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Published on: June 25, 2013
Anesthetic Management of an Adult Patient With Hyaline Fibromatosis Syndrome Undergoing Laparoscopic Colectomy: A
Mizuki Yoshikawa1, Tetsutaro Shinomura, Kaya Kishimoto
1From the Department of Anesthesia, Japanese Red Cross Otsu Hospital, Otsu, Japan.
Abstract:
Hyaline fibromatosis syndrome (HFS) is a rare autosomal recessive disorder characterized by hyaline fibrous depositions in the skin and internal organs. Contractured joints and gingival hypertrophy make airway management difficult in patients with HFS, while trunk deformities complicate surgical positioning. A 56-year-old woman with HFS underwent laparoscopic colectomy for sigmoid colon cancer. Her airway was secured by awake fiberoptic intubation, and general anesthesia was maintained uneventfully. This report discusses the oldest reported patient with HFS and is the first to describe the management of epidural anesthesia in a patient with HFS.
