gms | German Medical Science

Objective: Patients affected with von Hippel-Lindau disease usually develop multiple hemangioblastomas predominantly of the cerebellum, brain stem and spinal cord. The timing of surgery for these lesions is discussed controversially and depends on the anticipated surgical morbidity. Several surgical series on the outcome of resection of single hemangioblastomas have been published. However, the cumulative surgical morbidity of multiple surgeries is unknown.

Method: To investigate cumulative surgical morbidity in patients operated for multiple medullary hemangioblastomas, we analyzed all patients treated in our institution who were operated for at least two medullary hemangioblastomas. Functional grades were determined pre- and postoperatively according to the McCormick scale.

Results: Between 1996 and 2012 a total of 22 patients were treated surgically for at least two medullary hemangioblastomas. A total of 75 operations of medullary hemangioblastomas were performed in these patients. The lesions were located in the cerebellopontine angle, the medulla oblongata and the spinal cord. 13 patients had a total of two, 2 had a total of three, 4 had a total of five, 1 had a total of six, 1 had a total of eight and 1 had a total of nine medullary operations. The analysis of mean functional grades pre- and postoperatively showed a slight but relatively continuous clinical deterioration over the course of time. This was due to the natural history of the disease and to surgical morbidity. The deterioration resulting from the surgical treatment was determined by comparing McCormick grades pre- and postoperatively. This analysis of all 75 surgeries showed a mean deterioration of 0,35 (standard deviation 0,29), which did not significantly depend on the number of previous surgeries.

Conclusions: Based on our data the risk of surgical morbidity does not significantly increase with the number of previous surgeries for medullary hemangioblastomas. The results also confirm that neurological deficits once manifested preoperatively are usually not reversible. Therefore we recommend to consider microsurgical removal of lesions that show radiographic progression before significant neurological deficit occurs even in patients with multiple tumors and multiple previous medullary surgeries.