Neurofibromatosis during pregnancy, what about anesthesia?

Neurofibromatosis type 1 is a rare neurocutaneous condition that can have widespread and deleterious effects on various organ systems, including the spine with implications for the choice of anesthetic technique. We report the case of a parturient, who was received in the emergency room of the maternity ward of our hospital during the active phase of labor, without neuraxial imaging, in whom a cesarean section was indicated and performed under general anesthesia. Anesthesia for the parturient with neurofibromatosis is still not well documented, its management needs to be multidisciplinary, with a careful pre-conception planning. Key words: Neurofibromatosis, imaging, neuraxial anesthesia, pregnancy. Abbreviations: Neurofibromatosis type 1: NF-1 Citation: Amri AE, Elabdi F, Elkoundi A, Elhasnaoui MA. Neurofibromatosis during pregnancy, what about anesthesia? Anaesth. pain intensive care 2020;24(6):__ Received: 3 August 2019, Reviewed: 7 May 2020, 27 May 2020

Continuous Incremental Heavy Halo Traction Combined with Posterior-only Approach for Severe Cervical Kyphosis with Neurofibromatosis-1.

Background Surgical management of cervical kyphosis in patients with NF-1 is a challenging task. Presently, anterior-only (AO), posterior-only (PO) and combined anterior-posterior (AP) spinal fusion are common surgical strategies. However, the choice of surgical strategy and application of Halo traction remain controversial. Few studies have shown and recommended posterior-only approach for cervical kyphosis correction in patients with NF-1. The aim of this study is to evaluate the safety and the effectiveness of Continuous-Incremental-Heavy Halo Traction (CIH-HT) combined with posterior-only approach for treatment of cervical kyphosis with NF-1. Methods 19 patients with severe cervical kyphosis due to NF-1 were reviewed retrospectively between January 2010 and April 2017. All the cases underwent CIH-HT combined with posterior instrumentation and fusion surgery. Correction result, neurologic status and complications were analyzed. Results In this study, cervical kyphosis Cobb angle decreased from initial 63.0 ± 21.0 degrees to postoperative 10.8 ± 4.0 degrees(P＜0.01),with total correction rate of 92%, which consist of 44% from CIH-HT and 48% from surgical correction. JOA scores were improved from preoperative 13.6±1.6 to postoperative 16.0±1.0(P＜0.01). Neurological status was also improved. There was no correction loss and the neurological status was stable in mean 3.7 years follow-up. The incidence of complications was 36.8% (7/19). Six patients underwent local complications and one patient underwent a second surgery. Conclusion CIH-HT combined PO approach is safe and effective method for cervical kyphosis correction in patients with NF-1. A satisfied correction result, and successful bone fusion can be achieved via this procedure, even improvement of neurological deficits can also be obtained. Our study suggested that CIH-HT combined PO approach is another consideration for cervical kyphosis correction in patients with NF-1. Key words : Neurofibromatosis-1; Cervical kyphosis; Continuous-Incremental-Heavy Halo Traction; posterior-only approach;