A Case of Spinal Epidural Arteriovenous Fistulae with Scoliosis in Neurofibromatosis Type 1

DOI 2 Citations
  • Yoshida Shiro
    Department of Orthopaedic Surgery, Kurume University, Fukuoka, Japan
  • Kimura Yoshizo
    Department of Pathology, Kurume University, Fukuoka, Japan
  • Sato Kimiaki
    Department of Orthopaedic Surgery, Kurume University, Fukuoka, Japan
  • Sugita Yasuo
    Department of Pathology, Kurume University, Fukuoka, Japan
  • Suefuji Nobuko
    Department of Pathology, Kurume University, Fukuoka, Japan
  • Ohshima Koichi
    Department of Pathology, Kurume University, Fukuoka, Japan
  • Nagata Kensei
    Department of Orthopaedic Surgery, Kurume University, Fukuoka, Japan

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  • Neurofibromatosis type Iにともなう脊柱側弯症に胸髄硬膜動静脈瘻を合併した1例

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Abstract

Vascular abnormalities associated with neurofibromatosis type 1 (NF-1) occur frequently, but arteriovenous fistulae (AVF) rarely. This report describes a case of epidural AVF with scoliosis associated with NF-1 and resulting in death. A 74-year-old man with a medical history of NF-1 was admitted because of vomiting. The vital signs indicated shock with blood pressure of 54/30mmHg and heart rate of 120/min. Death followed 2 hours later. Histopathological findings showed a ruptured epidural AVF with neurofibroma. This was a very rare case of epidural AVF occurring in relatively rare association with NF-1, for which the in-hospital mortality rate is comparatively low. Our experience indicates that it is very important to screen patients with NF-1 for vascular abnormalities.

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