Anesthetic management of a parturient with segmental neurofibromatosis

Kevin Blackney,Mark J Mckeen,Yvonne Lai

doi:10.7243/2049-9752-3-5

Abstract

Abstract Segmental neurofibromatosis is a rare, but likely under reported form of neurofibromatosis. The traditional definition is that of dermatologic manifestations of neurofibromatosis, including neurofibromas and cafe-au-lait

Highlights

Segmental neurofibromatosis (SNF) is a rare form of neurofibromatosis with an estimated prevalence of 1 in 40,000 [1]. It typically presents with isolated dermatologic findings of neurofibromatosis type 1 (NF1), bilateral, deep tissue, multi-segmental, and systemic involvement is possible [2-7]
She had not had any prior neuraxial imaging, and evaluation of her condition was limited to dermatology and a genetics assessment
Induction of labor continued with misoprostol, but after receiving her second dose, the fetus developed persistent, late decelerations, and the decision was made to proceed with urgent cesarean delivery

Summary

Introduction

Segmental neurofibromatosis (SNF) is a rare form of neurofibromatosis with an estimated prevalence of 1 in 40,000 [1]. The patient’s initial diagnosis of SNF was made several years prior and was based on left axillary freckling with multiple tan macules and patches, as well as a patch on her left breast with a dark brown macule She had not had any prior neuraxial imaging, and evaluation of her condition was limited to dermatology and a genetics assessment. Once a more extensive understanding of SNF was obtained, the patient was re-evaluated, focusing on more extensive questioning of her neurologic status She denied any leg weakness, paresthesias, back pain, dizziness, or headaches. Induction of labor continued with misoprostol, but after receiving her second dose, the fetus developed persistent, late decelerations, and the decision was made to proceed with urgent cesarean delivery In this setting, the anesthesia team carefully evaluated the risks and benefits of neuraxial vs general anesthesia. The remainder of her hospital stay was unremarkable and she was discharged home with no change from her preoperative neurological status

Discussion

Findings

Conclusion