VNS implantation in a NF1 patient: massive nerve hypertrophy discovered intra-operatively preventing successful electrod

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CASE REPORT - FUNCTIONAL NEUROSURGERY - EPILEPSY

VNS implantation in a NF1 patient: massive nerve hypertrophy discovered intra-operatively preventing successful electrode placement. Case report Hussein Hamdi 1,2,3 & Gilles Brun 4 & Marc Zanello 5 & Maxine Dibué 6 & Yassine Beltaifa 1 & Stanislas Lagarde 7 & Agnes Trébuchon 7 & Fabrice Bartolomei 7 & Romain Carron 1,2 Received: 20 May 2020 / Accepted: 11 August 2020 # Springer-Verlag GmbH Austria, part of Springer Nature 2020

Abstract For the vast majority of surgeons, no specific investigation is necessary before vagal nerve stimulation (VNS) implantation. We report our intraoperative unexpected finding of a massively enlarged vagus nerve in a patient with neurofibromatosis type 1 (NF1). The nerve hypertrophy prevented wrapping the coils of the helical electrode. The patient had no signs of vagus nerve dysfunction preoperatively (no hoarseness or dysphonia). This exceptional mishap is undoubtedly related to NF1-associated peripheral nerve sheath tumors. Even though it is not advisable to routinely perform any imaging prior to VNS, in such specific context, preoperative imaging work-up, especially cervical ultrasound, might be judicious to rule out any asymptomatic enlarged left vagus nerve. Keywords Vagal nerve stimulation . Neurofibromatosis type 1 . Von Recklinghausen’s disease . Imaging . Cervical ultrasonography

Introduction Vagal nerve stimulation (VNS) is a well-established option for pharmaco-resistant epilepsy [10] [4] and chronic and recurrent depression [20] [6]. In experienced hands, This article is part of the Topical Collection on Functional Neurosurgery - Epilepsy * Romain Carron [email protected] 1

Department of Functional and Stereotactic Neurosurgery, Timone University Hospital, Aix Marseille University, Marseille, France

2

APHM, INSERM, INS, Inst Neurosci Syst, Aix Marseille University, Marseille, France

3

Functional Neurosurgery and Stereotaxy Unit, Neurological Surgery Department, Tanta University, Tanta, Egypt

4

Department of Neuroradiology, Timone University Hospital, Aix Marseille University, Marseille, France

5

Department of Neurosurgery, Sainte-Anne Hospital, Paris Descartes University, Sorbonne Paris Cité, Paris, France

6

Department of Neurosurgery, Heinrich Heine University, Düsseldorf, Germany

7

Epileptology Department, Timone Hospital, APHM, INSERM, INS, Inst Neurosci Syst, Aix Marseille University, Marseille, France

it is usually performed easily and is associated with a very low rate of complications [16] [13]. The surgery itself usually does not warrant any preoperative imaging, the appropriate diameter of the lead to be implanted being determined intra-operatively after dissection of the nerve. Some authors [1] [25] advocate to perform a laryngoscopy preoperatively to check the functional integrity of vocal cord mobility. The aim is mainly to rule out any pre-existing dysfunction that the procedure may deteriorate further, but to the best of our knowledge, there are no current recommendations of any preoperati