|STATE OF THE ART
|Year : 2018 | Volume
| Issue : 2 | Page : 62-67
Unilateral cleft lip: A review and current status
Anthony F Markus
Centre for Postgraduate Medical Research and Education, Bournemouth University, Poole, UK
|Date of Web Publication||26-Jul-2018|
Prof. Anthony F Markus
Centre for Postgraduate Medical Research and Education, Bournemouth University, Poole
Source of Support: None, Conflict of Interest: None
Primary surgery for cleft lip remains a challenge for all surgeons. This review seeks to identify the important underlying theories that have led to current practice, that will produce the best outcomes and to encourage collaboration in surgical practice, assessment and research in this area.
Keywords: Cleft lip, unilateral, surgery, review
|How to cite this article:|
Markus AF. Unilateral cleft lip: A review and current status. J Cleft Lip Palate Craniofac Anomal 2018;5:62-7
| Introduction|| |
The ideal approach to primary surgical correction of the unilateral cleft lip and nose remains elusive, principally because the fundamental surgical problem is often not clearly understood due to the complex anatomy of the deformity. Adequate and acceptable reconstruction can only be achieved when the surgeon fully appreciates both the normal and the pathologic spatial relationships and functions of the anatomical elements, particularly the involved muscular elements, and only then can facial balance be returned to as near as normal as possible.,,,,
With the exception of rare cases such as clefts associated, for example, with holoprosencephaly, in which there exists true tissue hypoplasia, the anomalies seen in labiomaxillary clefts result essentially from displacement, deformation, and functional hypotrophy of the underlying skeletal elements and the overlying soft tissues. This is particularly true of the maxillary segments, the dentoalveolar structures that they support and the nasal cartilages. It is also true of the nasolabial muscles which are all present on the cleft side but whose absence of normal insertions and the resultant dysfunctions are directly responsible for the apparent anomalies. The displacement, deformation, and functional hypotrophy also affect the mucocutaneous structures that border the cleft. Acknowledgment of these important facts has an important role in the selection of the most appropriate design of incision. The goal of primary surgery is not only to re-establish correct and normal nasolabial muscle insertions but also to restore the correct position of all other associated structures and in so doing promote normal or as near normal function, development, and growth of the face.
Veau  introduced the concept of “embryological” surgery, embodied somewhat later by Millard,, is given credence in histochemical studies of embryos between 16 and 22 weeks  which demonstrate that the deformities of the nasal cartilages, the premaxilla and maxilla and the associated musculature are recognisable at a very early stage in development. Compared to the non-cleft subject, these studies confirm the potential impact on growth of the mid-line structures. Delaire  stressed that it is essential to restore the connection between these anatomical units if facial growth in the cleft child is to proceed with any degree of normality.
| Importance of Muscle Reconstruction and Design of Incision|| |
The superficial muscles of the face are arranged in three interdependent groups forming three rings between the orbit and the chin. The first ring is formed on each side by the nasal constrictor muscles (or transverse nasal muscles) and the elevators of the alar region and the upper lip. It is important to remember that the constrictor muscles insert into the base of the nasal septum and incisive crest so that the lower part forms the floor of the nose and contributes to the nasal sill, playing an essential part in the sagittal and median orientation of the septum, vertical support of the lip, and proper function of the whole labionasal area. The elevator muscles (superficial and deep) are inserted into the anterior edge of the septum, just above the nasal spine. The nasolabial muscles form the first muscular ring and the upper half of the second ring of the anterior facial muscular chain. The upper half of the second ring, which corresponds to the upper lip, is formed by the upper part of the orbicularis muscle. This muscle is not, as so often represented on operative diagrams, formed solely by horizontal fibers, but it is made up of two different groups of muscles, joined by an intermediary group called “incisive”. These two groups are the oblique head of the nasolabial arch, which stretches from the columella and the sill of the nostril to the commissure, and the transverse head, which forms the fleshy component of the inferior part of the lip, at the level of the vermillion.
The correct position of the nasolabial (oblique) head determines the position of the free edge of the lip and the commissure, and consequently, function of the third muscular ring, the labio-mental. The advantage of meticulous reconstruction of the first nasolabial muscular ring is the better position and orientation of the nasal septum and the improvement of the position and shape of the alar and triangular cartilages. This improvement of the nose not only has an aesthetic advantage, but also it contributes to better nasal respiration and maxillary development.
Millard also stressed the importance of muscular reconstitution in the subnasal region and this is made easier by his skin markings and incisions. However, formal anatomic and morphological repair of the muscles of the nasal floor must be at an even deeper level. Since 1972, in all unilateral complete and incomplete clefts, Delaire systematically reconstructed not only the muscular sill but also the whole of the nasal floor before reconstituting the lip itself.
| Development of Skin Incisions for Unilateral Cleft Cheilorhinoplasty|| |
In unilateral cleft lips, as far as muscular surgery is concerned, the evolution of theories has led to considerable interest in the reconstitution of all the nasolabial muscles, in particular, those of the nasal floor and sill.
Despite the numerous eponymous techniques for closure of the unilateral cleft lip, all are derived from three basic approaches: The straight-line technique, the triangular flap technique, and the rotation-advancement technique. Each has its advantages and limitations so that no individual technique has gained universal acceptance. A surgeon's decision to use a certain technique is, therefore, often dependent on the extent of their training and exposure to the various techniques and their adoption of a particular approach. The outcome depends not only on choice of technique and training but also skill of the surgeon.
In comparing the various skin markings that have been used in the primary closure of unilateral clefts, there are two distinct periods. The first, up to 1949, was essentially straight line closures and involved lengthening of the lip on the cleft side, often at the expense of sacrificing, to some extent, the Cupid's Bow. In the second period, from 1950 onward, the unsightly deformity of the lip resulting from this sacrifice was avoided with the use of superior or inferior triangular flaps on the lateral side,, Because the resultant scar was difficult to correct at a later date, some techniques used both. During this period, very precise methods of measurement were developed in an attempt to obtain absolutely equal height of both sides of the cleft, for example, by Peiffer, who used a thin flexible metal wire and Malek who relied on very precise mathematical calculations.,
In 1957, Millard proposed a new method that he referred to as the rotation-advancement method  with rotation of the medial cleft element, increasing its length, and advancement of the lateral cleft element into the back cut near the columellar-labial junction. This technique has undergone numerous modifications since then, both by the Millard himself and many others but still remains the most commonly used in one form or another for unilateral cleft lip repair. Millard emphasized the importance of reestablishing symmetry of the lip and nose at the time of the initial lip repair. He did this by establishing normative measurements between certain anatomic landmarks and emphasized adherence to these measurements. However, these landmarks are often arbitrary and require considerable time and experience to master. Incorrect positioning of these landmarks leads to an asymmetric lip and an unsatisfactory esthetic result. Satisfactory results demand a comprehensive understanding of the underlying complex anatomy in this region, in particular, as Millard observed, around the philtrum.
Delaire initially used a combination of the markings and methods of calculation, but subsequently, after extensive anatomical studies, realized that the best way to obtain equal heights of both sides of the cleft lip was to correctly reconstitute all the labionasal muscles. After careful reconstruction and symmetrical functioning of the upper lip, the skin distends itself to provide the correct amount of covering, without any triangular flaps. Therefore, he did not make any measurements but instead very carefully differentiated between the different sectors of skin, according to whether it is of labial or nasal origin. These are then restored to their correct anatomo-functional position as determined by their underlying anatomical elements. This method has the great advantage of limiting the number and length of skin incisions to the existing limits between the skin of nasal origin and the skin of labial origin, not touching the skin of the philtrum nor the base of the columella and resulting only in a paramedian, more or less vertical scar and a small, horizontal scar just under the sill of the nostril.
Reddy et al. in a study of just under 800 patients compared two skin incisions used for primary closure of unilateral complete cleft lip (modified Millard and Peiffer) and sought to identify the most appropriate technique for clefts of varying morphology. No one technique of cleft lip repair consistently produced ideal esthetic and functional results. They concluded that certain preoperative anatomical features may lead the surgeon to choose one particular incision pattern in preference to another but found that one technique was essentially as good as the other, suggesting that the technique for closure of the underlying tissues is probably of more importance.
| Primary Rhinoplasty|| |
Primary correction of the nose in the unilateral cleft lip deformity has steadily received increased attention. Previously, the technique had fallen into disrepute with many cleft surgeons avoiding primary septal correction due to their fears that it may have an adverse effect on nasal, maxillary, and global facial development largely because of secondary scar contracture and stenosis., Primary correction of the nasal deformity associated with the unilateral cleft lip has today come to be accepted as the norm.,,,,,,,,,
It is well documented that the septum is deviated toward the noncleft side anteriorly. The anterior nasal spine, although seemingly displaced, remains a midline reference point. The approach to the septum advocated by Narayan and Adenwalla, for example, is through an incision over the mucoperichondrium on the cleft side, on the groove at the base of the septum. The mucoperichondrium is carefully stripped off the septal cartilage, followed by division of the septospinal ligament to expose the anterior border of the septal cartilage. They then make an incision at the junction of the cartilages with the underlying maxillary crest. The septal cartilage is freed from the vomer and the perpendicular plate of the ethmoid. A similar approach is described by others. Delaire, however, exposes the septum from both sides and sutures the transverse nasalis and depressor nasi septi to the base so as to overcome the curvature to the cleft side and return the septum to a central position under steady functional forces. Long-term outcome studies from many authors ,,, have confirmed that there is no deleterious effect in the long term to maxillary or nasal growth from septal cartilage repositioning. Reddy et al. compared outcomes with and without primary septoplasty using a standardized two-dimensional (2D) photographic analysis and found that nasal symmetry was better when septoplasty was carried out.
All of the nasal deformities are compounded by the skeletal base malposition on the cleft side. The debate continues whether there exists a primary hypoplasia of the cleft nasal elements with some feeling that a true hypoplasia exists, others not. However, comparative studies of the lateral crura of the alar cartilages in the cleft lip nasal deformity have shown that the cleft lateral crus is neither smaller nor histologically different from the noncleft cartilage. Most authors agree that there is displacement of the involved elements and that, in unilateral cases, the nasal deformity is bilateral so routinely operate on both alae.
There is general agreement that the nasal deformity in unilateral clefts is due to three major factors: imbalance of the facial musculature, hypoplasia of the skeletal base, and asymmetry of the skeletal base. Flattening of the cleft nostril rim is caused by the inferior position of the lower lateral cartilage. A significant factor in the causation of this deformity is the malposition and therefore abnormal action of the transverse nasalis. The transverse nasalis muscle is interspersed between the upper and lower lateral cartilages and its contraction increases the deformity. Together with the widening of the nostril margin and creation of an intranasal oblique ridge, the lateral splaying of the lower lateral cartilage widens the nose and depresses the dome on the cleft side, resulting in more techniques designed to overcome the problem, some shown to be more successful than others.
It is now recognized that primary repair of the unilateral cleft lip and nose needs to be performed in conjunction with muscular reconstruction of the lip. This muscle reconstruction is more complex than achieved by the traditional geometric arrangement of skin flaps. The evolution of functional muscular surgery for primary surgery of the unilateral cleft lip and nose has led to the development of a one-stage procedure with the aim of returning all the nasolabial muscles to as near normal a position as possible. Even if the initial result, at the end of operation, looks imperfect, the long-term results will always be better than in other techniques, in which greater importance is given to the immediate cosmetic appearance, at the expense of its future state.
| Periosteum|| |
There are differences of opinion regarding subperiosteal and supraperiosteal undermining in the belief that one may be detrimental to midfacial growth. However, wide periosteal undermining allows the advancement of the attached soft tissues, and therefore, not only coverage of an alveolar cleft but also mobilization of the muscles.
It is worth remembering that the superficial layer of periosteum (erroneously called the fibrous layer) is really the generating layer for the deep layer of periosteum (usually known as the cellular or osteogenic layer). It elaborates the bone from the progenitor cells and collagen, which had been supplied to it by the underlying generating layer. The simple undermining of the periosteum and raising it from the underlying bone is in itself no reason whatsoever to expect a reduction of either its generating activity or its osteogenic capacities. However, this can be altered if the external side of the fibrous layer of the periosteum is deprived of its normal muscular insertions and of its vascularization. Undermining at a supraperiosteal level causes much more disturbance to the physiology of the periosteum than when carried out at a subperiosteal level and indeed, the latter can even increase osteogenesis. In addition, variations in technique for repairing cleft lip and nasal deformities and the uniqueness of each cleft make comparison studies difficult. Surgical results are also influenced by other variables such as the use of presurgical orthodontic/orthopedic treatment, simultaneous gingivoperiosteoplasty, and specific timing of surgery, the particular procedure perhaps being only one of many important factors.
In summary, very wide periosteal undermining, extending from the nasal bridge to the top of the zygomatic bone and from the buccal sulcus to the infraorbital margin and posteriorly in the region of the maxillary junction with the small bones, has two main advantages. It facilitates mobilization of the large nasolabial muscles, with their periosteal insertions, and therefore, a satisfactory reconstruction of the normal muscular anatomy and physiology, and it promotes a bony bridge between the maxillary fragments, sometimes sufficient to prevent secondary bone grafting (which if necessary, can nevertheless still be performed).
| Current Considerations|| |
In the drive to improve outcomes, a number of seminal studies have been carried out. In Europe, a six-center study identified the best and worst outcomes, concluding that technique and number of patients treated were important factors in achieving best outcome. The Eurocleft study further identified a very large number of centers in Europe using almost as many different techniques. In the UK, long-term outcomes had been shown to be less than satisfactory; a national survey carried out as part of a review and reconfiguration of services, identified 81 surgeons at 57 centers treating an annual cleft birth rate of approximately 600. This led to recommendations that the number of centers and the number of surgeons is significantly reduced, along with the use of a single technique and proper training for all surgeons involved in cleft surgery, irrespective of their parent specialty. Not unsurprisingly, outcomes have improved significantly. Would this be possible in India? What is needed are long-term studies such as those spawned by the Clinical Standards Advisory Group report in the UK and based on audit of results and comparative long-term studies, for example, as from Joos.
Improved methodology for assessing outcome objectively has also progressed from results based on dental casts and photographs to more measured 2D and 3D studies.
Mosmuller et al. compared four different methods of assessment including 2D and 3D asymmetry and esthetic assessments and concluded that there is no widely accepted reliable scoring system for the assessment of nasolabial appearance in cleft patients. 3D facial images were acquired using 3D stereophotogrammetry in a study of outcomes following primary cheiloseptoplasty. After a 3D cephalometric analysis of the lip and nose was performed in both groups, linear and volumetric data were acquired in coming to their conclusions regarding facial appearance.
Among the various indices developed for measuring the results of treatment in patients born with unilateral cleft lip and palate (CLP), the GOSLON Yardstick index is very widely used to assess the efficacy of treatment and treatment outcomes. The results of a systematic review, however, showed a lack of evidence in the literature for the predictive validity of the index. Nevertheless, it remains a useful metric for comparative multicenter studies.
The cause of clefts is multifactorial, both genetic and environmental. Dixon et al. noted that, although there has been marked progress in identifying the triggers for syndromic CLP, the etiology of the more common nonsyndromic forms remains elusive. However, with a combination of epidemiology, careful phenotyping, genome-wide association studies, and analysis of animal models, several distinct genetic and environmental risk factors have been identified and confirmed for nonsyndromic CLP. These findings have advanced our understanding of developmental biology and created new opportunities for clinical translational research. India has tremendous potential to contribute by virtue of improving research expertise. The study of clefting in Andhra Pradesh by Reddy et al., for example, was an indicator of what can be achieved if records for such a diverse community are meticulous and comprehensive. The Millennium Development Goals 2015 and Sustainable Development Goals 2030 will focus efforts to improve service delivery. The contribution of major qualitative studies such as the Lancet Commission on Global Surgery  has identified the surgical needs and how best to resolve the unmet burden in low- and middle-income countries.
| Conclusion|| |
A review of this nature is of course very personal and impossible to be comprehensive. It aims perhaps to stimulate further reading. There will always be diverse thinking regarding the successful management of clef lip. The cleft community needs to rise above competing interests and pull together in providing best care, making every effort to advance their understanding of the problem and the challenges. A thorough understanding of the basis for surgery of the unilateral cleft is essential for progress in this field. The potential for truly cooperative working among the cleft community in India, whether through the national association or government agencies, is enormous. Of course, it is also essential that those employed by government fully understand the problems so that their input is meaningful.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Delaire J. The potential role of facial muscles in monitoring maxillary growth and morphogenesis. In: Carlson DS, McNamara JA, editors. Muscle Adaptation in the Craniofacial Region. Ann Arbor, MI: University of Michigan Press; 1978a. p. 157-80.
Markus AF, Delaire J, Smith WP. Facial balance in cleft lip and palate. I. Normal development and cleft palate. Br J Oral Maxillofac Surg 1992;30:287-95.
Markus AF, Delaire J, Smith WP. Facial balance in cleft lip and palate. II. Cleft lip and palate and secondary deformities. Br J Oral Maxillofac Surg 1992;30:296-304.
Delaire J. Theoretical principles and technique of functional closure of the lip and nasal aperture. J Maxillofac Surg 1978;6:109-16.
Joos U. The importance of muscular reconstruction in the treatment of cleft lip and palate. Scand J Plast Reconstr Surg Hand Surg 1987;21:109-13.
Veau, V. Bec-de-Lievre. Paris: Masson et Cie, 1938.
Millard DR Jr. Complete unilateral clefts of the lip. Plast Reconstr Surg Transplant Bull 1960;25:595-605.
Millard DR. The Unilateral Deformity. Cleft Craft – The Evolution of its Surgery. 1st
ed., Vol. 1. Boston: Little, Brown; 1976.
Precious DS. Unilateral Cleft Lip and Palate Chapter 3, Oral and Maxillofacial Clinics of North America 2000;12:399-420.
Delaire J, Chateau JP. How the nasal septum influences premaxillary and maxillary growth. Deductions from surgery of labiomaxillary clefts. Rev Stomatol Chir Maxillofac 1977;78:241-54.
Markus AF, Delaire J. Functional primary closure of cleft lip. Brit J Oral Maxillofac Surg 1993;11:261-91.
Randall P. A triangular flap operation for the primary repair of unilateral clefts of the lip. Plast Reconstr Surg Transplant Bull 1959;23:331-47.
Petit PP, Psaume, J. Le Traitement du Bec-de-Lievre. Paris: Masson et Cie, 1962.
Pfeifer G, Pirsig W, Wulff J, Wulff H. 1981 Lippen-Kiefer- Gaumenspalten – Chirurgische, otologische und sprachliche Behandlung; E. Reinhardt, Basel; 1981.
Malek R. Initial treatment of cleft lip and palate. Chir Pediatr 1983;24:256-67.
Malek R, Psaume J, Mousett MR, Trichet C. A new sequence and a new technique in complete Cleft lip and palate repair. In: Hotz, M, editor. Early Treatment of Cleft Lip and Palate. Toronto, Lewiston, NY, Bern, Stuttgart: Hans Huber Publication; 1986. p. 162-6.
Millard DR Jr. Refinements in rotation-advancement cleft lip technique. Plast Reconstr Surg 1964;33:26-38.
Reddy GS, Webb RM, Reddy RR, Reddy LV, Thomas P, Markus AF, et al.
Choice of incision for primary repair of unilateral complete cleft lip: A comparative study of outcomes in 796 patients. Plast Reconstr Surg 2008;121:932-40.
Berkley WT. Correction of nasal deformity in Cleft Lip and Palate Ed Grabb, WC, Boston: Little Brown & Co. 1971; p. 227-244.
Tan KK, Pigott RW. A morbidity review of children with complete unilateral cleft lip nose at 10 +/- 1 years of age. Br J Plast Surg 1993;46:1-6.
Delaire J. La cheilo-rhinoplastie primaire pour fente labio-maxillaire congenitale unilaterale. essai de schematisation d'une technique. Rev Stomat Chir Maxillofac 1975;76:193-216.
Delaire J. Intérét de la rhinoplasty primaire. Considérations techniques. Chir Pédiatr 1983;24:247-53.
McComb H. Primary correction of unilateral cleft lip nasal deformity: A 10-year review. Plast Reconstr Surg 1985;75:791-9.
McComb H. Anatomy of the unilateral and bilateral cleft lip nose. In: Bardach J, Morris HL, editors. Multidisciplinary Management of Cleft Lip and Palate. Philadelphia: W. B. Saunders; 1990. p. 144-9.
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.
Salyer KE. Primary correction of the unilateral cleft lip nose: A 15-year experience. Plast Reconstr Surg 1986;77:558-68.
Salyer KE, Bardach J. Unilateral Cleft Lip Repair in Surgical Techniques. In: Cleft Lip and Palate. St. Louis: Mosby; 1987.
Noordhoff M, Chen Y, Chen K, Hong K, Lo L. The surgical technique for the complete unilateral cleft lip-nasal deformity. Oper Tech Plast Reconstr Surg 1995;2:167-74.
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. [Full text]
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.
Joos U. Skeletal growth after muscular reconstruction for cleft lip, alveolus and palate. Br J Oral Maxillofac Surg 1995;33:139-44.
Markus AF, Precious DS. Effect of primary surgery for cleft lip and palate on mid-facial growth. Br J Oral Maxillofac Surg 1997;35:6-10.
Talmant JC. Nasal malformations associated with unilateral cleft lip. Accurate diagnosis and management. Scand J Plast Reconstr Surg Hand Surg 1993;27:183-91.
Gosla-Reddy S, Nagy K, Mommaerts MY, Reddy RR, Bronkhorst EM, Prasad R, et al.
Primary septoplasty in the repair of unilateral complete cleft lip and palate. Plast Reconstr Surg 2011;127:761-7.
Park BY, Lew DH, Lee YH. A comparative study of the lateral crus of alar cartilages in unilateral cleft lip nasal deformity. Plast Reconstr Surg 1998;101:915-20.
Delaire J, Precious DS, Gordeef A. The advantage of wide subperiosteal exposure in primary surgical correction of labial maxillary clefts. Scand J Plast Reconstr Surg Hand Surg 1988;22:147-51.
Shaw WC, Asher-McDade C, Brattström V, Dahl E, McWilliam J, Mølsted K, et al.
A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 1. Principles and study design. Cleft Palate Craniofac J 1992;29:393-7.
Clinical Standards Advisory Group. Cleft Lip and/or Palate. London: HMSO; 1998.
Mosmuller DG, Maal TJ, Prahl C, Tan RA, Mulder FJ, Schwirtz RM, et al.
Comparison of two- and three-dimensional assessment methods of nasolabial appearance in cleft lip and palate patients: Do the assessment methods measure the same outcome? J Craniomaxillofac Surg 2017;45:1220-6.
van Loon B, Reddy SG, van Heerbeek N, Ingels KJ, Maal TJ, Borstlap WA, et al.
3D stereophotogrammetric analysis of lip and nasal symmetry after primary cheiloseptoplasty in complete unilateral cleft lip repair. Rhinology 2011;49:546-53.
Buj-Acosta C, Paredes-Gallardo V, Montiel-Company JM, Albaladejo A, Bellot-Arcís C. Predictive validity of the GOSLON yardstick index in patients with unilateral cleft lip and palate: A systematic review. PLoS One 2017;12:e0178497.
Dixon MJ, Marazita ML, Beaty TH, Murray JC. Cleft lip and palate: Understanding genetic and environmental influences. Nat Rev Genet 2011;12:167-78.
Reddy SG, Reddy RR, Bronkhorst EM, Prasad R, Ettema AM, Sailer HF, et al.
Incidence of cleft lip and palate in the state of Andhra Pradesh, South India. Indian J Plast Surg 2010;43:184-9.
] [Full text]
Meara JG, Leather AJ, Hagander L, Alkire BC, Alonso N, Ameh EA, et al.
Global surgery 2030: Evidence and solutions for achieving health, welfare, and economic development. Lancet 2015;386:569-624.