Rhinoplasty in Cleft Lip-Nasal Deformity

Babak Azizzadeh, MD, FACS;  Donald B. Yoo, MD

Background/History

Owing to its central location on the face, the nose plays an intimate role in all social interactions.  Early surgical correction of these deformities allow for facilitated psychosocial development and a better opportunity for normalized social integration for these patients without the stigma of having a nose that appears abnormal.

The first attempted cleft lip repair is purported to have been around 400 AD by a Chinese physician.1 Surgical techniques advanced in the centuries since with modifications of initial straight line closures to the use of increasingly refined interdigitating flap closures.2-4  Early in the history of cleft nasal repair the functional and cosmetic results varied widely, from excellent to very poor.  Given the complex nature and inherent challenge of the repair, early plastic surgeons advocated staged repair of cleft lip and cleft nasal deformities.  Many expressed concern over the proper growth and development of the nose with primary or early surgical intervention, and deferred correction until adolescence or early adulthood5-9

Over time, there was a shift towards primary repair of nasal deformities at the time of cleft lip repair as the results of such interventions proved not to have the deleterious effects on nasal bone and cartilage development, and not fraught with complications as previously thought.10-12  The idea of reducing the psychosocial burden of growing up with a deformed nose also drove surgeons towards primary cleft nasal repair.  Multiple studies with long-term follow up confirmed satisfactory, durable results after primary repair of cleft lip and cleft nasal deformities.12,13 Paramount to successful treatment of patients with cleft lip-cleft nasal deformity is longitudinal, multi-disciplinary care. 

Epidemiology

The incidence of cleft lip and palate is approximately 1:1000, with a higher incidence in populations with a family history and a racial preponderance toward Hispanic and Asian populations.  Twenty percent of unilateral clefts involve only the lip, while 50% involve both lip and palate. 

Embryology

The embryologic development of the human face is a complex and intricate process.  In simplified terms, facial growth is produced by the fusion of 2 maxillary, 2 mandibular, and an unpaired frontonasal process, with a facial cleft resulting from incomplete or partial fusion of any of these processes.  Cleft lip and nasal deformities specifically result from failure of the maxillary processes to fuse normally with the frontonasal or intermaxillary processes.

The nose develops from a pair of ectodermal placodes located on the frontonasal or intermaxillary processes.  The central aspect of each placode invaginates and forms the lining of the nasal cavity, the philtrum, and the primary palate.  Mesodermal migration is critical in normal facial development, and it is theorized that a deficiency in mesoderm allows structures to split, forming clefts. 

Though there is not a single, universally accepted embryologic explanation for cleft formation, it is generally accepted that a lack of ectodermal and mesodermal elements, and a disruption in normal migration, contribute to the deformations found in cleft lip and nasal patients.  The processes leading to normal development are incompletely understood, but the etiology of facial clefting appears to be multifactorial, with environmental and genetic factors both playing a role.     

Pathogenesis/Anatomy

Nasal deformity in cleft patients results primarily from muscle deficiency, skeletal hypoplasia, and skeletal asymmetry.  The orbicularis oris consists of a superficial (pars superficialis) and deep (pars marginalis) component, the former of which normally inserts on either side of the midline.  In the cleft patient, this is misdirected towards the displaced alar base to attach inferolaterally at the pyriform aperture, contributing to the flattened and laterally displaced appearance of the alar base in these patients.

This muscle imbalance relative to the noncleft side also reorients the position of the lower lateral cartilage and subsequently from the normal oblique to a more horizontal configuration.  Insertion of the orbicularis oris on the base of the septum also distorts the septum and columella towards the noncleft side.  The columella is frequently shorter on the cleft side, with an inferiorly displaced medial crural footplate. 

Skeletal hypoplasia and malposition of the maxillary segments leads to deformity of all the nasal structures dependant on the underlying skeleton for support, including the alar base, lower lateral cartilages, septum, and nasal floor.  The nasal floor on the cleft side may be lower or absent altogether relative to the contralateral side.  The hypoplastic maxillary segment also exacerbates the flattening of the alar base and nostril, with downward displacement of the lateral crus of the lower lateral cartilage.  Relative to the noncleft side, the lateral crus is elongated and the medial crus shortened due to this inferolateral pull.

The appearance and location of the nasal tip is determined to a large extent by the size, shape and orientation of the lower lateral cartilages.  Due to the deformation of the lower lateral cartilage on the cleft side from the muscle discontinuity and skeletal deficiency, as well as septal deviation, the dome on the cleft side is typically lowered and obtuse, and the tip asymmetric.

The severity of septal deformity is variable.  Typically, the septum is dislocated from the maxillary crest towards the noncleft side resulting in a septal deflection towards the cleft side, commonly causing nasal obstruction on that side.  In addition, the inferior turbinate on the cleft side is also frequently hypertrophic, further adding to nasal obstruction on that side.          

Treatment

The goals of surgical treatment of cleft nasal deformity include: normal speech, aesthetic facial appearance, normal occlusion, and normal psychosocial development.  Most cleft lip-nose and palate patients will require multiple procedures throughout their treatment, and attention to proper technique and a focus on minimizing soft tissue scarring is critical to successful outcomes.  Early nasal reconstruction corrects major nasal deformity before it becomes even more distorted with age, allows for more normal socialization, and provides a better nasal airway from an early age.     

Perhaps the most challenging problems encountered in secondary cleft nasal repair result iatrogenically from poorly designed and poorly executed primary cleft nasal repair.  Owing to this fact, it is of utmost importance to recognize what can and can not be achieved at the time of primary repair.  Another important consideration is adequate identification and repair of the underlying skeletal deformities, as the lower lateral cartilages and nasal base rely on the skeletal base for integrity.    Such deformities should be addressed prior to attempting correction of the nasal base, columella, and nasal tip.  Without underlying structural support the small lower lateral cartilages of an infant lack the integrity to properly support the nasal tip and infratip lobule soft tissue. 

Once the extra-nasal structural elements are satisfactorily addressed, the nasal cartilage can be manipulated to restore the typical anatomic relationships.  The lower lateral cartilages can be exposed and released from their lateral attachments and repositioned to redefine the dome and tip defining points, increase the projection, and increase anteroposterior length of the nose.  A columellar strut graft is often a useful adjunct for providing additional structural support to the tip, and faciliting lengthening and projection of the nasal tip.  The columella is oriented obliquely and foreshortened on the cleft side, with its footplate displaced and tethered inferiorly, while the base is deviated to the noncleft side.  At times repositioning of the lower lateral cartilages is sufficient to restore the columella, while more severe cases require local flaps for correction. 

Septal deviation is inherent to cleft lip-cleft nasal deformity, and contributes greatly to the overall distorted external appearance of the nose in addition to creating functional nasal obstructive issues.  The caudal septum assumes an oblique position in the cleft patient and is displaced from the maxillary crest towards the cleft side, contributing to the displacement of the nasal tip towards the contralateral, noncleft side.  Correction of septal deviation is paramount to alleviating nasal obstruction in the cleft nose patient.  Some controversy exists regarding the optimal timing of septal surgery.  Some posit early septal surgery leads to a disruption in natural septal development, while others feel delaying it results in a disruption to normal maxillary growth.

Despite the move towards primary correction of cleft nasal deformities, secondary rhinoplasty procedures are often necessary to achieve optimal results.  Secondary rhinoplasty is best performed after the patient reaches bony maturity and maxillary deficiences have been treated appropriately with orthodontic and orthognathic procedures.  An open approach provides the best exposure to the nasal tip, and provides an unobstructed view of the middle vault and bony dorsum.  Autologous cartilage grafts, harvested from the septum, rib or ear, are often necessary to provide structural support and to restore acceptable contours.  Structural grafting is used to project the nasal tip, support the external nasal valves, and reconstruct the internal nasal valves.  Contour grafts facilitate nasal tip refinement, dorsal augmentation, and overall symmetry.   

 

Case Presentation (Babak Azizzadeh, MD, FACS;  Donald B. Yoo, MD)

 CC:  h/o right unilateral cleft lip s/p repair with residual oronasal fistula, cleft nose deformity

HPI:  A 32 year old female with a history of unilateral right cleft lip and cleft palate repair presents with residual right-sided oronasal fistula, nasal dysmorphia, and septal deviation towards the right.

Fig 1.  The patient’s right alar base sits in a more inferolateral position relative to the left non-cleft side.  The nostril on the right has a far more horizontal orientation and exhibits a weakened shape compared to the contralateral side. 

On external examination, the patient exhibits foreshortening of the columella, with the base oriented away from the cleft side.  The septum deviates towards the cleft side, displacing the tip towards the contralateral side.  Her right side demonstrates underdevelopment of the nasal bones and maxilla, causing inferolateral displacement of the right alar base and displacement of the nasal dorsum towards that side.   The malposition of the ala contributes to rim weakness of the right ala, resulting in a more horizontal shape to the nostril and a narrower aperture versus the left.  Overall, the patient’s nose exhibits a very deep radix with low dorsum, a low nasal starting point, and a greatly underprojected nasal tip.

Surgical correction involved direct excision and closure of the oronasal fistula with local tissue rearrangement and septorhinoplasty through an external approach.  As is the case in many cleft lip-nose patients, the cartilaginous septum was displaced laterally from the maxillary crest along the floor of the nose.  This displacement gives the septum an oblique orientation from the base view.  Correction involved separating the cartilaginous septum (quadrangular cartilage) from the maxillary crest and securing it to the midline with a 5-0 PDS suture.  Deviated portions of the septum were then removed, while preserving an adequate dorsal and caudal strut. 

Projection of the radix and dorsum was achieved by harvesting deep temporalis fascia through a small incision behind the hairline in the temporal fossa and harvesting auricular conchal cartilage.  The conchal cartilage was finely diced into 0.5 mm – 1 mm morsels and wrapped with the deep temporalis fascia to form a dorsal onlay graft to provide dorsal augmentation.  The alar rims were reconstructed with cartilage grafts to strengthen them and improve their symmetry.  Tip projection and symmetry was increased with a strut graft in the columella and a cap graft placed over the tip.

 

Works Cited

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  11. Smahel Z, Mullerova Z, Nejedly A. Effect of primary repositioning of the nasal septum on facial growth in unilateral cleft lip and palate. Cleft Palate Craniofac J. Jul 1999;36(4):310-3.
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