Anesthetic management of patients with ornithine transcarbamylase deficiency

Authors: SCHMIDT, JOACHIM¹; KROEBER, STEFANIE¹; IROUSCHEK, ANDREA¹; BIRKHOLZ, TORSTEN¹; SCHROTH, MICHAEL²; ALBRECHT, SVEN

Source: Paediatric Anaesthesia, Volume 16, Number 3, March 2006 , pp. 333-337(5)

Publisher: Wiley-Blackwell

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Abstract:

Summary

Ornithine transcarbamylase deficiency (OTCD) is the most common inborn error of the urea cycle. Several specific factors require care during anesthesia in patients with this condition to avoid metabolic decompensation with acute hyperammonemia and encephalopathy. We report monozygous twins with severe neonatal-onset OTCD undergoing general anesthesia twice each, with midazolam, s-ketamine, fentanyl and isoflurane in combination with surgical field infiltration with ropivacaine. Alternative pathway medication and high-caloric diet with 10% glucose solutions were continuously administered during the perioperative course. Both children were extubated within 10 min of the final suture, and their neurological state remained unchanged. Perioperatively, blood ammonia levels remained within the normal range.

Keywords: ornithine transcarbamylase deficiency; general anesthesia; perioperative management; hyperammonemia; alternative pathway therapy

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1460-9592.2005.01695.x

Affiliations: 1: Department of Anesthesiology, University of Erlangen-Nuremberg, Germany 2: Department of Pediatrics, University of Erlangen-Nuremberg, Germany

Publication date: 2006-03-01