Organ Donation and Moyamoya Disease

Abstract

To the Editor: The Moyamoya Syndrome is a rare cerebrovascular condition characterized by progressive stenosis of the intracranial internal carotid arteries and their proximal branches resulting in the development of severe collateral circulation and associated with a high risk of stroke (1Suzuki J Kodama N Moyamoya disease—A review.Stroke. 1983; 14: 104-109Crossref PubMed Scopus (692) Google Scholar). The Japanese term “moyamoya” was used for the first time in 1969. Suzuki and Takaku reported the presence of abnormally dilated collateral vessels appearing as “a hazy, like a puff of smoke”, corresponding to a network of dilated vessels occurring in the region of the circle of Willis (2Suzuki J Takaku A Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain.Arch Neurol. 1969; 20: 288-299Crossref PubMed Scopus (1754) Google Scholar). Moyamoya disease affects patients from many ethnic backgrounds, including American and European populations, with a higher incidence in persons from Asian heritage. The condition is rare with studies reporting an incidence of 0.086 cases per 100 000 persons in the United States (3Scott RM Smith ER Moyamoya disease and moyamoya syndrome.N Engl J Med. 2009; 360: 1226-1237Crossref PubMed Scopus (1106) Google Scholar). To our knowledge, there are no reports describing the use of organs from donors diagnosed with Moyamoya disease or studies evaluating the frequency of organ usage from individuals with catastrophic brain injury associated with this disease. Similarly, little is known about the complications associated with the use of solid organs from donors diagnosed with this rare condition. We recently evaluated a patient with massive hemorrhagic stroke who was diagnosed with Moyamoya disease (Figure 1:, Figure 2:, Figure 3:). The patient’s family agreed to allow organ donation (heart, kidneys, liver and lungs) resulting in successful organ transplantation in five transplant recipients. Evaluation of the patients after a short follow-up period (6 months) confirmed excellent allograft function in all recipients.Figure 2:Hematoxylin and eosin-stained section of the right middle cerebral artery, showing fibro–cellular intimal hyperplasia, intact and undulating internal elastic lamina and an attenuated media.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3:Trichrome-stained section of the right middle cerebral artery showing the intact nature of internal elastic lamina.View Large Image Figure ViewerDownload Hi-res image Download (PPT) We performed a review of the English language literature on the PubMed database using the terms “Moyamoya”, “organ donation” and “stroke” and we were unable to identify reports of successful transplantation using organs from donors diagnosed with Moyamoya disease. In addition, we performed a review of the UNOS database (dates: 1996–2010) and found that Moyamoya disease was reported rarely as cause of death among donors (three thoracic transplants and three abdominal transplants). These findings contrast with reports that suggest a higher number potential number of organ donors with Moyamoya disease and catastrophic brain injury. It is possible that this discrepancy may be explained by coding errors in the UNOS database or misdiagnosis of the disease. Moyamoya disease is considered a nontransmissible, nonatherosclerotic and noninflammatory condition and, therefore, there are no theoretical risks related to the use of organs from donors diagnosed with this condition. Nevertheless, Moyamoya disease may have extracranial manifestations. Moyamoya has been associated with coronary artery fistulas and stenosis (4St Goar FG Gominak SC Potkin BN Bilateral aortoostial coronary artery disease: Moyamoya of the heart?.Am J Cardiol. 1999; 83 (A1210.): 1296-1299Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar,5Gatti G Benussi B Gon L et al.Aortic valve replacement in an adult white male with moyamoya disease and coronary artery fistulas.J Cardiothorac Vasc Anesth. 2007; 21: 166-168Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar) and congenital heart disease (coarctation of the aorta, ventricular septal defect and valve stenosis; Ref. 6Lutterman J Scott M Nass R et al.Moyamoya syndrome associated with congenital heart disease.Pediatrics. 1998; 101: 57-60Crossref PubMed Scopus (65) Google Scholar), renal artery stenosis and renovascular hypertension (7Jansen JN Donker AJ Luth WJ et al.Moyamoya disease associated with renovascular hypertension.Neuropediatrics. 1990; 21: 44-47Crossref PubMed Scopus (35) Google Scholar,8van der Vliet JA Zeilstra DJ Van Roye SF et al.Renal artery stenosis in moyamoya syndrome.J Cardiovasc Surg (Torino). 1994; 35: 441-443PubMed Google Scholar). We believe that transplant physicians should continue using organs from donors with Moyamoya disease but must be aware of the possible extracranial associations. The long-term complications and outcomes associated with solid organ transplantation from donors diagnosed with Moyamoya disease require further investigation. The authors of this manuscript have no conflicts of interest to disclose as described by the American Journal of Transplantation. There was no funding source needed for this manuscript. The manuscript represents original work that is not being considered or has been accepted for publication elsewhere.