Abnormal fatty acid metabolism in spinal muscular atrophy may predispose to perioperative risks.
A 15 year old boy with SMA type II underwent spinal fusion and suffered a mitochondrial Reye-like catabolic crisis 4 days postop with hypoketotic hypoglycemia, lactic acidaemia, hyperammonemia and liver failure, with 90% coagulative necrosis and diffuse macro- and microvesicular steatosis, requiring orthotopic liver transplantation. This crisis responded in part to mitochondrial therapy and anabolic rescue. He made a dramatic sustained neurological recovery, though his post-transplant liver biopsies revealed micro- and macrosteatosis. We hypothesize that a combination of surgical stress-catecholamine induced lipolysis, prolonged general anaesthesia with propofol and sevoflurane, and perioperative fasting on a background of decreased β-oxidation were potential risk factors for the mitochondrial decompensation.
Duke Scholars
Published In
DOI
EISSN
Publication Date
Volume
Issue
Start / End Page
Location
Related Subject Headings
- Spinal Muscular Atrophies of Childhood
- Spinal Fusion
- Neurology & Neurosurgery
- Mitochondria
- Male
- Liver Failure
- Liver
- Hypoglycemia
- Hyperammonemia
- Humans
Citation
Published In
DOI
EISSN
Publication Date
Volume
Issue
Start / End Page
Location
Related Subject Headings
- Spinal Muscular Atrophies of Childhood
- Spinal Fusion
- Neurology & Neurosurgery
- Mitochondria
- Male
- Liver Failure
- Liver
- Hypoglycemia
- Hyperammonemia
- Humans