THE UNOPERATED ADULT BILATERAL CLEFT OF THE LIP AND PALATE By KHOO Boo-CHAI 1

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THE UNOPERTED DULT ILTERL CLEFT OF THE LIP ND PLTE y KHOO oo-chi 1 ILTERL cleft of the lip and palate is the most severe form of the common oral cleft deformity.

1 THE UNOPERTED DULT ILTERL CLEFT OF THE LIP ND PLTE y KHOO oo-chi 1 ILTERL cleft of the lip and palate is the most severe form of the common oral cleft deformity. In highly developed Western countries, the unoperated adult cleft is nowadays rarely seen, whereas in some areas of developing countries they are still present but in gradually diminishing numbers. This is because of increased awareness and availability of good surgical care. Studies on the adult cleft population have been carried out both in the living as well as in the dead and we have summarised the papers of current interest for easy reference (Table). However, studies confined only to adult bilateral clefts are rare (Davis, 1951 ; Hyslop and Wynn, 1952). Narula and Ross (197o) have recently studied facial growth in children with bilateral clefts. During the last 14 years we have had the opportunity of studying 95 adults with unoperated clefts of the lip and/or palate, and of these there were 27 cases with bilateral clefts. These constituted 25 per cent. of our adult cleft population. For purposes of this study, we have divided the 27 cases into three groups. The first group consisted of 12 patients who have had no repair done at all. The second group of IO patients only had a lip repair without repair of the cleft palate and the third group of 5 cases had their lips repaired together with surgical extirpation of the premaxilla. The palate was left unrepaired. These three groups presented us with a unique opportunity of studying the behaviour of the maxillary arch, one, with the oral muscular ring re-established early in life and the other, without. Our age limit was set at 15 years and above because this represents a relatively mature stage of development in our country. During this period, we also saw 67 personal cases of unoperated bilateral clefts outside the adult range (from birth to 14 years of age). The study of the adults with unilateral unoperated clefts will be embodied in a separate paper. We propose to discuss our population of bilateral clefts under t,~o main headings : (I) the lip and prolabium ; (2) the maxillary arch. The Lip and Prolabium.--We have noticed over a number of years the peculiarity of the prolabium as regards hair growth in our bilateral cases. Many of those (all of Mongoloid racial stock) who came for secondary lip revision do not have hair growth on the prolabium itself. We then examined carefully our males in the first group and we found that out of the nine males, eight had prolabiums which were barren of hair (Fig. I), and in the remaining one case, the hair growth was very sparse indeed. This observation is in keeping with that made earlier by Veau (1938) and more recently by Converse et al. (197o). However, Cronin (1957) and Marcks and associates (1957) whose cleft population consisted predominantly of Caucasians, have specifically stated that hair grew freely in the prolabium of their cases. Our own findings have taught us not to be too quick in condemning those operative methods which' incorporate the prolabium into the columeila in a bilateral lip repair. We agree with Pitanguy (1967) that in an adult with a very small prolabium this structure can be conveniently used in tow to lengthen the columella. The lateral lip elements, which are usually well developed and mobile, can then be easily brought across to the midline to form the upper lip. 1 ddress : 62I Geylang Road, Singapore I4.

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252 RITIStI JOURNL OF PLSTIC SURGERY The other factor which is of interest to us is the sizc of the prolabium.

mong the Mexicans, a severe underdevelopment of the prolabium and columella has been observed in all their bilateral cases (Ortiz-Monasterio et al., 1966).

The small prolabium is more frequently found in the complete clefts whereas the well-developed prolabium is found in the incomplete cleft.

F~ra (1968) working on a stillborn cleft population observed that in the incomplete bilateral cleft, the upper uncleft portion of the lip is well filled with muscle fibres which pass from the lateral

3 252 RITIStI JOURNL OF PLSTIC SURGERY The other factor which is of interest to us is the sizc of the prolabium. This is of some importance because the technique we use is to some extent dependent on it. mong the Mexicans, a severe underdevelopment of the prolabium and columella has been observed in all their bilateral cases (Ortiz-Monasterio et al., 1966). In our series, the prolabial size varied from small to well-developed. The small prolabium is more frequently found in the complete clefts whereas the well-developed prolabium is found in the incomplete cleft. This may be due to the peculiar blood supply and muscle arrangement. F~ra (1968) working on a stillborn cleft population observed that in the incomplete bilateral cleft, the upper uncleft portion of the lip is well filled with muscle fibres which pass from the lateral lip element to the prolabium. FIG. I and 13, Two cases of bilateral cleft lip, one repaired and one unrepaired, showing complete absence of hair on the prolabium. C, Study model of the unrepaired cleft lip patient showing contraction of the maxillary arch in the bicuspid reg.ion. The palate was intact. c In the adult with a well-developed prolabium, we have found that Millard's technique (I967) of bilateral lip closure with simultaneous lengthening of the columella with a fork flap is an extremely valuable one-staged procedure. The Maxillary rch.--when the lip, premaxilla and palate have been left undisturbed until adulthood, the lateral alveolar element gradually grew forward giving a fairly good profile (Fig. 2). We had only one case of marked protrusion of the premaxilla of the unoperated infant type. In this teenager (Fig. 3), the nasal stem was long (I I mm. beyond the alveolar margin) but the premaxilla was not rotated on the nasal stem. The right lateral alveolar element was behind and in contact with the nasal stem but there was a gap of 6 mm. on the left side. Whether this is a transitory phase before the alveolus grows more forward with age is problematical. However, when the lip was repaired, the premaxilla responded as in the infant and it was moulded back and assumed a well aligned position with the rest of the maxillary arch. rch collapse occurred in 5 out of IO cases (Fig. 4). Hypoplasia of the premaxilla

THE UNOPERTED DULT ILTERL CLEFT OF THE LIP ND PLTE 253 has been attributed to the surgica! set-back operation (F~ra and Hrivn~kova, I965).

In two cases, the premaxilla was small with one incisor present only.

We have not seen a case in which the premaxilla was too big giving rise to an undue prominence of the lip.

4 THE UNOPERTED DULT ILTERL CLEFT OF THE LIP ND PLTE 253 has been attributed to the surgica! set-back operation (F~ra and Hrivn~kova, I965). ut in this group there were two cases in which the premaxilla was grossly hypoplastic and was trapped anteriorly (Fig. 5). These were similar to the case described by Davis (I95I). In two cases, the premaxilla was small with one incisor present only. In the remaining six cases, the premaxilla was well developed, and in two of them there were four incisors present in the premaxilla. The other two cases had cleft of the lip only. We have not seen a case in which the premaxilla was too big giving rise to an undue prominence of the lip. The occlusal relationship of the molars in all our cases is relatively normal confirming that the deficiency is present in the anterior part of the maxilla in the neighbourhood of the clefts. FIG. 2 female aged 36 years showing a good profile without protrusion of the premaxilla. There is an anterior open bite. The lateral incisors were extracted before surgery. When the lateral lip elements were drawn apart, we see that the lateral alveolar elements have grown forward giving a good maxillary arch. The poor development of the mid-face frequently seen after surgery has been attributed to a number of factors, viz. contraction of scar tissue, disruption of blood supply and interference with growth centres. In 196o, Greet-Walker drew attention to a possibility that diminished growth potential inherent in the cleft deformity itself may be a factor in some of these cases. We had one case of maldevelopment of the middle third of the face with radiological evidence of hypoplasia of the body of the maxilla and maxillary retrognatism. Dr Oon Chong Lin, D.M.R.D. reported it thus : " The bone defect in the hard palate from the bilateral cleft deformity is best appreciated on the postero-anterior 20 radiograph. The deficiency measures ram. There is minimal bowing of the septum to the left together with hypertrophy of the nasal turbinates. n outstanding feature of the palate cleft is the under-development of the maxillae, with growth arrest notably in the antero-posterior direction. This had resulted in retrusion of the maxillae and a concave facial profile. The maxillary antra are poorly pneumatised. No significant arrest of growth in the vertical direction is noted. There is satisfactory apposition of the upper and lower teeth. The mandible appears normally developed and there is evidence of a mild prognatism." C

254 RITISII JOURNL OF PLSTIC SURGERY FIo. 3 Protrusion of the premaxilla of Ii mm.

Her inferior turbinate on the left side is grossly hylzertrophied.

protruded premaxilla after re-establishment of the oral musculature. The palate was repaired at a subsequent date.

5 254 RITISII JOURNL OF PLSTIC SURGERY FIo. 3 Protrusion of the premaxilla of Ii mm. beyond the alveolar margin resembling the infant type of arch collapse in a I9-year-old girl. Her inferior turbinate on the left side is grossly hylzertrophied. Study models of the maxillary arch before, and one year after lip repair showing spontaneous movcment of the protruded premaxilla after re-establishment of the oral musculature. The palate was repaired at a subsequent date. FIG. 4 FIG. 5 Fig male adult aged 20 showing a well-develooed, externally rotated 4-incisor premaxilla with a collapsed maxillary arch. Fig. 5.--Photograph shows a hypoplastic premaxilla in a 3o-year-old male which is trapped anteriorly. Note the small almost horizontally inclined incisors. The prolabium is small and hairless but the lateral lip element is well developed and extremely mobile. C FIG. 6 Maldevelopment of the middle third of the face with retarded growth of the septal cartilage giving rise to a snub nose in a 22-year-old male adult. Note the small premaxilla with a well-aligned maxillary arch. There was no hair growth on the small prolabinm.

THE UNOPERTED DULT ILTERL CLEFT OF THE LIP ND PLTE 255 He also had a snub nose due to retarded growth of the septal cartilage (Fig. 6).

We also detected a case of contraction of the maxillary arch in the bicuspid region in a case with a bilateral cleft of the lip only (Fig. I, c).

intervention (Muir and odenham, I966; Reichert, I97o). In this case, the condition was discovered pre-operatively. FIG.

6 THE UNOPERTED DULT ILTERL CLEFT OF THE LIP ND PLTE 255 He also had a snub nose due to retarded growth of the septal cartilage (Fig. 6). This deformity, for which we could not find any organic cause, could well fall within this group. We also detected a case of contraction of the maxillary arch in the bicuspid region in a case with a bilateral cleft of the lip only (Fig. I, c). This is an unusual and interesting finding because hitherto it has been known to occur only in those with post-alveolar or incomplete clefts of the lip and palate who have had prior surgical intervention (Muir and odenham, I966; Reichert, I97o). In this case, the condition was discovered pre-operatively. FIG. 7 This I8-year-old male patient had his premaxilla removed in early infancy during the repair of his lip. Note the canines in the midline and the malocclusion. The lateral view shows severe maxillary retrognatism. Could this be due to reduction in bulk from absence of the premaxilla? FIG. 8 The premaxilla of this 25-year-old male patient was surgically extirpaeed in early childhood to facilitate lip closure. Note " lateral drift "of the left palatal segment giving a wide palatal hiatus of I inch (25 mm.) Next, we were able to study a group of Io adults who only had their lips repaired in early childhood without subsequent closure of the palate. These cases did not have the benefit of pre- or post-operative orthodontics because this treatment was not available at that time in our country. It is interesting to note that in only one case was there an arch collapse with the premaxilla trapped in a forward position. It seems reasonable to postulate that when the labial support is re-established early in childhood, the oral muscular ring is an important factor in preventing the forward position of the premaxilla which was fairly frequent in the unoperated group. Whether there was an initial postoperative arch collapse we do not know. ut with growth and with softening of the lip scar, the lateral alveolar segment grew forward and outward beautifully. There were no cases of maxillary retrognatism. The cases in this group amply demonstrate that when the lip scar softens, there is a good chance for the maxillary arch to expand by

256 RITISlt JOURNL OF PLSTIC SURGERY itself with growth, and the premaxilla restrained by the repaired lip will gradually fit itself in a good arch position.

7 256 RITISlt JOURNL OF PLSTIC SURGERY itself with growth, and the premaxilla restrained by the repaired lip will gradually fit itself in a good arch position. These cases have a loosely hanging lip with an ample supply of tissues in the lateral lip element when they come up for secondary lip surgery. In our third and last group of five cases the premaxilla had been surgically extirpated (elsewhere) to facilitate primary lip closure. The palate was also left unrepaired. This single operative step on the premaxilla had a disastrous effect on the development of the middle third of the face. In four out of five cases there was severe maxillary retrognatism. In one case with a bilateral cleft involving the lip and the alveolus up to the incisive foramina, after surgical extirpation of the premaxilla and lip repair the lateral alveolar element grew forwards and met in the midline thus obliterating the gap in the alveolus (Fig. 7). In this case, we were not sure if the maxillary retrusion was due in some measure to the loss of bulk of the premaxilla. We have also noted a rather interesting observation of a "lateral drift" of one of the palatal segments when the premaxilla was surgically extirpated (Fig. 8). This gives rise to a wide (more than 2o ram.) palatal hiatus which makes surgical closure a bit difficult from the technical point of view. SUMMRY We were presented with an unique opportunity of studying 27 adults with unoperated bilateral cleft of the lip and palate. For the sake of convenience, these cases were divided into three groups. In the first group of I2 cases there was no repair done on either the lip or palate. Here we noted that half of the cases had various degrees of arch collapse. Hair growth on the prolabium was present in only one out of nine male adults. lso, the size of the prolabium, like that in infants, varied from small to well developed depending on the type of cleft present. There was one case of contraction of the maxillary arch in the bicuspid region which is a rare finding. In the second group of IO cases where the lip only had been repaired, we found that the oral musculature when re-established early had a salutary effect on the maxillary arch. The premaxilla was restrained by the repaired lip and when the lateral alveolar element grew forward, it nestled itself in good alignment in 9 out of IO cases. Finally, in our third group of five cases, we noted that extirpation of the premaxilla, the maxillary " cornerstone ", had a disastrous effect giving rise to maldevelopment of the middle third of the face in later life. The author would like to thank Oon Chong Lin, D.M.R.D. and Choo Teck Chuan, F.D.S.R.C.S., D.Orth.R.C.S., for their help in the study of these cases. REFERENCES THERTON, J. D. (I967). Morphology of facial bones in skulls with unoperated unilateral cleft palate. Cleft Palate Journal, 4, I8-3o. CONVERSE, J. M., HOGN, M. and DuI'UIS, C. C. (197o). Combined nose-lip repair in bilateral complete cleft-lip deformities. Plastic andreconstruetive Surgery, 45, lo CRONII% T. D. (I957). Surgery of the double cleft lip and protruding premaxilla. Plastic and Reconstructive Surgery, 19, DVIS,. D. (I95I). Unoperated bilateral complete cleft lip and palate in adult : Case report and comments. Plastic and Reconstructive Surgery, % DE JESUS, J. (I959)- comparative eephalometric analysis of non-operated cleft palate adults and normal adults. merican Journal of Orthodontics, 45, FR, M. (I968). natomy and arteriography of cleft lips in stillborn children. Plastic and Reconstructive Surgery, 42, FR, M. and HRIVNKOV, J. (1965). The problem of protruding premaxilla in bilateral total clefts. eta Chirurgia Plasticae, 7,

8 THE UNOPERTED DULT ILTERL CLEFT OF THE LIP ND PLTE GREEa WLKER, D. (196o). Clefts of the palate and timing of the operation. In Transactions of the International Society of Plastic Surgeons, 2nd Congr., I959, P. 40- Edinburgh : Livingstone. HYSLOP, V.. and WYNN, S. K. (I952). one flap technique in cleft palate surgery. Plastic and Reconstructive Surgery, 9, 97-io7. INNIS, C. O. (t962). Some preliminary observations on unrepaired hare-lips and cleft palates in adult members of the Dusun Tribes of North orneo. ritish Journal of Plastic Surgery, I5, Lw, F. E. and FULTON, J. T. (1959). Unoperated oral clefts at maturation. I. Study design and general considerations. merican Journal of Public Health, 49, I517-I524 MRCKS, K. M., TR~VSKIS,. E. and PYNE, M. J. (1957). ilateral cleft lip repair. Plastic and Reconstructive Surgery, rg, 4Ol-4O8. MESTRE, J., DE Jl~sos, J. and SUTELNY, J. D. (r96o). Unoperated oral clefts at maturation. ngle Orthodontist, 3o, MILLRD, D. R. Jr. (1967). ilateral cleft lip and a primary forked flap : preliminary report. Plastic and Reconstructive Surgery, 39, Mum, I. F. K. and ODENHM, D. C. (1966). Secondary repair of cleft lip and palate deformities. In " Modern Trends in Plastic Surgery, II ", p ed. Gibson, T., London : utterworth. NRtrL, J. K. and Ross, R.. (197o). Facial growth in children with complete bilateral cleft lip and palate. Cleft Palate Journal, 7, ORTIZ-MONSTERIO~ F., SERRNO,., RRER~ G., RODRIGUEZ-HOFFMN, H. and VINGERS~ E. (1966). study of untreated adult cleft palate patients. Plastic and Reconstructive Surgery, 38, PITNGUY, I. and FRNCO, T. (1967). Non-operated facial fissures in adults. Plastic and Reconstructive Surgery, 39, REICHERT, H. (197o). Osteoplasty in complete clefts of the secondary palate. ritish Journal of Plastic Surgery, 23, VEU, V. (1938). " ec-de-li~vre ". Paris : Masson. 257

OF THE LIP AND PALATE. By T. D. FOSTER, M.D.S., F.D.S., D.Orth.R.C.S. School of Dental Surgery, University of Birmingham

OF THE LIP AND PALATE. By T. D. FOSTER, M.D.S., F.D.S., D.Orth.R.C.S. School of Dental Surgery, University of Birmingham MAXILLARY DEFORMITIES IN REPAIRED CLEFTS OF THE LIP AND PALATE By T. D. FOSTER, M.D.S., F.D.S., D.Orth.R.C.S. School of Dental Surgery, University of Birmingham IN patients with repaired clefts of the