AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• References

The human face is basically composed of expressive tissue draped over an underlying skeletal framework. This pattern allows for the human capacity to express complicated emotions. The nose, as the most prominent facial feature, becomes the focus of psychologic and social attention when it is distinguished by anomalous features. In current practice, plastic surgeons are better able to reconstruct the faces of children with cleft features to near-normal anatomic form and physiologic function.¹ Surgeons are now able to counsel parents that with modern knowledge and technology, their children, whose appearance may seem somewhat disfiguring at birth, can be transformed into children with beautiful appearances and attractive smiles.

Multiple techniques are available to reconstitute the normal anatomy and physiologic function of the patient with cleft features. In the authors' experience, the final defining component receiving attention by parents, practitioners, and patients in achieving a normal appearance in a patient with cleft features currently is the nose. Despite the current trend for increasing attention toward nasal reconstruction at the time of primary lip repair, a margin still exists for achieving aesthetic improvement of the cleft lip nasal deformity. Optimal timing for reconstruction of the cleft lip nasal deformity remains somewhat unresolved.^{2, 3, 4, 5, 6}

As plastic surgeons continue to improve outcomes in the management of cleft lip defects, the repair of the nasal deformity becomes more important to families and patients to fulfill the goal of restoring the face to its full capacity of anatomic function and human emotional display. The nasal repair looms ever more important in modern society, in which appearance is paramount. The patient with cleft features should benefit from a satisfactory functional repair and from a satisfactory aesthetic repair. This correction must include the comprehensive reconstruction of the cleft nasal deformity.

History of the Procedure

The word harelip or lagocheilos is attributed to Galen.⁷ From this point, the history of cleft repair is a well-documented stepwise series of surgical refinements. A Chinese physician is credited with the first attempted harelip repair in approximately 400 AD.⁸ Straight-line closures of the mid-1800s were later modified by Hagedorn, who advocated the idea of interdigitating flaps for closures.⁹ Blair and Brown^{10, 11} and Brown and McDowell¹² later described the use of triangular flaps. Flap-closure and multiple-closure techniques progressed and developed into the 1950s and 1960s. Wynn and then Davis refined flap closure.¹² Millard popularized a technique that preserved the Cupid's bow and philtral dimple and reduced tension on the lip, thereby producing a more consistent cosmetic result.¹³

Until recently, the result of cleft lip nasal repair received less attention by plastic surgeons.⁸ Some early cleft surgeons, such as LeMesurier and Dennis Browne, admitted "complete nasal abdication."¹⁴ However, a satisfactory cosmetic result of the cleft nasal deformity was not ignored completely. In a 1931 paper discussing nasal abnormalities, Blair and Brown pointed out that the nose is the largest single feature of the face. They emphasized "that a pleasant face is important for employment and social reasons. Any blight in the nose makes the patient less than socially acceptable."¹¹

Even most 20th century surgeons debated the numerous problems related to the cleft lip and palate and quite frankly admitted that the nose was out of their surgical realm.² In 1925 in The Journal of the American Medical Association, Blair summarized the state of the repair of the harelip as varying from "nearly perfect to plain bad."¹⁰

Blair went on to describe abnormalities that could be fixed. Interestingly, he pointed out that the nasal deformities that were not addressed persisted when considering repair of the single cleft lip. Gillies wrote that the original deformities involving both the nose and lip were so complex that expecting any one primary operation to accomplish more than an aseptic closure was unreasonable. Future surgery would be needed to provide the desired result.¹³

Early plastic surgeons delayed nasal repair because they believed the deformity would naturally improve itself with eventual growth.¹⁵ In addition, primary attempts at nasal tip repair were temporary and frequently ineffective.^{16, 17, 18} In 1946, the so-called king of noses, Gustav Aufricht, wrote "the operation to correct skeletal asymmetry of the nose in association with harelip should be deferred until the age of sixteen or seventeen."¹⁹ In 1972, he reconfirmed his conservative view with the observation that many noses repaired in infancy eventually flattened out during adolescence: "The more material available at the age of sixteen or seventeen, the better the correction possible."²⁰

Based on these teachings, medical dogma evolved such that no surgery should be performed on the nasal deformity at the time of primary lip closure. This trend continued for quite some time. In 1968, Matthews advised against operating on the alar cartilage on patients younger than 16 years. He believed that during childhood, eventual secondary distortion would occur in the growing cartilage that "would doubtless reduce subsequent nasal growth or would inhibit growth all together."²¹ Proponents against early surgical intervention believed that it prevented the completion of the physiologic growth of the nasal cartilage.^{13, 22}

Such observations probably originated from surgeons who saw the results of nasal surgery performed in the late 1800s and the first half of the 1900s, when the vast majority of patients with cleft deformities were operated on by inexperienced surgeons who may have been general surgeons; orthopedic surgeons; or ear, nose, and throat surgeons. These individuals had no specific training or comprehensive understanding of the intricate facial structures and their associated physiologic processes. This experience led to so many patients with deformed and scarred noses seeking help that surgeons were taught to not operate on the nose primarily in the hopes that the child could then be operated on by a skilled reconstructive surgeon later in life.²³

As specially trained reconstructive surgeons used more uniform protocols to repair cleft nasal deformities, primary repair of nasal deformities received increased attention.²⁴ Additional clinical evidence proved more extensive surgery could be performed on children than previously believed, without interfering with future nasal cartilage growth and development.^{25, 26, 27, 28} Nasal repair at the time of the primary lip repair was preferred for various reasons.²⁹ According to Gelbe, "The nose should no longer be overlooked or relegated to a separate operation in the future".³⁰ To avoid having a child grow up with the psychological and social burdens of a deformed nose, more plastic surgeons concentrated on early primary nasal repair. In 1959, Berkeley stated, "Primary repair of the nose should reach the state much like the lip, with no need or prospect for secondary repair".¹⁴

In 1961, Stenstrom and Oberg proposed that the nasal tip deformity was caused by the imbalance of muscular pull on both the cleft and noncleft sides. They believed that by repositioning muscle and bony elements in proper dynamic anatomic alignment, the nose would develop into a more natural place, thereby avoiding further surgery.

A 1996 review by McComb confirmed that early surgery results in minimal interference with eventual growth. Since 1960, emphasis on primary repair of the cleft nasal deformity has increased.

Nasal reconstruction has now evolved into an important focus for primary cleft lip repair. Many surgeons believe early primary repair is durable and does not interfere with nasal growth and development.³¹ More importantly, patients are often freed from years of childhood and adolescent embarrassment.²⁰

Problem

See Embryology.

Frequency

In an epidemiologic study performed by Das et al, the incidence of total clefts in live-born infants was found to be 1.36 cases per 1000 live births for white neonates and 0.54 cases per 1000 live births for neonates who were not white.³² The incidence of cleft palate deformities is higher in populations who have a familial history of a similar deformity.³³

Etiology

Embryology

In utero developmental processes are complex. However, the production of the human face is even more complicated because it basically recapitulates the evolutionary history of vertebrate development.³⁴ To fully understand the intricacies of the cleft anomaly, clinicians must develop a comprehensive understanding of both normal and pathologic embryologic development.

Embryologic facial growth is produced by the fusion of 5 facial swellings: 2 maxillary, 2 mandibular, and an unpaired frontonasal.³¹ Incomplete or partial fusion of any of these swellings or processes results in the development of either a unilateral or a bilateral facial cleft. Specifically, plastic surgeons working with the cleft lip and nasal abnormalities are interested in anomalies resulting from the failure of the maxillary swelling to fuse with the frontonasal or intermaxillary processes. Although cleft lips and cleft palate often occur together, the 2 defects differ in their distribution. For this reason, they are considered distinct entities.

During the third week of embryogenesis, facial growth commences. At this point, the embryo has already divided into a 3-layered germ disc, including the face and its mesodermal precursor structures. At this time, it is ready for the future organogenesis that occurs during the fourth to eighth weeks. During the fourth week, the neurocrest develops and detaches from the lateral lips of the neurofold and migrates into different locations.

Some of the neurocrest cells are intimately involved in the formation of the mouth and nasal structures. These neurocrest cells migrate to the developing first 5 pairs of pharyngal arches on either side of the pharyngal foregut on day 22 and are important to the ultimate development of the cartilaginous rudiments of the nose and face. These arches correspond to numbers 1, 2, 3, 4, and 6 of the primitive vertebrae or branchial arches. Each arch consists of an outer covering of ectoderm, an inner covering of endoderm, and a core of mesoderm, which includes contributions from adjacent somites and the neurocrest cells. The arches are separated externally by ectoderm lying in pharyngal clefts and internally by endoderm lying in pharyngal pouches. In summary, the clinical spectrum of congenital facial clefts basically results from the failure of these facial processes to correctly fuse.

Simultaneously, nasal development commences as a pair of thickened ectodermal nasal placodes located on the frontonasal or intermaxillary processes. The central portion of each placode invaginates to form the epithelium of the nasal passage, while the raised margin of the placode gives rise to the nose, the philtrum of the upper lip, and the primary palate. Nasal passages are formed by the continued deepening of the nasal pits. At the end of the sixth week, the deepened nasal pits fuse to form a single, large ectodermal nasal sac lying superoposterior to the intermaxillary process.

From the end of the sixth week to the beginning of the seventh week, the floor and posterior wall of the nasal sac proliferate to form a thickening plaquelike thin fin of ectoderm separating the nasal sac from the oral cavity. This structure is termed the nasal fin. Vacuoles develop in the nasal fin and fuse with the nasal sac, thus enlarging the sac and thinning the fin to a thin membrane called the oral nasal membrane. This separates the sac from the oral cavity. This membrane ruptures during the seventh week to form an opening termed the primitive choana. At this stage, the floor of the nasal cavity is formed by a posterior extension of the intermaxillary process called the primary palate.

A commonly accepted process is that the facial mesenchyme originating from the cranial neurocrest cells later gives rise to the skeletal and connective tissues of the face (Siegel, 1985). Conversely, the ectoderm of the central face apparently plays the most significant role in ultimate nasal development. The epithelial wall of the upper lip is reinforced by migrating mesoderm, and 2 paired arches of ectoderm appear. These become the nose. Ectodermal cells proliferate and begin to sculpt an amorphous cell mass into a finite structure by cellular polarization that causes alignment in one specific plane.

Mesodermal migration is now considered the most important element in the reinforcement of developing facial structures. A lack of this mesoderm allows the structures to split apart, ultimately forming clefts. Other modifications of established theories suggest merging and fusion of local prominences. Apparently, up to the point of clefting, the embryonic structures are symmetric and equal in volume. However, once the cleft occurs, a developmental disparity in muscular tissue and cartilage is present. This situation gives rise to the cleft deformity and especially demonstrates the nasal cleft anomaly.³¹

Studies by Stark demonstrated that a minimum of ectodermal tissue is within the nose on the cleft side of an embryo with a clefting syndrome. With the asymmetry of tissue, an associated asymmetry of muscle or tissue tension is present. This further augments the developmental malposition and associated deformity.³⁵ This theory was popularized by Stenstrom, who demonstrated in cadaver dissections that the typical nasal deformity developed secondarily to tension on the columella and the alar base.^{36, 37}

While no single, proven embryologic explanation accounts for cleft formation and the associated nasal deformity, researchers generally accept that the clefting syndrome is caused by both a lack of ectodermal and mesodermal components and a subsequent mechanical deformation of tissue.⁶

The processes leading to normal development are undoubtedly complicated. Probably, relatively few alterations are responsible for the vast majority of clinical clefts. Environmental factors, from the oxygen concentration to the presence of adverse environmental factors such as phenytoin (Dilantin), which causes an increase in cleft formation, likely vary.

The occurrence of facial clefting appears to be based on the multifactorial effect of both genetic and environmental factors that determine the susceptibility of cleft formation.³⁸ According to this concept, when tissue susceptibility crosses the pathologic threshold, a cleft forms.³⁹

Pathophysiology

Anatomy and physiology of the cleft nose

In the typical symmetric face, the anatomic nose is distinctive as a single midline structure. Any slight asymmetry is immediately apparent and characteristic. The cleft nasal abnormality is usually confined to one side, but its characteristic features are constantly emphasized by comparison to the normal side.⁴⁰

Satisfactory repair of the cleft lip and nasal deformity begins with the surgeon's complete understanding of the normal anatomy and physiology of the nose and the normal features of the unilateral cleft lip nose. These consist of the following:⁴¹

• The columella appears shorter on the cleft side.


• The columella has an oblique position, with its base deviated to the noncleft side.


• The lateral crus of the lower lateral cartilage and the adherent skin are drawn into an S-shaped fold.


• The lateral crus of the lower lateral cartilage is longer on the cleft side.


• The lower lateral cartilage is displaced anteriorly on horizontal planes.


• The nasal tip is displaced in the frontal and horizontal planes following displacement of the lower cartilage.


• The nasal tip is asymmetric.


• The vestibular dome is excessively obtuse.


• The ala is flattened, resulting in a horizontal orientation of the nostril.


• The nostrils are asymmetrically proportioned.


• The entire nostril is in a retroposition.


• The base of the nostril is in a retroposition.


• The base of the ala is displaced laterally and/or posteriorly or inferiorly.


• The nasal floor is lower on the cleft side.


• A nasolabial fistula may be present.


• The caudal edge of the nasal septum and the anterior nasal spine are deflected into the noncleft vestibule.


• The nasal septum is deviated, resulting in varying degrees of nasal obstruction on the cleft side.


• The lower turbinate on the cleft side is hypertrophic.


• The maxilla is hypoplastic on the cleft side.


• The maxillary segment is displaced on the cleft side.


• The premaxilla and maxillary segments are displaced from the noncleft side.

Additional abnormalities may be associated with the bilateral cleft lip nose as follows:

• The columella is relatively short, and the prolabium may appear to be attached to the nasal tip.


• The nasal tip is flat and broad.


• The nasal alae are flat and sometimes drawn in an S-shaped fashion.


• The base of the ala is displaced laterally and sometimes inferiorly and posteriorly.


• Both nostrils are oriented in a horizontal position.


• The lower lateral cartilages are severely deformed.


• The nasal floor is absent.


• The caudal end of the septum and the nasal spine are displaced inferiorly, relative to the level of the alar bases.


• The nasal tip and nostrils are asymmetric.

Additional descriptions of the anatomy and morphology of these malformations have been well described by Huffman and Lierle. More asymmetry leads to a more noticeable nasal defect. These abnormalities become even more apparent as the child matures.³⁴ The nasal asymmetry peaks during the postpubertal growth spurt.⁴² Deviation of the nasal tip ends at the pubertal growth spurt.

Controversy persists regarding whether a deficiency of tissue is present on the cleft side or whether an imbalance exists in the arrangement of the tissue. Whether the cause is due to an intrinsic deficiency or secondary to normal structures being distorted has yet to be resolved.⁶ Farkas described the downward displacement of the alar bases in patients without clefts and in those with minimal nasal deformities. He attributed these changes to a lack of mesoderm.⁴³ This syndrome characterizes the cleft nasal deformity and is likely caused by a combination of tissue deficiency and abnormal mechanical factors.⁴³

Histologic studies by Atherton show the cartilage on the cleft side to be similar to the cartilage on the noncleft side. The difference appears to be in the form and anatomy rather than in their failure to develop. Facial cartilage in cleft fetuses demonstrates distortion rather than an overall deficiency of tissue.⁴⁴

After studying facial clefts in adults, Huffman and Lierle proposed that the deformity was due to tissue malposition of the cleft half and not to a relative size discrepancy. However, this does not seem to be true for bilateral clefts. In these situations, the opinion exists that a definitive lack of tissue is found in the columella.^{45, 35} Additionally, abnormal cranial anatomy is found in patients with clefts. Complete clefts are associated with increased height of the upper face, enlarged nasal cavities, decreased thickness of upper the lip, and a shortened maxillary depth.

In both complete and incomplete clefts, additional dental abnormalities, posterior displacement of the maxilla, and changes in the overall shape of the lower jaw can be found.²⁸ These further enhance the clinical findings characteristic of the cleft lip nasal deformity.

Salyer has emphasized the importance of recognizing these skeletal abnormalities.²⁹ McComb further stressed the value of repairing bony abnormalities to achieve a successful nasal repair. In the partial unilateral cleft, usually only the inferior portion of the nose is affected, leaving the underlying bone intact. In the complete cleft, all 3 layers (ie, mesoderm, endoderm, ectoderm) are separated, including the underlying bone. With the bilateral cleft, the premaxilla protrudes anterior to the retracted maxilla. This discontinuity of bony junctions must be recognized and reconstructed for successful nasal and cosmetic repair.⁴⁶ In fact, the bony problems in patients with repaired clefts may be caused by the surgery itself.⁴⁷

The absence of maxillary hypoplasia in class III malocclusion in patients with unrepaired cleft palates suggests the surgical procedures used to correct cleft lip and palate may have caused this condition. The resulting midface deficiency also may manifest at the nasal level.¹ In patients with cleft lip and palate and associated maxillary hypoplasia, maxillary advancement using the principles of distraction osteogenesis may be an alternative treatment technique to repair the bony structures and create a more acceptable cosmetic result.

Another advantage of maxillary distraction is that it can be applied to children. Delaying reconstruction of severe maxillary hypoplasia is no longer necessary. This can be performed with minimal morbidity when the child is as young as 5-6 years to improve the eventual cosmetic result.⁴⁸ This also creates an adequate platform for definitive nasal repair. Whether the nasal deformity is due to the lack of embryonic tissue or malformation of normal tissue, clearly the nasal deformity is affected by the severity of the cleft. The nasal floor is atrophic or absent, and in severe clefts, an associated deficiency in bone and muscle may be present.

The role of the facial muscles, especially in the orbicularis oris, in distorting the anatomy is another important consideration. Abnormal insertions or tractions of disorganized muscle may increase or accentuate the deformity as the face continues to grow.³⁷ Nasal tip skin and mucosae are usually normal. The lower lateral cartilage is subluxed on the cleft side and inappropriately lengthens the cleft side of the nose.²⁰ However, the quantity of soft tissue present in the nostril on the cleft side is sufficient to construct a nostril similar to the unaffected side.⁴

In the bilateral cleft, nasal structures are affected more substantially, with buckling and inferior displacement of the lateral cartilage and a short columella.

The most important physiologic function of the nose is to serve as an airway. The nasal airway may be compromised by the cleft lip nasal deformity. This is an extremely important feature to consider prior to initiating comprehensive surgical repair. These deformities tend to compromise the nasal airway, increase airway resistance, and encourage mouth breathing. The difference in airway size between patients with clefts and those without is approximately 30%, and approximately 60% of patients with clefts have difficulty with nasal breathing.⁴⁹

In addition to abnormalities of the nasal framework, associated soft tissue abnormalities, septal anatomic variations, or hypertrophy of the turbinates may be present. Careful presurgical considerations of the pathologic anatomy should take into consideration both the aesthetic aspects and the functional aspects of the nasal airway.⁵⁰

Although studies have demonstrated that the cleft nose grows with advancing age, it remains 30% smaller than the noncleft nose.⁵¹ This encourages mouth breathing and continued physiologic difficulties.

The cleft nose should be regarded as a complex syndrome, in which these anatomic deficiencies are present in varying degrees.

The nasal deformity is a result of abnormalities of the lip, alveolus, palate, and maxilla. Unilateral clefting, both complete and incomplete, results in a nasal deformity that may be caused by one or a combination of 3 major factors: (1) imbalance of the facial musculature, (2) hypoplasia of the skeletal base, and (3) asymmetry of the skeletal base.⁴¹ The nasal deformity associated with bilateral clefts varies based on whether the bilateral cleft is symmetric or asymmetric. In symmetric bilateral clefts, the premaxilla may be positioned entirely within the alveolar arch and may appear normal. In these patients, the problem is closely attached to the nasal tip with a very short columella.

In the unilateral cleft nose, the nasal discrepancy produces a nose that is pathognomonic for this syndrome. It is an anatomic defect that produces a horizontal nasal aperture on the cleft side and a vertical nasal aperture on the noncleft side. This appearance of a completely normal side and an abnormal side is pathognomonic for this distinct congenital anomaly.⁴⁰

Clinical

See Embryology.

INDICATIONS

Section 3 of 10  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• References

The cleft lip nasal deformity is of significant concern to both patients and parents. Patients can often experience feeding difficulties, middle ear disease, impaired speech, oronasal fistula, anomalous dentition, and cosmetic deformities. Surgical repair is considered the best treatment option.

RELEVANT ANATOMY

Section 4 of 10  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• References

See Pathophysiology.

CONTRAINDICATIONS

Section 5 of 10  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• References

Contraindications to surgical correction are related to the patient's desire to not have surgery or to the patient's overall medical status.

WORKUP

Section 6 of 10  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• References

Lab Studies

• The rule of 10 is the long-standing guideline to determine when a neonate is fit for surgical repair (Jones, MC). This rule mandates that the infant weigh at least 10 lb, be aged at least 10 weeks, and have a minimum hemoglobin level of 10 g/dL before surgery is considered. This rule has often excluded neonates younger than 28 days. However, new advances in neonatal anesthesia have made surgery possible in those excluded by these guidelines.
• Hemoglobin studies, hematocrit studies, and platelet counts are routinely obtained, and the neonate is evaluated by the appropriate anesthesia team. Any cardiac or respiratory problems are addressed prior to surgery with the appropriate referrals made, as needed.

TREATMENT

Section 7 of 10  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• References

Surgical Therapy

Surgical approach to the nasal defect

"A well mended harelip would pass unnoticed at a cocktail party were it not for the nose."¹³ Despite multiple technical procedures described, no one protocol has proven to be completely satisfactory in the repair of all cleft lip nasal deformities.¹⁸ Still, controversy remains as to the optimum corrective approach, the best techniques for exposure and repair, and most significantly, the timing of the correction.

Some surgeons believe that early nasal surgery (1) interferes with growth, resulting in nasal hypoplasia; (2) introduces scars, making secondary correction difficult; (3) damages infantile cartilage; and (4) makes repair technically harder because of the small size of the nose and immature cartilage.⁴

Reconstructive surgeons have been reluctant to perform rhinoplasty on a growing nose; however, the use of new, seemingly atraumatic operative techniques has created growing acceptance to correcting nasal deformities prior to puberty.¹⁷ However, even with these new atraumatic operative techniques, a loss of integrity of the cartilaginous nasal skeleton does not usually result in growth inhibition in the region of the mid face when the septum is not subjected to an operative procedure.²²

Current surgical procedures use multiple techniques and types of intervention to correct the residual nasal deformity.¹⁷ Prevailing opinion divides the approach. The first approach is to correct the skeletal deformity; then, the overlying nasal pyramid is modified. To achieve optimum surgical correction, the nasal floor is believed to require alignment with the alar base.⁵ The ubiquitous presence of maxillary deficiency was illustrated in 1996 by Kausman and Circkelair in a special study on minimal cleft lips that showed consistent maxillary bony defects.⁵²

Bony manipulation, including alignment of the septum, has been shown to have a positive effect on subsequent nasal development.²⁶ Correction of the residual cleft nasal deformity may be approached first by correcting the bony foundation and cartilaginous skeleton and the overlying nasal sutures.^{53, 54} The first stage includes orthodontic or orthopedic alignment of the maxillary nostril, closure and grafting of the alveolar cleft, and reconstruction of the floor.⁵⁵ The second stage is restoring airway patency, undermining and reconstituting the slumped alar cartilage, suturing the dome, creating symmetry, aligning the orbicularis oris, and increasing the tip projection of the nose.

Some surgeons even advocate approaching the nasal defect at the time of the primary lip repair,⁵⁶ while others, such as Mazzola, advocate delay of definitive nasal correction until the patient is older than 13-15 years. He recommends that the nasal repair be performed as a single second stage that includes performing septal surgery, correcting the slumped lower cartilage, suturing the dome, increasing the tip projection, creating symmetry of the width of the alar bases, and suturing the orbicularis oris muscle.⁵

An alternative type of repair is repositioning through so-called unit arrangement. This consists of repair of the deformed nostril approached through external skin incisions extending into the dorsal skin of the nostril. The anomalous portion is then rotated as a unit of skin cartilage and, occasionally, its underlying mucosa. The advocates of this technique do not consider the external scar a drawback.³

Other surgeons, such as Gorney, advocate that external scars should be avoided at all costs. These authors advocate that internal incisions should be used to expose the abnormal cartilage. They then are rearranged into more normal anatomic relationships and are held in position with a variety of fixation techniques to the upper lateral cartilage, the contralateral cartilage, or the normal opposite alar dome. Many authors have illustrated good results for repair of minor deformities.^{53, 57, 17, 54}

The advantage of this procedure is that it avoids an external scar. Early results appear cosmetically acceptable; however, the structural support elements are bolstered and can show signs of deterioration.

Whichever approach or technique is used, results may deteriorate in time. This may be due to scar formation, a difference in configuration between the ala on the cleft side and contralateral side, or a discrepancy between the lining of the nose and the skin coverage, resulting in dead space and resilient scar formation.¹⁷ Multiple techniques have been suggested to prevent subsequent deformation, such as suspending the slumped alar cartilage to a fixed point or using a cartilage stent to stabilize the columella.¹² The use of postoperative nasal splinting with silicone splints is popular in Asia and is believed to preserve the correction.⁵⁸ A dynamic nostril splint in surgery of the nasal tip was developed by Guarda in an attempt to keep the nose symmetric as it grows.¹⁶

Current thinking that considers every cleft lip nasal patient as a candidate for open rhinoplasty is based on wide exposure and good surgical control of the external approach. The external approach perhaps started with Duffenbach in 1845.⁵⁹ Multiple variations have been described and modified.⁵ The nasal approach, or "flying gull wing" incision, is placed on the infratip of the nose.⁶⁰ The incision extends from one alar margin to the other. Exposure is somewhat limited and does not allow good symmetric vision of all the distorted structures.

Blair initially described a midcolumellar approach in 1925.¹⁰ This approach has proved popular, and several authors modified it over the next half-century.^{1, 61} The incision starts along the nasal sill and extends up until the mid line of the columella over the dome. The incision then curves toward the affected side and ends at the lateral part of the lower lateral cartilage. The advantage is good exposure on the affected structures, but disadvantages include allowing upper rotation on the cleft side only.

A third or transcolumellar approach is a modification of the original Rethi approach.^{62, 57} The incision begins on one side, along the margin of the lower lateral cartilage. It then proceeds medially toward the caudal margin of the medial crus and continues along the columella up to its mid portion. The incision then continues in a similar way into the contralateral side. This approach allows repair and visualization of both sides of the nose.⁶³

Summary

The cleft lip nasal deformity has become more apparent to patients and parents. Without the continued capacity for successful reconstruction of the cleft lip, physicians will develop improvements in correction of the associated cleft lip nasal deformity. Additional reconstruction of the researched cleft nasal deformity can be performed when the patient is aged 4-6 years. Attention should be directed toward the development of symmetry with suture positioning and/or conchal grafts. This can be performed via either an open or a closed approach depending on the surgeon's preference and ability. Definitive correction should be delayed until the early teenage years. The authors strongly recommend an open approach for exposure and an anatomic approach to reconstruction, with autogenous tissue for replacement and/or augmentation. At this time, the surgeon can also address definitive correction of any septal abnormalities.

Some surgeons still advocate adolescence as the optimum time for definitive correction of the cleft lip nasal deformity. Repair of the nose at the time of lip surgery is difficult because of the small and delicate nature of neonatal cartilage. However, in the authors' opinion, long-term results are often unpredictable. The surgeon must weigh the possible advantages against the possible disadvantages in form and function due to potential growth inhibition. Rhinoplasty may be indicated before the normal growth spurt at puberty in patients with severe functional impairment, those with severe psychological problems, or to stop further expression of a deformity induced by septal growth.

Two issues must be addressed in order to support the continued acceptance of early nasal repair at the time of cleft lip repair. First, consider whether normal nasal growth and development will be interfered with, and second, consider whether the repair will remain stable, particularly through the adolescent growth spurt. Thus far, long-term evaluation and results have not been able to demonstrate any interference with nasal growth and the possible benefit of requiring fewer subsequent operations for a good cosmetic result.^{25, 29, 57, 27} Primary repositioning and manipulation of the nasal septum and changing its abnormal position in infancy have a positive effect on nasal development but not on maxillary growth.²⁶

Essential technical steps

• The skeletal base is aligned by presurgical orthopedics.


• The nasal skin is undermined widely from the nostril rim below to the nasion above in order to permit easy lifting of the alar cartilage.


• The first suture is placed to raise the alar cartilage to its normal symmetric position, thereby shortening the nose on the cleft side.


• The alar cartilage must be lifted with its attached nostril lining before the nasal floor is closed and before the lip is repaired.


• Adjustment of the lift sutures is performed following completion of lip repair.

Preoperative Details

Unilateral nasal cleft repair is often performed during cleft lip closure. The author advocates this combination of procedures. Presurgical orthodontics and gingivoperiosteoplasty accomplish nasal platform symmetry (Latham), which enables columella lengthening and correction of the alar cartilage position, as well as the flaring alar base at the time of rotation-advancement of the lip.⁶⁴ Nasal correction during cleft lip repair relieves the patient of the burden of an otherwise obvious nasal deformity that is noticeable even after secondary repair. Surgical intervention early in life was once thought to inhibit natural proportional growth of nasal cartilage, but this is demonstrably not the case.

Several steps are taken prior to cleft nasal repair to ensure the best possible outcome. Once the patient is of adequate weight (often >10 lb), maxillary segments can be aligned with presurgical orthodontics. The bony structures are usually approximated within a few weeks, allowing for a gingivoperiosteoplasty. This maneuver creates a more symmetrical platform for the lip and nose by normalizing the position of the alveolar segments and closing the anterior cleft.⁵⁶ These presurgical steps allow the septum to assume a more vertical position after the alignment of the 2 maxillary segments with the use of the presurgical orthodontics and gingivoperiosteoplasty. This sets the stage for cleft lip closure and primary nasal repair by age 6-7 months.⁵⁶

Intraoperative Details

The basic technique for correction of the unilateral cleft nasal deformity involves a C-flap, which lengthens the short side of the columella. Its tip is transposed into the upper portion of the lip cutback. Through a one-sided membranous septal incision, the columella can be advanced toward the nasal tip to increase symmetry. The unilateral membranous septal incision is extended to the alar margin, and the medial two thirds of the displaced alar cartilage can be accessed.⁶⁵

This technique helps prevent scar formation which can contribute to airway obstruction. The medial cruz is reconstructed when the freed alar cartilage of the cleft side is approximated to the normal alar cartilage on the noncleft side. The lengthened columella is advanced on the cleft side. The nostril sill is reconstructed and the alar flare is reduced, providing greater symmetry. The lateral lip flap is then advanced into the rotation gap to complete the lip construction as described by Millard.⁵⁶

COMPLICATIONS

Section 8 of 10  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• References

If the unilateral columella lengthening and alar base balance do not provide adequate nostril symmetry, the growth of the nose through puberty may produce a cosmetically unacceptable result. This problem can be corrected with a rhinoplasty when the patient is aged 16 years or older.⁵⁶

OUTCOME AND PROGNOSIS

Section 9 of 10  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• References

To date, long-term evaluation and results have not been able to demonstrate any interference with nasal growth and the possible benefit of needing fewer subsequent operations for a good cosmetic result.^{25, 29, 66, 57, 27} Primary repositioning and manipulation of the nasal septum and changing its abnormal position in infancy have a positive effect on nasal development but not on maxillary growth.²⁶

In support of early cleft lip nose repair, McComb reviewed his first 10 consecutive cases of primary cleft lip nose repair after 18 years.²⁷ His results supported the observation that growth of the cleft side of the nose is unaffected by early primary nasal surgery and that the vertical shortening of the nose by the alar lift technique is preserved into adult life. Residual nasal asymmetry resulted secondary to the septal deviation.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• References

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Article Last Updated: May 7, 2007