Cleft L ip and P alate

Mitchell L. Worley, MDa, Krishna G. Patel, MD, PhD

a,
*,
Lauren A. Kilpatrick, MDb

KEYWORDS
 Cleft lip  Cleft palate  Craniofacial malformations  Nasoalveolar molding

KEY POINTS
 Orofacial clefts are common congenital malformations that can occur in isolation or be
associated with additional abnormalities or genetic syndromes.
 Lip taping and nasoalveolar molding are low-risk interventions that can preoperatively
modify cleft defects to enhance surgical outcomes.
 Multiple techniques are available for repair of orofacial clefts and choice of technique
depends on cleft extent and surgeon preference.
 Early multidisciplinary evaluation and long-term follow-up of patients with orofacial clefting
are essential to achieving optimal clinical outcomes.

INTRODUCTION

Cleft lip and palate (CLP) are common congenital malformations affecting the head
and neck. The incidence of cleft lip with or without cleft palate (CL/P) is w1:1000,
and the incidence of isolated cleft palate (CPO) is w1:2500.1 The incidence of CPO
is uniform across different ethnicities, whereas CL/P varies with race (incidence in
Asian > Caucasian > African).2 Boys are more frequently affected with CL/P (2:1),
with an inverse ratio seen in CPO (male/female, 0.5:1).3,4

Causes of Cleft Lip and Palate

Although CL/P or CPO can occur in isolation (61.6%), they are frequently associated
with additional congenital anomalies or genetic syndromes (38.4%).5 Compared with
infants with CL/P, infants with CPO are more likely to have related congenital abnor-
malities (26.5% vs 52.5%, respectively).5 Common associated anomalies seen in chil-
dren with CPO include congenital heart defects (31.1%), hydrocephalus (11.2%), and
urinary tract defects (9.7%).4

Disclosure: No conflicts of interest to disclose.

a
Department of Otolaryngology–Head and Neck Surgery, Medical University of South Carolina,
135 Rutledge Avenue, MSC 550, Charleston, SC 29425, USA; b Department of Otolaryngology–
Head and Neck Surgery, University of North Carolina, 170 Manning Drive, Campus Box #7070,
Chapel Hill, NC 27599, USA
* Corresponding author.
E-mail address: [email protected]

Clin Perinatol 45 (2018) 661–678

https://doi.org/10.1016/j.clp.2018.07.006 perinatology.theclinics.com
0095-5108/18/ª 2018 Elsevier Inc. All rights reserved.
662 Worley et al

There is a strong genetic component to the development of orofacial clefts. More

than 200 genetic syndromes have been associated with cleft lip (CL) and 400 syn-
dromes with cleft palate (CP).6 Frequently discussed genetic syndromes with genes
associated with orofacial clefts include CHARGE syndrome (CHD7), velocardiofacial
syndrome (TBX1, COMT), and Apert syndrome (FGFR2).7 The genetic component to
orofacial clefting is also demonstrated in the increased recurrence rate among
affected families. Each child of an affected parent with a cleft has a 3% risk of having
an orofacial cleft. If one sibling is affected, the risk of a cleft in subsequent children is
5%. If both a sibling and parent are affected, there is a 14% risk of cleft formation in
subsequent children.8
Multiple environmental factors have also been linked with CLP. Risk factors include
smoking, pregestational and gestational diabetes, alcohol abuse, and certain anticon-
vulsants.9–11 Specific nutritional deficiencies that may contribute to the risk of clefting
include inadequate folate and vitamins B6 and B12.3,12 Providing further evidence for
the role of maternal nutrition in lip and palate development, a systematic review found
that maternal multivitamin use resulted in a 25% decrease in the odds of a child being
born with a cleft.13

Embryology of Cleft Lip and Palate

Lip embryologic development begins at gestational week 4 with the appearance of the
paired maxillary prominences and the unpaired frontonasal prominence (Fig. 1A–D). In
week 5, the medial and lateral nasal processes develop from the invagination of the
nasal placodes. The paired maxillary prominences extend medially in weeks 6 to 7
and meet the nasal processes to form the upper lip.
The primary palate develops from the fusion of the paired medial nasal prominences
in weeks 6 to 7 (Fig. 1E–H). This fusion forms the intermaxillary segment, which will
contain the central 4 incisors and hard palate anterior to the incisive foramen. At this
time, palatine processes or shelves also extend medially from the paired maxillary

Fig. 1. (A–D) Upper lip development sequence. (E–H) Soft and hard palate development
(Illustration by Emma Vought).
 Cleft Lip and Palate 663

prominences. Fusion of the palatal shelves to form the secondary palate begins in week
9 (fusion begins anteriorly at the incisive foramen and extends posteriorly to the uvula).

Classification of Cleft Lip and Palate

CLs can be classified as microform, incomplete, or complete. A microform CL de-
scribes a notch or groove in the soft tissues of the lip (Fig. 2A). All of the lip tissues
are present, but there is a notch at the vermilion-cutaneous junction. In contrast,
incomplete CLs involve dehiscence of the orbicularis oris and can be variable in their
involvement of the skin (Fig. 2B). A Simonart band refers to a thin band of soft tissue
that spans the superior aspect of an incomplete CL at the nasal sill. Complete CLs
extend through the length of the lip and into the nasal sill, leading to abnormal insertion
of the orbicularis oris onto the ala and columella (Fig. 2C). In addition, in bilateral CL

Fig. 2. (A) Microform right CL. (B) Incomplete left CL. (C) Complete right CL. (D) Bilateral
complete CL.
664 Worley et al

there is anterior displacement of the intermaxillary segment with absence of the orbi-
cularis oris within the intermaxillary segment (Fig. 2D).
CPs can likewise be classified by the extent of their anatomic involvement. Submucous
CPs are characterized by an underlying dehiscence of the palatal musculature, whereas
the overlying mucosa is intact. Because they do not have an associated mucosal defect,
detection of submucous CPs can be challenging. Physical examination findings associ-
ated with a submucous CP include midline notching of the hard palate, bifid uvula, and a
zona pellucida (blue line in the midline of the soft palate representing the lack of muscu-
lature and increased transparency). A cleft of the secondary palate involves a defect
extending posteriorly from the incisive foramen through the soft palate to the uvula. In
contrast, a cleft of the primary palate involves the palate anterior to the incisive foramen
extending to the alveolar arch. A complete CP involves both the primary and secondary
palates. Examples of the different types of CP are shown in Fig. 3. Note that primary and
secondary palate describe the palate by its embryologic origins. In contrast, the terms
hard and soft palate refer to the anatomic findings represented by the anterior bony pal-
ate and the posterior soft tissue/musculature palate, respectively.

Prenatal Diagnosis of Cleft Lip and Palate

Birthing and raising a child with orofacial clefting can be a source of psychological
distress for mothers and families.14,15 Antenatal diagnosis of an orofacial cleft allows
for prenatal counseling and can assist families in being prepared for the care of their

Fig. 3. (A) Submucous CP. (B) Incomplete CP. (C) Unilateral complete CP. (D) Bilateral
complete CP.
 Cleft Lip and Palate 665

future child.16,17 Prenatal diagnosis also allows families to meet the craniofacial team
members before birth and can help facilitate the recommended early postpartum eval-
uation of affected infants.
In the United States, anatomic ultrasonography studies are frequently obtained be-
tween 18 and 20 weeks’ gestation. The accuracy of two-dimensional (2D) ultrasonog-
raphy in detecting an orofacial cleft among low-risk patients is variable, with detection
rates of 0% to 70% reported in a systematic review.18 Infants with CPO are less likely
to be detected than those with CL/P.18 The prenatal ultrasonography detection rate is
likely dependent on multiple factors, including sonographer experience, gestational
age, and whether the laboratory routinely performs imaging of the face. Compared
with 2D studies, three-dimensional (3D) ultrasonography has an increased diagnostic
accuracy in general, and has shown an ability to detect a CP when a CL has been pre-
viously detected on 2D ultrasonography (up to 100% sensitivity).18,19

Early Multidisciplinary Evaluation

The American Cleft Palate-Craniofacial Association emphasizes the importance of
multidisciplinary treatment of these patients within the first few days of life.20 Given
the frequency of concomitant abnormalities, an early dysmorphology evaluation is
essential. If there are additional abnormalities, a comprehensive genetics evaluation
should be considered. Once an infant is connected to a craniofacial team, a coordi-
nator can also assist families in planning their follow-up care after discharge.
Frequently, patients with CLP require the care of multiple medical specialties
(Table 1) and should be followed in a multidisciplinary clinic until early adulthood.

Feeding Evaluation
Early feeding difficulties are common among infants with CLP. Issues with nutritional
intake can stem from difficulty creating a seal in patients with CL, or an inability to
generate the negative pressure or suction to feed in patients with CP.21 Alterations in

Table 1
Basic cleft care

Age Medical Treatments Surgery

Prenatal to birth  Genetic counseling —
 SLP consultation for feeding
0–5 mo  SLP for feeding and growth  CL repair
 Monitor hearing  Ear tubes (if COM)
 NAM (if indicated)
9–12 mo —  Palatoplasty
 Ear tubes (if COM)
1–4 y  Introduction to pediatric dentist —
 Assess language development
4–6 y  Assess for VPD  Corrective speech surgery
 Lip revision if needed
 Minor nasal surgery if needed
6–12 y  Orthodontics  Alveolar bone grafting
 Assess school/psychosocial adjustment
12–21 y  Orthodontics  Orthognathic surgery
 Definitive rhinoplasty

Abbreviations: COM, chronic otitis media; NAM, nasoalveolar molding; SLP, speech-language
pathology; VPD, velopharyngeal dysfunction.
666 Worley et al

these normal feeding patterns place children with orofacial clefts at increased risk for
poor weight gain and dehydration.22,23 Mothers with infants struggling with feeding
are also susceptible to depression, which can further complicate their children’s care.23
Given the prevalence of feeding difficulties in the cleft population, affected newborns
should be seen by a speech-language pathologist, who can perform an evaluation and
provide counseling. Typically, patients with CL are able to breastfeed, whereas those
with a CP are usually unsuccessful. The authors encourage families of children with
CPs to bottle feed for nutrition, but then they may breastfeed briefly as desired for mother
and infant bonding. There are multiple cleft-specific bottles, including the Haberman
Feeder, Pigeon Feeder, Mead Johnson Cleft Lip/Palate Nurser, and the Dr. Brown’s Spe-
cialty Feeding System. These bottles can be broadly classified into 2 types: assisted-
delivery (squeeze) versus rigid bottles. Assisted-delivery bottles (Haberman and Mead
Johnson) are compressible and allow parents to squeeze the reservoir and increase
the milk/formula flow. In contrast, rigid bottles (Pigeon and Dr. Brown’s) allow the infant
to release the flow of milk by compressing the specialized nipple. A Cochrane Review
evaluating squeezable versus rigid bottle use among patients with clefts found no evi-
dence for a difference in growth outcome based on bottle choice.24 As a result, families
should be counseled to use whichever bottle works best for their individual child.

Early Cleft Interventions

Both lip taping and nasoalveolar molding (NAM) are frequently used during the
neonatal period in an attempt to reduce the severity of a cleft deformity. Suggested
benefits of these interventions include decreased cleft width, improved nasal symme-
try, and improved psychological outcomes for caregivers.25–28 However, controversy
remains regarding the efficacy of individual techniques and there is a wide variation in
clinical practice patterns.29,30
Preoperative lip taping is commonly used in children with CLs during the neonatal
period.28,31 At our institution we routinely perform lip taping in infants with complete
CL. Beginning within 1 week of life, the tape is applied across the cleft while squeezing
the lip together (Fig. 4). Before discharge, families are instructed on how to apply the
tape daily and are scheduled for follow-up in the cleft surgeon’s clinic. The most
common complication of lip taping is skin irritation. If this is encountered, a dressing
can be placed to protect the skin before taping.
NAM is frequently used at our institution in patients with wide unilateral or bilateral
CL/P. Some proposed benefits of NAM include improved nasal symmetry in unilat-
eral CL deformities, increasing columellar length in bilateral CL deformities, and
improved alignment of the alveolar arches.26,32–35 However, NAM involves a commit-
ment from the family. An early family meeting should review the time commitment
and the need for frequent initial clinic visits for adjustments to the prosthetic.
Once the family and craniofacial team (cleft surgeon, prosthodontist) agree to pro-
ceed with NAM, a maxillary impression must be taken (typically within the first weeks
of life) (Fig. 5). The impression can be taken in the clinic or operating room depending
on the comfort level of the prosthodontist. The family is then seen by the prosthodon-
tist within a week for the initial fitting. The parents are instructed on how to apply the
NAM and it is worn 24 hours a day. Children are seen every 1 to 2 weeks in clinic for
up to 6 months to assess prosthetic fit and treatment effects. The most common re-
ported complications of NAM include skin irritation, poor compliance, and failure of
the device to remain in place.36 The last 2 complications emphasize the importance
of patient and family selection because a family not committed to the process is un-
likely to have satisfactory compliance or outcome. If NAM is not a possibility, the
family is encouraged to use lip taping.
 Cleft Lip and Palate 667

Fig. 4. Patient with bilateral CLP with lip taping in place to apply pressure to reposition the
intermaxillary segment posteriorly.

SURGICAL MANAGEMENT
Cleft Lip Preoperative Planning
Lip adhesion may be used for patients with a wide unilateral or bilateral CL who are
unable to complete either lip taping or NAM. This surgery is typically performed at
age 1 month in preparation for a second-stage lip repair.
Definitive lip repair commonly occurs at age 3 months (or 10 weeks) to avoid airway
difficulty associated with obligate nasal breathing in early infancy and postanesthetic
apnea. Other factors involved in timing of surgery include weight (ideally 4.5 kg
[10lb]) and hemoglobin level (ideally 10 g/dL), known as the rule of 10s and first
described by Wilhelmsen and Musgrave.37,38 Adequate completion of NAM or taping

Fig. 5. (A) NAM impression and prosthesis. (B) Patient with unilateral CLP with NAM in place.
(Courtesy of Betsy K. Davis, DMD, Medical University of South Carolina, Charleston, SC.)
668 Worley et al

may defer surgery. Caregivers should be counseled preoperatively regarding risks of

surgery to include dehiscence, hypertrophic scar, poor cosmesis, and presence of
nasolabial fistula. Technique of feeding (ie, bottle vs syringe) and need for hospitaliza-
tion after surgery should be discussed as well. Wound care after surgery may involve
gentle cleaning and application of ointment unless surgical glue is applied. If perma-
nent cutaneous sutures are used, a second procedure to remove the sutures will be
necessary.

Surgical Procedure
The investigators use either the Millard or Fisher technique for repair of the unilateral
CL and the Millard technique for repair of the bilateral CL.39–41 The Millard technique is
based on rotation-advancement principles, whereas the Fisher technique is described
as an anatomic subunit approach. Other rotation-advancement techniques by Moh-
ler42 and Noordhoff43 are also frequently used by surgeons in the United States.
The specific surgical technique is commonly used by a surgeon for repair of both
incomplete and complete CL.30 The goals of both unilateral and bilateral CL repair
include (1) oral competence via a complete orbicularis oris, (2) symmetry, and (3)
cosmesis. A tip rhinoplasty may be performed during this surgery to improve the con-
tour, shape, position, and width of the affected ala and nasal tip.
Lip repair or cheiloplasty is performed under general endotracheal anesthesia, typi-
cally with the use of an oral right angle endotracheal tube to position the endotracheal
tube inferiorly and out of the surgical field. Local anesthesia is used per surgeon pref-
erence. For the Millard repair, critical markings include the high and low points of the
Cupid’s bow, columellar base, alar bases, and choice of the appropriate high point of
Cupid’s bow on the lateral lip of the cleft side.41 The basic principle of the Millard repair
is to rotate the noncleft side inferiorly and advance the lateral lip of the cleft side. The C
flap originates from the medial non-CL and may be incorporated into the columella for
lengthening or the nasal sill. Mucosal M and L flaps can be rotated to bridge the alve-
olus and minimize the resulting fistula. The aforementioned markings, flaps, and
closure for the Millard repair are shown in Fig. 6. For the Fisher repair, 25 critical mark-
ings are used.39 A triangle is incorporated into the cutaneous skin at the cleft side phil-
tral column from the lateral lip using predetermined calculations related to the philtral
column height of the cleft and noncleft sides. The Rose-Thompson effect adds 1 mm
of lengthening. A second triangle is incorporated from the lateral lip vermilion as well.
Mucosal flaps again can be rotated. In both techniques, the orbicularis oris is recon-
structed. The Fisher repair markings, flaps, and closure are seen in Fig. 7.
The bilateral CL repair is distinct from the unilateral CL repair. The central prolabium
of the bilateral cleft defect does not contain orbicularis oris muscle and thus surgery
requires additional mobilization of the muscle across the premaxilla for adequate mus-
cle reapproximation. The columella is also typically deficient in the bilateral CL. Mark-
ings for the bilateral CL repair are similar to those for the unilateral CL repair by Millard.
The distinct difference is the advancement of the lateral lip vermilion to the midline to
approximate the central low point of Cupid’s bow inferiorly. Markings, flaps, and
closure for the bilateral CL repair are shown in Fig. 8.

Complications
Complications of unilateral CL repair include deficient vermilion or a whistle deformity,
under-rotation of the high point on the cleft side, muscular dehiscence, and nasal
asymmetry. Secondary surgery for the lip is typically considered at approximately
age 5 years or older.
 Cleft Lip and Palate 669

Fig. 6. Unilateral CL repair with Millard technique. (A) Surgical markings. (B) Flaps. (C) Post-
operative result.

Cleft Palate Preoperative Planning

Palatoplasty typically occurs between 9 and 12 months of age. Dorf and Curtin44
showed significantly improved speech outcomes if soft palate repair occurred before
age 12 months. Two-stage palate repair with early soft palate closure and delayed
hard palate closure is performed as well. Considerations for timing of surgery include
the surgery’s impact on the oropharyngeal airway and potential disruption of maxillary
growth caused by hard palate dissection.
Risks of surgery should be discussed with caregivers before surgery to include fis-
tula, velopharyngeal dysfunction (VPD), maxillary growth disturbance, and sleep-
disordered breathing.45 Short-term complications include hemorrhage, infection,
tongue edema, and respiratory difficulty. Need for postoperative hospitalization and
feeding plan (ie, bottle vs open-flow cup) should also be included in the preoperative
discussion. The goals of palatoplasty include restoration of an intact levator palatini
with palatal lengthening; ideally, surgery should have a low incidence of palatal fistula,
VPD, and maxillary growth disturbance.

Surgical Procedure
Surgical technique may be selected based on type of palatal involvement (ie, only soft
palate vs incomplete secondary palate vs complete secondary palate) and width of
the cleft between palatal shelves. The hard palate is repaired with axial mucoperios-
teal flaps based on the greater palatine arteries.46 Vomer flaps may be included to
670 Worley et al

Fig. 7. Unilateral CL repair with Fisher technique. (A) Surgical markings. (B) Flaps. (C) Post-
operative result.

aid in nasal closure. Relaxing incisions at the lateral aspect of the hard palate are used
for mobility; the lateral donor sites heal by secondary intention with remucosalization.
The soft palate is repaired with repositioning of the levator muscle into an intact sling
by varying techniques, including a straight-line repair, intravelar veloplasty, or double
opposing Z-plasty.46–48 Regardless of technique, a multilayer closure is used to mini-
mize risk of fistula formation. Markings, flaps, and closure of CPs are shown in Fig. 9.
Complications
Long-term complications of palatoplasty include palatal fistula, obstructive sleep ap-
nea (OSA), and VPD. Fistula formation may lead to nasal regurgitation of oral intake
and hypernasality. Techniques for palatal fistula closure are beyond the scope of
this article but can be complex. OSA has a higher incidence in patients with clefting;
screening for symptoms of sleep-disordered breathing should regularly occur in this
patient population.49 A polysomnogram is recommended before additional surgical
intervention given their high-risk status.50 VPD is discussed further later in this article.

COMMON COMORBIDITIES
Hearing and Middle Ear Disease
After the introduction of the newborn hearing screen (NBHS) in the United States,
more than 95% of newborn infants are screened in the perinatal period for hearing
 Cleft Lip and Palate 671

Fig. 8. Bilateral CL repair. (A) Surgical markings. (B) Flaps. A natal tooth is shown in the pre-
maxilla. (C) Postoperative result.

loss with the goal of early identification and intervention.51 Infants with a cleft anomaly,
especially CP, are at higher risk for referring the NBHS.52 Although this finding is often
attributed to middle ear effusion because of the high prevalence of middle ear disease
in children with CP, the incidences of sensorineural hearing loss, conductive hearing
loss (CHL), and mixed hearing loss are also higher in children with clefting.52–54
Eustachian tube dysfunction (ETD), including chronic middle ear effusion, is found in
at least 95% of patients with a cleft, specifically CP. The cause of chronic middle ear
disease is attributed to anatomic disruption of the tensor veli palatini and change in
eustachian tube compliance.55,56 CHL is found in most patients with a CP because
of middle ear findings.57 Interventions to improve hearing include placement of tympa-
nostomy tubes, palatoplasty, and/or hearing amplification.58,59 Most children with a
CP have improvement in ETD, middle ear disorders, and also hearing by approxi-
mately age 6 years.60 However, there remains a significantly increased incidence of
hearing loss in patients with a CP in subsequent years.58,61
Tympanostomy tube placement may occur in coordination with CLP repair or addi-
tional surgeries to minimize anesthesia exposure, as seen in Table 1. Patients with a
CP undergo a mean of 3 to 4 tympanostomy tube placement procedures before
672 Worley et al

Fig. 9. Palate repair. (A) Straight line palatoplasty surgical markings. (B) Straight line pala-
toplasty outcome. (C) Double opposing Z-plasty surgical markings. (D) Double opposing
Z-plasty outcome. (E) Mucoperiosteal flaps for hard palate closure; white arrow indicates
greater palatine artery vascular pedicle.

improvement in chronic middle ear disease.53 A greater risk of persistent CHL exists
among patients with multiple sets of tympanostomy tubes or cholesteatoma.53,62
Because of a higher incidence of cholesteatoma in at-risk patients with persistent
middle ear dysfunction, otologic follow-up into adulthood is recommended.53

Velopharyngeal Dysfunction
VPD is inadequate closure of the velopharynx during speech such that air escape oc-
curs through the nose during oral consonant production with hypernasal resonance.
Nasal phonemes (/m/,/n/,/ng/) occur naturally with nasal resonance and an open
 Cleft Lip and Palate 673

velopharynx; all other phonemes require closure of the velopharyngeal complex such
that the palate elevates to contact the posterior pharyngeal wall. VPD can be subca-
tegorized as velopharyngeal mislearning (often caused by articulation errors and
incorrect placement or closure), velopharyngeal incompetency (functional deficit), or
velopharyngeal insufficiency (structural or anatomic deficit).
VPD occurs in an estimated 20% of patients who previously underwent CP repair,
although the incidence of VPD varies widely in the literature.63 Risk factors for VPD
include history of CP, especially with delayed CP repair; submucous CP; change in
velopharyngeal anatomy (ie, adenoidectomy or maxillary advancement); syndromic
association including trisomy 21, 22q11.2 deletion syndrome, Kabuki syndrome; or
acquired pharyngeal dysfunction or hypotonia (ie, stroke, head injury, head and
neck cancer with anatomic defect, or radiation exposure). The double opposing
Z-plasty or Furlow palatoplasty has been shown to have improved speech outcomes
such that need for a secondary operation because of VPD is lower in children who un-
derwent Furlow repair compared with straight-line closure.64
Screening for VPD should begin at an early age (approximately 18 months) for chil-
dren with a CP and continue at regular intervals into early adulthood. A skilled speech-
language pathologist performs a perceptual assessment and/or objective testing us-
ing nasometry or pressure flow testing. Adequate speech assessment requires patient
maturity and may not be thorough until age 4 or 5 years. Additional testing for VPD in-
cludes nasal endoscopy and/or video fluoroscopy to better determine the pattern of
velopharyngeal closure and assist with surgical planning. Surgery may ideally be
tailored to closure pattern: coronal, sagittal, circular, or circular with Passavant ridge.
Speech therapy may be the primary treatment or an adjunctive treatment preoper-
atively and/or postoperatively. Therapy is the treatment of choice for velopharyngeal
mislearning. Often speech therapy is needed to correct compensatory articulation er-
rors even when surgery is required for an anatomic or structural deficit.
Patients who are not surgical candidates because of airway concerns, prior failed sur-
geries, existing comorbidities, position of internal carotid arteries, and so forth may be
candidates for a prosthesis. A partnership between speech-language pathology and
maxillofacial prosthodontics is advantageous to fashion an obturator or palatal lift for
these patients.65 Adequate maxillary dentition is important for prosthesis use.
Surgical interventions for VPD are ideally tailored to closure pattern based on
endoscopy or fluoroscopy findings and include (1) posterior pharyngeal wall augmen-
tation, (2) palatal lengthening, and/or (3) alteration of the velopharynx. Small central
gaps may be improved with injection pharyngoplasty; various materials have been
described for this procedure.66 If there is evidence of levator muscular dehiscence,
double opposing Z-plasty can be used to reorient the levator sling, positioning it pos-
teriorly with added palatal length.67 This secondary palatal technique may also be
used in combination with the sphincter pharyngoplasty. The velopharynx may be
altered via (1) sphincter pharyngoplasty or (2) posterior pharyngeal flap. Ideal candi-
dates for sphincter pharyngoplasty are patients with coronal closure patterns and
limited lateral pharyngeal wall motion. A sphincter is created via elevation of bilateral
superiorly based myomucosal flaps that are inset to the central posterior pharyngeal
wall at the level of velar closure.68 Posterior pharyngeal flap surgery involves elevation
of a central superiorly based myomucosal flap that is inset into a submucosal pocket
of the soft palate or the central soft palate. A pharyngeal flap should be considered in
patients with good lateral pharyngeal wall movement and sagittal or circular closure
patterns.69 Success rates between sphincter pharyngoplasty and pharyngeal flap
are similar.69,70 Complications after secondary speech surgery include persistent
674 Worley et al

VPD, dehiscence of flaps, and/or OSA. OSA is more commonly associated with
pharyngeal flap surgery, although it can occur after any oropharyngeal intervention.71

SUMMARY

Orofacial clefts are common congenital malformations with strong genetic and envi-
ronmental risk factors. In the perinatal period, feeding and nutrition can be a challenge
and the need for specialized feeders is common. Lip taping and NAM are early inter-
ventions that can be used to preoperatively modify cleft defects to enhance surgical
outcomes. Multiple techniques are available for repair of orofacial clefts and choice
of technique depends on cleft extent and surgeon preference. After definitive repair,
children remain at increased risk for middle ear disease and VPD and require
continued close follow-up with a multidisciplinary team.

Best Practices

What is the current practice?

CLP
Best practice/guideline/care path objectives
 Promptly identify children with orofacial clefts and coexisting congenital abnormalities.
 Ensure adequate nutrition, hearing, and speech development in children with orofacial
clefts.
 Optimize functional and cosmetic outcomes following CLP surgery.
What changes in current practice are likely to improve outcomes?
 Prompt multidisciplinary evaluation of newborns with orofacial clefts.
 Early consideration of the role of nonsurgical interventions to improve cleft symmetry (eg,
nasal taping).
Major recommendations
 The optimal time for initial evaluation by the multidisciplinary team is within the first few
days of life (level V).
 Multiple specialty bottles exist to assist in feeding children with orofacial clefts and there is
no difference in growth outcomes between bottle types (level Ia).
 Use of nasoalveolar molding can be considered for improvement of nasal alar symmetry and
columellar lengthening before CLP repair (level II–IV).
 CL repair should be considered around 3 to 6 months, and palate repair around 9 to
12 months (level V).
Summary statement
Early multidisciplinary evaluation of children with orofacial clefts is essential to optimizing
their preoperative management (feeding, consideration of NAM) as well as their postoperative
care following definitive cleft repair.
Data from Refs.20,24,33–35

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