Uncomplicated outcome after anesthesia for pediatric patients with Gaucher disease.
OBJECTIVE
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.
Ioscovich
Alexandr
A
Department of Anesthesiology, Shaare Zedek Medical Center, P.O. Box 3235, Jerusalem 91031, Israel.
Briskin
Alex
A
Abrahamov
Aya
A
Halpern
Stephen
S
Zimran
Ari
A
Elstein
Deborah
D
D000293:Adolescent; D000740:Anemia / Q000150:complications; D000758:Anesthesia; D001803:Blood Transfusion; D002405:Catheterization, Central Venous; D002648:Child; D002675:Child, Preschool; D005260:Female; D005776:Gaucher Disease / Q000150:complications*; D006801:Humans; D008297:Male; D011229:Preanesthetic Medication; D012189:Retrospective Studies; D013921:Thrombocytopenia / Q000150:complications; D016896:Treatment Outcome