While primary cleft lip nasal deformity has been well described, secondary cleft lip nasal deformity reflects the combination of residual deformity that follows primary operative maneuvers and growth-related nasal distortions. Secondary cleft lip nasal deformities are further associated with underlying skeletal and dentofacial abnormalities along with soft tissue constriction adding to the complexity of the deformity and posing major aesthetic and functional challenges to the multidisciplinary care team. Definitive rhinoplasties are performed to address these deformities and improve the quality of life in cleft patients following skeletal maturity and ideally after all underlying skeletal discrepancies have been corrected by orthognathic surgery. Maxillary advancement with or without mandibular setback is often required after careful planning and orthodontic preparation. Patients with cleft lip benefit tremendously from definitive rhinoplasty irrespective of inevitable residual discrepancies that remain and adjuvant therapies could enhance the overall outcome.
Key points
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All patients with a cleft lip deformity have an associated nasal deformity that ranges in severity.
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Cleft patients present with common anatomic differences, such as a maxilla deficiency in all 3 axes, that arise from intrinsic factors and because of previous surgery.
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Analysis and performance of orthognathic surgery should be conducted before definitive cleft rhinoplasty to optimize the overall result.
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A thorough understanding of the anatomy of the cleft nose aids the surgeon in selecting proper techniques for repair.
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When dealing with cleft patients, surgical technique must be altered in terms of the planned incision.
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Goals of definitive rhinoplasty include relief of nasal obstruction, creation of symmetry, and definition of the nasal base and tip, and management of nasal scarring and webbing.
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Septal reconstruction in the cleft nose is a key maneuver in cleft rhinoplasty.
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The surgeon must be able to use multiple treatment modalities to address the cleft patient and minimize relapse.
Introduction
Patients with clefts lip and/or palate require long-term multidisciplinary care to achieve functional occlusion and aesthetic facial balance. Because of the existing substantial asymmetry and insufficiency of underlying soft tissue and bone, the nasal deformity associated with congenital cleft lip/palate is a complicated condition that causes major aesthetic and functional challenges. Cleft patients have typical anatomic skeletal anomalies including bony deficits along the premaxilla and the pyriform rim as well as the shortage of the maxilla in all 3 axes, which are caused by both inherent causes and influenced by surgical interventions. To maximize the overall result, definitive cleft rhinoplasty should be evaluated for and performed following underlying skeletal correction. This may require orthognathic surgery to correct skeletal discrepancies. The purpose of this article is to review definitive rhinoplasty and orthognathic procedures in the treatment of patients with cleft lip and palate.
Anatomy and Pathophysiology of the Cleft Nasal Deformity
Nasal deformity exists in all forms of cleft lip with or without cleft palate. The degree of nasal deformity parallels the severity of labial clefting and is also worse whenever associated with clefting of the alveolus and secondary palate. Some common features of the cleft lip nasal deformity have been described. While long-term follow-up studies documented better outcomes and reduced complexity of operative maneuvers required during definitive rhinoplasty when primary “tip rhinoplasty” was performed at the time of unilateral and bilateral cleft lip repairs, it has been advised to conduct definitive rhinoplasty to correct secondary cleft lip nasal deformity after orthognathic correction and defer further intervention while the nose is still growing and changing rapidly until late childhood and young adulthood, especially for minor and moderate nasal deformities.
Unilateral cleft nasal deformity
The orbicularis oris insertion points are disturbed when there is a unilateral cleft . The orbicularis oris muscle joins to the ipsilateral aspect of the columella on the noncleft side and pulls the premaxilla, columella, and caudal nasal septum to the unaffected side. The orbicularis oris inserts into the ipsilateral alar base and pushes it laterally, inferiorly, and posteriorly on the cleft side. The cleft side’s lower lateral cartilage (LLC) is frequently deformed, contributing to the nasal deformity by providing a more blunted contour. Because of blunted medial crus and enlarged lateral crus, the cleft side’s nostril is much larger and laterally positioned to the unaffected side ( Fig. 1 A–C). Pathogenesis of the cleft lip nasal deformity is partly the result of an extrinsic factor caused by the misdirected pull of the muscle and also due to an intrinsic deficiency of the alar cartilage that makes it prone to the deformational forces by the mal-inserted perinasal and orbicularis oris muscle.
The nasal septum deviates toward the cleft side as the caudal septum deviates toward the unaffected side. Nasal obstruction is increased when there is a septal deviation and a narrowed nasal aperture. Internal vestibular webbing along the cleft side’s nose margin causes the margin to shift inward. Because the LLC is turned inward, the external nasal valve collapses, causing more nasal blockage.
Bilateral cleft nasal deformity
The existence of a bilateral hypoplastic maxilla with no nasal floor development is a notable observation in these patients. The alar base is caudally and laterally positioned, with substantial broadening. Because the lateral crus has grown longer and the medial crus has shrunk, the nasal tip has lost projection and contour. The presence of vestibular webbing can be seen on both sides. The nasal septum is normally near the middle of the nose, contributing to its symmetry ( Fig. 2 A–C) . In asymmetric cases the less affected side exerts a pushing strain on the septum, causing septal deviation to the ipsilateral side. ,
Secondary and acquired nasal deformity
Secondary cleft lip nasal deformity is defined as those distortions that persist despite primary operative maneuvers and are expressed as the combination of residual deformity, iatrogenic deformity, and growth-related nasal distortion. Residual deformity is a result of failure to correct, undercorrect, or relapse and is influenced by a variety of factors including the embryologic deficiencies of both the nose and underlying maxillary segments, and the diminished growth potential. Iatrogenic deformities are determined by the pattern of repair of the mal-inserted perinasal and orbicularis oris muscle at the time of primary surgery of the lip, the unintended results of surgical technical errors, and the level of orthodontics practiced within the overall management. Growth-related nasal distortions, also referred to as postsurgical growth potential, occur during puberty and are often worse in children with unilateral cleft lip with or without cleft palate. , ,
Facial Skeleton Characteristics of Patients with Clefts
Cleft patients are commonly present with a hypoplastic maxilla and a constricted maxillary dental arch. ( Fig. 3 : a–d and 4: a–c). On the other hand, unrepaired cleft patients have been reported to develop fairly normal maxillary growth. These observations directed many craniofacial surgeons to believe that multiple surgical interventions at an early age led to subsequent scar formation of contracture and were attributed as the sole cause of hypoplasia.
The severity of maxillary hypoplasia is related to the cleft type. In unilateral cases, the lesser maxillary segment is most commonly hypoplastic and is displaced superiorly, posteriorly, and medially. The maxillary midline is commonly deviated toward the side of the cleft. Bilateral cleft cases tend to present with an extremely narrow maxilla that results from the posterior alveolar segments collapsing medially. This can be seen clinically as a bilateral posterior cross-bite. The premaxilla may be either superiorly or inferiorly positioned and is often protruded. Patients with bilateral CL/P seeking orthognathic surgery notably suffer from multiple residual problems, such as scarring, agenesis of lateral incisors, alveolar defects, abnormal nasal anatomy, and upper lip muscular dysfunction.
Surgical management
Preorthognathic Consideration
Orthodontic preparation
Orthodontics can be used to align and improve the location of the maxillary segments before alveolar bone grafting (ABG). Maxillary expansion is accomplished with the use of fixed orthodontic appliances and expanders. Orthodontic treatment continues following ABG or scheduled to be completed when the patient reaches skeletal maturity in preparation for orthognathic surgery. The concept of surgery-first approach is seldom adopted in treatment protocols for cleft patients. The teeth closest to the cleft should be upright with excellent supporting bone on all aspects of the root surface when patients are orthodontically prepared for segmental advancement surgery.
Alveolar bone grafting
Repair of the alveolar cleft begins in the mid to late mixed dentition before the eruption of missing canine and guided by the eruption of its counterpart in unilateral cases. It has been demonstrated in multiple studies to have no effect on midface growth. , ABG facilitates orthodontic space closure, thus simplifying future orthognathic procedures. In a bilateral CL/P patient with a severely displaced premaxilla, surgical repositioning may be considered in conjunction with the ABG. ,
Preorthognathic speech pathology assessment
The presurgical workup should include a speech pathology evaluation. This includes clinical evaluation as well as instrumental testing to assess the risk of developing velopharyngeal insufficiency after surgery.
Orthognathic Surgery
Surgical planning
Orthognathic surgery in the cleft patient mainly involves maxillary advancement with the possible mandibular setback. When indicated, bimaxillary surgery will reduce the magnitude of both the maxillary and the mandibular movements needed to correct the underlying skeletal imbalance. This requires the fabrication of a surgical intermediate splint, for the relation of the new maxilla position to the existing mandible, and a final surgical splint, which relates the final mandibular position to the fixed final maxillary position.
Virtual surgical planning
The ability to design osteotomies and advance skeletal elements freely and carry out unlimited trials of maneuvers in the preoperative simulated environment with precise anthropometrics of these manipulations has revolutionized the virtual surgical planning (VSP) phase of craniofacial reconstruction. The full workflow for orthognathic surgery includes virtual planning of the surgical treatment, three-dimensional printing of customized surgical devices (surgical cutting guide and titanium fixation plates), and possible real-time intraoperative navigation. A surgical cutting guide can be used during surgery to pilot the osteotomy line that had been planned preoperatively at the computer and the custom-made fixation titanium plates will allow the desired repositioning of the maxilla.
Cleft orthognathic surgery has evolved from the simple maxillary Le Fort I advancement with the correction of dental malocclusion to the current model of patient-centered approach focusing on skeletofacial reconstruction using computer-assisted diagnosis and planning. With the rising popularity of virtual surgical planning, there has been renewed interest in “mandible-first surgery” in certain circumstances including a cleft maxilla when double jaw surgery is indicated. , Three-dimensional imaging and surgical simulation have provided valuable information for facial aesthetics and surgical feasibility in the preoperative planning of complex cases. These include cases involving bimaxilla, multiple segments, multidirectional movements, or those involving distraction. When distraction has been planned, distraction devices can be carefully selected and modified. In this way, accurate planning of the distraction vector can be achieved.
Improved access and reduced costs have seen the use of virtual surgical planning expand, and is, indeed, a tool to strive for perfection. VSP reduces the operation time, discomfort, and burden of care for patients and serves as an alternative to conventional model surgery in adult patients with CLP. However, Wu and coworkers reported outcome discrepancies from the virtual simulation of all rotational surgical corrections (roll, yaw, and pitch) being positively correlated to the degree of planned surgical movement and that patients with bilateral cleft lip and palate are more likely to incur outcome discrepancies in yaw correction with major maxillary advancement of more than 5 mm advancement. Similarly, Heifetz-Li and colleagues documented a notable minority of points that were under-corrected in the anterior–posterior plane when using patient-specific osteotomy guides and plates, and recommended increased intraoperative clinical judgment when interpreting these deviations.
Le Fort I advancement (see Fig 3 : g-j)
The Le Fort I osteotomy is performed in the standard fashion. A large amount of soft tissue scarring is common in cleft orthognathic patients. The most common symptom is a soft tissue constriction that restricts the mobility of the osteotomized maxilla. The application of constant and firm traction to the soft tissues limiting the maxillary advancement for a prolonged time is essential to achieve adequate advancement in many cleft cases. In cases with more than 10 mm of advancement, soft tissue traction for 20 to 30 minutes may be required to ensure a passive seating of the maxilla into the interdental splint. Mobilization and advancement are more difficult in bilateral CL/P cases. With large advancements, attention must be paid to the vascularity of the premaxilla. The mandibular teeth are wired into a prefabricated interdental splint, and the movable maxilla is subsequently inserted into the splint. Care must be given during this maneuver to ensure that the mandibular condyles are securely seated in the glenoid fossa. The maxilla is temporarily wired into the splint while the medial and lateral buttresses are rigidly fixed. The wires are removed and mouth opening is checked before closure.
Distraction osteogenesis
Maxillary distraction osteogenesis (DO) is now considered to be a valuable surgical option( Fig 4 : e–i). In a recent study, the distraction group demonstrated greater maxillary advancement, less horizontal relapse of the maxilla, and higher life satisfaction when compared with the osteotomy group. Distraction osteogenesis would be indicated in a subset of cleft patients with severe maxillary deficit requiring anterior advancement of more than 10 mm. Nowadays, the technique of trans-sutural distraction osteogenesis (TSDO) is used to treat complex forms of craniofacial deformities and proved to be an effective tool for midface advancement, particularly in growing children with CLP.
