We present here a case of gouty synovitis of the knee in a patient with partial hypoxanthine-guanine phosphoribosyl transferase deficiency (Kelley-Seegmiller syndrome), which is an inherited purine metabolic disorder. Magnetic resonance images and computed tomography showed a diffuse mass with stippled calcification around the posterior cruciate ligament (PCL) in the posterior intercondylar notch. Arthroscopic examination revealed that the articular surfaces and menisci in the affected knee were almost completely covered with white chalky monosodium urate (MSU) crystals. The diffuse mass around the PCL was composed of proliferative synovial villi covered with MSU crystals that looked like "snow covered trees". Arthroscopic total synovectomy was performed. The posterior trans-septal portal was especially useful for removal of the proliferative villi around the PCL. To our knowledge, this is the first report of arthroscopic examination in a patient with Kelley-Seegmiller syndrome.
Knee ; Posters [Publication Type] ; Transferases ; Synovitis ; Syndrome

A case is described of the staged surgical repair of thoracic aortic aneurysm after revascularization of single functioning ischemic kidney of a 68 year old man. A hitological evaluation of renal function was obtained before renal revascularization, which encouraging us to perform the repair of thoracic aortic aneurysm with less risk of post-surgical acute renal failure. In case of single ischemic kidney, renal revascularization should be preceded to other major surgeries in order to prevent renal shut down.

Between 1970 and October, 1992, 120 cases of abdominal aortic aneurysms (AAA) were treated for surgical repair. Thirteen of these cases (11%) were performed with simultaneous repair for coexistent visceral vascular diseases and other intestinal organ diseases. Another 9 patients (7.5%) were treated with coronary revascularization for combined ischemic heart disease. Six of these cases received both operations during the same hospital stay. Our surgical strategy for coexistent AAA and ischemic coronary artery disease is basically a staged operation. Coronary revascularization should precede AAA repair. Operative mortality was 1.1 percent for elective AAA repair. Long-term survival was 78% for elective surgery with a mean follow-up of 51 months, and 52% for emergency surgery with a mean follow-up of 46 months. Major risks for late death were malignant neoplasms and ischemic coronary artery disease. Survival rate of the 9 patients with successful concomitant coronary revascularization and AAA repair was 89% after 51 months of mean follow-up. We conclude that re-evaluation for coexistent ischemic heart disease is the most important point for management before and after AAA repair.

Among the 203 cases of aortic valvular surgery, we experienced 8 cases of acute coronary insufficiency during the early postsurgical period. Five cases suffered from right coronary insufficiency. The other 2 cases had left coronary failure, and the remaining case had both. The main symptom of right coronary failure was right ventricular dysfunction, resulting in inability to wean from cardiopulmonary bypass in 3 cases, and left ventricular dysfunction due to inferior myocardial infarction in 2 cases. On the other hand, the main symptom of left coronary insufficiency was acute left ventricular pump failure with a broad anteroseptal infarction, and cardiac arrest occurred in the other 2. All patients receiving an emergency coronary artery bypass graft survived. Two cases expired due to thromboembolism in the interposed graft to the left coronary ostium in Cabrol's or Piehler's procedures. In the 6 survivors we could not detect any recent coronary lesions by postsurgical coronary cineangiography. We suggest that the pathophysiology of this phenomenon was coronary artery spasm and a lack of coronary reserve capacity in severe left ventricular hypertrophy of aortic valvular disease combined with diastolic dysfunction. Prompt coronary artery bypass grafting and a careful myocardial protection using retrograde cardioplegic solutions might save patients in this critical condition and an appropriate decision on the surgical indications for aortic valvular surgery is necessary before the occurrence of left ventricular diastolic dysfunction and demand ischemia.

A 64-year-old man with a diagnosis of aortic valve stenosis presented with chest pain. The patient is a Jehovah's Witnesses and wanted surgery without blood transfusion. Therefore, we planned minimally invasive aortic valve replacement (MICS AVR) avoiding sternotomy. He underwent aortic valve replacement with a mechanical valve (ATS AP360 20 mm) through a right anterolateral thoracotomy at the fourth intercostal space. The value of hemoglobin was 11.2 g/dl after surgery. He recovered uneventfully and was discharged 17 days after surgery. MICS AVR has the advantage of less risk of bleeding, therefore MICS AVR is useful for Jehovah's Witness patients who refuse blood transfusion.