Cleft Lip Nasal Deformity Treatment & Management

Updated: Aug 30, 2017
  • Author: Maurice M Khosh, MD, FACS; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Treatment

Surgical Therapy

The techniques covered in this article do not represent an exhaustive review. No perfect surgical technique exists. Surgeons must tailor approaches to individuals and evolve techniques to best serve each patient. [2, 3, 4]

Nasal airway

The surgeon must aim to establish a functional nasal airway in addition to improving cosmesis. Adult studies have corroborated that postsurgically corrected noses maintain significantly smaller airways than noncleft noses. Hence, parallel goals of cosmetic and functional improvement must be served.

Septal deformity

Surgical correction aims at establishing a patent nasal airway while creating a more favorable external nasal appearance. Timing of septal surgery is controversial. One view is that early septal surgery disrupts natural septum growth, resulting in stunted nasal growth. Another view is that delay of septal surgery may cause unfavorable maxillary growth, especially if the child is a significant mouth breather. Clearly, conservative septal surgery, including reconfiguring septum shape by scoring portions of it, may be a compromise and may provide a meaningful solution. Consider the benefit of turbinate reduction or out-fracturing to increase nasal airflow. Exercise caution when performing surgery on abnormal anatomy, as vascularity and integrity of the structures may be less than optimal.

Columellar deformity

The columella is viewed as foreshortened in cleft lip nasal deformity. Two views on the best surgical method exist. Some surgeons argue that the columella requires primary correction, and others maintain that reshaping the nasal ala is sufficient to affect columellar length. [5, 6]

Proponents of reshaping believe that the columella owes its retracted appearance to the horizontal position of the nasal ala. Therefore, reseating the nasal ala in an anterior-posterior oval is thought to naturally elongate the columella. The Ivy modification of the Blair procedure entails medially and anteriorly rotating a laterally based nostril rim flap to lengthen the columella by repositioning the nasal ala, as depicted in the 1st image below. Similarly, the Dingman technique requires medially rotating a columellar and alar-based flap, as depicted in the 2nd image below.

Cleft lip nasal deformity. Ivy modification of the Cleft lip nasal deformity. Ivy modification of the Blair procedure. Laterally based nostril rim flap rotated medially inward.
Cleft lip nasal deformity. Dingman technique. Nost Cleft lip nasal deformity. Dingman technique. Nostril rotated via columellar and alar-based flap.

Many techniques are available for primary correction of the columella. The simple V-Y advancement flap may serve to lengthen the columella if adequate columellar width and upper lip tissue are present, as depicted in the 1st image below. Similar to the V-Y flap, an anteriorly pedicled rectangular flap may be elevated to provide greater length, as depicted in the 2nd image below. The Brauer-Foerster technique uses fan-shaped flaps along the medial and anterior margin of the alar rim, which can be pulled medially to increase columellar projection, as depicted in the 3rd image below.

The Cronin procedure involves simultaneous anterior bilobed flap advancement and posterior midline columellar flap advancement, as depicted in the 4th image below. Techniques that lengthen the columella may be more effective for patients with bilateral cleft lip for whom symmetric columella advancement is desired.

Cleft lip nasal deformity. V-Y advancement for col Cleft lip nasal deformity. V-Y advancement for columellar lengthening.
Cleft lip nasal deformity. Rectangular flap for co Cleft lip nasal deformity. Rectangular flap for columellar lengthening.
Cleft lip nasal deformity. Brauer-Foerster procedu Cleft lip nasal deformity. Brauer-Foerster procedure for columellar lengthening via medial rotation of bilobed flaps.
Cleft lip nasal deformity. Cronin procedure for co Cleft lip nasal deformity. Cronin procedure for columellar lengthening. Anteriorly based bilobed flap is advanced anteriorly, and posteriorly based columellar flap is advanced posteriorly.

More radical reconstruction is required when the columella is completely absent. The upper lip is the ideal donor site for reconstruction. However, the upper lip is often scarred or deficient secondary to cleft lip. Lower lip tissue may be transferred to the columella, or nasolabial flaps may be recruited. A full-thickness skin graft (FTSG) may be buried below upper lip skin for use in reconstructing the columella (ie, Ferris-Smith technique;, as depicted in the 1st image below. The Serre method involves burying a double length of FTSG (folded on itself and buried in the upper lip) to provide additional columellar length, as depicted in the 2nd image below. Auricular cartilage, especially from the helix, may serve to fashion a new columella, with the caveat that poor vascularity in the recipient site may hinder graft adherence.

Cleft lip nasal deformity. Ferris-Smith procedure. Cleft lip nasal deformity. Ferris-Smith procedure. Repair of total columellar loss; full-thickness skin graft (FTSG) is buried beneath upper lip skin and later rotated into place.
Cleft lip nasal deformity. Serre method. Repair of Cleft lip nasal deformity. Serre method. Repair of total columellar loss; doubled full-thickness skin graft (FTSG) is buried beneath upper lip skin and later rotated into place.

Alar deformity

Alar deformity is the major defect in cleft lip and/or nose. Surgical techniques may be categorized into external and internal approaches. In mild defects, the nostril may be repositioned by excising a wedge of skin anterior to the rim margin and advancing the rim forward, as depicted in the image below. Retaining a thin rim of skin at the rim margin prevents webbing at the soft triangle. Other external techniques described in columellar deformity, above (eg, Ivy modification of the Blair technique, Dingman technique) may also be used.

Cleft lip nasal deformity. Elevation of nostril ap Cleft lip nasal deformity. Elevation of nostril apex by excision of skin wedge.

Metz et al described a modified Van der Meulen technique for correction of the nasal entrance in patients with unilateral cleft lip; the surgery involved use of a nasal alar rim flap and anatomic repositioning of the alar cartilage. In the retrospective study, which involved 30 adult patients undergoing the surgery as a secondary procedure, the investigators reported that narrowing and lifting of the nasal tip was achieved in all patients, while the columella was lengthened by an average of 40% and nostril shape was modified from horizontally to longitudinally oval. [7]

Alar cartilage may also be delivered and repositioned without external incisions. For example, the alar cartilage is severed from its lateral attachment and advanced medially to be sutured to the contralateral ala in the Thomas Rees technique, as depicted in the 1st image below. Flattened contour of the ala may also be corrected by tacking the ala to the upper lateral cartilage, or, alternatively, to the nasal bone. The classic Tajima reverse-U technique has undergone many modifications. A reverse-U intranasal incision is made on the cleft side, the subcutaneous pocket is developed, and the deformed ala is sutured to the ipsilateral upper lateral cartilage, the contralateral upper lateral cartilage, and the contralateral ala, as depicted in the 2nd image below.

Cleft lip nasal deformity. Thomas Rees technique. Cleft lip nasal deformity. Thomas Rees technique. Lateral crus of lower lateral cartilage is severed from lateral attachment and advanced medially to be sutured to contralateral ala.
Cleft lip nasal deformity. Tajima reverse-U techni Cleft lip nasal deformity. Tajima reverse-U technique. A: Intranasal incision. B: Suture ala on the cleft side to (1) ipsilateral upper lateral cartilage, (2) contralateral cartilage, and (3) contralateral ala.

Onlay grafts may be required to achieve the ideal cosmetic result, despite best attempts at reconfiguring the ala via simple rotation or advancement. Grafts contribute to bulk for improved cosmesis and may serve to reinforce the atrophic weak alar cartilage. Septal cartilage may be harvested for immediate use or banked for future use in alar reconstruction; septoplasty is often required to restore nasal tip symmetry and increase nasal airflow. Some surgeons discourage septoplasty for the sole purpose of use in rhinoplasty because it may retard midfacial growth. Concha is an excellent source of cartilage for an onlay alar graft because it recreates the natural curvature of the ala.

Septal cartilage is straighter and more rigid than concha. Therefore, it may be best reserved for a columellar strut. The contralateral normal ala in unilateral cleft lips may be turned over to the defective side and used as an onlay graft. The cephalic border of the contralateral ala is divided but is kept pedicled medially and rotated over to the cleft side, as depicted in the image below, Various modifications of this technique exist. More extreme defects may be reconstructed with a C-shaped costal cartilage graft.

Cleft lip nasal deformity. Cartilage grafts. A: On Cleft lip nasal deformity. Cartilage grafts. A: Onlay alar graft. B and C: Turnover alar graft.

A study by Wei et al of patients with secondary unilateral cleft lip reported that nasal deformity (which in such cases can particularly involve the alar and nasal floor) can be successfully corrected with a double composite tissue Z-plasty technique, with the cartilage, muscle, and skin layers effectively restored. [8]

Dorsal deformity

Correction of septal deflection may be sufficient to reestablish normal dorsal symmetry. Advocates of early septal surgery cite the benefit of more normal nose growth, including the dorsal aspect. At times, osteotomies may be required to correct dorsal deflection. Onlay cartilage grafts have also been used to add bulk to the underdeveloped cleft side. Further projection may be gained through dorsal onlay bone grafting (eg, cranial, rib, iliac).

Bilateral cleft lip nasal deformity

The surgical techniques mentioned above focus on unilateral cleft lip deformities but also apply to bilateral deformities. Evolution of surgical techniques for treatment of bilateral cleft lip deformities has lagged behind treatment for unilateral deformities. This may reflect the relatively lower prevalence of bilateral cleft lip deformities. [9]

Computerized constructions reveal that bilateral cleft lip nose may be viewed as a duplicated image of unilateral cleft lip nose. Stigmata include a broad, flat, bifid tip; wide alar bases; and short columella—all of which contribute to the overall porcine nose appearance, as depicted in the image below. The nasal dorsum may exhibit lack of height, but it is usually within normal limits.

Cleft lip nasal deformity. Bilateral cleft lip nas Cleft lip nasal deformity. Bilateral cleft lip nasal deformity. Broad, flat, bifid tip; wide alar bases; and short columella. A: Frontal view. B: Profile view.

The hallmark of bilateral cleft lip nose is a short columella. Millard popularized what is perhaps the best known columellar lengthening technique, as depicted in the image below. Millard advocated the use of forked flaps, one from each side of the prolabium, which are banked within the nasal sills during primary lip repair until secondary rhinoplasty is performed. These flaps are then retrieved and rotated into the columella of the child when aged 2-4 years to achieve columellar lengthening.

Cleft lip nasal deformity. Millard classic forked Cleft lip nasal deformity. Millard classic forked flaps. One taken from each side of prolabium and banked within nasal sills during primary lip repair until secondary rhinoplasty.

However, McComb reviewed his cases of primary columellar repair using forked flaps and discovered that by adolescence, 3 unfavorable features developed: (1) the columella overlengthens, (2) the nasal tip broadens, and (3) downward drift of the lip-columellar junction occurs in conjunction with an unsightly transverse scar. [10] Therefore, McComb discourages borrowing tissue from the prolabium and favors using only nasal tissue to reconstruct the columella. McComb argues that surgical repair should focus on reestablishing normal alar shape, which in turn naturally elongates the columella. McComb achieves this result by suturing the medial crura of the alar cartilages together, which lengthens the columella and corrects the broadened nasal tip.

Mulliken states that the already flattened nasal ala continues to separate over time as a result of muscle tension. [11] Therefore, Mulliken favors primary alar correction, as does McComb, to achieve columellar length and proper nasal tip shape.

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Outcome and Prognosis

Cleft lip nasal deformity is a complex anomaly, and proper correction requires considerable surgical talent and experience. Surgeons must continue to tailor approaches to individuals and evolve techniques to best serve each patient. Children who benefit from good surgical repair have a chance to mature with fewer psychological sequelae from this deformity. [12, 13]

A study by Yoshimura et al indicated that nose growth can be adversely affected in children who undergo primary nasal repair simultaneously with primary cleft lip repair. At age 10 years, children in the simultaneous repair group (14 patients) had shorter noses and a greater upward tilt of the columella than did those who underwent cleft lip repair without simultaneous nasal repair (12 patients) and normal children in the control group. [14]

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