Congenital Facial Paralysis Treatment & Management

Updated: Jan 07, 2019
  • Author: Alan D Bruns, MD, FACS; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Treatment

Medical Therapy

Immediate medical treatment of congenital facial paralysis requires attention to eye care. Instill artificial tears in the eyes of a child every hour while the child is awake. Use ointment when the child is sleeping. Care must be taken when taping the eye and using patches to prevent the eyelashes from abrading the cornea. Frequent ophthalmologic evaluations are indicated to evaluate for corneal abrasions, epiphora, and entropion. [40]

Some have recommended treating traumatic facial paralysis in the newborn with observation and corticosteroids. [2] This approach is similar to treatment of adult acute facial paralysis. No prospective randomized studies are available that evaluate the efficacy of steroid use in the newborn with facial paralysis caused by birth trauma. Steroids can be considered during the 5-week observation period before decompression or exploration of the nerve is undertaken. A recent recommendation is that corticosteroid treatment or surgery should be withheld in neonates who present with uncomplicated facial nerve resulting from forceps trauma. [41] As the child ages, speech impediments may become more obvious because of difficulty with oromotor tone; therefore, speech therapy should be considered.

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Surgical Therapy

In general, more than 90% of traumatic facial nerve palsies recover spontaneously and thus surgery is not warranted; [31] no controlled study has shown an improved outcome following surgical nerve exploration and decompression. With surgery, the risk of an iatrogenic injury is high. However, surgical exploration may be considered in infants with poor prognostic factors that include a unilateral complete paralysis present at birth, hemotympanum, displaced fracture of the temporal bone, absence of voluntary and evoked motor unit response in all muscles innervated by the facial nerve by 3-5 days of life, and no improvement by 5 weeks of age. [31, 42]

Conversely, no procedures are available that can enable an infant to develop normal function of the facial nerve when the palsy is developmental in origin. Facial reanimation's goal is to minimize asymmetries and improve function. Surgical exploration in the newborn with facial paralysis is controversial. Issues regarding timing of facial rehabilitation are complex. The factors that are involved include ability of the infant to tolerate a surgical procedure, the unknown potential for recovery, and whether early surgical intervention can prevent future psychosocial problems for the child.

In addressing developmental and unresolved traumatic facial paralysis, some medical professionals advocate initial surgery during preschool to avoid the psychosocial problems associated with a physical abnormality. However, waiting until adolescence when facial growth is mature and the child is able to understand the risks and benefits of surgery also has merit. [10]

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Preoperative Details

For patients with congenital facial paralysis, many surgical procedures with varying indications for patients exist.

Decompression surgery

A general preoperative guideline for decompression surgery of the temporal bone after a traumatic injury is to determine if clinical and electrophysiologic tests reveal (1) complete unilateral paralysis (H-B grade VI), (2) evidence of temporal bone trauma based upon CT scanning and physical examination, (3) complete loss of function of the facial nerve at age 3-5 days, and (4) absence of improvement by age 5 weeks. As a reminder, after the nerve had been decompressed, and if Wallerian degeneration has occurred, the nerve regenerates at a rate of approximately 1 mm per day.

Neurorrhaphy

The best situation for repair of the facial nerve is when primary reanastomosis is possible between the transected ends; however, this is an uncommon occurrence in congenital paralysis. In developmental paralysis, a fibrotic remnant of the nerve or total absence of the nerve and traumatic paralysis is often caused by a crush injury rather than transection. Nerve ends may need to be débrided before anastomosis with 8-0 or 9-0 nylon sutures. The primary recommendation today is to use an epineurial repair because suture placement with fascicular or perineurial repair is difficult and may injure the axons. [43] The key factor in neurorrhaphy is reapproximation without tension.

Cable grafts

When a tension-free primary nerve repair is not possible, such as when a segment nerve has been crushed, a cable graft may be indicated. The most common donor nerves are the greater auricular, sural, and the medial and lateral antebrachial cutaneous nerves. The ansa cervicalis has been used as a donor nerve, because some evidence exists that motor nerve grafts are better than sensory nerve grafts. Cable graft anastomosis is accomplished using 8-0 or 9-0 nylon sutures to reapproximate the epineurium. With either primary nerve repair or cable grafting, the best possible outcome is generally with House-Brackmann Grade III facial function. [44]

Cross-face grafts

This procedure offers the potential to provide specific divisional innervation to its counterpart on the contralateral face. This technique may be combined with microvascular muscle grafts. It is not applicable in some patients with developmental palsies because the distal peripheral nerve and muscle are often impaired. This has been used in patients with hemifacial microsomia. Of the 9 patients younger than 1 year, 7 had symmetry at rest and voluntary movement and spontaneous facial expression at 18 months postoperatively. As the age of the child increased, the percentage of satisfactory outcomes decreased. [45]

Nerve transposition

This procedure is indicated when no known proximal facial nerve is available based upon MRI evaluation, physical examination, and topodiagnostic studies. The hypoglossal nerve provides the best crossover graft with minimal resultant lingual atrophy as seen in the image below. Facial nerve-hypoglossal nerve grafts are not indicated in many developmental paralysis because of the impairment of the distal peripheral nerve and neuromuscular junction (may be demonstrated on muscle biopsy). An ideal outcome of this technique is good symmetry at rest, some voluntary movement with synkinesis, and mass movement; however, no emotional facial expression is expected.

Surgical photo of a XII-VII crossover with jump gr Surgical photo of a XII-VII crossover with jump graft. The great auricular nerve graft is sutured to the proximal portion of the facial nerve and to a partial sectioned hypoglossal nerve (preserving approximately two thirds of the axons going to the tongue). Image courtesy of A.D. Bruns.

Muscle transfer

This procedure is indicated when distal nerves or neuromuscular junctions are absent or when significant atrophy is present. Children often have good facial tone at rest, and the risk of the surgery must be weighed carefully against the potential benefit of muscle transfer.

The usual donor muscles for transposition flaps include the masseter and temporalis muscles. Ideal results are good symmetry at rest and some voluntary motion; however, no emotional movement is expected. The temporalis muscle can be split and used to suspend the upper and the lower face.

Mini-temporalis transposition in association with facial nerve microsurgery may be a valuable alternative to free muscle transfer in selected cases. All patients demonstrated an increase in the observers' scores after mini-temporalis transfer in comparison with the scores granted preoperatively or after neural microsurgery. Highly motivated patients committed to postoperative motor reeducation exhibited the best results. [46] Often, a combination of temporalis and masseter muscle transfers is used to rehabilitate the upper and lower face. The trigeminal nerve innervates these muscles; thus, voluntary movement can be achieved with rehabilitation training.

Facial reanimation with free neuromuscular flaps is becoming an accepted standard treatment in patients with complete unilateral facial paralysis. This has been accomplished with a 2-stage technique with the gracilis muscle; recently, a single-stage reanimation technique with the latissimus dorsi may decrease recovery time for patients.

The 2-stage technique involves placing a cross-facial nerve graft in the first stage, followed by microneurovascular muscle transfer 10-12 months later. In these cases, the sural nerve is widely used as the nerve graft, and the gracilis is the preferred donor muscle. A short nerve graft may allow the second stage to be completed in 3.5-5 months. [47] In a study by Terzis and Olivares of pediatric patients, function and symmetry improvement was observed in all patients 2 years after free-muscle transfer, with functional and aesthetic gains increasing over time. Evidence indicated that the transplanted muscles grew in harmony with the craniofacial skeleton. [48]

A single-stage facial reanimation has been used to reduce recovery time. This involves one nerve anastomosis instead of 2, with a latissimus dorsi flap and long thoracodorsal nerve anastomosed to the facial nerve on the contralateral side. [49]

A study by Veyssière et al indicated that lengthening temporalis myoplasty is an effective treatment for congenital facial paralysis. The study, which included 34 patients, found that all of the patients with acquired congenital facial paralysis (11 cases) achieved a spontaneous smile by 9.5 months postoperatively, while 92.3% of those with isolated developmental congenital facial paralysis (12 out of 13 patients) achieved a spontaneous smile by 7.3 months postoperatively, and 90% of patients with syndromic congenital facial paralysis (9 out of 10 cases) obtained a spontaneous smile by 9.7 months postoperatively. [50]

Static sling

Children often have good facial symmetry at rest and do not significantly benefit from a static sling until the skin and subcutaneous tissue have matured and relaxed. Using a fascia lata sling to suspend the lower face from the zygoma provides symmetry at rest, but no voluntary or spontaneous movement is achieved. Functional improvement of chewing, fluid retention, speech articulation, smile symmetry, and ectropion is immediate. The psychological effect is also immediate, with achievement of self-esteem and acceptance by family and peers. [51, 52]

Eye protection

When eye protection is inadequate and corneal abrasions result, tarsorrhaphy, gold weights, and palpebral springs should be considered. Gold weights are likely the best option because they are simple to insert and easily removed. This procedure is rarely performed in the newborn because parents are often very capable of protecting the infant's eyes.

A first large series of thin-profile platinum eyelid weight implantations has been introduced for the treatment of lagophthalmos. This implant significantly reduces both capsule formation phenomena and extrusion compared with gold weights and could be considered an alternative to the more conventional gold implants. [53]

Treatment for CULLP

Several options are specific to CULLP. Most parents do not notice any defect except when the child is crying; therefore, surgical intervention in the isolated CULLP deformity is rarely indicated. Surgical procedures to weaken the nonaffected side with selective marginal mandibular neurectomy or botulinum toxin injections provide symmetry at rest. Other plastic-reconstructive options include wedge resection and fascia lata sling or cheiloplasty, plication or transposition of the orbicularis oris muscle, and digastric muscle transfer.

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Postoperative Details

The postoperative care of the newborn after facial rehabilitation is similar to any other surgical procedure. The child (if age appropriate) and parents should be instructed on exercises to improve facial rehabilitation.

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Follow-up

After facial reanimation, return of some function has been found to occur within 18 months. Long-term treatment involves evaluating for any donor site morbidity, including tongue atrophy in patients with facial nerve–hypoglossal nerve transposition, difficulty with mastication in patients with masseter or temporalis transfer, and examination of the donor sites for greater auricular or sural nerve grafts. Routine ophthalmologic examinations and physical therapy for facial expression exercises are included in the long-term treatment of patients. As the child ages, biofeedback can be used to facilitate training of the mimetic musculature after cable grafts, facial nerve–hypoglossal nerve transposition, and muscle transfers. In children with developmental facial nerve paralysis who often have other congenital abnormalities, attention to appropriate weight gain and developmental milestones is necessary.

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Complications

The complications of facial reanimation in the early postoperative period include infection, hematoma, and the production of facial paralysis on the unaffected side in the case of a cross-facial graft. Long-term complications relate to the failure of the reanimation technique and lingual atrophy when a facial nerve-hypoglossal nerve transfer has been performed.

The primary care provider should routinely observe infants with congenital facial paralysis to ensure adequate growth and development. The facial nerve is responsible for providing oral competence in the oral phase of swallowing through the orbicularis oris muscle. When deficit in innervation of this muscle is present, the infant may have great difficulty with feeding because the ability to suck is impaired. As the child ages, speech impediments may become more obvious because of difficulty with oromotor tone; therefore, speech therapy should be considered. Routine ophthalmologic examinations are also indicated to ensure that the eyes are adequately protected.

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Outcome and Prognosis

More than 90% of patients with facial nerve paralysis caused by trauma recover without treatment. When the palsy is of developmental origin the parents should be informed that the child will never have an entirely normal appearance. The best outcome expected in these cases is facial symmetry at rest, near symmetry with voluntary movement, and spontaneous emotive movement.

A study by Domantovsky et al of patients with Möbius syndrome who, at mean age 13.2 years, underwent smile reconstruction with a gracilis muscle transplant (with innervation via the motor nerve to the masseter), found that at a mean follow-up of 20.4 years, improvements in muscle movement had not diminished. [54]

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Future and Controversies

Much controversy exists regarding the timing of facial reanimation and the need for surgical exploration in children with congenital facial paralysis. Issues regarding the timing of reanimation are complex. Some health professionals advocate initial surgery during preschool to prevent the psychosocial aspects associated with a physical abnormality. However, waiting until adolescence when facial growth is mature and the child is able to understand the risks and benefits of surgery and participate in the decision making process also has merit.

No uniform assessment of facial function exists. The House-Brackmann scale is the most widely used, but it has only fair interrater reliability. A standardized program has been developed to permit data entry for facial function that simultaneously produces scores for each of the 6 most commonly used scales. This may progress to a method of acquiring videographs to quantify motion of relevant points of the face to provide a 3-dimensional surface scan to assist in evaluated surgical reanimation surgery.

A web-based data gathering and centralized analysis program with data and “face grams” has also been suggested because the patient population in any given program is small. This would then provide a larger pool of patient for randomized, double-blind studies to determine the effects of steroids or other treatments, thereby creating a better exchange of surgical ideas and innovations.

Other research on nerve growth will also improve clinical outcomes of facial paralysis patients in the future. [55]

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