Nasal deformity associated with typical cleft lip can cause aesthetic and functional issues that are difficult to address. The degree of secondary nasal deformity is based on the extent of the original cleft deformity, growth over time, and any prior surgical correction to the nose or lip. Repair and reconstruction of these deformities require comprehensive understanding of embryologic growth, the cleft anatomy, as well as meticulous surgical technique and using a spectrum of structural grafting. This article reviews cleft lip nasal deformity, presurgical care, primary cleft rhinoplasty, and definitive cleft septorhinoplasty with a focus on aesthetics and function.
Key points
- •
Considered by many rhinoplasty surgeons to be the most difficult to master, cleft lip septorhinoplasty is a challenge requiring understanding of the primary deformity and lip/nose repairs in infancy.
- •
Surgical planning to address the typical characteristics incorporate the typical grafting and suture techniques used in noncleft rhinoplasty, but adept use of cleft-specific techniques is required (alar hooding, alar base asymmetry, columellar shortening, deficiency in the piriform/premaxilla, and nostril shape).
- •
Skeletal disproportion from the cleft deformity plays a major role in the nasal deformity. Ideally the bony deficit is treated with appropriately timed alveolar cleft and premaxillary bone grafting.
- •
Septal or rib cartilage grafts should be used to create a stable caudal septal projection. The lower lateral cartilages are then set into position to the rotation and projection of choice using a tongue-in-groove technique.
- •
Thick-skinned nasal tips require conservative soft tissue debulking or soft tissue envelope thinning to improve tip contour and definition.
Introduction
Cleft lip and/or palate formation is the most common congenital craniofacial abnormality, constituting 1 in 500 to 1000 live births. Some variability in ethnicity has been noted, with higher incidence in Native Americans and Asian populations, and lower incidence in African Americans and Africans. Orofacial clefting seems to be multifactorial, associated with genetics and environmental factors.
The nasal deformity associated with typical cleft lip can result in significant aesthetic and functional issues that can be difficult to address. The nasal deformity has classic descriptions for the unilateral ( Box 1 ) and bilateral ( Box 2 ) cleft lip, but the nasal findings occur with significant variability along a spectrum of severity. The nose’s central position has an important role in facial aesthetics and the perception of normal facial features. In cleft nasal deformity the distortion of the nose can range from minimal to severe, emphasizing the importance of a patient-centered approach to repair. In order to maximize function and appearance through cleft septorhinoplasty, it is crucial to understand the embryologic origin of clefting and the anatomic structure in nasal cleft deformity.
- •
Grossly asymmetric
- •
Nose has longer appearance on cleft side
- •
Retrodisplaced cleft-side dome
- •
Base of columella deviated toward noncleft side
- •
Nostril is wider and retrodisplaced on cleft side
- •
Nostril margin on cleft side buckles inward because of bowing by internal vestibular web
- •
Deficient maxilla on cleft side (often absent nasal floor affecting piriform aperture)
- •
Posterolaterally displaced alar base and piriform margin on cleft side
- •
Anterolaterally displaced anterior nasal spine
- •
Deviated premaxilla, columella, and caudal septum toward noncleft side
- •
Posterolaterally displaced cleft-side dome of lower lateral cartilage (LLC)
- •
Increased angle between medial and lateral crura on cleft side
- •
Short medial crus on cleft side
- •
Long lateral crus on cleft side
- •
Upper lateral cartilage and LLC on cleft side are side by side rather than normal overlap
- •
Grossly symmetric
- •
Wide nose with broad and depressed tip
- •
Short columella
- •
Wide nostrils with inward collapsing margins
- •
Flared alae with bilateral vestibular webbing
- •
Posterolaterally displaced alar domes
- •
Increased angles of divergence between the medial and lateral crura
- •
Shortened medial crura
- •
Longer lateral crura
- •
Protrusive premaxilla
- •
Hypoplastic maxilla bilaterally
- •
Anterior nasal spine and caudal septum are inferiorly displaced relative to the alar bases
- •
Deficient or absent bony nasal floor
This article reviews cleft lip nasal deformity, presurgical care, primary cleft rhinoplasty, and definitive cleft septorhinoplasty, with a focus on restoring symmetry and contour to the shape, and maintaining or improving function.
Embryology
During embryologic development, the upper lip formation begins at approximately 4 weeks of gestation with completion at 3 to 4 months of gestation. Most facial skeleton and connective tissue is developed from a pluripotent population of cells and cranial neural crest (CNC) cells that show remarkable migratory abilities as well as ability for development into diverse cell types. Migration of the CNC cells into the frontonasal prominence facilitates the formation of the forehead, nasal dorsum, median and lateral nasal prominences, premaxilla, and the philtrum. This highly regulated process starts with paired bilateral maxillary prominences, derived from the first branchial arches, merging toward the midline to form the lateral aspect of the upper lip. Later in the development, as the CNC continues to migrate into the maxillary prominences, the medial and lateral nasal prominence join the premaxillary segment to form the nares, nasal tip, and philtral column ( Fig. 1 ).
This coordinated process involves a multitude of transcription factors, and regulation of growth signals. Disruption of the transcriptional or growth factor signals in development results in malformations of the upper lip, central alveolus, and/or the primary palate, including clefting of the lip and palate. Extent of cleft nasal deformity is associated with extent of interruption of the normal development, with a spectrum of varying severity of lip and associated nasal deformities. However, even when subtle, nasal deformities are always associated with cleft lips.
Anatomic deformity
Nasal deformity associated with unilateral and bilateral cleft have been well documented. Understanding the consistent skeletal and muscular dysmorphism and asymmetry is essential in providing the most cosmetic and functional repair.
Unilateral Cleft Lip Nasal Deformity and Dysfunction
In unilateral cleft lip nasal deformity, there are several well-described characteristics (see Box 1 ). There is asymmetry of the nasal tip and alar base caused by a deficiency of the maxilla on the cleft side ( Fig. 2 ). As a result, the cleft-side alar base does not merge in the midline, which contributes to the classically described unilateral cleft lip nasal deformity. These soft tissue asymmetries as well as the maxillary skeletal deficiency leave the cleft-side alar base more posterior, inferior, and lateral compared with the noncleft side.
The nasal tip asymmetry is caused by a dysmorphic shape of the cleft-side lower lateral cartilage (LLC). The cleft-side LLC has a short medial crus and a long lateral crus compared with the noncleft side. The asymmetry of the LLC and subsequent muscle pull generates a wide and horizontally oriented nostril. There is also discontinuity of the orbicularis oris on the cleft side and improper insertion into the columella on the noncleft side, which pulls the columella and caudal septum toward the noncleft side, with resultant posterior, lateral, and inferior displacement of the alar base on the cleft side. The nasal septum is then deviated toward the noncleft side caudally and bows dorsally toward the cleft side. In addition, the attachment of the upper lateral cartilage is affected by the irregularity of the LLC, which weakens the scroll region and compromises the internal nasal valve ( Fig. 3 ).
Bilateral Cleft Lip Nasal Deformity
Bilateral cleft lip nasal deformity shares many features with unilateral cleft lip nasal deformity but is grossly symmetric rather than asymmetric (see Box 2 ). There is often bony deficiency medially and inferiorly at the piriform of the maxilla. The premaxilla and overlying prolabium extend from the nasal septum in the complete bilateral cleft lip (a key point is that the amount of prolabial soft tissue is a key factor to the primary repair and the resulting nasal deformity). The alar bases are more posterior, inferior, and lateral in position. An underprojection of the nasal tip has been suggested to occur because of longer lateral crura and shorter medial crura of the LLC. The authors believe that the cartilage deformation of the LLCs includes nearly normal cartilage volume and size, but differences in how the cartilages are folded caused by the abnormal muscle attachments and distribution of forces caused by the bilateral cleft lip.
A broad and flat nasal tip with hooding of the alae are seen. Malpositioning of the prolabium and shortened medial crura results in a short columella with a wide base, which makes the nasal tip lack even more definition. The insertion of the orbicularis oris bilaterally into the alar base adds further to nasal alar base widening, which worsens with facial animation. The nasal septum is generally midline; however, if there is any asymmetry, the septum deviates toward the less affected side, where the muscle attachments are pulling the caudal septum. External and internal nasal valves can be affected because of poor upper lateral cartilage and LLC strength, which has potential to cause nasal breathing difficulties with valve collapse. Curvature of the lateral-most LLCs can create folds of redundant internal nasal valve nasal lining, also called plica vestibularis ( Fig. 4 ).
Presurgical management
Before surgical repair, patients with wide, complete cleft lip-palate may benefit from presurgical infant orthopedics (PSIO), which include appliances that can reposition the maxillary and nasal structures in more anatomic positions and allow for less wound tension after surgical repair, with the goal of potentially improving outcomes after primary repair ( Fig. 5 ).
Nasoalveolar Molding
Manipulation of the premaxilla before surgical repair for cleft lip and palate has been reported since the sixteenth century. Initially this included lip taping, elastics, orthodontics, and acrylic appliances. These devices were collectively referred to as PSIO. Over the years, maxillary arch devices were developed that connected the protruding premaxilla with lateral arch segments. Another option is the Latham device, which is used to shift the premaxilla and lateral alveolar segments by using a pin-retained appliance. The modern nasoalveolar molding (NAM) appliance was created by Grayson and colleagues. It combines an orthodontic device on the premaxilla and lateral alveolar segments with nasal stents. The device is fitted to the maxilla with molding and the stents are then fixed in place with taping.
After the introduction of NAM therapy, Grayson and Cutting noted improved appearance of the nose with a reduction in secondary nasal surgeries and decreased need for alveolar bone grafting. An oral appliance, such as the Hotz appliance or Zurich plates, functions as a static palatal plate once adapted to the cleft alveolus. The presence of increased serum maternal estrogen level allows nasal cartilages to be more readily corrected. The cartilage is made more flexible by the release of proteoglycans and hyaluronic acid caused by increased estrogen levels. The hormones that contribute to the increased elasticity of cartilage in a mother’s pelvis also is thought to affect a neonate’s ear and nasal cartilages. In addition, studies by Matsuo and colleagues also showed the ability of infant auricular cartilage to be molded.
The goal of NAM therapy is to bring the maxillary segments together and, in cases of bilateral cleft, to retract the premaxilla posteriorly to approximate the level of the maxillary segments. In addition, the nasal prongs serve the purpose of bringing the lip segments together, elongate the columella in the bilateral cleft, expand the nasal mucosal lining, and mold the lower lateral alar cartilages to more symmetric positions.
In infants with bilateral cleft, the lateral alveolar clefts are moved medially and the protruding premaxilla is moved medially and posteriorly. Next, nasal stents are applied to the retention arms with a soft denture material added to serve as a bridge between the 2 sides ( Fig. 6 ). The bridge functions as a nonsurgical method to lengthen the columella, which helps define the nasolabial junction. Applying gentle pressure to this area can also facilitate lengthening of the prolabium. As a result, the need for secondary columellar lengthening is reduced.
Evidence-Based Approach to Presurgical Infant Orthopedics
Use of PSIO is highly debated. The long-term outcomes of NAM therapy before primary surgical repair are controversial. Contributing factors to the controversy over NAM include a lack of clear objective outcome assessments, discrepancies in techniques used between cleft centers, and an ever-changing measure of success. Systematic reviews have not been able to identify negative impacts of PSIO, excluding NAM. There is also insufficient evidence of potential primary benefits such as ultimate facial growth, maxillary form, dental occlusion, or speech, and potential secondary benefits such as parental satisfaction or improvement in feeding (level II evidence).
Opponents of NAM propose that midface growth and dental arch shape may be inhibited by moving the maxillary segments. Furthermore, they suggest that nasal improvements have a significant rate of relapse. A 10% to 20% relapse rate in nostril width and height has been reported after NAM and unilateral cleft lip repair in the first year. However, this relapse could be countered by overcorrecting the cleft-side nostril.
In 2012, Abbott and Meara published a review supporting the effectiveness of NAM on the nasal form in unilateral cleft lip (level III evidence). After reviewing the evidence (levels II–V), the investigators concluded that although negative effects were unlikely, benefits in bilateral cleft deformity were also unclear. This lack of clarity is in part caused by a lack of well-designed and controlled outcomes assessments. The confounding variables in the studies reviewed, such as inconsistency in techniques of NAM and lip repair, degree of cleft severity, and the need for a long duration of follow-up, are difficult to control. In the future, studies should aim to incorporate multiple sites, rigid inclusion criteria, rigorous controls, and a multidisciplinary approach.
At present, in our practice, NAM is used in both unilateral and bilateral cleft repair with strong consideration of the socioeconomic impact on parents (ie, residing a long distance from a cleft center), parental compliance, and cleft severity.
Related posts:
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
