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Correction of Unilateral Cleft Lip Related Nose Deformity Animation 1

Correction of Bilateral Cleft Lip Related Nose Deformity Animation 2

Pearls

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  Ideally, the secondary cleft lip rhinoplasty should be performed at about the age of 14–15 years for a female and 17–18 years for a male.

  
  
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  The secondary cleft lip nose repair should be carried out after the maxillary deficiency has been corrected and the platform that supports the nose has been brought to a proper position and symmetry.

  
  
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  Nearly 60% of patients who have cleft lip nose deformity have difficulty breathing through the nose.

  
  
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  The major tenets of a proper correction of cleft lip nose deformity include complete exposure of the lower lateral cartilages, removal of the excessive soft tissues between the domes, dissection, and repositioning and fixation of the lateral crus to the columella strut.

  
  
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  It is crucial to recreate the central portion of the nasal tripod in a proper position with adequate strength by application of a columella strut, and application of a nasal spine, and maxillary and pre-maxillary graft if necessary.

  
  
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  A proper repositioning of the lateral crus may necessitate a V-Y advancement of the lateral crus with underlying lining.

  
  
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  An alternative to V-Y advancement is complete mobilization of the domes and the lateral crus on the cleft side, and rotation anteriorly to match the opposite cleft side without the lining. This will be more successful with bilateral placement of the lateral crura strut.

  
  
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  It is often necessary to debulk the ala and alar base on the cleft side and reposition the alar base medially.

  
  
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  It is crucial to remove a crescent-shaped piece of redundant soft triangle lining on the cleft side to aid adjustment of the nostril shape.

  
  
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  Elongation of the columella is achieved through placement of a columella strut, approximation of the footplates, trimming the soft triangle lining, which will convert the posterior portion of the infratip lobule to the columella, and placement of bilateral alar rim grafts.

In 1931, Blair & Brown called to attention the details of cleft-lip-related nasal deformities. Although Gillies & Millard suggested that repair of cleft lip nose deformity during the primary lip repair is unreasonable, this view has changed dramatically over the years. The initial argument was that, even if the nose is repaired properly during the cleft lip repair, additional surgery would be required at the time of puberty or later. It is often recommended that the operation to correct the skeletal asymmetry of the nose associated with cleft lip deformity is postponed until the age of 16–17. Broadbent & Woolf maintained that noses repaired during infancy will ultimately require additional procedures during adolescence. These strong views convinced most surgeons for decades that the cleft nose frame abnormalities should not be corrected during repair of the cleft lip. From the late 1960s, as pleasing results were produced with more finesse and more accurate alignment of the nasal base structures, convincing evidence was gradually offered to counteract the view opposing early repair of nasal deformity related to cleft lip.

Today, there is almost a consensus among craniofacial and pediatric plastic surgery leaders that correction of nasal tip asymmetry during primary cleft lip repair is advantageous and reduces the potential for additional surgery – or at least diminishes the magnitude of the surgery required as the patient matures. It is, however, crucial to understand that the deficiency in the maxilla inherent in the cleft lip complex disturbs the symmetry of the nose and reduces the support to the base of the nose on the cleft side, notwithstanding what procedures are carried out during the primary repair of the lip. It is therefore sensible to anticipate a secondary rhinoplasty in a number of patients who have undergone repositioning of the nasal frame during the primary surgery, because abnormal growth and skeletal deficiency will alter the position of the cartilages as the patient reaches adolescence.

Ideally, the secondary cleft lip nose repair should be carried out after the maxillary deficiency has been corrected and the platform that supports the nose has been brought to a proper position and symmetry. This should preferably take place around age 14–15 in a female and 17–18 in a male, when mandibular growth ceases, as documented by cephalometric X-rays. If the maxilla is advanced and the mandible continues to grow, additional maxillary advancement may be required, which can change the shape of the nose. This does not preclude correction of the nose abnormality under any circumstances before age 14–18. In certain cases a secondary cleft rhinoplasty may be deemed appropriate. Especially when the deformity is significant enough to induce psychological or functional disturbances, surgery can be considered earlier. However, the surgeon, the patient, and the family must have a clear understanding that, as skeletal maturation takes place and orthognathic surgery is completed, additional nose surgery may become necessary.

Patient Assessment

In general, cleft lip nose patients who undergo surgery are often pleased with the outcome, are not too fastidious, and do not complain about minor flaws. They are often well-adapted individuals who understand the reality of the deformity that they have to deal with. However, some patients have been the target of peer mockery throughout childhood and adolescence and have developed psychological disturbances that may merit attention prior to surgery. It is therefore essential to make sure that there are no underlying emotional imbalances that might prohibit surgery, since they may lessen or eliminate potential patient satisfaction.

Breathing difficulties should be explored very carefully in such patients, although lack of clinical symptoms does not necessarily mean that the patient does not experience airway occlusion, as indicated earlier. It is important to observe, as in other rhinoplasty candidates, whether the patient is a mouth-breather or mostly breathes through the nose. Additionally, investigation of any history of sinus infections and sinus headaches, which are common in such patients, is important. Learning about any complications that followed the primary repair and subsequent surgeries may help to reduce the potential for them to be repeated. It is also important to find out what tooth extraction and orthodontic work, if any, has been carried out to align the teeth.

On examination of the patient, depending on whether the cleft was unilateral or bilateral and whether or not it involves the alveolar segment and palatal cleft, there will be a great deal of variation in the presentation of the nasal deformity.

There are common shared features of cleft lip nose deformity. Common traits of unilateral cleft lip deformity are listed in Box 10.1 and the presentations of bilateral cleft lip deformity in Box 10.2 . Careful observation of the characteristics outlined in these boxes will help to formulate a precise surgical plan. However, it is again crucial to be familiar with abnormalities of the maxilla and mandible that may ultimately influence the outcome considerably. A gratifying outcome for cleft lip rhinoplasty is impossible without correction of the maxillary deficiency and/or excess growth of the mandible.

Nearly 60% of patients who have cleft lip nose deformity have difficulty breathing through the nose. Examination of the oral cavity often reveals occlusal abnormalities or the presence of some type of residual oronasal fistula. It is only after careful evaluation of the entire face that attention can be directed to the nose. Many patients with cleft lip nose deformity have thick skin with overactive sebaceous glands and acne. If acne is present, it should be controlled prior to the nasal surgery.

The nasal bones are commonly asymmetric and very wide. The upper lateral cartilages may be wide, especially in the case of bilateral clefts, where there may be a varying degree of extension of the cleft in the form of divergence of the upper lateral cartilages and nasal bones. The position of the domes and lower lateral cartilages, and therefore the width and projection of the tip, requires careful scrutiny. Assessment of the alar base symmetry may prove difficult because of the malposition and asymmetry of the lip and sometimes deviation of the chin. Observation of the size and orientation of the nostrils, the width of the columella, and the position of the lower lateral cartilages will often reveal a whole range of abnormalities. The architecture of the nostril sill is often distorted: it is commonly flat, scarred, and depressed because there is insufficient bone beneath it.

On the profile view, the radix is frequently shallow. The dorsal profile is often marred by a small hump. The nasolabial angle is usually narrow. The basilar view will disclose a short columella, abnormal orientation of the nostrils, and thickness of the alar base on the cleft side. The nostril sill and the floor can be more readily assessed in this view. Internal examination of the nose may often divulge some degree of stenosis, collapse of the external and internal valves, deviation of the septum, and presence of synechiae or even septal perforation.