Surgical Therapy
The surgical treatment of the cleft nose falls into 3 phases:
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Primary (at the same time as repair of the lip)
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Early secondary (in the child before skeletal maturity)
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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]
Preoperative Details
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.
Intraoperative Details
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]
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Maintain symmetry
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Secure primary muscle union
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Select the proper prolabial size and configuration
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Form the median tubercle and mucocutaneous ridge from lateral lip tissue
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Construct the nasal tip and columella by anatomic placement of the alar cartilages
LaRossa and Donath [46] emphasize the following:
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The maxilla also should be managed presurgically.
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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.
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The alar bases must be repositioned and the nasal floor repaired.
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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:
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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]
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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]
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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.
Lengthening the columella by an interdomal suture (blue) moves the lower lateral cartilages medially (red arrows) and defines the nasal tip.
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 Details
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.
Follow-up
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.
Complications
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]
Outcome and Prognosis
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:
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The columella was excessively long compared to control subjects, which led to larger-than-normal nostrils.
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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.
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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]
Future and Controversies
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.
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A diagram of normal nasal anatomy. Note the acute angle of genu of the lower lateral cartilages and the vertical orientation of the nares.
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A diagram of nasal anatomy of a person with bilateral cleft lip. Note the obtuse angle of the genu of the lower lateral cartilages and the horizontal alignment of the nares.
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Lengthening the columella by advancing skin from the prolabium (large red arrow) tends to increase the angle of the lower lateral cartilages (small red arrows) and broadens the tip.
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Lengthening the columella by an interdomal suture (blue) moves the lower lateral cartilages medially (red arrows) and defines the nasal tip.
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Intraoperative view of an open rhinoplasty on a patient with blilteral cleft lip.
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Intraoperative view of an open rhinoplasty on a patient with blilteral cleft lip, after placement of an intradomal suture.
