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Neurofibromatosis (NF) in Pregnancy

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Consults in Obstetric Anesthesiology

Abstract

The neurofibromatoses are a group of autosomal dominant diseases characterized by the formation of ectodermal and mesodermal tissue tumors [1]. These patients develop both benign and malignant tumors of the peripheral and central nervous system (CNS) and in other organs. Neurofibromas are benign tumors composed of neoplastic Schwann cells, nonneoplastic fibroblasts, mast cells, macrophages, pericytes, endothelial cells, and perineural cells [2]. Although the neurofibromatoses have common characteristics, two distinct forms have been recognized based on clinical and genetic findings, neurofibromatosis type 1 (NF1), formerly known as von Recklinghausen’s disease, and neurofibromatosis type 2 (NF2). Neurofibromatosis type 1 (NF1) is the most common form, but both could have multisystem involvement. Neurofibromas, the characteristic lesion of all forms, may complicate both general endotracheal anesthesia (GETA) and neuraxial anesthesia. The life expectancy of these patients appears to be shortened by approximately 8–15 years. The most common causes of death are from malignant peripheral nerve sheath tumors, central nervous system tumors, and vasculopathy [3].

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Correspondence to Ami Attali .

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Asif, A., Cook, S., Attali, A. (2018). Neurofibromatosis (NF) in Pregnancy. In: Mankowitz, S. (eds) Consults in Obstetric Anesthesiology. Springer, Cham. https://doi.org/10.1007/978-3-319-59680-8_111

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  • DOI: https://doi.org/10.1007/978-3-319-59680-8_111

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