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].
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Hirsch NP, Murphy A, Radcliffe JJ. Neurofibromatosis: clinical presentations and anaesthetic implications. Br J Anaesth. 2001;86(4):555–64.
Hirbe AC, Gutmann DH. Neurofibromatosis type 1: a multidisciplinary approach to care. Lancet Neurol. 2014;13(8):834–43.
Parsons CM, Canter RJ, Khatri VP. Surgical management of neurofibromatosis. Surg Oncol Clin N Am. 2009;18(1):175–96.
Terry AR, Barker Ii FG, Leffert L, Bateman BT, Souter I, Plotkin SR. Neurofibromatosis type 1 and pregnancy complications: a population-based study. Am J Obstet Gynecol. 2013;209:46.e1–8.
De Vitis LR, Tedde A, Vitelli F, Ammannati F, Mennonna P, Bono P, et al. Analysis of the neurofibromatosis type 2 gene in different human tumors of neuroectodermal origin. Hum Genet. 1996;97(5):638–41.
Cawthon RM, O’Connell P, Buchberg AM, Viskochil D, Weiss RB, Culver M, et al. Identification and characterization of transcripts from the neurofibromatosis 1 region: the sequence and genomic structure of EVI2 and mapping of other transcripts. Genomics. 1990;7(4):555–65.
Ramos-Zúñiga R, Saldaña-Koppel DA. Neurofibromatosis type 1 and pregnancy: the transformation of a nodular to cystic neurofibroma in the cervical region. Surg Neurol Int. 2015;6(Suppl 19):S487–9.
Mautner VF, Tatagiba M, Lindenau M, Fünsterer C, Pulst SM, Baser ME, et al. Spinal tumors in patients with neurofibromatosis type 2: MR imaging study of frequency, multiplicity, and variety. Am J Roentgenol. 1995;165(4):951–5.
Tadini G, Milani D, Menni F, Pezzani L, Sabatini C, Esposito S. Is it time to change the neurofibromatosis 1 diagnostic criteria? Eur J Intern Med. 2014;25(6):506–10.
Saif I, Seriki D, Moore R, Woywodt A. Midaortic syndrome in neurofibromatosis type 1 resulting in bilateral renal artery stenosis. Am J Kidney Dis. 2010;56(6):1197–201.
Cecchi R, Frati P, Capri O, Cipolloni L. A rare case of sudden death due to hypotension during cesarean section in a woman suffering from pheochromocytoma and neurofibromatosis. J Forensic Sci. 2013;58(6):1636–9.
Dham BS, Kwa DM, Campellone JV. Postpartum paraparesis from spinal neurofibroma. Spine J. 2012;12(7):e5–8.
Nelson DB, Greer L, Wendel G. Neurofibromatosis and pregnancy. Obstet Gynecol. 2010;116(Supplement):507–9.
Posma E, Aalbers R, Kurniawan YS, van Essen AJ, Peeters PMJG, van Loon AJ. Neurofibromatosis type I and pregnancy: a fatal attraction? Development of malignant schwannoma during pregnancy in a patient with neurofibromatosis type I. BJOG. 2003;110(5):530–2.
Geller M, Mezitis SGE, Nunes FP, Ribeiro MG, et al. Progesterone and estrogen receptors in neurofibromas of patients with NF1. Clin Med Pathol. 2008;1:93–7.
Xiong M, Gilchrest BA, Obayan OK. Eruptive neurofibromas in pregnancy. JAAD Case Rep. 2015;1(1):23–4.
Cesaretti C, Melloni G, Quagliarini D, Fogliani R, Zaina B, Bedeschi MF, et al. Neurofibromatosis type 1 and pregnancy: maternal complications and attitudes about prenatal diagnosis. Am J Med Genet Part A. 2013;161(2):386–8.
Dugoff L, Sujansky E. Neurofibromatosis type 1 and pregnancy. Am J Med Genet. 1996;66(1):7–10.
Segal D, Holcberg G, Sapir O, Sheiner E, Mazor M, Katz M. Neurofibromatosis in pregnancy. Maternal and perinatal outcome. Eur J Obstet Gynecol Reprod Biol. 1999;84(1):59–61.
Sharma JB, Gulati N, Malik S. Maternal and perinatal complications in neurofibromatosis during pregnancy. Int J Gynaecol Obstet. 1991;34(3):221–7.
Terry AR, Merker VL, Barker FG, Leffert L, Bateman BT, Souter I, et al. Pregnancy complications in women with rare tumor suppressor syndromes affecting central and peripheral nervous system. Am J Obstet Gynecol. 2015;213(1):108–9.
Jett K, Friedman JM. Clinical and genetic aspects of neurofibromatosis 1. Genet Med. 2010;12:1):1–11.
Mendonça FT, de Moura IB, Pellizzaro D, Grossi BJ, Diniz RC. Anesthetic management in patient with neurofibromatosis: a case report and literature review. Acta Anaesthesiol Belg. 2016;67(1):48–52.
Levy WJ, Latchaw J, Hahn JF, Sawhny B, Bay J, Dohn DF. Spinal neurofibromas: a report of 66 cases and a comparison with meningiomas. Neurosurgery. 1986;18(3):331–4.
Dounas M, Mercier FJ, Lhuissier C, Benhamou D. Epidural analgesia for labour in a parturient with neurofibromatosis. Can J Anaesth. 1995;42(5 Pt 1):420–4.
Spiegel JE, Hapgood A, Hess PE. Epidural anesthesia in a parturient with neurofibromatosis type 2 undergoing cesarean section. Int J Obstet Anesth. 2005;14(4):336–9.
Sakai T, Vallejo MC, Shannon KT. A parturient with neurofibromatosis type 2: anesthetic and obstetric considerations for delivery. Int J Obstet Anesth. 2005;14(4):332–5.
Ekwere IT, Edomwonyi NP, Imarengiaye CO. Anaesthetic challenges associated with achondroplasia: a case report. Afr J Reprod Health. 2010;14(142):149.
Naunheim MR, Plotkin SR, Franco RA, Song PC. Laryngeal manifestations of neurofibromatosis. Otolaryngol Head Neck Surg. 2016;154(3):494–7.
Cihangiroglu M, Yilmaz S, Topsakal C, Gok U, Altinsoy B, Cobanoglu B. Laryngeal neurofibroma associated with neurofibromatosis type 2. AJNR Am J Neuroradiol. 2002;23(10):1637–9.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2018 Springer International Publishing AG, part of Springer Nature
About this chapter
Cite this chapter
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
Download citation
DOI: https://doi.org/10.1007/978-3-319-59680-8_111
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-59679-2
Online ISBN: 978-3-319-59680-8
eBook Packages: MedicineMedicine (R0)