Management of anesthesia for surgery in prone position for a patient with hereditary neuropathy with liability to pressure palsies. A case report and narrative literature review

Keywords:

Hereditary neuropathy with liability to pressure palsies (HNPP); hereditary motor and sensory neuropathy; peripheral myelin protein 22 gene deletion (PMP22); intraoperative nerve injury; prone position; intraoperative care


Published online: Mar 29 2022

https://doi.org/10.56126/72.3.7

C.J. Moerman (*), T. Van Havenbergh (**), P. Van Houwe (***), S. Casaer (***)

Abstract

The reported case describes a patient with hereditary neuropathy with liability to pressure palsies (HNPP) requiring a neurosurgical laminotomy procedure for an arachnoid cyst with medullary compression. HNPP is a rare pathology requiring meticulous anesthetic and surgical management. We give a detailed overview of the planning, simulation, and performance of the anesthesia procedure in order to minimize all potential risk factors for perioperative nerve injury. During the preparation of this case, where the positioning was extremely challenging, we reviewed the available literature for guidance. A few papers report the use of neuraxial anesthesia in patients previously diagnosed with HNPP. No reports could be found on the management of general anesthesia and positioning for major surgery in those patients.