未经证实:在一些先天性胆道扩张(CBD)患者中,据报道,胆汁性肝硬化从新生儿期到婴儿期迅速发展。我们在此报告一个婴儿病例的CBD,在诊断时显示严重的胆汁性肝硬化,通过腹腔镜手术治疗。
UNASSIGNED:一名16个月大的女孩在我们县的一个偏远岛屿接受了肝功能障碍和胆管炎的保守治疗。在先前医院的影像学检查中发现巨大的扩张的胆总管后,她被转移到我们医院。对比增强计算机断层扫描显示胆总管扩张(最大直径:5cm),因此建议CBD。然而,她入院时的实验室数据显示营养状况差和严重的肝功能障碍(Alb,2.5mg/dl;AST,79IU/L;ALT,43IU/L;γ-GTP,491毫克/分升;D-bil,0.3mg/dl;CHE,90IU/L;NH3,123μg/dl)。我们最初通过胆囊进行腹腔镜探查和胆汁引流,注意到严重的肝纤维化类似于终末期肝硬化。在胆囊中放置引流管后,进行了胆管造影。胆管造影显示Todani型IVaCBD伴胰胆管畸形。无法确认造影剂流入十二指肠。患者在确定性手术前接受了7周的肝脏支持治疗和营养支持。随着肝脏合成能力的提高(Alb,4.0mg/dl;AST,82IU/L;ALT,78IU/L;γ-GTP,157毫克/分升;D-bil,0.2mg/dl;CHE,232IU/L;NH3,75μg/dl),我们在腹腔镜下进行了肝外胆管切除术和肝空肠吻合术。腹腔镜手术与肝活检一起成功进行。组织病理学,肝脏标本显示慢性肝炎和纤维化(F3A2)。胆道闪烁显像显示术后第15天胆汁排泄良好。术后过程顺利,患者在手术后第23天出院。6个月后穿刺肝活检显示慢性肝炎和纤维化的轻度改善(F2-3A1)。在门诊定期随访患者。
未经证实:严重的肝纤维化被怀疑是出生后CBD的持续胆汁淤积。具有严重肝纤维化的CBD可以通过具有肝保护治疗和免疫营养支持的两阶段手术避免肝移植。
UNASSIGNED: In some patients with congenital biliary dilation (CBD), biliary cirrhosis has been reported to rapidly progress from the neonatal period to the infantile period. We herein report an infantile
case of CBD that showed severe biliary cirrhosis at the diagnosis, which was treated by laparoscopic surgery.
UNASSIGNED: A 16-month-old girl underwent conservative therapy for liver dysfunction and cholangitis on a remote island of our prefecture. She was transferred to our hospital after the detection of a huge dilated common bile duct on imaging at the previous hospital. Contrast-enhanced computed tomography showed a dilated common bile duct (maximum diameter: 5 cm), thus suggesting CBD. However, her laboratory data on admission showed a poor nutritional status and severe liver dysfunction (Alb, 2.5 mg/dl; AST, 79 IU/L; ALT, 43 IU/L; γ-GTP, 491 mg/dl; D-bil, 0.3 mg/dl; CHE, 90 IU/L; NH3, 123 μg/dl). We initially performed laparoscopic exploration and bile drainage via the gallbladder, noting severe hepatic fibrosis resembling end-stage liver cirrhosis. After placing a drainage tube in the gallbladder, cholangiography was performed. Cholangiography showed Todani type IVa CBD with pancreaticobiliary maljunction. Contrast agent flowing into the duodenum could not be confirmed. The patient received liver-supporting therapy and nutritional support for 7 weeks before definitive surgery. Following the improvement of the hepatic synthetic capacity (Alb, 4.0 mg/dl; AST, 82 IU/L; ALT, 78 IU/L; γ-GTP, 157 mg/dl; D-bil, 0.2 mg/dl; CHE, 232 IU/L; NH3, 75 μg/dl), we performed extrahepatic bile duct excision and hepaticojejunostomy laparoscopically. Laparoscopic surgery was successfully performed along with liver biopsy. Histopathologically, the liver specimen showed chronic hepatitis and fibrosis (F3A2). Biliary scintigraphy showed good bile excretion at postoperative day 15. The postoperative course uneventful, and the patient was discharged on the 23rd day after surgery. A needle liver biopsy six months later showed mild improvement of chronic hepatitis and fibrosis (F2-3A1). The patient was regularly followed at the outpatient clinic.
UNASSIGNED: Severe liver fibrosis was suspected to be continuous cholestasis of CBD after birth. CBD with severe liver fibrosis may avoid liver transplantation by two-stage surgery with hepatoprotection therapy and immunonutritional support.