Unilateral Cleft Nasal Repair

The face is composed of soft tissue over an underlying skeletal framework. This construct allows for complex facial animations and expressions. The nose, positioned at the center of the face, is the most prominent facial feature. The face of a child with cleft lip and nose often draws attention that negatively impacts the child’s psychosocial development. In current practice, plastic surgeons are better able to reconstruct the faces of children with cleft lip and nose to near-normal anatomic form and physiologic function. ^[1] With early surgical correction, children born with cleft lip and nose have the chance to develop positive social interactions and better self-esteem early in life, along with good speech and attractive smiles.

Cleft nasal images are shown below.

While multiple techniques are available for the repair of cleft nasal deformity, the universal surgical principles are to restore the normal nasal anatomy and function affected by clefting and to anticipate the secondary deformities that appear with subsequent growth and development. The optimal timing for the reconstruction of the cleft lip nasal deformity was once unresolved. ^{[2, 3, 4, 5, 6]} Multiple long-term studies published by experienced cleft surgeons now show that early nasal reconstruction has no adverse effects on growth. ^{[7, 8, 9]} The current standard of care is to reconstruct the nose at the time of primary cleft lip repair. For information on cleft lip repair techniques, see Medscape Reference articles Unilateral Cleft Lip Repair and Craniofacial, Bilateral Cleft Lip Repair.

Although plastic surgeons continue to improve outcomes in the management of cleft lip and nose defects, secondary corrections and revisions are still common. The families of children with cleft lip and nose should be counseled that while primary nasal repair sets up a "more normal" vector for growth, their child may benefit from a touch-up revision before the school years and, ultimately, a formal rhinoplasty at the conclusion of orthodontic/orthognathic care. The ultimate goals of surgical care for children with cleft lip and nose are an aesthetic facial appearance, a beautiful smile, acceptable speech, and normal psychosocial development.

The word harelip or lagocheilos is attributed to Galen. ^[10] 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. ^[11] Straight-line closures of the mid-1800s were later modified by Hagedorn, who advocated the idea of interdigitating flaps for closures. ^[12] Blair and Brown ^{[13, 14]} and Brown and McDowell ^[15] 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. ^[15] 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. ^[16]

Until recently, the result of cleft lip nasal repair received less attention by plastic surgeons. ^[11] Some early cleft surgeons, such as LeMesurier and Dennis Browne, admitted "complete nasal abdication." ^[17] 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." ^[14]

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." ^[13]

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. ^[16]

Early plastic surgeons delayed nasal repair because they believed the deformity would naturally improve itself with eventual growth. ^[18] In addition, primary attempts at nasal tip repair were temporary and frequently ineffective. ^{[19, 20, 21]} 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." ^[22] 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." ^[23]

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." ^[24] Opponents of early surgical intervention believed that it prevented the completion of the physiologic growth of the nasal cartilage. ^{[16, 25]}

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. ^[26]

As specially trained reconstructive surgeons used more uniform protocols to repair cleft nasal deformities, primary repair of nasal deformities received increased attention. ^[27] Additional clinical evidence proved more extensive surgery could be performed on children than previously believed, without interfering with future nasal cartilage growth and development. ^{[28, 29, 30, 31]} Nasal repair at the time of the primary lip repair was preferred for various reasons. ^[32] According to Gelbe, "The nose should no longer be overlooked or relegated to a separate operation in the future." ^[33] 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." ^[17]

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. ^[34] 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. ^[30] In 2003, Salyer et al published their 33-year experience with unilateral cleft lip-nose repair, further advocating primary repair of the cleft nose. ^[9]

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. Most surgeons believe early primary repair is necessary, is durable, and does not interfere with nasal growth and development. ^{[35, 7, 9, 36, 37]} More importantly, patients are often freed from years of childhood and adolescent embarrassment. ^[23]

The most recent literature reports that nasal reconstruction at the time of primary lip repair improves the immediate appearance of the nose and has a positive effect on long-term growth, as the trajectory of the nasal growth is altered and less severe curvature of the nose is observed in the adolescent years. ^{[38, 39, 40]}

See Embryology.

Frequency

The incidence of cleft lip and palate is around 1:1000, with a racial preponderance toward Asian and Hispanic populations. In unilateral clefts, left-sided cleft lip is more common than right. Fifty percent of all unilateral clefts involve both lip and palate, while only 20% of unilateral clefts involve lip only. ^[41] The incidence of cleft palate deformities is higher in populations who have a familial history of a similar deformity. ^[42] Nasal deformity is seen with almost all cleft lips, with exceptions in the very mild clefts in which the ala base is unaffected.

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. ^[43] 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. ^[35] 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 cleft lip and nasal abnormalities are interested in anomalies that result 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 pharyngeal arches on either side of the pharyngeal 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 pharyngeal clefts and internally by endoderm lying in pharyngeal 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 plaque-like 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. ^[44] 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. ^[35]

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. ^[45] 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. ^{[34, 44]}

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. ^[46] According to this concept, when tissue susceptibility crosses the pathologic threshold, a cleft forms. ^[47]

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 (see the image below). ^[48]

See the list below:

• 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 descriptions of the anatomy and morphology of these malformations have been well described by Huffman and Lierle. ^[49] More asymmetry leads to a more noticeable nasal defect. These abnormalities become even more apparent as the child matures. ^[43] The nasal asymmetry peaks during the postpubertal growth spurt. ^[50] Deviation of the nasal tip ends at the pubertal growth spurt.

Previous controversy existed regarding whether a deficiency of tissue is present on the cleft side or whether an imbalance exists in the arrangement of the tissue. 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. ^[51]

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. ^[49] Histological studies by Atherton show the cartilage on the cleft side to be similar to the cartilage on the noncleft side. ^[52] 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. ^[52] A recent anatomic study by Li et al confirmed that, aside from the deformation and displacement of the cleft nasal cartilage, the difference in size and weight of cleft versus noncleft lower lateral cartilage is not significant. ^[53]

Unilateral clefting, both complete and incomplete, results in a nasal deformity that may be caused by one or a combination of 3 major anatomic factors: (1) imbalance of the facial musculature, (2) hypoplasia of the skeletal base, and (3) asymmetry of the skeletal framework. ^[54]

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. ^[44] 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. ^[23] 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 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. ^[31] These further enhance the clinical findings characteristic of the cleft lip nasal deformity. Salyer has emphasized the importance of recognizing these skeletal abnormalities. ^[32] McComb further stressed the value of repairing bony abnormalities to achieve a successful nasal repair. ^[28] 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.

Bony problems in patients with repaired clefts may be caused by the surgery itself. ^[55] The absence of maxillary hypoplasia and class III malocclusion in patients with unrepaired cleft palates suggests the surgical procedures used to correct cleft lip and palate may impair maxillary growth. The resulting midface deficiency also may manifest at the nasal level. ^[1] The concave facial profile is yet another characteristic appearance of the patient with cleft lip and nose as mandibular growth takes off in adolescence and the maxilla fails to keep up, resulting in a class III malocclusion.

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. ^[56]

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. ^[57]

Although studies have demonstrated that the cleft nose grows with advancing age, it remains 30% smaller than the noncleft nose. ^[58] This encourages mouth breathing and continued physiologic difficulties.

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. ^[48]

See Embryology.

The cleft lip nasal deformity is of significant concern initially to parents and families, and, later on, to the patients. The primary indication for surgical repair is appearance-related deformity. Patients may also have feeding difficulties, middle ear disease, impaired speech, oronasal fistula, anomalous dentition, and persistent cosmetic deformities. Secondary revisions are often indicated for secondary appearance deformities and nasal breathing obstructions.

See Pathophysiology.

Contraindications to primary surgical correction of the cleft nose are generally related to the patient's overall poor medical status or other anomalies that require further workup, such as cardiovascular or pulmonary problems. Poor nutritional intake and failure to thrive should also be resolved prior to surgery. In secondary rhinoplasty, a patient’s physical, developmental, or emotional immaturity or desire not to undergo nasal revisions should be considered contraindications to surgery.