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Journal of Neurosurgical Sciences 2019 October;63(5):548-65

DOI: 10.23736/S0390-5616.17.04153-4

Copyright © 2017 EDIZIONI MINERVA MEDICA

lingua: Inglese

Von Hippel-Lindau disease: when neurosurgery meets nephrology, ophthalmology and genetics

Francesco SIGNORELLI 1, 2 , Giovanni PISCOPO 3, Sophie GIRAUD 4, Silvana GUERRIERO 5, Antonio LABORANTE 6, Maria E. LATRONICO 6, Gennaro CHIMENTI 7, Rodolfo MADURI 8, Domenico CHIRCHIGLIA 9, Angelo LAVANO 8, Jacques GUYOTAT 2, Giovanni ALESSIO 5, Loreto GESUALDO 3

1 Service of Neurosurgery, Department of Clinical and Experimental Medicine, “Magna Græcia” University of Catanzaro, Catanzaro, Italy; 2 Service of Neurosurgery D, “Pierre Wertheimer” Neurologic and Neurosurgical Hospital, Lyon, France; 3 Unit of Nephrology, Dialysis, Transplantation, Department for Emergency and Organ Transplantation, AOU Policlinico, “Aldo Moro” University, Bari, Italy; 4 Department of Molecular and Clinical Genetics, Edouard Herriot Hospital, Lyon, France; 5 Department of Ophthalmology and Neuroscience, AOU Policlinico, “Aldo Moro” University, Bari, Italy; 6 Service of Ophthalmology, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Foggia, Italy; 7 Service of Ophthalmology, University of Siena, Siena, Italy; 8 Service of Neurosurgery, Department of Clinical Neurosciences, University Hospital of Lausanne (CHUV), Lausanne, Switzerland; 9 Department of Medical Sciences, “Magna Græcia” University of Catanzaro, Catanzaro, Italy



INTRODUCTION: Von Hippel-Lindau (VHL) disease is a dominantly inherited condition associated with tumors in multiple organs, whose treatment requires heightened multidisciplinary teamwork. Therefore, a document summarizing all the pertinent knowledge is needed to enhance coordination of care.
EVIDENCE ACQUISITION: A systematic review of the literature from the Medline, Embase and Cochrane Central databases was performed. From 1970 to 2017, all articles meeting specific inclusion criteria were included by at least one specialist physician for each field.
EVIDENCE SYNTHESIS: We included 95 articles, mostly dealing with genetics or management of VHL associated tumors in one organ system. There were no papers discussing the manifestations of VHL altogether, which was the aim of our paper.
CONCLUSIONS: VHL requires a multidisciplinary management to provide the highest quality of care. Coordination and communication between patients and caregivers is enhanced when knowledge is shared. Gathering together specialists in different domains around the production and reading of a comprehensive document such as the one hereby may contribute to this purpose.


KEY WORDS: von Hippel-Lindau disease; Brain; Retina; Hemangioblastoma; Carcinoma, renal cell; Pheochromocytoma

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