title: Anesthetic implications of ornithine transcarbamylase deficiency.
abstract: 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.
  
  
  OBJECTIVE
  To retrospectively review the medical records of children with OTCD to ascertain the nature and frequency of peri-procedural complications.
  
  
  METHODS
  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.
authors.affiliation: Department of Anesthesiology, College of Medicine, Mayo Clinic, Rochester, MN 55901, USA.
authors.forename: Andrea P
authors.initials: AP
authors.lastname: Dutoit
authors.forename: Randall R
authors.initials: RR
authors.lastname: Flick
authors.forename: Juraj
authors.initials: J
authors.lastname: Sprung
authors.forename: Dusica
authors.initials: D
authors.lastname: Babovic-Vuksanovic
authors.forename: Toby N
authors.initials: TN
authors.lastname: Weingarten
mesh_terms: D000758:Anesthesia / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D020163:Ornithine Carbamoyltransferase Deficiency Disease; D019990:Perioperative Care / Q000379:methods*; D012189:Retrospective Studies