Facial balance in cleft lip and palate. II. Cleft lip and palate and secondary deformities A. F. Markus.
J. Delaire.
W. P. Smith
Depnrtmenr oj’Ot14 and Musillqfuciul Surgety, Poole Generul Hwpital, Poole, Dorset, UK: Depurlment Stotnatdog~* and MuxillqJkiuI Surgcr): Cetltre Hospitulirr C~nivcrsitaire, Nunres. France
of
StiMM.4 R Y. The cleft abnormality is the cause of underdevelopment and subsequent loss of function. Primary cleft surgery and surgery to correct the secondary deformities of previous non-functional repair should aim to restore normal anatomy and physiology, with an emphasis on muscle reconstruction of the lip and soft palate if normal facial development is to be encouraged.
primary palate and these arc more pronounced when there is a complete cleft of the lip and palate. The results of repair are usually not so good in complete clefts of the lip and palate. especially if primary surgery has not restored the morphofunctional equilibrium between the soft tissues and the skeleton.
INTRODUCTION
Cleft lip and palate results from a failure of growth and fusion of the facial processes, secondary underdevelopment and, because of disturbance to the functional equilibrium from the divided muscles of the soft palate and upper lip, deformities of the underlying skclctal elements. It is not the result of hypoplasia of the soft tissues and underlying skeleton. except in uncommon conditions such as holoprosencephaly. Good results with primary surgery cannot bc obtained by merely repositioning the superficial tissues. The aim must be to restore the anatomy and therefore the function of the underlying muscles. In this way, one can expect more normal dcvclopment of the facial skeleton. These principles are not only important in primary surgery but are also invaluable in the correction of secondary deformities due to clefts. both during development and when growth has ceased. Careful muscle reconstruction. even at a later date, is essential for normal appearance and function of the lip.
FACIAL
Anomalies of the soft tissues in complete unilateral cleft of the lip and alveolus The naso-labial muscles (Fig. 1A): On the cleft side. the n~usclcs of the superior and middle rings of the anterior facial muscle chain cannot cross the cleft and insert normallv into the anterior border of the nasal septum on either side of the anterior nasal spine, so uniting with the muscles from the opposite side. This includes the transverse muscles of the nose (constrictor nares), the levator muscles of the upper
BALANCE
DEDUCTIONS ALVEOLUS
AND THERAPEUTIC IN COMPLETE CLEFT
LIP AND
In complete clefts of the lip and alveolus the superior and middle rings of the superficial facial muscle chains are interrupted on one or both sides in unilateral or bilateral clefts respectively. The alveolar cleft extends back to the palatal papilla but the palatal bone (secondary palate) remains intact. In complete clefts of the lip and palate. there is complete division of the primary and secondary palate. The initial skeletal anomalies are very different in the two types of cleft, not only in the primary but also the secondary palate. In cleft lip and alveolus. there is an undcrdevelopment and deformity of the skeletal elements on both sides of the cleft of the
Fig. IA - Complete unilateral out cleft palate). the anterior 296
cleft lip and alveolus facial muscle chains.
(with or with-
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lip and the nose and the oblique and horizontal heads of orbicularis oris. Similarly, the incisive muscle. instead of being inserted in the incisive fossa (slightly lateral to the median inter-incisive suture) is found on the anterior margin of the lateral segment, closely associated with the root of the canine tooth. All these muscles are retracted laterally and collapsed on to the underlying bone, hindering development. On the non-cleft side. the muscles have normal insertions into the nasal septum. the anterior nasal spine. the median cellular septum and fraenum of the upper lip, the premaxilla and the skin of the lip and columella. However. the pull which they exert is not opposed. as would normally be expected. resulting in ipsi-lateral displacement and deformity. T/w muco-cutaneous surjtices (Fig. 1B): On either side of the cleft lip. the skin is displaced and retracted by the underlying muscles. The nasal skin. which is finely punctate and less thick than the striate labial skin. drifts on to the upper lip. This is plainly visible. as is the external bulging of the labial skin which is retracted and thickened. The excess mucosa of the external and internal margins of the lip is abnormal, as it lacks mucosal glands (originally described by Veau (193X) as sterile mucosa). However. in our opinion. there is no hypoplasia of the skin or mucosa.
side by the muscular fibres which arc inserted into it and the periosteum of the adjacent nasal spine.
The alar cartilage of the nostril on the cleft side is not supported by the constrictor nares which normally passes behind the lateral crus and belovv the medial crus before inserting into the anterior nasal septum and the inferior border of the cartilaginous nasal septum. On the contrary. the lateral crus is pulled out and down by this muscle and the medial crus is pulled down by the muscles on the non-cleft side. This produces an overall flattening of the cartilage in relation to the opposite side. with distention and distortion of the two crura. Thcrc is no cartilaginous hypoplasia. The anterior margin of the septal cartilage is pulled towards the healthy
The skeletal anomalies on the two sides of the cleft are due to the abnormal insertion and function of the naso-labial muscles. Correct morpho-functional reconstruction of thcsc muscles and reconstitution of the anterior facial chains must be the main aim of primary surgery (Fig. 3). If this is achieved. the skeletal anomalies almost completely regress. Only persistent anomalies of the dento-alveolar region on either side of the cleft require orthodontic treatment
The Cartilages:
Fig. 1R - The skin and mucosa. 1 & I’: skin on the nasal sill drawn onto the face. 2 & 2’: rctractlon of the labial skin, 3 & 3’: the white roll and cleft margms. 4 & 4’: ~XCCSSmucosa.
Skeletal anomalies in complete unilateral cleft lip and alveolus (Fig. 2)
On the cleft side the anomalies arc characterised by with inversion of the canine undcrdevelopment. region and a groove on the adjacent external margin of the pyriform fossa (related to the inferior part of the transverse muscle of the collapsed nose). The deciduous canine tooth bud is more posterior than normal and tends, therefore. to erupt palatally. On the non-cleft side the anomalies are more complex. There is antero-medial rotation of the anterior part of the premaxilla towards the healthy side, a deviation of the anterior nasal spine and the median inter-incisive suture towards the healthy side and marked underdevclopment of the premaxilla on the cleft side. especially in the subalveolar part. Underdevelopment of the alveolus results directly in hypoplasia or even agenesis of the lateral incisor on this side. Therapeutic deductions in complete unilateral cleft lip and alveolus
Fig. 2 - Skeletal (without
anomahcs cleft palate).
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tuberosity) is displaced outwards, resulting in medial rotation of the anterior part. This usually happens a few days after birth (Fig. 4B) under the influence of contraction of the naso-labial muscles on the cleft side when the child is crying and feeding through a bottle. The entire greater segment is outwardly displaced as a result of which there is a deviation of the inferior part of the vomer. sometimes to nearly 90”, such that it may appear to bc forming part of the palatal vault. It is important to clearly distinguish the overlying mucosa from the adjacent palatal f%romucosa. There is also deviation of the scptal cartilage.
Fig. 3 - The efl’ect of rcstormg clcff tip and alveolus (wrhout
facial balance cleft paldtc).
In complcrc
unilateral
and possible secondary bone grafting at the age of 9-l I years. before eruption of the permanent canines (Bergland er al., 1986).
FACIAL BALANCE AND THERAPEUTIC DEDUCTIONS IN COMPLETE UNILATERAL CLEFT LIP AND PALATE Anomalies of the soft tissues in complete unilateral cleft lip and palate (Fig. 4) These arc similar to and arise for the same reasons as in clefts without cleft palate. The muco-cutaneous anomalies do not differ signilicantly from those seen in the absence of a palatal cleft. However. certain aspects arc more accentuated in relation to displaccment of the underlying dcnto-skeletal elements. These displacements are due to the break in continuity of the muscles of the soft palate and dysfunction of the associated muscles of the tongue. exaggerated by the complete division of the primary and secondary palate and. therefore, lack of balance and dysfunction of the naso-labial muscles. In very large clefts, the nasal cartilages will also be collapsed and flattened. When the cleft is straight but there is medial rotation of the anterior end of the lesser fragment. the alar cartilage will be more posterior and distorted. The nasal septum is also deviated to the non-cleft side. Skeletal anomalies in complete unilateral cleft lip and palate These represent the sum of all the anomalies seen in complete cleft lip and palate. In addition to the anomalies of the anterior region of the maxillary segment, identical to those in cleft lip and alveolus, the posterior part of the lesser segment (the maxillary
Facial
C Fig. 4 - Complete unllrlteral cleft tip. alveolus birth: (B) a few weeks laler. (C) after closure palate.
and palatc. (A) 31 of the lip and soft
the anterior nasal spine and the prcmaxilla to the non-cleft side. As previously discussed (Markus ef al.. 1992) the mandibular size and shape is altered by the position of the tongue, such that the symphyscal region is rclativcly more posterior. This results in posterior positioning of the lower lip which then lies behind the palatal aspect of the prcmaxilla, making the anterior rotation of the premaxilla worse. Therapeutic deductions in complete unilateral cleft lip and palate The severe skclctal anomalies are due to the cleft of the soft and hard palate. Attempts to minimise these anomalies by orthopacdic means. before closure of the lip. are often made. although not absolutely necessary. Early orthopaedic treatment. if used, should commence within the first 48 h. A plate will maintain the maxillary fragments in their correct relationships. preventing collapse of the lesser segment due to antero-medial rotation and rotation of the greater segment due to muscle activity (with simultaneous increase in distance between the maxillary tuberosities). It also acts as a palatal obturator, improving tongue posture and preventing it from lying between the margins of the cleft. This may encourage better development of the mandible and so facilitate feeding. Unfortunately, early orthopaedic treatment does have some disadvantages. It requires very active involvement and frequent intervention of the cleft team, as well as the family, and may also have an undesirable effect on the operative technique of some
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surgeons. All too often. too much confidence is placed in early orthopaedic treatment allowing the common operative techniques to be used unmodified. such that in the long term. imperfect results are perpetuated. The results are very much dcpcndent on the quality of the anatomical and functional correctness of the primary surgery. Early orthopaedic treatment may make surgery casicr but cannot compensate for inadequacies of surgical treatment. Primary closure of the soft palate bcforc closure of the lip encourages a reduction of the distance between the maxillary tuberosities, reducing the size of the cleft of the hard palate, improving the posture of the tongue and mandibular development. Malck and Psaume (1983) recommend carrying this out at the end of the 3rd month and closing the lip and hard palate at 6 months, utilising only the mucosa of the nasal floor. Before and after vcloplasty, they utilise early orthopacdic treatment. Since 1980. our preference is to close the soft palate and lip at the age of 6 months because at this time it is possible to carry out bcttcr muscle reconstruction. Simultaneous reconstruction of the deep facial and ccrv:cal muscle chains and the superficial facial muscle chains reduces the displacement of the bony fragments improving tongue position and function. which in turn improves the position of the maxillary segments and development of the mandible. Six months to I year later, the residual palatal cleft has usually become very narrow, allowing closure under optimal conditions and confining.surgery to the palatal libromucosa. It is also posstble at the second stage of primary surgery to perform a gingivo-periosteoplasly which allows the dcdicuous canine tooth to erupt into the correct position. If. at the time of the second operation. there are imperfections of muscle rcconstruction, revision can bc carried out simultaneously. This protocol makes early orthopacdic treatment unnecessary but requires a very meticulous technique of muscle reconstruction. In the long term, the best possible results can be achieved.
FACIAL BALANCE AND THERAPEUTIC DEDUCTIONS IN COMPLETE BILATERAL CLEFT LIP, ALVEOLUS AND PALATE The anomalies of the lateral segments, both soft tissue and skeletal, are similar to the unilateral form. The skeletal anomalies are. however. a little more accentuated with the inter-canine distance much reduced. Anomalies of soft tissue in complete bilteral cleft lip and alveolus On the external sides of the cleft these are similar to those seen in unilateral clefts (Fig. 1B). The nasolabial muscles on each side are collapsed and almost enclosed behind the prcmaxillary segment which itself is projected anteriorly (Fig. 5). In the premaxilla on the other hand. the anomalies are very different (Fig. 6). There is considerable retraction of the skin and the subcutaneous tissue of the columella and the
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Fig. 6 - .4nomalics of rhc muco-cutaneous surfaces in complctc bilateral lip (with or wIthout cleft palatc), (I) drlfrmg of nasal skin on IO fact, (2) rclractlon of skin on premaxllla and lateral segment: (3) d&Appearance of white roll on the latcrdl segments; (3) excess mucosa.
A
,n
n,
B Fig. 5 - Complctc bilateral the anterior facial muscle the anterior facial muscle of labelling refer IO Fig. 9
cleft hp and alveolus. (A) anomalies of chams (lateral VIW): (B) anomalies of chains (frontal view). (for explanation Mtrkus at [I/.. 1992. p 290).
prolabium is not distended as usual by the muscles, which remain entirely on the lateral parts of the cleft. The prolabium is very narrow and short, bulging underneath the columella. They arc often almost in contact. The premaxilla is projected forwards and upwards by the underlying dento-alveolar elements, which are similarly displaced. Skeletal anomalies in complete bilateral cleft lip and alveolus
The skeletal anomalies arc significant (Fig. 7). The premaxilla, instead of being well developed trans-
vcrscly on both sides of the median inter-incisive suture is projcctcd on an elongated ‘stalk’ (from the premaxillary-vomerinc suture to the tip of the anterior nasal spine). This is further accentuated by the incisor tooth germs. which are displaced forward. The intcrincisive suture is completely closed and barely visible. There are two reasons for failure of transverse development of the premasilla. The first is the abscncc of a union between each half of the premaxilla and the maxilla. The second is the lack of stimulus on the median inter-incisive suture by the median cellular septum and median fraenum of the upper lip. as there is no muscle insertion. The dento-skeletal anomalies arc fundamentally due to the anatomical and functional imbalance of the naso-labial muscles and the ‘septal system’. Primary surgery must aim to restore normal anatomy and, therefore. function of the muscles and the scptal system. It is necessary to re-insert the transvcrsc (constrictor) muscles of the nose, the oblique heads of the orhicularis oris muscle of the upper lip to the anterior border of the nasal septal cartilage (in front of the anterior nasal spine) and also properly reconoris to struct the horizontal heads of the orbicularis each other and to the median cellular septum and fraenum of the upper lip (Fig. 7B). In less severe cases where the upper lateral incisors are prcscnt, a near normal shape of the anterior part of the upper dental arch and sometimes. a return to near normal development of the prcmaxilla, with complete union to the maxilla, is possible. Secondary muscle reconstruction with simultaneous gingivopcriosteoplasty (with or without bone grafting) will encourage normal development. The length and anterior projection of the prcmaxillary segment should not be reduced in the course of carrying out a primary bilateral functional cheilorhino-plasty (always carried out in one operation). Wide sub-periostcal undermining (Delairc et al., 1988)
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Anomalies of the soft tissues and skeleton, and therapeutic deductions in bilateral complete cleft lip and palate (Fig. 8)
As in complete unilateral clefts. the distance between the maxillary tuberosities is increased and together with antero-medial rotation. the lateral segments come to lie behind the premaxilla. The distance between these segments and the premaxilla and also the alveolar crest of the anterior mandible may be considerable and make the surgery somewhat more difficult. As already discussed. the prcmaxilla must not bc set back because function of the naso-labial muscles anteriorly will ultimately product the desired result. It is also necessary to take account of the lengthening of the mandible after reconstruction of the soft palate. The best results will bc obtained by simple modifications to the timing and tcchniquc of surgery. At 4 5 months. closure of the soft palate and reconstitution of the naso-labial muscles on both sides is carried out.
A
A
Fig. 7 - Complstc bllatcral hp and alveolus (without cleft palate). (A) Skeletnl onomahcs; (B) After reconstruction of the naso-labial muscles
on the anterior aspect of the maxilla always allows the tissues to be brought across the premaxillary segment. Additionally. the tip of the anterior nasal spine is often too far forwards prior to primary surgery. If the naso-labial muscles are well reconstituted. its projection is rapidly reduced. Preservation of the length of the prcmaxilla is necessary to allow for the postopcrativc development of the lateral maxillary segments and advancement of the mandible.
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C Fig. 8 - Skclctal anomnhcs In complctc bilateral cleft hp. alveolus and palate. (A) at blrrh: (B) a few weeks Iatcr; (C) after prlmar! closure of the lip and soft palate.
In a very wide cleft lip. it is possible to convert it from complete to incomplete at the first operation and proceed to total repair at 7 or 8 months, with or without gingivoperiosteoplasty. At 14.-18 months. the residual palatal and alveolar cleft is closed with ginpivoperiostcoplasty. Any revision of the lip or soft palate can bc carried out at this stage. Orthodontic treatment in the deciduous dentition can. if necessary, be used from about the age of 4-5 years in casts of premaxillary protrusion, to restore, as soon as possible. the correct position and function of the upper deciduous canines.
plasia of the maxilla. and a progressive Class 111 deformity). These same sequelac. are seen if the cleft of the hard and soft palate is associated with a cleft of the lip and alveolus but tend to bc more severe because of the secondary sequcla of non-functional lip reconstruction. In complete unilateral cleft of the lip and alveolus (with or without cleft palate) problems arise if there is inadequate reconstruction of the naso-labial muscles and in particular, the muscles of the lloor of the nose, the nasal sill and the upper lip. Failure to restore the naso-labial muscles on the cleft side on to the nasal septum, the periosteum in the region of the anterior nasal spine and to the muscles of the opposite side, produces functional anomalies which have several adverse effects on the overall facial balance (Fig. 9). The naso-labial muscles on the lateral segments arc collapsed. exaggerating the underdcvelopment of the segment as well as posterior positioning and flattening of the alar cartilage, and nasal obstruction. The lack of muscle attachment on to the nasal septum allows the muscles on the unaffected side to deviate more and more to that side. The anterior border of the septum deviates into the healthy nostril which it partly obstructs. compounding the obstruction on the cleft side already present due to collapse of the alar cartilage. The asymmetrical muscular anatomy. a feature of commonly used methods of repair (Nicolau. 1983) is responsible for asymmetry of upper lip movements making it impossible for the patient to project the lip normally which is important for good dcvclopment of the premaxilla. This impaired function of the upper b
a
A
FACIAL BALANCE AND THERAPEUTIC DEDUCTIONS IN SECONDARY CLEFT LIP AND PALATE DEFORMITIES DUE TO NONFUNCTIONAL REPAIR When clefts of the lip and palate are well repaired. facial balance will be almost normal and in the long term. this will be manifest as normal facial development. At the end of adolescence aesthetic operations will not be required, with the exception of simple procedures on the nasal cartilages to improve symmetry, and revision of unsightly residual scars. Unfortunately. at present, in many patients this is not the cast and at the end of their development. they may have gross facial imbalance. We described earlier the skeletal anomalies of the face that result from a short and tight soft palate (Class III abnormalities with mandibular prognathism) and incorrect closure of the hard palate (hypo-
Fig. 9 - The cflects of incorrect muscle repair on the nasal septum, the anterior nasal spme and the muscles of the non-cleft side.
Facial lip favours retraction by the scar and exaggerates the activity of the lower lip which tends to become hypertrophied and projected more anteriorly. The difliculty in nasal respiration due to nasal obstruction. as well as naso-pharyngeal infections. favours habitual mouth breathing, with lowering of the tongue at rest and during swallowing. The lower position of the tongue favours posterior repositioning and a reduction in development of the prcmaxilla. anterior maxillary rctrusion and retroclination of the incisors. predisposing to a Class III malocclusion, which in turn. exaggcratcs the maxillary undcrdcvelopment and abnormal lip function. A vicious circle is established which can lead to considerable facial deformity in spite of the initial anomalies being minimal. In the absence of adequate preventive treatment. major surgical revision can bc carried out at the end of growth. This entails maxillary and/or mandibular osteotomics and sometimes multiple surgical procedures on the soft tissues of the lip and nose. Incorrect repair of complete bilateral cleft lip and alveolus results in even more major anomalies. In the prcmaxilla. thcsc deformities can vary dcpcnding upon the technique of lip reconstruction. If the horiyontal heads of the orbicularis are too tight across the premaxilla or if the median labial fraenum has been mutilated. the excessive retraction caused by the lip will rotate the premaxilla posteriorly resulting in its underdcvclopment and a Class III incisor relationship. If. on the other hand. all the naso-labial muscles are collapsed laterally, there is a distention of the soft tissues of the prolabium and columella with lowering, flattening and spreading out of the nasal dome. The prcmaxilla becomes globular and protrusive and projected in front of the lateral segments. A Class II dcnto-skeletal malformation develops and later correction can be difficult. Therapeutic deductions During the period of active growth. the deformity due to poor primary repair progressively worsens. Rehabilitation of the anatomical and functional balance of the naso-labial muscles and correction of the abnormal skeletal clcments is an indication for a secondary functional cheilo-rhinoplasty at the earliest possible opportunity. This necessitates a very wide sub-periostcal undermining over the antcro-lateral surface of the maxilla and careful fixation of the naso-labial muscles into the anterior border of the septal cartilage. A gingivo-pcriostoplasty should be performed, with or without osteoplasty, to close the alveolar cleft and a bone graft to the nasal spine if anchorage of the muscles to the septum would otherwise be inadequate. Post-operative functional physiotherapy is essential. Improvement of the dento-skeletal relationships can only be brought about after restoration of the naso-labial muscles. provided the skeletal relationships and the occlusion are not too abnormal. It is often necessary to carry out dento-facial orthopaedic treatment prior to functional cheilo-rhinoplasty. The
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dcnto-skeletal anomalies are those associated with anterior maxillary hypoplasia and a Class III deformity. Treatment may need to be carried out in two stages, firstly to expand the maxilla and secondly, to It is only apply forward traction to it, or rice wru. in the less sevcrc anomalies that these two types of malformations can be corrected simultaneously by using. for example, a quadhelix. Extra-oral traction can bc applied in an antero-posterior direction using an orthopaedic mask (Delaire & Vcrdon, 1983), a most cf?cctive way of moving the maxilla forwards. Good results are more easily achieved if the patient is young. preferably less than 9 or 10 years, if there is no fibrous connection between the vomer and the palate and if the anomalies of the naso-labial and soft palatal muscles have been previously (or will be very soon) corrected. Secondary surgical and orthopacdic treatment is not easy and dots not always give the desired results. Neverthclcss, they will always reduce the severity of the ultimate abnormalities when growth is completed. having recognised the need for secondary treatment, one should not delay in carrying it out. However, it is still possible to obtain some improvement if surgery is performed at the end of development. When active development has finished, equilibrium of the facial skeleton should be assessed. WC use the crania-facial architectural analysis (Dclaire, 1978) which in our opinion is the best method of identifying and quantifying the various deformities. Achieving the best results often requires combined osteotomics of the maxilla and mandible. including functional genioplasty to bring about a reduction in the height of the chin. The latter is not for aesthetic reasons but is often necessary to improve function of the labiomental muscles and allow bi-labial contact (Precious & Delaire, 1985). Normal equilibrium of the supcrficial facial muscles requires correct re-insertion of the naso-labial muscles. It is carried out at the same time as the orthognathic surgery which is facilitated by complete revision of the reconstructed lip (Schendcl & Delaire. 1981).
RARE CACSES BALANCE
OF ANOMALIES
OF FACIAL
There are certain rare causes of an abnormal facial balance seen in association with cleft lip and palate. A major reduction in length of the crania-facial area is one of the architectural factors which predispose to a Class III abnormality. Patients with a cleft lip and palate may have a reduction in the length of the cranial base and. therefore, a Class III relationship. although there may be an underlying tendency to prognathism or retrognathism. Cranio-facial asymmetry is often noted in infants with cleft lip and palate. In some cases it co-exists with anomalies of the cervical vertebrae (Horswell, 1991). such as hemivertebrae, which are responsible for problems with cephalic posture, which in turn gives rise to the asymmetry. Other situations exist in which asymmetry of the cranial base is associated with ipsi-lateral hemi-
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facial underdevclopment. This presents certain problems. especially if the maxilla on the opposite side is relatively advanced. It is particularly difficult to correctly rc-insert the naso-labial muscles. Retrusion of the lesser segment constantly favours a cross-bite on the cleft side and this is often exaggerated by surgery. The skclctal anomalies secondary to muscle dysfunction due to incorrect anchorace and the malocclusion are always compounded dur:ng facial development. At the end of growth, these crania-facial asymmetries are always difficult to correct. cvcn by combined osteotomies and bone grafts. It is necessary. therefore. to understand this type of asymmetry and to counter its adverse effects in all possible ways. that is, functional cheilo-rhinoplasty carried out very early, dentofacial orthopacdics and eventual postural therapy. cithcr functional or even surgical. possibly involving section of the Sterno-clcido-mastoid muscle.
DISCUSSION Clefts of the lip and palate arc not due to a fundamental loss of soft tissue or underlying skeletal elements, but to an underdevclopment, deformity and displacement under the influence of functional imbalance of the divided muscles of the soft palate and lip. In the long term. facial equilibrium is not achieved if the muscular anatomy is not restored. The surgeon must aim to restore the normal anatomy by functional reconstruction leading to correct overall facial balance. Therefore, it is necessary to know the fundamental conditions of this equilibrium in normal subjects, the anomalies in cleft lip and/or palate before and after operation and the best ways of correcting the imbalance. A knowledge of facial development and the eventual sequelac of certain traditional surgical techniques is essential. We have cmphasised the importance of respecting the palatal fibro-mucosa of the maxilla. two-stage repair of a cleft of the hard and soft palate and the importance of correct anchorage of the naso-labial muscles on to the anterior border of the nasal septum in both unilateral and bilateral clefts of the lip and alveolus. If in early childhood, the results obtained by primary surgery are not as good as expected. one should not delay revising the muscles of the soft palate and,’ or naso-labial muscles by carrying out a UVPP. and secondary functional cheilo-rhinoplasty respectively. However. in cases of maxillary underdcvelopment and a tendency to a Class III deformity. one should utilise early dento-facial orthopaedics if surgery is to be etl’ective. At the end of growth, surgical-orthopaedic correction of the dento-skeletal anomalies will result in a facial equilibrium which will be as near as possible to that of a patient without a cleft. The crania-facial analysis allows objectivity and quantification of the various anomalies in the sagittal and vertical plants. Certain anomalies of facial equilibrium in cleft lip and palate are not only due to the cleft but to other
important malformations of the cranial base. The long term consequences of these malformations ncccssitate additional procedures for the treatment of the facial clefts with which they are associated. Acknowledgements ‘l‘hc dulhros thank Mrs V. Jackson for prcp;~r~ngthe manuscript and Mr S. Rutherford. of the Mcd~cul Photography Department at Poole General Hospital. for the illustrations.
References Bergland. 0.. Scmb. G. & Abyholm, F. (1986). Elimlnalion of Ihc residual alveolar cleft by secondary bone grafting and subsequent orthodontic Ircatmenr. Cleft Palure Jw~w/. 23. 175. Delaire. J. (197X) L’analysc archltccturale Ed sIrucIurale cramolkcialc (de prolil). Prmciples Iheoriqucs. Quelques cxcmplcs d‘emplol cn chirurgie maxilla-faciale. HPWC tic Slomarogologie et de C‘hirurgir .Maxillr~- Fu~wle. 79. I. Delaire. J. & Vcrdon. P. (1983). L’cmploie dcs forces extraorales postero-anlcrlcurcs lourdes sur masque orrhopcdlquc dam le traitemcnt dcs scquelles dcnro-maxillairrs Jcs fcntes labiomaxlllo-palatines. C.‘hwurgie Pcdiointricprr. 24, 315. D&Ire. J.. Precious. D. & Gordccff. A. (1988).The advantage ol wide sub-pcriosteal exposure in primary surgical correction of labial maxillarv clclla. .Scon&uviun Jnurnnl ofP/r~~ic and Ret o,l.,rruc,i,‘c,-.Surgcr?. 22. 147. Horswell. B. B. (1991). The incldcncc and relationstun of ccrvlcal spine ,lnomalies in patlcnts with cleli lip and:or palate Jounlal of Oral a,ld Mu.xillc~facial Surger.~. 49.693. Malek, R. & Psaumc. J. (1983) Bases anatomlques et physiologiques du Iraitcmcnt primairr des fcntcs lahiopalatines umlatcralcs totales. At/o Odonrologu .Scondmawa, 152. 265. Markus. A. F.. D&ire, J. & Smith W P. (1992). Facial balance in clef1 lip and palate. I. Normal dc\clopment and cleft palate. Hriri,vh Journal oJ Owl UIIJ Maxillnfacrol Surgw~. 30,290. Nicolau. P. (1983) The orbicularls oris muscle: a functlonal approach IO its rcpalr In the clef1 lip. Brrrrsh Jt~urnul o/ Plorrrc Surgrrv. 36, I41 Precious. D. & Delaire. J. (19X5). Correction of ‘mtcrior mandlhular excess. Ihc functional gemoplastp. Ortrl Srrger~. Oral .2ledlrme. Oral Potho&!‘. 59. 229. Schcndel, S. & Dcl,urc. J. (1981). Funcliondl musculo-skeletal corrcslion of secondary unilateral clef1 lip dcformilies: combined lip-nose correction and Lc Fort I osteotomy. Journal CJJMo.xi//o- Facml Surpr~. 9. 108. Vcau. V (1938). Bee-dc-Lievre. Paris: .Llasson CI C. Ed.
The Authors A. F. Markus
E’DSKCS
Consultant W. P. Smith FDSRCS, FRCS Registrar Department of Oral and Maxlllofacial Poole General Hospital Poole Dorset BH I5 2J B
J. D&ire
Surgery
MD
Department of Stomatology and Maxillofaclal Centrc Hospitalicr Universirairc Nantcs 1:rance Correspondence
and requests for &prints
Pdpcr received I5 November Accepted 24 February 1992
1991
Surgery
IO Mr A. F. Markus
J. Delaire.
W. P. Smith
Depnrtmenr oj’Ot14 and Musillqfuciul Surgety, Poole Generul Hwpital, Poole, Dorset, UK: Depurlment Stotnatdog~* and MuxillqJkiuI Surgcr): Cetltre Hospitulirr C~nivcrsitaire, Nunres. France
of
StiMM.4 R Y. The cleft abnormality is the cause of underdevelopment and subsequent loss of function. Primary cleft surgery and surgery to correct the secondary deformities of previous non-functional repair should aim to restore normal anatomy and physiology, with an emphasis on muscle reconstruction of the lip and soft palate if normal facial development is to be encouraged.
primary palate and these arc more pronounced when there is a complete cleft of the lip and palate. The results of repair are usually not so good in complete clefts of the lip and palate. especially if primary surgery has not restored the morphofunctional equilibrium between the soft tissues and the skeleton.
INTRODUCTION
Cleft lip and palate results from a failure of growth and fusion of the facial processes, secondary underdevelopment and, because of disturbance to the functional equilibrium from the divided muscles of the soft palate and upper lip, deformities of the underlying skclctal elements. It is not the result of hypoplasia of the soft tissues and underlying skeleton. except in uncommon conditions such as holoprosencephaly. Good results with primary surgery cannot bc obtained by merely repositioning the superficial tissues. The aim must be to restore the anatomy and therefore the function of the underlying muscles. In this way, one can expect more normal dcvclopment of the facial skeleton. These principles are not only important in primary surgery but are also invaluable in the correction of secondary deformities due to clefts. both during development and when growth has ceased. Careful muscle reconstruction. even at a later date, is essential for normal appearance and function of the lip.
FACIAL
Anomalies of the soft tissues in complete unilateral cleft of the lip and alveolus The naso-labial muscles (Fig. 1A): On the cleft side. the n~usclcs of the superior and middle rings of the anterior facial muscle chain cannot cross the cleft and insert normallv into the anterior border of the nasal septum on either side of the anterior nasal spine, so uniting with the muscles from the opposite side. This includes the transverse muscles of the nose (constrictor nares), the levator muscles of the upper
BALANCE
DEDUCTIONS ALVEOLUS
AND THERAPEUTIC IN COMPLETE CLEFT
LIP AND
In complete clefts of the lip and alveolus the superior and middle rings of the superficial facial muscle chains are interrupted on one or both sides in unilateral or bilateral clefts respectively. The alveolar cleft extends back to the palatal papilla but the palatal bone (secondary palate) remains intact. In complete clefts of the lip and palate. there is complete division of the primary and secondary palate. The initial skeletal anomalies are very different in the two types of cleft, not only in the primary but also the secondary palate. In cleft lip and alveolus. there is an undcrdevelopment and deformity of the skeletal elements on both sides of the cleft of the
Fig. IA - Complete unilateral out cleft palate). the anterior 296
cleft lip and alveolus facial muscle chains.
(with or with-
Facial balance m cleft
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lip and the nose and the oblique and horizontal heads of orbicularis oris. Similarly, the incisive muscle. instead of being inserted in the incisive fossa (slightly lateral to the median inter-incisive suture) is found on the anterior margin of the lateral segment, closely associated with the root of the canine tooth. All these muscles are retracted laterally and collapsed on to the underlying bone, hindering development. On the non-cleft side. the muscles have normal insertions into the nasal septum. the anterior nasal spine. the median cellular septum and fraenum of the upper lip, the premaxilla and the skin of the lip and columella. However. the pull which they exert is not opposed. as would normally be expected. resulting in ipsi-lateral displacement and deformity. T/w muco-cutaneous surjtices (Fig. 1B): On either side of the cleft lip. the skin is displaced and retracted by the underlying muscles. The nasal skin. which is finely punctate and less thick than the striate labial skin. drifts on to the upper lip. This is plainly visible. as is the external bulging of the labial skin which is retracted and thickened. The excess mucosa of the external and internal margins of the lip is abnormal, as it lacks mucosal glands (originally described by Veau (193X) as sterile mucosa). However. in our opinion. there is no hypoplasia of the skin or mucosa.
side by the muscular fibres which arc inserted into it and the periosteum of the adjacent nasal spine.
The alar cartilage of the nostril on the cleft side is not supported by the constrictor nares which normally passes behind the lateral crus and belovv the medial crus before inserting into the anterior nasal septum and the inferior border of the cartilaginous nasal septum. On the contrary. the lateral crus is pulled out and down by this muscle and the medial crus is pulled down by the muscles on the non-cleft side. This produces an overall flattening of the cartilage in relation to the opposite side. with distention and distortion of the two crura. Thcrc is no cartilaginous hypoplasia. The anterior margin of the septal cartilage is pulled towards the healthy
The skeletal anomalies on the two sides of the cleft are due to the abnormal insertion and function of the naso-labial muscles. Correct morpho-functional reconstruction of thcsc muscles and reconstitution of the anterior facial chains must be the main aim of primary surgery (Fig. 3). If this is achieved. the skeletal anomalies almost completely regress. Only persistent anomalies of the dento-alveolar region on either side of the cleft require orthodontic treatment
The Cartilages:
Fig. 1R - The skin and mucosa. 1 & I’: skin on the nasal sill drawn onto the face. 2 & 2’: rctractlon of the labial skin, 3 & 3’: the white roll and cleft margms. 4 & 4’: ~XCCSSmucosa.
Skeletal anomalies in complete unilateral cleft lip and alveolus (Fig. 2)
On the cleft side the anomalies arc characterised by with inversion of the canine undcrdevelopment. region and a groove on the adjacent external margin of the pyriform fossa (related to the inferior part of the transverse muscle of the collapsed nose). The deciduous canine tooth bud is more posterior than normal and tends, therefore. to erupt palatally. On the non-cleft side the anomalies are more complex. There is antero-medial rotation of the anterior part of the premaxilla towards the healthy side, a deviation of the anterior nasal spine and the median inter-incisive suture towards the healthy side and marked underdevclopment of the premaxilla on the cleft side. especially in the subalveolar part. Underdevelopment of the alveolus results directly in hypoplasia or even agenesis of the lateral incisor on this side. Therapeutic deductions in complete unilateral cleft lip and alveolus
Fig. 2 - Skeletal (without
anomahcs cleft palate).
In
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tuberosity) is displaced outwards, resulting in medial rotation of the anterior part. This usually happens a few days after birth (Fig. 4B) under the influence of contraction of the naso-labial muscles on the cleft side when the child is crying and feeding through a bottle. The entire greater segment is outwardly displaced as a result of which there is a deviation of the inferior part of the vomer. sometimes to nearly 90”, such that it may appear to bc forming part of the palatal vault. It is important to clearly distinguish the overlying mucosa from the adjacent palatal f%romucosa. There is also deviation of the scptal cartilage.
Fig. 3 - The efl’ect of rcstormg clcff tip and alveolus (wrhout
facial balance cleft paldtc).
In complcrc
unilateral
and possible secondary bone grafting at the age of 9-l I years. before eruption of the permanent canines (Bergland er al., 1986).
FACIAL BALANCE AND THERAPEUTIC DEDUCTIONS IN COMPLETE UNILATERAL CLEFT LIP AND PALATE Anomalies of the soft tissues in complete unilateral cleft lip and palate (Fig. 4) These arc similar to and arise for the same reasons as in clefts without cleft palate. The muco-cutaneous anomalies do not differ signilicantly from those seen in the absence of a palatal cleft. However. certain aspects arc more accentuated in relation to displaccment of the underlying dcnto-skeletal elements. These displacements are due to the break in continuity of the muscles of the soft palate and dysfunction of the associated muscles of the tongue. exaggerated by the complete division of the primary and secondary palate and. therefore, lack of balance and dysfunction of the naso-labial muscles. In very large clefts, the nasal cartilages will also be collapsed and flattened. When the cleft is straight but there is medial rotation of the anterior end of the lesser fragment. the alar cartilage will be more posterior and distorted. The nasal septum is also deviated to the non-cleft side. Skeletal anomalies in complete unilateral cleft lip and palate These represent the sum of all the anomalies seen in complete cleft lip and palate. In addition to the anomalies of the anterior region of the maxillary segment, identical to those in cleft lip and alveolus, the posterior part of the lesser segment (the maxillary
Facial
C Fig. 4 - Complete unllrlteral cleft tip. alveolus birth: (B) a few weeks laler. (C) after closure palate.
and palatc. (A) 31 of the lip and soft
the anterior nasal spine and the prcmaxilla to the non-cleft side. As previously discussed (Markus ef al.. 1992) the mandibular size and shape is altered by the position of the tongue, such that the symphyscal region is rclativcly more posterior. This results in posterior positioning of the lower lip which then lies behind the palatal aspect of the prcmaxilla, making the anterior rotation of the premaxilla worse. Therapeutic deductions in complete unilateral cleft lip and palate The severe skclctal anomalies are due to the cleft of the soft and hard palate. Attempts to minimise these anomalies by orthopacdic means. before closure of the lip. are often made. although not absolutely necessary. Early orthopaedic treatment. if used, should commence within the first 48 h. A plate will maintain the maxillary fragments in their correct relationships. preventing collapse of the lesser segment due to antero-medial rotation and rotation of the greater segment due to muscle activity (with simultaneous increase in distance between the maxillary tuberosities). It also acts as a palatal obturator, improving tongue posture and preventing it from lying between the margins of the cleft. This may encourage better development of the mandible and so facilitate feeding. Unfortunately, early orthopaedic treatment does have some disadvantages. It requires very active involvement and frequent intervention of the cleft team, as well as the family, and may also have an undesirable effect on the operative technique of some
bal,mcc
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surgeons. All too often. too much confidence is placed in early orthopaedic treatment allowing the common operative techniques to be used unmodified. such that in the long term. imperfect results are perpetuated. The results are very much dcpcndent on the quality of the anatomical and functional correctness of the primary surgery. Early orthopaedic treatment may make surgery casicr but cannot compensate for inadequacies of surgical treatment. Primary closure of the soft palate bcforc closure of the lip encourages a reduction of the distance between the maxillary tuberosities, reducing the size of the cleft of the hard palate, improving the posture of the tongue and mandibular development. Malck and Psaume (1983) recommend carrying this out at the end of the 3rd month and closing the lip and hard palate at 6 months, utilising only the mucosa of the nasal floor. Before and after vcloplasty, they utilise early orthopacdic treatment. Since 1980. our preference is to close the soft palate and lip at the age of 6 months because at this time it is possible to carry out bcttcr muscle reconstruction. Simultaneous reconstruction of the deep facial and ccrv:cal muscle chains and the superficial facial muscle chains reduces the displacement of the bony fragments improving tongue position and function. which in turn improves the position of the maxillary segments and development of the mandible. Six months to I year later, the residual palatal cleft has usually become very narrow, allowing closure under optimal conditions and confining.surgery to the palatal libromucosa. It is also posstble at the second stage of primary surgery to perform a gingivo-periosteoplasly which allows the dcdicuous canine tooth to erupt into the correct position. If. at the time of the second operation. there are imperfections of muscle rcconstruction, revision can bc carried out simultaneously. This protocol makes early orthopacdic treatment unnecessary but requires a very meticulous technique of muscle reconstruction. In the long term, the best possible results can be achieved.
FACIAL BALANCE AND THERAPEUTIC DEDUCTIONS IN COMPLETE BILATERAL CLEFT LIP, ALVEOLUS AND PALATE The anomalies of the lateral segments, both soft tissue and skeletal, are similar to the unilateral form. The skeletal anomalies are. however. a little more accentuated with the inter-canine distance much reduced. Anomalies of soft tissue in complete bilteral cleft lip and alveolus On the external sides of the cleft these are similar to those seen in unilateral clefts (Fig. 1B). The nasolabial muscles on each side are collapsed and almost enclosed behind the prcmaxillary segment which itself is projected anteriorly (Fig. 5). In the premaxilla on the other hand. the anomalies are very different (Fig. 6). There is considerable retraction of the skin and the subcutaneous tissue of the columella and the
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h
Fig. 6 - .4nomalics of rhc muco-cutaneous surfaces in complctc bilateral lip (with or wIthout cleft palatc), (I) drlfrmg of nasal skin on IO fact, (2) rclractlon of skin on premaxllla and lateral segment: (3) d&Appearance of white roll on the latcrdl segments; (3) excess mucosa.
A
,n
n,
B Fig. 5 - Complctc bilateral the anterior facial muscle the anterior facial muscle of labelling refer IO Fig. 9
cleft hp and alveolus. (A) anomalies of chams (lateral VIW): (B) anomalies of chains (frontal view). (for explanation Mtrkus at [I/.. 1992. p 290).
prolabium is not distended as usual by the muscles, which remain entirely on the lateral parts of the cleft. The prolabium is very narrow and short, bulging underneath the columella. They arc often almost in contact. The premaxilla is projected forwards and upwards by the underlying dento-alveolar elements, which are similarly displaced. Skeletal anomalies in complete bilateral cleft lip and alveolus
The skeletal anomalies arc significant (Fig. 7). The premaxilla, instead of being well developed trans-
vcrscly on both sides of the median inter-incisive suture is projcctcd on an elongated ‘stalk’ (from the premaxillary-vomerinc suture to the tip of the anterior nasal spine). This is further accentuated by the incisor tooth germs. which are displaced forward. The intcrincisive suture is completely closed and barely visible. There are two reasons for failure of transverse development of the premasilla. The first is the abscncc of a union between each half of the premaxilla and the maxilla. The second is the lack of stimulus on the median inter-incisive suture by the median cellular septum and median fraenum of the upper lip. as there is no muscle insertion. The dento-skeletal anomalies arc fundamentally due to the anatomical and functional imbalance of the naso-labial muscles and the ‘septal system’. Primary surgery must aim to restore normal anatomy and, therefore. function of the muscles and the scptal system. It is necessary to re-insert the transvcrsc (constrictor) muscles of the nose, the oblique heads of the orhicularis oris muscle of the upper lip to the anterior border of the nasal septal cartilage (in front of the anterior nasal spine) and also properly reconoris to struct the horizontal heads of the orbicularis each other and to the median cellular septum and fraenum of the upper lip (Fig. 7B). In less severe cases where the upper lateral incisors are prcscnt, a near normal shape of the anterior part of the upper dental arch and sometimes. a return to near normal development of the prcmaxilla, with complete union to the maxilla, is possible. Secondary muscle reconstruction with simultaneous gingivopcriosteoplasty (with or without bone grafting) will encourage normal development. The length and anterior projection of the prcmaxillary segment should not be reduced in the course of carrying out a primary bilateral functional cheilorhino-plasty (always carried out in one operation). Wide sub-periostcal undermining (Delairc et al., 1988)
Facial balance III cleft Ilp and palate (II)
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Anomalies of the soft tissues and skeleton, and therapeutic deductions in bilateral complete cleft lip and palate (Fig. 8)
As in complete unilateral clefts. the distance between the maxillary tuberosities is increased and together with antero-medial rotation. the lateral segments come to lie behind the premaxilla. The distance between these segments and the premaxilla and also the alveolar crest of the anterior mandible may be considerable and make the surgery somewhat more difficult. As already discussed. the prcmaxilla must not bc set back because function of the naso-labial muscles anteriorly will ultimately product the desired result. It is also necessary to take account of the lengthening of the mandible after reconstruction of the soft palate. The best results will bc obtained by simple modifications to the timing and tcchniquc of surgery. At 4 5 months. closure of the soft palate and reconstitution of the naso-labial muscles on both sides is carried out.
A
A
Fig. 7 - Complstc bllatcral hp and alveolus (without cleft palate). (A) Skeletnl onomahcs; (B) After reconstruction of the naso-labial muscles
on the anterior aspect of the maxilla always allows the tissues to be brought across the premaxillary segment. Additionally. the tip of the anterior nasal spine is often too far forwards prior to primary surgery. If the naso-labial muscles are well reconstituted. its projection is rapidly reduced. Preservation of the length of the prcmaxilla is necessary to allow for the postopcrativc development of the lateral maxillary segments and advancement of the mandible.
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C Fig. 8 - Skclctal anomnhcs In complctc bilateral cleft hp. alveolus and palate. (A) at blrrh: (B) a few weeks Iatcr; (C) after prlmar! closure of the lip and soft palate.
In a very wide cleft lip. it is possible to convert it from complete to incomplete at the first operation and proceed to total repair at 7 or 8 months, with or without gingivoperiosteoplasty. At 14.-18 months. the residual palatal and alveolar cleft is closed with ginpivoperiostcoplasty. Any revision of the lip or soft palate can bc carried out at this stage. Orthodontic treatment in the deciduous dentition can. if necessary, be used from about the age of 4-5 years in casts of premaxillary protrusion, to restore, as soon as possible. the correct position and function of the upper deciduous canines.
plasia of the maxilla. and a progressive Class 111 deformity). These same sequelac. are seen if the cleft of the hard and soft palate is associated with a cleft of the lip and alveolus but tend to bc more severe because of the secondary sequcla of non-functional lip reconstruction. In complete unilateral cleft of the lip and alveolus (with or without cleft palate) problems arise if there is inadequate reconstruction of the naso-labial muscles and in particular, the muscles of the lloor of the nose, the nasal sill and the upper lip. Failure to restore the naso-labial muscles on the cleft side on to the nasal septum, the periosteum in the region of the anterior nasal spine and to the muscles of the opposite side, produces functional anomalies which have several adverse effects on the overall facial balance (Fig. 9). The naso-labial muscles on the lateral segments arc collapsed. exaggerating the underdcvelopment of the segment as well as posterior positioning and flattening of the alar cartilage, and nasal obstruction. The lack of muscle attachment on to the nasal septum allows the muscles on the unaffected side to deviate more and more to that side. The anterior border of the septum deviates into the healthy nostril which it partly obstructs. compounding the obstruction on the cleft side already present due to collapse of the alar cartilage. The asymmetrical muscular anatomy. a feature of commonly used methods of repair (Nicolau. 1983) is responsible for asymmetry of upper lip movements making it impossible for the patient to project the lip normally which is important for good dcvclopment of the premaxilla. This impaired function of the upper b
a
A
FACIAL BALANCE AND THERAPEUTIC DEDUCTIONS IN SECONDARY CLEFT LIP AND PALATE DEFORMITIES DUE TO NONFUNCTIONAL REPAIR When clefts of the lip and palate are well repaired. facial balance will be almost normal and in the long term. this will be manifest as normal facial development. At the end of adolescence aesthetic operations will not be required, with the exception of simple procedures on the nasal cartilages to improve symmetry, and revision of unsightly residual scars. Unfortunately. at present, in many patients this is not the cast and at the end of their development. they may have gross facial imbalance. We described earlier the skeletal anomalies of the face that result from a short and tight soft palate (Class III abnormalities with mandibular prognathism) and incorrect closure of the hard palate (hypo-
Fig. 9 - The cflects of incorrect muscle repair on the nasal septum, the anterior nasal spme and the muscles of the non-cleft side.
Facial lip favours retraction by the scar and exaggerates the activity of the lower lip which tends to become hypertrophied and projected more anteriorly. The difliculty in nasal respiration due to nasal obstruction. as well as naso-pharyngeal infections. favours habitual mouth breathing, with lowering of the tongue at rest and during swallowing. The lower position of the tongue favours posterior repositioning and a reduction in development of the prcmaxilla. anterior maxillary rctrusion and retroclination of the incisors. predisposing to a Class III malocclusion, which in turn. exaggcratcs the maxillary undcrdcvelopment and abnormal lip function. A vicious circle is established which can lead to considerable facial deformity in spite of the initial anomalies being minimal. In the absence of adequate preventive treatment. major surgical revision can bc carried out at the end of growth. This entails maxillary and/or mandibular osteotomics and sometimes multiple surgical procedures on the soft tissues of the lip and nose. Incorrect repair of complete bilateral cleft lip and alveolus results in even more major anomalies. In the prcmaxilla. thcsc deformities can vary dcpcnding upon the technique of lip reconstruction. If the horiyontal heads of the orbicularis are too tight across the premaxilla or if the median labial fraenum has been mutilated. the excessive retraction caused by the lip will rotate the premaxilla posteriorly resulting in its underdcvclopment and a Class III incisor relationship. If. on the other hand. all the naso-labial muscles are collapsed laterally, there is a distention of the soft tissues of the prolabium and columella with lowering, flattening and spreading out of the nasal dome. The prcmaxilla becomes globular and protrusive and projected in front of the lateral segments. A Class II dcnto-skeletal malformation develops and later correction can be difficult. Therapeutic deductions During the period of active growth. the deformity due to poor primary repair progressively worsens. Rehabilitation of the anatomical and functional balance of the naso-labial muscles and correction of the abnormal skeletal clcments is an indication for a secondary functional cheilo-rhinoplasty at the earliest possible opportunity. This necessitates a very wide sub-periostcal undermining over the antcro-lateral surface of the maxilla and careful fixation of the naso-labial muscles into the anterior border of the septal cartilage. A gingivo-pcriostoplasty should be performed, with or without osteoplasty, to close the alveolar cleft and a bone graft to the nasal spine if anchorage of the muscles to the septum would otherwise be inadequate. Post-operative functional physiotherapy is essential. Improvement of the dento-skeletal relationships can only be brought about after restoration of the naso-labial muscles. provided the skeletal relationships and the occlusion are not too abnormal. It is often necessary to carry out dento-facial orthopaedic treatment prior to functional cheilo-rhinoplasty. The
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dcnto-skeletal anomalies are those associated with anterior maxillary hypoplasia and a Class III deformity. Treatment may need to be carried out in two stages, firstly to expand the maxilla and secondly, to It is only apply forward traction to it, or rice wru. in the less sevcrc anomalies that these two types of malformations can be corrected simultaneously by using. for example, a quadhelix. Extra-oral traction can bc applied in an antero-posterior direction using an orthopaedic mask (Delaire & Vcrdon, 1983), a most cf?cctive way of moving the maxilla forwards. Good results are more easily achieved if the patient is young. preferably less than 9 or 10 years, if there is no fibrous connection between the vomer and the palate and if the anomalies of the naso-labial and soft palatal muscles have been previously (or will be very soon) corrected. Secondary surgical and orthopacdic treatment is not easy and dots not always give the desired results. Neverthclcss, they will always reduce the severity of the ultimate abnormalities when growth is completed. having recognised the need for secondary treatment, one should not delay in carrying it out. However, it is still possible to obtain some improvement if surgery is performed at the end of development. When active development has finished, equilibrium of the facial skeleton should be assessed. WC use the crania-facial architectural analysis (Dclaire, 1978) which in our opinion is the best method of identifying and quantifying the various deformities. Achieving the best results often requires combined osteotomics of the maxilla and mandible. including functional genioplasty to bring about a reduction in the height of the chin. The latter is not for aesthetic reasons but is often necessary to improve function of the labiomental muscles and allow bi-labial contact (Precious & Delaire, 1985). Normal equilibrium of the supcrficial facial muscles requires correct re-insertion of the naso-labial muscles. It is carried out at the same time as the orthognathic surgery which is facilitated by complete revision of the reconstructed lip (Schendcl & Delaire. 1981).
RARE CACSES BALANCE
OF ANOMALIES
OF FACIAL
There are certain rare causes of an abnormal facial balance seen in association with cleft lip and palate. A major reduction in length of the crania-facial area is one of the architectural factors which predispose to a Class III abnormality. Patients with a cleft lip and palate may have a reduction in the length of the cranial base and. therefore, a Class III relationship. although there may be an underlying tendency to prognathism or retrognathism. Cranio-facial asymmetry is often noted in infants with cleft lip and palate. In some cases it co-exists with anomalies of the cervical vertebrae (Horswell, 1991). such as hemivertebrae, which are responsible for problems with cephalic posture, which in turn gives rise to the asymmetry. Other situations exist in which asymmetry of the cranial base is associated with ipsi-lateral hemi-
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facial underdevclopment. This presents certain problems. especially if the maxilla on the opposite side is relatively advanced. It is particularly difficult to correctly rc-insert the naso-labial muscles. Retrusion of the lesser segment constantly favours a cross-bite on the cleft side and this is often exaggerated by surgery. The skclctal anomalies secondary to muscle dysfunction due to incorrect anchorace and the malocclusion are always compounded dur:ng facial development. At the end of growth, these crania-facial asymmetries are always difficult to correct. cvcn by combined osteotomies and bone grafts. It is necessary. therefore. to understand this type of asymmetry and to counter its adverse effects in all possible ways. that is, functional cheilo-rhinoplasty carried out very early, dentofacial orthopacdics and eventual postural therapy. cithcr functional or even surgical. possibly involving section of the Sterno-clcido-mastoid muscle.
DISCUSSION Clefts of the lip and palate arc not due to a fundamental loss of soft tissue or underlying skeletal elements, but to an underdevclopment, deformity and displacement under the influence of functional imbalance of the divided muscles of the soft palate and lip. In the long term. facial equilibrium is not achieved if the muscular anatomy is not restored. The surgeon must aim to restore the normal anatomy by functional reconstruction leading to correct overall facial balance. Therefore, it is necessary to know the fundamental conditions of this equilibrium in normal subjects, the anomalies in cleft lip and/or palate before and after operation and the best ways of correcting the imbalance. A knowledge of facial development and the eventual sequelac of certain traditional surgical techniques is essential. We have cmphasised the importance of respecting the palatal fibro-mucosa of the maxilla. two-stage repair of a cleft of the hard and soft palate and the importance of correct anchorage of the naso-labial muscles on to the anterior border of the nasal septum in both unilateral and bilateral clefts of the lip and alveolus. If in early childhood, the results obtained by primary surgery are not as good as expected. one should not delay revising the muscles of the soft palate and,’ or naso-labial muscles by carrying out a UVPP. and secondary functional cheilo-rhinoplasty respectively. However. in cases of maxillary underdcvelopment and a tendency to a Class III deformity. one should utilise early dento-facial orthopaedics if surgery is to be etl’ective. At the end of growth, surgical-orthopaedic correction of the dento-skeletal anomalies will result in a facial equilibrium which will be as near as possible to that of a patient without a cleft. The crania-facial analysis allows objectivity and quantification of the various anomalies in the sagittal and vertical plants. Certain anomalies of facial equilibrium in cleft lip and palate are not only due to the cleft but to other
important malformations of the cranial base. The long term consequences of these malformations ncccssitate additional procedures for the treatment of the facial clefts with which they are associated. Acknowledgements ‘l‘hc dulhros thank Mrs V. Jackson for prcp;~r~ngthe manuscript and Mr S. Rutherford. of the Mcd~cul Photography Department at Poole General Hospital. for the illustrations.
References Bergland. 0.. Scmb. G. & Abyholm, F. (1986). Elimlnalion of Ihc residual alveolar cleft by secondary bone grafting and subsequent orthodontic Ircatmenr. Cleft Palure Jw~w/. 23. 175. Delaire. J. (197X) L’analysc archltccturale Ed sIrucIurale cramolkcialc (de prolil). Prmciples Iheoriqucs. Quelques cxcmplcs d‘emplol cn chirurgie maxilla-faciale. HPWC tic Slomarogologie et de C‘hirurgir .Maxillr~- Fu~wle. 79. I. Delaire. J. & Vcrdon. P. (1983). L’cmploie dcs forces extraorales postero-anlcrlcurcs lourdes sur masque orrhopcdlquc dam le traitemcnt dcs scquelles dcnro-maxillairrs Jcs fcntes labiomaxlllo-palatines. C.‘hwurgie Pcdiointricprr. 24, 315. D&Ire. J.. Precious. D. & Gordccff. A. (1988).The advantage ol wide sub-pcriosteal exposure in primary surgical correction of labial maxillarv clclla. .Scon&uviun Jnurnnl ofP/r~~ic and Ret o,l.,rruc,i,‘c,-.Surgcr?. 22. 147. Horswell. B. B. (1991). The incldcncc and relationstun of ccrvlcal spine ,lnomalies in patlcnts with cleli lip and:or palate Jounlal of Oral a,ld Mu.xillc~facial Surger.~. 49.693. Malek, R. & Psaumc. J. (1983) Bases anatomlques et physiologiques du Iraitcmcnt primairr des fcntcs lahiopalatines umlatcralcs totales. At/o Odonrologu .Scondmawa, 152. 265. Markus. A. F.. D&ire, J. & Smith W P. (1992). Facial balance in clef1 lip and palate. I. Normal dc\clopment and cleft palate. Hriri,vh Journal oJ Owl UIIJ Maxillnfacrol Surgw~. 30,290. Nicolau. P. (1983) The orbicularls oris muscle: a functlonal approach IO its rcpalr In the clef1 lip. Brrrrsh Jt~urnul o/ Plorrrc Surgrrv. 36, I41 Precious. D. & Delaire. J. (19X5). Correction of ‘mtcrior mandlhular excess. Ihc functional gemoplastp. Ortrl Srrger~. Oral .2ledlrme. Oral Potho&!‘. 59. 229. Schcndel, S. & Dcl,urc. J. (1981). Funcliondl musculo-skeletal corrcslion of secondary unilateral clef1 lip dcformilies: combined lip-nose correction and Lc Fort I osteotomy. Journal CJJMo.xi//o- Facml Surpr~. 9. 108. Vcau. V (1938). Bee-dc-Lievre. Paris: .Llasson CI C. Ed.
The Authors A. F. Markus
E’DSKCS
Consultant W. P. Smith FDSRCS, FRCS Registrar Department of Oral and Maxlllofacial Poole General Hospital Poole Dorset BH I5 2J B
J. D&ire
Surgery
MD
Department of Stomatology and Maxillofaclal Centrc Hospitalicr Universirairc Nantcs 1:rance Correspondence
and requests for &prints
Pdpcr received I5 November Accepted 24 February 1992
1991
Surgery
IO Mr A. F. Markus
