Otolaryngol Clin N Am

40 (2007) 27–60

Cleft Lip and Palate

Oneida A. Arosarena, MD
Department of Otolaryngology, Temple University School of Medicine,
3400 North Broad Street, Kresge First Floor, Suite 102, Philadelphia, PA 19140, USA

Orofacial clefts are the most common craniofacial birth defects, second
only to clubfoot in frequency of major birth anomalies [1]. Patients who
have cleft lip or palate face significant lifelong communicative and aesthetic
challenges, and difficulties with deglutition. The complex medical, ancillary,
and psychosocial interactions necessary in the management of these patients
warrants a multidisciplinary team approach [2]. Care of the cleft patient can
be both challenging and rewarding.

Epidemiology
The overall incidence of orofacial clefting is typically quoted as 1 in 700
live births [3–5]. Cleft lip, with or without cleft palate (CL[P]), is an epide-
miologically and etiologically distinct entity from isolated cleft palate (CP)
[3,6]. Cleft lip is associated with cleft palate in 68% to 86% of cases [7]. The
incidence of CL(P) varies significantly by racial group and with socioeco-
nomic status, with an incidence of 1 in 1,000 births in whites, 1 in 500 births
in Asians and Native Americans, and approximately 1 in 2,400 to 2,500
births in people of African descent [3,7]. The incidence of CP does not
have the same ethnic heterogeneity and is typically quoted as 1 in 1,500 to
2,000 live births [4,5,7]. Between 60% and 80% of CL(P) patients are
male, but a predominance of female infants affected by isolated cleft palate
has been recognized [1,4,5,7]. Unilateral CL(P) is twice as common as bilat-
eral CL(P), and usually affects the left side [7].

Causative factors
Although most children who have orofacial clefts are otherwise normal,
the proportion of affected individuals who have recognized patterns of

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28 AROSARENA

malformation has increased steadily over the years as cleft teams have incor-
porated the services of geneticists and dysmorphologists (Tables 1 and 2).
More than 300 syndromes are known to be associated with orofacial cleft-
ing, but CP is more likely to be syndromic than CL(P). Approximately
14% to 30% of CL(P) cases are associated with multiple anomalies com-
pared with 42% to 54% of CP cases [3,6–8].
The cause of isolated orofacial clefting is believed to be multifactorial [1].
Although clefting tends to cluster in families, its inheritance is not usually
Mendelian and the discordance rate in monozygotic twins can be between
40% and 60% [4,6]. Several growth and transcription factors, receptors, po-
larizing signals, vasoactive peptides, cell adhesion proteins, extracellular
matrix components, and matrix metalloproteinases are involved in palatal
development. These biomolecules are expressed in a tightly controlled com-
plex cascade, disturbance of which can result in orofacial clefting [5]. CL(P)
has been associated with defects in the genetic loci for growth and transcrip-
tion factors transforming growth factor-alpha (TGF-a), TGF-b2, TGF-b3,
interferon regulatory factor-6 (van der Woude and popliteal pterygia
syndromes), T-BOX 22 (X-linked cleft palate with ankyloglossia), P63

Table 1
Multiple malformation syndromes associated with cleft lip with or without cleft palate
Recognized patterns with
Genetic disorders unknown genesis Teratogensa
Down syndrome Amniotic band sequence Anticonvulsant
phenotype
Smith-Lemli-Opitz syndrome Aicardi syndrome Fetal alcohol
syndrome
Aarskog syndrome Kabuki make-up syndrome Maternal diabetes
Coffin-Siris syndrome Craniofrontonasal dysplasia Maternal smoking
van der Woude syndrome Hypertelorism microtia Maternal folate
clefting syndrome deficiency
Waardenburg syndrome Focal dermal hypoplasia d
syndrome
Ectodermal dysplasia syndromes d d
(Ectrodactyly-ectodermal
dysplasia-clefting, Hay-Wells,
and Rapp-Hodgkin syndromes)
Distal arthrogryposis type 2 d d
Fryns syndrome d d
Popliteal pterygium syndrome d d
22q deletion syndromes (DiGeorge d d
syndrome, Shprintzen syndrome,
and CHARGE association)
Wolf-Hirschhorn syndrome d d
Basal cell nevus syndrome d d
Kallman syndrome d d
Nail patella syndrome d d
a
Indicates increased risk rather than direct causation [3,6,11–13].
 CLEFT LIP AND PALATE 29

Table 2
Multiple malformation syndromes associated with cleft palate
Recognized patterns with
Genetic disorders unknown genesis Teratogensa
Down syndrome Pierre-Robin sequence Anticonvulsant phenotype
Prader-Willi syndrome Goldenhar syndrome Fetal alcohol syndrome
Camptomelic dysplasia Kabuki make-up syndrome Thalidomide
Stickler syndrome Mobius sequence Dioxin
Holoprosencephaly Klippel-Feil syndrome Maternal smoking
de Lange syndrome Silver-Russell syndrome d
Spondyloepiphyseal dysplasia Beckwith-Wiedemann d
congenita syndrome
Treacher-Collins syndrome d d
Cleft palate–short stature d d
syndrome
22q deletion syndromes d d
(DiGeorge syndrome,
Shprintzen syndrome, and
CHARGE association)
Diastrophic dysplasia d d
Orofaciodigital syndrome type I d d
Otopalatodigital syndrome type I d d
Limb mammary syndrome d d
Nager syndrome d d
Smith-Lemli-Opitz syndrome d d
X-linked cleft palate with d d
ankyloglossia
Apert syndrome d d
Marfan syndrome d d
Turner syndrome d d
Cleidocranial dysostosis d d
a
Indicates increased risk rather than direct causation [3,6,9,10,56–58].

(ectodermal dysplasia syndromes), Msx1, and the goosecoid transcription

factor; for the vasoactive peptide endothelin-1 (22q deletion syndromes);
for the retinoic acid receptor-alpha and the fibroblast growth factor recep-
tor-1 (Kallman syndrome); for the cell adhesion molecule nectin-1 (ectoder-
mal dysplasia syndromes); and several other genes whose functions have not
yet been elucidated [3,9–13]. Similarly, CP has been associated with defects
in the genetic loci for TGF-a, TGF-b3, T-BOX 22, and P63 (limb mammary
syndrome); for the polarizing factor sonic hedgehog (holoprosencephaly);
and the extracellular matrix proteins collagen type II and procollagen
type XI (Stickler syndrome) [5,9,10,14]. Single gene disorders are believed
to cause only 15% of clefts. The phenotypic heterogeneity demonstrated
in the single gene disorders and variable penetrance, even in monozygotic
twins, suggest that environmental factors also contribute to orofacial cleft-
ing. The role of epigenetic influences, such as maternal smoking, maternal
alcohol use, folate deficiency or disordered metabolism, steroid and statin
30 AROSARENA

use, and retinoid exposure, is currently being investigated (see Tables 1 and 2)
[9,10,13,15].

Embryology
Human facial development begins during the fourth week of intrauterine
life when neural crest cells migrate and combine with the mesoderm to form
the facial primordia [5]. The philtrum and primary palate (that portion of
the palate and alveolus anterior to the incisive foramen) begin to form at ap-
proximately 35 days’ gestational age by the coalition, growth, and differen-
tiation of three embryonic prominences or processes (Fig. 1). The central
segment of the face, comprising the forehead, supraorbital ridges, nose, phil-
trum, and primary palate, is derived from the frontonasal process. The in-
termaxillary segment of the frontonasal process is itself formed by the
fusion of the two medial nasal prominences. This intermaxillary segment
gives rise to the philtrum and that portion of the maxilla that bears the in-
cisor teeth [14]. During the fifth and sixth weeks of intrauterine develop-
ment, medial growth of the maxillary prominences, derived from the first
branchial arches, results in fusion of the medial nasal and maxillary prom-
inences to form the upper lip and anterior alveolus. Failure of fusion results
in cleft lip and alveolus.
Formation of the secondary palate follows that of the primary palate.
The secondary palate (that portion of the palate posterior to the incisive fo-
ramen) forms through the fusion of two paired outgrowths of the maxillary
prominences, the palatal shelves (Fig. 2). The palatal shelves appear during
the sixth week of development as vertical projections into the oral cavity on
either side of the tongue. During the seventh week, the shelves elevate, as-
sume a horizontal orientation, and fuse, closing the secondary palate.
This fusion begins at the incisive foramen, progresses toward the posterior
palate, and is complete at about the 12th week of intrauterine life. Failure
of fusion results in a cleft palate (Fig. 3). The severity of the palatal cleft
varies from submucous clefting to complete bilateral clefting extending to
the maxillary alveolus [16]. Although the tongue does not participate in pal-
atal closure in the normal situation, altered tongue position may mechani-
cally block fusion of the palatal shelves, as in the Robin sequence. The
tongue musculature is known to become functional at about the time of pal-
atal shelf elevation [14].

Preoperative assessment
Initial assessment and identification of associated anomalies
The initial assessment of the infant born with an orofacial cleft includes
a birth history, thorough head and neck examination, and examination of
the infant’s extremities to identify associated malformations (see Tables 1
 CLEFT LIP AND PALATE 31

Fig. 1. (A-H) Intrauterine midfacial development, 5 weeks to 10 weeks. (From Moore KL. The
branchial apparatus and the head and neck. In: Moore KL, editor. Before we are born: basic embry-
ology and birth defects. 3rd edition. Philadelphia: WB Saunders; 1989. p. 134–58; with permission.)
32 AROSARENA

Fig. 2. Intrauterine development of secondary palate, 6 weeks to 12 weeks. (From Moore KL.
The branchial apparatus and the head and neck. In: Moore KL, editor. Before we are born:
basic embryology and birth defects. 3rd edition. Philadelphia: WB Saunders; 1989. p. 134–58;
with permission.)
 CLEFT LIP AND PALATE 33

Fig. 3. Infant with complete unilateral cleft palate.

and 2). A history of intrauterine growth retardation may indicate Smith-

Lemli-Opitz or Wolf-Hirschhorn syndrome. Down-slanting lateral canthi
may indicate Treacher-Collins or Aarskog syndrome, whereas up-slanting
lateral canthi and epicanthal folds indicate Down or Smith-Lemli-Opitz syn-
drome. Down-slanting lateral canthi with hypertelorism, blepharoptosis,
epicanthal folds and colobomata are physical signs present in Wolf-
Hirschhorn syndrome. Hypertelorism, blepharoptosis, and a simian crease
are also characteristic of Aarskog syndrome. Ankyloblepharon with entro-
pion and absent eyelashes indicate Hay-Wells syndrome.
Auricular abnormalities can occur with any of the 22q deletion syn-
dromes and Treacher-Collins syndrome. Unilateral microtia or anotia
with hemifacial microsomia typifies Goldenhar syndrome. An enlarged, cau-
liflower, or calcified auricle, and associated laryngotracheomalacia suggest
diastrophic dysplasia.
CL(P) or CP with lower lip pits is pathognomonic for van der Woude syn-
drome. A grimace should be elicited to assess facial nerve function to rule out
Mobius sequence. Digital malformations or agenesis characterize Aarskog,
Coffin-Siris, de Lange, Nager, Fryns, Smith-Lemli-Opitz, Silver-Russell,
limb mammary, ectrodactyly-ectodermal dysplasia-clefting and otopalatodi-
gital syndromes, and amnion rupture sequence [1,6,11]. Patients who have or-
ofaciodigital syndrome manifest hamartomas or lipomas of the tongue and
digital malformations. Infants who have Robin sequence, otopalatodigital
syndrome, Nager syndrome, Smith-Lemli-Opitz syndrome, Kabuki syn-
drome, Silver-Russell syndrome, and Stickler syndrome have characteristic
micrognathia [9,17,18]. As upper airway compromise complicates several of
the syndromes associated with CP, these patients may require immediate sta-
bilization by positioning, tongue-lip adhesion, mandibular distraction, or tra-
cheotomy in severe cases before palatal repair. Certainly, children who have
cleft with other malformations should be referred with their families to the
cleft team geneticist or dysmorphologist [19].
34 AROSARENA

Feeding
The most immediate concern in the care of the infant who has cleft, other
than the airway, is nutrition. The extent of the cleft often correlates with the
infant’s ability to feed. Patients who have clefts limited to the soft palate
usually have normal sucking, whereas infants who have hard palate clefts
are often unable to generate the negative pressure needed for normal suck-
ing because of the oronasal communication. Impaired sucking can lead to
weight loss and failure to thrive as the infant expends more energy in feeding
than he or she is able to ingest.
Early swallowing therapy is required in the infant who has a complete CP
to ensure near-normal feeding and growth. Parents can be taught to use
squeeze bottles with cross-cut nipples to increase the flow of formula in con-
cert with the infant’s suck. In general, most newborns who have clefts should
be able to ingest 2 to 3 ounces of formula with assistance within 20 to 30 min-
utes. Frequent burping is required during feeding because of aerophagia.
Alternatively, bottles with nipples specialized for CP feeding, such as the
Haberman feeder, can be used to limit air ingestion. Frequent assessments
by the cleft team speech and swallowing pathologist may be needed to estab-
lish parental confidence in feeding. Patients who fail to gain weight or dem-
onstrate excessive aerophagia may require placement of a palatal obturator
by the cleft team pedodontist. Patients who have CL(P) and associated pro-
truding premaxillae, particularly those who have bilateral clefts, should un-
dergo lip adhesion or premaxillary orthopedics at approximately age 12
weeks. Lip adhesion not only decreases the size of the palatal cleft by nor-
malizing the position of the premaxilla, but also restores the sphincter func-
tion of the orbicularis oris, which improves feeding. Monthly assessments by
the facial plastic surgeon are recommended to evaluate patient growth and
development, and more frequent follow-up by the cleft team pediatrician
may be needed in patients who have failure to thrive or developmental delay.
Otolaryngologic assessment
The abnormal insertion of the tensor veli palatini is believed to contribute
to Eustachian tube dysfunction, middle ear disease, and the conductive
hearing loss associated with CP. The placement of myringotomy tubes is
routine at the time of CP repair [7,20]. Because several multiple malforma-
tion syndromes associated with clefting (eg, Stickler syndrome, van der
Woude syndrome, Klippel-Fiel syndrome, Waardenburg syndrome, Down
syndrome, and diastrophic dysplasia) also manifest sensorineural hearing
loss, hearing assessment by auditory brainstem response testing or other
methods should be performed in the first months of life.
Psychosocial support
Families of infants who have clefts require counseling by a cleft team so-
cial worker or psychologist as they adjust to the stresses of caring for the
 CLEFT LIP AND PALATE 35

infant and frequent interaction with medical professionals. This is particu-

larly true of families whose infants were not diagnosed with a cleft while
in utero, who have limited resources or support, or who have infants who
have multiple anomalies. Stages of shock, denial, sadness, anger, and adap-
tation and reorganization have been described in parents of infants who
have clefts [21].

Unilateral cleft lip

Anatomy
The cleft lip deformity results from deficiency and displacement of soft
tissues, cartilage, and bone in the area of the cleft [7]. The principle muscle
of the lip is the orbicularis oris, which interdigitates with the other mimetic
muscles of the midface and lower face (Fig. 4A) [20]. In the cleft lip, there is
discontinuity of the orbicularis oris in the region of the cleft, and the fibers
of the orbicularis parallel the cleft margin, inserting on the alar base on the

Fig. 4. (A) Mimetic muscles of the lower face. (From Sykes J, Senders C. Pathologic anatomy of
cleft lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic
surgery. New York: Thieme Medical Publishers; 1993. p. 59; with permission.) (B) Abnormal
insertion of orbicularis oris in cleft lip. (From Sykes J, Senders C. Pathologic anatomy of cleft
lip, palate, and nasal deformities. In: Meyers AD, editor. Biological basis of facial plastic sur-
gery. New York: Thieme Medical Publishers; 1993. p. 61; with permission.)
36 AROSARENA

lateral side of the cleft, and on the columellar base and septum on the medial
side of the cleft (Fig. 4B) [7,19,20]. Moreover, the orbicularis oris is hypo-
plastic in the area of the cleft [20,22].
The abnormal muscular forces and maxillary osseous discontinuity result
in an outward rotation of the premaxillary-bearing medial segment and ret-
rodisplacement of the lateral segment (Fig. 5A). The muscular attachment
to the caudal septum is also believed to result in its displacement out of
the vomerine groove and into the noncleft nostril, which in turn results in
shortening of the columella. The philtrum is short on the cleft side, the
peak of Cupid’s bow is rotated superiorly, and the vermilion is also deficient
in the region of the cleft. In the nasal tip, the domes are separated and the
lateral crus is flattened on the cleft side [7].
The severity of cleft lip varies from clefts involving only the vermilion to
full-thickness clefts involving all tissue layers. A malformation consisting of
dehiscence of the orbicularis muscle with vermilion notching but intact over-
lying skin is termed a microform cleft [7,20]. An incomplete cleft lip spares
some of the superior portion of the upper lip. Anatomic dissections on still-
born infants reveal that the orbicularis oris muscle in the incomplete cleft lip
does not cross the cleft unless the cutaneous bridge is at least one third of the
height of the lip. Moreover, the orientation of the small amount of muscle
that bridges the cleft in this situation is abnormal [20,23].

Timing of cleft lip repair

Generally, cleft lip repair with primary tip rhinoplasty is performed at age
3 months. The patient’s overall health status, including the presence of other

Fig. 5. (A) Infant who has left complete cleft lip and palate. (B) Infant 1 month after rotation-
advancement repair and primary tip rhinoplasty. Note stenosis of nostril on left.
 CLEFT LIP AND PALATE 37

congenital anomalies, may dictate that repair of the cleft be delayed, how-
ever. Widely followed preoperative guidelines include the rules of ten:
weight at least 10 pounds, hemoglobin at least 10 g, white blood cell count
less than 10,000/mm3, and age more than 10 weeks [7,20,24]. Patients who
have wide complete unilateral clefts or bilateral clefts with marked premax-
illary protrusion may require staged repair with lip adhesion performed at
age 3 months and definitive repair performed at age 5 to 6 months. When
lip adhesion includes muscular repair across the cleft (eg, a Rose-Thompson
straight-line repair), it has the advantage of increasing the length of the cu-
taneous portion of the lip, which facilitates the definitive repair. Alterna-
tively, presurgical maxillary orthopedics may be used before lip repair in
the case of the wide cleft, but is associated with increased cost and burden
of treatment [7,25,26]. Lip adhesion may also be used with a passive molding
device to prevent collapse of the alveolar arch form [27].

Cheiloplasty techniques for the unilateral cleft lip

The first documentation of cleft lip repair occurred in the fourth century
AD in China. This simple technique involved freshening and approximation
of the cut cleft edges, and remained the standard of care until 1825 when von
Graefe proposed the use of curved incisions to allow lengthening of the lip.
His work provided the foundation for the Rose-Thompson technique and
other straight-line closure repairs introduced in the early 1900s [7,20]. The
straight-line closures, however, had the disadvantage of vertical scar con-
tracture leading to notching of the lip [20].
Several methods were developed to avert the scar contracture associated
with the straight-line closures. These included numerous geometric repairs
that were also designed to irregularize the lip scar [20]. In the 1950s, Tenni-
son and Randall introduced a triangular flap that created a Z-plasty in the
lower portion of the lip scar. All of these techniques produced scars that vi-
olated the philtrum, however [7,20].
The Millard rotation-advancement technique, introduced in 1957, is the
most widely used procedure for cleft lip repair because it places most of
the scar along the natural philtral border and is more flexible than the geo-
metric closures. Moreover, the Millard technique allows for complete mus-
cular repair and primary cleft rhinoplasty, and minimizes the discarding of
normal tissue. Its disadvantages include the need for extensive undermining
and the risk for nostril stenosis on the cleft side (Fig. 5B) [20]. The author’s
modification of the technique is described below.

Surgical technique
Flap design. Commonly used reference points for flap design are illustrated
in Fig. 6 and described as follows [20]:
Point 1: Center or low point of Cupid’s bow
Point 2: Peak of Cupid’s bow on noncleft side
38 AROSARENA

Fig. 6. Reference points for Millard rotation-advancement technique for unilateral cleft lip re-
pair. (From Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of
the unilateral cleft lip deformity. Facial Plast Surg 1993;9:169; with permission.)

Point 3: Peak of Cupid’s bow, medial side of cleft

Point 4: Alar base, noncleft side
Point 5: Columellar base, noncleft side
Point 6: Commissure, noncleft side
Point 7: Commissure, cleft side
Point 8: Peak of Cupid’s bow, lateral side of cleft
Point 9: Superior extent of advancement flap
Point 10: Alar base, cleft side
Point x: Back-cut point
The following measurements are made to ensure accuracy in marking the
reference points [20]:
1 to 2 ¼ 1 to 3 ¼ 2–4 mm
2 to 6 ¼ 8 to 7 z 20 mm
2 to 4 ¼ 8 to 10 ¼ 9–11 mm
3 to 5 þ x ¼ 8 to 9
Flap elevation. After the induction of general anesthesia, the patient is intu-
bated with an oral RAE tube. The reference points are marked, and the
patient’s lip is infiltrated with a few milliliters of local anesthetic with
1:200,000 epinephrine as a field block at the oral commissures to prevent dis-
tortion of the lip anatomy near the cleft. The rotation incision is marked
from point x to point 5 to point 3. A full-thickness rotation incision is
made and extended into the red lip to the wet line.
The advancement flap is elevated by incising along the vermilion-cutane-
ous junction from the height of Cupid’s bow medially (from point 8 to point
9, see Fig. 6) on the lateral margin of the cleft. This incision is also extended
 CLEFT LIP AND PALATE 39

into the red lip to the wet line (Fig. 7). The skin is elevated off of the orbi-
cularis oris muscle for approximately 1 cm on both sides of the cleft. Bilat-
eral gingivolabial sulcus incisions are made, which extend to the cleft
margins. The soft tissues of the lip and cheek are elevated off of the maxilla
in a supraperiosteal plane, using blunt dissection and preserving the infra-
orbital nerves. This elevation may continue as superiorly as the level of
the nasal bones to allow maximal flap advancement and rotation, and ten-
sionless closure if the cleft is wide. The orbicularis is freed from its abnormal
attachments to the columellar base and alar margin on the lateral side of the
cleft. The alar margin on the cleft side is released from its attachment to the
piriform aperture using an internal alotomy [20]. The advancement flap el-
evation is completed by incising along the nasal sill (point 9 to point 10, see
Fig. 6) but this incision may be extended to the alar facial groove if neces-
sary (point 9 to point 11 or 12, see Fig. 6). The c-flap is elevated after incis-
ing along the vermilion-cutaneous junction from the height of Cupid’s bow
medially on the medial margin of the cleft (Fig. 7).

Closure. The intraoral mucosa is closed with dissolvable suture. The gingi-
volabial sulcus mucosa may be attached to the nasal spine to elevate the gin-
givolabial sulcus. The orbicularis oris is reconstituted across the cleft with
semipermanent suture (Fig. 8). The alar base on the cleft side is medialized
by placement of a subcutaneous stitch from the alar base to the periosteum
of the nasal spine. The c-flap may be rotated into the nasal floor to prevent
stenosis of the nostril on the cleft side, or it may be discarded. The skin clo-
sure in the nasal floor is performed with 6-0 monofilament fast-absorbing
suture. The lip skin closure is performed with 5-0 monofilament subcuticular
sutures (Fig. 9). The skin closure is reinforced with surgical skin tape.

Fig. 7. Flaps in Millard rotation-advancement technique for unilateral cleft lip repair. (From
Ness JA, Sykes JM. Basics of Millard rotation-advancement technique for repair of the unilat-
eral cleft lip deformity. Facial Plast Surg 1993;9:171; with permission.)
40 AROSARENA

Fig. 8. Repair of orbicularis oris muscle. (From Ness JA, Sykes JM. Basics of Millard rotation-
advancement technique for repair of the unilateral cleft lip deformity. Facial Plast Surg
1993;9:174; with permission.)

Tip rhinoplasty. Primary cleft rhinoplasty lessens the cleft nasal deformity
[7,20,28]. Wide undermining of the nasal skin from the underlying nasal car-
tilages is performed either through the lip incision or an alar margin inci-
sion. The vestibular skin is not dissected from the alar cartilages to
minimize the risk for alar stenosis [20]. The dome on the noncleft side is

Fig. 9. Placement of rotation and advancement flaps. (From Ness JA, Sykes JM. Basics of Mill-
ard rotation-advancement technique for repair of the unilateral cleft lip deformity. Facial Plast
Surg 1993;9:173; with permission.)
 CLEFT LIP AND PALATE 41

delivered, and the dome is recreated and elevated in the cleft alar cartilage
using an interdomal suture. Alternatively, the dome on the cleft side may
be repositioned with external bolsters [7,20]. If an alar margin incision is
used, it is closed with 5-0 chromic gut suture. Arm splints are placed before
the patient is awakened.

Bilateral cleft lip

Anatomy
In the bilateral cleft lip, the orbicularis oris muscle inserts on both alar
margins, and no muscle fibers invade the prolabium. Unrestrained growth
of the vomer and nasal septum result in protrusion of the premaxilla
(Fig. 10) [7,26]. The prolabial skin is flat, lacking philtral ridges, a philtral
dimple, and Cupid’s bow. The columella is very short, and both lateral crura
are flattened, resulting in alar flaring [7]. The advantage of the bilateral cleft
lip is symmetry [29].

Fig. 10. (A, B) Infants who have bilateral cleft lip and maxillary protrusion; (C) Child in (B)
following lip adhesion.
42 AROSARENA

Cheiloplasty techniques for the bilateral cleft lip

Early bilateral cleft lip repair techniques involved excision of the premax-
illa and prolabium, resulting in an unnatural appearance to the upper lip
and deleterious effects on midfacial growth [7,26]. Later, premaxillary set-
back with vomerine osteotomies was popularized in the 1800s to manage
premaxillary protrusion. This technique was also associated with significant
midfacial growth hindrance, however [7,19]. Current bilateral cleft cheilo-
plasty techniques are modifications of Millard’s bilateral straight-line re-
pair. Modern principles that guide the repair of the bilateral cleft lip are
[7,29]:
Symmetry
Primary muscular continuity
Proper philtral size and shape
Formation of the median tubercle from lateral lip elements
Primary positioning of alar cartilages to construct the nasal tip and
columella

Surgical technique
Flap elevation. The patient’s lip is infiltrated with local anesthetic with
1:200,000 epinephrine as a field block at the oral commissures to prevent
distortion of the lip anatomy near the clefts. The prolabial skin is also
infiltrated with a small amount of local anesthetic. The prolabial vermil-
ion-cutaneous junction is incised. The mucosa of the prolabium is dissected
from the premaxilla in a supraperiosteal plane, and is turned down to line
the premaxillary gingivolabial sulcus.
The philtral flap is designed based on the child’s ethnicity, with suggested
dimensions of 6 to 8 mm of length, 2 mm of width at the columellar-labial
junction, and 3 to 4 mm of width between the peaks of Cupid’s bow for
Caucasians. These measurements are based on Mulliken and colleagues’
[30] prospective anthropometric study of 46 Caucasian children who had bi-
lateral clefts who were compared with normal children. Slightly wider phil-
tral flaps are used for children of other ethnicities, but these flaps should
rarely exceed 5 to 6 mm in width [7,30]. Because of the tendency for the phil-
trum to widen in children who have bilateral clefts, Mulliken’s philtral flaps
are designed with concave sides and a dart-shaped tip (Fig. 11) [29]. The re-
mainder of the prolabial skin may be discarded, or flanking strips of skin
may be deepithelialized on each side of the philtral flap for additional height
to re-create the philtral ridges [25,29]. In addition, small bilateral c-flaps
may be designed to line the nasal floor. The prolabial skin is elevated in
a supraperiosteal plane to the level of the nasal spine.
The advancement flaps are elevated by incising along the vermilion-
cutaneous junction from the height of Cupid’s bow medially on the lateral
lip elements. These incisions are extended into the red lip to the wet line.
 CLEFT LIP AND PALATE 43

Fig. 11. Repair of the bilateral cleft lip. (From Mulliken JB. Primary repair of bilateral cleft lip
and nasal deformity. Plast Reconstr Surg 2001;108:186; with permission.)

The mucosal flaps created by these incisions along the cleft margins are dis-
sected supraperiosteally, and sutured to the prolabial mucosa with absorb-
able suture, thus effecting bilateral gingivoperiosteoplasty.
The skin is elevated off of the orbicularis oris muscle for approximately
1 cm on the lateral elements of the cleft. Bilateral gingivolabial sulcus inci-
sions are made, which extend to the cleft margins. As with repair of the uni-
lateral cleft lip, the soft tissues of the lip and cheek are elevated off the
maxilla in a supraperiosteal plane. The orbicularis is freed from its abnor-
mal attachments to the alar margins using internal alotomies [20]. Elevation
of the advancement flaps is completed by incising along the nasal sills.
44 AROSARENA

Closure. The intraoral mucosa is closed as described for repair of the unilat-
eral cleft lip, and the orbicularis oris is reconstituted across the cleft. The
alar bases are medialized by placement of subcutaneous stitches from the
alar bases to the periosteum of the nasal spine. The dermis of each alar
base is sutured to the underlying muscle to prevent alar elevation with smiling
[29]. The skin closure in the bilateral nasal floor is performed with 6-0
monofilament fast-absorbing suture. The lip skin closure is performed with
5-0 monofilament subcuticular sutures. The skin closures are reinforced
with surgical skin tape.

Tip rhinoplasty. The nasal correction is performed through alar rim inci-
sions as described previously for the unilateral cleft nasal deformity. The
alar domes are recreated and elevated using intradomal and interdomal su-
tures. The alar margin incisions are closed and arm splints are placed before
the patient is awakened.

Postoperative care
After cleft lip repair, the patient is hospitalized until oral intake is suffi-
cient. Feeding is resumed with a syringe or cup. Arm splints are maintained
for the first two postoperative weeks to prevent patient disruption of the lip
repair.

Complications and their management

Notch in the vermilion. This complication indicates incomplete muscular re-
pair or dehiscence of the inferior portion of the orbicularis oris repair. It is
corrected by reapproximation of the lowest portion of the lip muscle. The
overlying mucosa may be excised, or a V-Y advancement performed.

Malalignment of Cupid’s bow or whistle deformity. This condition is fre-

quently caused by contracture of the lip scar, and can be prevented by place-
ment of a Z-plasty at the vermilion-cutaneous junction during primary
cheiloplasty. The scar may be excised secondarily, and the vermilion cor-
rectly repositioned with a Z-plasty to prevent recurrence of the deformity.

Absence of the median tubercle and part of Cupid’s bow. Commonly seen after
bilateral cleft lip repair, this problem is difficult to correct. Paired vermilion-
orbicularis flaps may be used to correct this deformity, or a cross lip flap
may be necessary [7,31].

Cleft palate
Anatomy
The normal palate consists of a bony anterior component and a posterior
soft-tissue component. Normal mobility of the soft palate is essential for
 CLEFT LIP AND PALATE 45

speech and swallowing function. This mobility depends on six paired mus-
cles that normally insert on the soft palate (Fig. 12A):
Levator veli palatini
Musculus uvulus
Superior constrictor
Palatopharyngeus
Palatoglossus
Tensor veli palatini
Of the six, the muscles that seem to have the greatest impact on velophar-
yngeal competence are the levator, the uvulus, and the superior constrictor.
The levator veli palatini pulls the soft palate, or velum, superiorly and pos-
teriorly, allowing it to appose the posterior pharyngeal wall. The musculus

Fig. 12. (A) Normal palate with insertions of levator veli palatini and musculus uvulus on mid-
line tensor aponeurosis. (From Senders CW, Sykes JM. Cleft palate. In: Smith JD, Bumsted
RM, editors. Pediatric facial plastic and reconstructive surgery. New York: Raven Press;
1993. p. 162; with permission.) (B) Cleft palate demonstrating insertion of levator on posterior
edge of hard palate and on edges of cleft. (From Senders CW, Sykes JM. Cleft palate. In: Smith
JD, Bumsted RM, editors. Pediatric facial plastic and reconstructive surgery. New York: Raven
Press; 1993. p. 163; with permission.)
46 AROSARENA

uvulus increases the bulk of the velum during its contraction, aiding in clo-
sure of the oropharyngeal-nasopharyngeal communication. The superior
constrictor is responsible for the sphincter function of the pharynx, moving
the pharyngeal walls medially during phonation and swallowing [4]. This
sphincter function can become critical in patients who have marginal velar
length or function who may compensate for their velar insufficiency with hy-
permobility of the superior constrictor. The palatopharyngeus may also play
a role in medialization of the pharyngeal wall and causes downward dis-
placement of the palate. The palatoglossus is also a palatal depressor that
is believed to be responsible for the production of nasal phonemes by allow-
ing controlled passage of air to the nasal chamber [4]. Both the palatophar-
yngeus and palatoglossus play important roles in swallowing. The tensor
moderates patency of the Eustachian tube.
In the cleft palate, the aponeurosis of the tensor veli palatini inserts onto
the bony edges of the cleft, rather than onto its normal insertion on the pos-
terior edge of the hard palate (Fig. 12B). Both the levator and tensor nor-
mally insert on the palatal aponeurosis. In the cleft palate the levator
sling is interrupted by insertion of the muscle onto the posterior edge of
the hard palate [1,4]. The function of the tensor is also compromised because
of its abnormal insertion, leading to inadequate ventilation of the middle ear
space. Because the aponeurotic attachment is more anterior in the cleft pal-
ate, the cleft palate is shorter than the normal palate [1,4].

Pathophysiology
The speech pathology associated with unrepaired cleft palate consists of
two components. The primary component of cleft speech pathology is directly
related to the oronasal communication, and is corrected surgically. It consists
of velopharyngeal dysfunction, hypernasality, and nasal air escape. Velo-
pharyngeal dysfunction refers to the inability of the soft palate to appose
the posterior pharyngeal wall and close the nasopharyngeal-oropharyngeal
communication during speech and swallowing. The sufficiency of the velum
depends on its length (adequacy) and muscular function (competence).
The secondary component of cleft speech pathology is a learned or com-
pensatory response in the unrepaired cleft palate or in the repaired palate
with an oronasal fistula or dysfunctional velum. This response consists of
glottal stops, pharyngeal fricatives, consonant substitutions, and decreased
consonant range that are carryovers from the child’s structural constraints
in early infancy and are difficult to correct with speech therapy. Such misar-
ticulations are best prevented by completing palatal repair by age 12 months
[1,4,32–34].
The goals of cleft palate therapy and repair are to:
Separate the oral and nasal cavities to prevent reflux of the food bolus
into the nares and promote nasal hygiene.
 CLEFT LIP AND PALATE 47

Normalize swallowing physiology by obliterating the oronasal communi-

cation and reconstructing a palate with a functional velum that has ad-
equate length.
Promote normal or near-normal speech by repair of the palatal cleft and
speech therapeutic intervention, while limiting the negative effects of
cleft palate repair on midfacial growth.
Perform mucoperiosteal repair of the cleft alveolus to correct anterior or-
onasal communication and facilitate possible bone growth across the
alveolar cleft, thus preventing tooth loss.

Timing of cleft palate repair

Despite the publication of numerous series on outcomes of cleft palate
repair, considerable controversy still exists as to the timing of cleft palate
and cleft alveolar repair. Many surgeons continue to advocate two-stage re-
pair of the cleft palate to limit the effects of hard palatal repair on maxillary
growth. Mucoperiosteal repair of the hard palate cleft is known to result in
subperiosteal scarring. Because midfacial growth occurs by bone deposition
by the hard palate periosteal osteocytes with concomitant osteoclastic bone
resorption along the nasal floor and in the maxillary sinuses, subperiosteal
scarring impairs midfacial growth. This midfacial growth impedance results
in a prognathic profile (Fig. 13). Advocates of two-stage palatal repair per-
form repair of the soft palate between 3 and 8 months of age while delaying

Fig. 13. (A, B) Teenaged patient with midfacial hypoplasia resulting from cleft palate repair in
infancy.
48 AROSARENA

hard palate repair until 15 months to 15 years of age. No significant differ-

ence in craniofacial morphology has been identified in children who had
palatal clefts repaired between age 8 months and 8 years [35–37]. If hard
palate closure is delayed until full facial growth has been attained, however,
this distortion is nearly eliminated at the expense of abnormal speech, which
may be difficult to correct [4,26,37,38]. Nevertheless, most craniofacial sur-
geons advocate complete repair of palatal clefts between age 9 and 12
months to prevent the detrimental effects of delayed repair on speech and
language development [2,4,7,34].

Palatoplasty techniques
The management of the CP has evolved from obturation in the 1700s; to
simple repairs of the cleft soft palate in the early 1800s; to two-flap complete
palatal repairs, such as von Langenbeck’s palatoplasty in the late 1800s
(Fig. 14); to repairs that lengthen the palate, such as the Veau-Wardill-
Kilner V-to-Y advancement technique in the 1930s (Fig. 15); to repairs
that not only close the palatal cleft and lengthen the palate but also correctly
align the palatal musculature (Table 3) [4]. Recreation of the levator sling
during CP repair has been associated with a higher probability of successful
speech development [7,39]. In their comparison study of Furlow palato-
plasty and von Langenbeck palatoplasty patients, Yu and colleagues [40]
found 98% of the patients who had undergone Furlow palatoplasty had ve-
lopharyngeal adequacy and excellent speech compared with 70% of the von
Langenbeck palatoplasty patients. The surgical technique selected for CP re-
pair depends on the extent of the cleft, and whether or not the cleft is uni-
lateral or bilateral.

Fig. 14. (A-C) Two-flap palatoplasty. (From Senders CW, Sykes JM. Cleft palate. In: Smith JD,
Bumsted RM, editors. Pediatric facial plastic and reconstructive surgery. New York: Raven
Press; 1993. p. 167; with permission.)
 CLEFT LIP AND PALATE 49

Fig. 15. Push-back palatoplasty. (From Senders CW, Sykes JM. Cleft palate. In: Smith JD,
Bumsted RM, editors. Pediatric facial plastic and reconstructive surgery. New York: Raven
Press; 1993. p. 165; with permission.)

Clefts limited to the soft palate

Although soft palate clefts can be simply closed by incising along the cleft
edges and reapproximating the nasopharyngeal mucosa and oral mucosa in
the midline, this technique does not easily allow for muscular repair, as the
muscle attaches on the posterior margin of the hard palate. Moreover, this
method does not increase palatal length, and the scar contracture of the
straight-line closure may even shorten the velum. For these reasons, the
author’s preferred method of soft palate cleft repair is the Furlow double
opposing Z-plasty, which concurrently lengthens the palate and correctly re-
aligns the levator veli palatini. Long-term studies have demonstrated im-
proved speech results and reduced rates of secondary surgery for
correction of velopharyngeal dysfunction when comparing the double op-
posing Z-plasty to other palatoplasty methods [7,40].

Surgical technique. After the induction of general anesthesia, the patient is

intubated with an oral RAE tube. The patient is positioned with his or her
neck extended, and a Dingman tongue gag is placed. The soft palate mucosa
is injected with 0.5% lidocaine with 1:200,000 epinephrine. It is important to
allow at least 10 minutes for the maximal vasoconstrictive effect of the epi-
nephrine to limit blood loss in the infant. During this time, tympanostomy
tube placement and/or intraoperative auditory brainstem response testing
may be performed.
The incisions for flap design described herein are for the right-handed
surgeon. The flap design may be reversed for the left-handed surgeon. The
mucosa is incised along the medial edge of the cleft bilaterally. On the right
side an incision is made along the posterior edge of the hard palate through
the mucosa and the attachments of the levator veli palatini to the hard pal-
ate (Fig. 16). An oral mucosal flap is developed on the right side, keeping the
50 AROSARENA

Table 3
Palatoplasty options
Technique Advantages Disadvantages
von Langenbeck’s Allows facile closure of Does not increase velar length
two-flap palatoplasty alveolar cleft Does not reorient levator sling
Longitudinal scar contracture
results in short palate
Denudes palatal bone
Veau-Wardill-Kilner Provides some increased Difficult to repair alveolar cleft
V-to-Y pushback palatal length with this technique
palatoplasty Does not reorient levator sling
Longitudinal scar contracture
can limit palatal lengthening
Denudes palatal bone
Furlow palatoplasty Significantly lengthens Increased operative time
velum compared to other methods
Recreates levator sling Technically challenging
Does not denude palatal Creates dead space between
bone oral and nasal mucosa that
fills with hematoma
Allows closure of alveolar Increased risk for palatal
cleft fistulae with wide clefts
Oral displacement of palatal
mucosa may negatively affect
speech
Combined two-flap/ Significantly lengthens Increased operative time
Furlow palatoplasty velum compared to other methods
Recreates levator sling Technically challenging
Allows closure of alveolar Denudes palatal bone
cleft

levator attached to the oral mucosa and separating it from the underlying
nasal mucosa. Care is taken not to incise the nasal mucosa at this time.
The oral mucosa is then elevated off the posterior edge of the hard palate
for a few millimeters with care being taken to preserve the greater palatine
neurovascular bundle. The soft tissue attachments to the pterygoid hamulus
are divided, and the tensor palatini is elevated out of the hamulus. Although
many surgeons describe fracturing of the hamulus to decrease tension on the
palatal closure, fracturing of the hamulus has been associated with damage
to the ascending palatine artery, which supplies the velar musculature [4,41].
The dissection then proceeds more laterally over the tensor into the space of
Ernst between the superior constrictor muscle and the pterygoids. This dis-
section results in increased medial rotation of the flap. Starting at the poster-
omedial edge of the cleft, the mucosa is elevated from the nasal surface of
the hard palate to allow mobilization of the nasal mucoperiosteum. The na-
sal mucosal flap on the right side is created by incising the nasal mucosa
from the uvula to the torus tubarius. The dissected area on the right side
can be packed with neurosurgical cottonoids soaked in 1:200,000 topical
epinephrine to limit blood loss during dissection of the flaps on the left side.
 CLEFT LIP AND PALATE 51

Fig. 16. (A-E) Double opposing Z-plasty soft palate cleft repair. (From Gage-White L. Furlow
palatoplasty: double opposing Z-plasty. Facial Plast Surg 1993;9:181–3; with permission.)

On the left side, the oral mucosal flap is developed by incising the mucosa
from the uvula to the maxillary tuberosity. The oral mucosa is then elevated
off of the levator palatini, leaving the levator attached to the nasal mucosa.
As with the right side, the oral mucosa is elevated off the posterior edge of
the hard palate, the soft tissue attachments to the hamulus are divided, the
tensor is elevated out of the hamulus, and the dissection is carried into the
space of Ernst. The oral mucosal flap is retracted, and the nasal mucosal flap
on the left side is created by detaching the nasal mucosa with the attached
levator from the posterior edge of the hard palate. A small cuff of nasal mu-
cosal tissue should be kept on the hard palate to facilitate closure.
At this point, closure of the palatoplasty begins by transposition of the
nasal mucosal Z-plasty flaps. The nasal mucosal flap from the right side is
sutured to the posterior hard palate nasal mucosa on the left side with ab-
sorbable suture. This closure begins adjacent to the left pterygoid hamulus
and proceeds from left to right. The left nasal mucosal flap is then trans-
posed and sutured to the mucosa at the base of the right pterygoid hamulus.
This mucosal flap is then sutured to the previously transposed right nasal
mucosal flap. A uvuloplasty is then performed before transposition of the
52 AROSARENA

oral mucosal flaps. The left oral mucosal flap is sutured to the mucosa of the
right maxillary tuberosity and then to the mucosa of the posterior edge of
the oral surface of the hard palate. The right oral mucosal flap is then trans-
posed and sutured to the left maxillary tuberosity and the previously trans-
posed left oral mucosal flap, completing the closure. The oral cavity,
oropharynx, nares, and nasopharynx are copiously irrigated and suctioned.
Arm splints are placed before the patient is awakened.

Unilateral complete cleft palate

Although some cleft surgeons suggest that the Furlow double opposing
Z-plasty technique be limited to clefts of the soft palate, the author has
used a modification of Furlow’s technique for closure of clefts involving
the hard palate [39]. Furlow’s technique for hard palate closure involves
closing the hard palate defect by oral displacement and tenting of the
hard palate mucoperiosteal flaps across the cleft, creating a dead space be-
tween the hard palate mucosal closure and the bone and nasal mucosal clo-
sure (Fig. 17) [4,39,42]. This dead space is believed to fill with hematoma,
which organizes into scar tissue [4,39]. Expansion of the hematoma is be-
lieved to contribute to fistula formation [4]. The risk for fistula formation
with this technique increases with clefts greater than 1 cm in diameter be-
cause of excessive tension on the midline area of closure [39]. The author
has experienced an unacceptable rate of fistulae at the junction of the
hard and soft palates with this technique and currently uses a combination
double opposing Z-plasty closure of the soft palate and two-flap closure of
the hard palate without fistula formation that is herein described.

Surgical technique. Both the soft and hard palate mucosae are injected with
0.5% lidocaine with 1:200,000 epinephrine. The mucosa is incised along the
medial edge of both the hard and soft palate cleft bilaterally. These incisions
extend to the premaxilla to repair the alveolar cleft, if present. Early

Fig. 17. Position of hard palate oral mucosa relative to nasal mucosa and underlying bone with
Furlow hard palate mucosal closure and two-flap hard palate mucosal closure. (Adapted from
Gage-White L. Furlow palatoplasty: double opposing Z-plasty. Facial Plast Surg 1993;9:181–3;
and Nguyen PN, Sullivan PK. Issues and controversies in the management of cleft palate. Clin
Plast Surg 1993;20:671–82; with permission.)
 CLEFT LIP AND PALATE 53

gingivoperiosteoplasty, in addition to promoting nasal hygiene and normal

speech development, has been shown to result in sufficient alveolar bone de-
velopment in 25% to 80% of patients to allow the eruption of primary and
permanent dentition [41,43]. The technique for repair of the unilateral com-
plete cleft palate is identical for that of repair of the soft palate cleft to the
point of transposition of the velar oral mucosal flaps. Before transposition
of the soft palate oral mucosal flaps, the repair of the bony palatal cleft is
completed.
The nasal closure is performed first by elevating the mucoperiosteum of
the nasal floor and vomer. If the cleft is wide, elevation of the nasal floor
mucoperiosteum may be carried laterally to the nasal sidewall to allow suf-
ficient medial advancement of the nasal floor mucosa to allow tensionless
closure. If the cleft is so wide that there is insufficient nasal floor mucoper-
iosteum, the vomerine mucoperiosteum is sutured to the ipsilateral palatal
shelf bone to close the nasal floor.
The hard palate mucosa is then incised from the maxillary tuberosities
and along the base of the alveolar bone to meet the incisions at the cleft
edges. Bilateral mucoperiosteal flaps are elevated, with care being taken to
preserve the greater palatine vessels. The velar oral mucosal flaps are trans-
posed, and the hard palate oral mucosal flaps are approximated in the mid-
line with interrupted vertical mattress sutures.
Gingivoperiosteoplasty is performed at this point if the cleft extends to
the alveolus. The mucosa on both the labial and lingual sides of the alveolus
is elevated on both sides of the cleft. Extensive mobilization of the mucosa
may be required to achieve tensionless closure. Recruitment of labial mu-
cosa for closure of the alveolar cleft is discouraged because this tethers
the lip. The alveolar mucosal flaps are approximated across the cleft. After
gingivoperiosteoplasty is completed, the hard palate oral mucosa may be
loosely tacked to the alveolar mucosa, or an obturator may be applied for
seven to ten days to facilitate adherence of the hard palate mucosa to the
palatal bone.

Bilateral complete cleft palate

Although the bilateral complete cleft palate is often wider than the uni-
lateral complete cleft palate, the principles of closure are the same. As de-
scribed for closure of the unilateral complete cleft palate, the double
opposing Z-plasty flaps are designed in the soft palate, and closure of the
soft palate nasal mucosa is completed. Bilateral vomer and nasal floor mu-
coperiosteal flaps are developed. Once the bilateral nasal floor closure is
completed, the hard palate oral mucosal flaps are developed. Although
the hard palate mucosal flaps may be mobilized completely and tethered
only on the greater palatine neurovascular bundles, significant tension
may still exist in the midline closure area at the hard palate–soft palate junc-
tion. This is because the double opposing Z-plasty closure of the soft palate
precludes large back-cuts in the soft palate oral mucosa to preserve the
54 AROSARENA

vascularity of the soft palate flaps. In such situations, the author has found
it useful to reinforce the palatal mucosal closure by suturing a strip of acel-
lular human dermal matrix (Alloderm Lifecell, Branchburg, New Jersey) to
the palatal mucosa in the midline before transposition of the soft palate oral
mucosal flaps and closure of the hard palate mucosal flaps. This technique
has been found useful in the prevention of fistulae when the palatal cleft is
wide and the mucosa of the palatal shelves is limited.

Postoperative care
Patients are observed postoperatively with continuous pulse oximetry for
the first 24 to 48 postoperative hours because of the risk for upper airway
edema and hemorrhage. A mist tent may be used. Intravenous hydration
is maintained and intravenous pain medications, supplemented with acet-
aminophen suppositories, are administered until the child demonstrates ad-
equate feeding behavior. Arm splints are placed and maintained for the first
two postoperative weeks to prevent patient disruption of the palatal repair.
Clear liquids are introduced by syringe or cup on the first postoperative day,
and nipple feeding is discouraged. A liquid diet is maintained for 1 to 2
weeks. Patients are discharged when oral alimentation is adequate [4].

Complications and their management

Respiratory compromise. Respiratory compromise in the immediate postop-
erative period can be life threatening and is related to upper airway edema
or excessive sedation. This complication is best prevented by close monitor-
ing of patients in a pediatric intensive care unit for the first 24 to 48 hours,
and may require intubation. The application of a mist tent postoperatively
may decrease upper airway obstruction.

Hemorrhage. Blood loss during cleft palate repair can be significant. This
problem can be controlled by injecting the palatine mucosa with an epineph-
rine-containing local anesthetic before surgery. In addition, neurosurgical
patties soaked in 1:200,000 topical epinephrine should be applied to open
areas during the procedure. Cauterization should be used conservatively
to preserve viability of the mucosal flaps.

Oronasal fistulae. The incidence of palatal fistula formation is reported to

be between 3.4% and 29% [4,44]. The success of fistula repair is limited
[4]. Previously closed oronasal fistulae tend to reopen during palatal expan-
sion [45]. Fistulae are usually the result of poor tissue quality and excess
wound tension. This emphasizes the importance of gentle tissue handling
with complete flap mobilization. In addition, the use of postoperative
arm splints decreases the risk for wound dehiscence caused by patient
manipulation [4].
 CLEFT LIP AND PALATE 55

Long palate. Excessive palatal length has been reported with the double op-
posing Z-plasty technique, leading to airway obstruction in neurologically
compromised children. The surgeon may consider straight-line repairs in
such clinical situations [39].

Secondary correction of cleft-related problems

Velopharyngeal dysfunction
The incidence of velopharyngeal dysfunction varies from study to study
and has been reported to be between 20% and 83%. It is believed to depend
on the type of palatal repair. Other contributing factors are the extent and
width of the cleft, which impact the amount of tissue available for repair; the
extent of undermining necessary, which in turn affects scarring; and the
length of the native velum. Velopharyngeal dysfunction manifests as in-
creased nasality, nasal emission, weakening of pressure consonants, and na-
sal reflux [33]. Speech therapy is initiated in the postoperative period and
continued until the dysfunction and compensatory misarticulations are cor-
rected. Persistent velopharyngeal dysfunction is best addressed surgically
with a pharyngeal flap procedure between the ages of 5 and 6 years, but at-
tendant risks of this procedure include the development of hyponasal reso-
nance and sleep apnea. The placement of a palatal prosthesis is another
treatment option, but is often poorly tolerated.

Alveolar bone grafting

Indications for alveolar bone grafting include stabilization of the max-
illary arch, provision of bony support to the teeth neighboring the cleft,
closure of oronasal fistulae (if present), elevation of the cleft nasal base,
and facilitation of orthodontic treatment or placement of titanium im-
plants. Secondary alveolar bone grafting is generally preferred to primary
grafting at the time of cleft lip or palate repair because of the adverse ef-
fects of primary grafting on facial growth [46,47]. Secondary alveolar bone
grafting is ideally performed before eruption of the permanent canine
tooth and, if possible, before eruption of the lateral incisor. Iliac crest can-
cellous bone is the most widely used donor site, but other sites include the
tibial shaft, mandibular symphysis, rib, and split calvarial bone [48]. Yen
and colleagues [49] reported a novel approach to closure of a large alve-
olar cleft that was too large for bone grafting because of soft tissue insuf-
ficiency. Orthodontic springs, elastics, and wires were used to transport
a posterior segment of bone containing two premolars in a patient with
bilateral cleft lip and palate. The authors report that other craniofacial
teams are studying distraction methods for closure of large alveolar
clefts.
56 AROSARENA

Midfacial hypoplasia
The timing of CP repair at age 9 to 12 months optimizes speech develop-
ment; however, it is recognized that palatal repairs that denude bone heal
with scar contracture resulting in midfacial growth distortion. This midfa-
cial growth retardation results in a prognathic profile (see Fig. 13). As im-
portant as the aesthetic consequences of midfacial hypoplasia are its
effects on speech. Class III malocclusion can affect production of all the
tongue tip and bilabial consonants. Anterior open bite and lateral open
bite may result in a lisp. Moreover, a lowered palatal vault, which can occur
with some types of hard palate cleft repair, can result in restricted tongue
mobility and distortion of the midline palatal groove necessary for produc-
tion of some sibilant consonants [32,33].
Correction of maxillary hypoplasia and retrusion usually requires trans-
verse maxillary expansion during the period of mixed dentition to correct
lingual crossbite deformities and orthognathic surgical correction during
the second decade of life. Anterior-inferior maxillary advancement with Le-
Fort I osteotomies and rigid fixation is plagued by relapse. The use of post-
operative protraction facemasks has been advocated to prevent surgical
relapse in cleft lip and palate patients following LeFort I osteotomy [50].
Maxillary distraction osteogenesis is associated with a reduced relapse
rate because of its ability to combine new bone deposition with bone remod-
eling and maxillary advancement. Skeletal-anchored distraction devices are
believed to be superior to tooth-borne devices in that the tooth-anchored de-
vices result in greater dental than skeletal movement [51,52].

Secondary septorhinoplasty
Correction of the unilateral cleft nasal deformity remains one of the most
challenging aspects of cleft surgical care as evidenced by the number of tech-
niques advocated for this problem. Dutton and Bumsted [53] use a three-
tiered approach to correction of the cleft nasal deformity. After performing
primary rhinoplasty at the time of lip repair, intermediate rhinoplasty is per-
formed after alveolar bone grafting and closure of the nasolabial fistula. The
goal of the intermediate rhinoplasty is to correct any residual lower cartilag-
inous deformity. An open rhinoplasty approach is used through V-Y ad-
vancement flaps from the upper lip to lengthen the columella (a Bardach
modification is used for the unilateral cleft lip nasal deformity). A Y-V
alar advancement may also be used to narrow the alar base, with fixation
of the base to the nasal spine with permanent suture. Delayed rhinoplasty
is then performed after puberty to correct any bony dorsal deformity and
various causes of nasal obstruction.
In a retrospective review from India, Ahuja [54] described radical correc-
tion of the nasal deformity in unilateral cleft lip patients who presented in
the second and third decade of life. None of these patients had undergone
 CLEFT LIP AND PALATE 57

primary nasal correction, orthodontic management, or alveolar bone graft-

ing before presentation. An open rhinoplasty approach was used. All of the
patients were treated with columellar lengthening on the cleft side, submu-
cous resection of the nasal septum with repositioning of the caudal strut, na-
sal dorsal augmentation, and bone grafting along the pyriform margin,
nasal floor, and alveolus. The nasal tip deformity was corrected with inter-
domal suturing, and the alar cartilages were fixed to the septum and upper
lateral cartilages with permanent sutures. Alar base repositioning was per-
formed with a V-Y advancement if excessive flaring of the ala was present.
Osteotomies were avoided. Ahuja reported good aesthetic results, while ac-
knowledging some persistent alar base depression, inadequate positioning of
the caudal septum, lack of tip definition, nostril asymmetry, and inadequate
dorsal augmentation. Although no improvement in occlusion can be ob-
tained with this technique, it is an acceptable corrective measure for patients
who present late for correction of cleft nasal deformities and cannot or will
not tolerate orthognathic and orthodontic procedures [54].

Future considerations
Care of the patient who has orofacial clefting may change considerably in
the not-too-distant future because of advances in the fields of tissue engi-
neering, genetics, and fetal surgery. Ongoing work in molecular develop-
mental biology will increase our understanding of the interactions of the
biomolecules involved in craniofacial development and tissue healing. This
insight will guide the application of tissue engineering techniques to not
only correct the maldevelopment associated with clefting in utero but to fa-
cilitate care of the pediatric and adult patient who has cleft lip and palate.
For example, the use of bone morphogenetic protein-containing bioresorb-
able implants has been suggested for repair of alveolar clefting to prevent
the morbidity associated with bone graft harvesting [55]. Similarly, as
knowledge of the human genome progresses and the various genes involved
in orofacial clefting are identified, in utero gene therapy may become feasi-
ble as methods of targeted gene delivery are refined. Moreover, a better
comprehension of the effects of environmental factors on gene expression
may lead to improvements in prenatal care that can significantly reduce
the incidence of clefting.
Fetal surgery is an exciting and promising prospect for children who have
various craniofacial anomalies. The advantages of fetal surgery include scar-
less wound healing if performed at midgestation and normalization of facial
growth. Fetal cleft lip repair has been demonstrated in various animal
models and CP repair has also been demonstrated in utero using a goat
model [55–60]. Fetal surgery poses significant risks to the mother and fetus,
however, including the risk for premature labor, even with the advent of en-
doscopic techniques. These risks make in utero cleft repair ethically unjusti-
fiable at this time [55].
58 AROSARENA

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