Diagnostic and therapeutic ERCP in the pediatric age group

The role and value of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric age group is not well established, because pancreatic and biliary diseases are less common in children. This however is not the case in areas like the Eastern Province of Saudi Arabia where sickle cell disease (SCD) and other hemoglobinopathies are common, with increased frequency of cholelithiasis and choledocholithiasis. The purpose of this study was to evaluate the indications, findings, safety and therapies of ERCP in children. One hundred and twenty five children had diagnostic and/or therapeutic ERCP as part of their management at our hospital. Their medical records were reviewed for: age at diagnosis, sex, Hb electrophoresis, indication for ERCP, findings, therapy and complications. There were 77 males and 48 females. Their age at presentation ranged from 5–18 year (mean 13.25 year). The majority of them had sickle cell disease (77.6%). The indications for ERCP were: obstructive jaundice (67.2%), recurrent biliary colic with or without jaundice (10.4%), acute and chronic pancreatitis (7.2%), postoperative bile leak (2.4%), cholangitis with obstructive jaundice (2.4%), hepatitis of unknown etiology (3.2%), cirrhosis of unknown etiology (4%), thalassemia with jaundice (0.8%), hemobilia (0.8%), acute cholecystitis with jaundice (0.8%), and sickle cell disease with ulcerative colitis and obstructive jaundice (0.8%). In six children, ERCP was done following laparoscopic cholecystectomy. ERCP was carried out under sedation in 91 (72.8%) children and under general anesthesia in 34. It was successful in 121 (96.8%) children while cannulation of the Ampulla failed in four. ERCP was normal in 43 children, but eight of them showed evidence of recent stone passage and in six, there were gallstones. In the remaining children, ERCP revealed: normal CBD with stones (18 patients), dilated CBD with stones (17 patients), dilated CBD without stones (19 patients), dilated biliary tree with stones (10 patients), dilated biliary tree without stones (six patients), bile leak (two patients), dilated biliary tree with stones and choledocho-duodenal fistula (one patient), choledochal cyst (two patients), septate gallbladder (one patient), normal ERCP with multiple pancreatic cysts (one patient) and biliary stricture (one patient). The following procedures were carried out: 35 had endoscopic sphincterotomy and stone extraction, 20 had endoscopic sphincterotomy, four had CBD stenting, one underwent removal of a stent, two had insertion of a nasobiliary tube and one had biliary endoprosethesis. There was no mortality. One had bleeding from the site of sphincterotomy which stopped after adrenaline injection. Four patients (3.2%) developed transient mild pancreatitis which settled conservatively. ERCP in the pediatric age group is safe both as a diagnostic and therapeutic procedure. ERCP can provide valuable information which aid in the diagnosis of biliary and pancreatic diseases in children as well as therapy with the technical feasibility of endoscopic sphincterotomy. This is specially so in the era of laparoscopic cholecystectomy, where ERCP should be the treatment of choice in children with CBD stones who are going or have previously undergone laparoscopic cholecystectomy.     

ERCP和MRCP在儿童胆胰疾病中的应用与评估

目的：探讨经内窥镜十二指肠孔头插管进行胰胆管造影（ERCP）和磁共振胰胆管造影（MRCP）在我国小儿胰胆疾病中应用的价值。方法：分析41例接受ERCP检查的患儿B超,CT,ERCP和MRCP的检查结果和ERCP的并发症,并和手术结果进行比较。结果：ERCP显示36例胆总管扩张,3例胆总管结石,1例反复发作的胰腺炎,胰管扩张,1例阴性;MRCP检查23例,胆管扩张20例,胆总管结石2例,阴性1例,ERCP的敏感性和特异性分别为95＝1％,97．4％,MRCP的敏感性和特异性为94．6％,94．8％,3例发生ERCP后并发症,主要是胰腺炎,发生率为4．6％,结论：ERCP和MRCP对小儿胰胆系统疾病的影像学诊断有很高的敏感性和特异性,可作为明确诊断的常规方法。     

The role of the accessory pancreatic duct of Santorini in pancreatic drainage in children (with emphasis on choledochal cyst patients)

Pancreatic drainage patterns have been studied by endoscopic retrograde cholangiopancreatography (ERCP) in four children with choledochal cyst (CC). The first two had mild or chemical pancreatitis; the third had a history of recurrent cholangitis and was diagnosed as chronic pancreatitis. The fourth manifested with severe, acute pancreatitis. All children were found to have an impaired flow through the papilla of Vater. In the first three cases, the duct of Santorini (DS) seemed to play an important role in the pancreatic drainage. In the fourth case, however, the duct was found to be absent. ERCP findings in additional eleven children without CC also were reviewed, and in this group the DS did not seem to play any significant role in pancreatic drainage. These results indicate that in children with common bile duct (CBD) anomalies, the DS may relieve the obstruction and ameliorate the pancreatitis.     

Idiopathic fibrosing pancreatitis presenting with obstructive jaundice in a child.

Idiopathic fibrosing pancreatitis (IFP) is a rare cause of obstructive jaundice in children. The obstruction is caused by circumferential compression of the intrapancreatic portion of the common bile duct (CBD). Various forms of biliary decompression, including surgical sphincteroplasty, choledochoduodenostomy, and Roux-en-Y choledochojejunostomy are employed as a treatment. We report a 14-year-old boy with IFP who was successfully treated by a temporary drainage of compressed CBD with the help of a stent placed by endoscopic retrograde cholangiopancreatography (ERCP). The diagnosis of IFP was confirmed histologically after open surgery. The patient remains well after 3.5 years of follow-up with no evidence of pancreatic insufficiency. This report offers an additional case of IFP causing obstructive jaundice treated by temporary internal drainage of CBD, which is safer and less invasive than biliodigestive surgical decompression.     

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.     

Diagnostic and therapeutic endoscopic retrograde cholangiopancreatography in children: a large series report

OBJECTIVES: Our goal is to evaluate the indications, findings, therapies, safety, and technical success of endoscopic retrograde cholangiopancreatography (ERCP) in children. METHODS: Our database was searched for patients 17 years of age or younger undergoing ERCP between January 1994 and March 2003. Additional information was obtained by chart review. The safety and technical success of ERCP were examined. Complications were classified by the consensus criteria. RESULTS: A total of 245 patients (95 M, 150 F; mean age 12.3 years) underwent 329 examinations. Indications included biliary pathology (n = 93), pancreatic pathology (n = 111), and chronic abdominal pain of suspected biliary or pancreatic origin (n = 41). The ERCP findings were bile duct stone(s) (n = 29), benign biliary stricture (n = 19), primary sclerosing cholangitis (n = 7), anomalous pancreaticobiliary union (n = 8), choledochal cyst (n = 5), bile duct leak (n = 6), malignant biliary stricture (n = 2), biliary atresia (n = 1), chronic pancreatitis (n = 44), pancreas divisum (n = 26), pancreatic duct stricture with (n = 6) or without (n = 9) leak, pancreatic tumor (n = 1), periampullary adenoma (n = 2), and sphincter of Oddi dysfunction (n = 65). Endoscopic therapies were performed in 71% of the procedures and included sphincterotomy, stone extraction, stricture dilation, endoprosthesis placement, snare papillectomy, and cystoduodenostomy. Thirty-two (9.7%) post-ERCP complications occurred and included cholangitis in 1 patient and pancreatitis in 31. The pancreatitis was graded mild in 24, moderate in 5, and severe in 2. No mortality related to ERCP occurred. CONCLUSIONS: Diagnostic and therapeutic ERCP results are similar in children and adults except for a lower incidence of malignant disease in children. Technical success rates are high. However, ERCP-related pancreatitis is not uncommon, and the risk and benefits should be carefully reviewed before proceeding. Outcome data are necessary and is currently being accumulated at our institution.     

Percutaneous management of tumoral biliary obstruction in children

Background There is limited experience of percutaneous biliary interventions in children although they are safe and effective procedures. Objective To evaluate the efficacy and safety of percutaneous management of tumoral biliary obstruction in children. Materials and methods Percutaneous biliary interventions were performed in eight children (six boys, two girls) with a mean age of 10.5 years (range 4–17 years). The interventions included percutaneous biliary drainage (five patients), percutaneous biliary drainage and placement of a self-expanding metallic stent (two patients), and percutaneous cholecystostomy (one patient). All patients had signs of obstructive jaundice and two had cholangitis. Results All procedures were successful. No procedure-related mortality was observed. Bilirubin levels returned to normal in four of the eight patients. Findings of cholangitis resolved in the two affected patients after the procedure and antibiotic treatment. Two patients underwent surgery after percutaneous biliary drainage procedures. A self-expanding metallic stent was placed in two patients with malignancy and the stents remained patent until death. Conclusion Percutaneous biliary interventions can be performed safely for the management of tumoral biliary obstruction in children.     

Late follow-up following internal drainage of choledochal cysts reassessment of necessity for reoperation

To evaluate late results in children who had been treated with an internal drainage procedure for a choledochal cysts, we carried out follow-up examinations and performed endoscopic retrograde cholangiopancreatography (ERCP). Internal drainage procedures were performed on a total of 17 patients prior to 1972. The follow-up period ranged from 18 to 30 years. ERCP was performed on 8 of 10 patients followed up. All had dilatation of the common bile duct; 7 had an anomalous pancreatobiliary junction; and there were no carcinomas detected. The procedures consisted of choledochocystoduodenostomy (CCD) in 12 patients, choledochocystojejunostomy (Roux-Y) (CCJRY) in 4, and cholecystoduodenostomy (CYD) in 1. Of the 12 patients who were treated with CCD, 3 were lost to follow-up, 3 died of liver cirrhosis, and 6 survived. Five of the 6 surviving patients needed a reoperation for cholangitis; in 1 of these severe atypical abnormalities of the cyst were found on pathologic examination. Of the 4 patients who were treated with CCJRY, 1 died of liver cirrhosis but the 3 survivors had an asymptomatic postoperative course common bile duct and an anomalous pancreatobiliary junction noted during the current work-up. The patient who was treated with CYD needed another operation for choledocholithiasis. Our conclusions for following patients who were treated with an internal drainage procedure in childhood are twofold: (1) for patients who had CCD, cyst excision is recommended; and (2) for patients who had CCJRY performed at the age of 10 years or less, cautious observation is recommended as long as the patient remains asymptomatic. Offprint requests to: Masafumi Naito     

Diagnostic and therapeutic endoscopic retrograde cholangiopancreatography (ERCP) in children and adolescents: experience in a single institution

INTRODUCTION: Experience in the use of endoscopic retrograde cholangiopancreatography (ERCP) for the investigation and treatment of pancreaticobiliary disorders is relatively limited in children. This report reviews the experience in a single institution with pediatric ERCPs and documents the indications, success rate, diagnostic and therapeutic yields, complications, and the impact on patient management. PATIENTS AND METHODS: The data of all consecutive patients aged < or = 18 years who underwent ERCP procedures between the years 1997 and 2007 were retrospectively identified through a computer database search. The database prospectively recorded the indications, findings, therapies, and complications. RESULTS: During the study period, 32 ERCP procedures were performed in 28 children with a median age of 13 (range 8 - 18) years. ERCPs were performed for biliary pathology in 21 (75 %) and for pancreatic pathology in 7 (25 %) patients. The most common biliary indications were suspected choledocholithiasis and postoperative bile leaks. Hydatid disease was the most common diagnosis that yielded bile leaks. The pancreatic indications were recurrent pancreatitis and traumatic pancreatic duct disruption. Cannulation of the desired duct was successful in all procedures. An endoscopic sphincterotomy, stone/sludge removal or a stent placement was performed in 20 (63 %) procedures. According to the long-term follow-up, avoidance from any further surgical interventions was achieved in 11 (65 %) children, in whom ERCP was undertaken as a therapeutic intervention. The complication rate was 6 % with the development of mild self-resolving pancreatitis in one patient and stent occlusion in another. CONCLUSIONS: ERCP in the pediatric population has a high success rate, both as a diagnostic tool and for therapeutic interventions, provided it is performed by experienced endoscopists. The delicate delineation of the anatomy by ERCP and its therapeutic potential make it absolutely superior to other less invasive tools such as magnetic resonance cholangiopancreatography.     

Cholestasis caused by biliary botryoid sarcoma

A 4-year-old boy with a short history of jaundice and hepatomegaly was studied by endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC). A large obstructing choledochal tumour was found at the level of cystic duct insertion and at surgery, a botryoid sarcoma was removed. Subsequent cytotoxic therapy was complicated by persistent dilatation of the extrahepatic bile ducts which necessitated eventual excision. There was no evidence of persistent tumour.     

Choledochal cysts: heterogeneity of clinical presentation

Over a 12-year period, 23 children were diagnosed as having choledochal cysts, of which 22 were type I and one was type II. The triad of right upper-quadrant abdominal pain, right upper-quadrant abdominal mass, and cholestatic jaundice was present in only four of 23 (17%). Mean time from the onset of symptoms to establishing the correct diagnosis was 20 months, and in one child the diagnosis was established only at autopsy. Six children had histologic evidence of biliary cirrhosis, and three developed portal hypertension despite surgical intervention. Both ultrasonography of the abdomen and endoscopic retrograde cholangiopancreatography (ERCP) were useful methods in establishing the diagnosis, but both tests did result in false negatives. Choledochal cysts are treatable causes of cholestatic jaundice in infants and children, but the intermittent and variable nature of their presentation renders clinical diagnosis difficult.     

产前诊断的先天性胆管扩张症手术时机探讨

目的 对产前诊断的先天性胆管扩张症的手术时机进行探讨.方法 回顾我院2006年至2010年收治的产前诊断10例先天性胆管扩张症患儿,入院手术年龄27 d至2岁,9例接受腹腔镜胆总管囊肿切除术,肝管空肠Roux-Y吻合术,1例接受开腹手术.观察其临床表现、超声和实验室指标、术中情况、术后恢复情况等.结果 产前囊肿均随孕周逐渐增大.产后5例患儿曾经出现黄疸,4例曾经出现白便.4例超声提示囊肿增大明显,6例ALT升高.术中造影见全部发生胆总管远端梗阻,其中Ⅰ型3例,Ⅳ型7例,3例可见胰胆合流异常.5例患儿术中及术后病理可见肝硬化、肝脏损伤表现.全部10例患儿术后恢复好.结论 产前诊断的先天性胆管扩张症可能是由于胆总管远端梗阻,造成胆管扩张形成.产前诊断先天性胆管扩张症的患儿,应密切观察,发现黄疸白便表现、ALT和AST升高明显、超声提示囊肿短期增大明显应尽快手术治疗.如无上述表现,可定期复查,但也应尽早手术根治.

Abstract：

Objective To present the therapeutic strategy for prenatally diagnosed congenital biliary dilatation (CBD).Methods From 2006 to 2010,10 patients with prenatally diagnosed CBD were treated at this center.Their age ranged from 27 days to 2 years old.Among the 10 patients,9 underwent the laparoscopic total cyst excision with Roux-en-Y hepatojejunostomy,1 was performed open surgery.The clinical features,ultrasonography,laboratory tests and postoperative complications were retrospectively analyzed.Results Results The ultrasonograohy revealed the biliary cysts' size increased with the gestational ages.After birth,5 of the 10 patients had jaundice,4 had acholic stools,4 patients' cysts increased in size,and 6 had elevated transaminases.Intraoperative cholangiography was performed,and distal common bile duct stenosis was found on all patients.Three of them were type Ⅰ of CBD,7 of them were type Ⅳ,and the other 3 had pancreaticobiliary malunion.Liver cirrhosis was found in 5 of the 10 patients.All patients recovered from the surgery.Conclusions Distal common bile duct obstruction is the leading etiology of the prenatally diagnosed CBD.Surgical intervention is recommended for the patients with jaundice,acholic stool,elevated transaminase or cysts obviously increasing in size.     

Management of childhood pancreatic disorders: a multidisciplinary approach

INTRODUCTION: Data on therapeutic endoscopy and radiologic interventions for the management of childhood pancreatic disorders are relatively limited. This study focuses on the multidisciplinary approach to the management of pancreatitis in children. PATIENTS AND METHODS: Children with pancreatic disorders were studied from January 1992 to May 2001. Acute pancreatitis (AP) was diagnosed by clinical evaluation, serum amylase more than three times normal, and morphologic abnormalities of the pancreas on imaging. Children with recurrent abdominal pain, pancreatic calcification or ductal stones on imaging, and pancreatic ductal changes on endoscopic retrograde cholangiopancreatography (ERCP) were diagnosed with chronic pancreatitis (CP). Patients were treated by gastroenterologists, surgeons, and interventional radiologists. Pancreatic exocrine insufficiency was diagnosed in appropriate settings. RESULTS: Fifteen children--6 with AP (posttrauma, 3; gallstone disease, 1; and viral, 1), 7 with CP, and 2 with pancreatic exocrine insufficiency--were diagnosed. Local complications observed in children with AP included pseudocyst in three, and infected acute fluid collection, right-sided pleural effusion, and ascites in one patient each. Complications of AP were managed with percutaneous catheter drainage (n = 3; pseudocyst, 2; infected fluid collection, 1), additional pancreatic duct stenting (n = 2), surgical drainage (n = 1), and octreotide for pleural effusion (n = 1). Signs of CP included abdominal pain (n = 7), obstructive jaundice resulting from lower common bile duct stricture (n = 2), and bleeding from gastroduodenal artery pseudoaneurysm (n = 1). Pancreatic duct stenting relieved pain in one patient, and steel coil embolization arrested bleeding from the pseudoaneurysm. Common bile duct strictures were managed by surgical bypass (n = 2), one of which required preoperative endoscopic bile duct stenting for management of cholangitis. Two other patients with CP required no intervention. CONCLUSION: A multidisciplinary approach of radiologic and endoscopic interventions and surgery are complimentary to each other in achieving successful outcomes of complicated childhood pancreatitis.     

Endoscopic internal biliary drainage in a child with malignant obstructive jaundice caused by neuroblastoma

We describe a 13-year-old girl who underwent insertion of a Flexima biliary stent for obstructive jaundice due to compression of the extrahepatic bile duct by an enlarged lymph node secondary to neuroblastoma. This novel endoscopic internal biliary drainage procedure was safe and effective even for a child, and improved her quality of life. We further review other treatment options available for malignant obstructive jaundice in children.     

Ischaemic cholangiopathy and sickle cell disease

We report a case of a 6-year-old girl of Afro-Caribbean origin, known to have sickle cell disease (SCD), with recurrent history of jaundice and abdominal pain. She was extensively investigated, including endoscopic retrograde cholangiopancreatography (ERCP), which revealed diffuse cholangiopathy of both extrahepatic and intrahepatic bile ducts. A pigtail stent was placed and balloon dilatation was performed for stricture of the extrahepatic duct. Since then, she remains well and asymptomatic. We suggest that cholangiopathy is the consequence of sickling in the end arteries of the biliary arterial tree.     

Endoscopic retrograde cholangiopancreatography in children.

Gastroduodenoscopy and retrograde cholangiopancreatography has been performed on 25 occasions in 20 children aged between 7 and 16. Radiographs of the clinically relevant duct or ducts were achieved in 96% of attempts, with no complications. The diagnostic information proved useful clinically; in particular it provided a precise map if biliary or pancreatic surgery was being contemplated. Several unexpected congenital duct anomalies were found. This and other recent reports, particularly from Germany, indicate that endoscopic retrograde cholangiopancreatography deserves greater application in children, and can also be used in babies.     

Endoscopic retrograde cholangiopancreatography in children

Gastroduodenoscopy and retrograde cholangiopancreatography has been performed on 25 occasions in 20 children aged between 7 and 16. Radiographs of the clinically relevant duct or ducts were achieved in 96% of attempts, with no complications. The diagnostic information proved useful clinically; in particular it provided a precise map if biliary or pancreatic surgery was being contemplated. Several unexpected congenital duct anomalies were found. This and other recent reports, particularly from Germany, indicate that endoscopic retrograde cholangiopancreatography deserves greater application in children, and can also be used in babies.     

Magnetic resonance cholangiopancreatography in primary sclerosing cholangitis in children

BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome of unknown aetiology. Diagnosis is based on clinical and laboratory data in conjunction with imaging of the biliary tree using endoscopic retrograde cholangiopancreatography (ERCP). OBJECTIVE:: To evaluate the clinical usefulness of MR cholangiopancreatography (MRCP) in the diagnosis of PSC in children. MATERIALS AND METHODS: Twenty-one children with clinical and laboratory suspicion of PSC were enrolled. MRCP was performed using a superconductive system with a phased-array coil. Rapid acquisition with relaxation enhancement (RARE) T2-weighted and half-Fourier single-shot turbo-spin-echo (HASTE) sequences were used. The distribution and extent of biliary tree changes were evaluated and classified according to Majoie's classification. A comparison between MRCP and ERCP was performed blind in all cases to evaluate the usefulness of MRI. RESULTS: In 13 cases (62%), MRCP showed abnormalities of the biliary tree which were considered positive for PSC, while in 8 cases there were no signs of PSC. Both MRCP and ERCP correctly identified changes in 13 cases and excluded abnormalities in 5. MRCP had a sensitivity of 81%, specificity of 100%, negative predictive value of 62%, positive predictive value of 100% and an accuracy of 85%. CONCLUSIONS: MRCP can be proposed as the preliminary non-invasive imaging modality for the diagnosis of PSC in children.