Cleft Lip Nasal Deformity - Breach in Confidence

What Is Cleft Lip Nasal Deformity (CLND)?

Cleft lip nasal deformity (CLND) is an abnormality of the nose often seen as an associated complication in patients with a congenital cleft lip, with or without a cleft palate. CLND patients suffer from cosmetic problems and impaired nasal airflow.

A nasal deformity can be present in individuals who do not have a cleft lip. However, it is mostly seen in patients with a cleft lip, and the surgeries done to correct the cleft lip seem to influence the extent of the nasal deformity, which is why it is termed as cleft lip nasal deformity. Treating patients with CLND is not a one-time affair; it is a complicated problem that needs to be addressed during multiple stages of the patient's life.

What Causes CLND?

CLND is primarily a birth defect that affects the soft tissue and the skeletal foundation in the individual. A couple of factors combine to deform the nose, they are-

1. Discontinuous Muscle Attachment - A muscle called orbicularis oris encircles the mouth, in healthy individuals, this muscle asserts equal force on the right and left side of the mouth, which is essential to perform motions related to the mouth in a symmetrical fashion.

In patients with a unilateral cleft lip, this muscle is essentially split either on the left or right side of the upper lip, disturbing normal physiology, and this also creates an imbalance in the force exerted by the muscle. As a result, the muscle on the cleft side of the lip retracts the lip and its attached side of the nose in an inferolateral direction. This retraction changes the shape and position of the nostril on the cleft side.

2. Irregular Cartilage - Soft tissues in the body (muscles, cartilage, ligaments, fat, etc.) modulate themselves according to the underlying bone. Patients with CLND have deviated nasal septum and retarded bone development (especially in the upper jaw).

To accommodate the altered septum, the cartilage in the lower end of the nose (lower lateral cartilage) on the cleft side becomes longer on the lateral side and short on the medial side, creating a wider dome appearance.

3. Maxillary Hypoplasia - Patients with cleft lip have retarded maxillary (upper jaw) growth, and this leads to poor skeletal support at the base of the nose leading to a flat and displaced nose extremity on the cleft side.

4. Deviated Nasal Septum - A deviated nasal septum, when combined with the unopposed force of the orbicularis oris muscle, will bend the septum on the cleft side. This reduces the nasal aperture leading to airway obstruction on the cleft side.

What Are the Symptoms of CLND?

The nasal deformity of a patient with CLND can be easily identified in the physical examination; the extent of the deformity is different for each individual. The following are a few features of a deformed nose-

• Malformation of the nasal tip (columella).

• Malposition of the ala (wing) of the nose.

• Deformities in the nasal pyramid and septum.

• Difficulty in breathing.

• Presence of scar tissue due to previous surgical interventions.

How Is CLND Treated?

Treating a patient with CLND is highly challenging because the deformity involves the skin, cartilage, mucosa, and skeletal platform. Cleft lip in patients with CLND is repaired as early as three months, and some surgeons prefer to perform rhinoplasty to treat the deformed nose at this time; some, however, prefer to do the rhinoplasty at a later stage to prevent any interference in the normal growth of the underlying bone.

Ideally, cleft lip nasal deformity is done in three stages-

Primary Rhinoplasty:

• This is done at the same time as the primary cleft lip repair. The idea behind this step is that early intervention allows the restoration of the shape and the potential for symmetrical nasal growth.

• In primary rhinoplasty, the lower lateral cartilage (triangular-shaped cartilages at the tip of the nose) are released, undermined, and repositioned to provide a better contour and symmetry of the nasal tip.

• The cleft lip and the lower lateral cartilage on the deformed side are treated as a unit, and the cleft lip incisions are extended into the lower lateral cartilage, which allows for differential movement of the ala and the lower lateral cartilage on the cleft side.

• After repositioning the nasal base and repairing the cleft lip, transnasal sutures are placed between the lower lateral cartilage on the cleft side and the upper lateral cartilage on the opposite side.

• These sutures create better symmetry and projection of the cleft-sided nasal dome.

Intermediate Rhinoplasty:

• It is done between the age of four to six years, just before the patient gets admitted to the school. This step allows the surgeon to correct any minor lip revisions and lateral vestibular webbing.

• The vestibular web represents five components of the nose that can change its appearance, and they are- the piriform rim, upper lateral cartilage, lower lateral cartilage, vestibular lining, and alar base.

• Any deformities in the above-mentioned structures are corrected during the intermediate rhinoplasty. This will achieve a more symmetric nose and also makes the future correction of nasal asymmetries easier.

• An open rhinoplasty helps to observe the geometric differences which can be corrected using suturing techniques. Cartilage grafting is not done at this stage of the treatment due to the residual growth potential of the nose.

Secondary Rhinoplasty:

• It is done after the completion of the facial growth at the age of 14 years to 16 years; this step of the treatment is similar to a traditional nose job. Cartilage grafts and other reinforcements form a major part of the treatment; they provide structural support, improve tip definition, and prevent wound collapse.

What Are the Surgical Considerations for CLND Repair?

It is important to provide a tailored treatment to patients with CLND because there is no standard treatment to correct the nasal asymmetry in a CLND patient, and many of these patients will have undergone multiple surgeries to treat associated cleft lip, cleft palate, and maxillary (upper jaw) deformities which can be quiet jarring.

Before the surgery, during the examination, the following features are to be evaluated-

• The maxillary (upper jaw) position is an important landmark that needs to be taken into account while preparing for the treatment. Many patients with CLND have retarded maxillary growth, which will need orthognathic surgery for overall facial harmony.

• In a patient in whom the traditional three-step rhinoplasty is not performed, the nasal examination must be done in a systematic manner. Previous surgeries to repair the cleft lip and/or palate will have altered the normal growth pattern, which will affect the planned operation.

• The features that need to be taken into consideration before the operation are the nasal tip asymmetry, distorted lower lateral cartilages on the cleft side, depressed nasal dome, deviated nasal septum, and flattened ala of the nose.

These considerations need to be addressed to perform a successful repair of the nasal deformity and prevent any complications and recurrences that might occur after the repair.

Conclusion

Repair of a nasal deformity in patients with CLND is quite tricky because treating the patient too early will lead to long-term effects on anatomical growth and, when treated too late, will increase the chances of obstruction of the airway along with a heavy psychosocial burden. Appropriate timing and the three-step rhinoplasty will improve the symmetry and prevent future growth deformities.