Anesthesia implications of Von Hippel–Lindau disease (vHLD)

🎨vHLD usually presents in young adults with cerebellar, medullary or spinal haemangioblastomas, retinal angiomatosis, renal cell carcinoma and phaeochromocytoma

🎨The frequency of phaeochromocytomas is 7–20%

🎨About 25% of patients with CNS haemangioblastomas subsequently turn out to have vHLD.

🎨Erythrocytosis and a high haematocrit are common and has to be searched for.

🎨Surgery for one manifestation of the disease may be complicated by the presence of an undiagnosed phaeochromocytoma. In this situation, pharmacological control of phaeochromocytoma should get more priority and surgery may have to be carried out in two stages

🎨Spinal anaesthesia may be dangerous in the presence of an undiagnosed cerebral or spinal tumour. Another point is, spinal cord haemangioblastomas can occur at more than one level. An MRI if already done, can help us to take a proper decision.

🎨Pregnancy may worsen the disease, by increasing the vascularity of tumours. Urgent and life saving neurosurgical intervention may become necessary: for e.g. when a spinal tumor bleeds, when a tumor obstructs CSF flow and causes acute hydrocephalus. Sometimes elective procedures like removal of a phaeochromocytoma which became evident during pregnancy has to be removed. It may become necessary to carry out these procedures during pregnancy or along with a Caesarean section.

🎨Surgery may be required for more than one lesion at the same time.

🎨So careful assessment should be made for lesions other than the one for which anaesthesia is required, and in particular for any symptoms and signs of cerebral, cerebellar or spinal cord tumours and phaeochromocytoma.

🎨In the situation in which two lesions are present, decisions may have to be made as to whether to operate simultaneously or separately . During pregnancy the management of the delivery must be carefully planned in advance.

🎨Although 24-h urinary screening for catecholamines can be performed, plasma normetanephrines and metanephrines are the most sensitive tests for detecting phaeochromocytomas in patients with family predisposition

Reference: Anaesthetic management of a patient with von Hippel–Lindau disease: a combination of bilateral phaeochromocytoma and spinal cord haemangioblastoma. European Journal of Anaesthesiology 13: 81–3. , Anesthesia Databook, 3rd edition