Introduction

 

Facial defects are commonly handled by either plastic or maxillofacial surgeons, depending on the dynamics of the surgical unit. Generally in the UK most traumatic, infectious and oncological facial defects are handled by maxillofacial surgeons, with congenital conditions such as clefts being handled by plastic surgeons, who must complete a requisite minimum number of cases per year.

 

In the USA, maxillofacial surgery is much less of an established speciality, with almost all complex reconstructions being carried out by plastic surgeons with fellowships in craniofacial surgery.

 

Classification

 

Facial defects can be split into sections using a surgical sieve type approach:

 

  •    CONGENITAL
      •  Cleft lip and palate
      •  Craniosynostosis
      •  Treacher-Collins Syndrome
      •  Hemifacial Microsomia
      •  Pierre Robin sequence

     

    •    INFECTION

     

    •    TRAUMA
      •  Initial management
      •  Fractures
      •  Lacerations

     

    •    NEOPLASIA
      •  Salivary gland tumours
      •  Oral cavity tumours

     

    Congenital

     

    Typically cleft lip and palate repairs are what one would associate with congenital plastic surgery and this has been covered in a separate section, however there are a number of lesser-known syndromes, these are all rare.

     

    Craniosynostosis (Figure 1)

    Craniosynostosis – This is the premature fusing of one or more of the cranial vault sutures. This takes place either in utero or shortly after birth and affects between 1 in 2,000 to 2,500 live births worldwide. It can be subdivided into syndromic and nonsyndromic causes.

     

    In nonsyndromic cases this affects in order of preference: Sagittal > Metopic > Coronal > Lambdoid > other sutures. They tend to affect only one suture, in syndromic cases multiple sutures are affected.

     

    Syndromic cases are classically either: Apert’s syndrome (multiple craniosynostosis, exorbitism, midfacial retrusion and symmetrical syndactyly of both hands and feet), Crouzon’s syndrome (similar facial features but no syndactyly) or Pfeiffer syndrome (similar facial features but also with broad thumbs and toes).

     

    The goal of treating these patients is to release the fused cranial sutures to allow normal cranial vault development.

     

    Treacher-Collins Syndrome (Figure 2)

    Treacher-Collins Syndrome is a rare (1 in 25,000 to 50,000), autosomal dominant disorder, which affects a gene on chromosome 5q with variable penetrance. This can cause downward slanting of the eyes, hypoplasia/aplasia of the zygoma, hypoplasia of the mandibula with micrognathia (small jaw), drooping of the lateral lower eyelids, and variable ear malformations with conductive deafness [Figure 2].

     

    The treatment of those affected by Treacher-Collins is very much multi-disciplinary and involves a carefully planned, tailored reconstruction (from 3D CT images) of autogenous bone with an autologous fat/soft tissue transfer, timed to fit with the physical and psychosocial development of the child.

     

     

    Hemifacial Microsomia is a rare (1 in 4,000 to 5,500) condition that affects the development of the structures in the middle and lower face: ear, mandible and maxilla. This can be either bilateral or unilateral and is not progressive, [Figure 3].

     

    It is the second most common facial birth defect after cleft lip and palate disorders.

     

    The extent to which the condition affects different individuals can be hugely variable, with the subsequent reconstruction dependent upon this.

     

    As in Treacher-Collins syndrome, autogenous bone and autologous fat/soft tissue transfer can be used in the treatment of hemifacial microsomia. 

     

     

    Pierre Robin Sequence is a rare (1 in 8,500 to 80,000) triad of micrognathia, glossoptosis (a posterior displacement of the tongue) and an obstruction of the upper airway. Cleft palate is also associated with this condition [Figure 4].

     

    Initial management of an affected individual is focused on the maintenance of the airway and regular feeds.

     

    Mandibular distraction can be used to lengthen the jaw, by creating an osteotomy and insertion of a device into this space, which gradually lengthens the jaw, promoting the formation of new bone.

     

     

    Infection

     

    The face is usually fairly resistant to the spread of infection due to its rich vascularity and drainage. However, there are a number of routes where infections are commonly allowed in. These are aero-digestive infections that have tracked up through the mediastinum and scalp, or orbital infections that have spread intracranialy through the venous drainage systems.

     

    Facial cellulitis is commonly seen and closely monitored by plastic surgeons and treated with a trust-specific regimen of IV antibiotics.

     

    Importantly, atypical mycobacterial infections should always be included in the differential diagnosis.

     

    Trauma

     

    Initial Management

     

    Facial trauma is usually dealt with by maxillofacial surgeons. It must be remembered that when assessing these patients the ATLS® principles of ABCDE should always be applied, as securing a definitive airway can be complex. Care should be taken when using a nasopharyngeal airway if there is any suspicion of a basilar skull fracture. Traumatic brain injury and C-spine injury should also be ruled out, as maxillofacial trauma is usually associated with more severe injuries.

     

    In the developed world, facial injuries are most commonly sustained through inter-personal violence. In the developing countries, they are more frequently a result of injuries subsequent to road traffic incidents, however, their incidence is declining with advancements in car safety.

     

    A careful history should be obtained, recording any neurological changes and noting the tetanus status of the patient. A systematic examination (secondary survey) should then be carried out to exclude the following: neurovascular compromise, any deformity, surgical emphysema (like touching Rice Krispies) or bogginess of the scalp suggesting an underlying skull fracture.

     

    Listed below are the classic findings of facial trauma and the fractures associated with specific signs:

     

    •  Racoon eyes (periorbital ecchymosis) – Basal skull fracture
    •  Battle’s sign (postauricular ecchymosis) – Basal skull fracture
    •  Otorrhoea – Basal skull fracture, condylar fracture
    •  Haemotympanum - Basal skull fracture
    •  Epistaxis – Nasal fracture
    •  CSF rhinorrhea – Cribriform plate fracture; Nasal, orbital and ethmoidal (NOE) fracture

     

    An oral examination is also necessary to assess for dentoalveolar and mandibular fractures. CT images are usually taken once the patient is stable in order to manage skeletal and soft tissue injuries, definitively and in a single operation.

     

    Fractures

    Mandible fractures are again dependent on the location; fractures to the body and ramus will often require open reduction and internal fixation (ORIF), whilst condular fractures and normal occlusion are often managed conservatively. Adequate reconstruction of the patient’s normal bite is essential.

     

    Le Fort (Figure 5)

     

    Maxilla fractures [Figure 5] are the best known of the maxillofacial fractures, with the Le Fort classifications of I – III. For this fracture to be classical the pteygoid plates should be involved. Class I is a horizontal fracture (orange line), while class II a pyramidal (green line), Class III is a transverse fracture (blue line) which is most likely to cause CSF rhinorrhoea.

     

    Generally, fractures to the medial wall of the orbit are managed conservatively with consultation from an ophthalmologist. Midface fractures, however, are often treated with an ORIF. Fractures to the nasal bone are usually seen by the ENT team, however plastic surgeons may become involved at a later stage, after the swelling has subsided, for correction under anaesthesia.

    Lacerations

     

    All facial lacerations should be referred to the on-call plastic surgery team for a prompt assessment. This involves injection with local anaesthetic, such as adrenaline, irrigation, any necessary debridement (to prevent tattooing of the skin) and careful closure of the relevant tissue planes. Particular care should also be taken when suturing the vermillion border of the lip, and the neurovascular status of the patient should always be documented. Such an approach is most likely to provide the optimum aesthetic and functional outcome.

     

    Neoplasia

     

    Facial neoplasms are, again, often the domain of maxillofacial and ENT surgeons, who have an excellent understanding of the steps on the reconstructive ladder and a good awareness of the aesthetic units of the face.

     

    Salivary Gland Tumours

     

    Salivary Gland Tumours encompass tumours of the parotid (80% benign), submandibular (55% malignant) and minor salivary glands (75% malignant).

     

    Again, general principles of history and examination apply. When examining a lump you should always remember the mnemonic:

     

    5 Students and 3 Teachers sat around a CAMPFIRE:

     

    •  Site, Size, Shape, Symmetry, Surface 
    •  Temperature, Tenderness, Transillumination 
    •  Consistency, Appearance, Mobility, Pulsatility, Fluctuance, Irreducibility, Regional lymph nodes & Edge

     

    A mass is more likely to be malignant if fixed or solid, associated with pain, change to the facial innervation or any lymphadenopathy. The standard investigation algorithm includes biopy by fine needle aspiration and initial imaging with ultrasound, with subsequent staging with CT or MR imaging.

     

    Benign tumours include:

     

    •  Pleomorphic adenoma, generally benign mixed, carries a high recurrence rate with local excision, and has the potential to become malignant if untreated for 15-20 years.
    •  Papillary cystadenoma lymphomatosum (aka Warthin's tumour), bilateral in 10% of cases, is unlikely to reoccur and rarely becomes malignant.

     

    Malignant tumours include:

     

    •  Mucoepidermoid carcinomas, the most common of the salivary gland tumours, peak in incidence in patients in their 20s to 50s, and have a prognosis that is tumour grade dependent.
    •  Adenoid cystic carcinomas, the second most common salivary gland tumours, peak in incidence of those in their 40s to 60s, and have a generally good prognosis, being slow growing and well-differentiated tumours that do not commonly spread via the lymphatics.
    •  Acinic cell carcinomas, affecting the parotid gland and, rarely, also appearing in the breast, are more common in those in their 40s to 50s, and are usually low-grade tumours with a good prognosis.
    •  Malignant mixed, adenocarcinomas arising from a benign tumour.

     

    The treatment for both benign and malignant conditions is surgical removal, with malignant tumours they are staged (TNM) and depending on the lymph node involvement, lymph node dissection and radiotherapy may take place. The prognosis of these tumours is closely related to their histological grade and metastasis.

     

    Oral Cavity Tumours

     

    Oral Cavity Tumours are tumours of the mouth, commonly related to a long smoking history. These have differing prognoses dependent upon the location of the tumour, with a worsening outcome the further away from the lip posteriorly.

     

    Very seldom handled by plastic surgeons, they are more often dealt with by ENT or maxillofacial specialists. Their treatment often involves significant neck dissections, radiotherapy and chemotherapy, especially if caught late. Most variants are squamous cell carcinomas and, as a result, are staged by the TNM classification. Reconstruction follows the surgical excision and often involves use of pedicled and/or free flaps and bone grafts to rebuild the affected mandible, neck and oral cavity.

     

    References & Further Reading

     

    1. Bullocks, J. et al. (2008) Plastic surgery Emergencies: Principles and techniques. Thieme publishers.
    2. Gardiner, M. & Borley, N. (2009) Oxford Specialist Training  - Training in Surgery the essential curriculum. Oxford University Press.
    3. Giele, H. & Cassell, O. (2008). Oxford specialist handbook of Plastic and reconstructive surgery. Oxford University Press.
    4. McGregor, A.D. & McGregor, I.A. (1995) Fundamental Techniques of Plastic Surgery: And Their Surgical Applications (9th edition). Churchill Livingstone.
    5. McGregor, A.D. & McGregor, I.A. (2000) Fundamental Techniques of Plastic Surgery: And Their Surgical Applications (10th edition). Churchill Livingstone.
    6. Nakhdjevani, A. (2007) Plastic Surgery Survival Guide to Trauma. RSM books.
    7. Song, D.H. et al. (2007) Plastic Surgery Essentials for Students – The American Society of Plastic Surgeons (7th edition). The American Society of Plastic Surgeons.

     

    Images

     

    1. http://img.medscape.com/pi/emed/ckb/neurology/1134815-1175718-1175957-1832066tn.jpg
    2. http://img.medscape.com/pi/emed/ckb/pediatrics_genetics/941088-946143-1581tn.jpg
    3. http://www.biomedcentral.com/content/figures/1471-5945-1-9-4.jpg
    4. http://img.medscape.com/pi/emed/ckb/otolaryngology/834279-844143-28tn.jpg
    5. http://upload.wikimedia.org/wikipedia/commons/thumb/6/67/LeFort3b.png/450px-LeFort3b.png (edited)

       

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