2018
DOI: 10.1007/s11748-018-0989-8
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Spontaneous ascending aortic rupture in a pregnant woman with neurofibromatosis type 1

Abstract: Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder that affects 1 in 3000 individuals. Vascular involvement in NF-1 is a well-recognized, but rare, feature of this disease. In pregnant women, the risk of aortic dissection or rupture is elevated during pregnancy and the postpartum period. We report a pregnant woman who had a history of NF-1 with a spontaneous ascending aortic rupture. This rupture was successfully treated by emergent surgery. The mother and the 28-week-gestation newborn recovered… Show more

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Cited by 5 publications
(6 citation statements)
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“…Two pathogenic mechanisms may account for the uncommon spontaneous aortic rupture in these cases: smooth muscle dysplasia and direct vascular invasion by neurofibromatous tissue, as well as ganglioneuromatous tissue invading the arterial wall. There was one case of spontaneous ascending aortic rupture in a pregnant woman with NF-1 in our review [ 12 ].…”
Section: Discussionmentioning
confidence: 99%
“…Two pathogenic mechanisms may account for the uncommon spontaneous aortic rupture in these cases: smooth muscle dysplasia and direct vascular invasion by neurofibromatous tissue, as well as ganglioneuromatous tissue invading the arterial wall. There was one case of spontaneous ascending aortic rupture in a pregnant woman with NF-1 in our review [ 12 ].…”
Section: Discussionmentioning
confidence: 99%
“…Several studies have suggested that risk factors for its onset included age over 40, smoking, hypertension, aortic dysplasia, Marfan syndrome and autosomal dominant connective tissue disease ( 10 , 11 ). Atsushi et al reported a case of spontaneous rupture of a thoracic aortic aneurysm that happened in a pregnant woman with type I neurofibromatosis, an autosomal dominant connective tissue diseaseat, at 30 weeks of gestation ( 12 ). Several studies have also suggested that diabetes may be a risk factor for AAA, although this has remained to be proved ( 13 , 14 ).…”
Section: Discussionmentioning
confidence: 99%
“…To the best of our knowledge, only limited reports exist which discuss these symptoms in pregnancy. 11,12) Vascular lesions due to NF1-induced vascular fragility are diverse and include aneurysms, arteriovenous fistulas, and arterial rupture. Comorbidity rates of these vascular lesions in NF1 range from 1.0 to 3.6%.…”
Section: Discussionmentioning
confidence: 99%
“…13) Ascending aortic rupture occurred in a previous NF1-complicated pregnancy. 11) The following mechanisms have been proposed for vascular fragility in NF1: (1) the direct infiltration of a neurofibroma into vascular mesothelium tissue, (2) ischemia of the vessel wall caused by the compression of a feeding artery by a neurofibroma, and (3) thinning of the vascular mesothelium and rupture of the elastic plate due to the growth of spindle-shaped cells in the vascular endothelium. These mechanisms lead to the formation of vascular lesions, vascular failure, and the weakening of the vessel wall in NF1 patients.…”
Section: Discussionmentioning
confidence: 99%