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。     

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.     

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.     

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.     

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.     

经内镜逆行胰胆管造影诊治小儿胆胰疾病

目的 探讨利用经内镜逆行胰胆管造影技术诊断和治疗小儿胆胰疾病的疗效.方法 收集新华医院2004年至2010年小儿经内镜逆行胰胆管造影的诊断及治疗病例共19例.患儿年龄14个月至16岁,其中男9例,女10例.疾病包括胆总管囊肿,肝内外胆管结石,胆胰管合流异常,胰管结石,慢性胰腺炎,whipple's术后胆肠吻合口狭窄等....     

Endoscopic biliary drainage management for children with serious cholangitis caused by congenital biliary dilatation

Congenital biliary dilatation (CBD) is usually associated with complications such as recurrent cholangitis, manifested as abdominal pain, vomiting, and jaundice. If cholangitis cannot be controlled by conservative treatment, a good therapeutic effect can be obtained through percutaneous biliary drainage or open T-tube drainage. We aimed to evaluate our experiences in biliary drainage through endoscopic retrograde cholangiopancreatography in children with cholangitis caused by CBD. From January 2014 to December 2017, 167 children with CBD were treated in our hospital. 17 patients (10.18%) with serious cholangitis caused by CBD underwent ERCP. There were 4 males and 13 females with an age range of 10–120 months (average 56.4 months). Placement of a biliary stent was attempted for biliary drainage through endoscopic retrograde cholangiopancreatography. Of the 17 patients studied, 13 children had jaundice and 15 had elevated aminotransferases. ERCP showed CBD in all patients and a common biliopancreatic duct in 12 of 17 patients (70.6%). Five patients underwent nasobiliary drainage and 12 patients underwent biliary drainage through double pigtail tubes. All patients achieved successful biliary drainage. Postoperative pancreatitis occurred in one patient. Biochemical indicators decreased significantly in 12 patients (70.6%) on the second postoperative day. The average length of hospital stay after surgery was 4.5 (range 3–7) days. No major complications related to ERCP were observed and all children had a good prognosis so far. Endoscopic biliary drainage is a safe, simple, and reliable technique. It can be used to resolve CBD-associated cholangitis, evaluate the biliary tract and pancreatobiliary duct junction, and guide pediatric surgeons to choose the right time and the correct procedure for CBD.     

Unusual causes of extrahepatic biliary obstruction in children: a case series with review of literature

This paper highlights the etiology, diagnosis, management and outcome in nine unusual cases of extrahepatic biliary obstruction in children. Extrahepatic biliary atresia and choledochal cyst constituted 127 out of 136 (93%) cases of all pediatric surgical biliary disorders managed between March 2000 and February 2007 at the reporting centre. However, nine children (aged 1.5–15 years) presented with uncommon causes like (1) idiopathic benign non-traumatic inflammatory stricture (n = 3), (2) idiopathic fibrosing chronic pancreatitis (n = 2), (3) post-cholecystectomy type 4 benign biliary stricture (n = 1), (4) post-acute pancreatitis pseudo-cyst of pancreas (n = 1), (5) non-Hodgkin's lymphoma (NHL) with extramural common bile duct compression and gall bladder perforation (n = 1), and (6) Langerhan cell histiocytosis (LCH, n = 1). The clinical features and the diagnostic work up of each group are discussed. A preoperative endoscopic/percutaneous biliary drainage was required in four children because of cholangitis at presentation. A biliary-enteric anastomosis was performed for all seven children in groups (1)–(4). The patients with NHL and LCH were referred for chemotherapy after establishing tissue diagnosis at laparotomy. With a follow-up period of 3 months to 7 years, seven children (with the exception of patients with NHL and LCH) are currently anicteric. This paper draws attention to some infrequently discussed causes of extrahepatic biliary obstruction in children. The management entails a carefully planned combination of endoscopic interventions, interventional radiology and surgery. The outcome in benign cases is usually satisfactory.     

青少年胰胆管畸形ERCP表现特点及临床意义

目的：探讨青少年胰胆管畸形的ＥＲＣＰ表现特点及其与胰胆系病变的关系。方法：应用Ｘ线电视录像和多轴位透视、摄片等技术研究２９例异常胰胆管连接（ＡＰＢＤＵ），８例胰腺分裂（ＰＤ）和３１例胆囊管低位汇入（ＬＯＣＤ）患儿的ＥＲＣＰ表现特点及其伴发疾病情况，并与５０例正常胰胆管连接者（对照组）比较。结果：①ＡＰＢＤＵ组胆管炎、胆管囊肿、慢性胰腺炎的发生率明显高于对照组（Ｐ＜０．０５）。ＡＰＢＤＵ组合并慢性胰腺炎的患儿以胰管增粗、迂曲等改变为主，而对照组主要表现为胰头变硬或增大等。②ＰＤ组慢性胰腺炎的发生率显著增高（Ｐ＜０．０１）。③ＬＯＣＤ组慢性胰腺炎和胆系结石的发生率显著增高。结论：ＥＲＣＰ是诊断胰胆管畸形的有效手段，胰胆管畸形与多种胰胆系病变存在病因学联系。     

腹腔镜在治疗小儿胰腺疾病中的应用

目的 探讨腹腔镜手术治疗小儿胰腺疾病的临床效果.方法 2005年10月至2010年12月应用腹腔镜手术治疗小儿胰腺疾病9例,其中胰腺假性囊肿1例,行囊肿内引流术;局灶性胰岛细胞增生症1例,行单纯胰腺肿瘤切除;弥漫性胰岛细胞增生症2例,胰腺假乳头状瘤3例,行保留脾脏的胰体尾切除术;胰管扩张2例,行扩张胰管空肠侧侧Roux...     

MR cholangiopancreatography in children: feasibility,safety, and initial experience

BACKGROUND: The indications for MR cholangiopancreatography (MRCP) in children, and its safety and findings, might differ from those in adults and are not well described. OBJECTIVE: To investigate the safety, feasibility, and accuracy of MRCP in children. MATERIALS AND METHODS: We reviewed all prospective MRCP reports, noting the indication, the use of secretin, endoscopic retrograde cholangiopancreatography (ERCP) findings, and patient outcomes. Two readers reviewed each MRCP study by consensus to rate duct visualization and compare pancreatic duct sizes before and after secretin administration (paired t-test). The likelihood of a normal versus an abnormal MRCP study was compared by gender, pancreatitis as the primary indication, secretin use, and whether ERCP was performed (Fisher's exact test), as well as age (t-test). RESULTS: A total of 85 MRCP studies were performed in children (mean age 10.3 years), most commonly for evaluation of pancreatitis (n=47, 55%); 41 (48%) used secretin and 39 (46%) used a negative oral contrast agent. Of the 85 studies, 72 (85%) had excellent image quality and 43 were normal. ERCP was performed after 16 of the 85 MRCP studies (19%); the diagnoses were concordant with those of MRCP in 13 (81%). There were 42 abnormal MRCP studies, and these were more likely to be in girls (P=0.03) and in children who had undergone ERCP (P<0.01). Secretin and the negative oral contrast agent were well-tolerated. Secretin improved duct visualization (P<0.001). CONCLUSION: MRCP safely and accurately depicted pancreaticobiliary anatomy in children. The use of secretin improved visualization of the pancreatic duct.     

Multidisciplinary management of surgical disorders of the pancreas in childhood

OBJECTIVES: To describe the frequency and range of pancreatic disorders in children requiring surgical intervention and to highlight the importance of multidisciplinary management. METHODS: An audit of all children under 17 years of age referred with surgical disorders of the pancreas or pancreatitis to a regional pediatric gastroenterology unit in the United Kingdom during a 10-year period. A retrospective chart review of clinical features, pathology and outcome was undertaken. RESULTS: Surgical intervention was required for the following pancreatic disorders: persistent hyperinsulinemic hypoglycemia of infancy (n = 4), pancreatic tumors (n = 5), pancreaticobiliary malunion (n = 12), pancreatic trauma (n = 6) and pancreatitis (n = 10). The indications for surgery in acute pancreatitis were a persistent pseudocyst (n = 1) and treatment of an underlying cause of pancreatitis (n = 4); in chronic pancreatitis, surgery was used to treat symptomatic pancreatic duct strictures (n = 4). One child died of a progressive lymphoma but all others who underwent surgery are alive and well. All 33 children with acute pancreatitis, including four with pancreatic necrosis, survived. CONCLUSIONS: Surgery for pancreatic disorders in children is rarely required but may be necessary a) for definitive management of primary pancreatic pathology, b) to treat sequelae of acute or chronic pancreatitis and c) to treat an underlying cause of pancreatitis. There is a broad spectrum of potential pathologies. These patients are best managed by a multidisciplinary team approach.     

Hepatobiliary and pancreatic complications of ascariasis in children: a study of seven cases

OBJECTIVES: This study presents seven cases of severe hepatobiliary and pancreatic complications of ascariasis in children. The authors describe the clinical, laboratory, and imaging findings, as well as the patients' clinical evolution. METHODS: These cases were studied within a period of approximately 1 year and included children younger than 11 years (mean age, 4.4 years). The authors reviewed their medical history and evaluated the results of their main diagnostic examinations. RESULTS: All of the patients had vomiting, abdominal pain, pallor, and abdominal distension at presentation. Passage of Ascaris lumbricoides in stool occurred in five cases, emesis with worms in three, fever in three, and hepatomegaly in two. Five patients had pancreatitis, of which two were necrohemorrhagic and one had pseudocyst of the pancreas. In three patients, A. lumbricoides was present in the pancreatic duct. Two patients had hepatic abscess (28.6%), and one of them also had cholangitis. One of the patients with pancreatitis also had signs of cholecystitis at presentation. CONCLUSIONS: Ultrasonography was the imaging diagnostic method of choice and demonstrated the presence of A. lumbricoides in the biliary and the pancreatic ducts, as well as signs of pancreatitis, cholecystitis, and hepatic abscess. Endoscopic retrograde cholangiopancreatography, used to confirm the diagnosis, was a fundamental procedure in the treatment, allowing the removal of worms from the biliary duct in four of seven patients.     

儿童Ⅳa型胆管扩张症的外科治疗探讨

目的探讨儿童Ⅳa型胆管扩张症临床特点及手术治疗。方法选取2015~2017年复旦大学附属儿科医院普外科收治的胆管扩张症患儿且临床资料较完整者,共135例,按Todani分型,Ⅰ型96例,Ⅱ型9例,Ⅲ型1例,Ⅳ型29例。按照纳入标准分组:Ⅳa型29例为A组;Ⅰc型肝内外胆管扩张且互相连续患儿36例设为B组,Ⅰa、Ⅰb型仅肝外扩张患儿56例设为C组,合计121例。回顾性分析患儿术前临床症状、影像学诊断、术中发现、手术过程及预后。结果各组中女性均多于男性,组间差异无统计学意义。以磁共振胰胆管成像(MRCP)为标准,B型超声肝内胆管扩张检出率为30.7%(20/65)。所有B型超声报告均提示第一肝门前囊性占位或胆总管扩张。术前伴随胰腺炎共25例(20.7%)。二次入院患儿共10例,3例经消炎治疗后好转,7例再次手术。术后随访中可见肝内胆管扩张持续存在患儿4例,其中2例无不适症状,予保守治疗,2例患儿行二次手术治疗,手术1个月后扩张胆管消失。结论术中应积极探查肝门处狭窄环及近端扩张胆管,并适当做肝门整形。儿童Ⅳa型胆管扩张症大多预后较好,妥善处理肝外胆道后,肝内胆管扩张术后大多能改善。胰腺炎与囊肿分型无关,术前伴发水肿型胰腺炎患儿多可一期手术。     

Hepatic duct stone associated with chlamydia sepsis: a rare condition in childhood

Stone formation in the biliary system is a rare condition in infants. A few cases of bile stones in the biliary tree have been reported with underlying predisposing factors, such as sepsis and antibiotic usage. This article describes a surgically treated 16-week-old infant with recurrent cholangitis who had a bile stone in the hepatic duct after chlamydia sepsis. Accepted: 4 February 1997     

儿童胆源性胰腺炎的临床特点

目的探讨儿童胆源性胰腺炎的临床特点。方法回顾分析2011年1月至2015年7月儿童胆源性胰腺炎10例的临床资料。结果 10例患儿中,男2例、女8例,平均年龄(6.1±4.9)岁,平均住院天数(8.4±3.8)d。胆囊结石4例,胆汁淤积4例,胆管结石2例,胆总管囊肿2例,胰腺分裂症1例。10例患儿均以腹痛为主要临床表现。均有血淀粉酶升高,最高值106~922 U/L;血脂肪酶升高6例,为22~2 000 U/L;血丙氨酸氨基转移酶异常6例,低钾血症2例,低血糖1例,酸碱失衡4例。腹部B超提示4例有胰腺炎改变,CT则提示均有胰腺炎改变。予禁食、补液、维持水电解质平衡、奥美拉唑抑制胃酸、奥曲肽抑制胰酶分泌等治疗。9例患儿内科保守治疗好转出院,1例转外科手术治疗。结论儿童胆源性胰腺炎发病率低,临床症状不典型,小年龄患儿胰胆管系统畸形较多见,内科保守治疗效果相对好。     

儿童Ⅲ型胰腺外伤六例治疗体会

目的 探讨儿童Ⅲ型胰腺外伤的治疗方法.方法 回顾性分析2010年1月至2015年6月收治的6例Ⅲ型胰腺外伤患儿的临床资料.本组均为男孩,年龄6岁4个月～11岁5个月.入院时均有不同程度腹痛,伴呕吐2例,伴发热1例;血淀粉酶(695.83±264.07) U/L,尿淀粉酶(4 163.67±5 546.53)U/L.CT检查,均提示胰管损伤可能,其中3例分别合并脾挫伤、左肾上腺挫伤加肺挫伤、左肾上腺挫伤.患儿均先给予生长抑素、禁食、静脉营养等保守治疗,但症状控制不满意.待血流动力学稳定后行逆行胰胆管造影(ERCP)加内镜鼻胰管引流术或胰管支架引流术.结果 6例ERCP造影提示1例胰头部、4例胰体部、1例体尾部主胰管损伤.行13次ERCP引流,2例经7次ERCP引流治愈;另4例6次ERCP引流症状控制不佳,3例穿刺引流,1例开放引流后治愈.6例随访胰管均修复、恢复通畅;2例超声检查示胰腺有局部细小囊性改变,无临床症状,淀粉酶恢复正常.结论 单独ERCP引流治疗儿童Ⅲ型胰腺外伤疗效不满意,有待更多临床总结;结合经皮穿刺引流和开放引流效果确切.儿童Ⅲ型胰腺外伤使用药物治疗加确切引流就能取得良好预后,应尽量避免复杂手术.     

Autoimmune pancreatitis in an 11-year-old boy

We report a case of histopathologically proven autoimmune pancreatitis in an 11-year-old boy. Abdominal US and MRI showed a focal swelling of the pancreatic head, the latter also showing delayed contrast enhancement. There was diffuse irregular pancreatic duct narrowing, compression of the intrapancreatic common bile duct, and mild proximal biliary dilatation on MR cholangiopancreatography. Laboratory results revealed normal serum IgG and subclass 4 with negative autoimmune antibodies, and slightly elevated carbohydrate antigen 19-9. This highlights the differentiation of autoimmune pancreatitis from pancreatic head cancer and, to a lesser extent, other forms of pancreatitis in children.     

儿童胰腺肿瘤精准手术切除策略探讨(附15例报告)

目的 探讨影响小儿胰腺肿瘤手术切除方式的因素及精准手术切除的可行性.方法 回顾性分析2002年10月至2013年7月收治的儿童胰腺肿瘤15例的临床资料.15例中,女10例,男5例;年龄4个月～14岁,平均8岁4个月.肿瘤位于胰头部9例,胰体尾部6例,伴肝转移1例,合并肝、肠系膜病变1例.结果 本组均接受手术治疗.肿瘤位于胰腺头部9例行保留幽门、胰十二指肠切除术7例,行肿瘤切除术2例,此2例肿瘤均来源于胰头钩突部;肿瘤位于胰腺体尾部6例,均行胰体/尾+脾切除术.病理检查报告:胰腺囊实性假乳头状瘤9例,胰腺母细胞瘤3例,浆液性囊腺瘤1例,胰岛细胞癌1例,促纤维增生性小圆细胞瘤1例.15例患儿术后恢复顺利,随访6个月～12年,促纤维增生性小圆细胞瘤1例患儿死亡,余者均存活.结论 手术切除肿瘤是治疗儿童胰腺肿瘤得重要手段,多数患儿仅通过手术切除肿瘤可长期存活.手术原则是尽可能保持胃肠道连续性,精准手术,尽可能多的保留正常的胰腺组织.影响决定小儿胰腺肿瘤术式的主要因素是肿瘤部位、周围重要组织是否受累肿瘤是否累及胆管、主胰管及脾血管.CT、MRCP属非侵袭性检查方法,可多平面分析,且无需注射造影剂,可对胆管及胰管状况肿瘤进行术前评估,为胰腺肿瘤确定精准手术方案提供依据.