Synchronous Todani types I and III choledochal cysts in a 10-month-old-infant: type IVb

A 10-month-old, previously healthy boy presented with one week of mild jaundice, light-colored stools and irritability. Abdominal sonography showed a large type I choledochal cyst and a separate, distinct cystic mass at the head of the pancreas. Magnetic resonance cholangiopancreatography was performed to evaluate the relationship of the two lesions. A type I choledochal cyst was confirmed, and a coexisting type III choledochocele was identified as the second cystic structure in conjunction with pancreaticobiliary malunion. The infant had complete resection of the type I choledochal cyst with Roux-en-Y hepaticojejunostomy, and anterior duodenotomy with marsupialization of the choledochocele. After five years of follow-up, the child is thriving and has had no recurrence of his symptoms. An exhaustive review of the literature identifies only one previous case of synchronous types I and III choledochal cysts, and this association is not clearly defined among the traditional classifications of type IV multiple choledochal cysts. Because operative management of a type III cyst requires the addition of a transduodenal approach, we encourage accurate reporting of mixed type choledochal cysts for the benefit of surgical planning, epidemiologic tracking and outcomes.     

Choledochal cysts: a nine-year review

Hospital records of 24 Chinese infants and children (7 males and 17 females) with choledochal cysts were reviewed. The patients were aged 1 day to 17 years. Abdominal pain, palpable abdominal mass and jaundice, either alone or in combination, accounted for most of the initial presentations. Twenty-two patients were classified as type I with surgery, while the other two patients were type V without surgery. Ultrasonography is a rapid and accurate diagnostic method in the initial evaluation. Cyst excision wth Roux-en-Y hepaticojejunostomy was the treatment of choice in this series. Chronic cholecystitis with or without inflammation of choledochal cyst was the most common pathological finding. Five patients underwent liver biopsies, showing four biliary cirrhosis and one portal fibrosis. So far, there is no evidence of malignancy in any patient.     

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.     

Duodenogastric reflux following biliary reconstruction after excision of choledochal cyst

Duodenogastric reflux (DGR) was assessed in patients surgically treated for choledochal cyst, with emphasis on two different biliary reconstruction methods: Roux-en-Y hepaticojejunostomy (HJ) and hepaticoduodenostomy (HD). Gastric bile monitoring with the Bilitec device revealed excessive DGR in patients in the HD group. Endoscopic findings demonstrated mild to moderate gastric mucosal erosion in patients after HD. In contrast, neither DGR nor gastritis was found in patients after HJ. This preliminary study suggests that HJ, rather than HD, should be recommended as a method of biliary reconstruction for pediatric patients with choledochal cyst. Careful observation of DGR should be continued in patients who have undergone HD.     

Impact of prenatal diagnosis on choledochal cysts and the benefits of early excision

Aim:   To evaluate the clinical outcomes of patients with prenatally diagnosed choledochal cysts compared with those diagnosed after birth and the optimal timing of definitive treatment.
Methods:   Retrospective review of all patients who underwent primary choledochal cyst excision and Roux-en-Y hepaticojejunostomy from 1996 to 2006 at a single institution.
Results:   A total of 45 patients were included. Ten (22.2%) of the patients had prior prenatal diagnosis. The mean age at operation for this group was 4.4 months and mean follow up was 55.9 months. There was no operative complication or late morbidity. For the post-natal diagnosis group, the mean age at operation was 5.7 years ( P  < 0.000) and mean follow up period was 69 months. The most common presentation in this group was abdominal pain (31.4%), followed by pancreatitis (28.6%) and symptoms of cholestasis (25.7%). Early post-operative morbidities occurred in two (5.7%) patients. On long-term follow up, two (5.7%) further patients in the post-natal group developed complications.
Conclusion:   Prenatal diagnosis of choledochal cysts results in earlier definitive surgery. More adverse complications were seen in those who had surgery at an older age. We therefore recommend early excision of choledochal cysts.     

不同年龄胆总管囊肿的临床与肝纤维化特点探讨

目的 探讨不同年龄胆总管囊肿的临床与肝脏纤维化特点与相互关系。方法 分析12例婴儿及36例幼儿、儿童胆总管囊肿的临床资料;肝组织HE染色观察纤维化程度和炎性细胞浸润情况,免疫组化染色观察细胞角化蛋白(AE1/AE3)、人类组织相容性抗原DR(HLA—DR)表达,并与18例胆道闭锁作对照。结果 婴儿胆总管囊肿黄疸出现率12/12,显著高于幼儿儿童组8/36(P<0.01);肝纤维化程度、小叶周边AE1/AE3阳性细胞和HLA—DR在肝脏表达显著高于非婴儿组(P<0.01),低于胆道闭锁组(P<0.01)。肝纤维化程度与黄疸及肝小叶周边AE1/AE3表达分级呈正相关(P<0.05)。结论 婴儿胆总管囊肿以黄疸为主要临床表现,其肝纤维化重于幼儿及儿童组,胆道梗阻和胆小管增生可能是其发生肝纤维化的原因。     

经腹腔镜手术治疗3个月内婴儿先天性胆总管囊肿

目的探讨经腹腔镜行囊肿切除、肝管空肠Roux-en-Y吻合治疗3个月以内先天性胆总管囊肿的可行性和治疗效果。方法本院2011年7月至2015年7月经腹腔镜手术治疗的3个月以内先天性胆总管囊肿患儿41例,其中男13例,女28例,均为囊肿型;手术年龄8~89 d,平均(50.2±23)d,体重2.7~6.47 kg,平均(4.47±1.01)kg。33例合并黄疸,8例大便颜色变浅,7例出现白陶土样便;31例术前检查转氨酶升高。全部病例均采用经腹腔镜完整囊肿切除,肝管空肠Roux-en-Y吻合术。结果 40例患儿成功完成手术,手术时间117~296 min,平均(186±42)min,出血量5 mL;1例损伤门静脉,中转开腹,出血50 mL;术后无一例发生肠瘘、胆瘘和胰瘘;随访5~53个月,无胆管狭窄、胆管炎、粘连性肠梗阻等发生。结论 3个月以内婴儿胆总管囊肿容易发生肝功能损害、肝脏纤维化,应尽早手术治疗。经腹腔镜手术治疗先天性胆总管囊肿安全有效。     

减少儿童腹腔镜下胆总管囊肿切除术并发症的对策

目的介绍儿童腹腔镜下胆总管囊肿切除、空肠肝管Roux-Y吻合术的初步临床经验,并就如何减少术中风险和术后并发症展开讨论。方法总结上海交通大学医学院附属新华医院儿外科和上海儿童医学中心外科的61例儿童胆总管囊肿经腹腔镜手术病例。其中男18例,女43例,年龄8 d～14岁,术前均行磁共振胰胆管造影(MRCP)并提示为Ⅰ型胆总管囊肿,囊肿直径1.2～12 cm,均未作外引流。结果 61例中54例成功经腹腔镜完成囊肿切除、空肠肝总管Roux-Y吻合术,7例分别因囊肿巨大、炎性粘连、出血或技术原因中转开腹。术后出现吻合口漏2例;随访2～43个月,胰头部囊肿形成2例,存在胰腺炎发作1例,有粘连性肠梗阻2例,均保守治疗后缓解。结论腹腔镜下儿童胆总管囊肿切除术具有显著的微创手术优点,但需要娴熟的镜下操作技能和经验的积累,减少手术并发症的风险是将该技术在临床推广的重要前提。     

A retrospective study of choledochal cyst: clinical presentation, diagnosis and treatment

The treatment and early diagnosis of choledochal cyst are very important to prevent complications of the disease. Delay in treatment can cause lethal complications like biliary cirrhosis and increased morbidity in patients. We studied 26 children with choledochal cyst retrospectively for their clinical presentation, and for the diagnostic tools and treatment modalities, used. The most common findings of choledochal cyst in our series were abdominal pain, vomiting, and ictures in contrast to the classical triad of the disease of jaundice, mass and pain. The most useful diagnostic tool was abdominal ultrasonography. For the treatment, we preferred Roux-en-Y hepaticojejunostomy to the internal drainage procedures, and the results were excellent with minimal mortality and morbidity. We underline prompt diagnosis and treatment to prevent late complications of the disease.     

A hepaticojejunostomy: technical errors with ‘twists and turns’

Excision of a choledochal cyst followed by biliary reconstruction with a Roux-en-Y hepaticojejunostomy is the treatment of choice for type I and IV choledochal cysts. We present a rare complication which was identified 8 years after the original reconstructive surgery. Conventional imaging modalities failed to identify the pathology. Only a contrast enhanced CT scan supported by image rendering software allowed for the visualization of the underlying chronic obstruction of part of the mesentery.     

Aplasia of the dorsal pancreas and choledochal cyst

The authors describe a rare case of choledochal cyst and aplasia of the dorsal pancreas complicated with chronic pancreatitis. A 9-year-old boy presented with obstructive jaundice. After biliary drainage using pericutaneous transhepatic gallbladder drainage (PTGBD) technique, the patient underwent choledochal cyst excision with Roux-en-Y hepaticojejunostomy. The association of choledochal cyst and aplasia of the dorsal pancreas with chronic pancreatitis has never been reported previously.     

Spontaneous rupture of a choledochal cyst in a 11-month-old girl]

Choledochal cysts are rare congenital malformations of the biliary tract. Though most cysts are diagnosed incidentally, some present directly with complications. We report on the case of an 11-month-old girl admitted for abdominal pain, fever and vomiting. Ultrasonography revealed intraabdominal fluid and the absence of a choledochal cyst diagnosed 2 months earlier. Laparotomy for suspected rupture of a choledochal cyst was planned and a choledochojejunostomy with Roux-en-Y was performed. Spontaneous rupture of a choledochal cyst is rare and occurs most frequently in children under the age of 4. The exact cause is yet unknown and several factors have been implicated. The most probable cause is the combination of pancreatic reflux and epithelial irritation of a weakened cyst wall. Choledochal cysts should preferably be treated as soon as the child is 6 months old. Complete excision of the cyst is mandatory because of the risk of malignant transformation.     

Choledochal cysts in infancy and childhood

Objective  To study the clinical presentation, management, and results of treatment of 41 consecutive cases of choledochal cyst (CC) managed from 1999 to 2006. Methods  The age of the patients ranged from 20 days to 11 years. Eleven cases were 1 year or less in age (infantile group), and 30 patients were more than 1 year old (classical pediatric group). Children less than 1 year old presented with jaundice (72%), hepatomegaly (54%) and clay-colored stools (63%); whereas those above one year in age presented with pain (83.3%) and jaundice (47.6%). Three cases had biliary perforation with localized or generalized biliary peritonitis. Ultrasonography (US) diagnosed/suggested CC in all 41 patients, however, contrast-enhanced computed tomographic (CECT) scan (n=12), radionuclide DISIDA scan (n=5), and magnetic resonance cholangiopancreatogram (MRCP) (n=3) also contributed to the diagnosis. 19 patients, including 4 from the ‘infantile’ group, had significant dilatation of intrahepatic biliary ducts. Results  Nine patients from the ‘infantile’ group had cystic type I CC (type Ic), while 2 patients had fusiform dilatation of the common bile duct (type if disease). In contrast, 21 of the older patients had type If disease while 9 had type Ic disease. Operative management included primary complete excision of the cyst with a Roux-en-Y hepaticodochojejunostomy (HDJ) (n=32), Lilly’s modification of submucosal resection with a HDJ (n=8), and cyst excision with a hepaticodochoduodenostomy (HDD) (n=1). All 3 patients with biliary perforation had primary excision of the CC with a HDJ. Unusual operative findings included accessory hepatic ducts (n=2), anteriorly placed common or right hepatic arteries (n=3), and partially or completely blocked proximal extrahepatic bile ducts (n=3). There were 2 postoperative deaths, one had cirrhotic liver disease with portal hypertension, the other had deep icterus with poor general condition. Both patients succumbed to overwhelming sepsis. One patient required reexploration and refashioning of the HDJ for biliary leak. Short-term follow-up was satisfactory in all 39 patients. Conclusion  Infants with CC constitute a distinct group with regard to clinical presentation and the pathological anatomy. CC should be kept in mind while evaluating neonates and infants with cholestatic jaundice, and older children with recurrent abdominal pain. Primary excision of the cyst with a HDJ provides satisfactory results in the management of the vast majority of cases of CC including those in infants, massive CC, and in the presence of biliary perforation and peritonitis.     

Comparison of hepaticoantrostomy and hepaticojejunostomy for biliary reconstruction after resection of a choledochal cyst

Twelve infants operated upon for choledochal cyst (CC) are reviewed with emphasis on the operative technique of biliary tract reconstruction, incidence of cholangitis, postoperative hypergastrinemia, biliary excretion, and upper gastrointestinal (GI) motility in a follow-up of 24 to 35 months. In 7 patients biliary reconstruction was performed with a Roux-en-Y hepaticojejunostomy (HJ), and in 5 with a hepaticoantrostomy (HAST). In the HJ group 4 patients had recurrent episodes of cholangitis and intermittent diarrhea and serum gastrin levels were significantly elevated in 5. Hepatobiliary scintigraphy showed unobstructed excretion of labelled bile through bile ducts into the Roux-en-Y loop, but with significantly delayed emptying of bile into the distal jejunum in all patients. Gastric emptying and upper intestinal passage were normal. In the HAST group no episode of cholangitis occurred and serum gastrin levels were within the normal range. Scintigraphically, hepatobiliary excretion, and duodenojejunal passage of labelled bile was normal, except in 1 patient who developed a postoperative stenosis of the left hepatic duct. Upper GI contrast studies demonstrated normal gastric emptying without reflux into the biliary system. These results suggest that biliary reconstruction with HAST can be performed safely with a low incidence of complications HAST offers a more physiologic method of biliary reconstruction after resection of a CC that allows bile to drain directly into the duodenum.     

小儿腹腔镜胆总管囊肿切除术45例

目的总结腹腔镜下小儿胆总管囊肿切除、肝总管空肠Roux-en-Y吻合术的经验。方法回顾性分析2010年1月至2012年12月我们收治的45例小儿先天性胆总管囊肿患儿临床资料,其中囊肿型32例,梭形扩张型13例,囊肿直径约1．2-5．5em,均在腹腔镜下行胆总管囊肿根治术,空肠Roux-en-Y吻合在腹壁外经脐部小切口进行,肝肠吻合在腹腔镜下完成。结果本组45例中,1例因炎症较重,游离囊肿前壁时渗血较多,解剖困难而中转开腹手术;1例术后6d进食后出现乳糜腹（经肠内、肠外营养14d后治愈）。其余43例均未出现并发症,痊愈出院。术后平均住院时间11d。结论初期开展腹腔镜下胆总管囊肿切除手术时要严格挑选病人,先从囊肿小、炎症轻、发病时间短的病例开始,逐渐积累经验后再扩大腔镜手术指征。腹腔镜手术具有创伤小、视野清晰、恢复快,并发症少等优点。腹腔镜下胆总管囊肿切除术是先天性胆总管囊肿的理想手术方法。     

紧贴囊壁剥离法切除胆总管囊肿的临床评价

目的：对紧贴囊壁剥离法（或称疝囊剥离法）切除胆总管囊肿的安全性及可行性进行评估。方法：回顾1984－1999年的胆总管囊肿113例，其中106例行囊肿切除、胆肠Roux-en-Y吻合术，均是采用敞开囊腔紧贴囊壁剥离法切除囊肿。结果：106例手术均顺利，无意外损伤，失血量、输血量绝大多数在200ml以内，3例未输血，无严重并发症及死亡.结论：敞开囊腔紧贴囊壁剥离法简化了手术操作和降低了手术难度，增加了手术安全性，因而被认为是切除胆总管囊肿的安全、有效、易掌握的方法。     

改良肝总管空肠Roux-Y型吻合术治疗小儿先天性胆总管囊肿

目的 探讨小儿先天性胆总管囊肿的治疗方法。方法 本组２８例，男９例，女１９例，年龄１０ｄ￣１４岁，均采用改良的肝总管空肠Ｒｏｕｘ－Ｙ型吻合术治疗先天性胆总管囊肿。结果 ２８例均痊愈出院。随访６个月￣８年，全组患儿未见出现腹痛、黄疸、发热。其中２０例患儿，经Ｂ超检查，未见胆管狭窄及扩张。Ｘ线钡餐检查，未见有钡剂反流入胆道。结论 改良肝总管空肠Ｒｏｕｘ－Ｙ型吻合术是治疗先天性胆总管囊肿的较好方法。     

Choledochal cysts: lessons from a 20 year experience

Cystic dilatation of the biliary tree is a rare congenital anomaly. To determine mode of presentation, diagnostic pitfalls, and long term outcome after surgery, 78 children (57 girls, 21 boys) with choledochal cyst treated between 1974 and 1994 were reviewed. Anatomical types were: Ic (n = 44), If (n = 28), IVa (n = 4), and V (n = 2); a common pancreaticobiliary channel was identified in 76% patients. Age at presentation ranged from 0-16 (median 2.2) years, six patients being diagnosed by prenatal ultrasonography. Of the 72 patients diagnosed postnatally, 50 (69%) presented with jaundice, associated with abdominal pain in 25 or a palpable mass in three, 13 (18%) presented with pain alone, and two (3%) with a palpable mass. The classic triad of jaundice, pain, and a right hypochondrial mass was present in only four (6%). Four children presented acutely after spontaneous perforation of a choledochal cyst, two presented with ascites and one cyst was discovered incidentally. Plasma and/or biliary amylase values were raised in 30 of 31 patients investigated for abdominal pain; seven had evidence of pancreatitis at operation. In 35 of 67 (52%) patients referred without previous surgery, symptoms had been present for more than one month, and in 14 of them for more than one year, before diagnosis. Delayed referral was due to misdiagnosis as hepatitis (n = 12), incomplete investigation of abdominal pain (n = 6), and failure to note the significance of ultrasonographic findings (n = 10). Two patients referred late died from liver failure. Of the 76 patients with type I or IV cysts, 59 underwent radical cyst excision and hepaticojejunostomy as a primary procedure and 10 as a secondary operation after previously unsuccessful surgery. Sixteen patients have been lost to follow up but most of the remainder are well after a mean period of 4.1 (0.1-13) years. Choledochal cysts are often misdiagnosed, but prognosis is excellent if radical excision is performed.     

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.