ABSTRACT
Mucolipidosis is an autosomal recessive lysosomal storage disorder that demonstrates a clinical resemblance to mucopolysaccharidosis. Accumulation of glycoproteins throughout the body causes dysfunction of several organs, in particular, valvular heart diseases are an important cause of mortality, however, there is no consensus guideline regarding the indications and optimal timing of the surgical repair because of the unclear and short natural history. Here we present 12- and 15-year-old siblings diagnosed with mucolipidosis who underwent aortic valve replacement. The senior sibling received redo-aortic valve replacement for prosthetic valve dysfunction 11 years after the initial surgery. A few surgical valve replacements in patients with mucopolysaccharidosis have been reported, however, there is no published case of aortic valve replacements in two siblings with mucolipidosis.
ABSTRACT
Infectious abdominal aortic aneurysm is a relatively rare disease, and there is no consensus regarding its surgical treatment. Medical infectious control should be concerned comparison with surgical treatment if there is sepsis, however we sometimes have no other choice but emergency operation for uncontrollable cases. In many reports, cryopreserved homografts were used as <i>in</i>-<i>situ </i>alternative grafts for infectious aortic aneurysms because they had some merits such as anti-infectious effects, suitability and so on. However the number of <i>in-situ </i>cryopreserved homograft replacement cases are few, and the long term result is unclear. We encountered a ruptured cropreserved homograft case 7 months after urgent <i>in-situ </i>cryopreserved homograft replacement. We report the case and refer to the relevans literature.
ABSTRACT
We encountered three cases of infra-renal infected abdominal aortic aneurysm in 2007 and 2008. Preoperative blood culture was positive in two of the three patients. All of the patients presented with fever of unknown origin. We replaced the affected segment of the abdominal aorta with a synthetic graft in 1 patient, and with a cryopreserved arterial homograft in the remaining 2 patients. An infected abdominal aortic aneurysm is a life-threatening condition. Diagnosis is often difficult, and emergency surgery may be necessitated by rupture of the aneurysm. Our experience suggests that computed tomography is effective for the diagnosis of infected aneurysms. The most effective surgical technique consists of complete resection of the aneurysm, <i>in</i>-<i>situ </i>replacement of the affected aortic segment with a synthetic graft or homograft, and omental coverage.