Anesthesia in a child with suspected peroxisomal disorder

Englbrecht JS, Maas M

Abstract

We present the case of an 8-year-old femalechild with suspected peroxisomal disorderrequiring general anesthesia for adenotomy,paracentesis and brainstem-evokedresponse audiometry. Peroxisomes are smallintracellular organelles that catalyse keymetabolic reactions. Peroxisomal disordersare a heterogeneous group of rare geneticdiseases. Anesthesia can be challenging asadrenal insufficiency, mental retardation,muscle weakness, risk of pulmonaryaspiration, airway complications, seizuredisorders and altered pharmacokineticsand pharmacodynamics can occur in thesepatients but guidelines for anesthesia donot exist due to the heterogeneity and rarityof these diseases and case reports are rare.Anesthesia was induced by sevofluranevia a face mask, followed by remifentaniland rocuronium for oral intubation afterintravenous access was obtained. Anesthesiawas maintained with sevoflurane andremifentanil. Dexamethasone was givenfor prophylaxis of postoperative nauseaand vomiting as well as perioperativeadrenal crises. Piritramide was given forpostoperative analgesia.With this approachanesthesia was uneventful. The tracheawas extubated with the patient awakeand she was taken to the recovery roomin a stable condition. The classificationand breadth of clinical manifestations ofperoxisomal disorders is complex and brieflysummarized. Anesthesiologists shouldconsider characteristics of their particularpatient’s form of peroxisomal disorder, as thismay greatly influence procedural planning.