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Part VI
Reconstruction of Congenital Defects
t.me/Dr_Mouayyad_AlbtousH
Unilateral Cleft Lip Repair
21
AryaNamin andRyanF.Brown
Introduction
Cleft lip deformity is due to failure of the fusion of the paired medial nasal prominences and paired maxillary prominences during the sixth and seventh weeks of
embryonic development to form the upper lip [1]. Routine use of prenatal ultrasonography has led to antepartum diagnosis, which can be made as early as 18weeks
gestation [2]. Cleft lip has a male predominance, with a 2:1 ratio [1]. Cleft lip aficts
Asians and Native Americans most commonly (1in 500 births), while it aficts
Africans (1 in 2500 births) least commonly [1]. Cleft lip deformity has been
described since ancient times, and unfortunately, these individuals were treated
poorly due to a lack of understanding of embryology and superstitions of the times
[3]. In the sixteenth century, an embryologic etiology of the cleft was suggested, and
our understanding of this deformity has continued to evolve since that time [3]. One
of the rst documented cleft lip repairs was undertaken in China around 400
A.D. when a physician stitched the edges of the defect together and required strict
dietary restrictions and voice rest for 100days postoperatively [4]. Efforts at repairing cleft lips were again reported beginning in the fteenth and sixteenth centuries,
which were largely described as sacricing the margins of the cleft and suturing
them together [3]. This typically resulted in a vertical scar and shortening of the lip
[3]. This technique prevailed until the mid-nineteenth century when various techniques utilizing aps were described to improve the outcomes of cleft lip surgery [5].
A. Namin
Department of Otolaryngology and Facial Plastic Surgery Associates, Fort Worth, TX, USA
R. F. Brown (*)
Department of Head and Neck Surgery/Facial Plastic Surgery, The Permanente Medical
Group, Santa Rosa, CA, USA
e-mail: Ryan.f.brown@kp.org
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2024
F. Sokoya, A. G. Vincent (eds.), Manual of Head and Neck Reconstruction,
https://doi.org/10.1007/978-3-031-65999-7_21
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A. Namin and R. F. Brown
In the 1940s and 1950s, various techniques utilizing lateral tissue aps were
described and gained popularity [6–8]. The introduction of the rotation- advancement
repair of the cleft lip deformity by Dr. Millard in 1955 offered a new technique that
preserved the three-fourths of a normal Cupid’s bow, one philtral column, and the
philtral dimple on the medial side of the cleft [9]. Dr. Millard’s technique has been
modied by himself and many other surgeons since its original description and is
the most popular method of repair today [9]. Mohler described his modication of
the rotation-advancement ap in which he placed the back cut into the columellar
base with the goal of creating a mirror image of the philtral column on the noncleft
side [10]. Similar to Mohler’s objective in preserving the philtral column of the
noncleft side, the anatomic subunit approximation technique also sought to preserve
the non-cleft phitral column and reconstruct a mirror image of this anatomy [11].
Anatomy
Lip development occurs during 4–8weeks of embryonic development [1]. Cleft lip
deformity is due to failure of the fusion of the paired medial nasal prominences and
paired maxillary prominences during the sixth and seventh weeks of embryonic
development to form the upper lip [1]. The primary palate forms from the fusion of
the paired medial nasal processes by 6weeks gestation, giving rise to the premaxilla
[1]. Unilateral cleft lip presents with a wide spectrum of severity ranging from complete to more subtle incomplete clefts. Incomplete cleft lips have cutaneous continuity between the medial and lateral lip segments. These lesser-form cleft lips have
been subdivided into three subgroups: minor-form cleft extends 3mm or more
above the normal Cupid’s bow peak, microform cleft is characterized by a notched
vermilion junction with Cupid’s bow peak elevated less than 3mm above the normal side, and mini-microform cleft is characterized by a discontinuous vermilion
without elevation of the Cupid’s bow peak and a hypoplastic mucosal free margin
[12]. Bilateral cleft lip similarly presents with a spectrum of severity, with the complete bilateral cleft lip representing about 54% of these cases [12]. Patients with
symmetrical incomplete cleft lip account for 22% of cases, and patients with asymmetrical cleft lip account for 23% of cases [12]. In bilateral complete cleft lip, the
premaxilla grows independently of the maxilla on either side and subsequently may
protrude considerably [1]. The columella is considerably shorter, the nasal tip is
broad and at, and the alar bases are overly wide [13]. The protrusive premaxilla
necessitates preoperative dentofacial orthopedics to allow for optimal results in a
single-staged repair [13].
In unilateral cleft lip, the premaxilla is outwardly rotated and projecting, while
the lateral maxillary segment is retropositioned and medially displaced [14]. The
nose has characteristic deformities in unilateral cleft lip. The caudal septum and
nasal spine are displaced into the noncleft nostril, and there is unilateral shortness
of the columella [14]. The lateral crura of the lower lateral cartilage is attened, the
alar base is displaced laterally, inferiorly, and posteriorly, and the vestibular lining
is decient [14]. The orbicularis oris muscle in the lateral lip inserts into the alar
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21 Unilateral Cleft Lip Repair
277
wing, and the muscle in the medial lip between the midline and the cleft is hypoplastic [14]. The curves of the normal upper lip have been likened to the shape of
Cupid’s bow throughout history [15]. In the normal lip, the cutaneous vermillion
junction, or white roll, appears to be histologically related to the pars marginalis of
the orbicularis oris [15]. The philtral columns are histologically related to decussating orbicularis oris muscle bers with musculocutaneous insertions [15]. In patients
with cleft lip, the pars marginalis of the orbicularis oris is underdeveloped; therefore, the white roll is not present around the cleft [15].
Indications
Treatment of cleft lip requires surgical repair. This typically involves a multidisciplinary team and can involve a course in presurgical orthopedics. The timing of
surgical repair and the decision of whether to address the nasal deformity at the time
of cleft lip repair is something that is debatable and inuenced by surgeon preference and case-specic variables [1, 16–18]. In regards to the timing of repair, the
rule of 10s has been proposed [19]. This requires the patient to weigh 10 pounds,
have a hemoglobin of 10mg/ dL, a white blood cell count of less than 10,000/m3,
and an age of 10weeks [1, 19]. Worldwide, it has been found that 66% of surgeons
repair cleft lip when the patient is 3–6months of age, while 33% of surgeons repair
the cleft lip when the patient is less than one month old [16]. The advantages of
waiting until 3–6months of age include allowing for a course of presurgical orthopedics and also to allow for additional growth. While some argue that anesthetic
risks are decreased, others argue that the anesthetic risk is not different between a
3-month-old child and a healthy full-term newborn [1, 16, 20]. Multiple groups
have reported good aesthetic outcomes with cleft lip repair during the rst month of
life in healthy full-term babies and have argued for the benets of improved feeding
and improved psychological effects for the family [16, 20].
During the rst part of the twentieth century, repair of the cleft lip nasal deformity was delayed for fear of disturbing growth. However, over the past several
decades, the safety and efcacy of primary rhinoplasty during the cheiloplasty have
been well reported on [18, 21, 22]. With the advent of nasoalveolar molding and
postoperative nasal stenting, long-term aesthetic results have improved beyond
what primary rhinoplasty alone could achieve. In a retrospective study examining
nostril height and width ratios at 5years of age after primary rhinoplasty, nasoalveolar molding, or nasoalveolar molding and primary rhinoplasty, it was found that
patients who underwent both nasoalveolar molding and primary rhinoplasty with
overcorrection of nostril height had improved outcomes [23]. Managing the bilateral cleft nasal deformity is particularly challenging, and the utility of presurgical
orthopedics in these patients has been described as well [17, 24].
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A. Namin and R. F. Brown
Preoperative Planning
Patients with cleft lips should be under the care of a multidisciplinary team, including a pediatrician, dietician, feeding/speech specialist, orthodontist, and social
worker. Weight gain should be carefully monitored in the preoperative period.
Patients with syndromes or comorbidities should be under the care of appropriate
pediatric specialists.
Efforts to minimize the cleft lip deformity preoperatively with the goals of
improving the ease of repair and hopefully long-term outcomes have long been
attempted [25–28]. It was noted that the cleft lip nasal deformity was largely due to
deformity of the lower lateral cartilage and nasal base and that auricular deformities
could be successfully managed in a nonoperative fashion in neonates [28].
Subsequently, nasal molding was undertaken to partially correct the nasal deformity
prior to denitive repair. Lip taping and adhesion were also described in attempts to
narrow wide clefts [25, 26]. While lip adhesion did narrow the cleft width, the disadvantages of lip adhesion being a separate general anesthetic and the potential for
scarring that would potentially impair or limit denitive repair have led this technique to be utilized less frequently during the current era [25]. Lip taping did not
require an anesthetic but did not have an effect on the nasal form or alveolar deformity. Lip taping is commonly utilized with nasoalveolar molding. The objectives to
improve the nasal, lip, and alveolar deformity prior to denitive repair eventually
led to the description and more widespread use of nasoalveolar molding.
An important consideration in preoperative planning is presurgical orthopedics
and, more specically, nasoalveolar molding, which was introduced by Grayson in
the late 1990s [27, 29]. It was found that molding techniques could improve the
nasal deformity in cleft lip prior to repair and, therefore likely improve the longterm outcome [28]. In nasoalveolar molding, an orthodontic device is made to mold
to the alveolar segments that narrow the gap and also have a nasal component that
extends from the alveolar device on a wire to mold the shape of the lower lateral
cartilage and nasal base [29–31]. The Grayson and Figueroa techniques are the two
most commonly utilized techniques in nasoalveolar molding, and a study comparing the outcomes of these techniques found no signicant differences in the longterm postsurgical outcomes [29]. The vast majority of studies support that
nasoalveolar molding improves the outcomes of cleft lip repair and that better outcomes can be achieved in more severe deformities [27, 30, 31]. The nasolabial
deformity in the bilateral complete cleft lip is particularly challenging to address,
and patients who have undergone preoperative nasoalveolar molding were found to
have signicantly improved columellar length and require signicantly less secondary nasal surgery [32].
Instruments/Equipment
• Bipolar electrocautery
• Skin hooks
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21 Unilateral Cleft Lip Repair
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• Forceps (Adson, 0.5mm, Bishop-Harmon, etc.)
• Fine-tipped dissecting scissors (tenotomy, converse, curved iris, etc.)
• Frazier tip suction
• Caliper
• Needle driver
• Elevator (Cottle, Freer)
• Dissolvable sutures
Surgical Technique
Marking
The surgical procedure begins with the marking of the anatomical landmarks with a
Caliper. Methylene Blue can be used, but the authors like to use a 25- or 27-gauge
needle and stab the tip of the needle into the tip of a blue marking pen. This tends to
run less than Methylene Blue. The needle can then be used to mark the lip landmarks and tattoo the dermis. If the tip of the needle penetrates the dermis of the skin,
then the surgical prep will not remove the markings.
The markings (see Fig.21.1) that are made include:
1. The depth of cupid’s bow (1).
2. The height of cupid’s bow on the noncleft side (2).
3. Then, use the distance between the depth of the cupid’s bow on the non-cleft side
and then mark the same distance from the cupid’s bow on the cleft side (3).
4. Measure the height of the philtral column on the noncleft side (4 to 2). Then
measure the philtral column on the cleft side (5 to 3).** The difference between
these two philtral columns will be the length/width of the C-ap.
5. Mark 1mm up on the columella, 3/5th of the way on the columella away from
the cleft. (The cutting line will be from 3 to 6).
6. Mark the back cut from the point marked on the columella at a 90-degree angle
(point 7). The length of the back cut will be the length of the C-ap measured in
step #4. (If there is a large asymmetry and a long C-ap is needed, the back cut
should not cross the philtral column, but once the philtral column is reached, the
cut should follow the philtral column inferiorly.)
7. Take the measurement from the noncleft side philtral column (4 to 2), and with
the caliper, nd a point medial to the alar base and vermillion on the cleft side
and mark that and the point on the cleft-side vermillion that equals that measurement (8 to 9).
8. Measure the visible lip where the cut through the red lip will happen on the cleft
and noncleft sides and try to have them match.
9. The aps in the surgery can be marked. The authors prefer to use the above
points and make dots, as they nd it less messy and does not smear as much. But
aps can be drawn out once the above points have been marked. The C-ap will
be skin only and is lateral to the 3–6 philtral column cut and does not include lip
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A. Namin and R. F. Brown
6
4
7
2
1
5
C
M
3
8
L
9
Fig. 21.1 Markings for unilateral cleft lip repair
mucosa. It should be based just posterior to the cleft-side medial crura in the
nose. The M-ap is the lip mucosa and vermillion just inferior to the C-ap skin
on the noncleft side. It is a superiorly based ap. The M-ap (medial ap) can be
used to help repair the nasal oor in a wide cleft. But it is best to save all tissue
until you are sure that it is not needed. The L-ap (lateral ap) is the lip vermillion and mucosa just medial to the planned philtral column cut on the cleft side
(8 to 9). It is a superiorly based ap and is always used to add tissue underneath
the nostril rim, so that the ala can be curved on the cleft side. It can also be used
to repair a nasal oor defect.
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The lip is then injected with local anesthetic with epinephrine to decrease
bleeding. The authors like to use a mixture of 1% lidocaine with 1:100,000 epinephrine and 0.25% plain Marcaine. The face is then prepped, and the patient is
draped in the usual sterile fashion (See Fig.21.2).
Surgical Steps/Incisions
1. Incise from the height of Cupid’s bow on the cleft side to the point marked on the
columella and then cut the measured length of the back cut (3 to 6). The authors
like to use an 11 blade to be able to make nice straight cuts along the planned
incision lines.
2. Cut along the vermillion lateral from the 3 to 6 cut (do not include any) and raise
the C-ap. Keep the C-ap thick so that it is well-vascularized. Extend the elevation of the ap to just posterior to the footplate of the medial crura in the cleftside nostril.
3. Raise the M-ap. If it is a complete cleft lip, then the M ap can be continued
posteriorly into the nose and onto the septum. This can be used to close a wide
nasal oor defect. Elevation of the septal ap is best done with a caudal or freer
elevator and should be elevated right on top of the cartilage.
4. Make the cut along the cleft-side that was marked 8 to 9. Once you reach the
vermillion it is possible to save a V shape of extra vermillion that can be used to
plump up the central lip.
Fig. 21.2 Pre-op markings and post-op result
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A. Namin and R. F. Brown
5. Cut along the upper gingival buccal sulcus of the cleft side. Then, elevate the soft
tissue that overlies the maxilla. The width of the cleft will determine how aggressive you need to be in this elevation. It is best to put a nger on the eye because
the distance is not far in infants. Care should be taken to not damage the infraorbital nerve. The authors prefer a supraperiosteal dissection.
6. Raise the L-ap. This is a mucosal ap. The superior cut of the ap will be cut
right up to the superior lateral edge of the head of the inferior turbinate and the
ap is based on the inferior turbinate. This cut should extend down to the piriform aperture and the soft tissue can be freed-up supraperiostally off of the maxilla and join the elevation that occurred through the upper gingival buccal sulcus
incision. This should free up the cleft side lip so that it can be advanced without
tension (See Fig.21.3).
7. Using ne-tipped scissors (curved iris or converse), insert them between the
medial crura and separate them. Then turn the scissors and come up over the
domes and over the lower lateral cartilages. Separate the overlying skin from the
cartilage. This will allow you to reposition the lower lateral cartilages more symmetrically. The cleft-side lower lateral cartilage, which is long and attened, can
nish being separated from the overlying skin by dissecting from the piriform
and upper gingival-buccal sulcus cuts. A completely dissected tunnel should
exist along the cleft side lower lateral cartilage and ala to break up abnormal
attachments and allow it to be repositioned. All muscle attachments to the alar
base on the cleft side should be removed.
8. Dissect free orbicular oris muscle pendants on both sides of the lip, but do not
dissect past the philtral columns.
Closing/Suturing
1. Inset the L-ap into the piriform aperture incision. This will provide extra tissue
to be able to curve the ala. If the nasal oor has a defect, the L-ap can be inset
into the piriform aperture incision but also bent over at its midpoint and attached
to the M-ap/septal ap on the non-cleft side. If present, the nasal oor defect
should be xed with a combination of the M-ap/septal ap and L-ap, and any
extra tissue should then be trimmed. Chromic or Vicryl suture can be used for
this step. It is an important step to ll in the nasal oor defect, as it an area hard
to reach in later cleft palate repair, and can leave an anterior stula.
2. Using a 3-0 or 4-0 Vicryl suture, grab the lateral gingiva-buccal tissue and
advance it medially and secure to the gingival periosteum. This will help take
tension off of the repair.
3. Close the orbicularis oris muscle together. Make sure that you rotate and advance
the lip and see where the muscle should line up on each side. Mattress sutures
can be used to try to evert the muscle to aid in creating a philtral column if
desired. Vicryl or Monocryl suture can be used. If the muscle is reapproximated
correctly, this should almost completely align the cleft side ala.
4. Place a deep layer of dissolvable suture such as Vicryl or Monocryl.
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