Anesthesia for obstetric patients with Gaucher disease: survey and review.
Pregnancy and delivery in patients with non-neuronopathic Gaucher disease, whether treated with enzyme replacement or untreated, are usually uncomplicated. Various factors may influence mode of delivery, vaginal or cesarean section, as well as type of anesthesia, general or regional, used during delivery. This retrospective review was intended to highlight some of the practical issues relating to obstetric anesthetic management, based on a review of the literature and experiences from a large referral clinic for Gaucher disease. In the past decade, there were 16 deliveries in 11 women in our institution. There were five normal vaginal deliveries, two vacuum extractions, one placental extraction, and eight cesarean sections. Platelet counts were 27-215 x 10(9)/L. Two spontaneous deliveries and one vacuum extraction were performed under epidural anesthesia; two other women having vaginal deliveries and one vacuum extraction were given i.v. analgesia; the fifth was given i.v. patient-controlled analgesia. The placental extraction was performed under general anesthesia. Seven of the women having cesarean deliveries received spinal anesthesia; the breech presentation required general anesthesia. There were no anesthesia-related side effects or complications, although there were some instances of post-partum bleeding irrespective of enzyme therapy. Gaucher disease affects multiple organs and can be a challenge to the anesthesiologist. Based on this survey we suggest that anesthetic management requires particular attention to hematological parameters before delivery. A multidisciplinary approach and extensive communication among obstetrician, hematologist and anesthesiologist is required to anticipate the possibility of post-partum hemorrhage, and preclude skeletal damage.
Ioscovich
A
A
Department of Anesthesia and the Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem, Israel.
Elstein
Y
Y
Halpern
S
S
Vatashsky
E
E
Grisaru-Granovsky
S
S
Elstein
D
D
D000328:Adult; D000767:Anesthesia, Epidural; D000773:Anesthesia, Obstetrical; D002585:Cesarean Section; D005113:Extraction, Obstetrical; D005260:Female; D005776:Gaucher Disease / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D005838:Genotype; D006801:Humans; D007585:Jews; D010348:Patient Care Team; D006473:Postpartum Hemorrhage / Q000209:etiology / Q000503:physiopathology; D011247:Pregnancy; D012189:Retrospective Studies