Professional Documents
Culture Documents
and Technique
Kenneth E. Salyer, M.D.,1 Shai M. Rozen, M.D.,1 Edward R. Genecov, D.D.S.,2
and David G. Genecov, M.D.1
ABSTRACT
This article presents the philosophy, technique, and personal and team approach
for treating children with unilateral cleft lip-nose deformities based on the senior author’s
36 years of experience. The treatment of unilateral cleft lip almost without exception must
involve correction of the nasal deformity. To obtain excellent results, the surgeon must
integrate technique, teamwork, and timing based on multidisciplinary protocols developed
over the years. Technique must involve broad dissection of the nasal and lip elements off
the abnormal skeletal base and delicate but wide dissection of the nasal cartilage to achieve
contour and tip projection. The team must include and assimilate surgical, orthodontic,
speech, and when necessary orthognathic viewpoints to achieve the optimal result. The
final goal is to bring the child to normal facial appearance at conversational distance. It is
important to realize that the treatment of the unilateral cleft is rarely one procedure at one
time but rather a culmination of several interventions precisely timed in the growing phase
of the child from infancy to adulthood.
KEYWORDS: Unilateral cleft lip, cleft lip, cleft lip and nose deformity, cleft
orthognathics, cleft orthodontics
T he term unilateral cleft lip is almost a misno- time period (Tables 1 and 2) rather than one-time ‘‘home
mer because nearly always the nose is an integral part of run’’ solutions are the rule rather than the exception.
the problem that must be addressed to obtain an im- The long-term protocols have evolved gradually
proved result. Based on more than three decades of over the years and consistently produce good to excellent
experience, this article presents the philosophy and the results. Passive perisurgical orthopedics is applied at
global approach to the treatment of the child with the 2 weeks of age. Other than the use of the passive device,
unilateral cleft lip. There are several key elements that the abnormal skeletal base is mainly ignored at this stage
must be considered to obtain the desired result. First, the and emphasis is put on the soft tissue repair of the cleft
nasal involvement is almost ubiquitous and therefore lip/nose complex. This is the most important surgical
must be addressed primarily at the time of lip surgery. stage in primary cleft lip/nose repair. When the palate is
Second, the treatment should always be multidiscipli- involved, two-flap palatoplasty is performed at 8 months
nary, involving the cleft surgeon, orthodontist, and of age. Approximately 35% of the authors’ patients need
speech pathologist when the palate is involved. Third, minor secondary correction of the lip and/or nose,
the problem confronted is dynamic over the time of the generally performed at preschool age, around 5 years
child’s development, and therefore protocols spanning a old. Definitive rhinoplasty is performed in most cases at
Cleft Lip Repair: Trends and Techniques; Editor in Chief, Saleh M. Shenaq, M.D.; Guest Editor, Joseph K. Williams, M.D., F.A.C.S., F.A.A.P.;
Seminars in Plastic Surgery, Volume 19, Number 4, 2005. Address for correspondence and reprint requests: Kenneth E. Salyer, M.D., International
Craniofacial Institute, Cleft Lip and Palate Treatment Center, 7777 Forest Lane, Suite C-717, Dallas, TX 75230. 1International Craniofacial
Institute, Cleft Lip and Palate Treatment Center, 2Private Practice, Dallas, Texas. Copyright # 2005 by Thieme Medical Publishers, Inc., 333
Seventh Avenue, New York, NY 10001, USA. Tel: +1(212) 584-4662. 1535-2188,p;2005,19,04,313,328,ftx,en;sps00183x.
313
314 SEMINARS IN PLASTIC SURGERY/VOLUME 19, NUMBER 4 2005
Figure 1 (A, B) Frontal and lateral views of secondary deformity due to inadequate dissection and release of lip and nasal components
referred to our institution for correction. Note the short lip and the left alar bucking. (C, D) Postoperative frontal and lateral views after
correction of secondary defect with appropriate release of all components
components. The alar base, the medial and lateral crus of ing degrees of maxillary deficiency in the unilateral cleft
the alar cartilage, the nasal dome, the columella, and the lip and nose deformity. Subsequent growth and the final
nasal septum—all are affected by the skeletal base, degree of deformity and outcome depend on the cleft
consisting of the alveolus, maxillary segments, and pal- dysmorphogenesis and the selection of surgical proce-
ate. The severity of the primary nasal deformity is dures and sequencing. Rehabilitation must address the
intimately related to the degree of displacement, abnor- three-dimensionality and growth over time to obtain the
mality, and hypoplasia of the maxillary segments. Con- desired result.
tinued hypoplasia and displacement of the maxillary In the base of the primary deformity of the nose is
segments, particularly the lesser segment, result in vary- the displacement of the lower lateral cartilage laterally
316 SEMINARS IN PLASTIC SURGERY/VOLUME 19, NUMBER 4 2005
and inferiorly on the cleft side. The nasal dome is mally locks the segments, producing additional scarring,
flattened and slumped in a downward position. The and does not consistently produce enough bone to
alar cartilage on the cleft side is flat and gives it a false support the teeth in the cleft defect to allow excellent
appearance of lengthening when compared with the orthodontic restoration. Close to half these patients need
noncleft side, which is abnormally displaced to the other future bone grafting. In the senior author’s opinion, it is
side. The relationship of the lower lateral cartilage to the key to ignore the skeletal abnormality at the early stage
septum is normal, but the septum itself is tilted because and reconstruct the soft tissue components consisting of
of the cleft deformity, thereby tilting the base of the nose skin, cartilage, and muscle.
toward the noncleft side and the tip of the nose toward For these reasons, the current authors’ team
the cleft side. The question that arises in our experience approach for the past 29 years, for all complete clefts
is whether the septum should also be translocated or involving the alveolus and maxillary segments, is to use
moved surgically. Anderl believes so; we feel it is not passive perisurgical orthopedics. In the first few days of
necessary. But the answer is in long-term analysis of the child’s life an impression is made on which the
results, which we are currently conducting. The key to orthopedic passive appliance is fabricated. This acrylic
correction of the cleft nasal deformity is dissecting free device initially prevents collapse of the maxillary seg-
and translocating the alar cartilage with its attached ments and aids in feeding. The primary objective of the
vestibular lining into a normal position, thereby estab- appliance is to control the segments once the lip is
lishing the normal vault and shape of the cartilage.5,6,8 closed. It functions as a guide to the maxillary segments
At this stage, the major deformity of the nose is and locks them into position after cheiloplasty and
corrected. When combined with complete freeing of before palatoplasty. They also aid or improve the nasal
the soft tissue envelope of the nose and correction of airway by providing a temporary midline closure and
the alar bases and floor of the nose, consistently good nasal septum after closure of the lip, thereby probably
results may be achieved at the time of primary correction. also contributing to more normal growth of the nasal
airway.
There is no scientific proof that passive orthope-
Lip Adhesion dics provide improved results—it is our empirical ap-
Lip adhesion may contribute to unnecessary additional proach based on team member interaction and influence.
scarring and abnormal tethering of the lip or nasal Based on clinical observation, the senior orthodontist
elements and therefore is an unnecessary procedure. believes that the appliance improves horizontal and
The current author evaluated early cases of 50 patients vertical skeletal deficiency by stimulating bone produc-
in a double-blind randomized fashion comparing those tion before and after lip closure; however this hypothesis
performed with and those without lip adhesions; im- remains to be proved.14 Cases in which the midline is
proved aesthetics in the group that did not undergo lip shifted more than 2 mm require moving the entire
adhesion brought the author to abandon this technique. maxilla using a Le Fort I maxillary osteotomy. It is easier
Still, many experienced cleft surgeons continue to use to perform this in infants treated with a passive perisur-
the lip adhesion12 with the purpose of treating the gical orthopedic appliance for midline and maxillary
abnormal skeletal base, making it easier for the surgeon deficiencies. The current authors believe that this con-
to close the lip at the expense of the overall aesthetic tributes to better symmetry of the alar bases on the
result for the lip and nose. Others have reported benefits deficient cleft side and improves septal deviation by
in using nonsurgical lip adhesion with tape.13 Lip guiding the maxillary segments into a more normal
adhesion may actually cause fixation or scarring of the anatomic relationship. No effort is directed at shifting
alar base or associated adjacent structures in an abnormal the deviated septum or changing the skeletal base
position, making it more difficult to obtain a definitive actively with this technique. The passive appliance
normal contour of the nose. allows better control of the maxillary segments before
the time of palatoplasty and is worn from infancy to the
time of the two-flap palatoplasty at the age of 8 months.
THE ABNORMAL SKELETAL BASE AND After palatoplasty, this method may decrease the
PREOPERATIVE ORTHOPEDICS amount of maxillary collapse by locking in the maxillary
segments.
Passive Perisurgical Orthopedics
Obtaining symmetry of the skeletal base is one of the
main long-term goals for complete correction of the cleft Active Presurgical Orthopedics
deformity. The attempt to achieve skeletal symmetry at Millard and Latham are credited with popularizing
infancy by active perisurgical orthopedics in cases of active presurgical orthopedics.15,16 This represents an
unilateral deformities is misguided treatment in the opposite philosophy to the senior author’s in that active
senior author’s opinion. Early periosteoplasty abnor- orthopedics with active force is used to alter the skeletal
UNILATERAL CLEFT LIP—APPROACH AND TECHNIQUE/SALYER ET AL 317
base prior to cleft lip nasal repair. Some advocates of this PROTRACTION AND DISTRACTION
philosophy have added a primary gingivoperiosteoplasty OSTEOGENESIS
to close the cleft alveolus. One report states that 60% of The proportional effect of surgical scarring versus the
these patients do not need bone grafting later.17 Essen- severity of the primary cleft dysmorphology on abnormal
tially, this means that 40% of the patients eventually growth seen in patients with cleft deformities is not
need a second procedure for bone grafting. In the senior completely clear. Although there is literature stating that
author’s hands, primary bone grafting yields a 96% in some third world countries certain untreated cleft
success rate. Therefore, a procedure that yields only cases have shown near-normal facial development,24 we
60% success and necessitates a second procedure is think even those untreated cases do not have normal
both unnecessary and detrimental to midfacial growth development of the small segment, especially in the
and outcome.18 For these reasons, periosteoplasty has severe cases. There is no conclusive evidence that scar-
been abandoned by some centers.19 Performing early ring from surgery is the sole reason for abnormal facial
distraction and completing early orthodontic treatment growth, although we consider it a major factor. We
in infants are, in the current authors’ opinion, detrimen- believe that the degree of tissue absence and cleft
tal to growth and development.20 Anterior cross bite and dysmorphogenesis most probably correlates with the
anterior open bite are frequent; therefore, premature degree of facial growth abnormality when careful surgery
surgery due to insufficient amount of bone to support is performed with minimal scarring. Compensation for
the teeth is detrimental. this abnormal growth may be performed in certain
As previously mentioned, the authors’ approach patients in the age group of 5 to 15 years by either facial
of ignoring the abnormal skeletal base at the time of protraction with a mask or distraction osteogenesis,
primary surgery has resulted in near-normal growth in depending on the amount of retrusion.
nearly 70% of the patients. With this approach of passive
orthopedics, palatal expansion at the average age of 5.5,
and bone grafting of the cleft deficiency at the time of Facial Protraction
tooth development, these patients do not require or- In cases with maxillary retrusion of 4 mm or less, the
thognathic surgery. Bone grafting at the age of 7 to 9 at Delaire facial protraction mask (Great Lake Orthodon-
the time of cuspid or lateral incisor development using tics, Tonawanda, NY) is used. At the senior author’s
immobilization at the time of grafting produces a 96% center, new bone production has been demonstrated
success rate in the authors’ experience.21,22 with advancement of the anterior nasal spine using
protraction in cleft patients. The posterior nasal spine
is retracted in our palatal flaps, probably due to the use of
Presurgical Nasoalveolar Molding posterior vomer flaps.25
Nasoalveolar molding has been used and advocated
in both unilateral and bilateral cleft cases.23 Through
expansion it probably creates more columellar tissue, Distraction Osteogenesis
which is needed in bilateral cleft deformities. In the After 13 years of evaluating distraction in about 250
most common cases of unilateral cleft lip the nasal and patients, it is our opinion that distraction, in its current
lip elements are present; therefore, the senior author state of art, should be used in major deformities or
does not believe nasoalveolar molding is needed in most retrusion of the maxilla of 12 mm or more. Distraction
unilateral deformities. In the rare Tessier facial cleft, offers balancing of the facial skeleton during growth,
there may be missing elements, in which cases tissue allowing improvement in appearance, speech, and
expansion is warranted. Nasoalveolar molding is quite occlusion—all of which improve self-esteem.
labor intensive, requiring weekly adjustments by an Although a majority of these patients require
experienced and dedicated orthodontist or surgeon. additional definitive skeletal surgery, distraction provides
More so, full compliance is needed from the parents of ongoing normalization of jaw relationships during growth
the infant and there are multiple frequent visits. Most and development, offering a major advance in cleft care. It
often this technique is used in combination with active is important to note that definitive orthodontic surgery is
presurgical orthopedics, which the senior author thinks more difficult after distraction. Osteotomies should avoid
is detrimental to growth. The unilateral cases presented any teeth or tooth buds. We need more data to evolve a
here do not warrant this technique. Because of the more definitive protocol for distraction.
complexity and labor intensiveness of this technique,
it is not practical nor available in most places in the
world. The New York group23 has provided this modal- COMMONLY SEEN SURGICAL ERRORS
ity and has shown excellent results in bilateral cases, AND PITFALLS
but the use in most unilateral cases is in our opinion Achieving good consistent results in primary repair of
unnecessary. cleft patients depends on in-depth understanding of the
318 SEMINARS IN PLASTIC SURGERY/VOLUME 19, NUMBER 4 2005
In the midline, muscle fibers may be slightly bunched performed, as used earlier by the senior author while
together to provide fullness to the midlip. developing the technique29 (Fig. 3).
The medial lip incision toward the columella is
performed with a number 67 Beaver blade. The lateral
Combined Correction of the Lip and Nose lip element is incised at the vermilion-cutaneous junc-
The peak of the Cupid’s bow on the cleft side is marked tion with a number 65 Beaver blade with attention to
on the vermilion cutaneous border (the white roll) at an including a piece of orbicularis muscle in the vermilion
equal distance from the midline to that of the noncleft flap to create the orbicularis marginalis. Now the lateral
side (Fig. 2). To facilitate the symmetric design of the dermal skin element is undermined and dissected off the
philtrum, a single arm skin hook may be placed in the muscle for a distance of 2 to 4 mm with careful hemo-
middle of the prolabium, retracting the prolabium to the stasis (Fig. 4). At this stage the medial lip element is
midline and then marking. Another important factor is
preincision marking of the wet line on the vermilion of
each side of the lip, which is critical for a good color
match of both sides of the lip and improved aesthetic
result as observed by Noordhoff.28 Also, when perform-
ing the transverse incision on the lateral lip a sliver of
orbicularis should be left on the new vermilion to provide
a full vermilion with orbicularis marginalis. As seen in
Figure 2, the incision on the lateral segment follows the
dotted line cephalically above the inferior turbinate. This
allows full access to the lower lateral cartilage to perform
its release from the skin envelope and partially from the
nasal lining. If more access is needed, the incision may be
extended further within the nose. Despite the extensive
freeing of the cartilage, the cartilage itself is not exposed.
The incision around the base of the ala on the lateral
rotation advancement flap is no longer performed. This
eliminates the scarring around the alar base but still
allows appropriate release of the alar cartilage. The Figure 4 The orbicularis oris muscle is pulled with pick-ups, and
the skin of the lateral lip segment is undermined from the
rotation incision on the medial lip may extend along underlying muscle at a distance of 2 to 4 mm. The medial lip
the base of the columella to provide additional length, segment is rotated downward and pulled with a hook until its
but no back cut or extension into the columella is height matches that of the lip segment on the cleft side.
320 SEMINARS IN PLASTIC SURGERY/VOLUME 19, NUMBER 4 2005
Figure 12 (A, C, E) Frontal preoperative views at age 2 months and postoperative views at ages 5 and 14. (B, D, F) Submental vertex
views respectively.
Figure 13 (A) Preoperative frontal view at age 3 months. (B) Postoperative frontal view at age 5.
(C) Postoperative frontal view at age 16. (D, E) Postoperative left and right lateral views at age 16.
reconstruction, and tip projection can be achieved at the currently popular and assists the surgeon in closure of the
time of the primary procedure. Of course, in many cases soft tissue by approximating the skeleton, but the au-
changes occur with growth that necessitate further thors believe that this causes abnormal locking position
secondary revisions. These changes are usually related of the maxillary segments, causing damage to the growth
to the septum and skeletal base. Using the described of the face, resulting in a more severe deformity when
treatment protocol, the results the senior author achieves growth is completed. Late results have demonstrated
at the primary repair of the soft tissue remain consistent this assumption.30 The use of periosteal flaps to close the
with subsequent growth. Failure to achieve satisfactory bony cleft early results in 40% of the patients needing
results should be apparent to the surgeon at the time of additional bone grafting.
completion of the primary repair. The authors’ technique attempts to eliminate the
Alignment of the skeletal base is not necessary at early stigmata of primary cleft lip-nose in young chil-
the time of primary repair. Passive devices may initially dren. It eliminates the alar buckling, a consistent finding
direct the segments toward each other after cheiloplasty of cleft nasal deformities prior to primary repair. When
and subsequently help prevent palatal collapse after performed by an experienced surgeon, the primary
palatoplasty. Active preoperative surgical orthopedics is procedure may create a near-normal external nose.
324 SEMINARS IN PLASTIC SURGERY/VOLUME 19, NUMBER 4 2005
Figure 14 (A) Preoperative frontal vertex view at age 1 month. (B) Postoperative
frontal view at age 8 months. (C) Postoperative frontal view at age 3 years.
When surgery is done, continued multidisciplinary treat- author’s series to date, there are no severe residual cleft
ment is continued based on a long-standing protocol, nasal deformities. However, the perfect nose without
and secondary procedures are performed, the result can deformity is unusual. Patients who have excessive scar-
be a normal, attractive child with minimally noticed ring of the nose and lip frequently have poor results.
deformity. This technique allows mobilization and Patients with compliant parents generally have good
repositioning of the alar cartilage and creation of a nasal contour throughout their growth after primary
near-normal nasal tip projection and alar base symmetry. surgery and may require only minimal surgery at a later
If the desired lip and nose shapes are not achieved, minor stage. With a critical eye on the results, the senior author
secondary procedures can be done, either before school reports that about 35% of the patients require early
age or when final surgical correction is performed. minor secondary procedures, generally performed before
It must be understood that subsequent treatment is the child begins school. Definitive repair after comple-
needed in all the patients. tion of growth is considered more of an aesthetic
Over the years, with gained experience, this correction to obtain optimal facial balance and harmony
procedure has been changed to give consistently im- rather than a major deformity correction. Almost all
proved and more predictable results. When the senior unilateral cases need septoplasty and turbinectomy dur-
author published his first 15 years of experience with this ing the course of care. The authors’ goal for these
technique, it showed that buckling of the alar cartilage children is to achieve facial harmony and balance, normal
and flattening of the nose were consistently eliminated speech, full dentition with normal occlusion, and a
on the cleft side as opposed to patients who had only lip beautiful smile, resulting in an attractive face with no
repair without treating the nose. Thus far, in the senior stigmata of clefting at a conversational distance.
UNILATERAL CLEFT LIP—APPROACH AND TECHNIQUE/SALYER ET AL 325
Figure 15 (A, C, E, G) Frontal views of the patient in Figure 14 at the ages of 5, 9, 16, and 17. (B, D, F, H) Occlusion in same ages
respectively (note orthodontic work over the years).
326 SEMINARS IN PLASTIC SURGERY/VOLUME 19, NUMBER 4 2005
Figure 17 (A–C) Lateral cephalograms of the same patient (Figs. 14–16) at ages 9, 16,
and 17.
and secondary cases. The senior author believes that cases.34 Their findings revealed that velopharyngeal
individually designed silicone nostril stents may further closure could be obtained in 92% of patients treated
improve this technique. Preoperative nostril expansion with two-flap palatoplasty at 8 months of age. Good to
using tissue expansion is unnecessary in patients with excellent facial aesthetic balance of the face, lip, and nose
unilateral cleft lip-nose defects because it is time con- could be achieved in all compliant patients receiving
suming and labor intensive. complete treatment. Excellent dental occlusion was
The authors are evaluating speech results, facial achieved with orthodontic treatment and orthognathic
balance, and dental occlusion in 50 random completed surgery when required.
328 SEMINARS IN PLASTIC SURGERY/VOLUME 19, NUMBER 4 2005