General Thoracic and Cardiovascular Surgery

, Volume 67, Issue 11, pp 979–981 | Cite as

Spontaneous ascending aortic rupture in a pregnant woman with neurofibromatosis type 1

  • Atsushi TateishiEmail author
  • Masahiro Okada
  • Mikizo Nakai
  • Yutaka Yokota
  • Yosuke Miyamoto
Case Report


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 uneventfully. During 7 years of follow-up, aorta of the patient shows no significant change. A review of the literature regarding the pathogenesis of this condition is also presented.


Thoracic aorta Neurofibromatosis Pregnancy 


Compliance with ethical standards

Conflict of interest

None of authors had a conflict of interest concerning this study, and none received outside support for this research.


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Copyright information

© The Japanese Association for Thoracic Surgery 2018

Authors and Affiliations

  1. 1.Department of Cardiovascular SurgeryOkayama Medical Center, National Hospital OrganizationOkayama CityJapan

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