腹腔镜在婴幼儿胆管发育不良与胆汁淤积症诊治中的应用

目的 探讨腹腔镜在胆管发育不良与胆汁淤积症诊断和治疗中的应用。方法 回顾性分析近年18例婴幼儿胆管发育不良与胆汁淤积症的临床资料。结果 所有病例均经腹腔镜行胆道造影明确诊断，其中胆管发育不良10例，胆汁淤积8例。10例胆管发育不良中，7例中转开腹行扩大肝门肝肠吻合术，3例胆道外引流、置管冲洗术；8例胆汁淤积中，2例单纯胆道减压、冲洗，6例胆囊造瘘，术中术后分别灌洗。结论 腹腔镜探查、胆道造影是诊断胆管发育不良与胆汁淤积症的简便、准确、易行的方法，胆管发育不良可行扩大肝门肝肠吻合术，或胆道减压、胆管灌洗术。胆汁淤积症的患儿，可行胆囊造瘘、胆道灌洗，并可免除开腹手术。     

小儿胆道急症腹腔镜下经皮胆囊造瘘13例临床分析

目的探讨小儿胆道急症的临床特点,总结腹腔镜下经皮胆囊造瘘在小儿胆道急症中的应用价值。方法回顾性分析2006年6月至2011年6月作者收治的13例胆道急症患儿临床资料,其中自发性胆道穿孔5例,胆总管囊肿并发急性胆管炎5例,结石或蛔虫梗阻并发急性胆管炎3例。患儿均在腹腔镜下行经皮胆囊造瘘。结果手术均成功实施,术后临床症状缓解,无胆道出血和胆瘘发生。自发性胆道穿孔5例,随访3个月至1年,胆道无异常发现,1例有胆总管扩张改变,目前仍在随访中,均未行二期手术。胆总管囊肿并发急性胆囊炎5例,4例在胆囊造瘘术1～3个月后行胆总管囊肿根治术,1例胆总管扩张回缩明显,目前仍在随访中。结石或蛔虫致胆道梗阻3例,术后13超复查,2例未见明显结石或蛔虫影像,胆道未见明显扩张。1例胆囊内仍有较大结石存在,于胆囊造瘘术后3个月行胆囊切除术。结论小儿胆道急症多在先天性胆道疾病的基础上并发。腹腔镜下经皮胆囊造瘘术治疗小儿胆道急症,可有效缓解胆道梗阻,术后根据情况行二期胆道处理,部分患儿甚至可以免除二期手术,是治疗小儿胆道急症的有效方法。     

腹腔镜在治疗胃肠外营养相关性胆汁淤积中的应用

目的 探讨腹腔镜胆道冲洗术治疗胃肠外营养相关性胆汁淤积(PNAC)的效果.方法 回顾性分析2007年11月至2011年3月在我院进行腹腔镜胆道冲洗治疗术的PNAC患儿临床资料,分析患儿围术期情况及手术前后胆汁淤积改善状况.结果 本组共13例患儿进行腹腔镜检查,1例术中诊断为胆道闭锁,12例诊断为PNAC的患儿均留置造瘘管,每天生理盐水冲洗,其中6例2周拔出造瘘管,4例4周拔管,1例6周拔管,1例8周拔管.经随访4～44个月,12例患儿均恢复良好.12例患儿初步诊断PNAC时直接胆红素(DBil)水平为(64.9±13.1)μmol/L,总胆汁酸(TBA)水平为(56.4±11.0)μmol/L,内科保守治疗2周后分别为(80.7±19.0)μmol/L和(64.0±16.2)μmol/L,DBil较诊断时升高(P<0.05),TBA无明显变化(P>0.05);术后2周与术前比较,DBil和TBA均明显降低,分别为(44.3±21.5)μmol/L和(35.0±20.1)μmol/L,差异有统计学意义(P<0.001).结论 腹腔镜辅助胆道冲洗治疗PNAC具有操作简单、直观、微创的优点,经胆道冲洗2周以上,治疗PNAC的疗效肯定.     

腹腔镜微创技术在婴儿阻塞性黄疸诊治中的应用

目的探讨腹腔镜微创技术在婴儿阻塞性黄疸诊治中的应用。方法回顾性分析我院2001年5月-2004年7月利用腹腔镜微创技术对37例婴儿阻塞性黄疸进行早期诊断治疗的临床资料。结果37例均利用腹腔镜技术行胆道探查、胆道造影明确诊断，其中婴儿肝炎综合征16例、胆道闭锁15例、胆总管囊肿6例，根据情况分别行胆道冲洗、胆囊造瘘、胆道重建等手术及肝活检术，无1例手术死亡。结论腹腔镜微创技术用于阻塞性黄疸的早期鉴别诊断及治疗有较高的临床实用价值，对胆道闭锁患儿争取了早期手术的时机，及时引流胆汁，避免肝脏进行性损害而可明显提高疗效；对婴儿肝炎综合征患儿，可镜下造影明确诊断，行胆囊置管及胆道冲洗，有利于疏通胆道，引流胆汁，减轻胆汁淤积对肝脏的进一步损害，可明显缩短疗程及提高疗效。     

经腹腔镜手术治疗Ⅰ、Ⅱ型胆道闭锁

目的 对经腹腔镜手术治疗Ⅰ型及Ⅱ型胆道闭锁的效果进行探讨.方法 自2003年3月至2007年7月,共收治患Ⅰ型及Ⅱ型胆道闭锁并胆管囊性扩张的患儿10例,其中Ⅰ型8例,Ⅱ型2例;男4例,女6例;年龄23～160 d,平均53.8 d.患儿均有黄疸、陶土样便等症状;伴有总胆红素、转氨酶明显升高;术前经B超等影像学检查证实有肝门部囊性扩张的胆管存在,平均直径1.5 cm(1.0～1.8 cm);10例经术中证实为Ⅰ、Ⅱ型胆道闭锁伴有肝外胆道囊性扩张.本组10例患儿行经腹腔镜扩张的胆管底部切除胆管空肠Roux-en-Y吻合术.术后平均随访26个月(4～51个月).结果 本组10例患儿行经腹腔镜手术全部成功,无中转开腹.手术时间平均为3.0 h(2.4～3.2 h),术中出血量5～10ml.术后胃肠平均通气时间为18 h(16～28 h),进食时间平均20 h(16～30 h);平均术后3 d(2～4 d)排黄色大便;腹腔引流放置时间平均58 h(48～72 h),平均术后10 d(7～16 d)黄疸减轻,6例患儿术后14 d总胆红素直接胆红素降至正常水平,3例患儿3个月降至正常水平,1例手术年龄5.5个月患儿虽获良好胆汁引流,胆红素水平有所降低,但因肝硬化严重,后来仍维持高水平,胆汁引流后肝功能改善不佳,术后28 d死于肝功能衰竭.转氨酶术后不同程度的下降.术后平均住院时间6.8 d(5～9 d).术后随访4～51个月,1例患儿于术后4周发生胆管炎,经抗炎后痊愈.其余患儿肝功能各项指标均正常,无胆管炎、吻合口狭窄、粘连肠梗阻等术后并发症.结论 经腹腔镜囊性扩张的胆管底部切除胆管空肠吻合术治疗Ⅰ、Ⅱ型胆道闭锁是一种安全有效、可靠的方法.     

产前诊断胆道闭锁影像学研究

目的：胆道闭锁早期诊断困难,而产前诊断更是极少发现。本文对产前诊断的胆道闭锁影像学特点进行探讨。方法回顾我院2010年至2012年收治的产前诊断9例产前诊断胆道畸形患儿,入院手术年龄24 d至2岁。全部患儿行腹腔镜胆道造影,4例诊断胆道闭锁,5例诊断先天性胆管扩张症,胆道闭锁患儿中2例接受腹腔镜下肝管空肠ROUX-Y吻合术,2例接受开腹肝门空肠吻合术。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4例患儿产前超声未见胆囊或胆囊显示不清。产前超声发现肝门部囊肿的3例患儿,囊肿小且均无明显增大,张力较高,呈规则圆形。产前诊断发现肝门囊肿的3例患儿术中证实为胆总管远端闭锁（Ⅰ型胆道闭锁）,未发现胆囊也未发现囊肿的1例患儿证实为Ⅲ型胆道闭锁。生后全部胆道闭锁患儿出现黄疸,最早出现在生后第2天,但都未出现陶土样便。全部胆道闭锁患儿囊肿大小形态无明显变化。全部患儿查ALT、AST、rGGT、直接胆红素和总胆红素进行性升高。2例接受腹腔镜下肝管空肠ROUX-Y吻合术,2例接受开腹肝门空肠吻合术。全部4例患儿术后恢复好。结论产前超声检查可以确定胆道闭锁,如果发现肝门部囊肿的胎儿,应定期接受超声检查,如果囊肿在孕期变化不明显,应怀疑囊肿型胆道闭锁。如产前超声未发现胆囊结构,则应怀疑为Ⅲ型胆道闭锁。生后应密切观察、超声、生化、黄疸情况。如果黄疸进行性加重可及早进行腹腔镜胆道造影及手术治疗。     

腹腔镜治疗小儿肝囊肿的临床研究

目的 分析小儿肝囊肿腹腔镜治疗的可行性及效果.方法 回顾性分析自2010年12月至2013年12月收治肝囊肿7例患儿的临床资料.其中,男3例,女4例;年龄12 d～3岁7个月;全部为单发囊肿.全部患儿行腹腔镜手术(经脐部单切口腹腔镜5例),及术中胆道造影.1例患儿行腹腔囊肿切除术,4例患儿行腹腔镜囊肿开窗术,2例患儿行腹腔镜囊肿胆囊吻合术.结果 无术中中转开腹.手术时间90～150 min,平均(102.9±23.6)min.出血量2～5 ml.无死亡病例,无创面出血,腹腔感染,腹腔积液等并发症.患儿术后3～7 d出院.术后每3个月门诊复查超声及生化指标,全部患儿肝功能及胆红素正常.囊肿切除及开窗术患儿无囊肿复发,胆囊吻合术患儿囊肿明显减小,无不适症状;无胆道感染、腹腔感染积液等并发症.结论 小儿肝囊肿应用腹腔镜及经脐部单切口腹腔镜治疗是安全有效的,如果囊肿与肝内胆管相通可考虑行囊肿胆囊吻合术.     

I型胆管闭锁的外科治疗

目的探讨I型胆管闭锁的外科治疗以及临床意义。方法2003—2011年,作者收治胆道闭锁患儿98例,其中伴有胆总管闭锁的I型胆管闭锁患儿5例,男3例,女2例,年龄62～127d。行胆囊-空肠吻合术2例,肝管-空肠吻合术3例。结果2例胆囊-空肠吻合术患儿退黄时间分别为术后10d和术后17d（退黄标准为总胆红素〈20μmol／L）;3例肝管-空肠吻合术患儿退黄时间分别为术后20d、1个月和2个月（退黄标准同前）。术后随访时间1—5年;2例胆囊-空肠吻合术患儿术后未见黄疸反复。3例肝管-空肠胆道重建手术患儿中,1例术后未见黄疸反复,1例术后5个月出现黄疸,诊断为胆管炎,经抗炎治疗后好转;1例反复发作胆管炎最终选择肝移植。结论术中胆道造影是诊断胆道闭锁的金标准;如果术中造影证实为胆总管闭锁,且胆囊与左、右肝管通畅,主张采取胆囊-空肠吻合术。肝管-空肠吻合容易造成吻合口狭窄;过度解剖肝门对于术后恢复不利。     

不同年龄胆道闭锁患儿手术效果分析

目的 分析胆道闭锁手术年龄与术后早期效果的关系以及较大年龄（＞90 d）患儿的Kasai手术指征.方法 2004-2010年复旦大学附属儿科医院收治胆道闭锁患儿452例,均经术中胆道造影确诊.手术采用标准的Kasai术,术后常规使用激素,对不同年龄组患儿术前肝功能、B超等资料及术后胆红素下降情况进行分析,总胆红素水平低于20 mmol/L定为黄疸完全消退.结果 将所有患儿根据年龄分为三组,手术年龄≤60 d者146例,手术年龄在60～90 d者222例,＞90 d者84例（90～10Od者33例,100～110 d 26例,110～120d 10例,120～ 130 d 8例,＞130 d 7例）.术前各年龄组总胆红素、直接胆红素、谷丙转氨酶无显著差异.术后2周,60 d以内组,胆红素下降水平最低（P＜0.05）,＜45 d患儿胆红素下降水平并未更加显著.术后3个月随访率为61.3％,各年龄组总胆红素水平无显著差异（F=0.132,P=0.970）.术后6个月随访率37.4％,90 d以上组总胆红素（58.09±58.55） mmol/L,90 d以内组总胆红素水平（27.67±30.60） mmol/L（P=0.226）.＞90d患儿,每间隔10d分成一组,各组间术后早期胆红素下降水平无差异（F=1.115,P=0.355）.＞90 d手术患儿两年自体肝生存率为36.1％.90 d以上患儿延误手术原因：39.2％因家长未重视,51.6％因误诊婴儿肝炎耽误治疗.结论 胆道闭锁患儿＞90 d并非手术绝对禁忌,多数患儿可取得较好的早期黄疸消退,部分患儿术后6个月可以有较好的肝功能恢复.     

胆道发育不良患儿的临床病理特点回顾

目的 探讨胆道发育不良患儿的临床表现、病理特点、诊断、治疗及预后,提高对该病的认识.方法 回顾性分析天津市儿童医院外科2010年6月至2016年11月收治的5例胆道发育不良患儿的发病情况、治疗过程,并通过复习国内外文献对胆道发育不良的疾病特点进行总结.结果 5例胆道发育不良患儿均因生后不久出现皮肤、巩膜黄染,内科治疗病情无改善入外科治疗;肝功能检查见胆红素水平升高,以直接胆红素升高为主,伴肝酶不同程度增高;超声检查示胆囊干瘪未充盈或胆囊腔狭小,胆总管显示不清.5例患儿行手术探查,术中造影显示肝内、外胆道通畅,管腔纤细;病理检查见部分汇管区中小叶间胆管缺失,小叶间胆管/汇管区＜0.5,诊断为胆道发育不良,予留置胆囊引流管,术后抗炎、补液、保肝及对症治疗.经2个月～6年门诊随访,5例患儿均存活至今,其中3例黄疸清除,1例皮肤仍黄染并伴有瘙痒,1例未退黄.结论 术前检查提示梗阻性黄疸患儿,术中造影提示胆道纤细,结合病理检查证实小叶间胆管/汇管区比例＜0.5,可以明确胆道发育不良的诊断.     

Surgical jaundice in infants: other than biliary atresia.

After biliary atresia, the lesions responsible for surgical jaundice in the infant are perforation of the common bile duct, choledochal cyst, bile plug syndrome, and miscellaneous congenital lesions in descending order of frequency. Perforation of the common bile duct commonly presents with an insidious onset of bilious ascites and is best treated by simple peritoneal drainage. Choledochal cyst usually presents later in childhood but presents in infancy if obstruction of the biliary tree is complete or near complete. Excision is the treatment of choice. Any condition leading to alteration in bile composition may cause bile plug syndrome. Spontaneous resolution is the rule: occasionally, intraoperative irrigation is necessary. Most miscellaneous lesions lend themselves to operative correction.     

Neonatal hepatitis syndrome with paucity of interlobular bile ducts in cystic fibrosis.

A male infant presenting with neonatal hepatitis syndrome, characterized by conjugated hyperbilirubinemia and very mild liver function test abnormalities, at 2 weeks of age was found to have no excretion of radioisotope into the intestinal tract on hepatobiliary scan. Liver biopsy revealed severe interlobular bile duct paucity. Other features of Alagille's syndrome were not present; other conditions frequently associated with interlobular bile duct paucity were also excluded. Subsequently, the infant was found to have cystic fibrosis. Cystic fibrosis is thus another disease that may be associated with paucity of interlobular bile ducts presenting as neonatal hepatitis syndrome, and this represents a different pathogenesis of cholestatic jaundice in neonates with cystic fibrosis besides those previously recognized.     

Foregut atresias and bile duct anomalies: rare,infrequent or common?!

The association of foregut atresias and bile duct anomalies is reportedly rare. We encountered five referrals within 2 years where the secondary diagnosis was missed at operation. Four patients initially presented on antenatal scans as a foregut atresia whereas the fifth presented at nine years with abdominal pain due to a choledochal cyst. The biliary anomalies (cholecysto-hepatic duct, liver cyst and choledochal cysts) in the first four presented as postoperative jaundice during infancy whereas the fifth patient developed subacute intestinal obstruction due to congenital duodenal stenosis at fifteen years. In the patients with duodenal atresia neither did the preoperative X ray reveal any distal bowel gas nor did the subsequent intraoperative cholangiograms reveal bifid common bile duct or pancreato-biliary malunion. Atresias were corrected by primary repair (duodenoduodenostomy for congenital duodenal obstruction in four patients and disconnection/ligation of tracheo-oesophageal fistula with oesophageal anastomosis in one patient). The biliary anomalies were corrected by excision of the abnormal bile ducts (choledochal cyst/liver cyst/cholecystectomy) with Roux en Y hepaticojejunostomy. All patients are asymptomatic and liver function and biliary dilatation has normalised. The association of foregut atresias and bile duct anomalies is not as rare as previously reported. Antenatal ultrasound suggesting either a foregut or a biliary anomaly should alert one to the association. Full radiological and/or imaging investigation may be indicated prior to corrective surgery of the primary anomaly.     

Bile acids in serum and bile of infants with cholestatic syndromes

The concentration of individual bile acids in serum was measured in 18 neonates and infants with various cholestatic conditions (extrahepatic biliary atresia, neonatal hepatitis syndrome, chronic intrahepatic cholestasis and posthemolytic cholestasis). The cholate/chenodeoxycholate ratio in serum was smaller than one in all patients with neonatal hepatitis syndrome or extrahepatic biliary atresia, cholestatic conditions which were accompanied by signs of liver cell injury. It was greater than one in the patients with chronic intrahepatic cholestasis. Administration of cholestyramine to patients with patent extrahepatic bile ducts decreased the total concentration bile acids in serum and elevated the cholate/chenodeoxycholate ratio. Thus, cholestyramine administration may be of diagnostic value for evaluation of bile duct patency in cholestasis of infancy. Differences between the bile acid pattern in serum and bile were observed. Thus, the cholate/chenodeoxycholate ratio was always higher in bile than in serum. 3beta-hydroxy-5-cholenoic acid found in serum was not detectable in bile. This finding suggests that impairment of biliary excretion rather than increased hepatic synthesis is responsible for elevation of this monohydroxy bile acid in serum.     

Bile duct atresia following extended right hepatectomy because of a tumor

A 19 month old male infant with a mesenchymal hamartoma of the liver underwent an extended right hepatectomy. Serum bilirubin gradually rose until 3 months after the surgery, and obstructive jaundice and acholic stools were manifested at 6 months. Percutaneous transhepatic cholangiodrainage was performed. Cholangiography showed dilation of the intrahepatic bile duct of the residual lateral segment and complete obstruction of the extrahepatic bile duct. A second operation for reconstruction of the biliary tract was performed 10 months after the first surgery. No aspect of an extrahepatic biliary tract was found. Histological inspection of a surgical specimen of remnant tissue revealed only cicatricial connective tissue without any biliary structures. The clinical course has been uneventful for 18 months since the second surgery. The cause of bile duct atresia in this case is strongly suggested to be an ischemic change due to devascularization of the extrahepatic biliary tract following hepatic resection because of a tumor. To prevent this kind of complication, hepaticoenterostomy should be performed close to the cut surface of the liver.     

Proliferation to Paucity: Evolution of Bile Duct Abnormalities in a Case of Alagille Syndrome

Alagille syndrome is an autosomal dominant disorder characterized by abnormalities in multiple organ systems, including the liver, and is caused by mutations in JAG1. Chronic cholestasis secondary to paucity of interlobular bile ducts is traditionally both a clinical and a pathologic hallmark of this disease at diagnosis. We describe the biliary changes on serial liver biopsies in a patient who presented with jaundice and extrahepatic stigmata of Alagille syndrome. Her initial specimens at 6 and 10 months of age demonstrated interlobular bile duct proliferation and cholestasis, suggestive of distal biliary obstruction. A specimen at 2 years of age showed near-total absence of interlobular bile ducts, with the classic histologic appearance of bile duct paucity. We present this case to underscore the potential pitfalls in interpreting cholestatic liver morphology in the absence of clinical information. The progression of bile duct abnormalities is discussed in the context of the role postulated for JAG1 in postnatal liver growth and development.     

Expression of osteopontin correlates with portal biliary proliferation and fibrosis in biliary atresia

The acquired or perinatal form of biliary atresia is a Th1 fibro-inflammatory disease affecting both the extrahepatic and intrahepatic bile ducts. Osteopontin (OPN) is a Th1 cytokine implicated in several fibro-inflammatory and autoimmune diseases. We examined the expression of OPN in acquired biliary atresia in comparison to normal liver and several pediatric cholestatic liver diseases. We also assessed OPN expression by cultured human bile duct epithelial cells. We found that liver OPN mRNA and protein expression were significantly increased in biliary atresia versus normal and other cholestatic diseases. OPN expression in biliary atresia was localized to epithelium of proliferating biliary structures (ductules and/or ducts) and bile plugs contained therein. No portal biliary OPN expression could be demonstrated in normal liver, syndromic biliary atresia, biliary obstruction not due to biliary atresia, and idiopathic neonatal hepatitis. OPN expression by human bile duct epithelial cells in culture was responsive to IL-2 and TNF-alpha. Our results demonstrate an up-regulation of OPN expression by interlobular biliary epithelium in biliary atresia, which correlates with biliary proliferation and portal fibrosis. These findings suggest a role for OPN in the pathogenesis of biliary atresia.     

先天性胆总管囊肿患儿胆汁成分分析及致石性研究

目的：探讨先天性胆总管囊肿患儿胆汁成分变化与结石形成关系。方法：对10例胆总管囊肿及9例非肝胆疾病手术患儿胆汁成分进行定量分析,包括总胆汁酸、4种初级胆汁酸、磷脂、胆汁蛋白、胆固醇及总脂含量等,并进行胆汁细菌培养。结果：①囊肿患儿胆汁中胆汁酸、磷酸、胆固醇、总蛋白及总脂浓度明显异常,以胆汁酸减少为显著。其肝胆汁、胆囊胆汁、囊肿胆汁中总胆汁酸浓度均明显降低,总脂浓度下降。易发生胆结石的主要原因之一;②囊肿患儿三种胆汁中四种初级结合型胆汁酸的含量及G／T,CA／CDCA均降低,胆汁酸组分比的变化,也是易产生结石的原因。囊肿中初级结合型胆汁酸的浓度相对降低,次级胆汁酸相对增加,可能对囊肿恶变发生的影响;③囊肿患儿肝细胞功能受损是导致CCC患儿胆汁成分发生改变的主要原因。结论：CCC患儿胆汁中主要固体成分存在明显异常,以胆汁酸减少及胆汁酸的组分比改变为著,是CCC患儿易发生结石的主要原因之一,导致CCC患儿胆汁成分改变的核心问题是肝功能受损所致。     

Surgery for biliary tract problems in children

Jaundice in the neonate is common and when not associated with symptoms or signs of systemic illness may at first be regarded with some complacency by both mother and health professional alike. When the cholestatic nature of the jaundice becomes apparent, a cause must be identified, if possible within 2 weeks of onset. Biliary atresia, choledochal cyst, inspissated bile syndrome and spontaneous perforation of the bile duct are the most frequent surgical causes. An investigation protocol includes stool observation, ultrasound scanning, biochemical liver function tests, a screen for infective and metabolic causes, a radioisotope excretion scan (hydroxyiminodiacetic acid HIDA scan) and, if necessary, operative cholangiogram and liver biopsy. Surgical management, if performed timeously, may prevent progression of parenchymal disease and in many cases can be curative. Delayed diagnosis leads to inevitable progression of liver damage with a poor long-term outlook. Cholelithiasis is being seen more frequently both in the infant and older child. Surgical intervention is required for symptomatic disease and for stones in the common bile duct. Tumours of the bile ducts are rare but are most likely to be malignant and should be referred to specialist centres for management.