CRANIOFACIAL SURGERY
DAVID
CRANIOFACIAL SURGERY: THE TEAM APPROACH DAVIDJOHN DAVID Cranio-Facial Clinic, Adelaide Children’s Hosfital and Royal Adelaide Hospital Craniofacial surgery offers a new hope to some grossly deformed people. T h i s complex surgery, which is based on a multidisciplinary team approach, needs to be carefully rationalized and regionalized to facilitate investigation, to improve planning, t o reduce the number of complications, and t o conserve financial resources. To date the CranioFacial Clinic a t t h e Adelaide Children’s Hospital and the Royal Adelaide Hospltal has reviewed 37 cases and operated upon 13 ‘of these. This work is presented together with a review of the team approach.
THE principles and practice of craniofacial surgery are now well established. This service is widely offered in a number of centres around the world to patients suffering from severe facial deformities. Paul Tessier (1967) has developed and perfected these techniques over the last decade, demonstrating that wide and adequate exposure of the craniofacial skeleton can be obtained by subperiosteal stripping of bones, cutting and repositioning the skeleton, and reconstructing the deficiencies by bone grafts. H e pioneered the team approach to these problems by combining with his neurosurgical colleagues to expose the base of the anterior cranial fossa, which is so often the keystone in the correction of these deformities. Because this kind of surgery is associated with a high risk of morbidity and mortality, there is a special need for the multidisciplinary approach. The team has been expanded to include all of the specialities necessary for the complete assessment and follow-up of these patients. Those units in which the surgery has been successfully pioneered and advanced have made their advances on this basis. The organization that has been established in South Australia will be described. The patients who have been assessed and those who have been operated upon will be discussed, together with the implications of organizing and maintaining such a service. Address for reprints : 326 South Terrace, Adelaide, South Australia 5000. AUST. N.Z. J.
SURG.,
VOL. 47-”o.
2,
APRIL,1977
THETEAM The composition of the team is shown in Table I. With the approach to surgery of a team of this magnitude, the risks can be kept to a minimum, but also, and more importantly, the results can be adequately measured so that the continuance of the concept of craniofacial surgery can be justified. The team must of TABLEI Craniofacial Team
Plastic surgeon Neurosurgeon Anasthetist Ophthalmologist E.N.T. surgeon
Orthodontist Oral surgeon Prosthodontist Psychiatrist Social worker Photographer
necessity be large. In the preoperative investigation it is responsible for data collection, diagnosis, and operative design, and the responsibilities of its members are as follows. ( a ) Plastic sztrgeon.-The plastic surgeon is the head of the team and as such assumes responsibility for patient care. Special training in neurosurgery, orthopzdic surgery, and maxillofacial surgery is recommended. It has been suggested that craniofacial surgery should become a separate speciality (Munro, 1975). This is probably an extreme point of view, but the plastic surgeon must not only be capable of performing the bony surgery, but also be able to correct the many difficult soft tissue problems that are always associated with craniofacial deformities. I93
CRANIOFACIAL SURGERY
( b ) Neurosurgeon.-It is important that the neurosurgeon’s role be seen as an extension of the one already played in the correction of skull deformities resulting from craniosynostosis. The neurosurgeon is responsible for the preoperative neurological assessment of the patient and, when the transcranial approach is used, for the exposure of the anterior cranial fossa. A high degree of cooperation is required throughout these procedures, with a blending of the techniques of plastic surgery and neurosurgery. (c) Anesthetist.-The importance of the anzesthetist’s role is highlighted by the fact that craniofacial operations are frequently in excess of 12 hours’ duration and are performed on patients whose ages range from five to 40 years. Large blood replacements are invariably necessary. ( d ) Orthodontist and oral surgeon.-These dental disciplines are involved in the planning procedures (wide infra) . Many patients need presurgical or postsurgical orthodontic treatment. TABLE 2
Routine Preoperatwe Skiagrams Routine skull and facial views Anteropostenor and lateral skull (cephalometnc) Anteropostenor and lateral tomography of orbits Anteroposterior and lateral chest
( e ) Ophthahologist. - The concept of craniofacial surgery is based on the fact that the orbits can be safely moved in three dimensions, therefore a detailed preoperative examination by a neuro-ophthalmologist is essential. A close examination of the following is of the greatest importance : interorbital distance ; interpupillary distance ; lateral canthal width ; p a 1 p e b r a 1 fissure width ; exophthalmometry ; visual acuity ; visual fields ; eye position ; muscle function ; and fundoscopy.
DAVID
( 9 ) Speech pathologist.-Surgery for deformity of the mid-face frequently involves sagittal displacement of the bones with a possible change in the relationship of the components of the velopharyangeal sphincter. Preoperative assessment involves an interview, speech recording and nasendoscopy. These procedures are repeated after operation. ( h ) Otorhinoluryngo2ogist.-Each patient is checked for middle and inner ear problems. Midfacial stenosis frequently creates secondary nasal and paranasal sinus problems which need to be assessed and managed by the ear, nose and throat surgeon. TABLE3 Craniafacial Deformities Assdssed in South Australia Crouzon’s syndrome ,. .. .. .. Apert’s syndrome 1 s t and 2nd branchial a&h syndrome’ ’ ‘’ Cleft lip and palate with maxillary hypoplasia Faciostenosis .. . . . . . . . . Hypertelorism .. ._ .. _ . .. Orbital dystopia ,. .. .. .. Uncorrected trauma .. .. .. ., Others .. .. .. .. .. ..
7 2
5
15 2
2
z I I
(i) Social worker. - Throughout time, people have associated mental retardation with a deformed face. An assessment of the patient’s intelligence should be made before operation. A thorough knowledge of the patient and his environment is needed for assessment as an indication for surgery, and as a base line for subsequent follow-up. The true worth of these surgical achievements can only be TABLE4 Osteotomks Performed Transcranial correction of hypertelorism Fronto-orbital advancement High Le Fort I osteotomy x 4 Le Fort I1 osteotomy Le Fort I1 osteotomy+mandibular osteotomy Le Fort Ill osteotomy, subcranial Le Fort I11 osteotomy+mandibular osteotomy Le Fort 111 osteotomy, transcranial
( f ) Radiologist.-The radiologist is responsible for taking and interpreting the skiagrams. The routine views taken are shown in Table 2. In addition, cerebral arteriograms, air studies and brain scans are often needed. The standard lateral and anteroposterior skull films, taken on a cephalostat, are used for the operative planning and to plot growth up to the time of surgery, and after operation until stability has been achieved.
measured in terms of patient satisfaction, and this measurement must of necessity be made by the psychosocial team. Patients and their families also need preoperative adjustment to the realities of the surgery. Often the severely deformed face cannot be made completely normal. In other patients the sudden drastic change makes them unrecognizable to their friends. Such patients will almost certainly need help in adjustment.
I93
AUST. N.Z. J. SURG., VOL. 47-”o.
2,
APRIL, 1977
DAVID
CRANIOFACIAL SUKGEKY
PATIENTS In the past year the South Australian craniofacial team has assessed 37 people with craniofacial anomalies, and operated on 13 of these (Table 3 ) . From the complex range of osteotomies available, we have undertaken the management of the types shown in Table 4. DESIGNINGTHE OPERATION The operative design cannot be done on an ad hoc basis in the operating theatre. It is necessary to have appropriate cephalometric
necessary to have access to the normal range of measurements for a female aborigine of a particular tribe before it was possible to correct her untreated craniofacial fracture. Many procedures will of necessity involve a simultaneous correction of the mandible as well as the orbitocranial skeleton. These corrections revolve around a correct occlusion, and for this purpose dental study models are cut and mounted on an articulator (Figure 2). In those cases where the middle third of the face is being repositioned we have used the
FIGURE I : (left) preoperative lateral transparent photograph ; (centre) the photograph is sectioned along the proposed osteotomy lines to produce the postoperative appearance ; (right) postoperative appearance after a mid-face advancement and mandibular recession.
skiagrams of the skull. The outline is traced and the standard lines and angles drawn. An accurately superimposable transparent photograph is taken as well. A tracing can be cut up and the angles repositioned into acceptable dimensions. The skiagram and the transparent photograph can be cut out and changed to give a suitable postsurgical prediction of appearance. The methods used have been based on those of Henderson ( 1974), with some modification by the members of the Department of Oral Pathology and Surgery at the University of Adelaide (Figure I ) . In this way the exact size of the defects to he created by the osteotomies and hence the size of the bone grafts can be predicted. Reference to a wide range of “normals” is essential. In one case it was AUST.N.Z. J. SURG.,VOL. 47-No.
2,
APRIL,1977
craniomandibular fixation with external rods fixed at a predetermined distance, so that when the maxilla is moved into occlusion with the mandible, the gaps at the osteotomy sites that must be filled with bone become apparent. The anzesthetic team bases its care on a complete biochemical and hamatological workup with central venous pressure, cardiac, and body temperature monitoring, a spinal catheter also being inserted when a transcranial approach is made. Blood replacement is titrated against the central venous pressure level. In those cases where the upper airway is threatened, a tracheostomy is routinely performed. Tessier (1971) established that the useful orbit can be displaced in the three spatial directions. This phenomenon is the basis of 195
CRANIOFACIAL SURGERY
FIGURE z : Model plan to demonstrate the proposed osteotomy.
FIGURE 3 : Preoperative and postoperative photographs of boy with Crouzon’s disease. The middle third of his face was advanced and lengthened. .AuST.
N.Z. 1. SURG., VOL. 47- NO.
2,
APRIL,1977
CRANIOFACIAL SURGERY
DAVID
many patterns of osteotomies now available to the craniofacial surgeon. Sagittal displacement of the orbits will be applicable to retrusions of the face or frontal bone related to craniosynostosis or faciostenosis. Transverse movement is applicable in the correction of hypertelorism. Vertical displacement will correct orbital malposition secondary to trauma or orbitofacid clefts. A combination of all the movements produces a rotational effect which can: ( i )
I n the view of Paul Tessier, the abnormal anatomy associated with craniofacial dysostosis makes each dissection an adventure in itself. The distorted relafionship between the orbital walls, and the anterior and the middle cranial fossE, presents obvious hazards (Figure 4). Adequate functional results can only be obtained by painstakingly careful dissection and reconstruction with as much as possible being done at the one time because experience has taught that operating for a second time on the orbits is always exceptionally difficult. So gross is the distortion of the anatomy encountered, especially in cases of craniofacial dysostosis, that no animal skull or even human cadaver can be used as a source of experimentation.
INCIDENCE AND EPIDEMIOLOGY Table 5 gives a list of the diseases that could be treated by the craniofacial team. The frequency of these deformities in the Australian community is uncertain. If we take a projection against the figures for Britain and the United States of America as shown on this TABLE5
FIGURE 4: Lateral skiagram of skull, showing the distorted relationship between the cranial base and the face in craniofacial dysostosis.
increase one or both orbital diameters; (ii) correct maxillary open bite; and (iii) lengthen a short face (Figure 3 ) . The principles of surgery are essentially simple. They are: I. Careful dissection of periorbitum and pericranium permits every osteotomy and every displacement without major risk to optic, oculomotor, palpebral, or lachrymal functions. 2. I t is possible to perform a one-stage correction of all displacements, malformations, and bony defects, as well as correction of the canthi and lachrymal systems. 3. For the orbital roof, the cranial route is the simplest and safest. The dura mater can be preserved or reinforced if necessary. 4. The orbitocranial and maxillofacial problems can be dealt with together as they are essentially inseparable. AUST. N.Z. J.
SURG.,
VOL.47-N0.
2,
APRIL,1977
Incidence New cases (live births) per year in AustraEa
Types
. . .. Craniofacial dpostosis rst, 2nd branchial arch syndromes Treacher-Collinssyndrome .. Hypertelorism .. ., .. Faciostenosis ,. .. .. Rare facial and orbital clefts .. Uncorrected trauma .. , . Severe cleft lip and palate ,.
.. .. ., .. .. .. .. ..
I I
: 170,000 : 6,000
I
:
10,000
? ? ? ? ?
-2
40
24 ? ? ? ? ?
table against our population of approximately 133 million, which produces approximately 240,000 live births per year, then some idea of the occurrence rate of these deformities in our community can be gained, although in precise terms this remains very obscure. The exact population that the craniofacial team should serve is obviously to some extent arbitrary. Paul Tessier has suggested that one team per 10 to 20 million people would he suitable, if the team performed onlv craniofacial surgery. On this basis, Ian Munro (197s) has suggested that Canada and the United States be served by seven craniofacial centres. It is a well-known fact, from experience gained by kidney transplant pro'97
GARRETT AND KOSSOFF
ULTRASONIC ECHOGRAPHY
grammes, that any centre performing less than 25 transplants per year has a considerably higher mortality than a centre performing more than 100 (Scribner, 1973). This fact, combined with the rapid rise in medical costs, would indicate that regionalization and rationalization of such a service are almost an economic as well as a medical necessity. CONCLUSIONS There is an obvious need for accurate diagnosis to enable records to be compiled to facilitate the epidemiological studies of craniofacial deformities. 2 . Accurate preoperative and postoperative measurements with access to standard records and routine follow-up to assess the late technical results are obviously necessary. 3. Because this surgery is difficult technically and carries a high morbidity and mortality rate, the craniofacial team must be skilled and experienced. 4. Accurate assessment of the psychosocial status of the patient and the subsequent followup results determine the true value of such surgery in the long term, namely the effect on the patient himself and his family. I.
5. Accumulation of skill in a central unit enables the development of the discipline and makes it possible for the teaching of a new generation of craniofacial surgeons. ACKNOWLEDGEMENTS I wish to acknowledge the contribution made by the other members of the South Australian Cranio-Facial Team, namely : Mr D. Simpson, neurosurgeon; Dr G. Keith, ophthalmologist ; Dr T. Allen, amsthetist ; Dr R. Edwards, anzesthetist ; Mr J. Rice, otorhinolaryngologist ; Dr M. Nugent, orthodontist; Dr J. Herd, oral surgeon; D r K. Moore, prosthodontist ; Mrs J. A. Barritt, social worker ; Miss C. Shepherd, social worker ; Mrs A. Bagnall, speech pathologist ; and members of the nursing staff and Photographic Departments of the Royal Adelaide Hospital and the Adelaide Children’s Hospitals. REFERENCES HENDERSON, D. (1g74), Brit. J. pkrst. Surg., 27: 287. MUNRO,I. R. (1g75), Plast. reconstr. Surg., 5 5 : 170. SCXIBNER, B. H. (1973)~J. Amer. med. Ass., 226: 1125. TESSIER,P. (1g67), Ann. Chir. blast., 12: 273. TESSIER, P. (1971). Transactions of Fifth International Congress of Plastic and Reconstructive Surgery, Butterworths, Melbourne : 903.
ULTRASONIC ECHOGRAFHY IN THE DIAGNOSIS OF ABDOMINAL DISEASE WILLIAMJ. GARRETTAND GEORGEKOSSOFF Royal Hospital f m W o m e n and Ultrasonics Institute, Sydney T h e place of two-dimensional ultrasonic echography in the diseases of the abdomen is outlined, and Illustrative cases are is safe and can be repeated as often as required. It does not radionuclide examinations, but complements those
IN 1959, when the dangers of ionizing radiation were under general discussion and ways and means were being sought to reduce radiation risk from diagnostic X-ray examina-
diagnosis of surgical described. T h e method replace radlologlcal or techniques.
Reprints : W. J. Garrett, F.R.A.C.S., Director, Department of Diagnostic Ultrasound, Royal Hospital for Women, Paddington, N.S.W. 2021.
tion, we established a programme of research in Sydney to develop machines using high frequency ultrasound for the two-dimensional display of anatomical sections. Ultrasound, did not appear to being carry the risk of tissue damage known to exist with ionizing radiation, and subsequent studies
198
AUST. N.Z.J. SURC.,VOL.47-No.
2,
APRIL, 1977
DAVID
CRANIOFACIAL SURGERY: THE TEAM APPROACH DAVIDJOHN DAVID Cranio-Facial Clinic, Adelaide Children’s Hosfital and Royal Adelaide Hospital Craniofacial surgery offers a new hope to some grossly deformed people. T h i s complex surgery, which is based on a multidisciplinary team approach, needs to be carefully rationalized and regionalized to facilitate investigation, to improve planning, t o reduce the number of complications, and t o conserve financial resources. To date the CranioFacial Clinic a t t h e Adelaide Children’s Hospital and the Royal Adelaide Hospltal has reviewed 37 cases and operated upon 13 ‘of these. This work is presented together with a review of the team approach.
THE principles and practice of craniofacial surgery are now well established. This service is widely offered in a number of centres around the world to patients suffering from severe facial deformities. Paul Tessier (1967) has developed and perfected these techniques over the last decade, demonstrating that wide and adequate exposure of the craniofacial skeleton can be obtained by subperiosteal stripping of bones, cutting and repositioning the skeleton, and reconstructing the deficiencies by bone grafts. H e pioneered the team approach to these problems by combining with his neurosurgical colleagues to expose the base of the anterior cranial fossa, which is so often the keystone in the correction of these deformities. Because this kind of surgery is associated with a high risk of morbidity and mortality, there is a special need for the multidisciplinary approach. The team has been expanded to include all of the specialities necessary for the complete assessment and follow-up of these patients. Those units in which the surgery has been successfully pioneered and advanced have made their advances on this basis. The organization that has been established in South Australia will be described. The patients who have been assessed and those who have been operated upon will be discussed, together with the implications of organizing and maintaining such a service. Address for reprints : 326 South Terrace, Adelaide, South Australia 5000. AUST. N.Z. J.
SURG.,
VOL. 47-”o.
2,
APRIL,1977
THETEAM The composition of the team is shown in Table I. With the approach to surgery of a team of this magnitude, the risks can be kept to a minimum, but also, and more importantly, the results can be adequately measured so that the continuance of the concept of craniofacial surgery can be justified. The team must of TABLEI Craniofacial Team
Plastic surgeon Neurosurgeon Anasthetist Ophthalmologist E.N.T. surgeon
Orthodontist Oral surgeon Prosthodontist Psychiatrist Social worker Photographer
necessity be large. In the preoperative investigation it is responsible for data collection, diagnosis, and operative design, and the responsibilities of its members are as follows. ( a ) Plastic sztrgeon.-The plastic surgeon is the head of the team and as such assumes responsibility for patient care. Special training in neurosurgery, orthopzdic surgery, and maxillofacial surgery is recommended. It has been suggested that craniofacial surgery should become a separate speciality (Munro, 1975). This is probably an extreme point of view, but the plastic surgeon must not only be capable of performing the bony surgery, but also be able to correct the many difficult soft tissue problems that are always associated with craniofacial deformities. I93
CRANIOFACIAL SURGERY
( b ) Neurosurgeon.-It is important that the neurosurgeon’s role be seen as an extension of the one already played in the correction of skull deformities resulting from craniosynostosis. The neurosurgeon is responsible for the preoperative neurological assessment of the patient and, when the transcranial approach is used, for the exposure of the anterior cranial fossa. A high degree of cooperation is required throughout these procedures, with a blending of the techniques of plastic surgery and neurosurgery. (c) Anesthetist.-The importance of the anzesthetist’s role is highlighted by the fact that craniofacial operations are frequently in excess of 12 hours’ duration and are performed on patients whose ages range from five to 40 years. Large blood replacements are invariably necessary. ( d ) Orthodontist and oral surgeon.-These dental disciplines are involved in the planning procedures (wide infra) . Many patients need presurgical or postsurgical orthodontic treatment. TABLE 2
Routine Preoperatwe Skiagrams Routine skull and facial views Anteropostenor and lateral skull (cephalometnc) Anteropostenor and lateral tomography of orbits Anteroposterior and lateral chest
( e ) Ophthahologist. - The concept of craniofacial surgery is based on the fact that the orbits can be safely moved in three dimensions, therefore a detailed preoperative examination by a neuro-ophthalmologist is essential. A close examination of the following is of the greatest importance : interorbital distance ; interpupillary distance ; lateral canthal width ; p a 1 p e b r a 1 fissure width ; exophthalmometry ; visual acuity ; visual fields ; eye position ; muscle function ; and fundoscopy.
DAVID
( 9 ) Speech pathologist.-Surgery for deformity of the mid-face frequently involves sagittal displacement of the bones with a possible change in the relationship of the components of the velopharyangeal sphincter. Preoperative assessment involves an interview, speech recording and nasendoscopy. These procedures are repeated after operation. ( h ) Otorhinoluryngo2ogist.-Each patient is checked for middle and inner ear problems. Midfacial stenosis frequently creates secondary nasal and paranasal sinus problems which need to be assessed and managed by the ear, nose and throat surgeon. TABLE3 Craniafacial Deformities Assdssed in South Australia Crouzon’s syndrome ,. .. .. .. Apert’s syndrome 1 s t and 2nd branchial a&h syndrome’ ’ ‘’ Cleft lip and palate with maxillary hypoplasia Faciostenosis .. . . . . . . . . Hypertelorism .. ._ .. _ . .. Orbital dystopia ,. .. .. .. Uncorrected trauma .. .. .. ., Others .. .. .. .. .. ..
7 2
5
15 2
2
z I I
(i) Social worker. - Throughout time, people have associated mental retardation with a deformed face. An assessment of the patient’s intelligence should be made before operation. A thorough knowledge of the patient and his environment is needed for assessment as an indication for surgery, and as a base line for subsequent follow-up. The true worth of these surgical achievements can only be TABLE4 Osteotomks Performed Transcranial correction of hypertelorism Fronto-orbital advancement High Le Fort I osteotomy x 4 Le Fort I1 osteotomy Le Fort I1 osteotomy+mandibular osteotomy Le Fort Ill osteotomy, subcranial Le Fort I11 osteotomy+mandibular osteotomy Le Fort 111 osteotomy, transcranial
( f ) Radiologist.-The radiologist is responsible for taking and interpreting the skiagrams. The routine views taken are shown in Table 2. In addition, cerebral arteriograms, air studies and brain scans are often needed. The standard lateral and anteroposterior skull films, taken on a cephalostat, are used for the operative planning and to plot growth up to the time of surgery, and after operation until stability has been achieved.
measured in terms of patient satisfaction, and this measurement must of necessity be made by the psychosocial team. Patients and their families also need preoperative adjustment to the realities of the surgery. Often the severely deformed face cannot be made completely normal. In other patients the sudden drastic change makes them unrecognizable to their friends. Such patients will almost certainly need help in adjustment.
I93
AUST. N.Z. J. SURG., VOL. 47-”o.
2,
APRIL, 1977
DAVID
CRANIOFACIAL SUKGEKY
PATIENTS In the past year the South Australian craniofacial team has assessed 37 people with craniofacial anomalies, and operated on 13 of these (Table 3 ) . From the complex range of osteotomies available, we have undertaken the management of the types shown in Table 4. DESIGNINGTHE OPERATION The operative design cannot be done on an ad hoc basis in the operating theatre. It is necessary to have appropriate cephalometric
necessary to have access to the normal range of measurements for a female aborigine of a particular tribe before it was possible to correct her untreated craniofacial fracture. Many procedures will of necessity involve a simultaneous correction of the mandible as well as the orbitocranial skeleton. These corrections revolve around a correct occlusion, and for this purpose dental study models are cut and mounted on an articulator (Figure 2). In those cases where the middle third of the face is being repositioned we have used the
FIGURE I : (left) preoperative lateral transparent photograph ; (centre) the photograph is sectioned along the proposed osteotomy lines to produce the postoperative appearance ; (right) postoperative appearance after a mid-face advancement and mandibular recession.
skiagrams of the skull. The outline is traced and the standard lines and angles drawn. An accurately superimposable transparent photograph is taken as well. A tracing can be cut up and the angles repositioned into acceptable dimensions. The skiagram and the transparent photograph can be cut out and changed to give a suitable postsurgical prediction of appearance. The methods used have been based on those of Henderson ( 1974), with some modification by the members of the Department of Oral Pathology and Surgery at the University of Adelaide (Figure I ) . In this way the exact size of the defects to he created by the osteotomies and hence the size of the bone grafts can be predicted. Reference to a wide range of “normals” is essential. In one case it was AUST.N.Z. J. SURG.,VOL. 47-No.
2,
APRIL,1977
craniomandibular fixation with external rods fixed at a predetermined distance, so that when the maxilla is moved into occlusion with the mandible, the gaps at the osteotomy sites that must be filled with bone become apparent. The anzesthetic team bases its care on a complete biochemical and hamatological workup with central venous pressure, cardiac, and body temperature monitoring, a spinal catheter also being inserted when a transcranial approach is made. Blood replacement is titrated against the central venous pressure level. In those cases where the upper airway is threatened, a tracheostomy is routinely performed. Tessier (1971) established that the useful orbit can be displaced in the three spatial directions. This phenomenon is the basis of 195
CRANIOFACIAL SURGERY
FIGURE z : Model plan to demonstrate the proposed osteotomy.
FIGURE 3 : Preoperative and postoperative photographs of boy with Crouzon’s disease. The middle third of his face was advanced and lengthened. .AuST.
N.Z. 1. SURG., VOL. 47- NO.
2,
APRIL,1977
CRANIOFACIAL SURGERY
DAVID
many patterns of osteotomies now available to the craniofacial surgeon. Sagittal displacement of the orbits will be applicable to retrusions of the face or frontal bone related to craniosynostosis or faciostenosis. Transverse movement is applicable in the correction of hypertelorism. Vertical displacement will correct orbital malposition secondary to trauma or orbitofacid clefts. A combination of all the movements produces a rotational effect which can: ( i )
I n the view of Paul Tessier, the abnormal anatomy associated with craniofacial dysostosis makes each dissection an adventure in itself. The distorted relafionship between the orbital walls, and the anterior and the middle cranial fossE, presents obvious hazards (Figure 4). Adequate functional results can only be obtained by painstakingly careful dissection and reconstruction with as much as possible being done at the one time because experience has taught that operating for a second time on the orbits is always exceptionally difficult. So gross is the distortion of the anatomy encountered, especially in cases of craniofacial dysostosis, that no animal skull or even human cadaver can be used as a source of experimentation.
INCIDENCE AND EPIDEMIOLOGY Table 5 gives a list of the diseases that could be treated by the craniofacial team. The frequency of these deformities in the Australian community is uncertain. If we take a projection against the figures for Britain and the United States of America as shown on this TABLE5
FIGURE 4: Lateral skiagram of skull, showing the distorted relationship between the cranial base and the face in craniofacial dysostosis.
increase one or both orbital diameters; (ii) correct maxillary open bite; and (iii) lengthen a short face (Figure 3 ) . The principles of surgery are essentially simple. They are: I. Careful dissection of periorbitum and pericranium permits every osteotomy and every displacement without major risk to optic, oculomotor, palpebral, or lachrymal functions. 2. I t is possible to perform a one-stage correction of all displacements, malformations, and bony defects, as well as correction of the canthi and lachrymal systems. 3. For the orbital roof, the cranial route is the simplest and safest. The dura mater can be preserved or reinforced if necessary. 4. The orbitocranial and maxillofacial problems can be dealt with together as they are essentially inseparable. AUST. N.Z. J.
SURG.,
VOL.47-N0.
2,
APRIL,1977
Incidence New cases (live births) per year in AustraEa
Types
. . .. Craniofacial dpostosis rst, 2nd branchial arch syndromes Treacher-Collinssyndrome .. Hypertelorism .. ., .. Faciostenosis ,. .. .. Rare facial and orbital clefts .. Uncorrected trauma .. , . Severe cleft lip and palate ,.
.. .. ., .. .. .. .. ..
I I
: 170,000 : 6,000
I
:
10,000
? ? ? ? ?
-2
40
24 ? ? ? ? ?
table against our population of approximately 133 million, which produces approximately 240,000 live births per year, then some idea of the occurrence rate of these deformities in our community can be gained, although in precise terms this remains very obscure. The exact population that the craniofacial team should serve is obviously to some extent arbitrary. Paul Tessier has suggested that one team per 10 to 20 million people would he suitable, if the team performed onlv craniofacial surgery. On this basis, Ian Munro (197s) has suggested that Canada and the United States be served by seven craniofacial centres. It is a well-known fact, from experience gained by kidney transplant pro'97
GARRETT AND KOSSOFF
ULTRASONIC ECHOGRAPHY
grammes, that any centre performing less than 25 transplants per year has a considerably higher mortality than a centre performing more than 100 (Scribner, 1973). This fact, combined with the rapid rise in medical costs, would indicate that regionalization and rationalization of such a service are almost an economic as well as a medical necessity. CONCLUSIONS There is an obvious need for accurate diagnosis to enable records to be compiled to facilitate the epidemiological studies of craniofacial deformities. 2 . Accurate preoperative and postoperative measurements with access to standard records and routine follow-up to assess the late technical results are obviously necessary. 3. Because this surgery is difficult technically and carries a high morbidity and mortality rate, the craniofacial team must be skilled and experienced. 4. Accurate assessment of the psychosocial status of the patient and the subsequent followup results determine the true value of such surgery in the long term, namely the effect on the patient himself and his family. I.
5. Accumulation of skill in a central unit enables the development of the discipline and makes it possible for the teaching of a new generation of craniofacial surgeons. ACKNOWLEDGEMENTS I wish to acknowledge the contribution made by the other members of the South Australian Cranio-Facial Team, namely : Mr D. Simpson, neurosurgeon; Dr G. Keith, ophthalmologist ; Dr T. Allen, amsthetist ; Dr R. Edwards, anzesthetist ; Mr J. Rice, otorhinolaryngologist ; Dr M. Nugent, orthodontist; Dr J. Herd, oral surgeon; D r K. Moore, prosthodontist ; Mrs J. A. Barritt, social worker ; Miss C. Shepherd, social worker ; Mrs A. Bagnall, speech pathologist ; and members of the nursing staff and Photographic Departments of the Royal Adelaide Hospital and the Adelaide Children’s Hospitals. REFERENCES HENDERSON, D. (1g74), Brit. J. pkrst. Surg., 27: 287. MUNRO,I. R. (1g75), Plast. reconstr. Surg., 5 5 : 170. SCXIBNER, B. H. (1973)~J. Amer. med. Ass., 226: 1125. TESSIER,P. (1g67), Ann. Chir. blast., 12: 273. TESSIER, P. (1971). Transactions of Fifth International Congress of Plastic and Reconstructive Surgery, Butterworths, Melbourne : 903.
ULTRASONIC ECHOGRAFHY IN THE DIAGNOSIS OF ABDOMINAL DISEASE WILLIAMJ. GARRETTAND GEORGEKOSSOFF Royal Hospital f m W o m e n and Ultrasonics Institute, Sydney T h e place of two-dimensional ultrasonic echography in the diseases of the abdomen is outlined, and Illustrative cases are is safe and can be repeated as often as required. It does not radionuclide examinations, but complements those
IN 1959, when the dangers of ionizing radiation were under general discussion and ways and means were being sought to reduce radiation risk from diagnostic X-ray examina-
diagnosis of surgical described. T h e method replace radlologlcal or techniques.
Reprints : W. J. Garrett, F.R.A.C.S., Director, Department of Diagnostic Ultrasound, Royal Hospital for Women, Paddington, N.S.W. 2021.
tion, we established a programme of research in Sydney to develop machines using high frequency ultrasound for the two-dimensional display of anatomical sections. Ultrasound, did not appear to being carry the risk of tissue damage known to exist with ionizing radiation, and subsequent studies
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