Cleft lip and palate patients represent one of the most challenging groups of patients for septorhinoplasty, presenting as a complex surgical obstacle for even the most seasoned surgeons. These individuals have undergone several surgeries throughout their lives, resulting in a considerable amount of scar tissue, significant asymmetries and structural deficits. Key factors in successfully treating cleft lip and palate patients are the reconstruction of the absent/asymmetric cartilages and the replacement of bony structures. The use of autogenous rib cartilage allows the surgeon to create various grafts as well as fortify the soft tissue to resist persistent soft tissue deformities.
Secondary (definitive) rhinoplasty is a complex surgical procedure due to the need for multiple surgical stages while dealing with aberrant anatomy.
Septal extension grafts are constructed from the interior aspect of the rib cartilage, while batten grafts are constructed from the curved portion.
Application of piriform rim/premaxilla grafts address asymmetry along the midface.
The use of adjuvant therapies (synthetic fillers and autologous fat grafting) may improve the overall result of the rhinoplasty.
The surgeon must be able to utilize multiple treatment modalities to address the cleft patient and minimize relapse.
Situated at the middle third of the face, the nose is one of the most common facial structures that is first identified by individuals. Any minute changes are easily identified and are unforgiving. Rhinoplasty is an incredibly intricate surgical procedure, as it involves manipulation of multiple parts, including the skin, cartilage, and bone. Cleft lip and palate patients pose a greater dilemma to the surgeon because of the presence of significant asymmetry and deficiency of underlying tissue and bone. Multiple rhinoplasties may be required in order to achieve esthetic results. The aim of this article is to discuss the secondary (definitive) rhinoplasty as well as adjuvant techniques when treating cleft lip and palate patients.
Unilateral Cleft and Acquired Nasal Deformity
In the presence of a unilateral cleft, the insertion points of the orbicularis oris are disrupted. On the noncleft side, the orbicularis oris muscle attaches to the ipsilateral aspect of the columella and forces the premaxilla, columella, and caudal nasal septum toward the unaffected side. , On the cleft side, the orbicularis oris inserts into the ipsilateral alar base and pulls the base laterally, inferiorly, and posteriorly. , The lower lateral cartilage (LLC) of the cleft side is commonly malformed and contributes to the nasal deformity, creating a more blunted contour ( Fig. 1 ). The nostril on the cleft side is significantly wider than the unaffected side and laterally positioned to the affected side because of a blunted medial crus and an elongated lateral crus , ( Fig. 2 ). It is important to note that as the caudal septum deviates toward the unaffected side, the remaining septum deviates toward the cleft side. The presence of septal deviation and decreased nasal aperture leads to an increase in nasal obstruction. Internal vestibular webbing along the nostril margin of the cleft side causes an inward displacement of the margin. The LLC is rotated inward leading to additional nasal obstruction because of a collapse of the external nasal valve; the position of the LLC creates a thicker and hooded appearance of the nasal ala. The internal rotation of the LLC is due to excess force from the aberrant position of the alar base and columella, forcing the cartilage posteriorly and inferiorly ( Fig. 3 ). LLC asymmetry and septal deviation require surgical intervention that includes an asymmetric lateral crura steal technique in addition to placement of a septal extension graft (SEG; Figs. 4 and 5 ).
Bilateral Cleft and Acquired Nasal Deformity
Bilateral and unilateral nasal cleft deformities share many characteristics, yet bilateral nasal cleft deformities tend to be more symmetric. , A significant finding in these patients is the presence of a bilateral hypoplastic maxilla with no development of the nasal floor. The alar base is positioned caudally and laterally with significant widening noted. The nasal tip has decreased projection and contour because of the elongation of the lateral crus and shortening of the medial crus , ( Fig. 6 ). The presence of vestibular webbing is noted bilaterally. The nasal septum is usually midline and contributes to the symmetric appearance of the nose. Alternatively, in asymmetry cases, the less affected side creates a pulling force on the septum, resulting in septal deviation to the ipsilateral side. ,
Surgical repair of a cleft nasal deformity is usually performed in multiple stages based on surgical timing: primary, intermediate, and secondary. These patients undergo cleft lip repair with simultaneous primary rhinoplasty around the age of 2 to 3 months. Current treatment guidelines recommend surgical intervention at 3 months of age to decrease the risk of surgical and anesthetic complications. An intermediate rhinoplasty is performed during the ages of 5 to 11 years, most commonly at ages 4 to 6 years, before school enrollment. , The timing of the secondary (definitive) rhinoplasty depends on the completion of facial growth and maturation, which varies between the genders. Facial maturation occurs between the ages of 14 and 16 years in girls and 16 to 18 years in boys. Additional factors that influence timing of definitive rhinoplasty include patient’s size, extent of deformity, and nose size.
Rhinoplasty performed at this stage alone is currently accepted as definitive surgical treatment by many surgeons. It is even considered the standard of care in the United States among other countries. The original notion that primary rhinoplasty can interfere with facial growth has been disputed by many recent studies. The primary target of this surgery is to obtain a symmetric nasal tip and alar base. There are many described techniques to correct the asymmetrical unilateral nasal deformity, most notably, the V-Y-Z plasty. , In general, the LLC is released and repositioned. , The alar base is separated from the pyriform aperture and maxilla to be repositioned symmetrically. , Nasal tip plasty is performed to enhance its projection. Moreover, the caudal nasal septum is repaired at this stage by attaching it to the anterior nasal spine. The use of a presurgical nasoalveolar molding device can be successful in shaping nasal cartilage within the first 6 weeks after birth, when the cartilage is more elastic because of high levels of circulating maternal estrogen.
This surgery is only performed when necessary. Some bilateral cleft nasal deformity patients undergo surgery at this stage to correct a severely asymmetrical nasal tip that was not repaired in primary rhinoplasty. , In addition, it aims at lengthening a shortened columella. For unilateral nasal deformities, it addresses any residual defects in LLC and lateral vestibular webbing. Septal repositioning and cartilage grafting are not performed at this stage; they are postponed until complete skeletal growth is achieved in adulthood.
Secondary/definitive intervention is usually required to correct secondary deformities and scarring that develop after primary cleft lip and nose repair. The success of primary rhinoplasty and the severity of secondary deformities are dependent on the surgeon’s skill and experience. An open approach is the most common technique used in definitive rhinoplasty, as it allows for enhanced exposure of muscle structure, cartilage, vestibular lining, and dense scarring. , , It also aids in precise positioning of the lateral lower cartilage, nasal base, and septum, as well as accurate grafting and suturing.
Goals of definitive rhinoplasty include nasal tip definition, removal of scar and fibrofatty tissue, and nasal obstruction repair ( Fig. 7 ). , In some cases, depending on the complexity of the cleft lip and palate deformity, a Le Fort procedure may be indicated to correct secondary maxillary hypoplasia. Definitive rhinoplasty is typically performed after repairing major skeletal defects with necessary orthognathic surgery.
Secondary cleft rhinoplasty is almost always performed via an open approach, giving access to different parts of the nose that need reconstruction along with direct visualization. The transcolumellar open incision is the most common access technique in definitive rhinoplasty, as it provides better exposure of the septal cartilage as well as the underlying bony and soft tissue structures. The incision is V- or W-shaped and is infracartilaginous. The incision is placed anterior to the footplate segments of the medial crura of the LLC. It is important to note that the medial crura are directly under the skin in the lateral portion of the columella; a superficial dissection is advisable in this area. A medial marginal columella incision is made approximately 1 to 2 mm from the columella edge and extends up to the domal recess. Once the skin flap is elevated, the cartilage and bone components can be examined thoroughly.
A deviated septum in cleft patients may lead to airway obstruction by way of physical stenosis and turbulent airflow. Correcting the septum involves resecting its cartilaginous and bony parts, including the vomer and perpendicular plate of the ethmoid bone. Simultaneously, it is best to maintain at least 1-cm width of dorsal and caudal septal segments, or L-strut, to preserve the tip and dorsal nasal support. , There should also be more than 40% fixation area, or contact point, between the nasal crest of the maxilla and L-strut when resecting the caudal septum. The contact point decreases the strain energy and stress values on the septum and relieves excessive load forces on the L-strut, thereby reducing the chances of future nasal deformities, such as a collapse of the dorsal septum or saddle deformity, and nasal tip ptosis. However, in some cases, the septal deviation is severe enough that the resection of the deviated segment and its replacement with a straight graft is necessary. The graft is obtained from septal cartilage, or the rib cartilage may be used if a larger segment is needed. Sutures through the upper lateral cartilages and septum are placed to apply force in the opposite direction of the L-strut deviation, thus correcting it and creating symmetry ( Fig. 8 ). In certain cases, a notch is created in the nasal spine using a straight osteotome to allow for a stronger attachment of the caudal strut. Spreader grafts and batten onlay grafts may be harvested from the resected parts of the quadrangular septal cartilage, but most often are obtained from the curved portion of the right sixth rib in order to have adequate shape, size, and strength of cartilage required in the cleft rhinoplasty. A mucoperichondrial flap is raised with caution not to perforate the surrounding mucosa and to allow excellent coverage of a large SEG from rib.