This article presents an overview of the cleft lip nasal deformity and its treatment. The complex pathologic changes to normal nasal anatomy are described, and treatment strategies for both unilateral and bilateral cleft lip patients are presented. The surgical technique for management of the cleft lip nasal deformity is discussed as it pertains to both primary and secondary correction.
Key points
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The nasal deformity in cleft patients is complex, and includes malpositioning and hypoplasia of the lower lateral cartilages.
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Nasoalveolar molding helps to facilitate correction of the cleft lip nasal deformity with repositioning of the cleft side ala at the time of primary lip repair.
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During primary repair, the nasal correction is completed before closure of the lip and nasal floor to avoid tethering forces.
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The staging of bilateral cleft lip repairs can provide a longer columella and sufficient lobule and tip projection.
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Secondary correction of the cleft nasal deformity uses cartilage grafts to create a new alar structure; it is not generally possible to achieve adequate elevation of the ipsilateral alar cartilage.
Cleft anatomy
Discussion of the cleft lip nasal deformity must take into account the treatment of the adjacent structures: the maxilla, the alveolus, and the lip. The cleft nasal deformity in a unilateral complete cleft has been well described by Ha and colleagues. To paraphrase, the characteristic features of a unilateral cleft nasal deformity include:
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Disruption of the muscle ring across the nasal sill;
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A splayed cleft-sided medial crus;
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Malposition and hypoplasia of the lower lateral cartilage;
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A flattened nasal dome;
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Pathologic tethering of the accessory chain of the lower lateral cartilage to the pyriform aperture; and
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Soft tissue deficiency of the nasal floor.
Other structural deformities on the cleft side include:
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A retrusive maxillary segment;
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A septum that deviates posteriorly (and toward the noncleft side);
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Abnormal insertions of the lip and cheek musculature to the alar base; and
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A vestibular lining deficiency.
Malfunction of the cleft ala external nasal valve results from:
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Alar base malposition;
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An imbalanced muscular pull; and
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Abnormal attachment of the cheek muscles to the lateral crus.
Tip projection is further compromised by a foreshortened columella, which lies obliquely with its base directed toward the noncleft side (as does the caudal septum). The tip deviates to the noncleft side, there is an obtuse angle between the middle and lateral crura, and the alar base is displaced posteriorly ( Fig. 1 ).
Cleft Lip Repair
Numerous authors have shown that preoperative nasoalveolar molding (in Millard’s words “to get the base right”) helps to facilitate rotation advancement closure of the cleft lip. Among the many benefits, this technique lengthens the lip on the cleft side, places the scar to match the contralateral philtrum column, achieves muscle reconstitution, allows for a gingivoperiosteoplasty (and closure of the anterior palate performed by some), as well as primary repositioning of the cleft side ala by the McComb technique or other ( Fig. 2 ). As such, an excellent result can be obtained that may not require further nasal surgery. Some surgeons may also choose to reposition the septum at this primary operation, and others wait.
The lip and nose are corrected at the first operation, usually completed at 6 months of age. The remainder of the palate is closed (if needed) at around 18 months, along with an extensive retropositioning of the soft palatal musculature using either a Furlow or Sommerlad technique. Alveolar bone grafting may still be required. If the soft tissue clefts of the alveolus and anterior palate are closed, however, this is an exceedingly easy procedure with a high success rate. Bone grafting can be performed at 5 or 6 years of age to provide bone for the eruption of the lateral incisor, if present.
Despite muscle repositioning, some patients may still develop velopharyngeal dysfunction. Of the available surgical treatments, the sphincter pharyngoplasty seems to be the most physiologic procedure for correction.
The Incomplete Bilateral Cleft
A bilateral cleft lip may have a complete cleft lip on one side with an incomplete cleft on the other, or may be 2 incomplete clefts. These can also be symmetric or asymmetric. This deformity is essentially 2 unilateral clefts with the previously mentioned deformities. We prefer to treat them as such, and repair the complete cleft first with a gingivoperiosteoplasty after nasoalveolar molding. The alveolar and anterior palate closure, as well as the nasal correction, are treated as described. A full, tension-free lip is obtained with a natural white roll and often a philtral dimple. After second stage closure, there will be reconstituted orbicularis oris muscle present in the prolabium.
Complete Bilateral Clefts
Most authors advocate a 1-stage procedure. Some discard significant portions of the prolabium to obtain a philtrum that is anthropometrically correct. Nothing is done to lengthen the lip, as is done in a unilateral cleft. Millard advocated delayed columellar lengthening with forked flaps, but most of the authors mentioned have been able to obtain adequate columellar length without them.
Almost all of the 1 -stage procedures that we have seen, including our own, have the following characteristics:
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Short upper lip height (often 5–6 mm);
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A tight upper lip that lays posterior to the lower lip on lateral view;
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Inadequate upper buccal sulcus despite a turndown of the prolabial vermillion;
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A missing or abnormal white roll; and
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A nasal deformity consisting of inadequate lobule and inadequate tip projection.
Because we were pleased with the results obtained with incomplete bilateral clefts, we began applying this staged technique for complete bilateral clefts. Because we feel that a bilateral cleft is no more than 2 unilateral clefts (with the single exception that there is no native muscle present in the prolabial segment), we treat a complete bilateral exactly as we do a unilateral: nasoalveolar molding and gingivoperiosteoplasty when permitted, followed by anterior palate closure, McComb nasal correction, and rotation advancement. The wider cleft side is repaired first, followed by the second stage 3 months later ( Fig. 3 ).
