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. Many centers more recently have presented results of children managed with preoperative orthopedic management followed by single stage correction (see Presurgical Orthopedic Therapy for Cleft Lip and Palate).

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.^[1] 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.^[2]

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.^[3] He begins by presurgical active orthopedics with an intraoral fixed device.^[4] 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.^[5]

Broadbent and Woolf

Broadbent and Woolf were among the first to describe simultaneous primary repair of the lip and nose.^[6] 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.^[7]

Mulliken

Mulliken was initially an advocate of the forked flap for reconstruction of the columella;^[4] He found the forked flaps then to be unnecessary, as he now lengthens the columella and reconstructs the nasal tip by suturing the genu of the LLCs together.^[8]

He also now advocates removing all of the prolabial skin except for a single central flap for reconstruction of the philtrum. Bilateral vestibular rim incisions, and alar base incisions are made. 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.^[9] The LLCs are freed on their anterior surfaces through the nasal incisions. The nasal floor, orbicularis oris, and lip repairs are then completed. The genu of the LLCs are then elevated and sutured 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. Labial closure is then completed.^{[10, 11, 12]}

Nakajima

Nakajima reported a series of 169 patients who had primary nasal cartilages repair through rim incisions similar to Mulliken's approach.^[13] 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.^[14]

McComb

In 1986, Harold McComb published a 10-year follow-up study of repairs he had accomplished using a 2-stage procedure.^[15] 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.^[16] 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.^{[16, 17]}

Salyer

Salyer performs his lip repair procedure, along with a limited nasal repair, when the patient is aged approximately 3 months.^[18] 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.^[19]

Salyer and Genecov have published 40-year follow-up studies of techniques, with good long-term results.^[20]

Noordhoff

Initially an advocate of a 2 stage procedure with forked flaps,^[21] Noordhoff now uses a 1-stage lip and primary nasal repair, performed when the patient is aged 3 months.^[22] 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.^[23] 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.^[24]

In 2004, Cutting and Grayson described the use of nasoalveolar molding (NAM), using a very elegant but work-intensive molding device.^[25] 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.^[26] 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, 27, 22]}

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.^[28]

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.^[29] 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.^[30] Recently, some studies suggest that in some situations, major gene loci may be operating.^[31]

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.^[32]

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.^[33] 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.^[34]

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."^{[8, 24]}

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.