|Year : 2015 | Volume
| Issue : 2 | Page : 92-97
Primary rhinoplasty at the time of unilateral cleft lip repair: A review and our protocol
Puthucode V Narayanan, Hirji Sorab Adenwalla
The Charles Pinto Centre for Cleft Lip, Palate and Craniofacial Anomalies, Jubilee Mission Medical College and Research Institute, Thrissur, Kerala, India
|Date of Web Publication||17-Aug-2015|
Dr. Puthucode V Narayanan
The Charles Pinto Centre for Cleft Lip and Palate, Jubilee Mission Medical College and Research Institute, Thrissur, Kerala
Source of Support: None, Conflict of Interest: None
The cleft lip nasal deformity has been well described. However, for a long time, cleft surgeons feared that repair of the cleft lip nose at the time of primary repair would cause a growth disturbance especially of the nose. Hence the nasal deformity was not repaired until later. However, from the time of Blair and Barrett Brown, it has been shown that there are no deleterious growth effect from primary nasal interventions. At our centre the senior surgeon has performed primary nasal correction including septal respositioning from the late 1960s. There has been no deleterious growth effect and the overall appearance of the nose has actually improved. This is now well established through many objective studies. Hence it is now imperative that the deformity of the nose including the septum be addressed at the time of primary unilateral cleft lip repair.
Keywords: Closed rhinoplasty, primary rhinoplasty, primary septal repositioning, unilateral cleft lip
|How to cite this article:|
Narayanan PV, Adenwalla HS. Primary rhinoplasty at the time of unilateral cleft lip repair: A review and our protocol. J Cleft Lip Palate Craniofac Anomal 2015;2:92-7
|How to cite this URL:|
Narayanan PV, Adenwalla HS. Primary rhinoplasty at the time of unilateral cleft lip repair: A review and our protocol. J Cleft Lip Palate Craniofac Anomal [serial online] 2015 [cited 2019 Nov 19];2:92-7. Available from: http://www.jclpca.org/text.asp?2015/2/2/92/162961
| Introduction|| |
The nose in a unilateral cleft lip child has some tell-tale deformities that have been well documented by Huffman and Lierle.  However, for a long time most cleft surgeons were reluctant to attempt a correction of the nasal deformity during primary cleft lip surgery. This was due to a notion that early nasal repair would cause a detrimental effect on the growth of the nose and the maxilla. 
Vilray Papin Blair and James Barrett Brown (1930) were among the first to attempt primary nasal correction with unilateral cleft lip repair.  They undermined the skin, thus, separating it from the cartilage and used mattress sutures tied over a bolster on the skin.
Gillies and Millard in 1953  also performed alar cartilage dissection and repositioned the septal cartilage after freeing it from its deviated position in the vomerine groove, straightened it, and sutured it to the lip muscle on the cleft side.
Berkeley (1959)  followed an aggressive primary correction of the cleft lip nose. He stressed the importance of straightening the septum. He resected the nasal spine and performed a rotation of the nose on the cleft side. He used a mid-line columellar incision for his primary repair.
| The Cleft Lip Nasal Deformity|| |
These have been well documented by Huffman and Lierle.  We shall list some of the significant components:
- The columella on the cleft side is short.
- The anterior nasal spine is displaced to the noncleft side.
- The anterior part of the nasal septal cartilage is also deviated to the noncleft side.
- The cleft side nostril is wider.
- The cleft side ala is buckled inward.
- The cleft side alar dome is retroplaced.
- The angle between the cleft side medial and lateral crura is obtuse [Figure 1].
| Evolution of the Concept of Repair of the Cleft Lip Nose|| |
Early cleft surgeons faced the dilemma on whether to repair the cleft lip nose primarily. Surgeons who shied away from primary correction feared that they would cause harm to the growth of the nose and the maxilla. There have been some animal studies to substantiate this claim.  However, this fear has been repeatedly been countered by numerous studies showing that primary repair causes no such deleterious effects.
As Millard notes,  Blair, Brown and McDowell, and their disciples have undermined thousands of alar cartilages without stunting nasal growth, and hence that aspect need not be a deterrent. Many early surgeons used external incisions. The results were generally unsatisfactory. Interest in primary rhinoplasty was rekindled by McComb and Coghlan.  When his procedure was first presented on 1975, some surgeons predicted drastic interference with subsequent development of the nose. On review, there did not appear to be any interference with growth. To a large extent, the children were spared the embarrassment of a deformed nose through their childhood years.
McComb felt that more harm was probably done by failing to correct the nasal deformity at the time of lip repair. The alar cartilage became locked and tethered in its displaced position by scar and the transverse shortage of nostril lining and growth of the nasal tip was altered. Secondary correction of the cleft lip nose was always more difficult to achieve.
This has been supported by others - Byrd et al.,  Haddock et al.,  Cussons et al.  as well. Cutting believed that primary nasal repair is durable and decreases the extent of secondary surgery in adolescents.
Anthropometric assessment has also shown no interference with nasal growth when the lip and nose were repaired simultaneously.  This study also showed better symmetry of the nostril and nasal dome projection and better correction of the alar flaring and overall balanced growth and development of the alar complex with primary nasal repair.
This has been our belief too. , The senior author has performed an aggressive correction of the cleft lip nose from the late 1960s on thousands of unilateral cleft lip children, and we have not found any detrimental effect on the growth of the nose. In fact, the overall shape and symmetry of the nose is better and the extent of secondary deformity is much less when compared to the patients who come to our center after having had primary surgery without any nasal intervention at some other centers.
Despite the plethora of evidence in favor of primary nasal correction, there continues to be a minority of cleft surgeons who believe that primary repair does not diminish the need for further operations and also that the magnitude of the secondary deformity is also not decreased. 
The advent of the modality of nasoalveolar molding (NAM) has made the cleft lip nasal deformity less severe during primary repair. However, NAM without any nasal surgical correction does not produce any lasting results. 
| Open/Closed/Semi-Open Rhinoplasty|| |
An open approach has been advocated by some authors. ,
However, many others, including us, use a closed approach. ,,,
A semi open method is also in vogue. ,,
We prefer the closed technique because we believe that we obtain results comparable with the open techniques.
| The Charles Pinto Centre Protocol for Primary Correction of the Cleft Lip Nasal Deformity|| |
At our center, the senior surgeon has been performing an aggressive closed rhinoplasty with septal repositioning in all complete unilateral cleft lips at the time of the primary repair. , All such lips are repaired using the Millard rotation-advancement procedure. The ala is approached from both the medial and the lateral aspects using a pair of curved Kilner Scissors [Figure 2]. The scissors are introduced medially through the incision at the base of the columella and laterally through the perialar incision. Dissection is carried out in the plane between the dorsal skin and the lower and the upper lateral cartilages on the cleft side, so that these cartilages are completely devoid of any skin attachments from the alar rim up to the nasal bones. A more limited dissection is also carried out on the noncleft side up to the dome. We do not attempt to separate the lower lateral cartilage from the vestibular lining as the cartilage is firmly adherent to the lining and we believe that it is almost impossible to separate the two in a closed dissection. The freed cleft side lower lateral cartilage is hitched to the noncleft side upper lateral cartilage using a bolster suture. An interdomal suture is also introduced through the noncleft side nostril to narrow the tip.
We use the Millard cinch suture to correct the alar flare. This suture (5-0 polypropylene) is taken from the noncleft side nostril through the membranous part of the septum and then through the paranasal muscles at the base of the ala and then again through the membranous septum to the noncleft side and tied there as a mattress suture. The perialar incision helps in identifying the paranasal muscles well, and aids in obtaining a good bite on them in order to correct the alar flare better. We have observed that when this perialar incision is avoided by some surgeons, the postoperative results do show some degree of alar flare in most patients.
Primary alar cartilage dissection has gained wide acceptance now. ,, However, when it comes to the repositioning of the deviated nasal septal cartilage primarily, there is still widespread reluctance on the part of cleft surgeons.
As already mentioned, Gillies and Millard (1953)  described the separation of the deflected septum from the vomerine groove, the straightening of the septum, and its suture to the cleft side lip muscles.
Berkeley (1959)  also stressed the straightening of the septum and resection of the anterior nasal spine.
However, the septal correction has still not gained wide acceptance. The Senior Surgeon at our center and his mentor Prof. Charles Pinto have aggressively addressed the problem of the deviated nasal septum from the late 1960s. , This was not accepted by cleft surgeons for a long time.
The nasal septum in unilateral cleft lip patients is always deviated to the noncleft side anteriorly, and the anterior nasal spine is similarly displaced to the noncleft side. We approach the septum by incising the mucoperichondrium on the cleft side over the groove at the base of the septum. The mucoperichondrium is carefully stripped off the underlying septal cartilage. The septospinal ligament is then identified anteriorly and divided to expose the anterior border of the septal cartilage. The cartilage is separated at its anterior border from the mucoperichondrium on the noncleft side. This is essential to avoid inadvertent shearing of the cartilage during the next step, which is the separation of the mucoperichondrium on the noncleft side from the cartilage. Separating the septal cartilage from the mucoperichondrium on both sides does no harm. The septal cartilage is freed from the perpendicular plate of the ethmoid and the vomer. The cartilage thus freed of its inferior and posterior attachments, still tends to buckle when repositioned toward the midline. To avoid this, a sliver of cartilage is excised off the inferior border. Previously, we also used to excise a wedge of cartilage from the anterior border. However, we have discontinued this now as we have realized the importance of the anterior most part of the septum for support of the tip of the nose in these patients. The cartilage still has a bow-stringing effect, and this is overcome by making scoring incisions on the cartilage on the noncleft side to make it flail [Figure 3]. The final step in the cartilage repositioning is the suturing of the cartilage to the newly constructed nasal floor on the cleft side. Ideally, the cartilage should be anchored to bone or periosteum in the mid line. However, in the cleft child, there is no such structure in the mid line. The anterior nasal spine is itself displaced to the noncleft side, as already mentioned. Hence, we compromise and overcorrect by suturing it to the soft tissue on the cleft side [Figure 4]. We believe that there will be a drift the cartilage medially with time. We can acknowledge that in most of our patients we do not need to perform any further correction of the septal position subsequently. We advocate this aggressive septal repositioning as we believe that it has not caused any adverse effects on nasal or maxillary growth. While we have not yet been able objectively to substantiate this belief due to logistical reasons, there are enough studies in literature to confirm our philosophy.
|Figure 3: Septal cartilage the shaded portion on the inferior aspect is excised|
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Anderl et al. and his group  have published their results after 35 years of septal repositioning and shown that there is no late damage and that secondary correction is necessary only in 20% of their patients. They also stress the functional improvement in breathing in their patients. They separated the septal cartilage from mucoperichondrium on both sides in their patients.
Smahel et al.  used X-rays to study the effects of primary septal cartilage separation from the maxilla and the nasal cartilages 15 years after primary correction. They concluded that after primary repositioning of the nasal septum patients had a more favorable nasal prominence and better vertical growth of the face than patients who did not have a primary septal correction. The maxillary retrusion in the two groups was similar.
Mulliken and Martínez-Pérez , have added septal work in his primary unilateral cleft lip repairs since 1995. His group has studied these patients using posteroanterior cephalograms retrospectively and found less sepal deviation and smaller contralateral turbinates in these patients. They agree with us that there is no growth disturbance to the nose from primary septoplasty.
Other studies have established using cephalograms that there is a long-term overall beneficial effect of primary septal correction on nasal symmetry and tip projection with no negative growth effects. 
| Columella|| |
As mentioned earlier,  the cleft side hemicolumella is always shorter in unilateral cleft lip patients. As advocated by Millard,  we use the C flap to lengthen the hemi columella in all our unilateral cleft lip patients. We have observed that most of our patients have symmetrical columella as a result of this. We use the excised sliver of septal cartilage as a columellar strut graft. When many of these patients undergo rhinoplasty subsequently, we do observe the presence and actual growth of this cartilage graft in many of them.
| Other Deformities|| |
There are some other associated deformities observed in the cleft lip noses.
| High Riding Nostril|| |
When there is a wide alveolar anteroposterior disparity between the medial and lateral elements, we note that the nostril base comes to lie at a more superior level than its counterpart on the noncleft side. We have used the unequal Z-plasty [Figure 5] and [Figure 6] described by Jackson  to prevent this deformity. Ever since we commenced the use of this refinement, the incidence of such high riding nostrils has diminished. We believe that this deformity can be completely eliminated only when the medial and lateral maxillary segments are at the same anteroposterior plane and this may be possible using NAM.
|Figure 6: Illustrations of Jackson's unequal Z-plasty on the nasal layer. (a) High riding nostril. (b) Z-plasty incisions marked|
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| Vestibular Web|| |
This is a nagging problem during cleft lip repair. Patel and Mulliken  believes that this has bony, cartilaginous, and soft tissue components. He uses intercartilaginous sutures under vision in his semi open approach and releases the attachments of the lateral crus from its piriform ligamentous attachments. In addition, he uses a lenticular excision of the web as he believes that there is an excess. This is in contrast to the belief of others that there is actually less of vestibular lining. , We too believe that the vestibular lining is to be preserved and are not sure if there is actual excess. Moreover, there is a need for all the available soft tissue lining during future rhinoplasties to avoid alar rim notching. The mentor of the Senior Surgeon Prof. Charles Pinto devised a Z-plasty that helps to address the web. ,, One has to be very meticulous during the dissection of the lining to avoid damage to the underlying cartilage [Figure 7]a and b. However, of late we do not perform this Z-plasty often as most of these children undergo preschool rhinoplasty and the web tissue is then used to build up a notch-free alar rim.
|Figure 7: Pinto's Z-plasty for nostril web (a) Marking. (b) Completed Z-plasty|
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With such an extensive closed primary alar cartilage shift and septal repositioning, we are able to obtain consistent long-term results [Figure 8]a, b and [Figure 9]a, b. The classical cleft lip nose stigmata of the grossly deviated septum, the grooved, slumped ala with a wide flare is almost never seen in our patients. However, most of them almost 80% - have a slight droop in the soft triangle. While this is not gross by any standards, it is still noticeable enough to invite ridicule from peers at school. Hence, we perform an open rhinoplasty at 5½-6 years in these children using a sutural technique to provide a symmetrical and stable cartilaginous framework. This has given good long-term results [Figure 10]a-c.
|Figure 8: (a) Before primary lip repair. (b) Long-term result - 15 years after primary unilateral cleft lip correction|
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|Figure 9: (a) Before primary unilateral cleft lip correction. (b) Long-term result- 11 years after the unilateral cleft lip correction|
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|Figure 10: (a) Before primary unilateral cleft lip correction. (b) Before preschool rhinoplasty. (c) 6 years after preschool rhinoplasty|
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We conclude that the components of the unilateral cleft lip nasal deformity have been well documented and that these can and should be addressed during the primary lip repair itself. Such intervention does not cause any growth disturbance in the long-term. There should no longer be a shadow of doubt about this. Similarly, repositioning of the nasal septum should also be taken up more enthusiastically by all cleft surgeons as this too has been shown to only produce better noses and it causes no harm even after 40 years of follow-up. The actual modality of intervention must of course be left to the virtuosity of the individual surgeon. We have described our protocol in detail.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Huffman WC, Lierle DM. Studies on the pathologic anatomy of the unilateral harelip nose. Plast Reconstr Surg (1946) 1949;4:225-34.
Sarnat BG. Normal and abnormal growth at the nasoseptovomeral region. Ann Otol Rhinol Laryngol 1991;100:508-15.
Millard DR Jr. Cleft Craft: Primary Nasal Correction, in the Unilateral Deformity. Boston, MA: Little Brown and Co.; 1976. p. 251-67.
McComb HK, Coghlan BA. Primary repair of the unilateral cleft lip nose: Completion of a longitudinal study. Cleft Palate Craniofac J 1996;33:23-30.
Byrd HS, El-Musa KA, Yazdani A. Definitive repair of the unilateral cleft lip nasal deformity. Plast Reconstr Surg 2007;120:1348-56.
Haddock NT, McRae MH, Cutting CB. Long-term effect of primary cleft rhinoplasty on secondary cleft rhinoplasty in patients with unilateral cleft lip-cleft palate. Plast Reconstr Surg 2012;129:740-8.
Cussons PD, Murison MS, Fernandez AE, Pigott RW. A panel based assessment of early versus no nasal correction of the cleft lip nose. Br J Plast Surg 1993;46:7-12.
Kim SK, Cha BH, Lee KC, Park JM. Primary correction of unilateral cleft lip nasal deformity in Asian patients: Anthropometric evaluation. Plast Reconstr Surg 2004;114:1373-81.
Adenwalla HS, Narayanan PV. Primary unilateral cleft lip repair. Indian J Plast Surg 2009;42 Suppl:S62-70.
Adenwalla HS, Narayanan PV. Unilateral cleft lip. In: Mani V, editor. Surgical Correction of Facial Deformities. New Delhi: Jaypee Brothers Medical Publishers (P) Ltd.; 2010. p. 144-52.
Guyuron B. MOC-PS(SM) CME article: Late cleft lip nasal deformity. Plast Reconstr Surg 2008;121:1-11.
Chang CS, Por YC, Liou EJ, Chang CJ, Chen PK, Noordhoff MS. Long-term comparison of four techniques for obtaining nasal symmetry in unilateral complete cleft lip patients: A single surgeon′s experience. Plast Reconstr Surg 2010;126:1276-84.
Thomas C, Mishra P. Open tip rhinoplasty along with the repair of cleft lip in cleft lip and palate cases. Br J Plast Surg 2000;53:1-6.
Trott JA, Mohan N. A preliminary report on open tip rhinoplasty at the time of lip repair in unilateral cleft lip and palate: The Alor Setar experience. Br J Plast Surg 1993;46:363-70.
Salyer KE, Genecov ER, Genecov DG. Unilateral cleft lip-nose repair: A 33-year experience. J Craniofac Surg 2003;14:549-58.
Mulliken JB, Martínez-Pérez D. The principle of rotation advancement for repair of unilateral complete cleft lip and nasal deformity: Technical variations and analysis of results. Plast Reconstr Surg 1999;104:1247-60.
Anderl H, Hussl H, Ninkovic M. Primary simultaneous lip and nose repair in the unilateral cleft lip and palate. Plast Reconstr Surg 2008;121:959-70.
Smahel Z, Müllerová Z, Nejedlý A. Effect of primary repositioning of the nasal septum on facial growth in unilateral cleft lip and palate. Cleft Palate Craniofac J 1999;36:310-3.
Ridgway EB, Andrews BT, Labrie RA, Padwa BL, Mulliken JB. Positioning the caudal septum during primary repair of unilateral cleft lip. J Craniofac Surg 2011;22:1219-24.
Gawrych E, Janiszewska-Olszowska J. Primary correction of nasal septal deformity in unilateral clefts during lip repair-a long-term study. Cleft Palate Craniofac J 2011;48:293-300.
Millard DR Jr. Cleft Craft: The Evolution of the Rotation and the Elevation of Flap c, in the Unilateral Deformity. Boston, MA: Little Brown and Co.; 1976. p. 217-28.
Jackson IT. Cleft lip and palate. In Mustarde JC, Jackson IT, editors. Plastic Surgery in Infancy and Childhood. 3 rd
ed. Churchill Livingstone. Edinburgh; 1988. p. 7.
Patel KB, Mulliken JB. Correction of the vestibular web during primary repair of unilateral cleft lip. Plast Reconstr Surg 2014;134:600e-7.
Noordhoff MS, Chen YR, Chen KT, Hong KF, Lo LJ. The surgical technique for the complete unilateral cleft lip-nasal deformity.Oper Tech Plast Reconstr Surg 1995;2:167-74.
Narayanan PV, Adenwalla HS. Unfavourable results in the repair of the cleft lip. Indian J Plast Surg 2013;46:171-82.
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