Orloff M J, Orloff M S, Daily P O, Girard B
Department of Surgery, University of California, San Diego Medical Center 92103-8999.
Am J Surg. 1994 Jan;167(1):96-102; discussion 102-3. doi: 10.1016/0002-9610(94)90059-0.
This report describes the long-term results of one-stage total gastrectomy and distal two-thirds esophagectomy, with reconstruction by esophagojejunostomy (16 Roux-en-Y; 2 interposition), in 18 adult patients with recurrent variceal hemorrhage due to unshuntable extrahepatic portal hypertension (EHPH) from occlusion of all major tributaries of the portal venous system. The etiology of portal venous occlusion was unknown in 11 patients, abdominal trauma in 3, peritonitis in 3, and thrombotic coagulopathy in 1. Almost half of the patients had their first episode of bleeding in childhood, and 83% experienced bleeding before 40 years of age. The severity of the problem was reflected by frequent previous bleeding episodes (mean: 12.8, range: 4 to 21), a large cumulative requirement for blood transfusions (mean: 129 units, range: 28 to 247 units), repeated, costly hospital admissions (mean: 15, range: 4 to 24), and numerous previous unsuccessful operations (mean: 4.4, range: 1 to 14). Blood transfusions transmitted serum hepatitis to three patients and AIDS to one, for an incidence of 22%. Bleeding recurred after repetitive endoscopic sclerotherapy in 10 patients and after various operations in 16 (failed portal-systemic shunts in 9, splenectomy in 16, devascularization procedures in 13). All patients had large esophageal and gastric varices on endoscopy, normal liver function, and widespread portal venous occlusion on visceral angiography. Radical esophagogastrectomy was usually a long and arduous operation because of dense adhesions, extensive collateral veins, and a scarred, contracted bowel mesentery due to previous operations. All patients survived the operation and are currently alive. No patient has had recurrent bleeding during 1 to 26 years of follow-up (mean: 13.9 years, 7 or more years in 14 patients). Quality of life has been good. It is concluded that radical esophagogastrectomy is the only effective treatment of unshuntable EHPH and that the operation should be performed promptly when this disease, which is associated with high mortality, high morbidity, and high costs, is diagnosed.
本报告描述了18例成年患者因门静脉系统所有主要分支闭塞导致不可分流的肝外门静脉高压（EHPH）而反复发生静脉曲张出血，接受一期全胃切除术和远端三分之二食管切除术，并通过食管空肠吻合术重建（16例Roux-en-Y式；2例间置术）的长期结果。11例患者门静脉闭塞的病因不明，3例为腹部外伤，3例为腹膜炎，1例为血栓性凝血病。几乎一半的患者在儿童期首次出血，83%的患者在40岁之前发生过出血。问题的严重程度体现在既往频繁的出血发作（平均：12.8次，范围：4至21次）、大量的累计输血需求（平均：129单位，范围：28至247单位）、反复且昂贵的住院治疗（平均：住院15次，范围：4至24次）以及众多既往未成功的手术（平均：4.4次，范围：1至14次）。输血导致3例患者感染血清性肝炎，1例感染艾滋病，发生率为22%。10例患者在反复内镜硬化治疗后出血复发，16例在接受各种手术后出血复发（9例门静脉分流术失败，16例脾切除术，13例去血管化手术）。所有患者内镜检查均显示有巨大食管和胃静脉曲张，肝功能正常，内脏血管造影显示广泛的门静脉闭塞。由于粘连致密、侧支静脉广泛以及既往手术导致肠系膜瘢痕化和挛缩，根治性食管胃切除术通常是一项漫长而艰巨的手术。所有患者均存活，目前仍在世。在1至26年的随访期间（平均：13.9年，14例患者随访7年或更长时间），无患者再次出血。生活质量良好。结论是根治性食管胃切除术是治疗不可分流的EHPH的唯一有效方法，当诊断出这种与高死亡率、高发病率和高成本相关的疾病时，应立即进行手术。
