eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Cosmetic Surgery

Cleft Lip Nasal Deformity

Samuel M Lam, MD, FACS, Department of Otolaryngology, Facial Plastic Surgery, Presbyterian Hospital of Plano
Maurice M Khosh, MD, FACS, Clinical Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, Columbia University College of Physicians and Surgeons; Private Practice, Head and Neck Surgical Group; Attending Surgeon, Lenox Hill Hospital, Manhattan Eye, Ear and Throat Infirmary, Columbia Presbyterian Medical Center, St Luke's-Roosevelt Hospital Center, Beth Israel Medical Center

Updated: Jun 1, 2009

Introduction

Cleft lip nasal deformity offers a unique challenge to the reconstructive surgeon for many reasons. First, clinical presentation of cleft lip varies widely, requiring a host of surgical techniques. Second, deformity may be quite severely asymmetric, making surgical correction difficult. Third, patients with cleft lip may have been previously subjected to numerous surgical interventions, leading to significant scar tissue in the operative site. Fourth, timing of rhinoplasty, whether synchronous or staged with cleft lip repair, is controversial. Fifth, this nasal anomaly affects the pediatric population, and the patient's growth affects surgical results. Conversely, the surgery may adversely compromise nose growth.

This article is intended as an introduction to basic clinical features of the deformity and to some surgical options for correction.

Cleft lip nasal deformity. A: Basal view. B: Frontal view.

History of the Procedure

Historically, cleft lip nasal deformity has received less attention than primary lip repair or has been ignored altogether. In pre-Columbian figurines, cleft lip was sculpted accurately, but nasal appearance was depicted incorrectly as normal. Rhinoplasty for cleft lip was not introduced until the turn of the 20th century. Most modern techniques had already been described by the 1920s-1940s, albeit with less refinement (eg, open rhinoplasty incisions were placed along the alar rim, leaving a conspicuous scar). It was not uncommon for patients to have undergone as many as 20 procedures for cleft lip/palate/nose. Currently, the literature offers numerous opinions regarding the best surgical approach and timing of intervention.

Problem

Patients with cleft deformity have cosmetic problems and impaired nasal airflow as a result of distorted anatomy. These patients have septal deflections, atretic nostrils, turbinate hypertrophy, and cleft lips and palates. Nasal rhinometry has demonstrated statistically significant findings of smaller airways in patients with cleft deformity when compared to patients without cleft deformity. Warren et al showed that children with unilateral cleft deformity have smaller airways than children with bilateral cleft deformity.¹ However, these differences do not persist over time. Although the cleft nose grows as the patient ages, it remains 30% smaller than that of patients without cleft lip deformity.

Etiology

Embryologic origin of cleft lip nasal deformity is not well understood. Curiously, classic nasal anomaly of patients with cleft lip may exist in absence of any cleft lip deformity owing to fetal derangements affecting only the nose. The nose is derived from two distinct tissue masses, the frontonasal and lateral facial complexes. Frontonasal processes that migrate over the forehead constitute the nasal placodes. In turn, nasal placodes develop into the medial and lateral processes, forming the columella and alae, respectively. Theory holds that mesodermal growth of the alae may follow growth of the central portion of the nose. Any disruption of this process may result in unilateral nasal deformity. Furthermore, incomplete union of the frontonasal and lateral facial complexes may contribute to clefting.

Presentation

Clinical presentation of cleft lip varies widely.

Deformity may be quite severely asymmetric.
Septal deflections, atretic nostrils, turbinate hypertrophy, and cleft lips and palates are presenting features.
Patients with cleft lip may have significant scar tissue from previous surgical interventions.

Indications

See Surgical therapy.

Relevant Anatomy

The major defect of cleft lip nasal deformity concerns the position of the ala. The ala lies inferior and lateral to the contralateral side; its relative position falsely lengthens the nose on that side. The ala rests on an underdeveloped premaxilla, which partly accounts for alar base lowering and horizontal nostril seating (see Image 1). The ala is often underdeveloped and weak, exhibiting a convoluted shape. This contributes further to dome lowering on the cleft side. Because of the abnormal ala, the columella is foreshortened and lies obliquely, with its base directed away from the cleft side. Bone growth is retarded on the cleft side; nasal bones and the nasal process of the maxilla are underdeveloped, causing the nasal dorsum to tilt to the cleft side. The septum may lie outside of the maxillary crest seat, and the cartilaginous portion may be buckled, both of which may cause nasal tip deviation.

Cleft lip nasal deformity. A: Basal view. B: Frontal view.

The nasal septum exhibits a wide range of abnormalities in addition to obvious external cosmetic deformities. When cleft palate coexists with cleft lip, significant bony abnormalities may occur along the nasal floor, including nasal-oral fistula. Bilateral cleft lips may also reveal a duplicated thickened septum, which is postulated to represent failed growth of 2 septa. Studies have confirmed the presence of significant septal deviation in patients with bilateral cleft lip when compared to patients without bilateral cleft lip. The septum tends to deviate toward the cleft side, with the cartilaginous base displaced off of the maxillary crest toward the cleft side. This septum malposition contributes to nasal tip tilt toward the noncleft side (see Image 2).

Cleft lip nasal deformity. Septum deviated toward cleft side and lying outside of maxillary crest.

Contraindications

Surgical intervention for cleft lip nasal deformity may be contraindicated in certain conditions. No absolute age minimum exists for nasal reconstruction. However, nasal surgery should be elected after age 5 years to permit sufficient physical and psychological maturity to develop before surgery is undertaken. Major septal surgery may be performed later in combination with or independent of external nasal surgery to allow for maximal time for development in this important facial growth center. As alluded to, psychological maturity is a critical consideration so that realistic and mutual goals may be set by the surgeon and patient before rhinoplasty surgery is undertaken.

Treatment

Surgical Therapy

The techniques covered in this article do not represent an exhaustive review. No perfect surgical technique exists. Surgeons must tailor approaches to individuals and evolve techniques to best serve each patient.

Nasal airway

The surgeon must aim to establish a functional nasal airway in addition to improving cosmesis. Adult studies have corroborated that postsurgically corrected noses maintain significantly smaller airways than noncleft noses. Hence, parallel goals of cosmetic and functional improvement must be served.

Septal deformity

Surgical correction aims at establishing a patent nasal airway while creating a more favorable external nasal appearance. Timing of septal surgery is controversial. One view is that early septal surgery disrupts natural septum growth, resulting in stunted nasal growth. Another view is that delay of septal surgery may cause unfavorable maxillary growth, especially if the child is a significant mouth breather. Clearly, conservative septal surgery, including reconfiguring septum shape by scoring portions of it, may be a compromise and may provide a meaningful solution. Consider the benefit of turbinate reduction or out-fracturing to increase nasal airflow. Exercise caution when performing surgery on abnormal anatomy, as vascularity and integrity of the structures may be less than optimal.

Columellar deformity

The columella is viewed as foreshortened in cleft lip nasal deformity. Two views on the best surgical method exist. Some surgeons argue that the columella requires primary correction, and others maintain that reshaping the nasal ala is sufficient to affect columellar length.

Proponents of reshaping believe that the columella owes its retracted appearance to the horizontal position of the nasal ala. Therefore, reseating the nasal ala in an anterior-posterior oval is thought to naturally elongate the columella. The Ivy modification of the Blair procedure entails medially and anteriorly rotating a laterally based nostril rim flap to lengthen the columella by repositioning the nasal ala (see Image 3). Similarly, the Dingman technique requires medially rotating a columellar and alar-based flap (see Image 4).

Cleft lip nasal deformity. Ivy modification of the Blair procedure. Laterally based nostril rim flap rotated medially inward.

Cleft lip nasal deformity. Dingman technique. Nostril rotated via columellar and alar-based flap.

Many techniques are available for primary correction of the columella. The simple V-Y advancement flap may serve to lengthen the columella if adequate columellar width and upper lip tissue are present (see Image 5). Similar to the V-Y flap, an anteriorly pedicled rectangular flap may be elevated to provide greater length (see Image 6). The Brauer-Foerster technique uses fan-shaped flaps along the medial and anterior margin of the alar rim, which can be pulled medially to increase columellar projection (see Image 7).

The Cronin procedure involves simultaneous anterior bilobed flap advancement and posterior midline columellar flap advancement (see Image 8). Techniques that lengthen the columella may be more effective for patients with bilateral cleft lip for whom symmetric columella advancement is desired.

Cleft lip nasal deformity. V-Y advancement for columellar lengthening.

Cleft lip nasal deformity. Rectangular flap for columellar lengthening.

Cleft lip nasal deformity. Brauer-Foerster procedure for columellar lengthening via medial rotation of bilobed flaps.

Cleft lip nasal deformity. Cronin procedure for columellar lengthening. Anteriorly based bilobed flap is advanced anteriorly, and posteriorly based columellar flap is advanced posteriorly.

More radical reconstruction is required when the columella is completely absent. The upper lip is the ideal donor site for reconstruction. However, the upper lip is often scarred or deficient secondary to cleft lip. Lower lip tissue may be transferred to the columella, or nasolabial flaps may be recruited. A full-thickness skin graft (FTSG) may be buried below upper lip skin for use in reconstructing the columella (ie, Ferris-Smith technique; see Image 9). The Serre method involves burying a double length of FTSG (folded on itself and buried in the upper lip) to provide additional columellar length (see Image 10). Auricular cartilage, especially from the helix, may serve to fashion a new columella, with the caveat that poor vascularity in the recipient site may hinder graft adherence.

Cleft lip nasal deformity. Ferris-Smith procedure. Repair of total columellar loss; full-thickness skin graft (FTSG) is buried beneath upper lip skin and later rotated into place.

Cleft lip nasal deformity. Serre method. Repair of total columellar loss; doubled full-thickness skin graft (FTSG) is buried beneath upper lip skin and later rotated into place.

Alar deformity

Alar deformity is the major defect in cleft lip and/or nose. Surgical techniques may be categorized into external and internal approaches. In mild defects, the nostril may be repositioned by excising a wedge of skin anterior to the rim margin and advancing the rim forward (see Image 11). Retaining a thin rim of skin at the rim margin prevents webbing at the soft triangle. Other external techniques described in Columellar deformity, above (eg, Ivy modification of the Blair technique, Dingman technique) may also be used.

Cleft lip nasal deformity. Elevation of nostril apex by excision of skin wedge.

Alar cartilage may also be delivered and repositioned without external incisions. For example, the alar cartilage is severed from its lateral attachment and advanced medially to be sutured to the contralateral ala in the Thomas Rees technique (see Image 12). Flattened contour of the ala may also be corrected by tacking the ala to the upper lateral cartilage, or, alternatively, to the nasal bone. The classic Tajima reverse-U technique has undergone many modifications. A reverse-U intranasal incision is made on the cleft side, the subcutaneous pocket is developed, and the deformed ala is sutured to the ipsilateral upper lateral cartilage, the contralateral upper lateral cartilage, and the contralateral ala (see Image 13).

Cleft lip nasal deformity. Thomas Rees technique. Lateral crus of lower lateral cartilage is severed from lateral attachment and advanced medially to be sutured to contralateral ala.

Cleft lip nasal deformity. Tajima reverse-U technique. A: Intranasal incision. B: Suture ala on the cleft side to (1) ipsilateral upper lateral cartilage, (2) contralateral cartilage, and (3) contralateral ala.

Onlay grafts may be required to achieve the ideal cosmetic result, despite best attempts at reconfiguring the ala via simple rotation or advancement. Grafts contribute to bulk for improved cosmesis and may serve to reinforce the atrophic weak alar cartilage. Septal cartilage may be harvested for immediate use or banked for future use in alar reconstruction; septoplasty is often required to restore nasal tip symmetry and increase nasal airflow. Some surgeons discourage septoplasty for the sole purpose of use in rhinoplasty because it may retard midfacial growth. Concha is an excellent source of cartilage for an onlay alar graft because it recreates the natural curvature of the ala.

Septal cartilage is straighter and more rigid than concha. Therefore, it may be best reserved for a columellar strut. The contralateral normal ala in unilateral cleft lips may be turned over to the defective side and used as an onlay graft. The cephalic border of the contralateral ala is divided but is kept pedicled medially and rotated over to the cleft side (see Image 14). Various modifications of this technique exist. More extreme defects may be reconstructed with a C-shaped costal cartilage graft.

Cleft lip nasal deformity. Cartilage grafts. A: Onlay alar graft. B and C: Turnover alar graft.

Dorsal deformity

Correction of septal deflection may be sufficient to reestablish normal dorsal symmetry. Advocates of early septal surgery cite the benefit of more normal nose growth, including the dorsal aspect. At times, osteotomies may be required to correct dorsal deflection. Onlay cartilage grafts have also been used to add bulk to the underdeveloped cleft side. Further projection may be gained through dorsal onlay bone grafting (eg, cranial, rib, iliac).

Bilateral cleft lip nasal deformity

The surgical techniques mentioned above focus on unilateral cleft lip deformities but also apply to bilateral deformities. Evolution of surgical techniques for treatment of bilateral cleft lip deformities has lagged behind treatment for unilateral deformities. This may reflect the relatively lower prevalence of bilateral cleft lip deformities.

Computerized constructions reveal that bilateral cleft lip nose may be viewed as a duplicated image of unilateral cleft lip nose. Stigmata include a broad, flat, bifid tip; wide alar bases; and short columella—all of which contribute to the overall porcine nose appearance (see Image 15). The nasal dorsum may exhibit lack of height, but it is usually within normal limits.

Cleft lip nasal deformity. Bilateral cleft lip nasal deformity. Broad, flat, bifid tip; wide alar bases; and short columella. A: Frontal view. B: Profile view.

The hallmark of bilateral cleft lip nose is a short columella. Millard popularized what is perhaps the best known columellar lengthening technique (see Image 16). Millard advocated the use of forked flaps, one from each side of the prolabium, which are banked within the nasal sills during primary lip repair until secondary rhinoplasty is performed. These flaps are then retrieved and rotated into the columella of the child when aged 2-4 years to achieve columellar lengthening.

Cleft lip nasal deformity. Millard classic forked flaps. One taken from each side of prolabium and banked within nasal sills during primary lip repair until secondary rhinoplasty.

However, McComb reviewed his cases of primary columellar repair using forked flaps and discovered that by adolescence, 3 unfavorable features developed: (1) the columella overlengthens, (2) the nasal tip broadens, and (3) downward drift of the lip-columellar junction occurs in conjunction with an unsightly transverse scar.² Therefore, McComb discourages borrowing tissue from the prolabium and favors using only nasal tissue to reconstruct the columella. McComb argues that surgical repair should focus on reestablishing normal alar shape, which in turn naturally elongates the columella. McComb achieves this result by suturing the medial crura of the alar cartilages together, which lengthens the columella and corrects the broadened nasal tip.

Mulliken states that the already flattened nasal ala continues to separate over time as a result of muscle tension.³ Therefore, Mulliken favors primary alar correction, as does McComb, to achieve columellar length and proper nasal tip shape.

Outcome and Prognosis

Cleft lip nasal deformity is a complex anomaly, and proper correction requires considerable surgical talent and experience. Surgeons must continue to tailor approaches to individuals and evolve techniques to best serve each patient. Children who benefit from good surgical repair have a chance to mature with fewer psychological sequelae from this deformity.⁴

Multimedia

Media file 1: Cleft lip nasal deformity. A: Basal view. B: Frontal view.

Media file 2: Cleft lip nasal deformity. Septum deviated toward cleft side and lying outside of maxillary crest.

Media file 3: Cleft lip nasal deformity. Ivy modification of the Blair procedure. Laterally based nostril rim flap rotated medially inward.

Media file 4: Cleft lip nasal deformity. Dingman technique. Nostril rotated via columellar and alar-based flap.

Media file 5: Cleft lip nasal deformity. V-Y advancement for columellar lengthening.

Media file 6: Cleft lip nasal deformity. Rectangular flap for columellar lengthening.

Media file 7: Cleft lip nasal deformity. Brauer-Foerster procedure for columellar lengthening via medial rotation of bilobed flaps.

Media file 8: Cleft lip nasal deformity. Cronin procedure for columellar lengthening. Anteriorly based bilobed flap is advanced anteriorly, and posteriorly based columellar flap is advanced posteriorly.

Media file 9: Cleft lip nasal deformity. Ferris-Smith procedure. Repair of total columellar loss; full-thickness skin graft (FTSG) is buried beneath upper lip skin and later rotated into place.

Media file 10: Cleft lip nasal deformity. Serre method. Repair of total columellar loss; doubled full-thickness skin graft (FTSG) is buried beneath upper lip skin and later rotated into place.

Media file 11: Cleft lip nasal deformity. Elevation of nostril apex by excision of skin wedge.

Media file 12: Cleft lip nasal deformity. Thomas Rees technique. Lateral crus of lower lateral cartilage is severed from lateral attachment and advanced medially to be sutured to contralateral ala.

Media file 13: Cleft lip nasal deformity. Tajima reverse-U technique. A: Intranasal incision. B: Suture ala on the cleft side to (1) ipsilateral upper lateral cartilage, (2) contralateral cartilage, and (3) contralateral ala.

Media file 14: Cleft lip nasal deformity. Cartilage grafts. A: Onlay alar graft. B and C: Turnover alar graft.

Media file 15: Cleft lip nasal deformity. Bilateral cleft lip nasal deformity. Broad, flat, bifid tip; wide alar bases; and short columella. A: Frontal view. B: Profile view.

Media file 16: Cleft lip nasal deformity. Millard classic forked flaps. One taken from each side of prolabium and banked within nasal sills during primary lip repair until secondary rhinoplasty.

References

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Chetpakdeechit W, Hallberg U, Hagberg C, Mohlin B. Social life aspects of young adults with cleft lip and palate: grounded theory approach. Acta Odontol Scand. 2009;67(2):122-8. [Medline].
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Liou EJ, Subramanian M, Chen PK. Progressive changes of columella length and nasal growth after nasoalveolar molding in bilateral cleft patients: a 3-year follow-up study. Plast Reconstr Surg. 2007;119(2):642-8. [Medline].
Millard DR. Cleft Craft. Vol 2. Boston, Mass:. Little Brown & Co;1977.
Numa W, Eberlin K, Hamdan US. Alar base flap and suspending suture: a strategy to restore symmetry to the nasal alar contour in primary cleft-lip rhinoplasty. Laryngoscope. 2006;116:2171-7. [Medline].
Peled IJ, Ramon Y, Ullmann Y. Wrap-around cartilage flap for correction of unilateral cleft lip nose deformity. Plast Reconstr Surg. Jun 1997;99(7):2085-8. [Medline].
Randall P. History of cleft lip nasal repair. Cleft Palate Craniofac J. Nov 1992;29(6):527-30. [Medline].
Sandham A, Murray JA. Nasal septal deformity in unilateral cleft lip and palate. Cleft Palate Craniofac J. Mar 1993;30(2):222-6. [Medline].
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Keywords

cleft lip nasal deformity, unilateral cleft lip, bilateral cleft lip, cleft-lip nasal deformity, hare lip, cleft deformity, cleft nose, cleft lip, nasal deformity

Contributor Information and Disclosures

Author

Samuel M Lam, MD, FACS, Department of Otolaryngology, Facial Plastic Surgery, Presbyterian Hospital of Plano
Samuel M Lam, MD, FACS is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Maurice M Khosh, MD, FACS, Clinical Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, Columbia University College of Physicians and Surgeons; Private Practice, Head and Neck Surgical Group; Attending Surgeon, Lenox Hill Hospital, Manhattan Eye, Ear and Throat Infirmary, Columbia Presbyterian Medical Center, St Luke's-Roosevelt Hospital Center, Beth Israel Medical Center
Maurice M Khosh, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Medical Association, and Triological Society
Disclosure: Nothing to disclose.

Medical Editor

Mimi S Kokoska, MD, Associate Professor, Department of Otolaryngology-Head and Neck Surgery, University of Arkansas for Medical Sciences; Chief, Department of Otolaryngology-Head and Neck Surgery, Central Arkansas Veterans Healthcare System
Mimi S Kokoska, MD is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American College of Physician Executives, American College of Surgeons, American Head and Neck Society, and Arkansas Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Robert M Kellman, MD, Professor and Chair, Department of Otolaryngology and Communication Sciences, State University of New York, Upstate Medical University
Robert M Kellman, MD is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Medical Association, American Neurotology Society, American Rhinologic Society, American Society for Head and Neck Surgery, Medical Society of the State of New York, and Triological Society
Disclosure: GE Healthcare Honoraria Review panel membership

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown

Further Reading