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KEYWORDS
Diagnosis Management Cleft lip nasal deformity
The cleft nasal deformity associated with congen- outlines surgical techniques and timing used to
ital cleft lip malformations presents a formidable minimize these deformities.
challenge for any facial plastic surgeon. The extent
of the nasal deformity may range from mild to se-
vere, and is related to the extent of the original lip ANATOMY OF THE CLEFT NASAL DEFORMITY
abnormality.1 A nasal deformity is associated with Unilateral Cleft Nasal Deformity
all cleft lip deformities, which is evidenced by the The nasal deformities associated with congenital
nasal tip and alar base asymmetry found in pa- unilateral cleft lips have been well described and
tients with mild microform clefting of the lip. are consistent.3,4 The extent of the typical nasal
The cleft nasal deformity is a three-dimensional deformity is related to the degree of deficiency of
abnormality involving all layers of the nose, begin- alar base support on the cleft side. The typical
ning with the skeletal platform and extending into characteristics of the unilateral cleft lip nose are
the vestibular lining, cartilaginous infrastructure, described in Box 1.
and external nasal skin.2 The fact that cleft noses The hallmark of the unilateral cleft lip nasal de-
have abnormalities in all tissue layers separates formity is a three-dimensional asymmetry of the
them in kind and difficulty from most cosmetic nasal tip and alar base (Fig. 1). The columella
rhinoplasties. and caudal nasal septum always deviate to the
The nasal abnormalities associated with con- noncleft side, secondary to an asymmetric, unop-
genital cleft lips are characteristic, varying only posed pull of the orbicularis oris muscle. The cleft
by the extent of the original deformity and by alar base is asymmetric and the cleft ala is dis-
whether the clefting of the lip is unilateral or bilat- placed laterally, inferiorly, and posteriorly to its
eral. The secondary cleft nasal deformity, how- noncleft counterpart.5 The nasal tip is also asym-
ever, is more variable. It relates to three factors: metric, with the cleft side lower lateral cartilage
The original congenital malformation (LLC) having a shorter medical crus and longer lat-
Any repositioning and surgical scarring result- eral crus than the LLC on the noncleft side (Fig. 2).
ing from prior surgical intervention The weakened and malpositioned cleft-side LLC
Alterations of the nose related to growth produces a nostril that is wide and horizontally
oriented.
Many surgical approaches and techniques have The nasal septum in the unilateral cleft lip nose is
been described to repair cleft nasal deformities. deviated caudally to the noncleft side, and is
Because the presenting patient with a congenital bowed posteriorly to the cleft side (Fig. 3).6 In
deformity is young, the surgical plan must account a study of 140 nasal septa in patients with unilat-
for patient growth and surgical scarring. The sur- eral lip clefting, Crockett and Bumstead7 found
geon should understand the pathophysiology of that the bony septum deviated into the cleft airway
the deformity and have a systematic surgical in 80% of these patients. Interestingly, most pa-
plan. This article describes the classic nasal ab- tients with clefting do not complain of nasal ob-
normalities associated with clefting of the lip, and struction despite having significant deviation and
facialplastic.theclinics.com
a
Facial Plastic and Reconstructive Surgery, Department of Otolaryngology, University of California, Davis, 2521
Stockton Blvd., Suite 7200, Sacramento, CA 95817, USA
b
Department of Otolaryngology, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Pung-
nap-2dong, Songpa-gu, Seoul 138-736, Korea
* Corresponding author.
E-mail address: jonathan.sykes@ucdmc.ucdavis.edu (J.M. Sykes).
Box 1
Characteristics of unilateral cleft lip nasal
deformity
Nasal tip
Medial crus of lower lateral cartilage shorter
on cleft side
Lateral crus of lower lateral cartilage longer
on cleft side (total length of lower lateral
cartilage is same)
Lateral crus of lower lateral cartilage may be
caudally displaced and may produce hood-
ing of alar rim
Alar dome on cleft side is flat and displaced
laterally
Columella
Short on cleft side
Base directed to noncleft side (secondary to
contraction of orbicularis oris muscle)
Nostril
Horizontal orientation on cleft side
Alar base
Displaced laterally, posteriorly, and inferiorly
Nasal floor
Usually absent
Nasal septum
Caudal deflection to the noncleft side and
posterior deviation to the cleft side
Fig. 2. Schematic diagram (A) and base photograph (B) of a left complete unilateral cleft and palate with obvious
asymmetry of the alar base.
Fig. 3. Bony skeleton of a patient with a left unilateral Fig. 4. Patient with incomplete left cleft lip showing
cleft lip showing obvious deviation of the nasal sep- obvious left nasal deformity with left alar hooding
tum to the noncleft side. The septum deviates to the and webbing of the left nasal vestibule. The alar
noncleft side because of asymmetric pull of the unop- base is deviated posteriorly, inferiorly, and laterally
posed orbicularis oris muscles. when compared with the noncleft side.
136 Sykes & Jang
Fig. 5. Two base photographs of patients with bilateral cleft lip and palate deformities. (A) The lip clefting is in-
complete and the columellar length is close to normal. (B) The manifestation of the cleft nasal deformity is much
greater secondary to a very large complete cleft of the lip and palate with a ‘‘locked out’’ premaxilla and
prolabium.
Fig. 6. Schematic diagram (A) and full face photograph (B) of a bilateral cleft lip deformity showing a short medial
crus and a long lateral crus being longer then normal.
Fig. 9. Schematic diagram (A) and intraoperative photograph (B) of a patient with unilateral clefting showing uni-
lateral dissection of the lower lateral cartilage from the external nasal skin.
present. In the bilateral deformity, asymmetry is investigators add intranasal incisions, such as
usually not an issue, and primary rhinoplasty is of- the intercartilaginous or marginal incision, to repo-
ten not performed. McComb and Coughlan13 have sition the LLC and to address alar hooding.
shown in an 18-year individual study that nasal The first step in primary cleft rhinoplasty is to ac-
and midfacial growth were not significantly differ- tively reposition the cleft alar base into an optimal
ent in children who underwent primary cleft rhino- three-dimensional position. This requires an inter-
plasty versus age-matched normal and cleft nal alotomy to completely free the alar base
controls who did not have primary rhinoplasty. Ad- attachment from the pyriform aperture (Fig. 8).
ditionally, these investigators showed that the An incision is made just lateral to the anterior at-
symmetry obtained with primary rhinoplasty was tachment of the inferior turbinate. With adequate
maintained into adult life. mobility, the alar base can be placed into a favor-
able, symmetric position.8
After mobilizing the cleft alar base or bases, the
Technique
LLCs are freed from the external skin. A lateral tun-
Primary cleft rhinoplasty can be accomplished by nel is created with scissors dissection over the lat-
using the standard incisions typically used for lip eral crus to the nasal dome (Fig. 9). This tunnel is
repair.18 No additional external incisions are typi- joined to a separate medial tunnel and the skin–
cally necessary. The rotation advancement repair soft tissue envelope is mobilized from the cartilag-
as described by Millard allows direct access to inous infrastructure (Fig. 10). If columellar length-
both the columella and the alar base. Some ening is required on the cleft side, a back cut
Fig.10. Schematic diagram (A) and intraoperative photograph (B) showing medial dissection of the medial crus of
the lower lateral cartilage from the columellar skin.
Cleft Lip Rhinoplasty 139
Fig.12. (A) Two-day-old patient with obvious left cleft alar deformity and cleft nasal base asymmetry. (B) The same
patient at 10 months of age after a unilateral rotation advancement cleft lip repair and primary tip rhinoplasty.
140 Sykes & Jang
Fig.13. (A) Intraoprative photograph of a patient with bilateral cleft lip showing forked flaps used to advance and
augment the columellar skin and lengthen nasal tip projection. (B) The same patient after suturing the forked
flaps into position and demonstrating lengthening of the columellar.
allows maximal visualization for diagnosis, and ad- of the LLC, or superiorly by dividing the ULC
equate exposure for placement and suturing of attachments to the nasal septum.6
structural grafts.
If the amount of skin is deficient in the columella,
skin and soft tissue can be advanced from the lip
NASAL SEPTOPLASTY
into the nose. In the unilateral cleft nasal deformity
with a short columella, a V-to-Y lip skin advance- Correcting septal deformities in the cleft patient is
ment can be used to increase columellar soft an important initial maneuver. Septoplasty serves
tissue. In the bilateral cleft nasal deformity, addi- to relieve functional nasal obstruction and provide
tional columellar lengthening can be obtained cartilage for graft material during the rhinoplasty.
with either a central V-to-Y skin advancement, or Additionally, nasal septal reconstruction allows
with upper lip forked flaps21 (Fig. 13). If columellar three-dimensional nasal base symmetry and sup-
soft tissue is sufficient, a standard inverted port to be established.
notched ‘‘V’’ incision is used to facilitate the exter- To reconstruct the caudal septum, the septo-
nal approach. maxillary ligaments must be divided and a strip
For the nasal septum, an endonasal approach of inferior cartilage usually must be removed. The
using a hemitransfixion incision may be per- caudal septum is then moved into the midline
formed. Alternately, the nasal septum may be and sutured into position with an anchoring suture
treated through an external approach by separat- to the nasal spine.6 If there is an intrinsic convexity
ing the soft tissue attachments of the medial crura or concavity to the nasal septum, structural
Fig. 14. (A) Schematic diagram of a left unilateral cleft lip with a columellar strut and dome suture to maximize
nasal tip and columellar symmetry. (B) Intraoperative photograph of a patient with a columellar strut sutured
into position to create three-dimensional symmetry.
Cleft Lip Rhinoplasty 141
Fig. 16. (A and B) Suturing of the cephalic margin of the lower lateral cartilage to the upper lateral cartilage to
diminish alar hooding on the cleft side.
142 Sykes & Jang
Fig. 18. A 17-year-old male before and after repair of left unilateral cleft lip with definitive external septorhino-
plasty. (A) Preoperative frontal view. (B) Postoperative frontal view. (C) Preoperative right lateral view. (D) Post-
operative right lateral view. (E) Preoperative left oblique view. (F) Postoperative left oblique view.
Cleft Lip Rhinoplasty 143
Fig. 19. A patient with a bilateral cleft lip and palate before and after definitive open septorhinoplasty. (A) Pre-
operative frontal view. (B) Postoperative frontal view. (C) Preoperative basal view. (D) Postoperative basal view.
(E) Preoperative right oblique view. (F) Postoperative right oblique view. (G) Preoperative right lateral view.
(H) Postoperative right lateral view.
144 Sykes & Jang
The cleft-side lateral crus of the LLC is concave, 8. Sykes JM, Senders CW. Surgical treatment of the uni-
contributing to the introverted alar contour.25,26 lateral cleft nasal deformity at the time of lip repair. Fa-
Treatment of the concave lateral crus of the LLC cial Plast Surg Clin North Am 1995;3:69–77.
can be accomplished with various methods. The 9. Stenstrom SJ. The alar cartilage and the nasal defor-
cartilage can be dissected from the underlying ves- mity in unilateral cleft lip. Plast Reconstr Surg 1966;
tibular skin, flipped, and replaced with the convex 38:223–31.
side outward (Fig. 17). Another technique involves 10. Cronin TD. The bilateral cleft lip with bilateral cleft of
placing an alar strut graft between the existing con- the primary palate. In: Converse JM, editor. Recon-
cave LLC and the vestibular skin. This underlay car- structive plastic surgery. Philadelphia: WB
tilage graft strengthens the lateral crus of the LLC Saunders; 1964.
and changes the concave nature of the cartilage. 11. Sarnat BG, Wexler MR. Growth of the face and jaws
Lastly, horizontal mattress sutures can be after resection of the septal cartilage in the rabbit.
placed to change the concave lateral crus into Am J Anat 1996;118:755–67.
a convex orientation. Whatever the technique 12. Bernstein L. Early submucous resection of nasal
used, the goal of these maneuvers is to change septal cartilage: a pilot study in canine pups. Arch
the concave alar cartilage into a convex one, im- Otolaryngol 1973;97:272–85.
proving the contour of the alar rim and the function 13. McComb HK, Coghlan BA. Primary repair of the uni-
of the external nasal valve.25 lateral cleft lip nose: completion of a longitudinal
study. Cleft Palate Craniofac J 1996;33:23–31.
14. Mulliken JB, Martinez-Perez D. The principle of
SUMMARY
rotation advancement for the repair of unilateral
Reconstruction of cleft lip nose is extremely chal- complete cleft lip and nasal deformity: technical var-
lenging. The congenital malformation involves all iations and analysis of results. Plast Reconstr Surg
tissue layers, from skeletal platform to external na- 1999;104:1247–9.
sal skin. Primary rhinoplasty is used to maximize 15. TerKonda RP, Sykes JM. Controversies and
nasal symmetry and minimize psychological ridi- advances in unilateral cleft lip repair. Curr Opin
cule for the child. This also allows the nose to Otolaryngol Head Neck Surg 1997;5:223–7.
grow in a symmetric fashion. Definitive rhinoplasty 16. Grayson BH, Santiago PE, Beecht LE, Cutting CB.
is performed using an external rhinoplasty Presurgical nasoalveolar molding in infants with cleft
approach. Structural grafting is employed to max- lip and palate. Cleft Palate Craniofac J 1999;36:
imize function, structure, support, and symmetry 486–98.
(Figs. 18 and 19). 17. Liou EJW, Subramanian M, Chen PKT, et al. The pro-
gressive changes of nasal symmetry and growth af-
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