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Asymmetries caused by premature craniosynostoses
Symposium of Facial Asymmetry
It primarily provides a tool for surgical planning and to a lesser degree for radiation therapy planning. Over the years, 3-D imaging has become applicable not only for cranio-maxillo-facial surgery (Marsh and Vannier, 1985; David et al., 1990) and orthopaedic surgery (Totty and Vannier, 1984), but also for neurosurgery, cardiovascular surgery, ENT surgery and general surgery (Zonneveld and Fukuta, 1995). The data that form the foundation of 3-D images can also be used for the manufacturing of models which are particularly useful in maxillofacial surgery (Zonneveld, 1994). At our institution, we have currently performed almost 1000 3-D imaging studies, over 500 of which deal with cranio-maxillo-facial deformities such as craniofacial synostosis, cranial- and craniofacial dysplasia, congenital- and acquired craniofacial neoplasms and craniofacial trauma. These studies were not primarily done to make a diagnosis, but to assess the extent and complexity of the deformity for the purpose of detailed surgical planning. In the beginning only bone deformities were imaged (Zonneveld et al., 1989a), including interactive disarticulation of joints such as the temporomandibular joint (Zonneveld et al., 1989b), but later the imaging of soft tissue structures became just as important; including the quantitative assessment of soft tissue volumes (Zonnereid et al., 1991a). Since 1989, we have used physical models, either to mimic complex surgery or to design implants. These models were usually milled from P U R foam using a numerically controlled milling machine. Although still using this technique (Zonnereid et al., 1991b; Zonneveld et al., 1992), at the same time we are testing the feasibility of a new technique called 'stereolithography'. This latter technique involves computer-controlled solidification of a photopolymer by means of ultraviolet light. Since 1993, we have begun using 3-D display techniques for producing autostereoscopic full-color hardcopies to enhance the understanding of spatial relationships in the 3-D images (Zonneveld et al., 1993). In this presentation we will discuss the various techniques and illustrate them with relevant case material. Given the theme of this conference, whenever possible, emphasis will be put on asymmetrical
congenital dysplasias (e.g. hemifacial microsomia, hemifacial hypertrophy, Romberg and Treacher Collins syndromes), different types of asymmetrical craniosynostosis (e.g. plagiocephaly), radiation-induced asymmetries (e.g. irradiated retionoblastoma), and trauma. References David, D. J., D. C. Hemmy, R. D. Cooter : Craniofacial
deformities. Atlas of three-dimensional reconstruction from computed tomography. Springer Verlag, New York, 1990 Hemmy, D. C., 1=. W. Zonneveld, S. Lobregt, K. Fukuta: A decade of clinical three-dimensional imaging: Part I. Historical development. Invest. Radiol. 29 (1994) [acc. for public.] Herman, G. T: Three-dimensional imaging medicine. A response to a critique by surgeons. Scan& J. Plast. Reconstr. Surg. 22 (1988) 61-67 Marsh, J. L., M. W. Vannier : Comprehensive care for craniofacial deformities. CV Mosby, St Louis 1985 Tessire, P. L., D. C. Hemmy : Three dimensional imaging in medicine. A critique by surgeons. Scan& J. Past Reconstr. Surg. 20 (1986) 3-11 Totty, W. G., M. W. Vannier : Complex musculoskeletal anatomy: analysis using three-dimensional surface reconstruction. Radiology 150 (1984) 173-17 Zonneveld, F. IV., S. Lobregt, J. C. van der Meulen, J. M. Vaandrager: Three-dimensional imaging in craniofacial
surgery. World J. Surg. 13 (1989a) 328-342 Zonneveld, F. IV., J. M. Vaandrager, M. F. Noorman van der Dussen, J. C. van der Meulen: Value of disarticulation of the
mandible in 3-D imaging. Proc. CAR'89, Springer Verlag, Berlin 1989b, 372-377 Zonneveld, F. W., L. Koornneef, D. WitteboLPost: Quantitative volumetric assessment of orbital soft tissue. Proc CAR'91, Springer Verlag, Berlin 1991a, 181 186 Zonneveld, F. W., M. F. Noorman van der Dussen, U. G. Kliegis, P. F. G. M. van Waes." Volumetric CT-based model milling in
rehearsing surgery. Proc CAR'91, Springer Verlag, Berlin 1991b, 347-353 Zonneveld, F. W., M. F. Noorman van der Dussen: Threedimensional imaging and model fabrication in oral and maxillofacial surgery. Oral and Maxillofac. Surg. Clin. N. Am. 4/1 (1992) 1%33 Zonneveld, F. W., A. M. Fermindez, A. Joel, A. G. M. Huitema, P. F. G. M. van Waes : The lenticular system. A full-colour
limited angle autostereoscopic alternative to holography [abs]. ECR'93 Book of Abstracts, Springer Verlag, Berlin 1993, 393 Zonneveld, F. IV., K. Fukuta : A decade of clinical threedimensional imaging: Part II. Clinical applications. Invest. Radiol. 29 (1994) [acc. for publ.] Zonneveld, F. IV.: A decade of clinical three-dimensional imaging: Part III. Image analysis, display options, and physical models. Invest. Radiol. 29 (1994) [acc. for puN.]
Asymmetries caused by premature craniosynostoses J. Mfihling
Dept. of OMF Surgery, University Hospital. Heidelberg, Germany Facial asymmetries are often caused by craniosynostoses. This implies premature fusion of one or more cranial sutures. The aetiology of craniosynostosis has not been classified as yet. The pathological impact on growth, however, has already been described by Virchow in 1852. He recognised that, as a consequence of synostosis, the cranial growth perpendicular to the
3
affected suture is inhibited while overgrowth is seen in the direction of the synostosis. Thus, depending on the localisation of the synostosis, typical deformities of the neuro- and viscerocranium develop. Vice versa, the malformation present allows to draw a conclusion with regard to the suture affected. Facial asymmetries are, in most cases, caused by
4
Journal of Cranio-Maxillo-Facial Surgery
synostosis of one coronal suture, but also by bilateral synostoses that may be of different degree of severity. The growth inhibition is not only confined to the neurocranium, but also may cause facial scoliosis. This malformation is called plagiocephalus, caused by synostosis o f the coronal suture. The forehead flattens on the affected side, at the same time the facial axis turns to the non-affected side because of the higher growth pressure in the direction of the affected suture. As a result, the eye axis and occlusal plane are tilted. Although the intracranial volume is restricted there will hardly ever be problems caused by the regionally increased intracranial pressure. Patients (parents) mainly seek treatment because of aesthetic reasons.
Fronto-orbital advancement is the rational treatment in these patients. The timing of the surgery, however, is of great importance, the best moment being the end o f the first year. This premature surgical interventional allows correction of the frontal deformation and normalisation of the regionally increased intracranial pressure. The surgical opening of the cranial sutures stops the disturbed growth of the facial region, so that, during further growth, the facial scoliosis will be eliminated. As a result, extensive surgical interventions as a rule, are not necessary at a later age !
Congenital anophthalmia and facial development A. Rodallec
Centre Med.-Chir. Ophtalmologique. Paris, France Congenital anophthalmia as well as severe microphthalmia usually leads to a micro-orbit associated with facial hemi-hypoplasia. This usually goes along with lack of growth and development of the eyelids and conjunctival sac. The insertion of an aesthetic eye prosthesis is virtually impossible under these circumstances. Stimulation of growth by inserting an intra orbital expansion device seems to be a logical procedure. If possible eye movements are stimulated as well, which in turn will also stimulate orbital growth. Since rapid growth takes place in the first year after birth, it is essential to intervene as early as possible to achieve a maximum result! The technique and results will be shown in this presentation.
Fig. 1. - If there is a lack of eye development,it is not only the bony orbit which does not develop, but all the facial bones contributing to the bony orbit (principally the malar, the frontal, the maxilla, and the greater wing of the sphenoid). Thus an isolated orbital congenital malformation will naturally evolve into a serious hemi craniofacialmalformation which is difficult to correct.
Orbital asymmetry H. F. Sailer
Dept. of Cranio-Maxillofacial surgery, University Hospital. Zurich, Switzerland Introduction/Definition We define orbital asymmetry as differences between both orbits concerning form, volume, position as well as differences in distance of the orbits from the facial midline. Evaluation and measurements are performed by
cephalometric posterior-anterior radiographs and superimposed tracings, quantitative computed tomography (Manson et al., 1986; Carls et al., 1994) and standardized reproducible photographs with superimposed tracings. In orbital asymmetry it is most important to first determine the facial midline as a reference line. In some patients with severe orbital and
It primarily provides a tool for surgical planning and to a lesser degree for radiation therapy planning. Over the years, 3-D imaging has become applicable not only for cranio-maxillo-facial surgery (Marsh and Vannier, 1985; David et al., 1990) and orthopaedic surgery (Totty and Vannier, 1984), but also for neurosurgery, cardiovascular surgery, ENT surgery and general surgery (Zonneveld and Fukuta, 1995). The data that form the foundation of 3-D images can also be used for the manufacturing of models which are particularly useful in maxillofacial surgery (Zonneveld, 1994). At our institution, we have currently performed almost 1000 3-D imaging studies, over 500 of which deal with cranio-maxillo-facial deformities such as craniofacial synostosis, cranial- and craniofacial dysplasia, congenital- and acquired craniofacial neoplasms and craniofacial trauma. These studies were not primarily done to make a diagnosis, but to assess the extent and complexity of the deformity for the purpose of detailed surgical planning. In the beginning only bone deformities were imaged (Zonneveld et al., 1989a), including interactive disarticulation of joints such as the temporomandibular joint (Zonneveld et al., 1989b), but later the imaging of soft tissue structures became just as important; including the quantitative assessment of soft tissue volumes (Zonnereid et al., 1991a). Since 1989, we have used physical models, either to mimic complex surgery or to design implants. These models were usually milled from P U R foam using a numerically controlled milling machine. Although still using this technique (Zonnereid et al., 1991b; Zonneveld et al., 1992), at the same time we are testing the feasibility of a new technique called 'stereolithography'. This latter technique involves computer-controlled solidification of a photopolymer by means of ultraviolet light. Since 1993, we have begun using 3-D display techniques for producing autostereoscopic full-color hardcopies to enhance the understanding of spatial relationships in the 3-D images (Zonneveld et al., 1993). In this presentation we will discuss the various techniques and illustrate them with relevant case material. Given the theme of this conference, whenever possible, emphasis will be put on asymmetrical
congenital dysplasias (e.g. hemifacial microsomia, hemifacial hypertrophy, Romberg and Treacher Collins syndromes), different types of asymmetrical craniosynostosis (e.g. plagiocephaly), radiation-induced asymmetries (e.g. irradiated retionoblastoma), and trauma. References David, D. J., D. C. Hemmy, R. D. Cooter : Craniofacial
deformities. Atlas of three-dimensional reconstruction from computed tomography. Springer Verlag, New York, 1990 Hemmy, D. C., 1=. W. Zonneveld, S. Lobregt, K. Fukuta: A decade of clinical three-dimensional imaging: Part I. Historical development. Invest. Radiol. 29 (1994) [acc. for public.] Herman, G. T: Three-dimensional imaging medicine. A response to a critique by surgeons. Scan& J. Plast. Reconstr. Surg. 22 (1988) 61-67 Marsh, J. L., M. W. Vannier : Comprehensive care for craniofacial deformities. CV Mosby, St Louis 1985 Tessire, P. L., D. C. Hemmy : Three dimensional imaging in medicine. A critique by surgeons. Scan& J. Past Reconstr. Surg. 20 (1986) 3-11 Totty, W. G., M. W. Vannier : Complex musculoskeletal anatomy: analysis using three-dimensional surface reconstruction. Radiology 150 (1984) 173-17 Zonneveld, F. IV., S. Lobregt, J. C. van der Meulen, J. M. Vaandrager: Three-dimensional imaging in craniofacial
surgery. World J. Surg. 13 (1989a) 328-342 Zonneveld, F. IV., J. M. Vaandrager, M. F. Noorman van der Dussen, J. C. van der Meulen: Value of disarticulation of the
mandible in 3-D imaging. Proc. CAR'89, Springer Verlag, Berlin 1989b, 372-377 Zonneveld, F. W., L. Koornneef, D. WitteboLPost: Quantitative volumetric assessment of orbital soft tissue. Proc CAR'91, Springer Verlag, Berlin 1991a, 181 186 Zonneveld, F. W., M. F. Noorman van der Dussen, U. G. Kliegis, P. F. G. M. van Waes." Volumetric CT-based model milling in
rehearsing surgery. Proc CAR'91, Springer Verlag, Berlin 1991b, 347-353 Zonneveld, F. W., M. F. Noorman van der Dussen: Threedimensional imaging and model fabrication in oral and maxillofacial surgery. Oral and Maxillofac. Surg. Clin. N. Am. 4/1 (1992) 1%33 Zonneveld, F. W., A. M. Fermindez, A. Joel, A. G. M. Huitema, P. F. G. M. van Waes : The lenticular system. A full-colour
limited angle autostereoscopic alternative to holography [abs]. ECR'93 Book of Abstracts, Springer Verlag, Berlin 1993, 393 Zonneveld, F. IV., K. Fukuta : A decade of clinical threedimensional imaging: Part II. Clinical applications. Invest. Radiol. 29 (1994) [acc. for publ.] Zonneveld, F. IV.: A decade of clinical three-dimensional imaging: Part III. Image analysis, display options, and physical models. Invest. Radiol. 29 (1994) [acc. for puN.]
Asymmetries caused by premature craniosynostoses J. Mfihling
Dept. of OMF Surgery, University Hospital. Heidelberg, Germany Facial asymmetries are often caused by craniosynostoses. This implies premature fusion of one or more cranial sutures. The aetiology of craniosynostosis has not been classified as yet. The pathological impact on growth, however, has already been described by Virchow in 1852. He recognised that, as a consequence of synostosis, the cranial growth perpendicular to the
3
affected suture is inhibited while overgrowth is seen in the direction of the synostosis. Thus, depending on the localisation of the synostosis, typical deformities of the neuro- and viscerocranium develop. Vice versa, the malformation present allows to draw a conclusion with regard to the suture affected. Facial asymmetries are, in most cases, caused by
4
Journal of Cranio-Maxillo-Facial Surgery
synostosis of one coronal suture, but also by bilateral synostoses that may be of different degree of severity. The growth inhibition is not only confined to the neurocranium, but also may cause facial scoliosis. This malformation is called plagiocephalus, caused by synostosis o f the coronal suture. The forehead flattens on the affected side, at the same time the facial axis turns to the non-affected side because of the higher growth pressure in the direction of the affected suture. As a result, the eye axis and occlusal plane are tilted. Although the intracranial volume is restricted there will hardly ever be problems caused by the regionally increased intracranial pressure. Patients (parents) mainly seek treatment because of aesthetic reasons.
Fronto-orbital advancement is the rational treatment in these patients. The timing of the surgery, however, is of great importance, the best moment being the end o f the first year. This premature surgical interventional allows correction of the frontal deformation and normalisation of the regionally increased intracranial pressure. The surgical opening of the cranial sutures stops the disturbed growth of the facial region, so that, during further growth, the facial scoliosis will be eliminated. As a result, extensive surgical interventions as a rule, are not necessary at a later age !
Congenital anophthalmia and facial development A. Rodallec
Centre Med.-Chir. Ophtalmologique. Paris, France Congenital anophthalmia as well as severe microphthalmia usually leads to a micro-orbit associated with facial hemi-hypoplasia. This usually goes along with lack of growth and development of the eyelids and conjunctival sac. The insertion of an aesthetic eye prosthesis is virtually impossible under these circumstances. Stimulation of growth by inserting an intra orbital expansion device seems to be a logical procedure. If possible eye movements are stimulated as well, which in turn will also stimulate orbital growth. Since rapid growth takes place in the first year after birth, it is essential to intervene as early as possible to achieve a maximum result! The technique and results will be shown in this presentation.
Fig. 1. - If there is a lack of eye development,it is not only the bony orbit which does not develop, but all the facial bones contributing to the bony orbit (principally the malar, the frontal, the maxilla, and the greater wing of the sphenoid). Thus an isolated orbital congenital malformation will naturally evolve into a serious hemi craniofacialmalformation which is difficult to correct.
Orbital asymmetry H. F. Sailer
Dept. of Cranio-Maxillofacial surgery, University Hospital. Zurich, Switzerland Introduction/Definition We define orbital asymmetry as differences between both orbits concerning form, volume, position as well as differences in distance of the orbits from the facial midline. Evaluation and measurements are performed by
cephalometric posterior-anterior radiographs and superimposed tracings, quantitative computed tomography (Manson et al., 1986; Carls et al., 1994) and standardized reproducible photographs with superimposed tracings. In orbital asymmetry it is most important to first determine the facial midline as a reference line. In some patients with severe orbital and