic-assisted resection of choledochal cysts in children is safe. They made a rapid recovery with a good cosmetic outcome.     

Bile infection contributes to intrahepatic calculi formation after excision of choledochal cysts

Intrahepatic calculi complicate choledochal cysts in 7–8% of patients. Although congenital stenoses and dilatation of the intrahepatic bile ducts are considered responsible for calculi formation, intrahepatic calculi are usually formed after cyst excision. In this study, bile specimens from patients with choledochal cysts were cultured for bacteria. Results were retrospectively analyzed among the following groups: the primary excision group, consisting of 97 patients undergoing cyst excision as a primary treatment (mean age 5.0 years), the internal drainage group, consisting of 13 patients who had previous cyst-enterostomy at cyst excision (mean age 20.2 years); and the hepatolithiasis group, consisting of 12 patients with postoperative hepatolithiasis (mean age 24.2 years). Bacteria were present in the bile of 10 patients (76.9%) in the internal drainage group and in all patients (100%) in the hepatolithiasis group, but present in only 17 patients (17.5%) in the primary excision group (p<0.01). Polymicrobial infection with Gram-negative enterobacteria such as Escherichia coli and Klebsiella species was predominant in the internal drainage and hepatolithiasis groups, while nonenteric bacteria were found in the primary excision group. Bile infection through bilioenterostomy may play an important role in intrahepatic calculus formation after excision of a choledochal cyst.     

伴有并发症的儿童胆总管囊肿的临床处理

目的 讨论合伴有各类并发症的儿童胆总管囊肿的临床处理及手术时机和方法.方法 回顾性分析2013年1月至2015年12月上海新华医院小儿外科收治的45例合伴有各类并发症的儿童胆总管囊肿患儿的临床资料.其中,胆道穿孔7例,胆源性胰腺炎9例,胆管炎、阻塞性黄疸、肝功能受损29例,保守治疗效果不佳.7例胆道穿孔中,5例胆汁性腹膜炎行囊肿外引流和二期根治术;2例隐匿性胆道穿孔一期行根治术.9例胆源性胰腺炎给予内镜下鼻胆管引流,待淀粉酶正常后一期行根治术.29例阻塞性黄疸伴肝功能受损的患儿中,17例发病年龄小于3个月行一期根治手术;12例发病年龄大于6个月患儿先行ERCP置鼻胆管引流,待黄疸消退、转氨酶正常后一期行根治性手术.结果 伴胆道穿孔的7例胆总管囊肿患儿中,2例在外引流期间出现水电解质紊乱,1例T管脱落,1例隐匿性穿孔在根治术后出血再次手术,余者术后均痊愈出院.9例胆源性胰腺炎患儿置鼻胆管引流后淀粉酶均恢复正常,根治术后均痊愈出院.并发急性胆管炎、伴有梗阻性黄疸、肝功能受损29例中,12例发病年龄大于6个月,其中11例行内镜下置鼻胆管引流后并发症改善行根治手术,1例ERCP失败后改行外引流和二期根治术;小于3月龄婴儿直接行一期根治性手术,术后均痊愈出院.所有患儿保持随访,术后随访时间1～3年.1例术后慢性胰腺炎史,1例胆管炎史,均通过药物治疗缓解症状.结论 对合伴有各类并发症的儿童胆总管囊肿选择合理的处理手段和合适的手术方式将有效减少并发症所造成的危害.     

Factors of biliary carcinogenesis in choledochal cysts.

The long-term complications of choledochal cysts include malignant degeneration of the epithelial lining of the biliary tract. Possible factors leading to malignant degeneration were examined by measuring amylase levels in the biliary tract, intraoperative biliary manometry and a histopathological study. 27 patients, 1 to 13 years of age, with choledochal cysts, were studied over a 13-year period. Amylase levels in the bile of choledochal cysts and the gallbladder were measured. The pressure profile of the biliary tract was recorded as the probe was withdrawn from the sphincter of Oddi (SO) to the distal end of the common bile duct. Hyperplasia of the gallbladder mucosa and malignancy of the biliary tract were investigated. Patients with high levels of biliary amylase had higher pressure differences between the SO and the duodenum than those with low levels of biliary amylase. The incidence of mucosal hyperplasia of the gallbladder mucosa was significantly higher in the fusiform type than in the cystic type. Adenocarcinoma in a cystic choledochal dilatation was found in a 12-year-old girl with high amylase levels. The cause of regurgitation of pancreatic juice into the biliary system might be due to a high pressure difference between the SO and the duodenum in addition to the lack of sphincter function at the abnormal junction of the pancreaticobiliary ductal system. Early diagnosis and surgical treatment to prevent the regurgitation leading to hyperplasia and malignancy of the biliary tract are important for children with choledochal cysts.     

Magnetic resonance cholangiopancreatography on postoperative work-up in children with choledochal cysts

This study aimed to assess the clinical usefulness of magnetic resonance cholangiopancreatography (MRCP) as a postoperative diagnostic tool in children with choledochal cysts. Magnetic resonance cholangiopancreatography was performed postoperatively in 19 patients and image quality was compared with that obtained by intravenous cholangiography spiral computed tomography (IVC-SCT). While the detectability by MRCP was inferior to that by IVC-SCT, MRCP highly (84.2%) depicted the anastomotic site together with the reconstructed bowel and intrahepatic bile ducts. Magnetic resonance cholangiopancreatography also clearly delineated the postoperative condition of pancreaticobiliary maljunction (PBM), residual distal common bile duct, common channel, and pancreatic duct. Since MRCP is noninvasive and requires neither radiation exposure nor a contrast agent, and is useful for detecting both anastomosis and pancreatico-biliary ducts around PBM, MRCP might be superior to IVC-SCT as an imaging technique for outpatient clinics performing long-term follow-up studies in children with choledochal cysts.     

经脐单切口腹腔镜肝管空肠Roux-en-Y吻合术治疗儿童胆总管囊肿的探讨

目的 评价经脐单切口腹腔镜治疗胆总管囊肿患儿的安全性和有效性.方法 回顾性分析2011年4月至7月间在我中心经脐单切口腹腔镜技术治疗的19例胆总管囊肿患儿预后和短期随访结果,并与我中心以往传统4孔腹腔镜手术治疗胆总管囊肿的疗效对比.结果 中位随访时间为3个月.其中2例患儿巾转为传统4孔腹腔镜手术,其余17例患儿成功完成经脐单切口腹腔镜下胆总管囊肿根治手术(平均手术年龄:3岁,男/女:5/12).早期单切口手术中1例患儿术后出现胆漏,腹腔引流l0d后自愈.单切口组的平均手术时间3.06 h与我院传统腹腔镜胆总管囊肿根治术时间3.04 h相比,差异无统计学意义(P=0.9t09).两组的术后平均住院时间(6.23 d比7.41d)、术后恢复进食时间(2.64d比2.86d)、腹腔引流留置时间(3.23d比3.05d)亦无明显差异(P=0.056、0.472、0.619).结论 经脐单切口腹腔镜应用传统腹腔镜器械治疗胆总管囊肿是安全的,短期随访结果与传统腹腔镜疗效相仿,为胆总管囊肿外科治疗提供了新的途径.     

先天性胆管扩张症合并肝内胆管扩张及复杂胆道畸形的诊断与治疗对策

目的研究先天性胆管扩张症合并肝内胆管扩张及复杂胆道畸形的特殊检查方法及其临床意义，探讨在标准术式即囊肿切除胆道重建的基础上对肝内胆管扩张及可能存在的复杂胆道畸形处理的方法。方法对于合并肝内胆管扩张患儿，以术中胆管造影、胆道镜观察等仔细进行术中检查，了解肝总管、胆囊管、左右肝管、肝内胆管扩张及狭窄情况，以及胰胆管共同通道的病理变化，同时仔细检查有无复杂胆道畸形的存在并予以手术处理。结果全组57例，33．3％合并肝内胆管扩张，其中10例为肝内胆管囊样扩张，其汇入肝总管的开口呈瓣膜状、隔膜状或细短管状狭窄。9例为自肝门向肝内胆管起始端逐渐变细的锥形扩张，无明显狭窄。2例合并迷走胆管，3例为左右肝管分别汇入扩张的胆总管囊肿。结论对合并肝内胆管扩张者进行相应的特殊检查具有重要的临床意义，可以指导在标准根治术，即囊肿切除胆道重建的基础上对近端肝内胆管狭窄矫治、可能存在的迷走胆管、复杂胆道畸形及远端胰胆管共同通道病变的处理。     

Spontaneous resolution of intrahepatic bile cysts in two patients with biliary atresia after hepatoportoenterostomy

Intrahepatic bile cysts were found in two girls after successful hepatoportoenterostomy for the treatment of extrahepatic biliary atresia, in the first case during a biliary tract infection; one of the cysts persisted after the infection had abated and finally resolved 3 years later. The cyst in the second case induced jaundice and resolved spontaneously after 3 months. Correspondence to: M.-S. Kong