Lillehei C W, Shamberger R C
Children's Hospital, and Harvard Medical School, Boston, MA 02115, USA.
J Pediatr Surg. 2001 Aug;36(8):1252-4. doi: 10.1053/jpsu.2001.25787.
BACKGROUND/PURPOSE: Middle aortic syndrome is a rare condition that involves narrowing of the abdominal aorta and its visceral branches. The authors propose staged vascular repair to minimize renal ischemia and facilitate use of native arterial tissue for reconstruction.
Three adolescents (age 8(1/2), 12(1/2), 13(1/2)) presented with severe hypertension. Subsequent evaluation showed coarctation of the abdominal aorta extending above the celiac axis. All 3 patients had bilateral renal artery stenoses. There also were tight stenoses of the celiac or superior mesenteric arteries. In the first stage the right renal artery stenosis was relieved. In the youngest patient, this was accomplished by balloon angioplasty. However, in the other 2, right renal autotransplantation was performed to the right iliac vessels using end-to-side anastomoses of the renal artery and vein. Cold perfusion was used. The second stage was performed 2 to 5 months later via a thoracoabdominal approach in 2 patients. A Dacron tube graft was utilized from above the coarctation to the iliac bifurcation. The left renal arteries were detached and anastomosed end to side to the bypass graft. In 1 child there were actually 3 separate renal arteries that required reimplantation. In the youngest patient the aortic narrowing was relieved by a long Dacron patch aortoplasty and interposition of an internal iliac artery graft to the left renal artery.
All 3 patients recovered well and returned to full activities. There was no measurable rise of BUN or serum creatinine postoperatively. Postoperative renal scans showed good renal perfusion bilaterally. Follow-up results 2 to 10 years later continue to show well functioning reconstructions.
A staged approach is an effective reconstruction for children with middle aortic syndrome which minimizes risk to renal function.
背景/目的：中段主动脉综合征是一种罕见疾病，表现为腹主动脉及其内脏分支狭窄。作者提出分期血管修复，以尽量减少肾缺血，并便于使用自体动脉组织进行重建。
3名青少年（年龄分别为8.5岁、12.5岁、13.5岁）出现严重高血压。后续评估显示腹主动脉缩窄延伸至腹腔动脉轴上方。所有3例患者均有双侧肾动脉狭窄。腹腔动脉或肠系膜上动脉也存在严重狭窄。第一阶段缓解右肾动脉狭窄。最年轻的患者通过球囊血管成形术完成。然而，另外2例患者，将右肾自体移植至右髂血管，采用肾动脉和静脉端侧吻合。使用了冷灌注。2至5个月后，2例患者通过胸腹联合入路进行第二阶段手术。使用涤纶人工血管从缩窄上方至髂总动脉分叉处。游离左肾动脉并将其端侧吻合至旁路移植血管。1例患儿实际上有3支独立的肾动脉需要重新植入。最年轻的患者通过长涤纶补片主动脉成形术缓解主动脉狭窄，并将髂内动脉移植至左肾动脉。
所有3例患者恢复良好，恢复了全部活动。术后血尿素氮或血清肌酐无明显升高。术后肾脏扫描显示双侧肾脏灌注良好。2至10年后的随访结果持续显示重建功能良好。
分期手术是治疗中段主动脉综合征患儿的有效重建方法，可将肾功能风险降至最低。
