CESAREAN-SECTION AND PHEOCHROMOCYTOMA RESECTION IN A PATIENT WITH VONHIPPEL-LINDAU DISEASE

Citation
D. Joffe et al., CESAREAN-SECTION AND PHEOCHROMOCYTOMA RESECTION IN A PATIENT WITH VONHIPPEL-LINDAU DISEASE, Canadian journal of anaesthesia, 40(9), 1993, pp. 870-874
Citations number
20
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Anesthesiology
ISSN journal
0832-610X
Volume
40
Issue
9
Year of publication
1993
Pages
870 - 874
Database
ISI
SICI code
0832-610X(1993)40:9<870:CAPRIA>2.0.ZU;2-L
Abstract
This report describes the anaesthetic management of a women with a ter m gestation, Von Hippel Lindau disease (VHLD), and a phaeochromocytoma , scheduled for a combined phaeochromocytoma resection and Caesarean s ection. Von Hippel Lindau disease is characterized by diffuse haemangi oblastomas of the central nervous system (CNS) and viscera. It is also associated with phaeochromocytomas and renal cell carcinomas. Patient s frequently have asymptomatic spinal cord and intracranial pathology. The patient and her fetus prevented a challenge because of the anaest hetic restrictions imposed by VHLD, and her pregnancy. She was also at risk of developing malignant hypertension from the phaeochromocytoma. The patient was not a candidate for regional anaesthesia because of t he possibility of spinal cord haemangioblastomas. She had received adr energic blockade with phentolamine (total 30 mg a day) and propranolol (total 40 mg a day) since the 27th wk of gestation in order to contro l hypertension secondary to the phaeochromocytoma. General anaesthesia was administered with aggressive management of hypertension with adre nergic blockers (labetalol 1.0 mg . kg-1 and esmolol 0. 75 mg . kg-1) and sodium nitroprusside 1.5 mug . kg-1 (total). Before delivery of th e baby, opiods, which could have resulted in a fetus with CNS depressi on, were avoided. After delivery, opioids (sufentanil 0.4 mug . kg-1 . hr-1) were used to limit the use of inhalational anaesthesia which ma y contribute to uterine atony Postoperative pain was managed with an i ntravenous narcotic infusion. Both patients had uneventful postoperati ve courses.