Extended Mohler repair

Nasoalveolar molding

Primary cleft lip and nasal reconstruction has been made more predictable by the development of nasoalveolar molding (NAM). Although the Mohler Cutting repair can be applied to any complete unilateral cleft lip deformity, regardless of severity, NAM leads to greater nasal symmetry, and facilitates gingivoperiosteoplasty. Most importantly, patients who are treated with NAM who are followed to facial maturity are found to have: improved nasal aesthetics compared to patients who did not undergo NAM, a low revision rate, and no detriment to anterior facial growth. Through weekly to biweekly modifications of the alveolar plate and nasal stent, NAM anatomically aligns the alveolar segments, lengthens the columella, increases nasal tip projection, folds the lower lateral cartilages towards its native form, transposes the lower lip and alar base towards the midline, and expands the intranasal lining. Therapy is usually completed within 8–10 weeks in patients with a unilateral cleft deformity. The summative effect of NAM is altering the severity of the cleft towards a mild phenotype. Therefore, the benefit of NAM lies in the ability for good surgeons to achieve excellent results and for experienced surgeons to more consistently obtain the near perfect result. In the author's practice pattern, NAM is used whenever feasible by the family.

Gingivoperiosteoplasty

The “boneless” bone graft has variable numbers of supporters and remains a topic of active debate. During primary cleft lip repair or cleft palate reconstruction, the mucosal edges of the alveolus may be connected to promote bony union between the free alveolar segments. Gingivoperiosteoplasty (GPP) was originally described by Skoog and later popularized by Millard, who used a Latham device to actively mold the alveolus prior to GPP.

Proponents state that the procedure will avoid, in most cases, the need for secondary alveolar bone graft. Indeed, successful GPP is demonstrated in 60% of patients with a unilateral cleft, circumventing the morbidity and financial burden of an additional surgery. Anterior growth appears to be unaffected through the age of mixed dentition and at the time of facial maturity. However, other reports have suggested some impairment of midface growth after GPP. Furthermore, the procedure is very technique dependent. Even a moderate dissection force can fracture the thin alveolar bone and destroy underlying tooth buds. Mucoperiosteal flap dissection must be limited to the mesial and distal faces of the alveolus with no dissection of the lingual or buccal faces. This requires perfect alignment of the alveolar segments and a gap of 1 mm. Pre-surgical orthopedics, therefore, is a necessary adjunct to gingivoperiosteoplasty.

Opponents state that gingivoperiosteoplasty has a limited success rate and will damage anterior facial growth as well as the developing teeth. Indeed, GPP has a variable success rate across institutions, implicating the technical challenges of the procedure. The majority of the clinical reports of gingivoperiosteoplasty are single institution experiences limiting a large volume analysis. Gingivoperiosteoplasty experienced resurgence after the development of nasoalveolar molding, and although initial reports at the age of facial maturity demonstrate no detriment to anterior facial growth, further analysis is likely required prior to broad acceptance of this innovative technique.

Tension-free reconstruction

The purpose of the suture is to join together vascularized tissue so that normal healing can occur. Normal healing does not occur when sutures are placed under tension. When considering the delicate reconstruction of the three-dimensional form of the lip and nose, this principle is taken a step further: even mild amounts of tension will result in relapse of the cleft deformity, widened scars, and predispose the patient to additional surgery to the lip and nose resulting in additional scars in these areas. Tension on closure, even mild tension, should not be addressed with sutures; this is an intervention which does not last. Tissues should be adequately freed until tension is completely eliminated in the critical aesthetic areas of the nose and upper lip skin. In cases where pre-surgical orthopedics is not used, extensive dissection may be required to completely free soft-tissue tension upon closure.

No lip adhesion

Lip adhesion is commonly advocated in patients with a wide unilateral or bilateral cleft where NAM or a similar form of pre-surgical orthopedics cannot be employed. This author's experience is that lip adhesions are unnecessary, even in the cases of very wide cleft lips, and can compromise surgical outcomes. Four points deserve mention in this regard:

• 1.

  L-flap reconstruction: The use of a lip adhesion precludes the use of an L-flap, whether based on the alveolus as described by Millard or pedicled on the lateral nasal wall, as advocated by Cutting and Grayson. Although a lip adhesion can bring the lip and alveolar segments in greater apposition, the alar base and nose are mildly affected by this procedure. The lip adhesion, therefore, may benefit lip reconstruction at the cost of nasal reconstruction. Without an L-flap, a pyriform aperture incision defect cannot be closed with mucosal tissue. Therefore, the intranasal incision is not made at the time of nasal repair or the incision is made but not reconstructed. Both of these will compromise the quality of nasal repair in the very patients who require the full armamentarium of reconstructive techniques: patients with wide cleft lip deformities.

• 2.

  Scar: In addition to eliminating certain flap options, a lip adhesion produces a scar in the center stage of the planned repair. Lip adhesion will therefore compromise the quality of the lip tissue and this is appreciable at the time of lip repair.

• 3.

  Complete cheek and nasal dissection: One of the great challenges of wide cleft lip repair is medial transposition of the lateral lip and alar base to achieve a tension-free closure. Although a lip adhesion can be useful in achieving this end, one can simply perform thorough cheek and lip dissection at the time of primary repair and achieve the same surgical ends of lip and alar base transposition, but without the cost of flap sacrifice and scar. This dissection requires a generous pyriform aperture incision and wide dissection in the perialar area and across the face of the maxilla. A supraperiosteal dissection plane is maintained to limit blood loss.

• 4.

  Anesthetic exposure: One of the most important considerations against lip adhesion is avoidance of a second anesthetic exposure. The operating room is a significant cost and performing primary cleft lip repair in one surgery instead of two will result in significant cost savings, even if pre-surgical orthopedics, such as NAM, are used. In low-resource regions of the world where (in certain cases) patient follow-up can be challenging, single-stage repair is a much more logical approach to patient care. Finally, as the negative effects to early anesthetic exposure are becoming increasingly appreciated by specialists, parents, and caregivers, streamlining care to single-stage interventions may come into greater demand.

Primary nasal repair

Previous studies have demonstrated the aesthetic benefit to primary cleft rhinoplasty as well as the safety of infant rhinoplasty to subsequent nasal growth. Although primary cleft rhinoplasty is advocated by most surgeons, not all rhinoplasties are created equal. The great enemy of the refined soft-tissue repair is scarring; therefore, primary cleft rhinoplasty should execute the best and most lasting change with the least amount of dissection. Any surgeon can perform a three layer nasal dissection at the time of infancy, then “touch up” the nose with an open rhinoplasty and cartilage grafts prior to elementary school, then refine the nose again (with an open rhinoplasty, of course) at the age of adolescence. However, one would predict that by the time facial maturity is reached, the density of scar surrounding the lower lateral cartilages and embedded in the nasal tip skin would preclude any aesthetic or functional result in the patient beyond the mediocre. Costal cartilage is advocated by many surgeons who perform adult cleft rhinoplasty; however, even these rigid grafts can be deformed by the steady, persistent force of cicatrix.

Primary cleft rhinoplasty, therefore, should be directed and limited in dissection. Although removal of fibrofat in the nasal tip can certainly result in greater tip refinement at the time of repair, will that same procedure be perceived as a benefit when attempting to refine the scarred tip at the age of 22? The benefit and detriment of different types of primary rhinoplasty is an area in which even modest scientific assessment is pending. Experienced cleft surgeons are familiar with the scarred nasal tip resulting from excessive operations that resists the most acrobatic surgical repair. The under-operated nose can be predictably improved, although perfection is elusive. The over-operated, scarred nose can produce unfortunate, complex, and insurmountable hurdles to an already challenging operation which, in many cases, can be avoided with proper planning and appreciation for the larger picture.

There are several principles of care which are advocated to produce the most change while limiting potential harm:

• 1.

  Nasoalveolar molding: Pre-surgical manipulation of the nose and alveolus results in a more predictable primary cleft rhinoplasty, which produces superior aesthetic results to the nose at the time of facial maturity compared to patients who have not undergone nasoalveolar molding. The requirement of secondary “touch up” surgery is decreased while increasing overall nasal symmetry.

• 2.

  Limited dissection: In the growing face, surgical planning should take into account the immediate and long-term effects of surgery. As adult cleft rhinoplasty remains one of the formidable challenges in plastic surgery, logic would dictate that limiting the amount of scar present in the nose at the time of facial maturity would be a wise investment for the patient. Therefore, the art of primary cleft rhinoplasty should include effecting the most change with the least surgical dissection. This philosophy falls somewhat counter to the culture of plastic surgery, which tends to advocate more complex and a greater number of procedures to reach the holy grail of the perfect result.

• 3.

  Indications for secondary rhinoplasty: The ideal treatment plan for patients with a cleft would be an effective primary cleft rhinoplasty at the time of cleft lip repair followed by a second rhinoplasty at the time of facial maturity. This goal can be achieved in many patients; however, several others may require a “touch up” surgery prior to reaching facial maturity. It is the author’s opinion that these patients are the ones most at risk for the formation of excessive scars within the nose. Secondary rhinoplasty on the growing face should be considered if there is direct and clear request by the patient or caregiver, not because the surgeon is trying to improve their result. When performing immature cleft rhinoplasty, consideration should be given to the consequences of the procedure. Aggressive cartilage dissection, resection of nasal tip fibrofat, and cartilage implants can all look favorable for a time after surgery; however, subsequent rhinoplasty at the time of facial maturity may be compromised by overzealous application of these techniques.