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
- •
The nasal deformity in cleft patients is complex, and includes malpositioning and hypoplasia of the lower lateral cartilages.
- •
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.
- •
During primary repair, the nasal correction is completed before closure of the lip and nasal floor to avoid tethering forces.
- •
The staging of bilateral cleft lip repairs can provide a longer columella and sufficient lobule and tip projection.
- •
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:
- 1.
Disruption of the muscle ring across the nasal sill;
- 2.
A splayed cleft-sided medial crus;
- 3.
Malposition and hypoplasia of the lower lateral cartilage;
- 4.
A flattened nasal dome;
- 5.
Pathologic tethering of the accessory chain of the lower lateral cartilage to the pyriform aperture; and
- 6.
Soft tissue deficiency of the nasal floor.
Other structural deformities on the cleft side include:
- 7.
A retrusive maxillary segment;
- 8.
A septum that deviates posteriorly (and toward the noncleft side);
- 9.
Abnormal insertions of the lip and cheek musculature to the alar base; and
- 10.
A vestibular lining deficiency.
Malfunction of the cleft ala external nasal valve results from:
- 11.
Alar base malposition;
- 12.
An imbalanced muscular pull; and
- 13.
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:
- 1.
Short upper lip height (often 5–6 mm);
- 2.
A tight upper lip that lays posterior to the lower lip on lateral view;
- 3.
Inadequate upper buccal sulcus despite a turndown of the prolabial vermillion;
- 4.
A missing or abnormal white roll; and
- 5.
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 ).
With the staged technique of bilateral cleft lip correction, we found the noses to be essentially normal. There was adequate columellar length and sufficient lobule and tip projection. The nasolabial angles were also found to be normal. In addition, the presence of a laxer, fuller upper lip allows maxillary development to proceed impeded.
Technique of Primary Nasal Correction
As indicated, the technique is the same whether one is dealing with a unilateral or bilateral cleft. We prefer to operate at 6 months of age.
The lip is marked for a rotation advancement repair, marking the high point of Cupid’s bow and measuring the distance from commissure to high point of the Cupid’s bow on the noncleft side. This measurement is transposed to the cleft side and marked. Nordhoff’s point (the last robust portion of the white roll) is marked, and may result in a slightly shorter transverse dimension of the lip on the cleft side. The rotation segment is incised with an adequate cutback directed toward but not across the contralateral philtral column. The advancement segment is incised, with a transverse incision extending across the nostril sill as far as, but not around, the alar base. The muscle is dissected extensively from the skin of the advancement segment, and only for a few millimeters on the rotation segment.
If nasoalveolar molding has been performed and the maxillary segments are 2 to 3 mm apart, a gingivoperiosteoplasty (in reality the dissection is supraperiosteal) is performed with turndown flaps from the alveolar segment extended back into an anterior vomer flap. This is completed so that the palatal surface of the alveolus and first 10 mm or so of the anterior palate are closed.
The next step in sequence, as advised by McComb is important: the nose is corrected before closure of the lip and the nostril floor. If the nose is done last, the lip closure may tether down the slumped alar cartilage.
The ipsilateral lower lateral cartilage and a portion of the contralateral are dissected completely from overlying skin with small, blunt-tipped Iris scissors. This is done through the incision at the base of the columella and at the alar margin. This dissection is carried up to the nasion and to the ipsilateral nasofacial junction. A Keith needle with a 4 to 0 Vicryl suture is passed through the desired position of the crus of the alar cartilage, usually about 3 mm lateral to the columella, and brought out through the skin at the contralateral nasion ( Fig. 4 ). This suture may need to repeated several times until it provides the exact slight overcorrection desired. When this has been accomplished, the correction is “locked-in” with vestibular effacement sutures that begin intranasally, pass through the alar crease, and then pass back through the same hole in the skin at a different angulation. Three to 4 of these sutures are passed to obliterate the vestibular web and provide a corrected ala and defined alar crease.
Once the alar cartilage has been corrected, a superiorly based mucosal flap is dissected from the vomer and septum using the same incision used to turn down the vomer flap. The septum is exposed at this point and can be straightened if required. The inner surface of the advancement flap is now dissected back to the turbinate and freed from the pyriform rim. These medial and lateral flaps can now be sutured together to close the nostril floor, with care being taken to maintain an adequate nostril caliber. This closure is brought all the way to the transverse nostril sill, using the Millard C flap to constitute a medial foot plate (it is not needed to lengthen the hemicolumella). Symmetry of the alar bases should be present. The buccal mucosa is then closed. Transverse suturing of the orbicularis muscle is performed, the white roll tattoo marks are sewn together, and the vermillion is closed in eversion. Finally, skin closure is carried out with 7-0 Vicryl after any required trimming is completed to have the incision perfectly match the contralateral philtral column. Lengthening of the lip is provided by an adequate cutback, and no triangular flaps should be required for lengthening ( Fig. 5 ).
