先天性胆总管囊肿诊治34例

先天性胆总管囊肿是儿童期常见胆道疾病,其发病机制仍在探索中。我院于1999年~2005年共收治34例先天性胆总管囊肿患儿,现报道如下。     

先天性胆总管囊肿诊治52例报道

目的探讨先天性胆总管囊肿的诊断与治疗方法。方法对我院2002年1月～2007年1月收治的52例先天性胆总管囊肿患儿进行回顾性分析。结果52例均经B超、CT、MRCP等检查诊断为先天性胆总管囊肿,并行手术治疗,手术方式为胆总管囊肿切除、肝门空肠Roux-Y型吻合术。全组无死亡病例,无重大并发症,预后良好。结论B超是诊断先天性胆总管囊肿的首选方法,胆总管囊肿切除、肝门空肠Roux-Y型吻合术是治疗先天性胆总管囊肿的首选方法。     

婴儿先天性胆总管囊肿16例诊治分析

胆总管囊肿是一种先天性疾病,可在各年龄组出现临床症状。临床上在3岁以后出现症状居多眼1演。我院近10年收住的先天性胆总管囊肿患儿65例,其中<12个月的婴儿16例,占24.6%。本文对此16例患儿作回顾性分析。1临床资料与结果1.1一般情况本组16例中,男6例,女10例。年龄5d～11个月。手术时年龄最小者42d,有10例<6个月。病史中单纯黄疸者7例,黄疸、腹块6例,黄疸、腹块并发热1例,仅有腹部肿块者1例,黄疸伴发热1例。14例肝功能指标有异常。6例入院前曾误诊为新生儿肝炎进行过治疗。所有病例均经B超检查或腹部CT检查确诊。16例入院后早期均采取非…     

产前诊断先天性胆总管囊肿一例报告及文献综述

随着产前影像学技术的提高和广泛应用．许多先天性畸形可以在产前明确诊断，本文报告1例产前诊断先天性胆总管囊肿患儿出生后早期的病程变化．对其进行文献综述总结。     

婴幼儿先天性胆总管囊肿临床分析

目的探讨婴幼儿先天性胆总管囊肿的临床特点。方法对24例婴幼儿先天性胆总管囊肿患儿的临床表现、手术前后肝功能测定、手术情况及肝脏活组织检查等进行回顾性分析,并与同期14例儿童先天性胆总管囊肿患儿比较。结果婴幼儿先天性胆总管囊肿临床表现以黄疸、腹部包块、白陶土大便为主,大多数患儿术前肝功能损害严重。24例胆总管扩张均为囊性扩张,其中21例囊肿远端明显狭窄、闭锁;21例行一期囊肿切除、肝总管一空肠Roux—Y吻合,囊肿外引流术,3例行二期囊肿切除、胆肠吻合术。肝脏活检12例存在肝硬化改变,9例存在肝硬化前期改变,3例肝脏组织正常。结论婴幼儿先天性胆总管囊肿有各自的临床及病理特点,胆道梗阻、胆汁淤积是肝脏损害的病理基础,应早期诊断,早期手术治疗,胆道梗阻解除后肝脏损害可恢复。     

巨大先天性胆总管囊肿1例

患儿，女，3岁。因腹部不适，逐渐膨大伴腹痛、黄疸二年入院。门诊以“腹部肿物性质待查”入院。家庭史无类似及遗传病患。     

先天性胆总管囊肿63例报告

目的总结先天性胆总管囊肿的诊断与治疗经验。方法回顾1994年～2006年我院收治的63例患儿的病例资料,对其诊断、手术方式及手术后近、远期并发症进行分析。结果本组入院前后均经B超、CT、MRCP等检查,诊断符合率分别为88.9%、80.0%、90.0%。其中46例行囊肿切除 肝总管空肠Roux-en-Y吻合术,6例行囊肿切除 肝总管十二指肠吻合术,6例行囊肿内引流术。术后近期并发症:伤口出血5例,胆汁漏7例,胆系感染2例,吻合口梗阻1例。远期并发症:返流性胆管炎8例.肠梗阻4例,胆系结石2例。结论B超检查是诊断先天性胆总管囊肿的首选方法,囊肿切除 肝总管空肠Roux-en-Y吻合术是一种较好的治疗方法。     

先天性胆总管囊肿43例诊治分析

目的　探讨先天性胆总管囊肿 (CCC)的诊断和治疗方法。方法　对 1998年 1月～ 2 0 0 3年 1月我院收治 43例 14岁以下CCC患儿性别、年龄、诊断依据、囊肿类型、手术时机和术式进行分析。结果　CCC发病年龄在 2岁以下者占 72 % ;男女比为 1:3 ;本组入院前后均经B超、CT、磁共振胆管成像 (MRCP)等检查 ,诊断符合率分别为 83 .7%、80 %、78.9% ,共 40例患儿行手术治疗 ,术式为囊肿切除 肝总管空肠Roux -en -Y吻合术 ,无 1例发生死亡或严重并发症。结论　B超是首选的检查手段 ,囊肿切除、胆道重建是理想的手术方法 ,胆肠吻合口的直径大小与是否发生吻合口狭窄有直接关系     

先天性胆总管囊肿九例综合报道

先天性胆总管囊肿又称胆总管扩张症,临床较少见,好发于女性,任何年龄均可出现症状,现将9例报告如下。临床资料一、一般资料9例中男3例,女6例,就诊年龄18天～14岁;病程最长14年。二、症状与体征发热5例,腹痛6例,黄疸7例,有白陶土大便史者4例,腹部肿块6例,肝肿大4例,压痛不明显,且有黄疸、腹痛,腹块典型三联征者2例。三、实验室及辅助检查白细胞<10×10~9/L6例,>10×10(?)/L 3例;尿胆红质阳性5例,尿     

先天性胆总管囊肿自发性穿孔24例诊疗分析

目的回顾性分析24例小儿先天性胆总管囊肿自发性穿孔患儿的临床特点及诊治经过,探索快速诊断、合理治疗的方法。方法对24例小儿先天性胆总管囊肿自发性穿孔的临床表现、诊断方法、治疗过程及预后进行回顾性分析。结果24例均一期行腹腔冲洗胆总管囊肿外引流术,3个月后行二期囊肿切除、肝总管空肠R0ux-Y吻合术,预后良好。结论胆总管囊肿自发性穿孔是小儿胆总管囊肿较为严重的并发症。胰胆合流是胆总管囊肿穿孔的主要原因,早期诊断,及时合理治疗,可明显降低并发症,术前腹腔穿刺对诊断有很大帮助,术中造影可明确病因。     

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.     

30例巨大胆总管囊肿的临床诊断和治疗分析

目的 探讨巨大胆总管囊肿在诊断及治疗方面的经验. 方法 对30例巨大胆总管囊肿患儿的临床资料进行回顾性分析.结果 本组30例患儿中,有4例手术后出血,1例术后出现胆瘘,经保守治疗成功.其余患儿住院期间恢复顺利.随访6个月至8年,患儿均无腹痛、发热、黄疸等异常情况. 结论 胆总管囊肿切除＋肝总管空肠Roux-Y吻合术治疗巨大先天性胆总管囊肿效果良好.     

Postnatal rupture of an antenatally diagnosed choledochal cyst: first case report

A 7-mo-old girl with an asymptomatic antenatally diagnosed choledochal cyst was referred to the regional paediatric surgical centre for advice on management. Following further radiological investigation and discussion with the supra-regional centre, a date for elective surgery was arranged. However, in the interim, she was admitted as an emergency following rupture of the cyst. She made an uneventful recovery following urgent laparotomy. Conclusion: We recommend prompt paediatric surgical referral following antenatal diagnosis of a presumed choledochal cyst. Delay in surgery may permit expansion and rupture of the cyst, even after a period of asymptomatic stability.     

Pancreatitis complicated with dilated choledochal remnant after congenital choledochal cyst excision

We describe here three cases of pancreatitis after congenital choledochal cyst excision. In these three cases, the choledochal remnant in the pancreas head was markedly dilated, probably because of an incomplete resection of the cyst at the primary operation, and an increase in intraluminal pressure of the pancreatic duct caused by a dynamic obstruction by a protein plug or a pancreatic calculus. Complete cyst excision, including the choledochal wall in the pancreas, is therefore strongly recomended.     

Traumatic rupture of a choledochal cyst masking as a duodenal hematoma

Choledochal cysts are rare, congenital malformations of the intra and/or extrahepatic biliary tree. We describe a case in which a patient was transferred to our hospital with a reported duodenal hematoma. The patient ultimately required exploration when his condition deteriorated. Laparotomy resulted in the discovery and successful treatment of a ruptured type IV-A choledochal cyst.     

Antenatal diagnosis of choledochal cyst: the first four cases

Four infants in whom choledochal cyst was diagnosed by maternal ultrasound examinations are reviewed. The infants, from different institutions, represent a spectrum with regard to clinical presentation and subsequent course. They show that choledochal cyst can develop at different stages of gestation and is often not associated with biliary obstruction or biliary atresia. Definitive surgery can be safely performed in the neonatal period and is the treatment of choice. Follow-up data indicate that despite marked differences in the initial severity of their hepatic parenchymal disease, all four infants have done well in their early years, with essentially normal liver function. These unique patients provide insights into the possible etiology and pathophysiology of choledochal cyst and suggest guidlines for future evaluation and management of such infants.     

Expression of matrix metalloproteinase 1 and 2 in congenital choledochal cyst and its clinical significance

Background  The pathogenesis of congenital choledochal cyst (CCC) is still not well elucidated. This study was conducted to detect the expression of matrix metalloproteinase-1 and 2 (MMP-1 and MMP-2) in CCC. The roles of MMP-1 and MMP-2 in CCC formation were also discussed. Methods  Specimens of CCC and gallbladders were taken from 53 patients with CCC, including 21 boys and 32 girls aged from 4 months to 17 years (mean: 6.5 years old). Twenty-eight normal gallbladders served as controls. Immunohistochemical method was used to detect the expressions of MMP-1 and MMP-2. Image analysis was made to quantitatively analyze the protein expression. Results  The expressions of MMP-1 and MMP-2 were located in epithelial cells, matrix cells, and inflammatory cells of the CCC wall. Various expressions were also found in the mucosa membrane cells of the CCC gallbladder. The expressions of MMP-1 and MMP-2 were low in the control group. Quantitatively, the expressions of MMP-1 and MMP-2 in the CCC wall were markedly higher than in the CCC gallbladder and the control group (P<0.01). There was no significant difference between the CCC gallbladder and the control group (P>0.05). Conclusions  The expression of MMP-1 and MMP-2 in the CCC wall is higher than in the CCC gallbladder and controls. The over-expression of MMP-1 and MMP-2 may be involved in the pathogenesis of CCC.     

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。     

Rapid enlargement of a choledochal cyst: antenatal diagnosis and delayed primary excision

A case of choledochal cyst (CC) antenatally diagnosed at 29 weeks' gestation is reported. Rapid enlargement of the cyst soon after delivery resulted in complete gastric outlet obstruction (GOO). The lesion was treated by external drainage as a temporary maneuver, with delayed cyst excision and hepaticoduodenostomy at the hepatic hilum performed at 81 days of age. Surgical treatment of CC in early infancy has been reported to be safe and effective. However, delayed primary excision would be an alternative procedure, especially in rare cases showing rapid enlargement resulting in GOO, since this choice has the potential advantage of allowing weight gain and improved nutritional status without risking interim complications due to the drainage procedure. Accepted: 24 March 1997