Document Detail


Pregnancy-related hemangioblastoma progression and complications in von Hippel-Lindau disease.
MedLine Citation:
PMID:  22875085     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
Carlijn Frantzen; Roeliene C Kruizinga; Sophie J van Asselt; Bernard A Zonnenberg; Jacques W M Lenders; Wouter W de Herder; Annemiek M E Walenkamp; Rachel H Giles; Frederik J Hes; Wim J Sluiter; Marielle G van Pampus; Thera P Links
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-8-8
Journal Detail:
Title:  Neurology     Volume:  -     ISSN:  1526-632X     ISO Abbreviation:  Neurology     Publication Date:  2012 Aug 
Date Detail:
Created Date:  2012-8-9     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
From the Department of Endocrinology (C.F., S.J.v.A., T.P.L.), Department of Medical Oncology (R.C.K., A.M.E.W.), and Department of Pathology (W.J.S.), University Medical Centre Groningen, Groningen, the Netherlands; Department of Gynaecology and Obstetrics (M.G.v.P.), Onze Lieve Vrouwe Gasthuis Amsterdam, Amsterdam, the Netherlands; Department of Internal Medicine (B.A.Z.) and Department of Nephrology (R.H.G.), University Medical Centre, Utrecht, The Netherlands; Department of Medicine, Division of General Internal Medicine (J.W.M.L.), Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands; Department of Medicine III (J.W.M.L.), Carl Gustav Carus University Medical Centre, Dresden, Germany; Department of Endocrinology (W.W.d.H.), Erasmus Medical Centre, Rotterdam, the Netherlands; and Department of Clinical Genetics (F.J H.), Leiden University Medical Centre, Leiden, the Netherlands.
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