Bilateral Cleft Nasal Repair

Although multidisciplinary care for the people affected by orofacial clefting has undergone many advances, surgical correction of the nasal deformities associated with bilateral cleft lip remains a challenge. Various single- and multiple-stage procedures have been used. Deformities may become apparent after further growth and development of the nose, making the bilateral cleft lip nasal deformity a 4-dimensional problem.

Many early surgical approaches for repairing the bilateral cleft lip and its nasal deformity in a single stage resulted in scarring and corrections that did not last. This led to the belief that primary repair may interfere with growth of the nasal cartilages and that the nasal deformity should not be corrected in a secondary staged procedure after nasal growth is complete. Soon, evidence began to refute this belief, and some have claimed that early surgery may assist growth.

Tan et al found in a survey of surgeons listed in the American Cleft Palate/Craniofacial Association that 108 (52%) of 210) performed primary nasal repair, and 102 (48%) of 210) preferred secondary nasal repair.[1]

Many centers have presented results of children treated with preoperative orthopedic management followed by single-stage correction (see Presurgical Orthopedic Therapy for Cleft Lip and Palate). Tan et al found in their survey that 71% of surgeons would perform some form of preoperative dentofacial orthopedics for a complete bilateral cleft lip; the most common technique was nasoalveolar molding (NAM), at 55% of those administering preoperative treatment.[1, 2, 3]

The history of the surgical treatment of the bilateral cleft lip nose is long and fascinating; for a more complete treatment, the reader is directed to Millard's Cleft Craft.[4] Below are some of the influential surgeons and a brief summary of their contributions.

Manchester

Manchester described fairly simple technique for repair of the bilateral cleft lip, in which the nose is minimally repaired, if at all.[5]

Millard

Millard maintained that the columella is actually shortened because the dislocated alar cartilages have not stretched the columella properly, and he designed a repair with this in mind.[6] He begins by presurgical active orthopedics with an intraoral fixed device.[7] If insufficient prolabium is available, lip adhesions may be used to enlarge the prolabium.

At the time of primary surgery, he is then able to make gingivoperiosteoplasties on the alveolar segments. During the lip repair, he banks prolabial forked flaps in the nasal floor ("whisker" flaps). When the patient is about 4 years old, Millard advances the forked flaps into the columella.[8]

Broadbent and Woolf

Broadbent and Woolf were among the first to describe simultaneous primary repair of the lip and nose.[9] They believed that early surgery certainly did not retard growth and might actually assist it. Their experiences taught them that a good primary repair endures; however, any deformities not fully repaired do not improve with time. Their method consists of making an incision in the superior cleft defect extending between the upper lateral cartilage (ULC) and lower lateral cartilage (LLC) to the tip. The superior border of the LLC is undermined, and the skin of the nasal tip and over the ULCs is freed. They made a midline incision of the nasal tip and sutured together the domes of the LLCs, which lengthened the columella. They also relocated the LLCs by pulling them upward and medial to the ULCs with sutures.

Cronin

Cronin advocated using floor of the nose tissue to lengthen the columella as a secondary procedure.[10]

Mulliken

Mulliken advocates lengthening the columella and reconstructing the nasal tip by suturing the genu of the LLCs together.[11] He initially used a single central vertical incision over the tip of the nose to expose the genu of the LLC, but later found that incision was unnecessary, as the LLCs could be approached through rim incisions.[12] He now advocates bilateral vestibular rim incisions and alar base incisions. The LLCs are freed on their anterior surfaces through the nasal incisions. He then elevates and sutures the genu of the LLCs together. The lateral portion of each dome is suspended to the ipsilateral ULC near the septum. The freed alar bases are held to the prolabium medially via a mattress suture from alar base to base. Excess tissue in the soft triangle is excised. When necessary, a ridge of vestibular lining is excised.[13, 14, 15]

Nakajima

Nakajima reported a series of 169 patients who had primary nasal cartilages repair through rim incisions similar to Mulliken's approach.[16] Nakajima brought his incision out onto the external skin near the tip. As the LLCs are moved superiorly, this external skin is folded under to form a "soft triangle." Fifteen-year follow-up showed good results.[17]

McComb

In 1986, Harold McComb published a 10-year follow-up study of repairs he had accomplished using a 2-stage procedure.[18] It involved lengthening the columella with forked flaps taken from the prolabium when the patient was aged 6 weeks, with repair of the lip and nasal deformity performed 6 weeks later. Later, he began to notice complications associated with this type of columellar lengthening and abandoned this technique in favor of another 2-stage procedure.[19] In describing his new procedure, he noted that, embryologically, the prolabium belongs to the lip and therefore should not be used to reconstruct the columella.

In McComb's current technique, preoperative orthopedic appliances are used. In his first stage, when the patient is aged 6-8 weeks, the nasal floor is repaired and lip adhesions are performed. A V-shaped incision is made above the nostril rim with the tip of the V ending over the dorsal columella. The nasal skin is widely undermined over the LLCs through the incision in the cleft margin. The soft tissue between the domes is removed, and the domes are then sutured together. The nasal flap is closed in a V-Y advancement, lengthening the columella by approximately 5 mm. Mattress sutures over bolsters are placed to eliminate any dead space in the tip. At the second stage, 1 month later, the prolabium is lifted and mucosalmuscular flaps are sutured behind it, completing the lip repair.[19, 20]

Salyer

Salyer performs his lip repair procedure, along with a limited nasal repair, when the patient is aged approximately 3 months.[21] Initial surgery entails bilateral superiorly based prolabial flap elevation developed around the flap to be used for philtral reconstruction. These are rotated into the nasal floor after bilateral vertical intranasal alar and alar base incisions allow freeing of the LLCs and rotation of the bases medially. The lip repair is then completed.

Further nasal reconstruction is achieved when the patient is aged 12-15 months. Incisions are made below each alar base running toward the midline. Bilateral horizontal rim incisions meet in the midline and extend up the columella. Through this incision, the alar cartilages and columellar skin are freed. The alar cartilages are advanced, and the domes are sutured together. This lengthens the columella and redefines the tip through advancement of nasal floor tissue. No permanent sutures are described between ULCs and LLCs. Instead, Dacron bolsters are placed and left for 6-7 days to promote LLC support, nasal tip projection, and lateral crux stabilization, which is thought to eliminate vestibular webbing.[22]

Salyer and Genecov have published 40-year follow-up studies of techniques, with good long-term results.[23]

Noordhoff

Initially an advocate of a 2 stage procedure with forked flaps,[24] Noordhoff now uses a 1-stage lip and primary nasal repair, performed when the patient is aged 3 months.[25] The alar bases and orbicularis are sutured to the nasal spine to attempt to prevent columellar drift. The domes are also sutured together at the tip to improve tip projection.

Cutting and Grayson

In 1993, Cutting and Grayson described a prolabial unwinding flap method as a single-stage reconstruction of the bilateral cleft lip and nasal deformity, relying on presurgical orthopedic appliances and sufficient prolabial size.[26] The repair involves an oblique, asymmetrical incision beginning at one side of the columellar base and continuing inferiorly and medially. This creates a flap that forms the inferior columella and philtrum. The asymmetrical nature of this reconstruction, however, led to a very high incidence of revision surgery, and they ultimately abandoned it.[27]

In 2004, Cutting and Grayson described the use of nasoalveolar molding (NAM), using a very elegant but work-intensive molding device.[28] Their protocol begins with a passive presurgical orthopedic device, fitted to the patient’s alveoli and adjusted weekly (see Presurgical Orthopedic Therapy for Cleft Lip and Palate). They progress to add wire outriggers that press up on the collapsed nasal vestibule. This is felt to remodel the cartilage and even create increased tissue for the subsequent less-involved surgical repair of the nose.[29] Cutting addresses the nasal tip by extending the prolabial flap via a transmembranous septal incision. The medial crura of the LLC are then elevated with the columellar flap. This avoids the problem of flap ischemia with the McComb approach, and it allows suturing the genu of the LLC together from a retrograde approach.[25, 28, 30]

Delaire

Delaire, on the other hand, offered the opinion that the best orthopedic treatment is an anatomic and functional surgical repair achieved in a single stage at approximately age 6 months. The columella can be lengthened at the time of the lip repair by precise repositioning of the lower lateral cartilages and good control of the healing process. He maintains that there is no skin deficiency of the columella, but he uses a nasal retainer to preserve the patency of the nasal valve.[31]

Surgical correction of nasal deformities associated with bilateral cleft lip is challenging, as deformities may become more apparent as the nose undergoes further growth and development.

Frequency

The worldwide reported incidence of cleft lip (with or without cleft palate) varies from 0.2 to 2.3 per 1000 births. Of the individuals with cleft lip, 10-25% have a bilateral cleft. Incidence of cleft lip and palate varies by race; Native Americans have an incidence of 1 in 278 births, Asians an incidence of 1 in 325 births, Caucasians an incidence of 1 in 750 births, and Africans an incidence of 1 in 2450 births. Males are more commonly affected than females by a ratio that varies from 1.5:1 to 2:1.[32]

The cause of cleft lip with or without cleft palate is still under debate. Cleft lip can be described as either syndromic or nonsyndromic. In nonsyndromic clefting, the multifactorial/threshold model has been developed, as common mendelian inheritance patterns were not present. This model describes a genetic susceptibility or threshold at which a phenotype is expressed in an individual influenced by environmental factors.[33] This model is supported by the observation that factors altering the electron transport chain, including hypoxia, carbon monoxide, phenytoin, and fetal alcohol syndrome, can increase the incidence of cleft lip.[34] Some studies suggest that in some situations, major gene loci may be operating.[35]

Approximately 14% of cleft lips, with or without cleft palate, are considered syndromic. Trisomies 13 and 21 both affect median nasal process (MNP) development, leading to an increased incidence of clefting. In Waardenburg syndrome, neural crest cells fail to develop properly; this syndrome is associated with deafness and patchy pigmentation absence as well as cleft lip and palate. Van der Woude syndrome manifests with lip pits due to abnormal salivary glands and is associated with cleft lip and palate.[36]

The face develops from 5 processes in the human embryo: the single frontonasal process and the paired maxillary and mandibular processes. These 5 processes consist mainly of neural crest tissue and are derived from the first pair of pharyngeal arches at approximately the fourth week of gestation. The frontonasal process begins to develop median and lateral nasal prominences around an olfactory pit at approximately the 33rd day of gestation. The maxillary processes continue to develop medially toward the median and lateral nasal processes of the frontonasal process.[37] The mesodermal penetration theory of Pohlmann and Veau emphasizes the importance of mesodermal migration and support of the fusing structures, without which the epithelial layers break down into a cleft.[38]

The indication for surgery is visible nasal deformity associated with a bilateral cleft of the lip that the patient or responsible party desires to be corrected.

Nasal anatomy

The bony structure of the nasal pyramid includes the following:

• The paired nasal bones

• The frontal processes of the maxilla

• The nasal processes of the frontal bone

• The bony septum, formed by the vomer and the perpendicular plate of the ethmoid

This osseous framework is supplemented by an extensive cartilaginous system that includes the following:

• Cartilaginous septum

• The paired upper lateral cartilages (ULCs)

• The paired lower lateral cartilages (LLCs) or alar cartilages

The LLCs are made up of the following 3 parts:

• The lateral crura, which provide support to the superior portion of the nostril

• The genu or dome, which provides projection and definition to the nasal tip

• The medial crura, forming the internal structure of the columella

The nasal cavity begins at the nares, or nostrils, extending posterior to the choanae. The nasal floor consists of the palatine process of the maxilla and the horizontal plate of the palatine bone.

Nasal anatomy is shown in the image below.

Anatomy of the bilateral cleft lip nasal deformity

The following deformities of the bilateral cleft lip nose have been well described by many authors:

• Shortened columella

• Broad flat nasal tip due to obtuse angle of the genu of the LLC

• Caudally displaced medial crura of the LLC

• Caudally, laterally, and dorsally displaced lateral crura of the LLC, causing a vestibular web

• Laterally displaced alar base, leading to nostrils that have a horizontal orientation

• Nasal floor deficits

• Paucity of alveolar bone at the cleft

• Protrusion of the premaxilla, which contributes to the deformity by decreasing the nasolabial angle

Bilateral cleft lip nasal anatomy is shown below.

The nasal deformity can vary considerably. In patients with bilateral incomplete clefts, little, if any, detectable deformity may be present. In patients with severe bilateral clefting, the deformity may be profound. Asymmetry of the nasal deformity and the premaxilla may present a challenge to the operating surgeon.

More recently, Mulliken and other authors have questioned whether the columella is truly shortened or if it merely appears short because of the splaying of the medial crura. They contend that the columellar tissues reside in the nasal tip and that the distal end of the columella has been displaced by the splaying of the medial crura. They advocate a change from "skin paradigm" of treatment to "cartilage paradigm."[11, 27]

In an attempt to lengthen the columella by advancing skin, the cartilage defect is worsened (see image below).

Any infant who is a candidate for surgery should be eating and gaining weight normally and should undergo a complete physical examination to determine the extent of any concurrent medical illnesses. The child should be free of respiratory infection and without any skin infection at the time of surgery. Any anomalies that compromise the patient's intraoperative or postoperative course may be contraindications to surgery.

The "Rule of Tens" is a set of classic guidelines for the suitability of an infant for surgery.

• Aged at least 10 weeks

• Weigh 10 lb (4.5 kg)

• Hemoglobin value of 10 g/100 mL

• WBC count less than 10,000/µL

Judgment as to whether a child is suitable for surgery requires good communication and respect between the surgical and anesthesia teams.

Laboratory studies are not routinely required in healthy infants. If the child's status is in any doubt, studies may be used preoperatively to confirm the child's suitability for surgery.

Expected results of a complete blood cell count are as follows:

• Hemoglobin value of at least 10 g/100 mL

• Hematocrit value of 35%

• WBC count of less than 10,000/µL

Chest radiographs are not routinely required in healthy infants. If the child's respiratory status is in any doubt, radiographs may be used preoperatively to confirm the absence of a respiratory infection or any other pulmonary anomalies that may compromise the patient's intraoperative or postoperative course.

The surgical treatment of the cleft nose falls into 3 phases:

• Primary (at the same time as repair of the lip)

• Early secondary (in the child before skeletal maturity)

• Late secondary (in the adolescent or adult after skeletal maturity)

Aggressive primary repair has the advantages of forming the cartilage before growth occurs,[28] but this may be unpredictable; some have advocated for waiting for skeletal maturity and performing secondary correction that point.[39, 40]

A protruding premaxilla places tension across a bilateral cleft lip repair; presurgical orthopedic appliances are often applied to correct this. See the topic Cleft Lip and Palate Orthodontic Appliances. Two categories of appliances are used, passive and active.

Passive appliances maintain the distance between the 2 maxillary segments while external force is applied to encourage posterior repositioning. This external force can be external taping,[41] a head cap with elastic straps across the prolabium,[42] or a even a surgical lip adhesion. The NasoAlveolar Molding (NAM) device of Grayson and Cutting is an elaborate example of a passive orthopedic system.[43, 44]

Active appliances are fixed intraorally and apply traction through mechanical means such as elastic chains, screws, and plates. The Latham device, used by Millard, is an active orthopedic device.[7]

Presurgical orthopedic devices are a controversial topic in cleft treatment. See the discussion in the topic Presurgical Orthopedic Therapy. Arguments against their use include possible long-term growth effects, occlusal misalignment, and feeding difficulty.

Primary repair

No single procedure can successfully repair clefts of various severities. The ultimate goal of the surgery should be to achieve the best possible result by the time the child is ready to attend school, with a view to final open rhinoplasty, as necessary, at craniofacial maturity. Some principles should be kept in mind.

Mulliken has elucidated some of the principles of primary treatment of the bilateral cleft lip with nasal deformity.[45]

• Maintain symmetry

• Secure primary muscle union

• Select the proper prolabial size and configuration

• Form the median tubercle and mucocutaneous ridge from lateral lip tissue

• Construct the nasal tip and columella by anatomic placement of the alar cartilages

LaRossa and Donath[46] emphasize the following:

• The maxilla also should be managed presurgically.

• The lower lateral cartilage (LLC) should be released from any soft tissue attachments, including the vestibular lining, to prevent any tendency for the cartilages to return to their previous positions.

• The alar bases must be repositioned and the nasal floor repaired.

• Bolstered sutures or stents are useful in maintaining the final cartilage shape.

Delaire has emphasized the physiologic reconstruction of cleft lip and nasal deformities with focus on the muscle reconstruction not only of the lip but around the nose.[47]

There are 3 approaches for primary correction of the alar cartilage, as follows:

• The first is a conservative technique with no extra incisions. The alar cartilages are approached medially from the prolabium by tunnelling under the columella and laterally from the alar bases. This can be combined with a limited amount of subperichondrial nasal septal dissection to allow for the repositioning of the medial crura.[39]

• The second approach is an anterograde technique in which the prolabial incision is continued subcutaneously anterior to the medial crura up the lateral aspect of the columella. Trott and Mohan extend the columellar incision into the intranasal rim incision.[48] This allows direct access to the medial and lateral crura as in a conventional open rhinoplasty, but it may compromise the prolabial blood supply. Thomas has published a large series treated with this approach, without loss of the prolabium from ischemia.[49]

• The third is a retrograde technique with dissection posterior to medial crura using an extended prolabial incision up through the membranous septum. The alar cartilage is then accessed from behind. The advantage of this method is that the prolabial blood supply is well maintained and the medial crura can be repositioned superiorly to support the lengthened columella.[30] The disadvantage is that access to the alar dome is difficult and direct visualization of the alar cartilages is seldom possible. Cutting et al emphasized the importance of dissecting the fibroadipose tissue between the alar domes to allow for apposition of the cartilages. He combined a retrograde approach with intranasal rim incisions; this provides good access to the alar dome for direct suturing without compromise of the prolabial blood supply.[50]

Early secondary repair

Secondary repair has been directed toward columellar lengthening and narrowing the broad nasal tip. The "cartilage paradigm" is part of the modern approach to this surgery.[51, 52] Composite skin cartilage grafts from the ear may be required.[53] A useful technique in the situation of midline tissue deficiency and scarring is the Abbé cross-lip flap.[54] (For more information, see Medscape Reference article Craniofacial, Bilateral Cleft Lip Repair.)

Late secondary repair

After the patient's face is skeletally mature, the cleft nasal deformity can be treated with open rhinoplasty techniques in addition to the techniques available for early secondary repair.[55] (For more information, see Rhinoplasty, Basic Open Technique.) These techniques allow the surgeon to use more advanced techniques of structural support of the nose.[56] (For more information, see Structural Support for Correction of Cleft Nasal Deformity.) See the image below.

The use of a lining mucosal flaps,[57, 58] a V-to-Y advancement flap in the columella, and spreader grafts in the middle nasal vault[59] are useful adjunctive techniques.

Postoperative care is designed to prevent stress that may cause dehiscence. Many authors advocate 2 weeks of syringe feeding and arm bolsters with nothing placed in the mouth or over the lip and nasal base (ie, no pacifiers). Authors have shown good results without such onerous restrictions.

The repair is reviewed after 2 weeks, and the nasal stents are removed if they have not already fallen out. The wound should be assessed for any signs of infection or dehiscence.

The standard of care for treatment of children affected with cleft lip and palate is to be enrolled in a multidisciplinary clinic throughout childhood.[60] Residual deformities can be assessed at long-term team follow-up appointments to determine whether any further surgery is necessary.

One of the potential complications that must be kept in mind in operating on the nose an infant that is an obligate nasal breather has the potential for creating obstructive sleep apnea.

A possible complication that may be encountered in single-stage procedures is the loss of a portion of the prolabium. This may occur if its blood supply is compromised by simultaneously separating the prolabium from the premaxilla and performing a nasal tip dissection. Cutting believes that his retrograde approach to the nasal tip is less likely to compromise this circulation.[27, 28] Indeed, he has even shown good results combining this approach with the rim incisions of Mulliken.[61] Thomas et al showed that the open rhinoplasty techniques could be applied to primary bilateral lip repair without incidence of loss of the prolabial flap.[49, 62]

Hypertrophic scarring is possible, even in this age group. Noordhoff et al feel that early recognition and massage of the lip scar are important in prevention.[25]

One of the ironies of caring for children with clefts is that the final outcome of treatment is not known until the patients are adults, at which point the surgeon may be nearing retirement. Surgeons are, therefore, indebted to those surgeons who have the experience of large numbers of patients and who have documented their long-term results. Macomb found few unfavorable outcomes until his patients reached adolescence.[18, 19, 20] The New York University group has shown good 12-year anthropometric results using their nasoalveolar molding protocol.[63] These experiences highlight growth as the "fourth dimension" in an already complex problem.

Many of the unfavorable outcomes of cleft nasal surgery are described as occurring after the forked flap technique. McComb originally lengthened the columella with forked flaps taken from the prolabium. He found that the following 3 deformities developed as patients approached adolescence:

• The columella was excessively long compared to control subjects, which led to larger-than-normal nostrils.

• The base of the columella tended to drift inferiorly onto the upper lip, creating a more obtuse nasolabial angle and allowing the scars from the forked flap to drift downward and become more noticeable.

• The nasal tip remained broad as the cartilages continued to splay at the domes.

Cutting and Grayson describe another potential problem with using forked flaps.[26] When forked flaps banked in the whisker position are rotated into the columella, the rotation can cause the upper lip to bunch up. Additional tissue excision and reopening of old scars may be necessary to correct this complication. Additionally, it rotates a noticeable midline scar into the columella. Cutting and Grayson point out that procedures that use prolabial skin exclusively to reconstruct the columella and use the lateral lip segments to reconstruct the entire lip place tension on the lip and maxilla.[26] This tension can lead to midfacial retrusion.

Garri et al compared the result of their secondary nasal reconstruction for bilateral cleft nose; they found the results of open rhinoplasty superior to composite grafting for columellar advancement.[64] This is further evidence that the "cartilage paradigm" yields better outcomes than the "skin paradigm."

Lee et al presented a study that showed improved appearance in children with bilateral cleft lip nasal deformity treated with nasoalveolar molding (NAM).[50]  In a retrospective study of infants with bilateral cleft lip and palate in whom nasoalveolar molding was performed prior to primary reconstructive surgery, Mancini et al found that nasolabial symmetry was improved after the molding process by position changes in the subnasale and labium superius. Columella length increased from 1.4 to 4.71 mm after nasoalveolar molding.[65]

The number and variety of methods for treatment of the bilateral cleft lip nasal deformity indicate the difficult nature of the problem. Perfect anatomic reconstruction is impossible, even if completed within the first few months of life. The possibility of fetal correction of bilateral cleft lip is intriguing; however, the benefits of fetal surgery must outweigh the risks of miscarriage and fetal demise.