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Plastic Surgery - Regional Paediatric Burns and Plastic Surgery Service

Photograph from SmileSpace project, part of Alder Hey's facial palsy serviceFacial palsy is a weakness in the muscles of the face. This is most commonly caused by damage to the facial nerve that provides the nerve supply to the fine muscles that are responsible for facial movement. This results in an inability to perform normal facial expressions.

Facial palsy diagramThis palsy can be due to damage to the nerve at any point along its course from the brain through the skull and in the face where it forms five main branches. The palsy can be complete (a total loss of function) or partial (a weakness rather than complete loss of movement) affecting all divisions of the nerve or only specific branches. As a result, there is great variability in the way that children with facial palsy are affected.

Causes of facial palsy

There is a great deal of information on the Facial Palsy UK website and we recommend you spend some time exploring it; here we outline the impact facial palsy can have and some of the numerous causes.

Congenital

In many cases, a child is born with facial palsy and no specific cause is found. It won’t be anything that has been done by you during pregnancy and is likely a spontaneous event during development. In some cases it occurs with other features that define a recognisable syndrome. Such cases are very rare.

Acquired

Facial palsy can be acquired through trauma (such as skull fractures and facial lacerations) or as a consequence of tumours that occur anywhere along the path of the nerve from the brain to the face. The most common cause of a facial palsy (usually temporary) is during childbirth, especially if forceps are used. Bell Palsy is less common in children than in adults but in both instances, we know that prompt treatment with steroids can result in a good outcome.

Functional impact

In adults, facial palsy has significant functional impact. In other words, the function of the eyes, nose and mouth are impaired causing symptoms such as red and irritated eyes, blocked nose and inability to keep food and liquid in the mouth. Such problems in children are less common as the functional problems above are partly due to sagging of the tissues of the face, which happens as we age. Therefore the main issues that affect children are psychological as a consequence of their facial and smile asymmetry.

Psychological impact

Very young children are oblivious to the condition until they start to interact with their peers. Typically at nursery or primary school age the child will notice the difference in their smile. Sometimes other children will enquire or the parents will ask questions “at the school gates”. Around the age of 3-5 years, the child may ask why their own smile is different from others. In older children, especially if there is a change of peer group it may become a focus of bullying. The single most important factor in a child’s reaction to their difference is the parental reaction to their difference. Parents that show concern about their child’s facial difference project their anxieties onto the child who then develops a negative self-perception.

Case study – Isaac’s story

Isaac has the very rare Moebius Syndrome and his mother has very kindly allowed us to reproduce an email she sent to our service. As a response to this email, we are in the process of establishing a multidisciplinary team that not only deals with the facial palsy but all aspects of Moebius Syndrome, such as sleep disturbance and limb anomalies.

She outlines her journey in her own words below:

“I am not sure if I can begin to describe how difficult our journey has been over the last few years due to Isaac’s condition and I am sure this is reflected through many parents’ experiences.  I think the rareness of Isaac’s condition adds to the complexity.  I will give you a brief summary just so that you understand why such a centre is so important to us.

Isaac was born by caesarean as he was breach.  It was a straightforward delivery and all seemed well initially.  By the evening we realised he was not feeding and by early hours they took him to special care to be fed by tube. The next day the tube was removed and we were told to try to feed him with a bottle, which was an extremely stressful time as no one know why he could not feed.  The paediatrician visited and said that he might have a genetic condition or might just be an odd looking child! This was the first time we knew there was anything seriously wrong and to be told our newborn looked strange was not something that we expected and was extremely traumatic.  I was told I had to feed Isaac every other hour day and night to ensure he had sufficient milk but not given any tools to do so except a prem teat.  No mention was ever made of a Habermann feeder or similar and so it was a struggle from the beginning.  After 5 days we were allowed to leave hospital and simply told he must not lose weight but not given anything to help us feed him.

Isaac was tested for the main genetic conditions but as there is no genetic test available for Moebius the tests returned normal.  We were told we would have regular paediatrician appointments but otherwise no suggestion or further investigations as to what was wrong.  Isaac had low muscle tone and a severe squint.  We were referred to the eye surgeon in Chester who saw us when Isaac was 6 months.  Prior to this we had spoken to the paediatrician to say Isaac had not smiled but he felt Isaac might have sight problems and that is why he did not smile.  We saw the eye surgeon and he reported back to the paediatrician.  We have subsequently learned that at 6 months the eye surgeon told the paediatrician that he suspected Moebius but we were not told until our routine appointment.  The consultant simply said he had Moebius, gave a brief explanation and said that he was just as he was and would never smile.  We were given no advice, no information leaflet and no support.  I even asked him to write it down for me, as I did not know how to spell it.

This brief description was only the beginning of our experience and it is for this reason I am passionate about the need for medical professionals to receive more training and knowledge about these conditions.  For example had we been told about a Habermann feeder we may have been able to feed Isaac easier and would not have had to suffer months of gruelling feeding.  Had there been a greater awareness about Moebius we might not have had to wait 8 months to find out what was wrong and if information was available we could have left that waiting room with the knowledge we needed.

At 9 months Isaac nearly died as we did not know he would have feeding problems.  We had researched Moebius but were not aware of all the implications.  We gave Isaac a biscuit that was suitable for a 6 month old baby but he choked so severely I had to call an ambulance which took 25 minutes to arrive.  Luckily I was able to clear his airways.  Information about feeding issues could have prevented this.

As Moebius Syndrome is so rare, there was no specialist we could turn to for help and we had to call around and speak to consultants just to identify who we should be seeing.  The Moebius Research Trust was invaluable support as at the time it had a forum where we could speak with other parents.  It was basically a collection of desperate adults all just trying to help one another understand how best to help their child.  Recognising the need to highlight Moebius we have tried to raise awareness where possible and have had articles published in The Independent, The Telegraph, local newspapers and have appeared on ITV Wales several times along with a German news program.  If you google “Isaac Hughes” you can see many, many articles.  Following this, we have been contacted by several parents who were actually able to diagnose their child’s condition after learning about Isaac.  It really should not be the case that parents are having to self diagnose and whilst I understand it is a rare condition there must be a better way to ensure it is on paediatricians mind when presented with a child with limited facial movement.” 

It is in response to emails such as these that we established our multidisciplinary team in order to try to coordinate appointments for our most complex patients into as few visits as possible.

Assessment

Structure of the facial palsy multidisciplinary teamA multidisciplinary team (MDT) is widely recognised as the best way to manage patients that have complex problems that impact on many areas of their health. It comprises a team of individuals each with their own expertise that have a common strategy for treatment of a particular condition. As children do not like sitting in a room being stared at by many strangers we have adopted a “virtual MDT” approach whereby an initial consultation will be with one of the team members and subsequent consultations will be coordinated with those professionals needed to address specific issues.

We believe the MDT will advance the care of our patients by using a standardised approach with robust data collection and outcomes studies to allow us to evaluate patient experience and maintain standards to an international level.

Meet the team

emmaPhysiotherapy: Helen Hartley and Rebecca Pratt are our therapists that employ a range of techniques to treat some of the symptoms of facial palsy such a facial spasm as synkinesis as well as having a key role in post-operative rehabilitation following smile reconstruction.

Psychology: Rachel Mumford and her team provide psychological support to those children and families that experience difficulties coping with facial palsy.

WendySpeech and Language Therapy: Wendy Blumenow is also a member of the craniofacial service and provides speech therapy to the small number of patients that have issues in this area.

Neurology: Dr Ram Kumar and Dr Stefan Spinty are the neurologists on the team who is involved in diagnosis and treatment of facial palsy. Dr Kumar graduated in medicine at University of Cambridge in 1995. He has been a consultant paediatric neurologist at Alder Hey since 2007. Dr Kumar has contributed chapters to a number of textbooks and is actively engaged in research, undergraduate and postgraduate multiprofessional education.

Neurosurgery: Mr Conor Malluci is our neurosurgeon that has a practice in brain tumours; some patients will experience a facial palsy following tumour removal and Mr Malluci is our link for children that present with facial palsy that is found to be due to a tumour. He has just finished his tenure as chairman of the British Paediatric Neurosurgery Group and continues to chair their research group. He is currently deputy editor of the British Journal of Neurosurgery and the associate editor of the European journal, “Child’s Nervous System”.

Ear Nose and Throat: Mr Ravi Sharma is our ENT surgeon.

MariaPlastic surgery: We have a dedicated ward for our facial palsy patients with individual cubicles and en suite facilities for families. Mr Adel Y Fattah is the lead for the facial palsy service having trained in Toronto with Dr Ron Zuker. Maria Kelly is our specialist nurse and is responsible for liaising between specialties in order to coordinate appointments.

Theatre Team: We have a dedicated core group of theatre staff that perform our facial reanimation and microsurgery cases.

Options for treatment

Botox for Smile and Facial Asymmetry

A very common form of smile asymmetry is mandibular division palsy leading to “asymmetric crying facies”. This is so called as it is often first noted in babies when they cry. It is caused by depressor anguli oris muscle (DAO) weakness, as a consequence of palsy of the mandibular division that innervates this muscle. A number of options exist to treat this including surgery to use a muscle in the neck to recreate the muscle action (digastric transfer) or more commonly procedures to weaken the normal muscle on the unaffected side. The latter doesn’t treat the problem, but does restore symmetry, which is the noticeable feature. One method of doing this is to perform an operation via an incision inside the mouth and remove a portion of the muscle. This is permanent and once performed cannot be easily reversed. Our preferred method is to use botox to temporarily weaken the muscles. The aim of this is to weaken the DAO muscle to the same extent as on the affected side. As it is not permanent, repeated injections are required two or three times a year.

botoxThe injection is painlessly performed in the clinic (or under sedation) using an ultra-fine needle. It takes a few seconds to perform the treatment then you are able to go home. It takes 2-3 days for any improvement to be seen. The effect should last for about three to six months but everyone is different. An example is seen below. In general, the age at which it is performed is determined by the child actively seeking treatment, usually around the age of nine and above.

Surgery

We offer a full range of surgeries to restore facial animation. The most common procedures we use for established (long term) facial palsy are described below. In many cases, the options for treatment include doing nothing which is a good option if you are unsure about treatment as leaving things as they are will not usually have any effect on the condition or necessarily worsen it. Many procedures are tailored to the individual needs of the patient. For example, recent injury to the facial nerve on one side may be amenable to a cross face nerve graft where we can run a nerve graft from one side of the face to the other to restore muscle function using carefully hidden incisions.

For established (long term) facial palsy in which the muscles of the face do not work and the child is seeking solutions to recreate a smile we offer three main procedures outlined below.

Option 1. Two-stage free muscle transfer

Widely regarded as the Gold Standard for treatment, is the use of a “free muscle transfer” which can be a one or two stage operation. The two-stage operation involves a first stage termed a cross face nerve graft that uses a nerve from the back of the calf to take the activity of the normal facial nerve on the unaffected side to the affected side. This first operation involves a one to three-night stay in hospital and most importantly will have no effect on the smile. Using two small transverse incisions in the back of the calf of the leg, the “sural” nerve is identified and removed. This nerve supplies some sensation to the outer border of the foot and its removal will result in some loss of sensation; most patients do not report this to be a problem. Meanwhile on the unaffected side of the face, a facelift incision running from the hair, immediately in front of the ear and curving behind the ear and partly onto the neck is made and the skin is lifted up to expose the branches of the facial nerve. The nerve has many branches like that of a tree and a special instrument is used to test each of the branches to find a branch that creates a strong smile. Once that branch is found, it is cut and the nerve graft from the calf is sutured to it using microsurgery. Other branches that remain will preserve the smile to that side of the face. The graft is then threaded through to the other side of the face by tunnelling under the skin via a small incision inside the upper lip so that there are no other scars on the face.

After the operation, we wait 6-12 months for the nerve fibres to grow from the unaffected side of the face down the nerve graft to the affected side. We can often test for this growth by tapping over the course of the nerve, which will create a tingling sensation. During this waiting phase the patient may feel unusual sensations or tingling which is a good sign showing that the nerve is growing; it usually grows around 1mm per day. It is important to remember that there will be no restoration of smile after this first stage.

The second stage operation involves taking part of the gracilis muscle from the inside of the thigh and transferring it up to the affected side of the face so that it can power a smile. The patient will spend roughly a week in hospital after this procedure.

The muscle is usually taken from the opposite side using an incision down the inside of the upper thigh, which is usually well hidden. Part of the muscle is used and together with the nerve that activates it and the blood vessels that keep it alive, it is transferred to the face. The affected side of the face is opened in the same way as the first operation and the muscle is attached to the inside of the lips using stitches. Using microsurgery, the blood supply to the muscle is restored by putting the artery and vein together and finally the nerve is sutured to the nerve graft that was placed in the first operation using the incision in the mouth.

After the operation we need to wait 6-12 weeks for the nerve to grow down to the muscle before we start to see any movement. Initially, the movements are small and weak but with a programme of physiotherapy and exercises in the mirror the muscle will get stronger over a number of months.

Option 2: One stage free muscle transfer

panelThe one-stage version of the operation does not use the facial nerve of the other side but uses a nerve that creates a biting action (“the motor nerve to masseter muscle”). Essentially, this version is the second stage operation only where the electrical input to the muscle is from the nerve to the biting muscle instead.

The main difference between the two operations is that in the one stage operation the patient will need to consciously bite to smile, whereas in the two-stage operation the smile will be spontaneously activated whenever the nerve on the other side works. Nonetheless, good results have been reported with the one-stage operation and many have found that the brain “re-wires” itself so that a conscious need to bite eventually goes and the smile may even be spontaneous. The other difference is that the biting muscle will create a stronger smile and so for some children that have a particularly strong smile, this may be the better option.

Option 3. Labbé Transfer

Another option to restore smile is a single stage procedure that also uses one of the biting muscles and doesn’t require any microsurgery.  This is called the Labbé procedure after the French surgeon that described it. In the temple when you bite you’ll feel a muscle contract called temporalis.This is attached to the jawbone just in front of the ear with a strong tendon.

The Labbé procedure involves a scar across the top of the head inside the hair to expose this muscle. The muscle is then freed from the skull and allowed to slide down toward the face. Then using an incision inside the mouth the tendon is freed up from the jawbone and re-routed to the corner of the mouth. When the patient bites, the muscle will create a smile.

Whatever the technique, all are limited by the fact that we are using a single muscle to pull in one direction that best mimics the natural facial muscles that are small and can pull in a multitude of different ways to create subtle facial expression. The techniques above are unable to create such subtle movements but are able to create a smile that will be relatively symmetrical to the other side.

Research

SmileSpace project at Alder HeyOur group is actively engaged in research and are currently investigating the 3D anatomy of the face using 3D photographs of the normal population to define what constitutes a normal smile as part of our SmileSpace project.

Development of the Face-Q Kids, a patient reported outcome measure for facial palsy (as part of an international collaboration with the creators of the Face-Q, Breast-Q and Cleft-Q).

Publications

  • Snyder-Warwick AK, Fattah AY, Zive L, Halliday W, Borschel GH and Zuker RM (2014). The degree of facial movement following microvascular muscle transfer in pediatric facial reanimation depends on donor motor nerve axonal density. Plast Reconstr Surg. 135: 370e-381e
  • Fattah AY, Gurusinghe AD, Gavilan J, Hadlock TA, Marcus JR, Marres H, Nduka C, Slattery WH and Snyder-Warwick AK (2014). Facial nerve grading instruments: Systematic review of the literature and suggestion for uniformity. Plast Reconstr Surg. 135: 569-579
  • Fattah AY, Gavilan J, Hadlock TA, Marcus JR, Marres H, Nduka C, Slattery WH and Snyder-Warwick AK (2014). Survey of Methods of Facial Palsy Documentation in Use by Members of the Sir Charles Bell Society. Laryngoscope 124: 2247-51.
  • Fattah AY, Ravichandiran K, Zuker RM and Agur AMR (2013). Three-dimensional study of the musculotendinous and neurovascular architecture of the gracilis muscle: applications to free functional muscle transfer. J Plast Reconstr Aesthet Surg 66: 1230-1237
  • Chang Y, Cantelmi D, Wisco J, Fattah A, Hannam A and Agur AMR (2013). Evidence for the functional compartmentalisation of the Temporalis Muscle: a three dimensional study of innervation. J Oral Maxillofac Surg 71: 1170-1177
  • Davies JC, Fattah A, Ravichandiran M and Agur AMR (2012). Clinically Relevant Landmarks of the Frontotemporal Branch of the Facial Nerve. Clinical Anatomy 25: 858-865
  • Fattah A, Borschel GH, Manktelow RT, Bezhuly M and Zuker RM (2012). CME: Facial Palsy and Reconstruction. Plastic and Reconstructive Surgery 129: 340-352e
  • Davies J, Fattah A, Ravichandiran M and Agur AMR (2012). Redefining Pitanguy’s line: A three dimensional analysis of the fronto-temporal branch of the facial nerve. Clinical Anatomy 25: 934
  • Fattah A, Ravichandiran K, Zuker RM and Agur AMR (2012). Creation of a 3D Digital Model of the Gracilis Model: Application to Free Functioning Muscle Transfer. Canadian Journal of Plastic Surgery 20: 135
  • Snyder-Warwick A, Zive L, Fattah A, Halliday W, Borschel G and Zuker RM (2012). Axonal Counts in Facial Reanimation. Canadian Journal of Plastic Surgery 20: 141
  • Snyder-Warwick A, Fattah A, Placheta E, Zive L, Borschel G and Zuker RM (2012). Functional Outcomes in Facial Reanimation: Comparison of Innervation Sources. Canadian Journal of Plastic Surgery 20: 141
  • Fattah A, Cypel T, Donner E, Wang F, Alman BA and Zuker RM (2011). The First Successful Lower Extremity Transplantation: 6-year Follow up and Implications for Cortical Plasticity. American Journal of Transplantation 11: 2762-2767
  • Fattah A, Borschel GH and Zuker RM (2011). Facial Nerve Injuries in Children. Journal of Craniofacial Surgery 22: 782-788
  • Invited Contributions
  • Sadri A and Fattah AY (2014). Facial Palsy and Reconstruction in Children. In Clarke R (Ed). Paediatric Otolaryngology: Practical Clinical Management. Thieme, Stuttgart, Germany (in press).
  • Davies JC, Agur AMR and Fattah AY (2013). Anatomic Landmarks for Localization of the Branches of the Facial Nerve. OA Anatomy 1(4):33
  • Fattah A, Zuker RM and Borschel GH (2011). Smile Reconstruction in Children: The Toronto Approach. American Society of Peripheral Nerve Newsletter, Spring 2011.
  • Fattah A, Zuker RM, Borschel GH and Manktelow RT (2011). Facial Reanimation in Children and Adults. In Boyd, B and Jones, N Eds. Operative Microsurgery. McGraw-Hill, New York (in press).

Information for professionals

We are nationally designated to provide facial palsy care to children across England up to the age of 18 and beyond. We take all referrals related to facial palsy or suspected facial palsy; we are here to help you diagnose and treat your patients optimally. Please feel free to contact us for advice. Our main domains are:

Syndromic Cases: We will see all patients that have facial palsy as part of a syndrome and are developing our Moebius Syndrome service.

Obstetric facial palsy: We will see all cases of facial palsy following difficult birth in order to reassure and document rate of recovery.

Acute facial trauma: we will see facial nerve injury following trauma including skull base trauma. Lacerations to the face seen before 72 hours have much better outcomes in terms of our ability to match up nerve ends using intra-operative neurostimulation.

Planning and counselling prior to oncologic resection: We can counsel patients on what to expect should facial nerve injury follow oncologic resection and communicate with oncologic surgeons to coordinate immediate reconstruction if appropriate.

Established facial palsy: we will see all cases and consider suitability for smile reconstruction and facial reanimation, as well as treating issues such as synkinesis and facial spasm.

Work up: In general, no specific investigations need to be performed prior to referral but any results to date are very helpful when included with the referral letter.

For further information email facialpalsy@alderhey.nhs.uk or call 0151 252 5386. We want to make your experience of using our service as easy as possible.

How to refer

Referrals will be accepted via the Regional Paediatric Burns and Plastic Surgery Service and the Choose and Book system.

Email: facialpalsy@alderhey.nhs.uk

Fax: 0151 252 5932

Choose and Book: Please search under Alder Hey Plastic Surgery

Address:

Facial Nerve Programme
Regional Paediatric Burns and Plastic Surgery Service
Alder Hey NHS Children’s Foundation Trust
Eaton Road
Liverpool
L12 2AP

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