Perioperative care of a child with Zellweger syndrome.

Abstract

Zellweger syndrome (ZS) is an autosomal recessive dis- order characterized by defects in the structure, function, or number of peroxisomes, which are essential for the β- oxidation of very-long-chain fatty acids. Disordered pe- roxisome function leads to a wide range of metabolic ab- normalities and end-organ involvement including renal, hepatic, neurologic, and adrenal dysfunction. We report an 18-month-old girl with ZS requiring general anesthe- sia during placement of bilateral cochlear implants. The potential perioperative concerns of ZS are discussed, pre- vious reports of anesthetic care reviewed, and options for anesthetic care presented.

Keywords

• zellweger syndrome; peroxisomes; fatty acid metabo- lism; β-oxidation