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