Anesthesia in achild with suspected peroxisomal disorder

Anesthesia in achild with suspected peroxisomal disorder We present the case of an 8‑year-old female child with suspected peroxisomal disorder requiring general anesthesia for adenotomy, paracentesis and brainstem-evoked response audiometry. Peroxisomes are small intracellular organelles that catalyse key metabolic reactions. Peroxisomal disorders are a heterogeneous group of rare genetic diseases. Anesthesia can be challenging as adrenal insufficiency, mental retardation, muscle weakness, risk of pulmonary aspiration, airway complications, seizure disorders and altered pharmacokinetics and pharmacodynamics can occur in these patients but guidelines for anesthesia do not exist due to the heterogeneity and rarity of these diseases and case reports are rare. Anesthesia was induced by sevoflurane via a face mask, followed by remifentanil and rocuronium for oral intubation after intravenous access was obtained. Anesthesia was maintained with sevoflurane and remifentanil. Dexamethasone was given for prophylaxis of postoperative nausea and vomiting as well as perioperative adrenal crises. Piritramide was given for postoperative analgesia. With this approach anesthesia was uneventful. The trachea was extubated with the patient awake and she was taken to the recovery room in a stable condition. The classification and breadth of clinical manifestations of peroxisomal disorders is complex and briefly summarized. Anesthesiologists should consider characteristics of their particular patient’s form of peroxisomal disorder, as this may greatly influence procedural planning. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Der Anaesthesist Springer Journals

Anesthesia in achild with suspected peroxisomal disorder

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Publisher
Springer Journals
Copyright
Copyright © 2017 by Springer Medizin Verlag GmbH
Subject
Medicine & Public Health; Anesthesiology; Emergency Medicine; Intensive / Critical Care Medicine; Pain Medicine
ISSN
0003-2417
eISSN
1432-055X
D.O.I.
10.1007/s00101-017-0379-0
Publisher site
See Article on Publisher Site

Abstract

We present the case of an 8‑year-old female child with suspected peroxisomal disorder requiring general anesthesia for adenotomy, paracentesis and brainstem-evoked response audiometry. Peroxisomes are small intracellular organelles that catalyse key metabolic reactions. Peroxisomal disorders are a heterogeneous group of rare genetic diseases. Anesthesia can be challenging as adrenal insufficiency, mental retardation, muscle weakness, risk of pulmonary aspiration, airway complications, seizure disorders and altered pharmacokinetics and pharmacodynamics can occur in these patients but guidelines for anesthesia do not exist due to the heterogeneity and rarity of these diseases and case reports are rare. Anesthesia was induced by sevoflurane via a face mask, followed by remifentanil and rocuronium for oral intubation after intravenous access was obtained. Anesthesia was maintained with sevoflurane and remifentanil. Dexamethasone was given for prophylaxis of postoperative nausea and vomiting as well as perioperative adrenal crises. Piritramide was given for postoperative analgesia. With this approach anesthesia was uneventful. The trachea was extubated with the patient awake and she was taken to the recovery room in a stable condition. The classification and breadth of clinical manifestations of peroxisomal disorders is complex and briefly summarized. Anesthesiologists should consider characteristics of their particular patient’s form of peroxisomal disorder, as this may greatly influence procedural planning.

Journal

Der AnaesthesistSpringer Journals

Published: Nov 8, 2017

References

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