© Springer International Publishing AG 2018
George B. Wanna, Matthew L. Carlson and James L. Netterville (eds.)Contemporary Management of Jugular Paragangliomahttps://doi.org/10.1007/978-3-319-60955-3_1313. Cranial Nerve VII Rehabilitation
(1)
Department of Otolaryngology, Vanderbilt University, 1215 21st Avenue South, Nashville, TN 37232, USA
Keywords
MicrosurgeryNeurotologySkull base surgeryFacial nerveCranial nerveRehabilitationParagrangliomaGlomus tumorSurgeryRadiationOtologyJugular veinTemporal boneFacial nerveReanimationNerve graftIntroduction
Jugular paragangliomas (JPs) arise from the paraganglion cells located within the adventitia of the jugular bulb [1]. Although typically slow growing and histologically benign, JPs commonly invade the middle ear, mastoid, and neural compartment of the jugular foramen, thereby causing facial nerve and lower cranial nerve (IX, X, XI, XII) deficits. Furthermore, operative intervention and radiotherapy may also result in cranial nerve deficits, and it is important to be aware of and manage cranial nerve injury to minimize their clinical impact on the patient.
In this chapter, we will discuss management of CN VII paralysis and facial nerve rehabilitation. Lower cranial nerve deficits will be addressed in the following chapter when discussing the management of speech and swallowing dysfunction (CN IX–XII) in the setting of JPs.
Risk of Injury
Tumor Invasion
Preoperative cranial neuropathies are common findings, occurring in 10–50% of patients diagnosed with JPs [1, 2]. The lower cranial nerves are most commonly involved; however, facial nerve involvement occurs in as high as 10% of patients [3, 4]. This functional deficit is most commonly caused by direct invasion of the vertical (mastoid) segment of the facial nerve, and symptoms can include a wide range of findings including frank paralysis or paresis to more subtle facial twitching or hemifacial spasm [5].
Posttreatment Deficit
Treatment practices for JPs vary among physicians and institutions; however, when intervention is required, management consists of total or subtotal surgical excision and/or radiotherapy. When surgery is performed, preoperative embolization of the lesion is also often utilized. All treatment options have risks and benefits that must be considered as they relate to each individual patient; here we will focus on the implications for facial nerve function.
Selective transfemoral arterial embolization of the tumor’s feeding vessels is utilized to decrease operative blood loss which allows for better visualization during resection and reduction in operative time. This is thought to decrease postoperative morbidities and improve the chances of achieving a total resection [1]. At the author’s institution, we most commonly embolize with a nonadhesive mixture of ethylene vinyl alcohol, dimethyl sulfoxide (DMSO), and micronized tantalum powder (Onyx; Covidien, Ireland). Cranial neuropathies, including CN VII weakness, have occurred as a direct result of embolization. Gartrell et al. reported a ~ 6% theoretical risk that a patient may harbor a vascular pattern that increased the risks of ischemic injury to the facial nerve if embolization takes place. The facial nerve becomes more vulnerable to ischemic injury when its extratemporal segment derives its vascular supply solely from the stylomastoid artery, a branch from the occipital artery. Similarly, in patients without this vascular pattern, ischemic injury becomes more likely with increasing number of vessels embolized [6].
Surgical removal of tumor poses a more direct risk to the facial nerve. Decisions balancing surgical exposure, extent of tumor removal, and protection of the facial nerve are common surgical dilemmas. Postoperative facial nerve paralysis has been reported as high as 25.5% in patients who underwent total and subtotal resections of JPs [7, 8].
Facial nerve function is also placed at increased risk when facial nerve rerouting is performed to enhance surgical exposure for tumor resection. When compared to leaving the facial nerve in a fallopian bridge (i.e., non-rerouting), short rerouting techniques (mobilizing the mastoid segment from stylomastoid foramen to the second genu) result in similar facial nerve function outcomes. However, when long anterior rerouting (mobilizing the facial nerve from the stylomastoid foramen to the geniculate ganglion), a substantial decline in postoperative facial nerve function is noted. A recent literature review by Odat et al. that included 15 studies and 688 patients examined this issue. Patients in the non-rerouting and short rerouting groups had postoperative House-Brackmann scores of I–II in 95% and 90%, respectively, whereas only 67% of patients in the long rerouting group retained a House-Brackmann score of I–II [9]. This greater risk of facial nerve dysfunction with greater mobilization may reflect the extent of tumor burden and/or vascular and traumatic changes as a result of mobilization, and decisions must be made on a case-by-case basis. The values provide a guide for operative planning and patient discussions regarding treatment options and anticipated outcomes.
Radiotherapy is a well-validated alternative to surgery that has evolved greatly over the past two decades due to its effectiveness and reported lower morbidity when compared to radical surgical resection [7, 10]. Overall tumor control rates have been reported between 89–100% in the literature, and because of this, radiosurgery is sometimes preferred as the primary treatment modality in select patients [8, 11, 12] Although radiosurgical techniques have limited morbidity, complications may still occur in a small percentage of patients. The most commonly reported cranial nerve complication occurs in CN VIII and lower cranial nerves. One study by Scheick et al. looked at long-term complications of stereotactic radiosurgery with a mean follow-up of 5.3 years. This study showed 7 of 11 (64%) patients reporting worsening CN VIII function, 3 patients (27%) having deficits in CN IX and X, and a 10% rate of injury to CN VII, XI, and XI [12]. Additionally, a meta-analysis by Ivan et al. supported these rates of lower cranial nerve injury by reporting CN IX–XII injury ranging from 9–12% of cases treated with stereotactic radiosurgery [8, 11]. There are studies, however, that have also shown stability and even improvement of cranial nerve function, including improvement in CN VII10. Due to the heterogeneity of reporting results of facial nerve function. House-Brackmann grading is difficult to quantify and compare across these studies, and long-term data are required to further characterize tumor control and functional outcomes.
Facial Nerve Repair and Reanimation
As treatment techniques have evolved, the methodology of facial nerve management has advanced as well. As a result of refined radiotherapy, subtotal resection has become a reliable and attractive option for surgeons, especially for tumors intimately involved with the facial nerve or lower cranial nerves [7]. However, facial nerve paresis, from rerouting or from unintentional injury, may occur, and a thorough understanding of repair techniques and management practices will enable appropriate rehabilitation when required.
Facial Nerve Repair and Reinnervation Procedures
Primary Anastomosis
The importance of maintaining facial function combined with improved adjuvant therapies has enabled a more conservative approach toward disease intimately involved with the facial nerve. Although surgical injury or resection is becoming less common, intraoperative sacrifice of the facial nerve still may be required in some select cases. In these circumstances, the best course of action is primary coaptation when tension-free anastomosis is possible. Primary anastomosis, rather than interposition nerve graft, limits the number of splice interfaces to one rather than two. Both suture techniques as well as fibrin glue have been described when anastomosing the intratemporal segment of the facial nerve [13–15]. Typically two or three interrupted 8–0 or 9–0 monofilament sutures are placed through the epineurium to provide approximation and alignment of the fascicular units. However, the intratemporal and intracranial segments of the facial nerve do not have an epineurium layer to assist with anastomosis. In these circumstances both suture repair and sutureless anastomosis techniques have been described. When sutures are used, they are placed superficially through the perineurium with attempts to avoid disruption of the fascicular units as much as possible. In areas where bony anatomy can assists with keeping the nerve fascicles of the proximal and distal ends of the nerve in alignment, absorbable materials such as Gelfoam (Pfizer Inc., New York, NY), collagen matrix, and fibrin adhesive have been used to assist with neural coaptation [15, 16].
Intratemporal facial nerve mobilization can provide added length when required. However, the blood supply received from the periostium of the fallopian canal is compromised with rerouting, placing theoretic limits on the length of consequence-free mobilization. Despite this limitation, primary anastomosis is thought to be optimal for resected segments 18 mm or less [15]. When tension-free closure is not possible, other repair techniques must be employed.
Interposition Graft
Interposition grafting is the next best option to primary repair. Multiple donor nerves have been described with the most common being the great auricular nerve, medial antebrachial cutaneous nerve, and sural nerve. The great auricular nerve has the advantage of anatomic proximity to JPs and is often times within the surgical field. The path of the great auricular nerve is classically described as originating midway along the SCM coursing superiorly along the lateral aspect of the muscle for approximately 6 cm until it branches into anterior and posterior branches [17]. The medial antebrachial cutaneous nerve and sural nerve provide the advantage of length and are able to provide up to 25 cm of nerve for grafting. The harvesting of these latter nerves requires separate surgical sites with additional morbidities; however, using stair-step incisions provide acceptable cosmetic results. When interposition grafts are anticipated, it is important to counsel patients preoperatively on hypesthesia and loss of sensation in sensory distributions of graft options. The periauricular area is likely to be numb as a result of extensive surgery around the auricle, again favoring the greater auricular nerve. Dorsolateral foot and medial/ulnar surface of the forearm, though well tolerated and “worth” the trade-off for a good donor nerve, are an additional morbidity to be evaluated in the context of patient preference.
With primary repair and interposition grafting, initial return of facial function can be seen at 6 months postoperatively with maximal facial function taking as long as 12–18 months [15]. For these reasons, additional facial reanimation procedures are not typically considered until 1 year postoperatively when satisfactory results have not been obtained. A satisfactory and optimal result in the setting of interposition grafting is considered a House-Brackmann score of III or IV. Therefore, adjuvant procedures may be indicated depending on individual patient’s results and personal expectations.
Transposition Grafts
Transposition grafts are an excellent option for facial nerve reinnervation and are often used when a suitable proximal nerve stump is not available, when interposition grafting has failed, or when facial function has not returned in cases where the nerve was not transected. The timing of this technique is an important factor to consider. Although the utility of earlier intervention is under investigation, most clinicians will allow 12–18 months to pass prior to implementing transposition grafting for dynamic facial reanimation. Proponents for earlier repair site the risk of distal motor end plate degeneration as a reason to intervene sooner; however, this must be balanced with the risk of interrupting a regenerating facial nerve. Electromyography (EMG) can be used to help investigate the progress of neural regeneration. EMG begins to demonstrate fibrillation potentials approximately 2–3 weeks after nerve transection, signifying viable but denervated muscle end plates. As neural regenerating is taking place, EMG demonstrates polyphasic action potentials and observation for facial nerve recovery should be considered. For successful reinnervation, a functional motor end plate-muscle unit must be present; therefore, patients are offered transposition grafting when preoperative work-up reveals fibrillation potentials without signs of polyphasic action potentials on EMG. Conversely, if both fibrillation and polyphasic action potentials are absent, there is irreversible fibrosis and facial muscle atrophy resulting in poor candidacy for reinnervation procedures [15, 17].
Several cranial nerves have been evaluated for use in transposition grafting due to the anatomic location and robust amount of myelinated motor axons. Ipsilateral cranial nerves V, IX, XI, and XII have been used in addition to the contralateral cranial nerve VII [17]. A special consideration that must be made with JPs is that ipsilateral lower cranial nerves may not always be suitable given the potential for disease- and treatment-related injury. Furthermore, paresis of the hypoglossal nerve from transposition in a patient that already has a high vagal nerve injury may have devastating consequences to upper aerodigestive function. As with harvesting sensory nerves for interposition grafts, motor deficits with transposition grafting are important to discuss preoperatively with patients and may need to be addressed postoperatively to limit the morbidity associated with resection of these motor nerves.