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Cleft Lip Nose
C l e f t Li p N o s e Jonathan M. Sykes, MDa,*, Abel-Jan Tasman, MDb, Gustavo A. Suárez, MDc KEYWORDS Cleft lip rhinoplasty Secondary rhinoplasty Surgical techniques Open rhinoplasty technique Closed rhinoplasty technique
KEY POINTS A three-dimensional understanding of the anatomy of the cleft nose aids surgeons in selecting the proper technique for repair. Advantages of early surgical intervention include minimizing the deformity as the child grows and lessening asymmetries to allow optimal nasal growth. Analysis and performance of orthognathic surgery should be done before nasal surgery to optimize the overall result. Goals of the secondary rhinoplasty include relief of nasal obstruction, creation of symmetry and definition of the nasal base and tip, and management of nasal scarring and webbing. Septal reconstruction in the cleft nose is a key maneuver in cleft rhinoplasty.
The nasal deformity associated with congenital cleft lip is a complex defect that results in significant aesthetic and functional problems. The defect involves all tissue layers, including the bony platform of the nose, the inner nasal lining, the cartilaginous infrastructure, and the external skin. The extent of the deformity varies with the degree of lip abnormality; it may be unilateral or bilateral and subtle or complete.1 In many patients with congenital clefts, the secondary nasal deformity is minimal. However, the appearance of the nose in some patients with clefts is often the feature that is the most noticeable to the observer. The variability of the secondary cleft nasal deformity is related to the original deformity, scarring from previous surgeries on the lip and nose, and changes related to growth.2 In addition, many patients with clefts have significant nasal obstruction and functional problems.3
The goal of complete care of the cleft nasal deformity is to minimize functional problems and to maximize the appearance of the nose. This goal requires the surgeon to have an understanding of the pathophysiology of clefting, and the three-dimensional nature of the cleft nasal deformity. This article discusses the anatomy and pathophysiology of the cleft lip nasal deformity and the timing of the various repairs needed, and provides a philosophic understanding of a selection of techniques currently used to repair the cleft nasal deformity.
ANATOMY AND EMBRYOLOGY OF THE CLEFT NASAL DEFORMITY During normal development, the paired median nasal processes fuse to form the premaxilla, philtrum, columella, and nasal tip. The bilateral maxillary processes form the lateral aspects of the upper lip.4,5 Cleft lip deformities result from a failure of the fusion of the median nasal processes
Disclosures: Neither author has any financial or other disclosures with regard to this article. a Division of Facial Plastic and Reconstructive Surgery, Department of Otolaryngology, University of California Davis, 2521 Stockton Boulevard, Suite 6203, Sacramento, CA 95817, USA; b Rhinology and Facial Plastic Surgery, Department of Otolaryngology-Head and Neck Surgery, Cantonal Hospital, Rorschacher Strasse 95, St. Gallen 9000, Switzerland; c Department of Otolaryngology - Head and Neck Surgery, Bellvitge University Hospital, Feixa Llarga s/n, L’Hospitalet de Llobregat, Barcelona 08097, Spain * Corresponding author. 2521 Stockton Boulevard, Suite 6200, Sacramento, CA 95817. E-mail address: [email protected] Clin Plastic Surg 43 (2016) 223–235 http://dx.doi.org/10.1016/j.cps.2015.09.016 0094-1298/16/$ – see front matter Ó 2016 Elsevier Inc. All rights reserved.
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INTRODUCTION
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Sykes et al with the maxillary processes. Interruption of this embryonic process creates malformation of some or all of the upper lip, central alveolus, and primary palate. The extent of the associated cleft nasal deformity is related to the extent of the interruption of the normal developmental fusion process. The characteristic unilateral and bilateral cleft nasal deformities can occur along a spectrum of severity. In patients with incomplete cleft lips, these nasal deformities are less pronounced.6,7 Even though the nasal defects may be subtle, there is always a nasal abnormality associated with cleft lips.
Unilateral Cleft Lip Nose Deformity In patients with complete, unilateral cleft lip, the maxilla on the cleft side is deficient. Because of this, the alar base on the cleft side does not fuse in the midline and is positioned more posterior, lateral, and inferior than the alar base on the noncleft side.5 Consequently, the lateral crus of the lower lateral cartilage (LLC) on the cleft side is lengthened and the medial crura is shortened in relation to the LLC on the noncleft side. The septum is attached to the noncleft maxilla inferiorly, which causes the septum to be deviated to the noncleft side caudally, and bowing dorsally toward the cleft side. The attachment of the upper lateral cartilage to the LLC is affected by the change in position of the LLC, which effectively weakens the scroll region and causes compromise of the internal nasal valve. In addition, the abnormal insertion of the orbicularis oris muscle causes an asymmetric pull on the caudal septum. This pull also adds to the characteristic anterior septal deflection to the noncleft side (Fig. 1).
Bilateral Cleft Lip Nose Deformity In patients with complete, bilateral cleft lip, the maxilla is deficient bilaterally, which allows the prolabium to have unopposed anterior growth. The alar bases are displaced in a more posterior, lateral, and inferior position than occurs without clefting. The deficient skeletal base leads to longer lateral crura of the LLC bilaterally and short, splayed medial crura.8 This creates an underprojected, broad, and flat nasal tip. The columella is short because of the malposition of the prolabium and the shortening of the medial crura. The short columella makes the broad and snubbed nasal tip even more pronounced. Insertion of the septopremaxillary ligament is usually symmetric, thereby causing no alteration in the anterior septum/columella unit. Bilateral insertion of the orbicularis oris musculature into the alar base
Fig. 1. Basal view of the primary unilateral cleft lip deformity showing deviation of the columella toward the noncleft side, widening of the nasal floor, displacement of the alar base, and flattening of the LLC (stars represent the domes, lines depict caudal septum [midline] and lateral crus [most lateral]).
contributes to the widening of the nose and flattening of the LLC (Fig. 2).
TREATMENT Timing of the Cleft Nasal Repair The decision to perform early nasal surgery on children with clefts is based on several factors. These factors include the extent of the deformity and the potential scarring and impact of the procedure on nasal growth. Advantages of early surgical intervention include minimizing the deformity as the child grows, lessening asymmetries to allow optimal nasal growth, and creating favorable conditions for future surgery.
Fig. 2. Basal view of the bilateral cleft deformity. The columella is usually deviated toward the less complete side of the deformity.
Cleft Lip Nose Historically, controversy has existed as to whether primary tip rhinoplasty was a positive influence on the eventual appearance of the nose in patients with clefts. Major septal work and cartilaginous dissection has been thought to negatively affect nasal growth.9 However, no experimental or clinical studies have ever proved that minor manipulations (without resection) of the nasal tip or nasal base interfere with future nasal growth.10 For these reasons, most contemporary surgeons agree that the ideal repair of a cleft nasal deformity is performed in 2 stages. The first includes alteration in the nose at the time of lip repair (primary rhinoplasty), delaying a definitive repair until the patient has completed facial growth (secondary rhinoplasty). In female patients, secondary rhinoplasty is generally performed around 15 to 17 years of age, and in male patients at approximately 16 to 18 years of age.5
Presurgical Nasoalveolar Molding Presurgical nasoalveolar molding (PNAM) can be used in patients with wide or very asymmetric clefts to (1) reposition the malaligned alveolar segments, (2) narrow the cleft gap, (3) improve nasal tip symmetry in unilateral clefts, (4) elongate the
columella, and (5) expand the nasal soft tissues in bilateral clefts (Fig. 3). PNAM uses an intraoral alveolar molding device with nasal molding prongs. This technique requires a dedicated orthodontist and a motivated family that understands the treatment goals. If properly used, PNAM can lessen the tension across the lip wound and lessen the nasal deformity.11 Primary rhinoplasty can then be performed to improve nasal appearance and optimize nasal growth.
Primary Rhinoplasty The purpose of primary rhinoplasty is to close the anterior nasal floor, to relocate the displaced alar base, and to bring early symmetry to the nasal base and tip.5 This approach allows for both a functional and aesthetic improvement without jeopardizing nasal and facial growth. After the cleft lip incisions are made and the primary lip dissection is completed, the muscle and soft tissues of the alar base are separated from their maxillary attachments. The malpositioned alar base is freed by creating an internal alotomy at the anterior head of the inferior turbinate. If adequate soft tissue dissection of the alar base is performed, the cleft alar base can be
Fig. 3. (A) Initial placement of PNAM in a child with a right complete cleft lip and palate. (B) Four months after initial placement of PNAM. Note the narrowed cleft gap, narrowed lip gap, and improved overall symmetry of lip and nasal base. (C) Five months after surgery. (D) Five years after surgery.
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Sykes et al repositioned (during closure) in the optimal threedimensional position. The LLC on the cleft side is then dissected from its cutaneous attachments by creating a medial and a lateral tunnel just superficial to the LLCs. These subcutaneous tunnels are connected and allow the cleft LLC to be repositioned in a more symmetric fashion. Care is taken not to violate the vestibular skin, avoiding the complication of secondary adhesions and nostril stenosis. Primary cleft rhinoplasty begins with closure of the nasal floor and sill. This closure is first started with reapproximation of the musculature of the nasal base, which allows the cleft alar base to be reconstructed in a manner that mirrors the noncleft alar base. Closure of the nasal sill is performed with 5-0 chromic catgut sutures. It is important not to narrow the sill too much. A nasal base that is too wide is easy to narrow secondarily, whereas a stenotic sill is difficult to widen later. The other component of primary cleft rhinoplasty is to reposition the cleft nasal tip into a more projected, symmetric position. After the nasal sill is reestablished and the lip is repaired in a layered fashion, the cleft LLC is repositioned.12 This step is achieved with internal mattress or tieover external bolsters. The new dome has a lengthened medial crus and a shortened lateral crus. The resulting nasal tip is more symmetric, defined, and projected (Fig. 4).
Intermediate Rhinoplasty Intermediate rhinoplasty is defined as any nasal surgery performed between the time of initial lip repair and the time of definitive rhinoplasty when the patient reaches facial skeletal maturity. The use of intermediate rhinoplasty in patients with unilateral cleft nasal deformities has decreased as surgeons have become more adept at primary rhinoplasty. However, many patients have significant nasal deformities that have not been adequately repaired after their initial cleft lip procedures. In these cases, performing intermediate rhinoplasty minimizes the social stigmata associated with a more noticeable nasal deformity.3
Orthognathic Surgery In cleft patients with significant dentofacial deformities, surgery to correct the skeletal abnormalities and to optimize the dental occlusion is often necessary. Orthognathic surgery has the advantage of maximizing the skeletal profile, enhancing nasal appearance, and improving the malocclusion that commonly accompanies
Fig. 4. After completion of primary cleft lip rhinoplasty. Note that the suture securing the bolsters is tightened until a slight blanch is seen.
oral clefting. Therefore, analysis of the facial skeleton should be done before nasal surgery to determine whether skeletal repositioning is necessary. Studies have shown that it is the cleft palatoplasty that is responsible for restriction of maxillary growth in an anteroposterior and a transverse dimension. This condition often results in maxillary hypoplasia (with a resulting underjet) and transverse maxillary width restriction (with a resulting buccal crossbite)13 (Fig. 5A). Skeletal correction of the hypoplastic cleft maxilla requires advancement, and often widening, of the maxilla. In most instances, preoperative orthodontic treatment (usually 12–18 months) is necessary to align the dental arches. This treatment can minimize the occlusal deformity and often decreases the amount of movement necessary during orthognathic surgery (Fig. 5B). There are 2 basic approaches that can be used to correct the skeletal deformities associated with congenital clefting. The first approach is conventional orthognathic surgery, including maxillary advancement and widening with or without mandibular setback. The other approach is to perform a standard Le Fort I maxillary osteotomy and placement of distraction
Cleft Lip Nose
Fig. 5. (A) Lateral view of a patient with a cleft lip deformity. Note the class III malocclusion as a result of the maxillary hypoplasia (underjet). (B) After the use of distraction osteogenesis and secondary septorhinoplasty.
osteogenesis (DO) devices. If conventional orthognathic surgery is planned, upper and lower jaw surgery is often required for adequate skeletal correction.13 The maxilla is often scarred from prior palatal surgery, precluding a large advancement to adequately correct the underjet. Operating on both jaws allows the surgeon to maximize the skeletal relationship and correct the class III malocclusion. The DO approach uses either an internal distractor or a rigid external distractor (RED) device (Fig. 6). Use of DO allows the surgeon to progressively advance the maxilla. This method is often necessary to correct significant cleft jaw discrepancies. After completing the maxillary osteotomy and placing the distraction device, a waiting (latency) period is allowed before distraction begins. The maxilla is then distracted over period of 4 to 6 weeks before bony consolidation. DO grows both bone and soft tissue and improves the skeletal base that supports the nose (Fig. 7). Orthognathic surgery may delay the timing of the definitive septorhinoplasty, but has rewarding effects on the overall result.
Secondary (Definitive) Rhinoplasty Once the patient has reached facial skeletal maturity, definitive septorhinoplasty can be performed. Structural reconstruction of the cleft nose often requires cartilage grafting material from the rib or septum to achieve adequate support. The goals of the secondary rhinoplasty are the creation of symmetry and definition of the nasal base and tip, relief of nasal obstruction, and management of nasal scarring and webbing. The extent of the secondary nasal deformity varies according to several factors, including the extent of the original lip and nose defect, any surgery performed between birth and definitive rhinoplasty, and the specific nasal growth.
SURGICAL TECHNIQUES Approaches The approach to definitive cleft septorhinoplasty varies according to surgeon preference and the requirements of the reconstruction. It is important to clearly identify the key factors contributing to the
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Fig. 6. (A) After placement of the RED device. (B) Four weeks after placement of the RED device.
nasal deformity and select the most appropriate approach to achieve the surgical goals. These goals include septal reconstruction and treatment of the nasal tip, alar rim, alar base, columella, and nasal sill. In most instances, all these goals can be corrected by using either the external or endonasal approach. The preference of one of the senior authors (JMS) is to use an open or external approach. In most instances, the traditional inverted-V columellar incision is used. When there is significant lack of columellar soft tissue in unilateral clefting, the incision can be modified onto the cleft lip with a V-to-Y closure to increase columellar soft tissue (Fig. 8). Techniques have been described that recruit tissue from the lip repair with a sliding chondrocutaneous flap. This flap provides additional tissue to augment the vestibular lining and reduce the alar-columellar web, and, when combined with the open rhinoplasty, the chondrocutaneous flap can permit tip stability and lip refinement.14 The preference of the other senior author (AJT) is to use a closed or endonasal approach in secondary cleft rhinoplasty. Several incisions may be used to access the nasal infrastructure while obviating a transcolumellar incision. The
advantages of the endonasal approach include maintaining an intact skin–soft tissue envelope. When cartilaginous grafts are placed under an intact envelope, the surgeon is better able to immediately visualize the impact of the graft on the eventual nasal contour. Another potential advantage of the endonasal approach is maintenance of vascular supply to the skin, allowing increased tension when placing structural grafts. With the open approach, lengthening or projecting grafts can make eventual wound closure difficult. Multiple endonasal incisions (transfixion, infracartilaginous, and intercartilaginous) are often used to obtain adequate exposure to correct the deformity (Figs. 9 and 10).
Septal Reconstruction In the unilateral cleft deformity, the asymmetric unopposed pull of the orbicularis oris muscles and the deficient bony maxilla causes the septum to deviate to the noncleft side anteriorly. In the bilateral cleft lip deformity, the nasal septum is usually midline, being deviated caudally to the less involved side if asymmetry of the lip exists.15
Cleft Lip Nose
Fig. 7. (A) Preoperative lateral view of the patient shown in Fig. 6. (B) Lateral view after DO and secondary rhinoplasty.
Repair of the cleft septum is challenging and is the foundation of the rhinoplasty. Complete septal reconstruction requires adequate exposure and complete breakdown of the ligamentous attachments that contribute to the septal deviation. The septum can be approached by either an open or endonasal approach. If the external approach is used, the anterior septum is exposed
Fig. 8. Bilateral cleft lip rhinoplasty approached with a V incision to recruit skin from the lip into the columella.
by separating the ligaments that connect the 2 medial crura. If the endonasal approach is used, a complete transfixion incision is joined to bilateral intercartilaginous incisions to obtain the exposure necessary for reconstruction. In either case, adequate caudal and dorsal struts should be preserved while deviations in the cartilage and bone are corrected. To return the caudal septum to the midline, the surgeon often must remove a strip of cartilage inferiorly, allowing the septum to swing over the nasal spine (Fig. 11). This position can be maintained by suturing the cartilage to the spine with a 5-0 long-acting absorbable monofilament suture. If the septal support is not sufficient after resection of the deviated segments, reconstruction with cartilage grafts is needed to maintain adequate central segment support. Septal support can be achieved with a variety of grafting methods. The septum can be supported with a caudal septal extension graft (SEG) or a caudal batten graft (Figs. 12 and 13). These grafts can be fashioned from different autologous materials (septal cartilage, costal cartilage, ethmoid plate bone). The SEG can be sutured to the caudal
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Fig. 9. A 24-year-old patient with unilateral cleft lip nose deformity who underwent revision rhinoplasty: scar tissue resection, caudal septal reconstruction, LLC reconstruction. Preoperative (A–C). Early postoperative (D–F).
Cleft Lip Nose
Fig. 10. The surgical steps (A–G) for the patient shown in Fig. 9.
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Fig. 11. (A) Caudal septal deflection. (B) After swinging-door maneuver.
end of the existing septum in an end-to-end or end-to-side technique. The important concept is that, at the conclusion of grafting and repositioning of the septum, the caudal aspect of the septum is straight and well supported. Another graft that aids in support of the caudal and dorsal septum is the extended spreader graft. Spreader grafts are thin, long pieces of cartilage placed between the septum and the upper lateral cartilage and can improve the cross-sectional airway of the internal nasal valve. These
grafts can also help correct dorsal external deviations.16 In patients who have severe nasal septal deviation (in which resection, repositioning, and cartilage grafting are inadequate), an extracorporeal septoplasty may be required. This technique involves explantation of the septal cartilage, reshaping of this cartilage on the operative field (out of the patient), and reimplantation of the septum with fixation both caudally and dorsally.17 Often, this technique is combined with cartilage
Fig. 12. Caudal septal batten graft placement.
Fig. 13. Caudal SEG placement.
Cleft Lip Nose grafting for support and strength of the reimplanted septum. Regardless of the technique used, at the end of the septoplasty, the septum should be straight and well supported to maximize the cleft airway and to allow adequate tip resuspension.
Treatment of the Nasal Tip The nasal tip in patients with congenital clefting of the lip is poorly supported. In the unilateral deformity, the tip is asymmetric secondary to the short medial crus on the cleft side (Fig. 14). In the bilateral deformity, the tip is usually underprojected and the columella is short. Tip techniques are therefore designed to improve tip symmetry, definition, and projection. After the nasal septum is straightened and supported, the nasal tip can be resuspended on the septum to improve tip support and projection. This technique, termed the tongue-in-groove (TIG) technique, allows the tip to be projected, deprojected, lengthened, or shortened.18 In most clefts, the cleft side nasal tip needs projection and rotation, because the secondary nasal deformity usually has underprojection and hooding of the cleft tip. The TIG technique involves suture fixation of the medial crura of the LLCs to the caudal end of the nasal septum. Typically, the cleft side alar cartilage has to be advanced more than the noncleft side to improve the flattening of the cleft LLC and enhance overall tip symmetry. Another method used to improve support and projection is the columellar strut cartilage graft. The columellar strut graft is a sturdy piece of cartilage that is placed between the medial crura of the LLCs. The medial crura can be advanced on this graft and suture fixated to enhance projection and support. The LLC may also be vertically divided. On the cleft side, this maneuver is usually performed
Fig. 14. The classic finding in unilateral cleft nose deformity.
lateral to the existing dome. Division of the cartilages lateral to the dome increases the medial crural element and projection of the nasal tip. After division of the LLC is performed, the cartilages are reconstituted with suture. After the central tip segment is supported with one of these maneuvers, a cartilaginous tip graft can be added to camouflage irregularities and improve tip definition. These tip grafts are typically suture fixated with 6-0 permanent monofilament suture.
Treatment of the Cleft Alar Rim The cleft side lateral crus of the LLC is usually concave. This concavity is often associated with alar malposition, with the cartilage often being inferiorly displaced in relation to the position of the noncleft side LLC. The concavity of the alar rim often causes external nasal valve collapse and a functional nasal deformity. Treatment of the malpositioned alar rim can be accomplished with a variety of techniques, including cartilage grafting and/or suture repositioning. The cleft side lateral crus can be supported with (1) an alar rim graft, (2) an alar strut graft, (3) an alar turn-in flap, (4) excision and turnover of the entire lateral crus of the LLC with resuturing of the segment (the so-called flip-flop of the LLC)19 (Fig. 15). The alar rim graft is placed inferior to the existing cartilage in a nonanatomic position and helps to support and strengthen the LLC. The alar strut graft, also known as the lateral crural strut graft, is placed on the deep surface of the LLC, with the graft being sutured to the undersurface of the cartilage. The lateral extent of this graft is typically placed in a pocket at the pyriform aperture. Both of these grafts aid in supporting the alar rim, elevating the level of the alar rim and repositioning the rim laterally. The LLC turn-in flap uses the cephalic portion of the LLC. In most rhinoplasty procedures, the cephalic portion of the LLC is resected and discarded. In the turn-in flap technique, this previously resected cartilage is transposed on a pedicle and sutured to the undersurface of the remaining LLC in order to strengthen and support the LLC and to flatten the preexisting concavity. The flip-flop technique involves dissecting the lateral crura of the LLC off the underlying vestibular skin, excising this portion, turning it over, and resuturing it to the vestibular lining. This procedure changes the shape of the alar rim from concave to convex. All of these maneuvers are designed to strengthen and reposition the malformed alar rim cartilage. If there is still significant malposition of the cleft LLC after these maneuvers are
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Fig. 15. (A) Alar turn-in flap of the cephalic aspect of LLC. (B) Lateral crural strut graft. (C) Alar batten graft.
completed, the lower LLC can be sutured to the upper lateral cartilage or to the nasal septum to reposition the alar rim more superiorly.
Treatment of the Alar Base The alar base is often asymmetric and abnormal in shape. The cause of this deformity is related to the poorly supported skeletal nasal base, and to any surgery performed before the definitive rhinoplasty. In many cases, the insertion of the lateral alar rim (the alar-facial junction) is malpositioned as a result of the original cleft lip repair. A small malposition during the cleft lip repair can result in a larger disparity with growth. For this reason, the cleft alar-facial junction often needs to be repositioned to create alar base symmetry. Another common secondary deformity that is a result of the original cleft lip repair is a lack of complete closure of the sill of the nose. This defect occurs when the superior portion of the orbicularis oris muscle is incompletely closed. This defect causes a lack of symmetry of the alar base at the level of the nasal sill. This deformity is often obvious to the observer and creates a noticeable abnormal shape to the inferior aspect of the nostril (Fig. 16). Reconstruction of this deformity requires reopening of the superior aspect of the lip and realignment of the muscle. Although sill deformity
Fig. 16. Basal view of the unilateral cleft deformity showing the volume deficit in the nasal sill area along with the classic associated deformity.
Cleft Lip Nose is most commonly corrected with muscle dissection and closure, a dermal flap can be added to augment the base of the nose at the nasal sill. If the patient has a small deficiency at the nasal sill and does not want surgery, augmentation with injectable fillers can help with nasal base symmetry.
SUMMARY The nose in patients with congenital cleft malformations is often the facial feature that is most noticeable to the observer. The secondary nasal deformity is variable and is affected by the extent of the original cleft, growth, and by any intervening surgery to correct the lip or nose. Repair of secondary cleft nasal deformities is challenging. Successful reconstruction requires an understanding of the pathologic anatomy, adequate exposure to perform techniques, and attention to structural grafting to overcome scarring and provide support. Often, graft material from the septum, rib, and/or ear is required. Attention must be paid to both function and appearance.
REFERENCES 1. Sykes JM, Senders CW. Pathologic anatomy of cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic surgery. New York: Thieme Medical Publishers; 1993. p. 57–71. 2. Sykes JM, Senders CW. Surgical management of the difficult nose. Oper Tech Otolaryngol Head Neck Surg 1990;1(4):219–24. 3. Shih CW, Sykes JM. Correction of the cleft-lip nasal deformity. Facial Plast Surg 2002;18(4):253–62. 4. Capone R, Sykes J. Evaluation and management of cleft lip and palate disorders. In: Papel ID, editor. Facial plastic and reconstructive surgery. 3rd edition. Stuttgart (NY): Thieme; 2009. p. 1059–60. 5. Sykes JM, Jang YJ. Cleft lip rhinoplasty. Facial Plast Surg Clin North Am 2009;17(1):133–44, vii. 6. Blair VP. Nasal deformities associated with congenital cleft of the lip. JAMA 1925;84:185–7.
7. Huffman WC, Lierle DM. Studies on the pathologic anatomy of the unilateral hare-lip nose. Plast Reconstr Surg 1949;4:225–34. 8. Coleman J, Sykes J. Cleft lip rhinoplasty. In: Papel ID, editor. Facial plastic and reconstructive surgery. 3rd edition. Stuttgart (NY): Thieme; 2009. p. 1082. 9. Sarnat BG, Wexler MR. Growth of the face and jaws after resection of the septal cartilage in the rabbit. Am J Anat 1966;118:755–67. 10. McComb HK, Coghlan BA. Primary repair of the unilateral cleft lip nose: completion of a longitudinal study. Cleft Palate Craniofac J 1996;33:23–31. 11. Grayson BH, Maull D. Nasoalveolar molding for infants born with clefts of the lip, alveolus, and palate. Clin Plast Surg 2004;31(2):149–58, vii. 12. Mulliken JB, Martinez-Perez D. The principle of rotation advancement for the repair of unilateral complete cleft lip and nasal deformity: technical variations and analysis of results. Plast Reconstr Surg 1999;104:1247–9. 13. Sykes J, Rotas N. 3rd edition. Orthognathic surgery in cleft lip and palate patient. FPS Cln NA, vol. 4. Philadelphia: WB Saunders; 1996. p. 351–75. 14. Wang TD, Madorsky SJ. Secondary rhinoplasty in nasal deformity associated with the unilateral cleft lip. Arch Facial Plast Surg 1999;1:40–5. 15. Crockett D, Bumstead R. Nasal airway, otologic, and audiologic problems associated with cleft lip and palate. In: Bardach J, Morris HL, editors. Multidisciplinary management of cleft lip and palate. Philadelphia: WB Saunders; 1990. 16. Haack J, Papel ID. Caudal septal deviation. Otolaryngol Clin North Am 2009;42(3):427–36. 17. Most SP. Anterior septal reconstruction: outcomes after a modified extracorporeal septoplasty technique. Arch Facial Plast Surg 2006;8(3):202–7. 18. Kridel RW, Scott BA, Foda HM. The tongue-in-groove technique in septorhinoplasty. A 10-year experience. Arch Facial Plast Surg 1999;1(4):246–56. 19. Murakami CS, Barrera JE, Most SP. Preserving structural integrity of the alar cartilage in aesthetic rhinoplasty using a cephalic turn-in flap. Arch Facial Plast Surg 2009;11(2):126–8.
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KEY POINTS A three-dimensional understanding of the anatomy of the cleft nose aids surgeons in selecting the proper technique for repair. Advantages of early surgical intervention include minimizing the deformity as the child grows and lessening asymmetries to allow optimal nasal growth. Analysis and performance of orthognathic surgery should be done before nasal surgery to optimize the overall result. Goals of the secondary rhinoplasty include relief of nasal obstruction, creation of symmetry and definition of the nasal base and tip, and management of nasal scarring and webbing. Septal reconstruction in the cleft nose is a key maneuver in cleft rhinoplasty.
The nasal deformity associated with congenital cleft lip is a complex defect that results in significant aesthetic and functional problems. The defect involves all tissue layers, including the bony platform of the nose, the inner nasal lining, the cartilaginous infrastructure, and the external skin. The extent of the deformity varies with the degree of lip abnormality; it may be unilateral or bilateral and subtle or complete.1 In many patients with congenital clefts, the secondary nasal deformity is minimal. However, the appearance of the nose in some patients with clefts is often the feature that is the most noticeable to the observer. The variability of the secondary cleft nasal deformity is related to the original deformity, scarring from previous surgeries on the lip and nose, and changes related to growth.2 In addition, many patients with clefts have significant nasal obstruction and functional problems.3
The goal of complete care of the cleft nasal deformity is to minimize functional problems and to maximize the appearance of the nose. This goal requires the surgeon to have an understanding of the pathophysiology of clefting, and the three-dimensional nature of the cleft nasal deformity. This article discusses the anatomy and pathophysiology of the cleft lip nasal deformity and the timing of the various repairs needed, and provides a philosophic understanding of a selection of techniques currently used to repair the cleft nasal deformity.
ANATOMY AND EMBRYOLOGY OF THE CLEFT NASAL DEFORMITY During normal development, the paired median nasal processes fuse to form the premaxilla, philtrum, columella, and nasal tip. The bilateral maxillary processes form the lateral aspects of the upper lip.4,5 Cleft lip deformities result from a failure of the fusion of the median nasal processes
Disclosures: Neither author has any financial or other disclosures with regard to this article. a Division of Facial Plastic and Reconstructive Surgery, Department of Otolaryngology, University of California Davis, 2521 Stockton Boulevard, Suite 6203, Sacramento, CA 95817, USA; b Rhinology and Facial Plastic Surgery, Department of Otolaryngology-Head and Neck Surgery, Cantonal Hospital, Rorschacher Strasse 95, St. Gallen 9000, Switzerland; c Department of Otolaryngology - Head and Neck Surgery, Bellvitge University Hospital, Feixa Llarga s/n, L’Hospitalet de Llobregat, Barcelona 08097, Spain * Corresponding author. 2521 Stockton Boulevard, Suite 6200, Sacramento, CA 95817. E-mail address: [email protected] Clin Plastic Surg 43 (2016) 223–235 http://dx.doi.org/10.1016/j.cps.2015.09.016 0094-1298/16/$ – see front matter Ó 2016 Elsevier Inc. All rights reserved.
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INTRODUCTION
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Sykes et al with the maxillary processes. Interruption of this embryonic process creates malformation of some or all of the upper lip, central alveolus, and primary palate. The extent of the associated cleft nasal deformity is related to the extent of the interruption of the normal developmental fusion process. The characteristic unilateral and bilateral cleft nasal deformities can occur along a spectrum of severity. In patients with incomplete cleft lips, these nasal deformities are less pronounced.6,7 Even though the nasal defects may be subtle, there is always a nasal abnormality associated with cleft lips.
Unilateral Cleft Lip Nose Deformity In patients with complete, unilateral cleft lip, the maxilla on the cleft side is deficient. Because of this, the alar base on the cleft side does not fuse in the midline and is positioned more posterior, lateral, and inferior than the alar base on the noncleft side.5 Consequently, the lateral crus of the lower lateral cartilage (LLC) on the cleft side is lengthened and the medial crura is shortened in relation to the LLC on the noncleft side. The septum is attached to the noncleft maxilla inferiorly, which causes the septum to be deviated to the noncleft side caudally, and bowing dorsally toward the cleft side. The attachment of the upper lateral cartilage to the LLC is affected by the change in position of the LLC, which effectively weakens the scroll region and causes compromise of the internal nasal valve. In addition, the abnormal insertion of the orbicularis oris muscle causes an asymmetric pull on the caudal septum. This pull also adds to the characteristic anterior septal deflection to the noncleft side (Fig. 1).
Bilateral Cleft Lip Nose Deformity In patients with complete, bilateral cleft lip, the maxilla is deficient bilaterally, which allows the prolabium to have unopposed anterior growth. The alar bases are displaced in a more posterior, lateral, and inferior position than occurs without clefting. The deficient skeletal base leads to longer lateral crura of the LLC bilaterally and short, splayed medial crura.8 This creates an underprojected, broad, and flat nasal tip. The columella is short because of the malposition of the prolabium and the shortening of the medial crura. The short columella makes the broad and snubbed nasal tip even more pronounced. Insertion of the septopremaxillary ligament is usually symmetric, thereby causing no alteration in the anterior septum/columella unit. Bilateral insertion of the orbicularis oris musculature into the alar base
Fig. 1. Basal view of the primary unilateral cleft lip deformity showing deviation of the columella toward the noncleft side, widening of the nasal floor, displacement of the alar base, and flattening of the LLC (stars represent the domes, lines depict caudal septum [midline] and lateral crus [most lateral]).
contributes to the widening of the nose and flattening of the LLC (Fig. 2).
TREATMENT Timing of the Cleft Nasal Repair The decision to perform early nasal surgery on children with clefts is based on several factors. These factors include the extent of the deformity and the potential scarring and impact of the procedure on nasal growth. Advantages of early surgical intervention include minimizing the deformity as the child grows, lessening asymmetries to allow optimal nasal growth, and creating favorable conditions for future surgery.
Fig. 2. Basal view of the bilateral cleft deformity. The columella is usually deviated toward the less complete side of the deformity.
Cleft Lip Nose Historically, controversy has existed as to whether primary tip rhinoplasty was a positive influence on the eventual appearance of the nose in patients with clefts. Major septal work and cartilaginous dissection has been thought to negatively affect nasal growth.9 However, no experimental or clinical studies have ever proved that minor manipulations (without resection) of the nasal tip or nasal base interfere with future nasal growth.10 For these reasons, most contemporary surgeons agree that the ideal repair of a cleft nasal deformity is performed in 2 stages. The first includes alteration in the nose at the time of lip repair (primary rhinoplasty), delaying a definitive repair until the patient has completed facial growth (secondary rhinoplasty). In female patients, secondary rhinoplasty is generally performed around 15 to 17 years of age, and in male patients at approximately 16 to 18 years of age.5
Presurgical Nasoalveolar Molding Presurgical nasoalveolar molding (PNAM) can be used in patients with wide or very asymmetric clefts to (1) reposition the malaligned alveolar segments, (2) narrow the cleft gap, (3) improve nasal tip symmetry in unilateral clefts, (4) elongate the
columella, and (5) expand the nasal soft tissues in bilateral clefts (Fig. 3). PNAM uses an intraoral alveolar molding device with nasal molding prongs. This technique requires a dedicated orthodontist and a motivated family that understands the treatment goals. If properly used, PNAM can lessen the tension across the lip wound and lessen the nasal deformity.11 Primary rhinoplasty can then be performed to improve nasal appearance and optimize nasal growth.
Primary Rhinoplasty The purpose of primary rhinoplasty is to close the anterior nasal floor, to relocate the displaced alar base, and to bring early symmetry to the nasal base and tip.5 This approach allows for both a functional and aesthetic improvement without jeopardizing nasal and facial growth. After the cleft lip incisions are made and the primary lip dissection is completed, the muscle and soft tissues of the alar base are separated from their maxillary attachments. The malpositioned alar base is freed by creating an internal alotomy at the anterior head of the inferior turbinate. If adequate soft tissue dissection of the alar base is performed, the cleft alar base can be
Fig. 3. (A) Initial placement of PNAM in a child with a right complete cleft lip and palate. (B) Four months after initial placement of PNAM. Note the narrowed cleft gap, narrowed lip gap, and improved overall symmetry of lip and nasal base. (C) Five months after surgery. (D) Five years after surgery.
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Sykes et al repositioned (during closure) in the optimal threedimensional position. The LLC on the cleft side is then dissected from its cutaneous attachments by creating a medial and a lateral tunnel just superficial to the LLCs. These subcutaneous tunnels are connected and allow the cleft LLC to be repositioned in a more symmetric fashion. Care is taken not to violate the vestibular skin, avoiding the complication of secondary adhesions and nostril stenosis. Primary cleft rhinoplasty begins with closure of the nasal floor and sill. This closure is first started with reapproximation of the musculature of the nasal base, which allows the cleft alar base to be reconstructed in a manner that mirrors the noncleft alar base. Closure of the nasal sill is performed with 5-0 chromic catgut sutures. It is important not to narrow the sill too much. A nasal base that is too wide is easy to narrow secondarily, whereas a stenotic sill is difficult to widen later. The other component of primary cleft rhinoplasty is to reposition the cleft nasal tip into a more projected, symmetric position. After the nasal sill is reestablished and the lip is repaired in a layered fashion, the cleft LLC is repositioned.12 This step is achieved with internal mattress or tieover external bolsters. The new dome has a lengthened medial crus and a shortened lateral crus. The resulting nasal tip is more symmetric, defined, and projected (Fig. 4).
Intermediate Rhinoplasty Intermediate rhinoplasty is defined as any nasal surgery performed between the time of initial lip repair and the time of definitive rhinoplasty when the patient reaches facial skeletal maturity. The use of intermediate rhinoplasty in patients with unilateral cleft nasal deformities has decreased as surgeons have become more adept at primary rhinoplasty. However, many patients have significant nasal deformities that have not been adequately repaired after their initial cleft lip procedures. In these cases, performing intermediate rhinoplasty minimizes the social stigmata associated with a more noticeable nasal deformity.3
Orthognathic Surgery In cleft patients with significant dentofacial deformities, surgery to correct the skeletal abnormalities and to optimize the dental occlusion is often necessary. Orthognathic surgery has the advantage of maximizing the skeletal profile, enhancing nasal appearance, and improving the malocclusion that commonly accompanies
Fig. 4. After completion of primary cleft lip rhinoplasty. Note that the suture securing the bolsters is tightened until a slight blanch is seen.
oral clefting. Therefore, analysis of the facial skeleton should be done before nasal surgery to determine whether skeletal repositioning is necessary. Studies have shown that it is the cleft palatoplasty that is responsible for restriction of maxillary growth in an anteroposterior and a transverse dimension. This condition often results in maxillary hypoplasia (with a resulting underjet) and transverse maxillary width restriction (with a resulting buccal crossbite)13 (Fig. 5A). Skeletal correction of the hypoplastic cleft maxilla requires advancement, and often widening, of the maxilla. In most instances, preoperative orthodontic treatment (usually 12–18 months) is necessary to align the dental arches. This treatment can minimize the occlusal deformity and often decreases the amount of movement necessary during orthognathic surgery (Fig. 5B). There are 2 basic approaches that can be used to correct the skeletal deformities associated with congenital clefting. The first approach is conventional orthognathic surgery, including maxillary advancement and widening with or without mandibular setback. The other approach is to perform a standard Le Fort I maxillary osteotomy and placement of distraction
Cleft Lip Nose
Fig. 5. (A) Lateral view of a patient with a cleft lip deformity. Note the class III malocclusion as a result of the maxillary hypoplasia (underjet). (B) After the use of distraction osteogenesis and secondary septorhinoplasty.
osteogenesis (DO) devices. If conventional orthognathic surgery is planned, upper and lower jaw surgery is often required for adequate skeletal correction.13 The maxilla is often scarred from prior palatal surgery, precluding a large advancement to adequately correct the underjet. Operating on both jaws allows the surgeon to maximize the skeletal relationship and correct the class III malocclusion. The DO approach uses either an internal distractor or a rigid external distractor (RED) device (Fig. 6). Use of DO allows the surgeon to progressively advance the maxilla. This method is often necessary to correct significant cleft jaw discrepancies. After completing the maxillary osteotomy and placing the distraction device, a waiting (latency) period is allowed before distraction begins. The maxilla is then distracted over period of 4 to 6 weeks before bony consolidation. DO grows both bone and soft tissue and improves the skeletal base that supports the nose (Fig. 7). Orthognathic surgery may delay the timing of the definitive septorhinoplasty, but has rewarding effects on the overall result.
Secondary (Definitive) Rhinoplasty Once the patient has reached facial skeletal maturity, definitive septorhinoplasty can be performed. Structural reconstruction of the cleft nose often requires cartilage grafting material from the rib or septum to achieve adequate support. The goals of the secondary rhinoplasty are the creation of symmetry and definition of the nasal base and tip, relief of nasal obstruction, and management of nasal scarring and webbing. The extent of the secondary nasal deformity varies according to several factors, including the extent of the original lip and nose defect, any surgery performed between birth and definitive rhinoplasty, and the specific nasal growth.
SURGICAL TECHNIQUES Approaches The approach to definitive cleft septorhinoplasty varies according to surgeon preference and the requirements of the reconstruction. It is important to clearly identify the key factors contributing to the
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Fig. 6. (A) After placement of the RED device. (B) Four weeks after placement of the RED device.
nasal deformity and select the most appropriate approach to achieve the surgical goals. These goals include septal reconstruction and treatment of the nasal tip, alar rim, alar base, columella, and nasal sill. In most instances, all these goals can be corrected by using either the external or endonasal approach. The preference of one of the senior authors (JMS) is to use an open or external approach. In most instances, the traditional inverted-V columellar incision is used. When there is significant lack of columellar soft tissue in unilateral clefting, the incision can be modified onto the cleft lip with a V-to-Y closure to increase columellar soft tissue (Fig. 8). Techniques have been described that recruit tissue from the lip repair with a sliding chondrocutaneous flap. This flap provides additional tissue to augment the vestibular lining and reduce the alar-columellar web, and, when combined with the open rhinoplasty, the chondrocutaneous flap can permit tip stability and lip refinement.14 The preference of the other senior author (AJT) is to use a closed or endonasal approach in secondary cleft rhinoplasty. Several incisions may be used to access the nasal infrastructure while obviating a transcolumellar incision. The
advantages of the endonasal approach include maintaining an intact skin–soft tissue envelope. When cartilaginous grafts are placed under an intact envelope, the surgeon is better able to immediately visualize the impact of the graft on the eventual nasal contour. Another potential advantage of the endonasal approach is maintenance of vascular supply to the skin, allowing increased tension when placing structural grafts. With the open approach, lengthening or projecting grafts can make eventual wound closure difficult. Multiple endonasal incisions (transfixion, infracartilaginous, and intercartilaginous) are often used to obtain adequate exposure to correct the deformity (Figs. 9 and 10).
Septal Reconstruction In the unilateral cleft deformity, the asymmetric unopposed pull of the orbicularis oris muscles and the deficient bony maxilla causes the septum to deviate to the noncleft side anteriorly. In the bilateral cleft lip deformity, the nasal septum is usually midline, being deviated caudally to the less involved side if asymmetry of the lip exists.15
Cleft Lip Nose
Fig. 7. (A) Preoperative lateral view of the patient shown in Fig. 6. (B) Lateral view after DO and secondary rhinoplasty.
Repair of the cleft septum is challenging and is the foundation of the rhinoplasty. Complete septal reconstruction requires adequate exposure and complete breakdown of the ligamentous attachments that contribute to the septal deviation. The septum can be approached by either an open or endonasal approach. If the external approach is used, the anterior septum is exposed
Fig. 8. Bilateral cleft lip rhinoplasty approached with a V incision to recruit skin from the lip into the columella.
by separating the ligaments that connect the 2 medial crura. If the endonasal approach is used, a complete transfixion incision is joined to bilateral intercartilaginous incisions to obtain the exposure necessary for reconstruction. In either case, adequate caudal and dorsal struts should be preserved while deviations in the cartilage and bone are corrected. To return the caudal septum to the midline, the surgeon often must remove a strip of cartilage inferiorly, allowing the septum to swing over the nasal spine (Fig. 11). This position can be maintained by suturing the cartilage to the spine with a 5-0 long-acting absorbable monofilament suture. If the septal support is not sufficient after resection of the deviated segments, reconstruction with cartilage grafts is needed to maintain adequate central segment support. Septal support can be achieved with a variety of grafting methods. The septum can be supported with a caudal septal extension graft (SEG) or a caudal batten graft (Figs. 12 and 13). These grafts can be fashioned from different autologous materials (septal cartilage, costal cartilage, ethmoid plate bone). The SEG can be sutured to the caudal
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Fig. 9. A 24-year-old patient with unilateral cleft lip nose deformity who underwent revision rhinoplasty: scar tissue resection, caudal septal reconstruction, LLC reconstruction. Preoperative (A–C). Early postoperative (D–F).
Cleft Lip Nose
Fig. 10. The surgical steps (A–G) for the patient shown in Fig. 9.
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Fig. 11. (A) Caudal septal deflection. (B) After swinging-door maneuver.
end of the existing septum in an end-to-end or end-to-side technique. The important concept is that, at the conclusion of grafting and repositioning of the septum, the caudal aspect of the septum is straight and well supported. Another graft that aids in support of the caudal and dorsal septum is the extended spreader graft. Spreader grafts are thin, long pieces of cartilage placed between the septum and the upper lateral cartilage and can improve the cross-sectional airway of the internal nasal valve. These
grafts can also help correct dorsal external deviations.16 In patients who have severe nasal septal deviation (in which resection, repositioning, and cartilage grafting are inadequate), an extracorporeal septoplasty may be required. This technique involves explantation of the septal cartilage, reshaping of this cartilage on the operative field (out of the patient), and reimplantation of the septum with fixation both caudally and dorsally.17 Often, this technique is combined with cartilage
Fig. 12. Caudal septal batten graft placement.
Fig. 13. Caudal SEG placement.
Cleft Lip Nose grafting for support and strength of the reimplanted septum. Regardless of the technique used, at the end of the septoplasty, the septum should be straight and well supported to maximize the cleft airway and to allow adequate tip resuspension.
Treatment of the Nasal Tip The nasal tip in patients with congenital clefting of the lip is poorly supported. In the unilateral deformity, the tip is asymmetric secondary to the short medial crus on the cleft side (Fig. 14). In the bilateral deformity, the tip is usually underprojected and the columella is short. Tip techniques are therefore designed to improve tip symmetry, definition, and projection. After the nasal septum is straightened and supported, the nasal tip can be resuspended on the septum to improve tip support and projection. This technique, termed the tongue-in-groove (TIG) technique, allows the tip to be projected, deprojected, lengthened, or shortened.18 In most clefts, the cleft side nasal tip needs projection and rotation, because the secondary nasal deformity usually has underprojection and hooding of the cleft tip. The TIG technique involves suture fixation of the medial crura of the LLCs to the caudal end of the nasal septum. Typically, the cleft side alar cartilage has to be advanced more than the noncleft side to improve the flattening of the cleft LLC and enhance overall tip symmetry. Another method used to improve support and projection is the columellar strut cartilage graft. The columellar strut graft is a sturdy piece of cartilage that is placed between the medial crura of the LLCs. The medial crura can be advanced on this graft and suture fixated to enhance projection and support. The LLC may also be vertically divided. On the cleft side, this maneuver is usually performed
Fig. 14. The classic finding in unilateral cleft nose deformity.
lateral to the existing dome. Division of the cartilages lateral to the dome increases the medial crural element and projection of the nasal tip. After division of the LLC is performed, the cartilages are reconstituted with suture. After the central tip segment is supported with one of these maneuvers, a cartilaginous tip graft can be added to camouflage irregularities and improve tip definition. These tip grafts are typically suture fixated with 6-0 permanent monofilament suture.
Treatment of the Cleft Alar Rim The cleft side lateral crus of the LLC is usually concave. This concavity is often associated with alar malposition, with the cartilage often being inferiorly displaced in relation to the position of the noncleft side LLC. The concavity of the alar rim often causes external nasal valve collapse and a functional nasal deformity. Treatment of the malpositioned alar rim can be accomplished with a variety of techniques, including cartilage grafting and/or suture repositioning. The cleft side lateral crus can be supported with (1) an alar rim graft, (2) an alar strut graft, (3) an alar turn-in flap, (4) excision and turnover of the entire lateral crus of the LLC with resuturing of the segment (the so-called flip-flop of the LLC)19 (Fig. 15). The alar rim graft is placed inferior to the existing cartilage in a nonanatomic position and helps to support and strengthen the LLC. The alar strut graft, also known as the lateral crural strut graft, is placed on the deep surface of the LLC, with the graft being sutured to the undersurface of the cartilage. The lateral extent of this graft is typically placed in a pocket at the pyriform aperture. Both of these grafts aid in supporting the alar rim, elevating the level of the alar rim and repositioning the rim laterally. The LLC turn-in flap uses the cephalic portion of the LLC. In most rhinoplasty procedures, the cephalic portion of the LLC is resected and discarded. In the turn-in flap technique, this previously resected cartilage is transposed on a pedicle and sutured to the undersurface of the remaining LLC in order to strengthen and support the LLC and to flatten the preexisting concavity. The flip-flop technique involves dissecting the lateral crura of the LLC off the underlying vestibular skin, excising this portion, turning it over, and resuturing it to the vestibular lining. This procedure changes the shape of the alar rim from concave to convex. All of these maneuvers are designed to strengthen and reposition the malformed alar rim cartilage. If there is still significant malposition of the cleft LLC after these maneuvers are
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Fig. 15. (A) Alar turn-in flap of the cephalic aspect of LLC. (B) Lateral crural strut graft. (C) Alar batten graft.
completed, the lower LLC can be sutured to the upper lateral cartilage or to the nasal septum to reposition the alar rim more superiorly.
Treatment of the Alar Base The alar base is often asymmetric and abnormal in shape. The cause of this deformity is related to the poorly supported skeletal nasal base, and to any surgery performed before the definitive rhinoplasty. In many cases, the insertion of the lateral alar rim (the alar-facial junction) is malpositioned as a result of the original cleft lip repair. A small malposition during the cleft lip repair can result in a larger disparity with growth. For this reason, the cleft alar-facial junction often needs to be repositioned to create alar base symmetry. Another common secondary deformity that is a result of the original cleft lip repair is a lack of complete closure of the sill of the nose. This defect occurs when the superior portion of the orbicularis oris muscle is incompletely closed. This defect causes a lack of symmetry of the alar base at the level of the nasal sill. This deformity is often obvious to the observer and creates a noticeable abnormal shape to the inferior aspect of the nostril (Fig. 16). Reconstruction of this deformity requires reopening of the superior aspect of the lip and realignment of the muscle. Although sill deformity
Fig. 16. Basal view of the unilateral cleft deformity showing the volume deficit in the nasal sill area along with the classic associated deformity.
Cleft Lip Nose is most commonly corrected with muscle dissection and closure, a dermal flap can be added to augment the base of the nose at the nasal sill. If the patient has a small deficiency at the nasal sill and does not want surgery, augmentation with injectable fillers can help with nasal base symmetry.
SUMMARY The nose in patients with congenital cleft malformations is often the facial feature that is most noticeable to the observer. The secondary nasal deformity is variable and is affected by the extent of the original cleft, growth, and by any intervening surgery to correct the lip or nose. Repair of secondary cleft nasal deformities is challenging. Successful reconstruction requires an understanding of the pathologic anatomy, adequate exposure to perform techniques, and attention to structural grafting to overcome scarring and provide support. Often, graft material from the septum, rib, and/or ear is required. Attention must be paid to both function and appearance.
REFERENCES 1. Sykes JM, Senders CW. Pathologic anatomy of cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic surgery. New York: Thieme Medical Publishers; 1993. p. 57–71. 2. Sykes JM, Senders CW. Surgical management of the difficult nose. Oper Tech Otolaryngol Head Neck Surg 1990;1(4):219–24. 3. Shih CW, Sykes JM. Correction of the cleft-lip nasal deformity. Facial Plast Surg 2002;18(4):253–62. 4. Capone R, Sykes J. Evaluation and management of cleft lip and palate disorders. In: Papel ID, editor. Facial plastic and reconstructive surgery. 3rd edition. Stuttgart (NY): Thieme; 2009. p. 1059–60. 5. Sykes JM, Jang YJ. Cleft lip rhinoplasty. Facial Plast Surg Clin North Am 2009;17(1):133–44, vii. 6. Blair VP. Nasal deformities associated with congenital cleft of the lip. JAMA 1925;84:185–7.
7. Huffman WC, Lierle DM. Studies on the pathologic anatomy of the unilateral hare-lip nose. Plast Reconstr Surg 1949;4:225–34. 8. Coleman J, Sykes J. Cleft lip rhinoplasty. In: Papel ID, editor. Facial plastic and reconstructive surgery. 3rd edition. Stuttgart (NY): Thieme; 2009. p. 1082. 9. Sarnat BG, Wexler MR. Growth of the face and jaws after resection of the septal cartilage in the rabbit. Am J Anat 1966;118:755–67. 10. McComb HK, Coghlan BA. Primary repair of the unilateral cleft lip nose: completion of a longitudinal study. Cleft Palate Craniofac J 1996;33:23–31. 11. Grayson BH, Maull D. Nasoalveolar molding for infants born with clefts of the lip, alveolus, and palate. Clin Plast Surg 2004;31(2):149–58, vii. 12. Mulliken JB, Martinez-Perez D. The principle of rotation advancement for the repair of unilateral complete cleft lip and nasal deformity: technical variations and analysis of results. Plast Reconstr Surg 1999;104:1247–9. 13. Sykes J, Rotas N. 3rd edition. Orthognathic surgery in cleft lip and palate patient. FPS Cln NA, vol. 4. Philadelphia: WB Saunders; 1996. p. 351–75. 14. Wang TD, Madorsky SJ. Secondary rhinoplasty in nasal deformity associated with the unilateral cleft lip. Arch Facial Plast Surg 1999;1:40–5. 15. Crockett D, Bumstead R. Nasal airway, otologic, and audiologic problems associated with cleft lip and palate. In: Bardach J, Morris HL, editors. Multidisciplinary management of cleft lip and palate. Philadelphia: WB Saunders; 1990. 16. Haack J, Papel ID. Caudal septal deviation. Otolaryngol Clin North Am 2009;42(3):427–36. 17. Most SP. Anterior septal reconstruction: outcomes after a modified extracorporeal septoplasty technique. Arch Facial Plast Surg 2006;8(3):202–7. 18. Kridel RW, Scott BA, Foda HM. The tongue-in-groove technique in septorhinoplasty. A 10-year experience. Arch Facial Plast Surg 1999;1(4):246–56. 19. Murakami CS, Barrera JE, Most SP. Preserving structural integrity of the alar cartilage in aesthetic rhinoplasty using a cephalic turn-in flap. Arch Facial Plast Surg 2009;11(2):126–8.
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