Appendix A: Organisation of a Craniofacial Unit The requirements of patients with craniofacial abnormalities are very complex and demand a multidisciplinary approach. Many body systems are affected, and every detail of patient management has to be given due attention. Care begins at birth and continues until the patient and his family have been relieved of the burden of the anomaly. A team is needed, capable of delivering expert patient care, and representative of all the relevant disciplines. Data, in the form of histories, physical examinations, and special investigations, are needed in planning treatment, and such data should be used to the maximum scientific effect, to improve present methods of management, still far from satisfactory, and to expand knowledge of the biology of cranial growth and its disorders. Craniofacial Units Sporadic craniofacial procedures performed by a surgeon on an irregular basis invite disaster. Tessier (1971a) estimated that each craniofacial centre should serve a population of 10 to 20 million people, provided that the team performed only craniofacial surgery and treated at least 50 new cases annually. As a consequence of Tessier's example and teaching there are now centres of acknowledged excellence in Paris and Nancy, attracting patients not only from France but also from North Africa and elsewhere. In North America there are now important craniofacial centres in Philadelphia, New York, Boston, Toronto, and Mexico City. Munro (1975) proposed that North America should be divided into seven regions, six for the United States and one for Canada, each serving populations of 20 to 40 million people. He believed that such centres would allow a concentration of multidisciplinary skills and accumulation of experience in the treatment of craniofacial anomalies. This in turn would stimulate laboratory studies into the pathogenesis of craniofacial anomalies. The concept of regionalisation and rationalisation has now been accepted in many countries and has been given some authority by the U.S. National Institutes of Health, which have undertaken to fund only certain designated craniofacial centres. A more diffuse, but nevertheless important, role of the centralised service is to act as a focal point for the manipulation of public opinion towards a more humane attitude towards those people with severe craniofacial deformities. Most of these patients have normal intelligence and the potential for happy and useful lives-if they are not crushed by social ostracism or lifelong institutional care. ' In Australia, craniofacial surgery was begun in several widely separate cities. Independently, groups of enthusiasts in Adelaide (David 197:7) and Brisbane (Atkinson et al. 1979) trained themselves in this very exacting field and undertook to see and to treat patients referred from other specialists in Australia, New Zealand, and South-East Asia. We describe in this section the organisation of the South Australian Craniofacial Unit, as it has evolved since it was established in 1975. The Team 297 The Team The following disciplines are represented: plastic surgery, neurosurgery, ear nose and throat surgery, ophthalmic surgery, medical genetics, neuroradiology, anaesthetics, psychiatry, orthodontics, dentistry, oral and maxillofacial surgery, prosthodontics, speech pathology, nursing, social work, photography, and administration. With such a large and sometimes temperamental cast of actors, there is need for co-ordination at all stages. This is done by the head of the team assisted by the craniofacial registrar, who is usually a surgical or scientific trainee. The unit secretaries play indispensable administrative parts. The anaesthetists are also concerned in co-ordination, especially in the preoperative assessments. Some of these, such as fundoscopy and blood-taking, are distressing for children and can be done during the anaesthetic procedures needed for X-ray investigations. The Plastic Surgeon The plastic surgeon is the head of the team and assumes responsibility for patient care. Special training in neurosurgery, orthopaedic surgery, and maxillofacial surgery is recommended. It has been suggested that craniofacial surgery should become a separate speciality '(Munro 1975). At the time when this was first mooted, it was probably an extreme point of view; however, with the emergence of many excellent craniofacial units around the world and the related need for plastic surgeons who can devote the majority of their time to this particular sub speciality, Munro's proposal has much more validity. The plastic surgeon (craniofacial surgeon) must not only be able to perform the bone surgery, but also to correct the many difficult soft tissue problems associated with craniofacial deformities. The Neurosurgeon It is important that the neurosurgeon's role be seen as an extension of his established role in the correction of the calvarial deformities resulting from craniosynostosis. The neurosurgeon has to assess the various neurological dangers inherent in each type of craniofacial procedure and to give warning if unusual circumstances make these prohibitive. The neurosurgeon is responsible for the preoperative neurological assess­ ment of the patient, and he is the operative partner when the transcranial approach is used for the exposure of the anterior cranial fossa. A high degree of co-operation is required throughout these procedures, with a blending of the techniques of plastic surgery and neurosurgery. The neurosurgeon is responsible for postoperative supervision when there is any risk of intracranial bleeding, infection, or visual loss. The Anaesthetist The importance of the anaesthetist's role is highlighted by the fact that craniofacial operations may last 10 h or more, and are performed on patients whose ages range from infancy to middle age. Large blood replacements are frequently necessary. Because of this, all craniofacial procedures should be administered by the same anaesthetic team so that experience in this exacting work is built up. Intubation and airway maintenance may be difficult because of associated upper airway stenosis and 298 Appendix A Organisation of a Craniofacial Unit jaw anomalies. The anaesthetist is also responsible for the immediate postopera1ive management in the intensive care unit. Two anaesthetic teams work with the South Australian Craniofacial Unit, one for children and one for adults. The Ophthalmologist and the Orthoptist The concept of craniofacial surgery is largely based on the fact that the orbits can be safely moved in three dimensions. Detailed preoperative and postoperative examination by the neuro-ophthalmologist and by the orthoptist are essential. The measurements detailed on p. 77 are necessary in operative planning. Examination of visual acuity, visual fields, binocular vision, eye positions, muscle function and fundoscopy are of obvious importance. If any ophthalmological complications arise, then the ophthalmologist must diagnose and be directly involved in their treatment. New methods ofcomputerised monitoring of vision are very promising, but they are no substitute for careful clinical examination before and after operation. The ENT Surgeon Each patient is examined to exclude aural disease or malformation. Faciostenosis frequently causes abnormalities in the physiology of the nasal airways and paranasal sinuses which should be assessed and managed by the ENT surgeon. The Geneticist Because. so many of the craniosynostoses and craniosynostosis syndromes have a genetic background, the involvement of the medical geneticist in diagnosis aad subsequent counselling is of the utmost importance. The genetic evaluation includes a detailed family and pregnancy history including questions about environmental and medical agents and teratogens. Recording of this information for the description of syndromes is also important. The Orthodontist, Dentist, and Oral Surgeon The dental specialists are involved in the planning procedures. They attend to dental hygiene, make the dental casts and the cephalometric tracings. In the preoperative period they apply the orthodontic appliances for intermaxillary fixation. The orthodontist collects baseline cephalometric data for longitudinal facial growth studies in the various areas of craniofacial deformity, and is also involved in the management of such residual dental problems as are amenable to treatment by orthodontic techniques. The Prosthodontist and the Maxillofacial Technician The prosthodontist fashions prosthetic appliances for patients with jaw anomalies when these are required and also makes splints for those edentulous patients who may require intermaxillary fixation. A specialist maxillofacial technician has been trained The Team 299 to make facial and cranial moulages, to cut and mount the dental casts, and to fashion the cast metal splints used in intermaxillary fixation. He is also occasionally asked to make artificial eyes and ears. The Radiologist The radiologist is responsible for making and interpreting the X-ray investigations discussed in Chap. 7. Routine skull radiographs are always examined as a first step. Three-dimensional tomography of the orbits and skull base is usually needed, and its interpretation needs much experience. Computerised axial tomography is becoming a routine requirement. The standard lateral and anteroposterior skull films taken on a cephalostat are used for operative planning and to plot growth up till the time of surgery, and after operation till stability has been achieved. The Pyschosocial