Annals of the Royal College of Surgeons of England (1984) vol. 66

New perspectives in the management of

severe cranio-facial deformity*

DAVID J DAVID FRCS FRACSSouth Australian Cranio-Facial Unit Adelaide Children's Hospital, Royal Adelaide Hospital Australia

Key words: CRAN IOSYNOSTOSIS; FRONTO-ETHMOIDAL MENINGOENCEPHALOCELE; CRANIO-FACIAL TRAUMA; CRANIO-FACIAL CLEFTS;CRANIO-FACIAL SURGERY

SummaryIt is postulated that craniosynostosis is due to a growth abnormality in

all or part ofthe cranial capsule. Release ofthe stenosedpart in thefirstmonths of life will re-establish the balance between the rapidlygrowing brain and eye, and the cranial capsule. Three periods foroperative treatment are described: early, intermediate and late. Only in

the early period can operative treatment restore normal growthdynamics; in the late period the aim is correction of an establisheddeformity.

The relationship between cranial clefts and frontonasal en-

cephaloceles is explored. If the space-occupying encephalocele is

removed early, the distortedfacial bones adopt a more normal position,whereas cranial clefts do not respond to early operation by remoulding.

The treatment of the acquired deformities of acute cranio-facialtrauma have taken on new perspectives with the application of themulti-disciplinary approach and surgical techniques developed in thetreatment of congenital deformities resulting in considerable reductionin the period of hospitalisation.

Introduction'Monstrosities contribute to rectify our opinions.... From wrong

construction of parts arises unnatural action, which by studyingwe may discover the natural action.' John Hunter (1).

John Hunter introduced a scientific attitude towards surgerythat has produced a more logical pattern of approach.However, his capacity to advance the practice of surgery was

distinctly limited by the inadequacies of the techniques of hisday (the tools of surgery changed very little from Romantimes until the mid-19th century). Hunter's ideas of in-ductive and deductive thought have been combined withmodern technology to the utmost effect in solving theproblems of severe cranio-facial deformity. The most signifi-cant advance in this area was taken by Paul Tessier (2) in1967 when he developed a two-stage combined cranio-facialapproach to the orbito-cranio complex. It is this approachwhich has allowed cranio-facial surgery to develop to thesophisticated multi-disciplinary level practised in a numberof units around the world today.

Such a unit has been functioning in South Australia since1975. Centred at the Adelaide Children's Hospital and alsooperating at the Royal Adelaide Hospital, this Unit in-corporates staff from several major hospitals and institutionsin South Australia and since 1977 the Unit has established a

liaison with New Zealand, Hong Kong, Malaysia, Singa-

Address for reprints: DJ David, S.A. Cranio-Facial Unit, AdelaideChildren's Hospital, North Adelaide, S.A., Australia, 5006.* Based on a Hunterian Lecture given at Royal College of Surgeonson May 26th 1983The Editor would welcome any comments on this paper by readers

pore, Papua-New Guinea and Fiji. Tessier indicated that aunit should serve about 30000000 people. Munro extra-polated this concept to 7 units in North America. The size ofthe area will vary with the skills of the general plasticsurgeons. As cranio-facial techniques pass into general use,fewer cases will need to be referred to a cranio-facial unit, butthe need for such units will remain to deal with the extremedeformity, or the failure of conventional treatment. Since1975 the South Australian Cranio-Facial Unit has beenreferred 496 patients; 301 operations were performed, 104 ofwhich were transcranial and 197 subcranial. The aim of thispaper is to present new approaches in the management ofthree categories of severe cranio-facial deformity:(1) The craniosynostoses.(2) Fronto-ethmoidal meningoencephaloceles and their re-

lationship to cranio-facial clefts of the fronto-nasalregion.

(3) Acute cranio-facial injuries.Management of the craniosynostosesCraniosynostosis is premature fusion of the cranial suturesand is frequently associated with skull deformity. RudolphVirchow (3) noted the relationship in 1852 and explainedthat premature fusion of many of the sutures could reducethe cranial capacity; the concept of cranio-stenosis, ornarrowing of the skull.Virchow noted that premature fusion of one suture results

in cessation of growth in the direction perpendicular to thatsuture, and the compensatory over-growth across othersutures results in various cranial deformities.As clinical interest in malformation of the skull intensified,

it became evident that some of the more severe cases ofcraniosynostosis were associated with other birth defects asseen in Crouzon, Apert and Carpenter Syndromes, etc. Inmany cases there were serious associated facial malforma-tions. Surgery designed to decompress the brain could notand did not improve these and new techniques were neededtogether with a new surgical philosophy. In contrast toVirchow's purely mechanical theory, Moss (4) has arguedthat premature sutural fusion is secondary to more funda-mental dysplasia of the skull base. In his view, synostosis is asymptom not a cause of deformity.

This view is not wholly acceptable. For surgeons, the mostcompelling evidence of the role of the sutures is the responseto adequate resection of fused sutures. The re-appearance ofa previously obliterated suture after resection surely suggeststhat regional cranial growth has been released by operationallowing the brain to expand in a more normal way. It seemslogical therefore to give some role to the fused sutures in theproduction of skull deformity.

Management of severe cranio-facial deformity 271

It appears likely that the individual deformities associatedwith craniosynostosis represent dyscephalies due to distortedgrowth of the entire cerebral capsule; including cranial base,vault, pericranium and dura as well as bone. The hypothesisthus expressed that craniosynostosis is a regional failure ofskeletal growth affecting a number of tissues is little morethan a descriptive hypothesis. It still does not shed any morereal light on the fundamental cause except in the minority ofcases that are genetically determined.When craniosynostosis is extreme there may be restriction

of growth of the cerebral capsule producing raised cranialpressure and threatening vision and intellectual capacity.This situation is described as craniostenosis.

In the same way one can speak of orbitostenosis. Theorbits are the incomplete bony capsule of the expandingeyeball and orbital sutural synostosis has been described.The orbital cavity becomes shallow and wide and the eyeballis extruded forwards.

Delaire et al. (5) in 1963 used the analogous term 'facio-stenosis' to describe the midface hypoplasia of Crouzonsyndrome. It is uncertain whether the maxillary hypoplasiaof Crouzon or Apert syndromes represents an intrinsic localgrowth failure associated with premature sutural fusion orwhether it is secondary to a primary dysplasia of the skullbase acting both on the growth of the vault and the facialskeleton.

In summary, premature sutural fusion is seen as animportant local manifestation of an underlying defect in thegrowth of the skull. This defect may be regional as in thesimple calvarial deformities, or it may be generalised.Examples of the generalised disorders of skull growth areseen in the metabolic craniosynostoses and in the more severetypes of complex cranio-facial deformity.Thus far, the concept is in accordance with Moss' argu-

ment that craniosynostosis is not a primary disease process.There is evidence that premature calvarial sutural fusion hasvery real significance in the dynamics of abnormal cranio-cerebral growth. It results in a relatively unyielding cerebralcapsule which fails to respond normally to the forces exertedby the expanding brain. There is now experimental (6) aswell as surgical evidence to support the classical concept ofthe role of the sutures in determining to some extent thenature and severity of the deformity of the skull vault. So aconcept of regional skeletal growth failure emerges. It istempting to take it a little further when one reviews X-rayevidence that suggests that regional growth failure in thevault seems to be exerting upward tension on the developingorbito-frontal region, perhaps through dural tension lines.This would be a reversal of Moss' concept of the basedistorting the calvarium by dural attachment. It is not yetpossible to say whether premature fusion of the facial sutureshas similar autonomous influence in determining the charac-ter of facial deformities; even if future research shows that

this is not so, we believe that the deformities also representthe outcome of regional skeletal growth.The principles of treatment are therefore based on a

knowledge of the pathological process.The surgical relevance of maturity varies in different types

of craniosynostosis and this can be relevant in choosing thetime to operate. We have therefore found it important toconsider treatment in three somewhat arbitrary epochs, asdescribed by David, Poswillo and Simpson (7).(1) The early stage-the first 12 months of life.(2) The intermediate stage-up to 9 years.(3) The late stage 10 years or more.

THE EARLY STAGE

Operation may be performed:(a) for the release of existing pathology, eg exorbitism or

raised intracranial pressure, or(b) prevention or minimising of future cranial and/or facial

deformity.Analysis of the more complex synostoses have led to somenew perspectives.Frontal plagiocephaly, where the deformity results from a fusionofone coronal suture and its basal extensions, or, in the moresevere hemicranial plagiocephaly, from premature fusion ofcoronal, squamosal and lambdoid sutures.The operation of linear craniectomy has been superseded

by a modification of a logical and very elegant operationdevised in Toronto (8). This represents an application ofTessier's original concept of fronto-orbital advancement(Fig. la and b). The procedure can be used as a bilateralfronto-orbital advancement in cases of turricephaly (9).

In these forms of craniosynostosis the facial component ofthe deformity is considered secondary, yet where the primarydefect is left uncorrected the secondary facial problems maybe severe. The effects of early craniectomy on reducingsecondary facial deformity are encouraging but as yetunsubstantiated.Cranijo-facial syndromes Decompressive operations for cranio-stenosis are frequently necessary. The first stage is a coronalcraniectomy and fronto-orbital advancement with subtem-poral craniectomy. The second stage, 2-3 weeks later,consists of parasagittal and lambdoid craniectomies. Theseoperations have relieved the raised intra-cranial pressurewell.

THE INTERMEDIATE STAGE

Operations at this -stage are performed for(a) threats to function, the most common of which are the

effects of extrusion of the- globes and/or recurrent raisedintracranial pressure,

(b) psycho-social distress.Considering this period between 1 and 9 years separately isjustified because the child is now too old to reap the

a bFIG. 1 Infant with frontal plagiocephaly associated with unilateral coronal synostosis, (a) before and (b) after unilateral advancement of thefronto-orbital complex and frontal bone.

272 David J. David

prophylactic benefits of early treatment, yet too young toachieve a definitive cosmetic and functional result. A rangeof procedures is performed during this time, intermediate inthe total treatment plan. In the younger child a fronto-orbital advancement may be sufficient, whereas in the olderchild the more radical operation of fronto-orbital advance-ment and anterior maxillary shift may be indicated.

THE LATE STAGE

These are invariably for psycho-social and cosmetic in-dications but have associated functional advantages.(a) Correction of exorbitism resulting from orbito-stenosis.(b) Release of the upper airway constriction resulting from

faciostenosis.(c) Improved occlusion.In this period the surgical options are more complex. It isnow possible to move parts of the cranial and facial skeletonin three dimensions in order to correct all deformitiesassociated with craniosynostosis. In simple plagiocephaly,fronto-orbital advancement of one or both sides may beadequate. Frontal depression and asymmetry of the mid andlower face may be corrected by adding facial osteotomies tothe fronto-orbital advancement. The osteotomies of the mid-facial skeleton are conveniently categorised in terms of LeFort's classifications of facial fractures, Le Fort I, II and III.All three sections enter the region of the pterygomaxillaryfissure, where the maxilla and palatine bone must bedetached from the pterygoid process. Of course the oste-otomy lines do not exactly duplicate Le Fort's fractures, but

the comparison gives a useful shorthand identification ofthese complex mid-facial procedures. The following pro-cedures have been used in the late stage.(a) Sub-cranial Le Fort III osteotomy and facial advance-

ment, done when the anatomy of the cranial base isrelatively normal and no calvarial reconstruction isneeded.

(b) Transcranial Le Fort III osteotomy and facial advance-ment when the anatomy of the cranial base is such thatthe temporal lobes or the contents of the cribriformfossae are at risk during osteotomy. The procedure maybe combined with a fronto-orbital advancement (Fig. 2aand b). Combined facial osteotomies may be appro-priate, eg Le Fort III and Le Fort I sections. In caseswith exophthalmos and naso-maxillary retrusion butnormal dental occlusion the upper maxillary complexcan be advanced independently of the palate anddentition. This is termed Le Fort III minus Le Fort Isection. Le Fort III and I sections can be combinedwhen the upper jaw has to be moved independently ofthe orbito-nasal complex and it is expected that cor-rection by onlay bone grafts would be unsatisfactory.When there is hypertelorism as well as maxillary hypo-plasia, Le Fort III and Le Fort I sections can becombined with transcranial paramedian naso-ethmoidalresections and orbital shifts.

(c) Transcranial orbital translocations are now standardprocedures for the correction of hypertelorism andorbital dystopia. They may be combined with frontal

' I 1)FIG. 2 Chinese girl with Crouzon syndrome, (a) before and (b) after transcranial Le Fort III osteotomy and facial advancement combinedwith fronto-orbital advancement.

Management of severe cranio-facial deformity 273

FIG. 3 Boy with craniofrontonasal dysplasia (Cohen II syndrome)translocations and nasal reconstruction.

reshaping. They have been performed for the hyper-telorism of Cohen syndrome and severe frontal plagio-cephaly (Fig. 3a and b).

From the study of the 'unnatural action' produced by thedeformity has emerged an hypothesis on which is based arational approach to surgery.

Relationships between fronto-ethmoidalmeingoencephaloceles and cranio-facial cleftsMany attempts have been made to classify clefts of thecranio-facial region. Tessier has made an anatomical classifi-cation which describes clefts arranged around the orbit,numbered 0 to 14. 'This attempt at classification of cleftsdoes not prevent a more sophisticated or detailed explanat-ion, but rather it provides an immediate reference to theexact location and character ofwhat is being described' (10).

Mazzolla (11) has produced a morphological classificationof malformations to the fronto-nasal area based on embryo-logical studies. Mazzolla uses the term 'fronto-nasal dys-raphia' to include median clefts of nose, possibly equivalentto Tessier's clefts 0, 14 and 1, 13. Fronto-nasal dysraphia alsoincludes cysts and fistulae of the fronto-nasal region. Heincludes fronto-ethmoidal meningoencephaloceles in themedian cleft nose group. Difficulties with classification ofdefects in this area provide a challenge to the use of theHunterian techniques ofobservation, induction of principles,and the use of these principles in the further management ofproblems.

b)and resultant hypertelorism (a) before and (b) after transcranial orbital

Case materialSeventeen cases of fronto-ethmoidal meningoencephalocelewere compared with 13 cases of midline facial clefts of themedian and paramedian cleft nose variety. Cephaloceleappears to be the appropriate generic name for a congenitalherniation of brain through a skull defect; the alternativename is craniumbifidum, in the attractive but unprovenassumption that these conditions are the cephalic equivalentof spina bifida. The contents of the herniae are usuallymeningoencephaloceles which can be classified in four maingroups:

(i) Occipital.(ii) Parietal.

(iii) Basal.(iv) Sincipital.The cincipital group have been further classified bySuwanwela (12) basing his classification on that of VonMeyer (13) into fronto-ethmoidal meningoencephaloceles:

(i) Naso-frontal.(ii) Naso-ethmoidal.(iii) Naso-orbital.

Interfrontal encephaloceles.Cranio-facial clefts.

The description 'fronto-ethmoidal' is most appropriatebecause it describes the site of the cranial end of the defectwhich is always through the position of the foramen caecumat the junction of the frontal (membranous) and ethmoidal(cartilaginous) bones. The crista galli is at the posteriormargin of the defect.

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274 David J. David

MorphologyCRANIAL END OF THE BONY DEFECT

The exit hole from the anterior fossa was always at thejunction of the frontal bone and the ethmoidal bone at thesite of the foramen caecum (Fig. 4). Behind this, that is atthe posterior margin of the cranial defect, was the cribriformplate with the cristi galli at its anterior end. The cribriformplate was tilted downwards, deepening the central portion ofthe anterior cranial fossa. The anterior end of the cribriformplate lies much lower than the posterior end; the cribriformplate thus forms an angle of 45-50 degrees with the horiz-ontal. The defect varied in size and shape; all naso-frontaldefects were round and central; all naso-orbital defects werebilobed (2 patients from this group were previously operatedand one side of the exit holes had been obliterated by metalmesh); 2 of the naso-ethmoidal type were bilobed, in 1 casethe defect was lozenge shaped and central and the remainderwere round.

a

FIG. 4 Three-dimensional computer reconstruction ofa CAT scan ofa child withi fronto-ethmoidal meningoencephalocele. The rota-tional view shows the anterior and middle fossae from above, withthe exit hole of the meningoencephalocele at the junction of thefrontal bone and ethmoidal bone.

THE FACIAL END OF THE BONY DEFECT

Naso-frontal The defect lies at the junction of the frontal andnasal bones with the nasal bones attached to its inferiormargin. The defect varies in shape (Fig. 5a and b).Naso-ethmoidal The facial defects lie between the nasal bonesand the nasal cartilages. The nasal bones are deformed, oftenbroadened with crimped margins. The fronto-nasal angle isobliterated, producing the appearance of an overhangingledge. (Fig. 6a and b).

If the facial defect is confined to the nasal pyramid it issmall and oval, and the medial walls of the orbit are notinvolved; if, however, the meningoencephalocele is largerand the facial defect extends more laterally, then the anteriormargins of the medial orbital walls are eroded and becomecrescent shaped.Naso-orbital type presents on the face through defects in themedial orbital walls situated in the frontal process of themaxilla and the lacrimal bones (Fig. 7a, b and c). The bonytract is long and shaped like an inverted 'Y'. The inverted 'Y'may be symmetrical. The meningoencephalocele comes

through a frontal process of the maxilla onto the face leavingthe nasal bone intact anteriorly and the lacrimal bone andlateral plate of the ethmoid intact posteriorly. However,

bFIG. 5 (a) Aboriginal infant -with naso-frontal variety of fronto-ethmoidal meningoencephalocele. The facial defect lies at thejunction offrontal and nasal bones, with the nasal bones attached tothe inferior margin. (b) Three-dimensional reconstruction of CATscan.

during its passage through the substance of the ethmoid thelateral plate is pushed laterally forming a bony tunnel by theextruded cranial contents. The orbital soft tissue mass mayextend over the medial orbital rim onto the face producing agroove or flattening of the inferior orbital margin medially.

GENERAL FEATURES OF THE BONY FACIAL SKELETON

In all cases the face appears to be longer than normal. Thepyriform aperture with nasal cartilages is compressed fromabove and displaced inferiorly. There is telecanthus. This isusually not severe compared with clefts, and is of the Tessiersecond degree variety, with normal lateral canthal dis-tance. Six patients had malocclusions which may be relatedto the deformity in that the vertical plate of the ethmoidbone being attached to a tilted cribriform plate is itself retro-displaced and this may result in some maxillary hypoplasia.The naso-ethmoidal type of encephalocele has a direct effecton the nasal septal cartilage pushing it downwards andbackwards. It is as though the encephalocele has blown outonto the face through the weakened junction of the frontaland ethmoidal bones and displaced the otherwise normal

Management of severe cranio-facial deformity 275

6a

6bFIG. 6 (a) Malaysian boy with naso-ethmoidal variety of fronto-ethmoidal meningoencephalocele. The facial defect lies between thenasal bones and nasal cartilages, with erosion of the anteriormargins of the medial orbital walls. (b) Three-dimensional recon-struction of CAT scan.

FIG. 7 (a) Malaysian woman with naso-orbital variety of fronto-ethmoidal meningoencephalocele. The encephalocele presents onthe face through defects in the anterior medial orbital walls. Thenasal bone is intact anteriorly. (b,c) Three-dimensional recon-struction of CAT scan. Endotracheal tube in place for anaesthetic.

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276 David J. David

orbits and nasal capsule widening the orbits and lengtheningthe face.

In contrast to clefts which have a deficiency of tissue attheir margins, the defects of the fronto-ethmoidal meningo-encephaloceles are like tunnels or 'blow-holes', and they arelined by normal tissues.SOFT TISSUE

Pathological constituents of the encephalocele vary. Nopatients had extension of the ventricular system into thedefect. Some patients had a small amount of normal-lookingbrain in the neck of the encephalocele but usually the tissuelooked atrophic. Nine patients had previous surgery, 7 hadintercranial operations only and 2 had intracranial andfacial operations.

Those cases where the neck of the encephalocele has beendivided at previous surgery did not show significant spon-taneous atrophy of the facial extension suggested by Naim-Ur-Rahman (14). This tissue appears to remain in enoughbulk to produce the significant distortion of the face.

Soft tissue mass of the meningoencephalocele may extendinto the orbits and fuse with the peri-orbitum, but may flowover the infra-orbital rim which becomes indented anddepressed and onto the face to be associated with abnormalskin which is discoloured and may be scarred from previousulceration and healing. The skin is often thickened andcrusty.OCULAR PROBLEMS

with a degree of orbital dystopia. Fig. 8 shows the increasedintercanthal distance, increased interpupillary distance andrelatively normal lateral canthal distance in these patients.

NEUROLOGICAL PROBLEMS

Three patients had a degree of mental retardation, 5 hadhydrocephalus and 2 had epilepsy.

The interfrontal type of sincipital meningoencephalocelereferred to by Suwanwela has not been seen by us. However,such a case is presented and described in Tessier's article onfacial clefts (10). It appears from the description to be indeeda midline facial cleft where the interfrontal meningo-encephalocele is secondary to the clefting which continuesdown the midline of the nose into the maxilla and hasproduced a short, wide face with severe hypertelorism.

Median clefts of the nose (Tessier 0,14 and 1,13)Thirteen such cases have been studied including 2 cases ofsyncephalic twin. In all of these cases the facial height isdecreased, the interpupillary distance, intercanthal distanceand lateral canthal distance is much increased comparedwith the fronto-ethmoidal meningoencephalocele. There is adeficiency of both bone and soft tissue adjacent to the cleftsand the degree of hypertelorism is greater than with thefronto-ethmoidal meningoencephaloceles.

ThesisFour patients had decreased visual acuity, 3 patients had Fronto-ethmoidal meningoencephaloceles are fundamen-squints, 8 presented with lacrimal drainage disfunction and 3 tally different in origin from the midline clefts. The deformity

a bFIG. 8 Naso-frontal variety of fronto-ethmoidal meningoencephalocelle (a) before and (b) after transcranial translocation of both orbits, andnasal reconstruction.

Management of severe cranio-facial deformity

is related to the space-occupying extruded brain and is notintrinsic to the bones. Early complete surgery should allowthe developing brain and eyes to mould the skeleton. Theforces generated by the nasal airway, speech and masticationwill remodel the facial deformity. The midline clefts of thenose have a primary deficiency of tissue. The abnormality isintrinsic to the tissues themselves and early surgery will notbe expected to reduce the final deformity.

TreatmentAll operations were performed by the cranio-facial approachthrough a bicoronal scalp flap. Sub-periosteal dissection ofthe orbits was performed. Where indicated the skin over themidline soft tissue deformity was excised. Lower facialosteotomies were performed before the bifrontal craniotomy.The neck of the meningoencephalocele was isolated by acombined intradural and extradural approach. Brain wasinspected and as much as possible was conserved. The neckof the encephalocele was transected and the dural defectrepaired. The remaining orbital cuts were then made. Themedial orbital walls are often defective in the naso-ethmoidaltype and the angle of the cribriform plate is so steep that thetranslocated orbits come to overlay the cribriform plate.Canthopexies and nasal bone grafts are performed asnecessary.

Eleven patients had both orbits moved (Fig. 8a and b).Two patients had one orbit moved and 4 patients had medialorbital wall osteotomies plus canthopexies, this being theoperation of choice in the first year of life (Fig. 9a and b).

DiscussionThe logical classification of sincipital encephaloceles hasbeen suggested, establishing a relationship between clefts ofthe nose and hypertelorism and short faces and fronto-ethmoidal meningoencephaloceles with its three sub-groups.It has been previously suggested that sincipital meningo-encephaloceles are one of the neural tube defects to beconsidered with anencephaly and myelomeningocele as avarying expression of a single developmental aberration.

However, the sincipital meningoencephaloceles of the fronto-ethmoidal type pose some difficulties to this interpretation.They do not have the circumstantial supporting evidence ofsibling affectation present in the other neural tube defects.They also show quite a different geographical distributionaffecting people in Malaysia, Thailand, Burma, Pakistanand Southern Russia but being rare in Europe and NorthAmerica.Thus in their epidemiology the sincipital frontal-

ethmoidal meningoencephaloceles show remarkable peculi-arities and in the present state of knowledge it seems unwiseto lump them in with other neural tube defects. It mayindeed express-some unknown environmental agents, per-haps dietetic.There are obvious functional differences between the

meningoencephaloceles and the clefts; mere division of theneck of the encephalocele is not enough to wither the distalextent of the extruded tissue and prevqnt distortion of thedeveloping facial skeleton. Cranio-facial surgery is recom-mended with removal of the extruded brain and the appro-priate osteotomies and bone graft in the first 3 months of lifeif possible.

Cranio-facial fracturesHunter stated that 'the principles of our art are not less necessary tobe understood than the principles of other sciences . . .'. Theprinciples established from studies of some congenital de-formities have been effectively applied in the treatment ofsevere cranio-facial trauma. I wish to trace the evolution ofthe techniques of treatment in the South Australian Cranio-Facial Unit and outline the current management.

MaterialForty cases with severe cranio-facial fractures are analysedspanning the period of change from one approach to theother. Seventeen patients had CSF rhinorrhoea and weretreated conservatively, that is, by reduction and treatment offacial component of the fracture only.

In each of these cases the CSF rhinorrhoea ceased postope-

a bFIG. 9 Naso-frontal variety of fronto-ethmoidal meningoencephalocele (a) before and (b) after transcranial reduction of encephalocele andmedial orbital wall osteotomies, canthopexies and nasal reconstruction.

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278 DavidJ. David

ratively. The second group of 23 patients required both aneurosurgical procedure and a plastic surgical procedure.

CLASSIFICATION

In addition to the Le Fort classification which deals with themiddle third of the face, we have come to consider thepattern of the cranial component of the fractures as:

Trans-frontalTrans-orbitalTrans-basal: trans-ethmoidal

trans-sphenoidal

TRADITIONAL TREATMENT

In the early 1970s the treatment of such fractures in ourInstitution represented a compromise between two extremeviews. At one end of the spectrum was a neurosurgical view(15) that patients with persistent CSF rhinorrhoea due to afracture of the cranial base retained a high risk of meningitiseven if the leak ceased spontaneously. At the other end of thespectrum was the plastic surgical view that if CSF rhinor-rhoea stopped with reduction of the facial fracture the risk ofmeningitis was negligible.The situation existed where neurosurgeons whose

experience was largely derived from cranial fractures whichextended into the base of the skull rarely took the pattern ofthe facial fracture into account. The plastic surgeons rarelyconsidered the problems of brain, dura and scalp.

PHASE OF DEVELOPING CO-OPERATION

Initial co-ordination of disciplines was brought about by ajoint assessment of the patient and radiographs in the first

week after injury. Conventional tomography was initiallyused to determine the pattern of the naso-ethmoidal com-ponent of the fracture. Our neurosurgical colleagues wereprincipally interested in whether the fracture could be seencrossing the floor of the anterior cranial fossa. It soon becameclear that the tomograms shed more light on all componentsof the fracture. With an atmosphere of co-operation and theinformation provided by the tomography an individual planfor treatment of a particular patient would be made. Duringthese years of inter-disciplinary treatment, the correction ofthe facial skeleton was usually performed first, unless urgentneurosurgery was necessary. The neurosurgical correctionwas then left for a number of weeks and done as a secondindividual procedure.MODERN TECHNIQUES

With increased influence of the Cranio-Facial Unit, the earlycombined neurosurgical and plastic surgical approach wasdeveloped. Of 23 cases requiring both neurosurgical andplastic surgical intervention, 10 have been treated by a singlecranio-facial operation between the first and sixteenth daypost-accident. In cases treated before 1979 separate plasticand neurosurgical operations were performed from 1 to 14weeks apart.The indications for combined early treatment became

clear.(i) Cranio-facial fractures with intra-cranial air or intra-

cranial blood requiring urgent neurosurgical inter-vention.

(ii) X-ray evidence of significant disruption of the frontalbone, orbital roofs or base of skull.

'I h)FIG. 10 (a) Man with extensive cranio-facial fractures on presentation after accident. (b) After recovery from combined neurosurgical andcranio-facial primary repair.

Management of severe cranio-facial deformity 279

The conventional tomography of the 1970s has beensupplanted by CAT scanning with varying degrees ofsophisticated reconstruction. At the present time we are ableto produce three-dimensional reconstruction of considerabledetail.

TECHNIQUES

A bicoronal scalp flap is turned down to expose the superiororbital margin. The frontal zygomatic and naso-ethmoidregions are dissected. Neurosurgical repair of the durathrough a frontal craniotomy takes precedence followed byreduction and fixation of all fractures. Fine wiring of thefronto-orbital components is necessary and is often combinedwith external fixation. The latter has to be placed above thecraniotomy.

Because such surgery exposes the dura and CSF spaces tothe nasal airway, particular care is taken about sepsis.Preoperative and postoperative antibiotic cover is used.There have been no serious postoperative complications in

any of these patients since the implementation of the com-bined surgical approach. Uncorrected cranio-facial fracturesproduce hideous deformities. The early combined cranio-facial approach has been proved an effective way to restorethese people to normality (Fig. lOa and b). The outstandingresult from this approach is that the period of hospitalisationis reduced by 50%0.

ConclusionThe study of abnormal growth pattern in craniosynostosissyndromes during the post-natal period has led to a workinghypothesis of the pathogenesis of the deformity. This has ledin turn to a rational approach to the timing of surgery.The concept of a fundamental difference in the genesis of

the deformity associated with fronto-ethmoidal meningo-encephaloceles and cranio-facial clefts determine the timingand type of surgery.From such studies a system of management has been

effectively applied to the early treatment of patients withcranio-facial fractures.From the study of the 'wrong construction of parts'

acquired cranio-facial deformity can be managed moreeffectively.

It is to John Hunter that we are indebted for applying theinductive system of observation and experiment to the studyof disease, producing general principles which we are thenable to apply to solve further problems, allowing us to reachout and extend our surgical care into the future.

ReferencesI Hunter J. In: Palmer J F, ed. Lectures on the principles of

surgery, the collected works of J. Hunter. London, Longman,1835.

2 Tessier P, Guiot G, Rougerie J, Delbet JP, Pastorra J.Osteotomies cranio-naso-orbitales hypertelorism. Ann ChirPlast 1967; 12:103.

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