Anesthetic implications of ornithine transcarbamylase deficiency

Andrea P. Dutoit, Randall R. Flick, Juraj Sprung, Dusica Babovic-Vuksanovic, Toby N. Weingarten

Research output: Contribution to journalArticlepeer-review

10 Scopus citations


Background: Ornithine transcarbamylase deficiency (OTCD) is an X-linked urea cycle disorder associated with potentially fatal episodes of hyperammonemia. Children with OTCD often require anesthesia. There is insufficient information regarding perioperative complications and optimal management of anesthesia in these patients. Aim: To retrospectively review the medical records of children with OTCD to ascertain the nature and frequency of peri-procedural complications. Methods/Materials: The electronic medical records of Mayo Clinic patients with OTCD who underwent anesthesia between the dates of January 2003 and September 2009 were reviewed. Results: Nine patients with OTCD underwent 25 anesthetics using a variety of anesthetic techniques, including four major surgeries. Eleven procedures were performed prior to OTCD diagnosis and those patients were not receiving therapy for a urea cycle disorder. In the other cases, patients were on a variety of therapies for OTCD. Fourteen patients were outpatient procedures. Clinical signs of postoperative metabolic decompensation did not occur. Conclusions: In this series, patients with OTCD tolerated anesthesia well. Choice of perioperative management of OTCD and the choice of anesthetic technique should be individualized and based on clinical circumstances, but should have the underlying aim of minimizing protein catabolism. It appears patients with stable OTCD may undergo minor procedures as outpatients safely.

Original languageEnglish (US)
Pages (from-to)666-673
Number of pages8
JournalPaediatric Anaesthesia
Issue number7
StatePublished - Jul 2010
Externally publishedYes


  • General anesthesia
  • Ornithine transcarbamylase deficiency
  • Pediatric anesthesia

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Anesthesiology and Pain Medicine


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