title: Anesthetic management of a patient with Alport-leiomyomatosis syndrome.
abstract: 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.
authors.affiliation: Department of Anesthesiology and Resuscitology, Okayama University Medical School, 2-5-1 Shikata-cho, Okayama 700-8558, Japan.
authors.forename: Motohiko
authors.initials: M
authors.lastname: Hanazaki
authors.forename: Ken
authors.initials: K
authors.lastname: Takata
authors.forename: Keiji
authors.initials: K
authors.lastname: Goto
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Katayama
authors.forename: Masataka
authors.initials: M
authors.lastname: Yokoyama
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Morita
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Shirakawa
authors.forename: Tomoki
authors.initials: T
authors.lastname: Yamatsuji
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Naomoto
mesh_terms: D000768:Anesthesia, General; D001249:Asthma / Q000150:complications; D004938:Esophageal Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D016629:Esophagectomy; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D018231:Leiomyomatosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D008279:Magnetic Resonance Imaging; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D055815:Young Adult
references.citation: Eur J Pediatr Surg. 1998 Dec;8(6):358-63
references.pmid: 9926306
references.citation: J Thorac Cardiovasc Surg. 1999 Oct;118(4):760-4
references.pmid: 10610420
references.citation: Semin Pediatr Surg. 1994 Nov;3(4):259-66
references.pmid: 7850366
references.citation: Gastroenterology. 2001 Jan;120(1):216-20
references.pmid: 11208731
references.citation: Nephrol Dial Transplant. 1997 Apr;12(4):776-80
references.pmid: 9141011
references.citation: Am J Dig Dis. 1975 Jul;20(7):684-90
references.pmid: 1146792
references.citation: Am J Hum Genet. 1998 Feb;62(2):253-61
references.pmid: 9463311
references.citation: Am J Med Genet A. 2003 Jun 15;119A(3):381-5
references.pmid: 12784310
references.citation: J Biochem. 1998 May;123(5):767-76
references.pmid: 9562604