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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

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 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.

Keywords

Thoracic aorta Neurofibromatosis Pregnancy 

Notes

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.

References

  1. 1.
    Oderich GS, Sullivan TM, Bower TC, Gloviczki P, Miller DV, Babovic-Vuksanovic D, et al. Vascular abnormalities in patients with neurofibtomatosis syndrome type I: clonical spectrum, management, and results. J Vasc Surg. 2007;46:475–84.CrossRefGoogle Scholar
  2. 2.
    Park YJ, Park KM, Oh J, Park HS, Kim JS, Kim YW. Spontaneous aortic rupture in a patient with neurofibromatosis type 1. J Korean Surg Soc. 2012;82:261–5.CrossRefGoogle Scholar
  3. 3.
    Tapp E, Hickling RS. Renal artery rupture in a pregnant woman with neurofibromatosis. J Pathol. 1969;97(2):348–402.CrossRefGoogle Scholar
  4. 4.
    Saitoh S, Matsuda S. Aneurysm of the major vessels in neurofibromatosis. Arch Orthop Trauma Surg. 1998;117:110–3.CrossRefGoogle Scholar
  5. 5.
    Smok DA. Aortopathy in pregnancy. Semin Perinatol. 2014;38:295–303.CrossRefGoogle Scholar
  6. 6.
    Manalo-Estrella P, Barker AE. Histopathologic findings in human aortic media associated with pregnancy. Arch Pathol. 1967;83:336–41.PubMedGoogle Scholar

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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