Dumortier J, Vaillant E, Boillot O, Poncet G, Henry L, Scoazec J-Y, Partensky C, Valette P-J, Paliard P, Ponchon T
Fédération des Spécialités Digestives, Hôpital Edouard Herriot, Lyon, France.
Endoscopy. 2003 May;35(5):446-50. doi: 10.1055/s-2003-38779.
While hemorrhagic complications of portal cavernoma are frequent, compression of the bile ducts by portal cavernoma is uncommon and treatment is still a matter for debate. We report here six new cases in order to describe: (a) the clinical, biological, and morphological features of this condition, and (b) the long-term results of a combined endoscopic and surgical treatment. The median age of patients at the time of diagnosis was 36.5 years. The circumstances of diagnosis were acute cholangitis (n=3), asymptomatic biological cholestasis (n=1), pruritus, jaundice and asthenia (n=1) and jaundice alone (n=1). Portal cavernoma and bile duct dilatation were confirmed by abdominal ultrasonography with pulsed color doppler and endoscopic retrograde cholangiography (ERC). Gallstones were found in four patients. Following stenting of the bile duct, there was a good outcome in two patients. In four patients, after failure of prolonged endoscopic treatment, second-line surgical portal-systemic shunting allowed removal of the biliary stent, and no recurrence of disease. In conclusion, biliary involvement in portal cavernoma is now a well-recognized entity, and our results suggest that combined endoscopic and surgical treatment could be required.
虽然门静脉海绵样变的出血并发症很常见，但门静脉海绵样变压迫胆管并不常见，治疗方法仍存在争议。我们在此报告6例新病例，以描述：（a）这种情况的临床、生物学和形态学特征，以及（b）内镜和手术联合治疗的长期结果。诊断时患者的中位年龄为36.5岁。诊断情况为急性胆管炎（n = 3）、无症状性生化胆汁淤积（n = 1）、瘙痒、黄疸和乏力（n = 1）以及仅黄疸（n = 1）。通过腹部超声检查（脉冲彩色多普勒）和内镜逆行胆管造影（ERC）确诊门静脉海绵样变和胆管扩张。4例患者发现胆结石。胆管支架置入术后，2例患者预后良好。4例患者在长期内镜治疗失败后，二线手术门体分流术使胆管支架得以取出，且疾病无复发。总之，门静脉海绵样变累及胆管现在是一个公认的实体，我们的结果表明可能需要内镜和手术联合治疗。
