Caesarean section and phaeochromocytoma resection in a patient with Von Hippel Lindau disease

Abstract

This report describes the anaesthetic management of a women with a term gestation, Von Hippel Lindau disease (VHLD), and a phaeochromocytoma, scheduled for a combined phaeochromocytoma resection and Caesarean section. Von Hippel Lindau disease is characterized by diffuse haemangioblastomas of the central nervous system (CNS) and viscera. It is also (labdtalol 1,0 mg' kg -t et esmolol 0,75 mg" kg -I)

Bilateral phaeochromocytoma during pregnancy

“…Following bilateral adrenalectomy for bilateral phaeochromocytoma, with a recurrence at an extra-adrenal site during pregnancy a successful pregnancy outcome has been reported (9). Combining tumour resection of radiologically localised tumours with caesarean section has been reported to be successful by many workers (10,11). Ultrasound scan ning for remnant tumour being negative in our patient, and the advanced state of her pregnancy, with satisfac tory fetal growth in the third trimester, justified our con servative approach to her management.…”

Reproductive disturbances in multiple neuroendocrine tumor syndromes

“…Pheochromocytomas or paragangliomas in pregnant MEN, NF1, VHLD, and PGL patients require careful evaluation and design of surgical and medical treatment (Hopkins et al 1989, Joffe et al 1993, Hamilton et al 1997, Demiraran et al 2001, Kariya et al 2005.…”

Caesarean section for a woman with Von Hippel-Lindau disease

“…2 The anaesthetic literature is limited regarding ideal anaesthetic management and both neuraxial and general anaesthesia have been used successfully for caesarean section. [5][6][7][8][9][10] Neuraxial blockade could cause cerebellar herniation in patients with raised ICP with either a deliberate or inadvertent dural puncture. There is also the risk of rupturing a spinal haemangioblastoma with either spinal or epidural needle.…”