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The anatomy of the nose

Nose deformity

When a child is born with a cleft nose or cleft lip, the nose can also be involved. The extent to which the nasal deformity exist depends on whether or not the cleft is complete, and whether the cleft is uni- or bilateral. The nose deformity in an unilateral, complete cleft lip consists of a displacement of the nasal tip, which is tilted away from the cleft. Also the nasal septum remains attached to the nasal spine but is curved away from the cleft. The lower lateral cartilage is splayed out and therefore has an M configuration rather than a normal C shape. This cartilage also is displaced caudally, so there is no overlapping with the upper lateral cartilage anymore. When compared to the non-affected site, the lower lateral cartilage is smaller and the nasal bone has a more obtuse angle to the maxilla. Another notable malformation is the short hemi-columella .

Besides the nasal cartilage, also the bone which forms the nasal floor is affected. In minor clefts it may be slightly wide and atrophic, but in more severe clefts it is absent. This has a direct impact on the vestibular lining and its intimate relationship to the alar cartilages .

In bilateral cleft patients, the problems above are present on both sides of the nose.In most cases the deformity is symmetrical, though sometimes the clefting process is different which leads to an asymmetrical nasal malformation. But in bilateral clefts, the premaxilla is a very important structure which defines the severity of the cleft. As the premaxilla becomes more protrusive, the columella nasi is shortened, along with the medial crura of the lower lateral cartilages. The lateral cartilages are pulled to the side and backwards, which results in a separation of the nasal tip. The nasal floor suffers the same effects as in the unilateral cleft, depending on the severity of the cleft .

These deformities lead to functional problems involving breathing and speaking and therefore the nose needs to be reconstructed. This is done in two surgeries, the first performed at the age of 2 or 3 months, together with the definitive lip repair. The second surgery, when necessary, is performed in the adolescence.

Primary Nasal Correction

Unilateral Clefts

Nasal correction is performed at 2-3 months of age in conjunction with lip closure. It is important for the future shape of the nose that the lip is repaired with the Millard rotation-advancement technique which is described above. This technique facilitates the repair of the muscle layer at the level of the nasal spine, which takes care of the right position of the nasal floor. Elongation of the hemi-columella is also obtained with this procedure, whereas the incision provides good access for the alar dissection. The nasal dissection begins on the medial side. The two medial crura are separated from each other with fine iris scissors, entering at the base of the columella. This undermining is continued to the dome superiorly and under the mucosa posterior to the columella. The base of the ala is freed from the maxilla through the lateral lip incision. The dissection then proceeds subcutaneously to the nasal bones and medially to the alar dome on the opposite site. Inferiorly, the alar cartilage is freed from the skin, extending the undermining around the alar rim from the base of the ala to the medial crus and continuing inside the nostril to the caudal edge of the alar cartilage. At this point, the cartilage remains attached to the nasal mucosa only and can be easily mobilized to a normal position. Then the mucosa of the lateral nasal wall is liberated until the alar base can be rotated medially to the columellar base without tension. The tip of the flap made by the alar base is sutured to the nasal mucosa at the back and to the lip and the columellar skin on the front side. To maintain the position of the cartilage three or four pull-out sutures are used. Three sutures are usually necessary, at the vertex of the dome, at the midsection of the ala, and at the tip of the lateral alar. The sutures are fixed to the skin with tape and are removed in 3 days. The results of the nasal correction are clear immediately; the alar domes and the nostrils become symmetrical, and the cartilage shifts to a higher position, similar to the normal side. When properly performed, the nasal correction surgery produces permanent results without any alteration of nasal growth.

Bilateral Clefts

The technique to operate a bilateral cleft is similar to that for unilateral cleft repair, but a little more difficult because there is no normal side anymore so there is no point of reference. The alar cartilages are broadly freed from the skin and form the upper lateral cartilages, the domes are mobilized medially and sutured to each other, and the alar bases are rotated medially. The alar cartilages are shifted to a normal, more cephalic position with pull-out sutures. The short columella is a problem which can’t be solved yet during this procedure. A following procedure is necessary.

Secondary nasal correction

Unilateral clefts

The unilateral cleft lip nose presents the following problems: a descended alar cartilage, a decreased projection and lateralization of the alar dome, notching of the alar rim, an asymmetrical nostril base, severe septal deviation that includes all segments. The aims of the operation are the same as in a conventional rhinoplasty, namely to produce the optimal aesthetic and functional result. This operation takes place from age 3 to adolescence. The alar cartilage-rim-base is repositioned, and a complete septoplasty is performed. To avoid teasing, significantly deformed cleft noses are treated prior to entering school. At this age the alar cartilage is sufficiently well defined to maintain support of the alar rim after suture plication. Various techniques are used, which technique is used depends on the degree of the deformity. In mild deformities a technique with a cleft sided rim incision with mobilization of the alar cartilage from mucosa and overlying skin is used. The cartilage is then symmetrically repositioned, either with complete stent sutures to the overlying skin or by plication to the opposite dome cartilage. When more significant correction is required, an open tip rhinoplasty is used. This includes removal of the skin of the nose by making an incision at the columellar base. Now the nasal skeleton lies free, so there is a good access to the nasal septum. In the older child or teenager, greater attention is given to cartilage shaping, dome suturing and plication, thinning of subcutaneous tissue and fat found between the cartilages, cartilage augmentation (which are auricular cartilage grafts to a weak cleft-sided alar cartilage in particular), osteotomies, and septal realignment to effect a more aesthetic result.

Bilateral clefts

Secondary correction of the bilateral cleft nose deformity is done by the same steps as at the unilateral. But in some cases there is not enough cartilage to make a perfect projection of the nose tip. In this case some septal, auricular or rib cartilage grafts are needed. Septal deviation is common but is usually less severe than in unilateral clefts, but a septoplasty is required in almost every case, providing excellent grafting material. An explicit problem in bilateral cleft patients is lengthening of the columella. There are many techniques to achieve more tissue volume of the columella. But overall, it seems clear that skin of the nasal floor, the upper lip or both is needed, which can be rotated. In the Cronin technique, a V-Y plasty is used to lengthening the columellar tissue. This means that there are made incisions in the lines of a V, and after sliding the two segments apart, these incisions are closed in the lines of an Y. The incisions are made at the base of the columellar, which forms an inverted V. Another technique is the Millard forked flap technique. This technique is based on two forked flaps on each side of the columella, which are sutured in the midline. With this technique, the nostrils are rotated a little bit.