This Article Résumé de cet Article Full Text Full Text (PDF) Submit a scholarly reply Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Ioscovich, A. Articles by Elstein, D. Search for Related Content PubMed PubMed Citation Articles by Ioscovich, A. Articles by Elstein, D.

Uncomplicated outcome after anesthesia for pediatric patients with Gaucher disease

[Évolution sans complications après l’anesthésie de patients pédiatriques atteints de la maladie de Gaucher]

Alexandr Ioscovich, MD^*, Alex Briskin, MD^*, Aya Abrahamov, MD, Stephen Halpern, MD, Ari Zimran, MD and Deborah Elstein, PhD

^* From the Department of Anesthesiology, and
the Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem, Israel; and
the Department of Anaesthesia, Sunnybrook and Women’s College Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada.

Address correspondence to: Dr. Deborah Elstein, Gaucher Clinic, Shaare Zedek Medical Center, P.O. Box 3235, Jerusalem 91031, Israel. Phone: +972-2-655-5143; Fax: +972-2-651-7979; E-mail: elstein{at}szmc.org.il

Purpose: The purpose of this retrospective review was to highlight clinical issues relating to anesthetic management in children who present with Gaucher disease-specific features that may impact on anesthetic management and surgical outcome. Previous reports have dealt primarily with neuronopathic forms where neurological dysfunction determined the mode of anesthesia. To date, no series of routine surgeries in pediatric patients with non-neuronopathic Gaucher disease has been published.

Methods: All surgeries performed in children with Gaucher retrospectively analyzed.

Results: There were 31 procedures under anesthesia in 15 pediatric patients. Twenty-seven of these (87%) involved either insertion or removal of a central venous catheter. There was no correlation between disease severity and the need for blood transfusion postoperatively [required in only eight cases (25.8%), including a total hip replacement]. No difficult intubations or other airway problems were recorded. Positioning of two patients, because of gibbus and prior to hip replacement, respectively, required special attention.

Conclusions: We record our experience in surgeries in children with mild, non-neuronopathic type I and severe neuronopathic type III Gaucher disease, who had relatively short surgeries under general anesthesia. Attention to hematological parameters in particular can minimize postoperative bleeding, the most serious complication.