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The early management of bilateral cleft of lip and palate
THE EARLY MANAGEMENT OF BILATERAL CLEFT OF LIP AND PALATE By DENIS GLASS,L.D.S.R.C.S., D.D.O. Consultant Orthodontist, Plastic and Reconstructive Surgical Centre, East Grinstead, Sussex
TBE incidence of cleft lip and palate occurring in the Anglo-Saxon race has been estimated at 1-75o to i-I,OOO live births (Fogh-Andersen, 1942, 1961 ; Grace, 1943 ; Hixon, 1951 ; Sesgin and Stark, 1961 ). Figure I shows the distribution of the various types of cleft taken from i,ooo cases attending the East Grinstead cleft lip and palate centre and shows that about 18 per cent. of clefts are bilateral. T h e important feature of this type of cleft is that it divides the maxilla into three separate segments, two lateral maxillary segments and one premaxillary segment, providing many problems for those responsible for their solution. It is now an accepted fact that all clefts have a tissue deficiency and this deficiency is mesodermal ; the failure of the embryonic tissues to develop is due to lack of mesodermal penetration (Stark, 1954). Although the amount of tissue deficiency must affect the degree of deformity, the width of the cleft and position of the segments are also dependent on other factors. T h e most obvious example is undoubtedly the displaced premaxilla which in some cases occupies a position anterior to the tip of the nose and bears no relationship to its correct place between the maxillary segments. How does it reach that position ? In normal development, the embryonic maxillary processes fuse at about the fifth week of intrauterine life. Then the mesodermal elements penetrate the first branchial arch and meet and eventually become the upper and lower maxillary processes. T h e whole face, including the lips, is now invaded by mesial advancement of the second branchial arch which supplies all the muscles of the face, especially the circumoral muscles. At the same time the maxillary clip of Callender advances to the midline to overlap and envelop the true premaxilla (Fig. 2) (Wood et aL, 1967). In bilateral clefts of lip and palate, the position of the three maxillary components is dependent on many factors. The lateral maxillary segments assume a position of stability determined by the soft tissue forces of the tongue and cheeks, and mesial collapse of these segments is often seen in untreated cases of all ages ; this is due to buccinator pressure acting on a defective maxillary arch. The protruded position of the premaxilla is caused by pressure from the lower lip and tongue acting on a premaxilla which has no restraifiing maxillary continuity and no intact circumoral musculature to prevent forward growth of the vomer, In bilateral clefts, therefore, the defective and displaced premaxiUa, held forward under the tip of the nose by overgrowth of the vomer, is forwardly displaced far from its correct position as the keystone of the arch and despite the many techniques available, only limited improvement rather than perfection can be expected. Presurgical Dental Orthopmdic Treatment.--This early appliance therapy is carried out during the months before lip closure. Its object is to align the maxillary segments and to retract the premaxilla to a position where surgical closure is made easier. The appliance using extra-oral traction on the prolabium pulls back this segment into contact with the lateral segments which themselves may be aligned to assist lip closure (Hotz, 1964; Maisels, 1966). 13o
]BILATERAL
CLEFT
OF LIP
AND
131
PALATE
T h e benefits claimed for this t r e a t m e n t are as follows : i. I t facilitates lip closure. 2. T h e m o t h e r m a y be relieved to think some t r e a t m e n t is being done. 35 3
2
q;tqh ?t ,'o"
Y
I PREMAXILLA
MAXILLA
UNILATERALONLY UNILATERAL INCIDENCE OFCLEFT TYPES - I000 CLEFTS FIG. i
Fig. I.--Types of cleft occurring in a sample of i#oo cleft lip and palate cases attending the East Grinstead cleft lip and palate centre for treatment. Bilateral clefts form 18 per cent. of the sample. Fig. 2.--Diagram of the facial aspect of the maxilla ; note that the premaxilla never appears on the face but is enclosed by the maxilla.
#
t
t,. ,
~
FIG. 2
However, these benefits are not universally accepted (Pruzansky, I964) and in m y opinion-I. 2. 34. 5-
it does not stimulate maxillary growth ; it does not produce " clinical or b o n y u n i o n " o f the segments ; it delays the time o f lip closure until the child is 9 to I2 months old ; it draws attention to the child's deformity at h o m e ; it provides added burdens o f travel and absence f r o m h o m e to the neglect of the rest of the family ; 6. it involves the orthodontist and technicians in work of doubtful value.
F r o m the work o f H u d d a r t et al. (I966) on this p r o b l e m in which he studied treated and untreated cases and f r o m m y own experience there is no apparent difference in the two groups o f children in later years. However, this therapy does seem to have a beneficial psychological effect on the orthodontist and surgeon concerned.
P r e s u r g i c a l O r t h o p m d i c s a n d P r i m a r y B o n e G r a f t i n g . m P r e s u r g i c a l orthopaedic treatment combined with early or p r i m a r y b o n e grafting has become a popular
132
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method of treatment in some centres (Georgiade et al., 1964 ; Horton et al., 1964 ; Brauer and Cronin, 1965 ; Nylen, 1966 ; Schuchardt, 1966 ; Monroe et al., 1969). However, Johansen (1965) in his Northcroft Memorial Lecture (reviewing over IOO cases treated in this way during a period of IO years), concluded that the results were disappointing and stated that he has discontinued this method of treatment.
A
B C FIG. 3 Bilateral cleft of lip and palate (left) before lip closure, (right) following lip closure at 3 months. Note the favourable upper and lower lip contact producing a lip seal. Below: Models--A, before lip closure ; B, I½ years old before palatal closure ; C, 3 years old. Note the good arch shape produced by the reconstruction of the upper lip. No dental orthopmdic treatment has been carried out. At the East Grinstead centre no primary bone grafts are carried out, as the cleft lip and palate team feel that the benefits, if any, which might result from this treatment are out of alI proportion to the severity of the surgical procedure involved. T h e recent work by Joss (1962) and Skoog (1967) of boneless bone grafting, may produce better results. As orthodontist, I am responsible for the final alignment of the bony segments of the maxilla and to join these segments by bone before uniting the soft tissues of the face and before restoring the muscle function of cheeks and lips is, in my opinion, of doubtful value. Furthermore, collapse of the maxillary segments does occur in some cases despite the bone graft, especially in the mixed dentition age and the correction of this collapse in these cases necessitates long and tedious orthodontic treatment.
BILATERAL CLEFT OF LIP AND
PALATE
133
Lip C l o s u r e . - - T h e timing of lip closure varies considerably from centre to centre, but at East Grinstead is usually completed by 3 months of age. The premaxilla now comes under the influence of the united muscle of the upper lip and moves rapidly to a position of stability in contact with the lateral maxillary segments. In favourable cases, the
Fro. 4 Patient B . S . Top left: bilateral cleft of lip and palate before surgery. Note severe displacement of premaxilla. Top right: following vomerine resection, premaxillary setback and lip closure at 4 months. Below: same patient at 9 and 14 years old. There is no diminution in the nasal or facial growth.
premaxillary retraction will allow normal upper to lower lip contact and will establish a normal lip seal so vital to the stability of the premaxillary segment (Fig. 3). When considering the timing of lip closure it is a pity that the work by Straith et al. (I955) and McCash (I957) has not received more attention. Lip closure in the first week of neonatal life under local anaesthesia would seem to have many beneficial effects : I. The uniting of the muscles of the lip at the earliest possible time.
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BRITISH JOURNAL OF PLASTIC SURGERY
2. The restoration of the normal muscle function of the face with its beneficial effect on growth and development as well as feeding. 3. The automatic retraction of the premaxilla. 4. The avoidance of a general anmsthetic. 5. The beneficial psychological effect on the mother by the immediate removal of the gross disfigurement and the fact that the mother and child can return home to their family and neighbours with the worst disfigurement corrected.
FIG. 5 Models of patient B . S . Top left: premaxilla is displaced forward z5 ram. Top right: following resection and premaxillary setback, prior to palatal closure. Below: (left) maxillary arch at 7 years ; (right) maxillary arch at i i years.
Vomerine R e s e c t i o n . - - T h e repositioning of the premaxilla by vomerine resection has been, and still is, severely criticised, but in cases where the premaxilla is displaced as far forward as the tip of the nose and the tissue deficiency combined with the soft tissue pattern of the lips is considered unfavourable for normal lip closure, this is undoubtedly the treatment of choice (Fig. 4). Mr C. R. McLanghlin has been doing this operation at East Grinstead in selected cases for I4 years (McLaughlin, 1956), and the eldest patient is now I4 years old. The results are excellent, and from an orthodontic point of view, both muscle function and arch relationship as well as facial contour are a great improvement on many cases treated in the standard way.
BILATERAL CLEFT OF
LIP
AND PALATE
I35
Poor results by this method of treatment have been caused by poor assessment of the problem with inadequate planning and removal of too much vomerine bone or incomplete fixation of the premaxilla during bony union. Hence certain criticisms should be directed at the execution, not the method.
Fig. 6.--Patient M. L. Boy aged 3½ years, unfavourable lip posture is preventing the repaired upper lip from retracting the premaxiUa. The premaxiUa is therefore outside the normal circumoral musculature. Fig. 7.--Left: unoperated bilateral cleft showing the lower lip posture behind the premaxilla and inside the upper lip segments. Right: thumb sucking and tongue thrusting can displace the premaxilla.
FIG. 6
FIG. 7
The setback is timed when the lip is closed at 3 months, the vomerine bone removed is always less than appears necessary and the premaxilla is firmly refixed by a midline Kirschner wire through the front of the premaxilla into the vomer ; care is taken to keep the pin in the midline and thus avoid the developing teeth. The pin is retained for four weeks when union will have occurred (Fig. 5). In some cases following the standard treatment of lip closure with no premaxillary setback, the premaxiUa will not fall back to contact the lateral segments and a very poor result is obtained (Fig. 6). This is due to a combination of circumstances : (I) The
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FIG. 8 Patient M . L . Splint fitted to stabilise the premaxilla following vomerine resection. This is retained for at least one month.
FIG. 9 Patient M. L. Following removal of the Kirschner wire and the splint, it may be necessary to retract the premaxilla still further to establish incisor relationship. This simple orthodontic appliance is very effective.
BILATERAL CLEFT OF LIP AND PALATE
137
premaxilla was severely protruded. (2) The arch relationship shows a Class II malocclusion associated with a normal sized maxilla articulating with a smallmandible, thus preventing the upper and lower lip contact necessary for lip seal. Instead, the lower lip rests permanently inside the premaxilla preventing its retraction by the now united upper lip. (3) There may be a powerful tongue thrusting habit. (4) The child may suck his thumb (Fig. 7).
A
B C FIG. II Models of Patient M. L. A, At 3½ years of age before premaxillary setback. B, Following premaxillary setback. C, At 4 years of age, there is now normal upper to lower jaw relationship. In these cases a vomerine resection can give excellent results at 3 to 4 years of age when co-operation from the child permits. The procedure is the same as for the early setback, except that a small acrylic splint is prepared before surgery. The premaxilla is set back and pinned to the vomer. At this time the premaxillary segment and the two maxillary segments are freshened in the alveolar area to give bony union, and further stabilised by the splint which unites the three segments of the maxilla (Fig. 8). The pin is removed at four weeks but the splint which is removable is retained for three months. When the splint is removed the lower lip posture is carefully studied, and if there is no lip seal, and the lower lip impinges on the lingual side of the premaxilla, failure is inevitable unless active orthodontic treatment is carried out to retract the upper teeth to a position of stability inside the lower lip (Fig. 9). To achieve this it may be necessary to correct the collapse of the lateral segments at the same time as the premaxilla is retracted (Figs. IO and II).
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Surgical Closure o f the P a l a t e . - - T h e surgical closure of the hard and soft palate is usually done at East Grinstead at about I½ years, the main object being to separate the nasal from the oral cavity and to provide a muscular mechanism able to perform the intricate movements of speech and deglutition. The provision of intelligible speech is by far the most important part of the rehabilitation of these children, for without adequate speech their opportnnities today are very restricted.
FIG, I2 TWO bilateral clefts of lip and palate. Right: 3 months of age. Left: I5 years of age. Neither has had any surgery b u t both show considerable maxillary arch collapse.
Much has been written about the effect of surgery on maxillary growth and development and the surgeon has received more than his fair share of criticism (Graber, I949 ; Jolleys, t954 ; Yansy, I955 ; Bill et al., I956 ; Swanson et al., t956 ; Jesus, t959). " Surgical damage of growth centres " , " Surgical scar tissue contraction of the maxilla" are expressions handed down from textbook to textbook. Today, with the team approach to the cleft problem, and the skill of modern plastic surgery, scar tissue contraction of the maxilla is rarely seen. Many research workers (Scott, I953, I955 ; Sarnat, I958 ; Latham and Burston, I964) now consider that growth centres do not occur in the maxillary complex of bones but that the entire maxilla is in itself capable of overall growth which is closely co-ordinated with the expanding growth of the cartilage of the nasal capsule and careful surgery has little effect on this growth. Only when unnecessary surgical procedures are carded out, as for example when repeated attempts are made to close a palatal fistula, is growth affected. Scar tissue seldom occurs in mucoperiosteum. After competent surgery, the new tissue cannot be differentiated from the normal surrounding mucoperiosteum. The maxillary contraction which is usually blamed on scar tissue is mostly due to the extraoral pressure of the muscles of the face acting on a divided maxilla. Even in unoperated adult bilateral clefts (Glass, I956) maxillary contraction may be seen (Fig. i2), but once the lip has been repaired the pressure exerted by repaired orbiculads otis on the maxillary segments will cause rapid collapse of the segments. This occurs long before the palate is closed and is not related to the surgery.
B I L A T E R A L C L E F T OF L I P AND P A L A T E
I39
Dental Orthopmdic T r e a t m e n t . - - A f t e r the surgical closure of the palate in this Centre, no treatment except speech therapy is carried out until the child is about 4 years of age. Then dental orthopmdic treatment to realign the segments of the maxilla is started (Glass, 1959). The timing of this treatment depends on the co-operation of the child and the presence of a good anchorage to retain the appliance. The object of the treatment is to expand the maxillary segments and allow the repositioning of the premaxilla in its correct relationship to the lateral segments. This produces a normal
shaped maxillary arch into which the tongue can move during speech and swallowing ; it produces normal occlusion of the two jaws thus improving the functional activity of the mouth and the improved position of the segments changes the facial contour. In most clefts, the collapse of the segments occurs in the anterior part of the lateral segments, with the fulcrum in the region of the first molar tooth. To correct this it will be necessary to fit an appliance which produces anterior expansion with no posterior expansion. Figure 13 shows the appliance used here for all cleft palate expansions. It consists of two segments of acrylic joined by a 1-25 mm. heavy stainless-steel wire bent in the form of a double C spring. The middle loop is inactive and is joined by acrylic to cover any fistulm which may be present in the palate. The lateral segments are anchored to the teeth of the buccal segments by four Adams cribs and further retention is achieved by capping these teeth. This will give any type of individual expansion, and is ideally suited to give the rapid anterior expansion necessary in these cases. It is simple to construct, easy to wear and, being removable, is easy to keep clean. Expansions of over I cm. can be achieved with the one appliance, and it is unusual to need more than one appliance to complete the expansion of the maxillary segments. It also carries an acrylic palatal part which covers anterior fistula in the hard palate during expansion.
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B R I T I S H JOURNAL OF PLASTIC SURGERY
The disadvantages of the screw expansion plates are : I. Two or three plates are necessary to give the required amount of expansion. 2. Anterior expansion alone is difficult to achieve. 3. The opening of the midline fissure as the screw is turned allows food and air to escape into the nose if there is an anterior palatal fistula.
FIG. 14 Before and after expansion to produce improved arch relationship.
When the maxillary expansion is completed (Fig. I4), and there is a good upper to lower jaw occlusion, the segments are retained by a small prosthesis, carrying any missing lateral incisors. This must be worn until the permanent teeth erupt. CONCLUSION
The successful management of bilateral clefts depends on : I. A careful study of the soft tissue behaviour associated with the cleft. 2. Early lip and palatal surgery by a competent plastic surgeon. 3- Rapid dental orthopaedic correction of the three maxillary segments at 4 to 6 years. 4. A course of treatment as short and intense as possible followed by long periods of rest. I wish to thank the plastic surgeons at the Queen Victoria Hospital, East Grinstead, for their help in the preparation of this paper, and especially Mr C. R. McLaughlin and Mr R. L. B. Beare for co-operation in the treatment of cases shown in Figures 4 and 5 and Figures 6 and IO respectively. I am also indebted to Mr P. Broadbery and the staff of the Photographic Department for the colour slides and the published pictures. REFERENCES BILL, A. H., MOORE, A. W. and CoE, H. E. (1956). T h e time of choice for repair of cleft palate in relation to the type of surgical repair and its effect on the bony growth of the face. Plastic reconstr. Surg. x8, 469-473. BRAImR, R. O. and CRONIN,T. D. (1964). Dental orthopmdics and primary bone grafts. Cleft Palate ft. I, 31. FOGH-ANDERSEN,P. (1942). " Inheritance of Hare Lip and Cleft Palate ", p. 226. Copenhagen : Busck. -(1961). Incidence of cleft lip and palate : constant or increasing. Acta chir. scand. I22, lO6-111.
BILATERAL CLEFT OF LIP AND PALATE GEORGIADE, N. C., PICKRELL, K. L. and QUINN, G. W. (1964). Early bone grafts in alveolar palatal clefts. Cleft Palate J. I, 43. GLASS, D. F. (1956). Maxillary growth and its application to unoperated clefts of lip and palate. Trans. Eur. Orthodont. Soc., pp. 249-258. - - - - (1959). Rehabilitation of the child with cleft lip and palate. Dent. Pract. IO, 4. GRABER,T . M . ( I 9 4 9 ) . Craniofacial morphology in cleft lip and palate deformities. Surgery Gynec. Obstet. 88, 359. GRACE, L. G. (1943). Frequency of occurrence of cleft palates and hare lips. J. dent. Res. 22, 495-497. HlXON, E. H. (1951). A study of the incidence of cleft lip and palate in Ontario. Can. ft. Publ. Health, 42, 5o8-511. HORTON, C. E., CRAWFORD,H. H., ADAMSON,J. E., BUXTON,S., COOPER, R. and KANTER,I. (1964). Cleft Palateff. 1, 25. HOTZ, R. (1964). Early treatment of cleft lip and palate. Int. Symposium, April 9-1I, University of Zurich. Berne : Huber. HUDDART~A. G., NORTH, J. F. and DAVISj M. E. (1966). Observations on the treatment of cleft lip and palate. Dent. Pract. 16, 265-274. JESUS, D. E. (1959). A comparative cephalometric analysis of the non-operated cleft palate adult and normal adults. Am. ft. Orthodont. 45, 61-62. JOHANSEN, B. (1965). Primary bone grafting in clefts. Northcroft Memorial Lecture. Br. Soc. Study Orthodontics. (Not published.) JOLLEYS, A. (1954). A review of the results of operations on cleft palates with reference to maxillary growth. Br. ft. plast. Surg. 7, 229Joss, G. (1962). Evaluation of rotation advancement method for unilateral cleft lip repair. Br. J. plast. Surg. 15, 349. LATHAM,R. A. and BURSTON,W. R. (I964). The effect of unilateral clefts of lip and palate on maxillary growth. Br. ft. plast. Surg. 27, IO. McCAsH, C. R. (1957). Neonatal repair of cleft lip and palate. Br. ft. plast. Surg. 9, 235. McLAUGHLIN, C. R. (1956). Plastic surgery. In " British Encyclopmdia of Medical Practice and Medical Progress ", p. 154. London : Butterworths. MAISELS, D. O. (1966). Early orthopmdic treatment of clefts. Cleft Palate ft. 3, 76. MONROE, C. W., GRIFFITH, B. H., ROSENSTEIN, S. W. et al. (1968). Correction and preservation of arch form in complete clefts. Plastic reconstr. Surg. 41, lO8-112. NYLEN, B. (1966). Surgery of alveolar clefts. Plastic reconstr. Surg. 37, 42. PRUZANSKY, S. (1964). Pre-surgical dental orthopmdics. Cleft Palateff. I, 164-187. SARNAT, B. G. (1958). Palatal and facial growth in macaca rhesus monkeys with surgically produced palatal clefts. Plastic reconstr. Surg. 22, 29-41. SCHUCHARDT, K. (1966). Primary bone grafts in clefts of lip, alveolus and palate. In " Modern Trends in Plastic Surgery II ". Ed. Gibson, T., p. zi4. London : Butterworths. SCOTT, J. H. (1953). The cartilage of the nasal septum. Br. dent. ft. 95, 37-43. - (1955). Dent. Pract. 5, 208-214SESGIN, M. Z. and STARK, R. B. (1961). The incidence of congenital defects. Plastic reconstr. Surg. 27, 261. SKOOG, T. (1967). Use of periosteum and Surgicel tg~ for bone restoration in congenital clefts of the maxilla. Scand. ft. plast. Surg. I, 113. STARK,R. B. (1954). The pathogenesis of harelip and cleft palate. Plastic reconstr. Surg. 13, 2o-39. STRAITH, R. E., TEASLEY,J. L. and MOORE, L. T. (1955). Local anmsthesia in the newborn cleft repair. Plastic reconstr. Surg. 16, 125. SWANSON, L. T., MACCOLLUM,D. W. and RICHARDSON, S. O. (I956)- The evaluation of dental problems in cleft palate patients. Am. ft. Orthodont. 42, 749. WOOD, N. K., WRAGG, L. E. and STUTEVILLE,O. M. (I967). The premaxilla : embryological evidence that it does not exist in man. Anat. Rec. I58, 485. YANSY, M. (1955). Lateral cephalometric study of skeletal pattern in cleft lip and palate patients from six to thirteen years of age. Am. ft. Orthodont. 41, 236.
141
TBE incidence of cleft lip and palate occurring in the Anglo-Saxon race has been estimated at 1-75o to i-I,OOO live births (Fogh-Andersen, 1942, 1961 ; Grace, 1943 ; Hixon, 1951 ; Sesgin and Stark, 1961 ). Figure I shows the distribution of the various types of cleft taken from i,ooo cases attending the East Grinstead cleft lip and palate centre and shows that about 18 per cent. of clefts are bilateral. T h e important feature of this type of cleft is that it divides the maxilla into three separate segments, two lateral maxillary segments and one premaxillary segment, providing many problems for those responsible for their solution. It is now an accepted fact that all clefts have a tissue deficiency and this deficiency is mesodermal ; the failure of the embryonic tissues to develop is due to lack of mesodermal penetration (Stark, 1954). Although the amount of tissue deficiency must affect the degree of deformity, the width of the cleft and position of the segments are also dependent on other factors. T h e most obvious example is undoubtedly the displaced premaxilla which in some cases occupies a position anterior to the tip of the nose and bears no relationship to its correct place between the maxillary segments. How does it reach that position ? In normal development, the embryonic maxillary processes fuse at about the fifth week of intrauterine life. Then the mesodermal elements penetrate the first branchial arch and meet and eventually become the upper and lower maxillary processes. T h e whole face, including the lips, is now invaded by mesial advancement of the second branchial arch which supplies all the muscles of the face, especially the circumoral muscles. At the same time the maxillary clip of Callender advances to the midline to overlap and envelop the true premaxilla (Fig. 2) (Wood et aL, 1967). In bilateral clefts of lip and palate, the position of the three maxillary components is dependent on many factors. The lateral maxillary segments assume a position of stability determined by the soft tissue forces of the tongue and cheeks, and mesial collapse of these segments is often seen in untreated cases of all ages ; this is due to buccinator pressure acting on a defective maxillary arch. The protruded position of the premaxilla is caused by pressure from the lower lip and tongue acting on a premaxilla which has no restraifiing maxillary continuity and no intact circumoral musculature to prevent forward growth of the vomer, In bilateral clefts, therefore, the defective and displaced premaxiUa, held forward under the tip of the nose by overgrowth of the vomer, is forwardly displaced far from its correct position as the keystone of the arch and despite the many techniques available, only limited improvement rather than perfection can be expected. Presurgical Dental Orthopmdic Treatment.--This early appliance therapy is carried out during the months before lip closure. Its object is to align the maxillary segments and to retract the premaxilla to a position where surgical closure is made easier. The appliance using extra-oral traction on the prolabium pulls back this segment into contact with the lateral segments which themselves may be aligned to assist lip closure (Hotz, 1964; Maisels, 1966). 13o
]BILATERAL
CLEFT
OF LIP
AND
131
PALATE
T h e benefits claimed for this t r e a t m e n t are as follows : i. I t facilitates lip closure. 2. T h e m o t h e r m a y be relieved to think some t r e a t m e n t is being done. 35 3
2
q;tqh ?t ,'o"
Y
I PREMAXILLA
MAXILLA
UNILATERALONLY UNILATERAL INCIDENCE OFCLEFT TYPES - I000 CLEFTS FIG. i
Fig. I.--Types of cleft occurring in a sample of i#oo cleft lip and palate cases attending the East Grinstead cleft lip and palate centre for treatment. Bilateral clefts form 18 per cent. of the sample. Fig. 2.--Diagram of the facial aspect of the maxilla ; note that the premaxilla never appears on the face but is enclosed by the maxilla.
#
t
t,. ,
~
FIG. 2
However, these benefits are not universally accepted (Pruzansky, I964) and in m y opinion-I. 2. 34. 5-
it does not stimulate maxillary growth ; it does not produce " clinical or b o n y u n i o n " o f the segments ; it delays the time o f lip closure until the child is 9 to I2 months old ; it draws attention to the child's deformity at h o m e ; it provides added burdens o f travel and absence f r o m h o m e to the neglect of the rest of the family ; 6. it involves the orthodontist and technicians in work of doubtful value.
F r o m the work o f H u d d a r t et al. (I966) on this p r o b l e m in which he studied treated and untreated cases and f r o m m y own experience there is no apparent difference in the two groups o f children in later years. However, this therapy does seem to have a beneficial psychological effect on the orthodontist and surgeon concerned.
P r e s u r g i c a l O r t h o p m d i c s a n d P r i m a r y B o n e G r a f t i n g . m P r e s u r g i c a l orthopaedic treatment combined with early or p r i m a r y b o n e grafting has become a popular
132
B R I T I S H JOURNAL OF P L A S T I C SURGERY
method of treatment in some centres (Georgiade et al., 1964 ; Horton et al., 1964 ; Brauer and Cronin, 1965 ; Nylen, 1966 ; Schuchardt, 1966 ; Monroe et al., 1969). However, Johansen (1965) in his Northcroft Memorial Lecture (reviewing over IOO cases treated in this way during a period of IO years), concluded that the results were disappointing and stated that he has discontinued this method of treatment.
A
B C FIG. 3 Bilateral cleft of lip and palate (left) before lip closure, (right) following lip closure at 3 months. Note the favourable upper and lower lip contact producing a lip seal. Below: Models--A, before lip closure ; B, I½ years old before palatal closure ; C, 3 years old. Note the good arch shape produced by the reconstruction of the upper lip. No dental orthopmdic treatment has been carried out. At the East Grinstead centre no primary bone grafts are carried out, as the cleft lip and palate team feel that the benefits, if any, which might result from this treatment are out of alI proportion to the severity of the surgical procedure involved. T h e recent work by Joss (1962) and Skoog (1967) of boneless bone grafting, may produce better results. As orthodontist, I am responsible for the final alignment of the bony segments of the maxilla and to join these segments by bone before uniting the soft tissues of the face and before restoring the muscle function of cheeks and lips is, in my opinion, of doubtful value. Furthermore, collapse of the maxillary segments does occur in some cases despite the bone graft, especially in the mixed dentition age and the correction of this collapse in these cases necessitates long and tedious orthodontic treatment.
BILATERAL CLEFT OF LIP AND
PALATE
133
Lip C l o s u r e . - - T h e timing of lip closure varies considerably from centre to centre, but at East Grinstead is usually completed by 3 months of age. The premaxilla now comes under the influence of the united muscle of the upper lip and moves rapidly to a position of stability in contact with the lateral maxillary segments. In favourable cases, the
Fro. 4 Patient B . S . Top left: bilateral cleft of lip and palate before surgery. Note severe displacement of premaxilla. Top right: following vomerine resection, premaxillary setback and lip closure at 4 months. Below: same patient at 9 and 14 years old. There is no diminution in the nasal or facial growth.
premaxillary retraction will allow normal upper to lower lip contact and will establish a normal lip seal so vital to the stability of the premaxillary segment (Fig. 3). When considering the timing of lip closure it is a pity that the work by Straith et al. (I955) and McCash (I957) has not received more attention. Lip closure in the first week of neonatal life under local anaesthesia would seem to have many beneficial effects : I. The uniting of the muscles of the lip at the earliest possible time.
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2. The restoration of the normal muscle function of the face with its beneficial effect on growth and development as well as feeding. 3. The automatic retraction of the premaxilla. 4. The avoidance of a general anmsthetic. 5. The beneficial psychological effect on the mother by the immediate removal of the gross disfigurement and the fact that the mother and child can return home to their family and neighbours with the worst disfigurement corrected.
FIG. 5 Models of patient B . S . Top left: premaxilla is displaced forward z5 ram. Top right: following resection and premaxillary setback, prior to palatal closure. Below: (left) maxillary arch at 7 years ; (right) maxillary arch at i i years.
Vomerine R e s e c t i o n . - - T h e repositioning of the premaxilla by vomerine resection has been, and still is, severely criticised, but in cases where the premaxilla is displaced as far forward as the tip of the nose and the tissue deficiency combined with the soft tissue pattern of the lips is considered unfavourable for normal lip closure, this is undoubtedly the treatment of choice (Fig. 4). Mr C. R. McLanghlin has been doing this operation at East Grinstead in selected cases for I4 years (McLaughlin, 1956), and the eldest patient is now I4 years old. The results are excellent, and from an orthodontic point of view, both muscle function and arch relationship as well as facial contour are a great improvement on many cases treated in the standard way.
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Poor results by this method of treatment have been caused by poor assessment of the problem with inadequate planning and removal of too much vomerine bone or incomplete fixation of the premaxilla during bony union. Hence certain criticisms should be directed at the execution, not the method.
Fig. 6.--Patient M. L. Boy aged 3½ years, unfavourable lip posture is preventing the repaired upper lip from retracting the premaxiUa. The premaxiUa is therefore outside the normal circumoral musculature. Fig. 7.--Left: unoperated bilateral cleft showing the lower lip posture behind the premaxilla and inside the upper lip segments. Right: thumb sucking and tongue thrusting can displace the premaxilla.
FIG. 6
FIG. 7
The setback is timed when the lip is closed at 3 months, the vomerine bone removed is always less than appears necessary and the premaxilla is firmly refixed by a midline Kirschner wire through the front of the premaxilla into the vomer ; care is taken to keep the pin in the midline and thus avoid the developing teeth. The pin is retained for four weeks when union will have occurred (Fig. 5). In some cases following the standard treatment of lip closure with no premaxillary setback, the premaxiUa will not fall back to contact the lateral segments and a very poor result is obtained (Fig. 6). This is due to a combination of circumstances : (I) The
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FIG. 8 Patient M . L . Splint fitted to stabilise the premaxilla following vomerine resection. This is retained for at least one month.
FIG. 9 Patient M. L. Following removal of the Kirschner wire and the splint, it may be necessary to retract the premaxilla still further to establish incisor relationship. This simple orthodontic appliance is very effective.
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premaxilla was severely protruded. (2) The arch relationship shows a Class II malocclusion associated with a normal sized maxilla articulating with a smallmandible, thus preventing the upper and lower lip contact necessary for lip seal. Instead, the lower lip rests permanently inside the premaxilla preventing its retraction by the now united upper lip. (3) There may be a powerful tongue thrusting habit. (4) The child may suck his thumb (Fig. 7).
A
B C FIG. II Models of Patient M. L. A, At 3½ years of age before premaxillary setback. B, Following premaxillary setback. C, At 4 years of age, there is now normal upper to lower jaw relationship. In these cases a vomerine resection can give excellent results at 3 to 4 years of age when co-operation from the child permits. The procedure is the same as for the early setback, except that a small acrylic splint is prepared before surgery. The premaxilla is set back and pinned to the vomer. At this time the premaxillary segment and the two maxillary segments are freshened in the alveolar area to give bony union, and further stabilised by the splint which unites the three segments of the maxilla (Fig. 8). The pin is removed at four weeks but the splint which is removable is retained for three months. When the splint is removed the lower lip posture is carefully studied, and if there is no lip seal, and the lower lip impinges on the lingual side of the premaxilla, failure is inevitable unless active orthodontic treatment is carried out to retract the upper teeth to a position of stability inside the lower lip (Fig. 9). To achieve this it may be necessary to correct the collapse of the lateral segments at the same time as the premaxilla is retracted (Figs. IO and II).
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Surgical Closure o f the P a l a t e . - - T h e surgical closure of the hard and soft palate is usually done at East Grinstead at about I½ years, the main object being to separate the nasal from the oral cavity and to provide a muscular mechanism able to perform the intricate movements of speech and deglutition. The provision of intelligible speech is by far the most important part of the rehabilitation of these children, for without adequate speech their opportnnities today are very restricted.
FIG, I2 TWO bilateral clefts of lip and palate. Right: 3 months of age. Left: I5 years of age. Neither has had any surgery b u t both show considerable maxillary arch collapse.
Much has been written about the effect of surgery on maxillary growth and development and the surgeon has received more than his fair share of criticism (Graber, I949 ; Jolleys, t954 ; Yansy, I955 ; Bill et al., I956 ; Swanson et al., t956 ; Jesus, t959). " Surgical damage of growth centres " , " Surgical scar tissue contraction of the maxilla" are expressions handed down from textbook to textbook. Today, with the team approach to the cleft problem, and the skill of modern plastic surgery, scar tissue contraction of the maxilla is rarely seen. Many research workers (Scott, I953, I955 ; Sarnat, I958 ; Latham and Burston, I964) now consider that growth centres do not occur in the maxillary complex of bones but that the entire maxilla is in itself capable of overall growth which is closely co-ordinated with the expanding growth of the cartilage of the nasal capsule and careful surgery has little effect on this growth. Only when unnecessary surgical procedures are carded out, as for example when repeated attempts are made to close a palatal fistula, is growth affected. Scar tissue seldom occurs in mucoperiosteum. After competent surgery, the new tissue cannot be differentiated from the normal surrounding mucoperiosteum. The maxillary contraction which is usually blamed on scar tissue is mostly due to the extraoral pressure of the muscles of the face acting on a divided maxilla. Even in unoperated adult bilateral clefts (Glass, I956) maxillary contraction may be seen (Fig. i2), but once the lip has been repaired the pressure exerted by repaired orbiculads otis on the maxillary segments will cause rapid collapse of the segments. This occurs long before the palate is closed and is not related to the surgery.
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Dental Orthopmdic T r e a t m e n t . - - A f t e r the surgical closure of the palate in this Centre, no treatment except speech therapy is carried out until the child is about 4 years of age. Then dental orthopmdic treatment to realign the segments of the maxilla is started (Glass, 1959). The timing of this treatment depends on the co-operation of the child and the presence of a good anchorage to retain the appliance. The object of the treatment is to expand the maxillary segments and allow the repositioning of the premaxilla in its correct relationship to the lateral segments. This produces a normal
shaped maxillary arch into which the tongue can move during speech and swallowing ; it produces normal occlusion of the two jaws thus improving the functional activity of the mouth and the improved position of the segments changes the facial contour. In most clefts, the collapse of the segments occurs in the anterior part of the lateral segments, with the fulcrum in the region of the first molar tooth. To correct this it will be necessary to fit an appliance which produces anterior expansion with no posterior expansion. Figure 13 shows the appliance used here for all cleft palate expansions. It consists of two segments of acrylic joined by a 1-25 mm. heavy stainless-steel wire bent in the form of a double C spring. The middle loop is inactive and is joined by acrylic to cover any fistulm which may be present in the palate. The lateral segments are anchored to the teeth of the buccal segments by four Adams cribs and further retention is achieved by capping these teeth. This will give any type of individual expansion, and is ideally suited to give the rapid anterior expansion necessary in these cases. It is simple to construct, easy to wear and, being removable, is easy to keep clean. Expansions of over I cm. can be achieved with the one appliance, and it is unusual to need more than one appliance to complete the expansion of the maxillary segments. It also carries an acrylic palatal part which covers anterior fistula in the hard palate during expansion.
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The disadvantages of the screw expansion plates are : I. Two or three plates are necessary to give the required amount of expansion. 2. Anterior expansion alone is difficult to achieve. 3. The opening of the midline fissure as the screw is turned allows food and air to escape into the nose if there is an anterior palatal fistula.
FIG. 14 Before and after expansion to produce improved arch relationship.
When the maxillary expansion is completed (Fig. I4), and there is a good upper to lower jaw occlusion, the segments are retained by a small prosthesis, carrying any missing lateral incisors. This must be worn until the permanent teeth erupt. CONCLUSION
The successful management of bilateral clefts depends on : I. A careful study of the soft tissue behaviour associated with the cleft. 2. Early lip and palatal surgery by a competent plastic surgeon. 3- Rapid dental orthopaedic correction of the three maxillary segments at 4 to 6 years. 4. A course of treatment as short and intense as possible followed by long periods of rest. I wish to thank the plastic surgeons at the Queen Victoria Hospital, East Grinstead, for their help in the preparation of this paper, and especially Mr C. R. McLaughlin and Mr R. L. B. Beare for co-operation in the treatment of cases shown in Figures 4 and 5 and Figures 6 and IO respectively. I am also indebted to Mr P. Broadbery and the staff of the Photographic Department for the colour slides and the published pictures. REFERENCES BILL, A. H., MOORE, A. W. and CoE, H. E. (1956). T h e time of choice for repair of cleft palate in relation to the type of surgical repair and its effect on the bony growth of the face. Plastic reconstr. Surg. x8, 469-473. BRAImR, R. O. and CRONIN,T. D. (1964). Dental orthopmdics and primary bone grafts. Cleft Palate ft. I, 31. FOGH-ANDERSEN,P. (1942). " Inheritance of Hare Lip and Cleft Palate ", p. 226. Copenhagen : Busck. -(1961). Incidence of cleft lip and palate : constant or increasing. Acta chir. scand. I22, lO6-111.
BILATERAL CLEFT OF LIP AND PALATE GEORGIADE, N. C., PICKRELL, K. L. and QUINN, G. W. (1964). Early bone grafts in alveolar palatal clefts. Cleft Palate J. I, 43. GLASS, D. F. (1956). Maxillary growth and its application to unoperated clefts of lip and palate. Trans. Eur. Orthodont. Soc., pp. 249-258. - - - - (1959). Rehabilitation of the child with cleft lip and palate. Dent. Pract. IO, 4. GRABER,T . M . ( I 9 4 9 ) . Craniofacial morphology in cleft lip and palate deformities. Surgery Gynec. Obstet. 88, 359. GRACE, L. G. (1943). Frequency of occurrence of cleft palates and hare lips. J. dent. Res. 22, 495-497. HlXON, E. H. (1951). A study of the incidence of cleft lip and palate in Ontario. Can. ft. Publ. Health, 42, 5o8-511. HORTON, C. E., CRAWFORD,H. H., ADAMSON,J. E., BUXTON,S., COOPER, R. and KANTER,I. (1964). Cleft Palateff. 1, 25. HOTZ, R. (1964). Early treatment of cleft lip and palate. Int. Symposium, April 9-1I, University of Zurich. Berne : Huber. HUDDART~A. G., NORTH, J. F. and DAVISj M. E. (1966). Observations on the treatment of cleft lip and palate. Dent. Pract. 16, 265-274. JESUS, D. E. (1959). A comparative cephalometric analysis of the non-operated cleft palate adult and normal adults. Am. ft. Orthodont. 45, 61-62. JOHANSEN, B. (1965). Primary bone grafting in clefts. Northcroft Memorial Lecture. Br. Soc. Study Orthodontics. (Not published.) JOLLEYS, A. (1954). A review of the results of operations on cleft palates with reference to maxillary growth. Br. ft. plast. Surg. 7, 229Joss, G. (1962). Evaluation of rotation advancement method for unilateral cleft lip repair. Br. J. plast. Surg. 15, 349. LATHAM,R. A. and BURSTON,W. R. (I964). The effect of unilateral clefts of lip and palate on maxillary growth. Br. ft. plast. Surg. 27, IO. McCAsH, C. R. (1957). Neonatal repair of cleft lip and palate. Br. ft. plast. Surg. 9, 235. McLAUGHLIN, C. R. (1956). Plastic surgery. In " British Encyclopmdia of Medical Practice and Medical Progress ", p. 154. London : Butterworths. MAISELS, D. O. (1966). Early orthopmdic treatment of clefts. Cleft Palate ft. 3, 76. MONROE, C. W., GRIFFITH, B. H., ROSENSTEIN, S. W. et al. (1968). Correction and preservation of arch form in complete clefts. Plastic reconstr. Surg. 41, lO8-112. NYLEN, B. (1966). Surgery of alveolar clefts. Plastic reconstr. Surg. 37, 42. PRUZANSKY, S. (1964). Pre-surgical dental orthopmdics. Cleft Palateff. I, 164-187. SARNAT, B. G. (1958). Palatal and facial growth in macaca rhesus monkeys with surgically produced palatal clefts. Plastic reconstr. Surg. 22, 29-41. SCHUCHARDT, K. (1966). Primary bone grafts in clefts of lip, alveolus and palate. In " Modern Trends in Plastic Surgery II ". Ed. Gibson, T., p. zi4. London : Butterworths. SCOTT, J. H. (1953). The cartilage of the nasal septum. Br. dent. ft. 95, 37-43. - (1955). Dent. Pract. 5, 208-214SESGIN, M. Z. and STARK, R. B. (1961). The incidence of congenital defects. Plastic reconstr. Surg. 27, 261. SKOOG, T. (1967). Use of periosteum and Surgicel tg~ for bone restoration in congenital clefts of the maxilla. Scand. ft. plast. Surg. I, 113. STARK,R. B. (1954). The pathogenesis of harelip and cleft palate. Plastic reconstr. Surg. 13, 2o-39. STRAITH, R. E., TEASLEY,J. L. and MOORE, L. T. (1955). Local anmsthesia in the newborn cleft repair. Plastic reconstr. Surg. 16, 125. SWANSON, L. T., MACCOLLUM,D. W. and RICHARDSON, S. O. (I956)- The evaluation of dental problems in cleft palate patients. Am. ft. Orthodont. 42, 749. WOOD, N. K., WRAGG, L. E. and STUTEVILLE,O. M. (I967). The premaxilla : embryological evidence that it does not exist in man. Anat. Rec. I58, 485. YANSY, M. (1955). Lateral cephalometric study of skeletal pattern in cleft lip and palate patients from six to thirteen years of age. Am. ft. Orthodont. 41, 236.
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