Anesthetic management of a patient with Alport-leiomyomatosis syndrome

Motohiko Hanazaki, Ken Takata, Keiji Goto, Hiroshi Katayama, Masataka Yokoyama, Kiyoshi Morita, Yasuhiro Shirakawa, Tomoki Yamatsuji, Yoshio Naomoto

Research output: Contribution to journalArticlepeer-review

4 Citations (Scopus)


We report the anesthetic management of esophagectomy for a patient with Alport-leiomyomatosis syndrome. A 23-year-old woman complained of dysphagia and severe chest pain. Her chest X-ray, computed tomography (CT), and magnetic resonance imaging (MRI) showed an enlarged esophagus, in contact with the trachea, heart, aorta, and large vessels. She frequently experienced severe asthma attacks. Because various risks in both respiration and circulation, especially in anesthesia induction, were of concern, her right femoral vessels were exposed, for the emergency use of percutaneous cardiopulmonary support (PCPS), prior to anesthesia induction. Anesthesia was induced and maintained with propofol, fentanyl, and vecuronium. Esophagectomy was performed uneventfully and no severe events were seen in anesthesia management. Alportleiomyomatosis syndrome is a very rare disease. When we are involved in the anesthetic management of a patient with this disease, evaluation of the influence of the enlarged esophagus on both respiration and circulation, and careful preparation for emergence, are very important.

Original languageEnglish
Pages (from-to)453-455
Number of pages3
JournalJournal of Anesthesia
Issue number3
Publication statusPublished - 2009
Externally publishedYes


  • Alport syndrome
  • Alport-leiomyomatosis syndrome
  • Anesthetic management
  • PCPS

ASJC Scopus subject areas

  • Anesthesiology and Pain Medicine


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