Current Considerations in the Management of Facial Nerve Palsy

REVIEW

CURRENT OPINION Current considerations in the management of facial nerve palsy

Charles Kim and Gary J. Lelli Jr

Purpose of review Facial nerve palsy is a potentially devastating condition that can arise from many different causes. Appropriate management is complicated by the wide spectrum of clinical presentation and disease severity that characterizes this condition. As such, recent studies have focused on augmenting our understanding of the underlying anatomy and pathophysiology of facial nerve palsy, while also exploring different treatment options. Recent findings There have been a multitude of radiologic investigations that have delineated anatomical considerations pertinent to facial neuropathy, whereas various grading schemes and software programs have been developed to facilitate the clinical assessment of patients. Furthermore, a wide variety of medical and surgical treatment options have been proposed – whereas some are variants of previously described methods, others represent novel approaches. Summary Appropriate management of facial nerve palsy is dependent on a multitude of factors and must be tailored to patients on an individual basis. The studies summarized in this article highlight the recent advancements geared toward refining the assessment and treatment of patients with facial neuropathy. Keywords Bell’s palsy, exposure keratopathy, facial nerve palsy, facial synkinesis

INTRODUCTION Ramsay Hunt syndrome, herpes simplex virus, The facial nerve (cranial nerve VII) is intimately human immunodeficiency virus, Lyme disease, and involved in the innervation of the facial muscles. meningitis, as well as conditions such as sarcoidosis As a result, any degree of dysfunction can have and Gullian–Barre syndrome. However, the vast significant functional and aesthetic ramifications. majority of cases are idiopathic in nature; in a Given the wide-reaching clinical manifestations retrospective review published in 2002, Peitersen of facial neuropathy, appropriate treatment often [1] found that 70% of facial palsy cases are ultimately involves a multidisciplinary approach. Ophthalmo- diagnosed as Bell’s palsy. logists are frequently called upon to optimize Chronic systemic conditions such as hyper- the health and function of the cornea, which can tension and diabetes mellitus have been implicated be compromised in the setting of inadequate blink- as risk factors for Bell’s palsy. Jorg et al. [2] recently ing and malpositioning of the midface and eyelid. performed a systematic review of facial neuropathy This review will focus on the current concepts in the setting of severe hypertension and found that pertinent to the ocular assessment and management this association was strongest in children – the of patients with facial nerve dysfunction. We will median age in their cases was 9.5 years. In addition, briefly review some well established treatment modalities, while highlighting medical and surgical advancements that have been published over the Department of Ophthalmology, Weill Cornell Medical College, New York, last 12 months. New York, USA Correspondence to Gary J. Lelli Jr., MD, Department of Ophthalmology, Weill Cornell Medical College, 1305 York Avenue, 12th Floor, New York, NY 10021, USA. Tel: +1 646 962 3182; fax: +1 646 962 0602; e-mail: CAUSE [email protected] Facial neuropathy can result from a wide variety Curr Opin Ophthalmol 2013, 24:478–483 of causes, including infectious processes such as DOI:10.1097/ICU.0b013e3283634869

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KEY POINTS Table 1. House–Brackmann grading system Grade Features Facial nerve palsy can arise from a multitude of causes, although most cases are idiopathic. I. Normal Normal facial function in all areas Patients with facial neuropathy can exhibit a wide II. Mild Slight weakness noticed on close inspection spectrum of clinical manifestations owing to the dysfunction complex innervation pattern of the facial nerve. No synkinesis, contracture, or hemifacial spasm Recently published radiographic studies have focused Normal symmetry and tone at rest on developing a better understanding of the underlying Moderate-to-good forehead function anatomic considerations pertinent to facial neuropathy. Complete eye closure with minimal effort A variety of nonsurgical treatment modalities, ranging Slight asymmetry of mouth from scleral contact lenses to systemic steroids, has III. Moderate Obvious but not disfiguring difference been explored and described in the literature. dysfunction between the sides Surgical management of patients with facial neuropathy Noticeable but not severe synkinesis, encompasses both static and dynamic techniques, contracture, or hemifacial spasm which are directed at addressing the periocular Normal symmetry and tone at rest changes seen in these patients. Slight-to-moderate movement of forehead Complete but asymmetric eye closure with effort Slightly weak mouth movements with Riga et al. [3] examined a group of 56 patients with maximum effort Bell’s palsy and found that patients with abnormal IV. Moderately Obvious weakness and disfiguring hemoglobin A1c values had more severe presenta- severe asymmetry tions [House–Brackmann grade V/VI (Table 1)] than dysfunction nondiabetics, although this did not appear to have Normal symmetry and tone at rest prognostic ramifications. No forehead movement Incomplete eye closure ANATOMY Asymmetry of mouth with maximum effort V. Severe Only barely perceptible motion Cranial nerve VII has a multitude of sensory dysfunction and motor functions (Table 2). The motor fibers Asymmetry at rest originate in the tegmentum of the caudal pons No forehead movement and exit at the pontomedullary junction, at which Incomplete eye closure with only slight point they run separately from the sensory fibers movement of lid with maximal effort prior to their joint entry into the internal acoustic Slight movement of corner of mouth canal as the facial nerve. & VI. Total No movement Kondo et al. [5 ] performed morphometric paralysis analyses of the facial nerve and found a significant decrease in the number of myelinated axons with Adapted with permission [4]. age (r ¼0.77; P < 0.01), perhaps explaining the increased susceptibility of elderly patients to Bell’s the canal were significantly smaller on the affected palsy, as well as their delayed recovery course. There side of patients with Bell’s palsy compared with the was no change in the transverse area of individual contralateral side. Furthermore, they also confirmed myelinated axons over time (r ¼0.01; P ¼ 0.96). that the labyrinthine segment is the most narrow In addition, there was no difference in the number portion of the facial canal. or transverse area of axons between affected and Vaid et al. [7] used HRCT and MRI to demon- unaffected sides or based on sex. strate the clinical significance of the geniculate The facial nerve runs 20–30 mm within the fossa, which they found to be involved in a multi- facial canal, representing the longest bony course tude of pathologic processes – including infections, of any cranial nerve. As a result, it is particularly trauma, schwannoma, hemangioma, meningioma, susceptible to injury from trauma and edema. and malignancy – responsible for causing facial Murai et al. [6] utilized high-resolution computed nerve palsy. Furthermore, enlargement of the geni- tomography (HRCT) with multiplanar reconstruc- culate fossa may indicate an underlying fracture tion to demonstrate that the mean cross-sectional in the setting of traumatic facial paralysis, as area of the labyrinthine and horizontal segments of demonstrated by Mu et al. [8].

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Table 2. Sensory and motor functions of cranial nerve VII

Sensory Motor

External ear (via nervus intermedius) Muscles of facial expression (including orbicularis and frontalis) Taste fibers on anterior two-thirds of tongue Posterior belly of digastric muscle (via chorda tympani) Oropharynx (below palatine tonsil) Stylohyoid muscle Stapedius muscle (middle ear) Lacrimal secretion (via pterygopalatine ganglion) Salivary secretion (via chorda tympani)

Upon its exit from the stylomastoid foramen, Computer Evaluation software is an accurate and the facial nerve becomes extracranial. It passes sensitive tool for obtaining objective measures of between the superficial and deep lobes of the parotid facial position. gland, where it divides into the temporofacial The primary ocular manifestations of facial and cervicofacial trunks, prior to further division nerve palsy are related to exposure keratopathy that into the temporal, zygomatic, buccal, mandibular, can develop in response to the lagophthalmos and cervical branches. The temporal and zygomatic and paralytic ectropion often seen in these patients. branches then innervate the orbicularis oculi, As a result, careful attention must be paid to the whereas the buccal and cervical branches contribute positioning of the upper and lower eyelids, as well to supplying the inferior orbicularis. as the force, frequency, and velocity of the blink Recent studies have described different response, and the severity of lagophthalmos. approaches to accessing the facial nerve and its These anatomical considerations are particularly branches. For example, Chatellier et al. [9] found vital in dictating the nature and extent of treatment, that the zygomatic branch of the facial nerve could as discussed below. be reliably located using a reference point 2.5 cm The tearing often experienced by patients anterior to the intertragic notch. Similarly, Feng with facial neuropathy can be multifactorial in et al. [10] utilized HRCT to describe an optimized cause, with a reflexive component stemming from surgical approach to the vertical segment of the exposure and poor production and distribution of facial nerve by implementing the promontorium the tear film, as well as epiphora related to abnormal tympani and tympanic ring as reference points. eyelid positioning and lacrimal pump failure. Furthermore, Call et al. [14&] utilized infrared video meibography to show that weakness of CLINICAL ASSESSMENT the orbicularis oculi can induce morphological Originally described in 1985, the House–Brackmann changes in the lower eyelid, resulting in increased Grading System (HBGS) provides clinicians with meibomian gland dysfunction. These findings were a standardized method of assessing patients with corroborated by the work of Shah et al. [15] , who facial paralysis and monitoring their clinical course demonstrated significant differences in lower eyelid (Table 1) [4]. Although other systems have also been meibomian gland function, tear break-up time, described, the HBGS remains widely favored because eyelid abnormality, and digital pressure in eyes of its relative simplicity and ease of use. affected by cranial nerve VII palsy compared with In 2013, Alicandri-Ciufelli et al. [11] described control. the Rough Grading System (RGS), an even more Although imaging may potentially be diag- simplified scale comprised of six grades in order nostic, Jun et al. [16] did not find any prognostic of increasing severity. They found that the RGS value in obtaining MRI imaging in the setting of provided a slightly higher level of interobserver acute idiopathic facial nerve palsy, as the degree of agreement than the HBGS (0.59 vs. 0.46) and facial nerve enhancement did not correlate signifi- reported a high degree of correlation between the cantly with the HBGS during the early or late stages two systems. of the condition. Lee et al. [12] described a strong correlation between the HBGS and Facogram, a digital scoring system designed to provide more objectivity TREATMENT to clinical assessment without the need for any Many factors are involved in determining the direct clinician time. Similarly, Hadlock and Urban appropriate approach to the treatment of patients [13] demonstrated that the Facial Assessment by with facial neuropathy, including the underlying

480 www.co-ophthalmology.com Volume 24 Number 5 September 2013 Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. Management of facial nerve palsy Kim and Lelli cause, expected duration of nerve dysfunction, of orbicularis oculi function. However, the func- anatomical manifestations, severity of symptoms, tional blink recovery in the steroid group only and objective clinical findings. reached 64% of that seen in the control group over the 84-week follow-up period (36% recovery in the untreated group). Similarly, Berg et al. [22] demon- Medical and nonsurgical management strated significant improvement on treatment with Lubrication with artificial tears and ophthalmic steroids in patients with mild-to-moderate presen- ointment remains the mainstay of treatment for tations (HBG I/II). most patients, and is typically considered the first line. These measures can be further augmented with Surgical management the use of an eye patch or moisture chamber. Scleral contact lenses have recently been A multitude of surgical options are available for explored as a method of protecting the cornea from patients afflicted with facial nerve palsy. Adequate exposure keratopathy. Gire et al. [17&&] published closure of the eyelids can be accomplished via static a case series consisting of four patients with and dynamic procedures involving the upper and facial neuropathy and severe exposure keratopathy lower eyelids. who experienced dramatic corneal stabilization and restoration with significant improvement in visual Static techniques acuity following placement into prosthetic replace- Tarsorrhaphy (temporary or permanent) can be ment of the ocular surface ecosystem (PROSE) performed to address lagophthalmos and associated devices. The PROSE device is a gas-permeable scleral exposure keratopathy, using either a medial or a lens comprised of fluorosilicone-acrylate polymers lateral approach. In addition, ectropion repair is that is filled with preservative-free saline solution. commonly indicated in the setting of facial nerve Weyns et al. [18&&] reported similar success in a group palsy, given the frequency of paralytic ectropion of three patients with postsurgical facial paralysis that is seen in this population of patients. who were managed with scleral contact lenses. Repair can be accomplished through a tarsal strip Recent studies have also explored the efficacy of procedure, which may also involve a medial spindle botulinum toxin as an independent therapeutic operation to address punctal ectropion. modality. Yucel and Arturk [19] reported signifi- Iyengar and Burnstine [23] reported the use of cant improvement in the corneal symptoms and fascia lata suspension of the lower eyelid as the decreased use of artificial tears in 15 patients with primary intervention in a group of 22 patients facial neuropathy who received an injection of with facial nerve palsy. They noted symptomatic 7.5 units of botulinum toxin-A directly into the relief in all patients as well as improvement levator palpebrae superioris muscle. The duration in lagophthalmos (6.5–2.9 mm), while maintaining of induced ptosis was found to exceed 10 weeks. excellent aesthetic results. Whereas previous groups have explored the Concurrent lower eyelid retraction can be utility of magnetic implantation in patients with addressed with the use of subtarsal spacers designed facial neuropathy, Barmettler and colleagues recently to elevate the lower eyelid margin. However, described a temporary nonsurgical magnetic midface elevation and skin grafting may be necess- tarsorrhaphy system involving the use of magnet- ary if the amount of anterior lamella is inadequate. embedded spectacles on normal individuals with Furthermore, biomaterials can be used in lieu of skin magnets also affixed to their upper eyelid. They grafting in patients with poor tissue quality. Borrelli found that magnet fixation to the eyelid did not et al. [24] recently described the use of decellularized affect palpebral fissure height and that all 13 indi- porcine-derived membrane (Tarsys) to correct lower viduals in their study had improved eyelid closure, eyelid retraction in a patient with facial nerve palsy. with 10 achieving complete closure (A.R.M. Barmet- Initially described by Smellie in 1966, implan- tler et al., IOVS 2012;1037:ARVO E-Abstract D1242). tation of gold weights into the upper eyelid is a well In 2012, the American Academy of Neurology established method of diminishing lagophthalmos. released an updated guideline report stating that Braun et al. [25] also reported excellent results using systemic steroids should be offered to patients with platinum weights in this fashion. new-onset Bell’s palsy to increase the chance of An alternative approach to the treatment facial nerve recovery (Level A recommendation) of lagophthalmos includes levator lengthening. [20&&]. In concert with this recommendation, Originally described by Tessier in 1969, levator VanderWerf et al. [21] found that treatment with lengthening via aponeurosis interposition was oral prednisolone within 5 days of symptom successfully utilized by Guillou-Jamard et al. [26] onset accelerated the electromyographic recovery in a group of 29 patients. This procedure can also

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be performed in conjunction with an external Facial synkinesis blepharorrhaphy in order to elevate the lower eye- Following recovery from Bell’s palsy, 15–20% of lid, as described by Krastinova-Lolov [27,28]. patients develop facial synkinesis [35,36], stemming First described by Ballance and Duel in 1932, from aberrant nerve regeneration. Facial synkinesis decompression of the facial canal represents another is characterized by findings such as eyelid option for surgical intervention. Hato et al. recently closure and epiphora with mastication, as well as reported their experiences decompressing the tym- aberrant facial and neck contraction with volitional panic and mastoid segments of the canal with con- movements. current placement of basic fibroblast growth factor- Terzis and Karypidis [37&&] reported a study impregnated biodegradable gelatin hydrogel around consisting of 31 patients suffering from facial syn- the nerve. Within their group of 20 patients, they kinesis. They found that various combinations of found that the rate of complete recovery was 75.0%, cross-facial nerve grafting, secondary microcoapta- compared with 44.8% by the conventional decom- tions, Botox, and biofeedback could be utilized to pression method and 23.3% on steroids alone [29]. effectively manage synkinesis. They also reported similar findings in the setting of pediatric patients Dynamic techniques [38]. In 1934, Gilles described a method of achieving dynamic eyelid closure that involved the transfer and extension of pedicled temporalis muscle across CONCLUSION the upper and lower eyelids to the medial canthal The studies on facial nerve palsy outlined above ligament, allowing blinking to be triggered by represent only a small fraction of the articles that mastication. Since this report, many additional have been published on the subject over the last methods of dynamic eyelid closure have been 12 months. Nevertheless, these studies enhance described in the literature. our understanding, characterization, and treatment Dynamic correction of paralytic lagophthalmos of patients with facial neuropathy, and will frequently involves transfer of the temporalis undoubtedly serve as the impetus for the develop- muscle, which is effective and can provide strong ment of new treatment modalities in the years to eyelid closure over an extended period of time [30]. come. Given the vast array of treatment options that Kurita et al. [31] performed a transfer of the tempo- are currently available, it is vital for the ophthalmo- ralis muscle to the eyelid in a group of three patients logist to apply an individualized management plan who had previously undergone dynamic smile that accounts for the complex manifestations of reconstruction using the temporalis. Each patient facial nerve palsy as well as the goals and desires also underwent multiple static procedures for eyelid of the patient. closure and concurrent latissimus dorsi muscle transfer to replace cheek movements. Successful Acknowledgements eyelid closure was achieved in all three cases. None. Reanimation of paralyzed muscles using adjacent motor nerves has also been well documented. Conflicts of interest Hayashi et al. [32] performed an anastomosis of the Neither author has received any grants or has any hypoglossal and facial nerves for facial reanimation conflicts of interest related to the content of this article. in a set of 36 patients with facial paralysis following The authors do not have any financial interests to excision of large vestibular schwannomas. They declare. achieved excellent success rates, which were depend- This research received no specific grant from any funding ent on the location of nerve splitting, although they agency in the public, commercial, or not-for-profit did note a high rate of tongue atrophy in their series. sectors. Interestingly, Corrales et al. [33] used hypoglossal– facial nerve anastomosis to treat patients with central facial nerve palsies and achieved successful REFERENCES AND RECOMMENDED reanimation rates that were comparable with those READING treated for peripheral dysfunction. Papers of particular interest, published within the annual period of review, have been highlighted as: The masseter nerve can also be utilized for the & of special interest purposes of facial nerve grafting. Hontanilla and && of outstanding interest Additional references related to this topic can also be found in the Current Marre [34] found that reanimation via direct World Literature section in this issue (p. 518). masseteric–facial coaptation led to better symmetry 1. Peitersen E. Bell’s palsy: the spontaneous course of 2500 peripheral and faster onset of movement compared with facial nerve palsies of different etiologies. Acta Otolaryngol Suppl 2002; hemihypoglossal transposition. 549:4–30.

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