A case report of anesthesia for a child with Hajdu-Cheney syndrome.

Abstract

Hajdu-Cheney syndrome is an extremely rare disorder characterized by progressive skeletal acro-osteolysis, which results in extremity fractures and scoliosis often requiring surgical treatment from childhood. A unique facial structure and deformity of the cervical spine is associated with a difficult airway. We report here a 10-year-old girl with Hajdu-Cheney syndrome who developed progressive basilar impression and medullary compression for which foramen magnum decompression was performed. After slow induction of anesthesia, we were able to perform fiberoptic orotracheal intubation via a VBM bronchoscope airway. This case report contributes to the accumulation of knowledge about anesthesia for this rare syndrome.

• Authors: Satoshi Yamaguchi, Kyoichi Nakamura, Yukio Takahashi
• Journal: Journal of anesthesia (J Anesth) Vol. 27 Issue 6 Pg. 949-50 (Dec 2013) ISSN: 1438-8359 [Electronic] Japan
• PMID: 23728363 (Publication Type: Case Reports, Journal Article)

Topics

• Anesthesia, General (methods)
• Child
• Female
• Hajdu-Cheney Syndrome (physiopathology, surgery)
• Humans