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." [16] Despite multiple technical procedures described, no one protocol has proven to be completely satisfactory in the repair of all cleft lip nasal deformities. [60, 21] Still, controversy remains as to the optimum corrective approach, the best techniques for exposure and repair, and, most significantly, the timing of the correction. [61]
Some surgeons believe that early nasal surgery (1) interferes with growth, resulting in nasal hypoplasia; (2) introduces scars, making secondary correction difficult [62] ; (3) damages infantile cartilage; and (4) makes repair technically harder because of the small size of the nose and immature cartilage. [4]
Reconstructive surgeons historically have been reluctant to perform rhinoplasty on a growing nose; however, the use of prudent operative techniques has created growing acceptance to correcting nasal deformities prior to puberty. [20] With less traumatic techniques, a loss of integrity of the cartilaginous nasal framework does not usually result in growth inhibition in the region of the mid face when the septum is not subjected to aggressive resection. [25] While primary repositioning and manipulation of the nasal septum and changing its abnormal position in infancy have a positive effect on nasal development, it may have a negative effect on maxillary growth. [63, 29]
Preoperative Details
Unilateral nasal cleft repair is often performed during cleft lip closure. The author advocates this combination of procedures. Presurgical orthopedics with or without gingivoperiosteoplasty accomplish nasal platform symmetry, 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.
Several steps are taken prior to cleft nasal repair to ensure the best possible outcome. [65] Maxillary segments can be aligned with presurgical orthopedics. The bony structures are usually approximated within a few weeks, allowing for a gingivoperiosteoplasty if desired by the surgeon. 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. [66] This may be combined with nasal molding as described by Grayson. [67]
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 nasal molding. This sets the stage for cleft lip closure and primary nasal repair by age 6-7 months. [66] While nasal alveolar molding (NAM) improves the nasal shape both preoperatively and immediately after surgery, long-term results are less definitive. [68, 69] Although the general perception is that NAM has a positive effect on the cleft nose morphology, there is variability in both protocol and outcome between different treatment centers. [70, 71] Further long-term studies are needed to confirm the efficacy of this intensive early intervention. [72, 73, 74, 75] Presurgical NAM should not be considered adequate to replace rhinoplasty at the time of primary lip and nose repair. [76]
NAM also has the added burden of weekly visits for the patient and the parents; it should be tailored to a select population. Alternatives to presurgical orthopedics include lip adhesion and external lip taping. While these methods do not improve nasal shape directly, they help to narrow the cleft and makes for a less tense closure at the time of lip and nose repair.
Intraoperative Details
Primary cleft nasal deformities
Current surgical practice is to repair the nasal defect at the time of the primary lip repair. While surgical techniques varies between surgeons, [77] the general concepts are as follows:
-
Freeing the abnormal nasal cartilage from the overlying skin envelope to correct the slumped lower lateral cartilage
-
Supraperiosteal undermining over the maxilla to adequately mobilize the lateral nasolabial elements
-
Suturing the dome and/or suspending the lower lateral cartilage to the upper lateral cartilage [78] to reconstruct the nasal tip
-
Centralizing the caudal portion of the cartilaginous septum without aggressive resection
-
Controlling the width of the alar base with alar cinch sutures
-
Closing the nasal floor
-
Reconstructing the nasal sill with lateral lip element advancement
-
Transmucosal-transcutaneous effacement suturing to eliminate vestibular webbing, if necessary
Access to the nasal cartilaginous framework does not require external incisions. Aside from the incisions used for the cleft lip repair, and intranasal incision carried upward just anterior to the inferior turbinate releases the lateral crus from the piriform and helps to centralize the deformed lower lateral cartilage. A rim incision can be used if interdomal suture placement for tip support is desired, especially when a resection of the rim is contemplated to refine nostril symmetry primarily. Although the piriform rim is routinely depressed and retruded, adequate mobilization of the alar base and careful closure of the orbicularis muscle may prevent postoperative alar depression. [79]
Children who underwent primary nasal repair at the time of lip repair had improved symmetry and more balanced-growth than children who did not received primary nasal repair, with increased nasal tip projection, decreased nasal width, and near normal columellar length in the first 3 years of life. [80]
Despite different techniques used, the results usually deteriorate in time, even with intentional over-correction at the time of primary repair. This may be because of scar formation, a difference in configuration between the ala on the cleft side and contralateral side, a discrepancy between the lining of the nose and the skin coverage, [20] or differential nasal growth between the cleft and the noncleft sides. [68] The images below show a cleft lip and nose repair.
The nasal cartilage is displaced laterally, inferiorly, and posteriorly, and has inadequate form and support.
Cleft nose repair at the time of cleft lip repair with rim incision, interdomal suturing, and alar base cinching. These techniques restore the position and tip support for the cleft nose.
Secondary cleft nasal deformities
Currently, secondary rhinoplasty is the norm rather than the exception when it comes to cleft lip and nose deformity. [81] Ideally, the timing of the definitive secondary rhinoplasty should be when the patients are at bony maturity and all necessary orthodontic and orthognathic procedures have been completed. [82] The appearance of the nose depends not only on its form but also on its cephalometric relation to the facial skeleton. Advancing the maxilla after a definitive rhinoplasty is akin to pulling out the foundation after a house has been built. Careful counseling of young adults with cleft lip and nose in this situation is crucial, as many are rightfully anxious to proceed with their secondary rhinoplasty.
Secondary nasal deformities may be separated by regions into the dorsum, the tip, and the ala. Each region has algorithms to address issues of form, symmetry, and projection. [8] Historically, unit arrangement was used to address deformed nostril 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. [3] This technique has largely fallen out of use for secondary cleft rhinoplasty because of the unpredictability of the external nasal scars.
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 various 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. [83, 84, 20, 67] The major advantage is that it avoids an external scar. Early results from this approach appeared cosmetically acceptable; however, the structural support elements are bolstered and can show signs of deterioration.
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. [85] The external approach perhaps started with Duffenbach in 1845. [86] Multiple variations have been described and modified. [5] The nasal approach, or "flying gull wing" incision, is placed on the infratip of the nose. [87] 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. [13] This approach has proved popular, and several authors modified it over the next half-century. [1, 88] 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. [89, 84] 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. [90]
Regardless of surgical incision, a combination of nasal osteotomies, cartilage rearrangement, septoplasty, and rib/septal/ear cartilage struts and grafts are needed to create symmetry, a more refined tip, improved projection, and better nasal airway in the patient with cleft nasal deformities. [8, 82]
Postoperative Details
After the surgery, patients are generally kept overnight in the hospital for pain control and airway observation. In the younger patients, they must be able to maintain hydration orally prior to discharge from the hospital. Bottle feeding should not have a negative effect on postoperative wound healing, but the use of pacifier should be discouraged. The use of elbow splints helps to discourage very young patients from playing with the incisions. The authors routinely use preoperative antibiotics and postoperative oral antibiotics for 5-7 days to prevent nasal tip infections.
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. [15] Wong et al described implanting a resorbable plate as an internal nasal splint but had problems with postoperative exposure and extrusion. [91] 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. [19] The use of postoperative nasal silicone splints is also believed to preserve the correction. [92] The authors occasionally employ postoperative silicone splinting in primary nasal repairs and use either silicone splints or nasal packing more routinely in secondary rhinoplasties. If aggressive undermining of the nasal skin was performed, external nasal splints (Aquaplast or similar) are also helpful to immobilize the redraped skin.
Follow-up
Postoperative patients are seen on a weekly basis until the surgeon is satisfied with healing. Cleft lip and nose repair is a major event for many parents and families, since the patient looks very different after surgery. Availability and reassurance in the immediate postoperative period are important as families adjust to their "new" baby. Routine follow-up with a comprehensive cleft team on an a frequent basis is necessary for multidisciplinary evaluation of the child's growth and development.
Complications
The most common complication in cleft rhinoplasty is asymmetry. Patients and their parents should be counseled extensively regarding expectations and the need for future procedures. 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. [83]
Intraoperative bleeding and anesthesia-related complications such as postoperative laryngospasms, aspiration, and prolonged hospitalization should be discussed. Nasal tip infection is yet another potential complication due to multiple sutures placed to maintain protection. Despite careful surgical techniques, postoperative scar formation is unpredictable and can result in obvious hypertrophic scarring.
Outcome and Prognosis
The approach to the cleft lip nasal deformity has evolved over the years. The previous hesitation of primary nasal repair at the time of lip repair has largely been assuaged by long-term studies that have shown no detrimental effects on nasal growth with early surgery. In support of early cleft lip nose repair, McComb reviewed his first 10 consecutive cases of primary cleft lip nose repair after 18 years. [30] 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.
Nonetheless, even with primary repair, most children need secondary nasal revisions of varying magnitude. Definitive open septorhinoplasty should be delayed until the teenage years and after maxillary advancement, should it be necessary. [93]
Future and Controversies
Controversies still exist regarding presurgical orthopedics, nasal molding, the optimal timing of surgery, what technique to use, and how aggressive the surgical intervention should be prior to skeletal maturity [94] . However, the ultimate goal for children with cleft lip, nose, and palate is to "achieve optimal facial balance and harmony, normal speech, beautiful smile, and full dentition with normal occlusion, resulting in an attractive face with stigmata of clefting at a conversational distance." [9] The future for the plastic surgeon is to achieve this goal with the fewest procedures and the lowest burden on patients and their families.
-
Typical appearance of cleft nasal deformities.
-
Cleft nasal deformity can be seen even in patients with incomplete cleft lip.
-
The nasal cartilage is displaced laterally, inferiorly, and posteriorly, and has inadequate form and support.
-
Cleft nose repair at the time of cleft lip repair with rim incision, interdomal suturing, and alar base cinching. These techniques restore the position and tip support for the cleft nose.
-
Additional view of patient shown above.
