Endoscopic retrograde cholangiopancreatography in children with symptomatic pancreaticobiliary maljunction: A retrospective multicenter study

BACKGROUND Endoscopic retrograde cholangiopancreatography(ERCP)has been widely used in pediatric patients with cholangiopancreatic diseases.AIM To evaluate the efficacy,safety,and long-term follow-up results of ERCP in symptomatic pancreaticobiliary maljunction(PBM).METHODS A multicenter,retrospective study was conducted on 75 pediatric patients who were diagnosed with PBM and underwent therapeutic ERCP at three endoscopy centers between January 2008 and March 2019.They were divided into four PBM groups based on the fluoroscopy in ERCP.Their clinical characteristics,specific ERCP procedures,adverse events,and long-term follow-up results were retrospectively reviewed.RESULTS Totally,112 ERCPs were performed on the 75 children with symptomatic PBM.Clinical manifestations included abdominal pain(62/75,82.7%),vomiting(35/75,46.7%),acholic stool(4/75,5.3%),fever(3/75,4.0%),acute pancreatitis(47/75,62.7%),hyperbilirubinemia(13/75,17.3%),and elevated liver enzymes(22/75,29.3%).ERCP interventions included endoscopic sphincterotomy,endoscopic retrograde biliary or pancreatic drainage,stone extraction,etc.Procedure-related complications were observed in 12 patients and included post-ERCP pancreatitis(9/75,12.0%),gastrointestinal bleeding(1/75,1.3%),and infection(2/75,2.7%).During a mean follow-up period of 46 mo(range:2 to 134 mo),ERCP therapy alleviated the biliary obstruction and reduced the incidence of pancreatitis.The overall effective rate of ERCP therapy was 82.4%;seven patients(9.3%)were lost to follow-up,eight(11.8%)re-experienced pancreatitis,and eleven(16.2%)underwent radical surgery,known as prophylactic excision of the extrahepatic bile duct and hepaticojejunostomy.CONCLUSION ERCP is a safe and effective treatment option to relieve biliary or pancreatic obstruction in symptomatic PBM,with the characteristics of minor trauma,fewer complications,and repeatability.

Endoscopic diagnosis of pancreaticobiliary maljunction

Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a junction of the pancreatic and bile ducts located outside the duodenal wall, usually forming a markedly long common channel. As the action of the sphincter of Oddi does not functionally affect the junction in PBM patients, continuous pancreatobiliary reflux occurs, resulting in a high incidence of biliary cancer. PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst) and PBM without biliary dilatation (maximal diameter of the bile duct ≤ 10 mm). The treatment of choice for PBM is prophylactic surgery before malignant changes can take place. Endoscopic retrograde cholangiopancreatography (ERC P) is the most effective examination method for close obs ervation of the pattern of the junction site. When the communication between the pancreatic and bile ducts is maintained, despite contraction of the sphi ncter on ERCP, PBM is diagnosed. In these pat ients, levels of pancreatic enzymes in the bile are gene rally elevated, due to continuous pancreatobiliary reflux via a long common channel. Magnetic resonance cholangiopancreatography and 3D-computed tomography can diagnose PBM, based on findings of an anomalous union between the common bile duct and the pancreatic duct, in addition to a long common channel. Endoscopic ultrasonography and intraductal ultra sonography can demonstrate the junction outside the duodenal wall, and are useful for the diagnosis of asso ciated biliary cancer. Gallbladder wall thickness on ultra so nography can be a screening test for PBM.

Carcinogenesis and chemoprevention of biliary tract cancer in pancreaticobiliary maljunction

Pancreaticobiliary maljunction (PBM) is a high risk factor for biliary tract cancer. In PBM, since the pancreatic duct and bile duct converge outside the duodenal wall beyond the influence of the sphincter of Oddi, pancreatic juice and bile are constantly mixed, producing a variety of harmful substances. Because of this, the biliary mucosa is repeatedly damaged and repaired, which causes an acceleration of cell proliferative activity and multiple gene mutations. Histological changes such as hyperplasia, metaplasia, and dysplasia ultimately result in a high incidence of carcinogenesis. In a nationwide survey by the Japanese Study Group on PBM, coexisting biliary tract cancer was detected in 278 of the 1627 registered cases of PBM (17.1%). Of these cases, in those with dilatation of the extrahepatic bile duct, cancer was often detected not only in the gallbladder but also in the bile ducts. More than 90% of cancer cases without dilatation of the extrahepatic bile duct develop in the gallbladder. Standard treatment for PBM is a cholecystectomy and resection of the extrahepatic bile duct. However, cholecystectomy alone is performed at nearly half of institutions in Japan. Conversely, reports of carcinogenesis in the remnant bile duct or pancreas after diversion surgery are steadily increasing. One of the causes for this is believed to be an accumulation of gene mutations which were present before surgery. Anticancer drugs are ineffective in preventing such carcinogenesis following surgery, thus the postoperative administration of chemopreventive agents may be necessary.

Magnetic resonance cholangiopancreatography study of pancreaticobiliary maljunction and pancreaticobiliary diseases

AIM: To discuss the imaging anatomy about pancreaticobiliary ductal union, occurrence rate of pancreaticobiliary maljunction (PBM) and associated diseases in a Chinese population by using magnetic resonance cholangiopancreatography (MRCP).

Importance of early diagnosis of pancreaticobiliary maljunction without biliary dilatation

AIM:To clarify the strategy for early diagnosis of pancreaticobiliary maljunction(PBM) without biliary dilatation and to pathologically examine gallbladder before cancer develops.METHODS:The anatomy of the union of the pancreatic and bile ducts was assessed by using endoscopic retrograde cholangiopancreatography(ERCP).Patients with a long common channel in which communication between the pancreatic and bile ducts was maintained even during sphincter contraction were diagnosed as having PBM.Of these,patients in which the maximal diameter of the bile duct was less than 10 mm were diagnosed with PBM without biliary dilatation.The process of diagnosing 54 patients with PBM without biliary dilatation was retrospectively investigated.Histopathological analysis of resected gallbladder specimens from 8 patients with PBM without biliary dilatation or cancer was conducted.RESULTS:Thirty-six PBM patients without biliary dilatation were diagnosed with gallbladder cancer after showing clinical symptoms such as abdominal or back pain(n = 16) or jaundice(n = 12).Radical surgery for gallbladder cancer was only possible in 11 patients(31%) and only 4 patients(11%) survived for 5 years.Eight patients were suspected as having PBM without biliary dilatation from the finding of gallbladder wall thickening on ultrasound and the diagnosis was confirmed by ERCP and/or magnetic resonance cholangiopancreatography(MRCP).The median age of these 8 patients was younger by a decade than PBM patients with gallbladder cancer.All 8 patients underwent prophylactic cholecystectomy and bile duct cancer has not occurred.Wall thickness and mucosal height of the 8 resected gallbladders were significantly greater than controls,and hyperplastic changes,hypertrophic muscular layer,subserosal fibrosis,and adenomyomatosis were detected in 7(88%),5(63%),7(88%) and 5(63%) patients,respectively.Ki-67 labeling index was high and K-ras mutation was detected in 3 of 6 patients.CONCLUSION:To detect PBM without biliary dilatation before onset of gallbladder cancer,we should perform MRCP for individuals showing increased gallbladder wall thickness on ultrasound.

Relationship between pancreaticobiliary maljunction and gallbladder carcinoma: a meta-analysis

BACKGROUND: Reports on the relationship between pancreaticobiliary maljunction (PBM) and gallbladder carcinoma (GBC) are conflicting. The frequency of PBM in GBC patients and the clinical features of GBC patients with PBM vary in different studies. DATA SOURCES: English-language articles describing the association between PBM and GBC were searched in the PubMed and Web of Science databases. Nine case-control studies fulfilled the inclusion criteria and addressed the relevant clinical questions of this analysis. Data were extracted independently by two reviewers using a predefined spreadsheet. RESULTS: The incidence of PBM was higher in GBC patients than in controls (10.60% vs 1.76%, OR: 7.41, 95% CI: 5.03 to 10.87, P<0.00001). The proportion of female patients with PBM was 1.96-fold higher than in GBC patients without PBM (80.5% vs 62.9%, OR: 1.96, 95% CI: 1.09 to 3.52, P=0.12). GBC patients with PBM were 10 years younger than those without PBM (SMD: -9.90, 95% CI: -11.70 to -8.10, P<0.00001). And a difference in the incidence of associated gallstone was found between GBC patients with and without PBM (10.8% vs 54.3% OR: 0.09, 95% CI: 0.05 to 0.17, P<0.00001). Among the GBC patients with PBM, associated congenital dilatation of the common bile duct was present with a higher incidence ranging from 52.2% to 85.7%, and 70.0%-85.7% of them belonged to the P-C type of PBM (the main pancreatic duct enters the common bile duct). No substantial heterogeneity was found and no evidence of publication bias was observed.CONCLUSIONS: PBM is a high-risk factor for developing GBC, especially the P-C type of PBM without congenital dilatation of the common bile duct. To prevent GBC, laparoscopic cholecystectomy is highly recommended for PBM patients without congenital dilatation of the common bile duct, especially relatively young female patients without gallstones.

Rare variant of pancreaticobiliary maljunction associated with pancreas divisum in a child diagnosed and treated by endoscopic retrograde cholangiopancreatography: A case report

BACKGROUND Pancreaticobiliary maljunction(PBM) is an uncommon congenital anomaly of the pancreatic and biliary ductal system, defined as a union of the pancreatic and biliary ducts located outside the duodenal wall. According to the Komi classification of PBM, the common bile duct(CBD) directly fuses with the ventral pancreatic duct in all types. Pancreas divisum(PD) occurs when the ventral and dorsal ducts of the embryonic pancreas fail to fuse during the second month of fetal development. The coexistence of PBM and PD is an infrequent condition.Here, we report an unusual variant of PBM associated with PD in a pediatric patient, in whom an anomalous communication existed between the CBD and dorsal pancreatic duct.CASE SUMMARY A boy aged 4 years and 2 mo was hospitalized for abdominal pain with nausea and jaundice for 5 d. Abdominal ultrasound showed cholecystitis with cholestasis in the gallbladder, dilated middle-upper CBD, and a strong echo in the lower CBD, indicating biliary stones. The diagnosis was extrahepatic biliary obstruction caused by biliary stones, which is an indication for endoscopic retrograde cholangiopancreatography(ERCP). ERCP was performed to remove biliary stones. During the ERCP, we found a rare communication between the CBD and dorsal pancreatic duct. After clearing the CBD with a balloon, an 8.5 Fr 4-cm pigtail plastic pancreatic stent was placed in the biliary duct through the major papilla. Six months later, his biliary stent was removed after he had no symptoms and normal laboratory tests. In the following 4-year period, the child grew up normally with no more attacks of abdominal pain.CONCLUSION We consider that ERCP is effective and safe in pediatric patients with PBM combined with PD, and can be the initial therapy to manage such cases,especially when it is combined with aberrant communication between the CBD and dorsal pancreatic duct.

Synchronous gallbladder and pancreatic cancer associated with pancreaticobiliary maljunction

We report the case of a 46-year-old woman who presented with chronic intermittent abdominal pain without jaundice; abdominal ultrasonography showed thickening of the gallbladder wall and dilatation of the bile duct. Endoscopic retrograde cholangiopancreaticography showed pancreatobiliary maljunction with proximal common bile duct dilatation. Pancreatobiliary maljunction was diagnosed. A computed tomography scan of the abdomen showed suspected gallbladder cancer and distal common bile duct obstruction. A pancreatic head mass was incidentally found intraoperative. Radical cholecystectomy with pancreatoduodenectomy was performed. The pathological report showed gallbladder cancer that was synchronous with pancreatic head cancer. In the pancreatobiliary maljunction with pancreatobiliary reflux condition, double primary cancer of the pancreatobiliary system should be awared.

A case of gallbladder carcinoma associated with pancreatobiliary reflux in the absence of a pancreaticobiliary maljunction:A hint for early diagnosis of gallbladder carcinoma

A 62-year-old man with progressive thickening of the gallbladder wall visited our outpatient clinic. The biliary amylase level in the common bile duct was 19900 IU/L and that of the gallbladder was 127000 IU/L, although endoscopic retrograde cholangiopancreatography revealed no pancreaticobiliary maljunction. Histology demonstrated a moderately differentiated adenocarcinoma of the gallbladder. Pancreatobiliary reflux and associated gallbladder carcinoma were confirmed in the present case, in the absence of a pancreaticobiliary maljunction. Earlier detection of the pancreatobiliary reflux and progressive thickening of the gallbladder wall might have led to an earlier resection of the gallbladder and improved this patient＇s poor prognosis.

Spontaneous necrosis of solid gallbladder adenocarcinoma accompanied with pancreaticobiliary maljunction

A 71-year-old Japanese man with acute cholecystitis and an incarcerated gallbladder （GB） stone was admitted. Plain ultrasonography （US） incidentally detected a mass-like lesion in the fundus. Doppler US revealed that this elevated lesion had no blood flow. Computed tomography showed a relatively low-density mass, measuring 5 cm x 4 cm in diameter, with no positive enhancement. Magnetic resonance imaging showed a mass in the fundus with a slightly low intensity on Tl-weighted images and a slightly high intensity on T2-weighted images. We were agonized in making the qualitative diagnosis of mass-like lesions of the fundus, such as a benign tumor, cancer, or debris. We performed laparoscopic cholecystectomy, because the incarcerated GB stone clearly caused acute cholecystitis. Intra-operative cholangiography clearly revealed pancreaticobiliary maljunction. Amylase levels in the common bile duct and gallbladder were quite high. The elevated lesion in the fundus dearly showed severe necrosis. Although this necrotic nodule included non-viable adenocarcinoma cells, viable cancer cell nests were located in the muscularis propria and subcutaneous layer. Histopathological examination confirmed a solid adenocarcinoma. Thus, we diagnosed it as a gallbladder cancer, based on histopathological analysis of the resected specimen. We therefore undertook radical surgery, including wedge resection of the liver, radical dissection of regional lymph nodes, and resection of the extrahepatic bile duct. Histopathological findings revealed no cancer, hyperplasia or dysplasia in the additionally resected specimens. The patient was finally staged as T2, N0, H0, P0, M（-）, stage Ⅱ. We present the first case of spontaneous necrosis of solid gallbladder adenocarcinoma, with a review of previous studies.

Carcinoma of the papilla of Vater following treatment of pancreaticobiliary maljunction

Pancreaticobiliary maljunction(PBM)is frequently associated with biliary cancer due to reflux of pancreatic enzymes into the choledochus,and even after surgery to correct the PBM such patients still have a risk of residual bile duct cancer.Here,we report the case of a 59-year-old female with carcinoma of the papilla of Vater which developed 2.5 years after choledochoduodenostomy for PBM.During the postoperative follow-up period,computed tomography obtained 2 years after the first operation demonstrated a tumor in the distal end of the choledochus,although she did not have jaundice and laboratory tests showed no abnormalities caused by the previous operation.As a result,carcinoma of the papilla of Vater was diagnosed at an early stage,followed by surgical cure.For early detection of periampullary cancer in patients undergoing surgery for PBM,careful long-term follow-up is needed.

Liver, biliary and pancreatic injuries in pancreaticobiliary maljunction model in cats

BACKGROUND： Pancreaticobiliary maljunction is a high risk factor of pancreatitis and biliary tract cancer. How this maljunction affects the liver remains obscure. This study aimed to examine the effects of pancreaticobiliary maljunction on the liver, pancreas and gallbladder in a cat model.METHODS： A model of choledocho-pancreatic side-to-side ductal anastomosis was created in ten cats. Before the procedure,a small piece of tissue from the liver, pancreas and gallbladder was collected as a control. The common channel formation was checked by cholecystography. The livers, pancreases and gallbladders of these cats were harvested for histological examination. The expression of proliferating cell nuclear antigen in the gallbladder was examined with immunohistochemistry. RESULTS： Seven of the 10 cats survived for 6 months after surgery. The color of the liver was darker in the PBM model than the control specimen, with nodules on the surface. Histological examination showed ballooning changes and inflammatory infiltrations and the histopathological score increased significantly（P〈0.05）. Also, mitochondria swelling and lipid droplet in cytoplasm were observed under an electron microscope. The pancreas also appeared darker in the PBM model than the control specimen and dilated pancreatic ducts were found in three cats. Histopathological examination revealed vascular proliferation and inflammatory infiltration with numerous neutrophils. Gallbladder epithelial cells were featured by expanded endoplasmic reticulum, increased intercellular space and cellular nucleus deformation. The positive cells ofproliferating cell nuclear antigen were increased significantly（P〈0.05）. CONCLUSION： The present study demonstrated that pancreaticobiliary maljunction can lead to the injuries of the liver, pancreas and gallbladder.

Application of imaging techniques in pancreaticobiliary maljunction

Imaging techniques are useful tools in the diagnosis and treatment of pancreaticobiliary maljunction(PBM).PBM is a precancerous lesion often relative to the disease of the pancreas and biliary tract,for example,cholecystolithiasis,protein plugs,and pancreatitis.For patients with PBM,early diagnosis and timely treatment are highly important,which is largely dependent on imaging techniques.The continuous development of imaging techniques,including endoscopic retrograde cholangiopancreatography,magnetic resonance cholangiopancreatography,computed tomography,ultrasound,and intraoperative cholangiography,has provided appropriate diagnostic and therapeutic tools for PBM.Imaging techniques,including non-invasive and invasive,have distinct advantages and disadvantages.The purpose of this paper is to review the application of various imaging techniques in the diagnosis and treatment of PBM.

胰胆管合流异常相关性胆囊癌研究进展

胰胆管合流异常(PBM)是一种先天性胰胆管畸形,常会引起反复发作的胆管炎、胰腺炎。越来越多的证据表明PBM是一种癌前病变,在胆囊癌(GBC)的发生和发展中起着重要的作用。由PBM所致GBC称为PBM相关性GBC。因此,PBM的早期识别及诊治对于降低GBC的风险至关重要。本文对PBM的概况和诊疗进展以及PBM相关性GBC的临床特征、发生机制及诊疗进展加以综述,旨在显示早期诊治PBM的临床价值,从而降低PBM相关性GBC发生率。

内镜下CCEST微创干预治疗胰胆管合流异常患者35例分析

背景胰胆管合流异常(pancreaticobiliary maljunction,PBM)患者的共同通道(common channel,CC)过长导致Oddi括约肌的作用不直接影响到胰胆连接处,继而胰液和胆汁相互反流,造成胆道或胰腺的各种病理改变.探索内镜下微创缩短胰胆管CC通道在PBM中的应用及其机制变得尤为重要,为患者提供又一个治疗的技术选择.目的探讨经内镜下十二指肠乳头括约肌共同通道切开术(endoscopic common channel sphinctertomy,CCEST)微创干预治疗PBM患者的临床效果及安全性.方法观察分析CCEST内镜下微创干预治疗的35例PBM患者,其中BP型19例、PB型16例,复杂型0例.35例患者均行经内镜逆行胰胆管造影(endoscopic retrograde cholangiopancreatography,ERCP)下CCEST并分析内镜下微创干预治疗对PBM患者的影响作用及并发症等情况.结果35例PBM患者中,男性12例,女性23例,平均年龄为(46±26.38)岁(3岁-89岁).所有患者均给予内镜下CCEST,以缩短共同通道.35例PBM患者中合并胆、胰管结石患者18例(51.43%),14例患者首次ERCP取石成功,成功率为78%(14/18).ERCP微创干预治疗术后1 wk,35例患者中血清生化、C-反应蛋白(C-reactive protein,CRP)指标及胆汁淀粉酶较术前均明显下降(P<0.05).5例患者出现轻度ERCP术后胰腺炎,经对症治疗后均未出现术后出血、消化道穿孔和重症胰腺炎等严重并发症.经过对35例患者(22.05±10.67)mo的随访,其中1例术后确诊胆管癌而行外科手术;有4例患者因结石复发再次行ERCP治疗,也取得满意疗效.治疗总有效率为88.6%(31/35).结论PBM起病较隐匿,ERCP可协助明确PBM诊断.CCEST通过缩短胆胰管汇合部共同通道,早期改善PBM患者胆胰管引流,是内镜微创治疗PBM患者的有效安全方法.

十二指肠镜治疗儿童无明显胆管扩张型胰胆管合流异常的疗效及预后分析

目的探讨十二指肠镜治疗无明显胆管扩张型胰胆管合流异常(pancreaticobiliary maljunction without obvious biliary dilatation,PBM-nonOBD)患儿的手术疗效及预后不良相关因素。方法回顾性分析复旦大学附属儿科医院自2020年1月至2022年12月收治的内镜治疗PBM-nonOBD患儿的临床资料(包括人口学资料、临床症状、实验室检查及影像学资料),并对患儿进行随访。采用单因素分析及多因素Logistic回归分析十二指肠镜治疗PBM-nonOBD患儿不良预后的危险因素,并绘制受试者工作特征(receiver operating characteristic,ROC)曲线分析相关危险因素的预测价值。结果本研究共纳入44例患儿,随访时间(19.7±8.6)个月,治愈率为54.5%(24/44),其中治疗有效24例(为治疗有效组),治疗无效20例(为治疗无效组)。术后不良事件以十二指肠镜逆行性胆胰管造影术后胰腺炎最常见(7/44,15.9%),其中27.3%(12/44)的患儿最终需接受根治术,15.9%(7/44)的患儿需接受再次内镜治疗。治疗有效组胰胆管合流异常(pancreaticobiliary maljunction,PBM)分型以B型和D型为主,占比分别为41.7%(10/24)和37.5%(9/24)。单因素分析结果显示,年龄偏小、胰胆共同管直径较长、胆总管最宽直径较宽是PBM-nonOBD患儿内镜手术后预后不良的相关因素(P<0.05);多因素Logistic回归分析发现,年龄偏小(OR=1.645,95%CI:1.645~2.309)及胰胆共同管直径较长(OR=0.720,95%CI:0.720~0.968)是PBM-nonOBD患儿预后不良的独立危险因素(P<0.05),曲线下面积(area under the ROC curve,AUC)分别为0.838(95%CI:0.719~0.958)和0.731(95%CI:0.567~0.894),最佳截断值分别为4.9岁和8.8 mm。结论十二指肠镜手术创伤小,不会导致严重并发症,可有效缓解部分PBM-nonOBD患儿症状;年龄偏小和胰胆共同管长度较长可能与十二指肠镜治疗PBM-nonOBD预后不良相关。

内镜逆行胰胆管造影在胰胆管合流异常诊治中的应用

胰胆管合流异常(PBM)是一种胆胰管发育异常的先天性畸形,长期发展会导致胆胰系统肿瘤的发生,因此早发现早诊治是一个重点。内镜逆行胰胆管造影术(ERCP)作为PBM的诊断金标准,不仅能缓解急性临床症状,而且可以作为后期外科手术的桥接手段。

Metachronous cancer of gallbladder and pancreas with pancreatobiliary maljunction

Pancreaticobiliary maljunction is a congenital anomaly in which the junction between the pancreatic duct and the common bile duct is located outside the sphincter of Oddi.It is well known that pancreaticobiliary maljunction is frequently associated with carcinoma of the biliary tract.We report a case of metachronous cancer of the gallbladder and pancreas associated with pancreaticobiliary maljunction and cystic dilatation of common bile duct in a 68-year-old Tunisian woman who underwent a cholecystectomy for acute cholecystitis.The pancreatic tumor was an adenosquamous carcinoma.Pancreaticobiliary maljunction allows for pancreatobiliary or biliopancreatic reflux which may induce biliary tract carcinoma.Few cases of multifocal cancer associated with this anomaly have been reported.The association with pancreatic carcinoma remains rare.Close attention should be given to both the biliary tract system and pancreas during the long-term follow-up of patients with pancreaticobiliary maljunction,especially after they have undergone a choledochojejunostomy.

Pancreaticobiliary reflux as a high-risk factor for biliary malignancy:Clinical features and diagnostic advancements

Pancreaticobiliary junction is composed of complex structure with which biliary duct and pancreatic duct assemble and go out into the ampulla of Vater during duodenum wall surrounding the sphincter of Oddi.Although the sphincter of Oddi functionally prevents the reflux of pancreatic juice,pancreaticobiliary reflux(PBR) occurs when function of the sphincter of Oddi halt.The anatomically abnormal junction is termed pancreaticobiliary maljunction(PBM) and is characterized by pancreatic and bile ducts joining outside of the duodenal wall.PBM is an important anatomical finding because many studies have revealed that biliary malignancies are related due to the carcinogenetic effect of the pancreatic back flow on the biliary mucosa.On the other hand,several studies have been published on the reflux of pancreatic juice into the bile duct without morphological PBM,and the correlation of such cases with biliary diseases,especially biliary malignancies,is drawing considerable attention.Although it has long been possible to diagnose PBM by various imaging modalities,PBR without PBM has remained difficult to assess.Therefore,the pathological features of PBR without PBM have not been yet fully elucidated.Lately,a new method of diagnosing PBR without PBM has appeared,and the features of PBR without PBM should soon be better understood.

经内镜逆行胰胆管造影术治疗儿童胰胆管合流异常的临床研究

目的探讨经内镜逆行胰胆管造影术(endoscopic retrograde cholangiopancreatography,ERCP)治疗胰胆管合流异常(pancreaticobiliary maljunction,PBM)的临床效果及预后。方法回顾性分析2014年1月至2020年12月解放军总医院第七医学中心收治的60例PBM患儿的临床资料,根据治疗方法分为ERCP组(n=30)和外科手术组(n=30)。分析患儿的临床资料、辅助检查和预后情况。统计学方法采用Wilcoxon秩和检验、Mann-Whitney检验、χ^(2)检验或Fisher确切概率法。结果ERCP依据日本胰胆功能障碍研究小组(Japanese study group on pancreaticobiliary maljunction,JSGPM)-PBM分型标准,以A型(10例,33.3%)、B型(10例,33.3%)为主,而外科手术组以C型(11例,36.7%)、D型(14例,46.7%)为主,差异有统计学意义(P<0.05)。两组患者的手术成功率均为100%。ERCP组与外科手术组的恢复进食时间[5.0(2.6,6.4)与9.0(8.0,12.0)d,Z=3.489,P<0.001],术后住院时长[8.0(5.3,13.5)与17.0(14.0,20.8)d,Z=6.408,P<0.001],住院总费用[2.8(2.2,4.1)与7.3(5.4,9.8)万元,Z=5.562,P<0.001],ERCP组均低于外科手术组。术后两组患儿的肝胆功能实验室检查指标呈下降趋势。随访6个月,ERCP组的术后疾病缓解率为86.2%(25/29),外科手术组的术后疾病缓解率为63.0%(17/27);随访12个月,ERCP组的术后疾病缓解率为96.0%(24/25),外科手术组的术后疾病缓解率为88.5%(23/26),两组比较差异均无统计学意义(P值均>0.05)。结论对JSGPM-PBM分型中的A型及B型且病情急性变化的PBM患儿进行ERCP治疗,可有效缓解症状,改善预后。