RESUMEN
OBJECTIVE: We studied the reciprocal effect of pregnancy and von Hippel-Lindau (VHL) disease by analyzing the influence of pregnancy on VHL disease-related lesions and VHL disease on pregnancy outcome. METHODS: Medical charts and imaging reports from the VHL disease expertise centers in the Netherlands were used to retrospectively assess lesion progression score before and after pregnancy and to obtain data on pregnancy outcome and VHL disease-related lesions. The Friedman test was used for analysis (p ≤ 0.05). Twenty-nine patients were studied (48 pregnancies, 49 newborns). RESULTS: The progression score of cerebellar hemangioblastomas significantly changed between the single MRI scan before and the 2 scans after pregnancy (p = 0.049) (n = 12). Fetal mortality rate was 2% (n = 1) caused by maternal pheochromocytoma. Maternal VHL disease-related complications occurred in 17% (n = 8) of all pregnancies. In 4 patients, a life-threatening situation emerged: hydrocephalus due to cerebellar hemangioblastoma (n = 2) and pheochromocytoma (n = 2). CONCLUSIONS: Pregnancy in patients with VHL disease induces cerebellar hemangioblastoma progression and causes a high VHL disease-related pregnancy complication rate. We recommend intensified surveillance of patients with VHL disease, especially of cerebellar hemangioblastomas during preconception care and pregnancy.