Cleft Lip And Palate Pdf Download

This chapter is a comprehensive overview regarding the treatment of cleft lip and palate (CLP) patients. Epidemiology with global aspects are discussed. Etioilogy, genetics, and embryology are described in the initial part of the chapter. The importance of an interdisciplinary approach and treatment planning are discussed. The section on surgical treatment includes all aspects of CLP surgery, such as cleft lip repair, cleft palate repair, and alveolar cleft repair. Correction of secondary deformities like maxillary hypopla-sia, oronasal fistulas, and lip–nose deformities is another important part of the chapter. The importance of jaw– orthopedic and speech specialists is emphasized.

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Chapter 45

Cleft Lip and Palate:

An Overview

Radhika Chigurupati, Andrew Heggie, and Krishnamurthy Bonanthaya

This chapter is a comprehensive overview regarding the

treatment of cleft lip and palate (CLP) patients. Epidemiology

with global aspects are discussed. Etioilogy, genetics, and

embryology are described in the initial part of the chapter.

The importance of an interdisciplinary approach and treat-

ment planning are discussed. The section on surgical treat-

ment includes all aspects of CLP surgery, such as cleft lip

repair, cleft palate repair, and alveolar cleft repair.

Correction of secondary deformities like maxillary hypopla-

sia, oronasal fistulas, and lip–nose deformities is another

important part of the chapter. The importance of jaw–

orthopedic and speech specialists is emphasized.

Global burden of birth defects: cleft lip and palate

Epidemiology

Etiology and genetics

Embryology

Classification

Interdisciplinary management of the cleft individual

Prenatal diagnosis

General assessment

Feeding and nutrition

Ear, nose, and throat evaluation

Presurgical orthopedics

Cleft lip repair

Unilateral cleft lip

Bilateral cleft lip

Repair of cleft palate

Timing of repair

Surgical anatomy

Principles and techniques of palate repair

Speech and velopharyngeal dysfunction

Correction of oronasal fistulae

Orthodontic management of the cleft individual

Alveolar cleft repair

Replacement of absent teeth in the line of the cleft

Surgical correction of maxillary hypoplasia

Technical considerations for cleft orthognathic surgery

Conventional orthognathic surgery vs. distraction osteogenesis

Surgical correction of secondary lip and nose deformities

Secondary lip deformities

Cleft nasal deformity

Cleft septorhinoplasty

Global burden of birth defects:

cleft lip and palate

More than four million children are born with birth

defects worldwide every year. Craniofacial anomalies

comprise a large fraction of all human birth defects,

less frequent only than congenital heart disorders

and clubfoot. Cleft lip with or without palate (CL/P)

is the most common craniofacial birth defect with an

estimated quarter of a million affected babies born

each year in the world. This malformation shows

considerable variation across geographic regions and

ethnic groups and has significant medical, psycho-

logical, social, and economic ramifications.1 It is a

costly public health problem with an average lifetime

treatment cost per child in the US estimated to be

roughly $101 000.2

The World Health Organization (WHO) and most

cleft organizations across the globe recommend inter-

disciplinary care by a team of specialists. In reality,

however, surgical and non-surgical treatment is often

fragmented and dictated by socio-economic factors

and access to medical facilities. In developing coun-

tries, particularly in rural areas, care is often neglected

due to social beliefs and lack of awareness, or initiated

late due to restricted resources and inadequate access.

The delay in treatment and intermittent care by local

or overseas cleft mission surgeons, combined with

incomplete follow-up, results in poor outcomes with

unnecessary complications. More recently, some

humanitarian non-profit organizations supporting

cleft care have changed their aid philosophy. They

are identifying centers with a potential to deliver

quality care in low- and middle-income countries and

4 Dentofacial Deformities

providing support for the physicians, staff, and hos-

pital infrastructure, helping to establish parameters

of care, as well as monitoring treatment outcomes at

these centers.3

Birth defects are emerging as a cause of neonatal

mortality in countries that have made progress in

control of infectious diseases and malnutrition. The

strategies proposed to reduce the global impact of

birth defects include: (1) effective family planning,

genetic counseling, and prenatal diagnosis; (2) educa-

tion for couples to decrease maternal exposure to

avoidable environmental risk factors such as tobacco,

alcohol, and teratogenic medications; (3) improving

periconception maternal intake of micronutrients

such as folic acid (400 μg); and (4) improving the

availability of medical and surgical care locally for

the affected infants. National leadership and commit-

ment are essential for proper surveillance of birth

defects, infant mortality, and to monitor the clinical

and cost effectiveness of various interventions.4,5

The WHO initiative for collaborative craniofacial

anomalies research has identified areas of uncertainty

in clinical care and efforts are being made to conduct

trials with sufficiently large samples of patients to

provide evidence for treatment strategies. Initial

research efforts have focused on addressing surgical,

anesthetic, and nursing care for patients with cranio-

facial anomalies in developing countries. Surgical

techniques for repair of various cleft sub-types and

correction of velopharyngeal insufficiency are being

evaluated. Adjunctive services such as use of prophy-

lactic ventilation tubes, presurgical orthopedics, psy-

chological counseling, speech therapy, and feeding

interventions before and after surgery are also being

monitored and assessed. An international database of

craniofacial anomalies has been established to

improve answers to questions relevant to individuals

with cleft and craniofacial anomalies, their families,

and health care providers.6

Epidemiology

Cleft lip with or without palate (CL/P) has an aver-

age birth prevalence of 1:700 ranging from 1:500 to

1:2000, depending on the race (Table 45.1). There are

wide ethnic variations with highest occurrence in

Native Americans (3.6:1000), followed by Asians

(2.1:1000 Japanese births and 1.7:1000 Chinese births),

Caucasians (1:1000), and lowest in those of African

descent (0.3:1000). Cleft of palate only (CP), which

differs genetically from CL/P, has birth prevalence

rate of 1:2000 and is more similar across all popula-

tions.7–13 About half of the oral clefts involve lip and

palate (46%), a third of the clefts involve only the pal-

ate (33%), and clefts of lip alone account for 21%. CL/

P is more often unilateral than bilateral and more

common in males than females. The unilateral defects

occur more often on the left side than the right side.

Clefts of lip occur in the ratio of 6:3:1 for unilateral

left, unilateral right, and bilateral.14 CP is more com-

mon in females and more often associated with other

developmental anomalies.

Clefts are referred to as non-syndromic and syn-

dromic, based on their association with other anoma-

lies. About 50% of CP and 10% of CL/P are associated

with a syndrome.15–17 Some common syndromes

associated with cleft lip and palate include Van der

Woude, Treacher Collins syndrome, Down syn-

drome, oro-facial digital syndrome, Opitz syndrome,

craniofacial microsomia, and fetal alcohol syn-

drome.18 Nearly half of the syndromic cleft palate

presentations are associated with the triad of micro-

gnthia, glossoptosis, and airway obstruction (Pierre

Robin sequence). The most common syndromic pre-

sentations of this triad are Stickler's syndrome,

accounting for 25%, and velo-cardio-facial (VCF) syn-

drome, accounting for 15% of all syndromic cleft pal-

ate individuals.19

Etiology and genetics

Non-syndromic CLP is a complex trait with multifac-

torial etiology, resulting from gene–gene and gene–

environmental interactions. Identification of key

genes contributing to the genesis of orofacial clefts

will help in early diagnosis, disease prevention, or

possibly developing adjunctive therapies.The most

recent estimates suggest that anywhere from 3–14

genes contribute to cleft lip and palate.20,21 Candidate

genes and loci responsible for non-syndromic CLP

have been identified on chromosomes 1, 2, 4, 6, 11, 14,

17, and 19.22–24 Two genes IRF6 and MSX-1 now seem

to explain about 15% of isolated CLP. Important con-

tributors to CL/P are variants of interferon regulatory

growth factor (IRF 6) gene, whose function is related

to the formation of connective tissue. Mutations in

IRF6 lead to Van der Woude and popliteal pterygium

syndromes. Mutations in other genes, TBX22, FGFR1,

and P63, also contribute to syndromic clefts.25,26

Table 45.1 Epidemiology of oral clefts

Distribution of oral clefts

Cleft lip and palate 46%

Cleft palate only 33%

Cleft lip only 21%

Cleft lip and palate

Average birth prevalence 1:700

More common in males

Unilateral > bilateral

Left side > right side

Association with other anomalies 10%

Cleft palate only

Average birth prevalence 1:2000

More common in females

Association with other anomalies 50–60%

Cleft Lip and Palate: An Overview 5

Aberrant transforming growth factor beta-3 (TGF-β 3)

signaling plays a role in the pathogenesis of cleft

palate.

Environmental factors that contribute to the etiol-

ogy of facial clefting disorders include cigarette

smoking,27–30 folic acid deficiency during the pericon-

ceptional period,31,32 maternal exposure to alcohol

and teratogenic medications such as retinoids, corti-

costeroids, and anticonvulsants (phenytoin and

valproic acid).23 Co-sanguinous marriages, maternal

diabetes, and obesity have also been linked to an

increased risk of orofacial clefts. Less consistent asso-

ciations have been found between clefts and maternal

viral infections such as rubella and varicella.6,19

Studies conducted to determine the risk of having

a child with CL/P show that every parent has about a

0.14% (1:700) chance of having a child with a cleft.

The risk of recurrence of a cleft condition is deter-

mined by a number of factors, including the number

of family members with clefts, their relationship to

family members with clefts, race and sex of the affect-

ed individuals, and the type of cleft. Studies show

that the recurrence risk for first-degree relatives is

about 3.3% for CL/P and for isolated CP it is 2%.33

Once parents have a child with a cleft the risk of hav-

ing a second child with a cleft is about 2–5%, and after

two affected children that risk rises to 9–12%.34,35 In

twins with CLP and those with isolated CP, the con-

cordance is far greater for monozygotic twins than

for dizygotic twins.19 Parents and young adults

should be counseled appropriately by a geneticist so

that they are in a better position to make decisions

about future pregnancies.

Embryology

The embryologic development of the face begins at

4 weeks after conception from the neural crest ecto-

mesenchyme that forms five prominences; the fronto-

nasal process, and paired maxillary and mandibular

processes surrounding a central depression. During

the fifth and sixth weeks of embryonic development,

bilateral maxillary processes derived from first bra-

chial arch fuse with the medial nasal process to form

the upper lip, alveolus, and the primary palate

(Fig. 45.1).

The lateral nasal process forms the alar structures

of the nose. The lower lip and jaw are formed by the

mandibular processes. This process of formation of

the face is the consequence of a cascade of processes

that involve cell proliferation, cell differentiation, cell

adhesion, and apoptosis. Failure or error in any of

these cellular processes that lead to fusion of the

medial nasal process with the lateral nasal and maxil-

lary process can cause orofacial clefts (Fig. 45.2). The

Naso-optic

furrow

Eye

1st pharyngeal

groove

Hyoid arch

Nasal pit

Lateral nasal

prominance

Developing

eyelids

Globular

prominance

Cardiac

swelling

Mandibular

prominance

Fig. 45.1 (a) Schematic diagram showing embryonic develop-

ment of face at 6 weeks. (b) Electron microscopy showing the

development of face of a 37-day-old human embryo. The nasal

pit (NP) is surrounded by the medial nasal process (MNP) and

lateral nasal process (LNP) and maxillary process (MAX).

(Adapted and redrawn from Sperber, GH. Craniofacial

Development. Hamilton: BC Decker Inc, 2001.)

(a)

(b)

(a)

(b)

Fig. 45.2 Orofacial clefts resulting from errors during embry-

onic development of face. (a) Unilateral cleft of upper lip.

(b) Bilateral cleft of the upper lip. (c) Midline cleft of the upper

lip and nose. (d) Median mandibular cleft. (Adapted and

redrawn from Sperber, GH. Craniofacial Development . Hamilton:

BC Decker Inc, 2001.)

6 Dentofacial Deformities

molecular events that underlie these cellular process-

es are under the control of a strict array of genes that

include fibroblast growth factors (FGFs), sonic hedge-

hog (SHH), bone morphogenic proteins (BMPs), and

members of the transforming growth factor beta

(TGF-β ) superfamily and other transcription factors.10

The formation of the secondary palate begins during

the sixth week after conception from the two palatal

shelves, which extend from the internal aspect of the

maxillary processes. During the eighth week, these

bilateral maxillary palatal shelves after ascending to

an appropriate position above the tongue, fuse with

each other and the primary palate. A disruption in

the fusion of these embryonic components can occur

due to delay in elevation of the palatal shelves from

vertical to horizontal, defective shelf fusion or post-

fusion rupture resulting in a cleft of the secondary

palate (Fig. 45.3).36

Classification

In order to standarize documentation and com-

municate effectively, various types of classification

systems have been described. The early Veau classifi-

cation included groups 1–4 with increasing severity

of clefting:

• group 1 – cleft of the soft palate;

• group 2 – cleft of the hard and soft palate up to

incisive foramen;

• group 3 – complete unilateral cleft lip and palate;

• group 4 – complete bilateral cleft lip and palate.

However, this classification is not always adequate

to document the variations. The more sophisticated

schematic diagrams, such as the one described by

Kernahan and Stark have been used recently

(Fig. 45.4).37 Berkowitz used a simple classification

for labio-palatine clefts:

1. Clefts of lip and alveolus.

2. Clefts of primary (including lip) and secondary

palate.

3. Clefts of secondary palate only.

4. Submucous cleft.

Interdisciplinary management of

the cleft individual

The idea of interdisciplinary care is to coordinate

treatment by multiple specialists in a timely fashion

with an aim of achieving normality in all aspects,

including feeding, breathing, speech, hearing, align-

ment of teeth, appearance, and overall psychological

and physical development. The timing of surgical

and non-surgical interventions should coincide with

the physical, cognitive, and social development of the

child (Table 45.2). Cleft teams generally include a

craniomaxillofacial surgeon, pediatrician, nurse

practitioner, speech pathologist, orthodontist, social

(a)

(c)

(b)

(d)

Fig. 45.3 Coronal scanning electron microscopy photos of human embryo showing the stages of formation of the palate at 8–9

weeks. (a) Development of palate showing palatal shelves and tongue position. (b) The vertical orientation of the palatal shelves on

either side of tongue. (c) Palatal shelves elevate and (d) fuse with each other and the nasal septum in the midline.

Cleft Lip and Palate: An Overview 7

worker, and geneticist. Experience in Scandinavian

countries and a multicenter Euro-cleft study has dem-

onstrated that standardization, centralization, and

participation of surgeons who perform a large num-

ber of cleft procedures produce better surgical results

in terms of speech, appearance, and facial growth.38

However, this cannot be applied to all countries, par-

ticularly those with a high volume of cleft individuals

and a limited number of care facilities.

Prenatal diagnosis

Interdisciplinary team care begins with prenatal

diagnosis and parental counseling. With the advent

of sophisticated high resolution three-dimensional

(3D) ultrasonography and genetic tests for screening

of birth defects, intrauterine diagnosis of cleft lip is

possible. Early diagnosis of a cleft of the lip should

alert the obstetrician of the possibility of other mal-

formations that may require further investigations.

While early diagnosis may help parents to be better

prepared, the advent of such a capability raises both

ethical and psychological issues, such as dilemma of

termination of birth. Physicians and surgeons have

to inform parents that CLP in the absence of other

major systemic anomalies is a treatable non-life-

threatening condition. The cleft team also can discuss

feeding issues, timing of lip and palate surgery, and

help establish contact with support groups for the

family.39

Transvaginal ultrasonography may reveal a cleft

of lip as early as 11 weeks whereas 16–20 weeks is

ideal for transabdominal ultrasonography. Several

factors may influence the accuracy of ultrasound

studies: sophistication of the scanning equipment;

experience and skill of the sonographer; number of

weeks into pregnancy; position of the baby while

scanning; amount of amniotic fluid; maternal body

structure; and severity of cleft.

Premaxillary protrusion is an important clue to the

presence of cleft lip and cleft palate and may be more

conspicuous than the cleft itself. The presence of a

paranasal echogenic mass favors the presence of

bilateral cleft lip and cleft palate. Clefts of palate alone

are rarely visualized on ultrasound.40 The majority of

the cases of orofacial clefts are detected antenatally. A

recent study by Johnson et al . showed that the fre-

quencies of prenatal diagnosis for CLP, cleft lip only,

and CP only were 33.3%, 20.3%, and 0.3%, respective-

ly.41 Although the benefits of fetal healing have been

well documented, at this time there is no indication

for intrauterine repair of the cleft lip deformity as the

Nose

Nasal floor

Lip

Alveolus

Hard palate

Hard

palate

Soft

Fig. 45.4 Kernahan and Stark classification of clefts. Kernahan's

striped-Y classification: areas 1 and 5 represent the right and

left sides of nasal floor, 2 and 6 represent the right and left sides

of the lip respectively. The alveolus is represented by areas 3

and 7, the hard palate anterior to the incisive foramen by areas

4 and 8, the hard palate posterior to the incisive foramen by

areas 9 and 10, and the soft palate by area 11. (From Millard Jr

DR. The unilateral deformity. In: Cleft Craft: The Evolution of its

Surgery, Vol 1. Boston, MA: Little, Brown, 1977; 52.)

Table 45.2 Interdisciplinary care of the cleft patient

Prenatal

Diagnosis and parental counseling

0–6 months

General assessment for associated anomalies

ENT evaluation – breathing, feeding, swallowing, and hearing

Presurgical orthopedics (0–3 months)

Primary lip repair ( 3–4 months)

6 months – 2 years

Speech and oral sensory motor assessment

Grommets/ear tubes (as needed)

Primary palate repair ( 9–12 months)

Preschool: 3–5 years

Dental care

Speech assessment and therapy (continue as needed)

Assess need for lip revision

Childhood: 6–12 years

Correction of velo-pharyngeal dysfunction (as needed)

Orthodontic treatment – phase I

Alveolar cleft repair (8–11 years)

Adolescence: 13–18 years

Orthodontic treatment – phase II

Orthognathic surgery (if needed) – 14–16 years (female),

16–18 years (male)

Revision chielo-rhinoplasty

Replacement of missing teeth (as needed)

8 Dentofacial Deformities

risk of fetal surgery is far too high both for the fetus

and mother for correction of this non-life-threatening

condition.

General assessment

Every child born with a CL/P should be thoroughly

assessed by complete physical examination and nec-

essary diagnostic tests to check for associated sys-

temic abnormalities, including congenital heart, renal,

or airway anomalies. If the child is delivered in a non-

medical facility or a small hospital they should be

referred to a tertiary hospital with specialists or a cra-

niofacial team for further evaluation. A proper airway

assessment, and counseling for nutrition and feeding

should be initiated immediately.

Feeding and nutrition

Feeding is one of the first concerns in a child born

with a CL/P. Parents should be taught how to feed

the baby and informed about various feeding nipples

that can deliver more milk under less pressure. The

goal is to provide adequate nutrition to satisfy the

caloric requirements and avoid failure to thrive. The

team nurse generally provides the parents with infor-

mation on feeding before birth or immediately after

birth. Children with cleft lip only without a cleft pal-

ate may have some difficulty in creating a seal around

the nipple but generally can be breast fed before and

after lip surgery. However, the presence of a cleft pal-

ate makes it difficult to create a negative pressure that

is necessary to feed. Four randomized clinical trials

conducted with a total of 232 babies showed that

squeezable bottles may be easier to use than rigid

bottles for children with CL/P. There was no statisti-

cal difference found in growth outcomes of children

with CL/P who were fitted with a passive palatal

appliance to help with feeding and those without an

appliance.42,43 A feeding tube is rarely necessary

except in infants with other associated anomalies,

particularly airway anomalies.

Ear, nose, and throat evaluation

A proper airway assessment should be priority for a

newborn with congenital craniofacial anomalies.

Infants are obligate nasal breathers. It is important to

check if there is obstruction at any level in the upper

or lower airway, including the nares and choanae.

Children born with a cleft of the palate may have

associated micrognathia, glossoptosis, and airway

obstruction (Fig. 45.5). In these children, one should

look for signs of increased effort while breathing,

stridor, weight loss, and failure to thrive. Parents

should be informed to watch for an abnormal breath-

ing pattern or respiratory distress, particularly during

upper respiratory tract infection. If there are signs of

obstruction, a pediatric otolaryngologist should con-

sulted to perform an endoscopic evaluation of upper

and lower airway to look for possible any cause of

obstruction.

An audiology assessment is recommended soon

after birth to check for hearing abnormalities. Children

with cleft palate exhibit a higher frequency of otitis

media prior to palate repair than those without clefts.

Middle ear ventilation disorders due to eustachian

tube dysfunction can cause conductive hearing loss.

This can also contribute to speech and language delay

in these children. Although not as common as con-

ductive hearing loss, sensorinerual hearing deficits

exist within the cleft population and it has an affect

on speech perception and clarity, as well as auditory

comprehension skills.44,45 Early speech and language

stimulation and an initial speech evaluation no later

than 6 months after birth is recommended for chil-

dren with clefts of palate.

Presurgical orthopedics

The benefits of presurgical orthopedics include better

alignment of the alveolar segments and premaxilla,

tension-free approximation of the cleft lip edges,

and improvement of nostril symmetry and shape.

Presurgical orthopedics was introduced in the man-

agement of clefts by McNeill and Burston. They initi-

ated the use of a palatal acrylic plate in order to bring

the collapsed maxillary alveolar segments into proper

alignment prior to lip surgery. Latham described use

of an active expansion device to align the collapsed

lateral maxillary segments and retract the premaxilla

in complete bilateral clefts, and to achieve symmetry

of the alveolar arch in complete unilateral clefts.46,47

Grayson and others have shown that gentle appli-

cation of presurgical orthopedic forces to mold the

alveolar segments and the nostrils within 0–3 months

of birth has shown some benefits in correction of the

nasal deformity in children with complete bilateral

CLP and wide unilateral clefts. Nasoalveolar mold-

ing increases the surface area of the nasal mucosal

lining. It also helps with elongation of the columella

and making the columella upright. This preoperative

expansion of the nasal lining allows suturing of inter-

domal cartilages without tension and decreases wid-

ening of the nose (Fig. 45.6).48–51

Fig. 45.5 Child with Pierre Robin sequence (PRS) showing a

wide cleft of palate, small lower jaw, and a retropositioned

tongue in the cleft obstructing the airway.

Cleft Lip and Palate: An Overview 9

There is a wide variation in the availability of

expertise and cost of treatment when it comes to

infant presurgical orthopedics or nasoalveolar mold-

ing. In recent years, several centers have reported the

adoption of this technique to improve the outcomes

of lip and nose repair, especially in complete bilateral

CLP.52,53 It is important to evaluate these recent stud-

ies critically for their overall clinical and cost effec-

tiveness. Non-surgical lip adhesion with tape is a

cost-effective and simple technique that can bring

the alveolar segments closer to facilitate cheiloplasty

in infants with wide clefts of the lip and palate

(Fig. 45.7).

Fig. 45.6 (a) Baby with a bilateral cleft lip and palate showing

protrusion of premaxilla before nasoalveolar molding.

(b) Nasoalveolar molding appliance. (c) Nasoalveolar molding

appliance in position to expand nasal soft tissues and align the

alveolar cleft segments. Note the position of premaxilla in

(d) frontal and (e) lateral views after nasoalveolar molding.

(Courtesy of Dr. Oberoi, UCSF Center for Craniofacial

Anomalies)

(a) (b)

(c)

(d)

(e)

Fig. 45.7 (a–d) These photos illustrate that simple presurgical

orthopedic techniques, such as lip taping, can be used to align

the cleft alveolar segments prior to lip repair.

(a) (b)

10 Dentofacial Deformities

Cleft lip repair

The goal of primary lip repair is to reconstruct a func-

tional lip with minimal scarring and normal appear-

ance. The timing for primary lip repair is usually

between 3 and 6 months after birth. Most craniofacial

centers follow the rule of ten's to ensure that the

infant is fit for the surgical procedure. This rule

implies that the infant should be at least 10 weeks of

age, weigh at least 10 lbs, and have a hemoglobin

level of at least 10 g/100 ml. Some centers have

reported lip repair in children with lower hemoglobin

levels (8 g/100 ml) with no deleterious effects. In

low- and middle-income countries, infants are often

malnourished and proper feeding and nutrition

counseling is essential to prepare them for lip surgery

by 3 months.54 Lip repair is performed under general

anesthesia with an Oral RAE® endotracheal tube

taped to the midline of lower lip without distorting

the commissure. Postoperative care includes keeping

the wound clean by preventing crusting and using

antibiotic cream; in some centers arm restraints are

also used for 7–10 days.

A surgical lip adhesion may be preferred as an ini-

tial surgical procedure within 6–8 weeks after birth in

some centers. Lip adhesion helps to align the maxil-

lary alveolar segments and achieve a tension-free

definitive lip repair at a later date. Good approxima-

tion of the alveolar segments also allows the surgeon

to perform a gingivoperiosteoplasty at the time of

definitive cheiloplasty. The disadvantages of convert-

ing the complete cleft lip to an incomplete one by lip

adhesion are the need for an extra operation and the

possibility of excising more tissue at the time of

definitive lip repair. Non-surgical orthopedic tech-

niques during the first 6–8 weeks after birth, as

described earlier in this chapter, can produce good

alignment of the alveolar segments.

Unilateral cleft lip

Surgical anatomy

Unilateral cleft lip is an asymmetric deformity that

presents with a multitude of inherent anatomic varia-

tions (Fig. 45.8). The most visible anatomic abnormali-

ties of the complete unilateral cleft lip and nose

deformity are due to the abnormal position of the

orbicularis oris muscle. After fetal dissections, Fara

noted that in a complete cleft lip, the fibers of the

orbicularis oris muscle instead of proceeding hori-

zontally from the commissure towards the midline

turn upward along the margins of the cleft. The fibers

of the orbicularis terminate medially beneath the base

of the columella and laterally beneath the alar base

and periosteum of the piriform rim.55 It is these

abnormal muscle attachments and pull, that cause

the typical bulge on the unrepaired cleft lip, distor-

tion of the ala of the nose, and deflection of anterior

nasal spine and septum of the nose. The nasal defor-

mity is proportionate to the severity of clefting. There

is slumping of the alar cartilage on the cleft side lead-

ing to an asymmetric nasal tip. The alar base is dis-

placed laterally, inferiorly, and posteriorly leading to

a widened nasal aperture. There is shortening of the

medial crus of the alar cartilage and lack of overlap of

upper and lower lateral cartilages. The columella and

the caudal edge of the septum and anterior nasal

spine are deviated to the non-cleft side.56

Evolution of unilateral cleft lip repair

Several surgeons, including, Rose (1891), Thompson

(1912), Blair (1930), Le Mesurier (1949), Tennison and

Randall (1952), and Skoog (1974), have contributed to

the evolution of cleft lip repair, but the most popular

technique was introduced by Millard (1955) who

described the rotation–advancement concept. Today

(a) (b)

(c)

Fig. 45.8 (a–c) Variations of the unilateral cleft lip and palate deformity. The typi-

cal features of the abnormal anatomy in UCLP are seen in these photos. There is

vertical shortening of the lip at the cleft margins, obicularis oris muscle fibers ter-

minate medially beneath the base of the columella and laterally beneath the alar

base. Fibers of orbicularis oris in the lateral segment are more hypoplastic and do

not extend up to the cleft margin. Note the discrepancy of vermilion width with

excess vermilion at the cleft margin on the lateral side compared to the medial side.

The maxillary alveolus exhibits asymmetry with an upward and outward rotation

of the greater segment.

Cleft Lip and Palate: An Overview 11

various modifications of the rotation–advancement

technique by Millard are used to repair the unilateral

cleft lip deformity.57 In Millard's technique the medial

flap is rotated downward to achieve length, while the

lateral flap is advanced (Fig. 45.9). It is an extremely

versatile procedure that the surgeon can modify or

adjust while operating. The advantage of this tech-

nique is that the suture line lies on the recreated phil-

tral column and incision allows easy access for

primary rhinoplasty to reposition the nasal septum,

lower lateral cartilage, and alar base. The main disad-

vantage is that the inexperienced surgeon requires

good surgical judgment during the operation as it is

not based on exact measurements. The triangular flap

technique, described by Tennison and Randall, is

based on exact measurements, can be reproduced

well, and used more easily in wide clefts of the lip.58

Principles of repair of unilateral cleft lip and

nose

An adequate repair of the unilateral lip deformity

should correct the alignment of the orbicularis oris

muscle, create a cupid's bow and philtral column on

the affected side. In the unilateral defect the normal

side can be used as a guide to identify the key points

and to plan the incisions on the cleft side. Despite

inherent variations there are some similarities that

form the basis of the guiding principles in surgical

repair of this deformity (Fig. 45.10).57

1. Rotation or lengthening of shortened vertical

height of lip. The difference in the vertical length

of the lip from the height of the cupid's bow to the

base of the columella between the non-cleft side

and the cleft side indicates the amount of rotation

and back cut necessary. The medial lip element

(non-cleft side) is rotated inferiorly to achieve ade-

quate length and symmetry of the cupid's bow.

2. Advancement of flap of tissue from lateral to

medial. The flap of tissue from lateral should be

advanced in to the lip on the medial segment. It is

important to release the abnormally inserted para-

nasal and facial muscles at the alar base by sub-

periosteal dissection in order to approximate the

edges without tension.

3. Retaining cupid's bow and creating a philtral col-

umn. Approximation of the cleft edges should be

achieved without loss of natural landmarks: the

cupid's bow, philtral dimple, and philtral column.

The scar of union of cleft edges should be placed

along a natural line – the philtral column. Creating

a good philtral column requires proper approxi-

mation of the muscle and tension-free closure of

the overlying skin.

4. Muscle reconstruction. The muscle bellies should

be dissected within the skin and mucosal envelope.

The muscles should be approximated with inter-

rupted mattress sutures. The muscles at the base of

the columella and ala should be dissected and

approximated to help reposition the distorted

nasal structures.

5. Restoration of the alveolar continuity. If the alveo-

lar segments are closely approximated a gingivo-

periosteoplasty can be performed at the time of

primary lip repair to achieve a continuous alveolar

arch.

6. Primary repair of the distorted nasal anatomy.

This requires wide undermining of the skin drape

(a)

(b)

Fig. 45.9 (a) Markings of the key points and incisions for repair

of the unilateral cleft lip using the Millard rotation–advance-

ment principle: lengthening the shortened vertical height of the

lip on the medial side to match the lateral, and advancing the

flap of tissue from lateral to medial. (b) Markings of the key

points and incisions for repair of the unilateral cleft lip using

the triangular flap technique.

Fig. 45.10 Unilateral cleft lip repair. (a) Preoperative and

(b) postoperative views of lip repair performed by a modified

Millard technique.

(a) (b)

12 Dentofacial Deformities

cal cupid's bow. The premaxilla is protuberant and

sometimes deviated to one side making tension-free

approximation of muscle and cleft margins difficult.

The orbicularis oris muscle which is in the lateral lip

elements inserts at the alar base on each side. The

accompanying nasal deformity consists of a columella

that is abnormally short, a wide nasal tip, and a flared

alar base due to the malpositioned, splayed alar carti-

lages. The variations of bilateral CLP deformity are

shown in the Fig. 45.12.

Evolution of bilateral cleft lip repair

The evolution of repair of the bilateral cleft lip over

the years has shown that it is best to repair both sides

of the cleft lip at the same time. In many centers today,

primary nasal correction is performed along with the

repair of the lip. The observation that the alar carti-

lages are splayed and rotated caudally was important

in the evolution of primary rhinoplasty in cleft lip

repair. The fear of growth interference when nasal

cartilage is manipulated during primary repair has

been one of the reasons for delaying nasal repair. For

many years forked flaps were used to lengthen the

columella, but McComb and Mulliken emphasized

the importance of early positioning of the alar carti-

lages and unraveled the problem of the short colu-

mella and broad nasal tip in the bilateral cleft lip.

McComb, after reviewing his initial work over a peri-

od of 15 years, found that the nostril shape was still

abnormal, the tip remained broad, and columella was

very long.62 He proposed primary nasal correction by

repositioning the alar cartilages through a vertical

skin incision.63 Over the years, the techniques that

have been used for repair of the bilateral cleft lip

and nose deformity include: the straight line closure

or veau III operation; Tennison's triangular flap

technique (similar to unilateral repair); Millard's

technique; Manchester, Skoog, Black, and Mulliken

techniques.57,64

Principles of repair of bilateral cleft lip and

nose

The basic principles guiding repair of the bilateral

cleft lip deformity are: maintaining symmetry; estab-

(a)

(b)

Fig. 45.11 These figures show primary nasal deformity correc-

tion in the unilateral cleft lip. (a) Wide undermining of the skin

drape over the lateral crura of the alar cartilages. (b) Suturing of

the alar dome.

over the lateral crura of the alar cartilages and

repositioning of the nasal septum with a suture

(Fig. 45.11). Long-term results of primary rhino-

plasty by McComb and others show that the out-

comes are better and there is no decrease in overall

size or nasal growth inhibition after primary

correction.59–61

Bilateral cleft lip

Surgical anatomy

Bilateral cleft lip repair is much more challenging and

the results are often less satisfactory than those of

unilateral cleft lip. Complete bilateral clefts of lip are

rare, accounting for only 10% of cleft lips and there-

fore the experience in treating these deformities is

limited. The typical anatomical abnormalities that

make the bilateral cleft lip deformity so difficult to

repair are the absence of muscle in the prolabial seg-

ment, resulting in lack of philtral dimple, philtral col-

umns, white roll margin and the median tubercle.

The prolabium lacks the angular peaks and the typi-

Fig. 45.12 (a, b) Variations of the bilateral cleft lip and palate

deformity. These photos show the typical anatomical abnor-

malities seen in individuals with complete bilateral clefts of lip

and palate: lack of muscle in the prolabium, lack of the angular

peaks and typical dip of cupid's bow, lack of a definite philtral

ridge or dimple in the prolabium, abnormally short and wide

columella, lack of adequate labial sulcus depth in the premaxil-

lary area, and flat nasal tip and flared alar base.

Cleft Lip and Palate: An Overview 13

lishing muscle continuity; designing the prolabial

flap to achieve appropriate philtral width and shape;

forming a cupid's bow and median tubercle from the

lateral labial tissue; and finally repositioning the alar

cartilages to construct the nasal tip and columella

(Fig. 45.13). The principles outlined here are those of

the Mulliken simultaneous lip and nose repair.

1. Establishing symmetry. Performing both sides

simultaneously allows achievement of symmetry.

2. Designing a prolabial flap of appropriate width.

The design and width of the prolabial flap should

be narrow and biconcave to avoid a wide abnor-

mal philtrum as the child grows. This width should

be roughly 4 mm at the columella base and 6 mm at

the peak of the cupid's bow. The mucosa of the

prolabium can been used either to deepen the sul-

cus in the premaxillary region or to reconstruct the

lip. The author's preference is to use it to deepen

the labial sulcus which is usually shallow.

3. Forming the cupid's bow and median tubercle

from lateral lip elements. The prolabium usually

has a very narrow strip of vermillion and hence, in

the majority of cases, reconstruction of the vermil-

lion in the mid portion of the lip presents a difficult

problem. The incision on the prolabium is made at

the mucocutaneous junction and lateral lip vermil-

lion flaps are be used to reconstruct of the central

portion of the vermillion or the tubercle.

4. Establishing muscle continuity. In a complete

bilateral cleft the prolabium is devoid of muscle

fibers. Establishing continuity of muscle beneath

the skin of the prolabium is the only way to recon-

struct a normal functioning lip. The muscle fibres

that are inserted at the alar base on each side are

released and reoriented to be approximated in the

midline with vertical mattress sutures. This may

be difficult, particularly at the upper edge of the

lip in wide clefts or a cleft with protruding pre-

maxillae. While a sulcular incision bilaterally

beneath the lateral lip elements is essential to

mobilize the lip and achieve tension-free closure,

the debate has centered on whether the incision

should be subperiosteal or supraperiosteal. The

author's preference is to perform a subperiosteal

dissection following Delaire's principles.65,66

5. Reconstruction of the nasal tip and columella. The

lower lateral cartilages should be mobilized ade-

quately from the overlying skin by wide dissection

and undermining. The splayed cartilage domes

can be approached by combining rim incisions

with the prolabial flap. The lower lateral cartilages

are repositioned anatomically and fixed with

interdomal mattress sutures to create the tip and

columella.

6. Repositioning the alar base. The nasal musculature

must be mobilized by performing a subperiosteal

dissection along the piriform rim and the wide alar

base must be cinched with a suture to the base of

the anterior nasal spine.

7. Management of the premaxilla. A protruding pre-

maxilla in a complete bilateral CLP can impede the

approximation of the lip wound edges with a

tension-free closure. This can result in a wide scar

and a poor cosmetic result. It is therefore impor-

tant to reposition the premaxilla in the appropriate

position with presurgical orthopedics whenever

possible.

Repair of cleft palate

The goals of palate repair are to normalize speech by

surgical approximation and realignment of the aber-

(a)

(b)

(c)

Fig. 45.13 Repair of bilateral cleft lip and palate. (a) Key points

to be marked for bilateral cleft lip repair. (b) Designing prolabial

flap. (c) Forming cupid's bow and median tubercle from lateral

lip elements. (d) Preoperative and (e) postoperative views of a

child with bilateral cleft lip repaired by modified Mulliken

technique.

14 Dentofacial Deformities

rant attachments of the palatal muscles, and to seal

the communication between the oral and nasal cavi-

ties without fistulae.

Timing of repair

The timing of palate repair to achieve optimal speech

with minimal facial growth disturbance has been

one of the more debated issues in cleft literature.

Historically, cleft repair of the hard palate was

delayed to minimize impairment of maxillofacial

growth. It is now well accepted, and evidence in the

literature shows, that speech outcomes are better

when soft and hard palate repair is completed before

speech development.67 Palate surgery is therefore

timed according to the infant's speech developmental

stage rather than chronologic age. For most children

developing normally, this is around 9–12 months.

The majority of the surgeons repair the palate (i.e.

hard and soft palate) in one stage before 12 months of

age.68 Some recommend a two-stage repair with soft

palate repair as early as 3–6 months, at the time of

primary lip repair, and hard palate by 12–15 months

of age.69 Children with cleft palate often have other

anomalies and it may be necessary to modify the tim-

ing of repair in the presence of comorbidities, particu-

larly airway anomalies. Repair of the palate may be

delayed up to 14–16 months of age if there are con-

cerns of airway obstruction. Premature babies and

infants with micrognathia are particularly at increased

risk for postoperative episodes of apnea after palate

repair.69

Surgical anatomy

Cleft of the palate can range from a minor submucous

cleft affecting only the soft palate to a complete bilat-

eral cleft affecting the primary and secondary palate.

It is important to look for overt signs of a submucous

cleft if there is any suspicison. These signs include a

bifid uvula, notching of the posterior nasal spine or

translucency in the mid palatine region of the soft

palate due to lack of muscle.

The muscles of soft palate that help with the func-

tion of speech and swallowing include the levator

palatini, tensor palatini, palatopharyngeus, palato-

glossus, and musculus uvulae. The soft palate in a

non-cleft individual acts as a muscular valve that can

lift superiorly and posteriorly to appose the pharyn-

geal wall and achieve velopharyngeal closure during

speech. In a child with an unrepaired cleft the soft

palate cannot function as a muscular valve. This is

due to the abnormal orientation and attachment of

the muscles, primarily the levator palatini. The bun-

dles of the levator on each side are longitudinally

directed to insert into the posterior edge of the pala-

tine bone instead of joining in the midline in a trans-

verse orientation and inserting into the palatine

aponeurosis (Fig. 45.14).70,71 In addition, the sphincter

action of the palatoglossus, palatopharyngeus, and

superior constrictor muscles at oropharyngeal aper-

ture is compromised leading to velopharyngeal

insufficiency. The tensor palatini muscle fibers, which

control the opening of the eustachian tube and aerate

the middle ear, do not function optimally, often lead-

ing to chronic otitis media.

Eustachian tube

Soft palate

Superior fibers of

superior constrictor

Palatine aponeurosis

(from tensor)

Ten so r Ten sor

Palatine process

of maxilla

Vomer

Levator

Superior

constrictor

Hamulus

Aponeurosis

Maxilla

(a) (b)

Fig. 45.14 (a) The muscle attachments in a normal palate.

(b) Note abnormal attachments of muscle in a cleft palate. (c) A

cleft of the secondary palate. (d) A bilateral cleft palate where

neither palatal shelves fuse with the nasal septum, leaving a

wide cleft in the midline.

(c)

(d)

Cleft Lip and Palate: An Overview 15

Principles and techniques of palate

repair

The main principle of cleft palate repair is to detach

and retropose the abnormal insertion of the levator

palatini and join the muscles of both halves of the soft

palate in the midline at the junction of the middle and

posterior third of the soft palate, in order to achieve

proper elevation of the soft palate.72 In the hard

palate, the most important principle is to reflect

mucoperiosteal flaps based on the grater palatine

arteries which emerge from the greater palatine fora-

men bilaterally at the postero-lateral area of the hard

palate.

Cleft palate surgical treatment dates back to the

1760s when a French dentist, Le Monnier, first

attempted repair. Several other surgeons, including

Philbert Roux, Carl Ferdinad Von Graefe, and Johann

Dieffenbach, subsequently described techniques to

repair the palate. It was Von Langenbeck, who first

described the use of mucoperiosteal flaps for cleft

palate surgery.73,74 Kriens, in 1969, first introduced

the concept of an anatomical approach to veloplasty

by restoring the levator sling.72 The choice of surgical

technique depends of the type of cleft. At the time of

the primary palatoplasty the ears should be inspected.

If there is evidence of serous otitis, a myringotomy is

performed and fluid aspirated with placement of

grommets or ventilating tubes in the myringotomy

incisions.

The two-flap palatoplasty is a commonly used

surgical technique for repair of the complete unilater-

al and bilateral cleft of the palate. The edges of the

cleft are incised from the alveolus to the base of the

uvula and bilateral full-thickness mucoperiosteal

flaps are reflected (Fig. 45.15). The levator palatini

muscles are released and dissected to be repositioned

horizontally and sutured. Bilateral releasing incisions

are made to decrease the tension in the midline.75

For the cleft of the secondary palate a Von

Langenbeck repair can be used. In this technique

bilateral releaxing incisions are made and the muco-

periosteum is elevated to complete the stripping and

closure of nasal layer, muscle, and oral layers as

shown in Fig. 45.16a. The Veau–Wardwill–Kilner (V–

Y pushback) technique, named after Thomas Kilner,

Victor Veau, and William Wardwill, is used less often

for repair of cleft of secondary palate. In this tech-

nique the oral mucosa is divided anteriorly, which

may lengthen the palate but leaves areas of exposed

bone in the anterior hard palate that can potentially

cause maxillary growth disturbances. The double

reversing Furlow Z-plasty was introduced by

Dr. Leonard Furlow Jr in 1978.76 This technique uses

two reversed Z-plasties of the oral and nasal mucosa

to repair the cleft. It has two advantages: restoring

the normal anatomic position of the levator palatini

in the middle and posterior third of soft palate

and increasing soft palate length (Fig. 45.16b).

Complications of palatoplasty include postoperative

bleeding, airway obstruction, wound dehiscence, and

fistula formation. Care should be taken to achieve

adequate intraoperative hemostasis and careful post-

operative monitoring is essential to avoid airway

obstruction.68

Speech and velopharyngeal

dysfunction

Children with palatal clefts are at risk for a wide

range of speech problems related to resonance, articu-

lation, phonation, learning, and language delay.

These speech abnormalities can be caused by velo-

pharyngeal insufficiency, oronasal fistula, weak lip

pressure, abnormal tongue pressure, malpositioned

teeth, abnormal jaw relationship, neuromuscular

dysfunction, and conductive or sensorineural hearing

loss. It is important to identify and associate the cause

(a)

(b)

Fig. 45.15 (a) Classic two-flap palatoplasty showing two pedi-

cled palatal mucoperiosteal flaps and closure of muscle and

nasal mucosa. (b) The two flaps are brought together by good

approximation of the oral layer over the nasal layer.

16 Dentofacial Deformities

with effect. Assessment of speech should begin as

early as 6 months of age and be monitored through-

out adolescence.

Speech in cleft individuals often has a nasal quality.

This perceived hypernasality during speech is typi-

cally due to incomplete closure of the velopharyngeal

port which separates the nasal cavity from the oral

cavity during speech production. Typically, velopha-

ryngeal insufficiency (VPI) refers to the inability of

the soft palate and the posterior and lateral pharyn-

geal walls to come together to create a seal during

speech production. Nasal air escape during speech

may be due to an unrepaired submucous cleft of the

palate or after palatal repair due to lack of sufficient

soft palate length or impaired movement secondary

to scar tissue. It may be due to improper position of

the palatal musculature impeding elevation of the

soft palate, or ineffective oropharyngeal sphincter

closure, or due to fistulae in the hard or soft palate.

Assessment of speech for VPI has to be both clini-

cal and instrumental. Clinical assessment of resonance

characteristics is best performed as a child's articula-

tory repertoire develops. Non-instrumental testing

utilizing visualization of airflow with a reflecting

mirror and nasal pinching often assists with the pre-

diction of velopharyngeal function during speech. If

deficits are identified, then further assessment using

nasendoscopy to assess posterior and lateral pharyn-

geal wall motion or videoflouroscopy is indicated.

Nasopharyngoscopy is a diagnostic tool for evalua-

tion of velopharyngeal function that helps the sur-

geon to make a decision regarding the need for

therapeutic intervention. Small-diameter pediatric

flexible endoscopes with a good light source and topi-

cal anesthetic for nasal mucosa will help children

become more compliant with this procedure.

Studies show the need for surgical correction of

VPI range from 4–30%.77,78 The most effective method

for correction of VPI is controversial but the choice of

the procedure depends on cause and the where the

abnormality is found: lateral or posterior pharyngeal

wall.79 A posterior flap pharyngoplasty is indicated

when there is limited or lack of posterior wall motion.

The flap may be superiorly or inferiorly based. A

superiorly based flap, with the base at the level of the

tubercle of the atlas and insertion into the soft palate,

is more popular than the inferiorly based flap. A lat-

eral pharyngoplasty is advocated for managing

decreased lateral wall motion by creating a dynamic

sphincter to control the size of the pharyngeal orifice.

This was first described Orticochea and modified by

Jackson.80 The sphincter is created by posterior tonsil-

lar pillars, including the palatopharyngeus muscle,

which are raised and sutured end to end.81

Correction of oronasal fistulae

One of the complications of primary cleft palate repair

is failure of healing or breakdown of wound resulting

in oronasal fistulae. Fistulae can occur at any location

in the hard or soft palate. The reports on incidence of

fistula formation are variable and range from 2–43%.

Incidence of oronasal fistulae depends on several

variables, including experience of the surgeon and

age at the time of repair; it is less often related to the

type of repair and severity of cleft deformity. The

most significant variable seems to be the experience

of the surgeon.77,82,83

Repair of the fistula varies according to its ana-

tomical location, whether it is in the anterior or poste-

rior part of the palate. Various local pedicled flaps

(b)

(a)

Fig. 45.16 (a) The Von Langenbeck technique of palatoplasty is generally used for clefts of the secondary palate. (b) Schematic dia-

gram of Furlow Z-plasty technique, showing the reverse opposing double Z-plasty of muscle and mucosa.

Cleft Lip and Palate: An Overview 17

have been described to close the defect. Posteriorly or

anteriorly based tongue flaps, musculo-mucosal flaps

based on the facial artery, a temporalis flap, or local

palatal mucoperiosteal flaps can be used to close fis-

tulae. More recently, interpositional grafts made of

acellular dermis have been used to achieve tension-

free closure of oronasal fistulae.84 It is important to

have at least a two-layered closure without tension

and maintain good supply to the flap in order to

achieve a watertight seal of the defect without recur-

rence. Almost all of the fistulae can be repaired by

local pedicled flaps as described above. However,

occasionally a vascularized flap may be helpful in

closure of a scarred palate with a large defect.

Orthodontic management of the

cleft individual

The orthodontist plays an important role in the care

of the cleft individual during infancy, mixed denti-

tion and permanent dentition (Table 45.3). Presurgical

orthopedic treatment is facilitated by the orthodontist

at 2–10 weeks after birth in infants with wide clefts

and poorly aligned alveolar segments or a protruding

premaxilla. In the early mixed dentition phase, chil-

dren with complete clefts of lip and palate often have

a posterior and anterior crossbite. The crossbite is

asymmetric in unilateral clefts, affecting mainly the

lesser segment or cleft side. In bilateral clefts, there is

collapse of both lateral segments with a bilateral pos-

terior crossbite and protrusion of the premaxilla. The

goal of orthodontic treatment in this phase is to pre-

pare for repair of the alveolar cleft by expanding the

maxillary alveolar segments, and correcting the posi-

tion of rotated maxillary incisors. The use of a quad

helix or a screw expansion device allows greater

expansion in the anterior maxillary arch (Fig. 45.17).

Monitoring facial growth during childhood and

early adolescence helps to identify early signs of max-

illary hypoplasia and allows intervention if indicated.

Maxillary growth may be restricted in the vertical,

transverse, and antero-posterior dimensions in chil-

dren with complete CLP. These children may benefit

from maxillary protraction using a reverse-pull head-

gear or early maxillary osteotomy and distraction

osteogenesis to minimize the severity of deformity.85

The typical orthodontic treatment for a cleft patient

following the eruption of the permanent teeth con-

sists of maintaining maxillary arch width after repair

of alveolar cleft, and alignment of teeth with full fixed

appliances. This should be timed appropriately based

on need for surgery, the individual's growth poten-

tial, and ability to cooperate and maintain oral

hygiene. Extraction of teeth may be necessary, partic-

ularly in the mandibular arch, if there is arch length

deficiency. When surgery is indicated orthodontic

treatment is coordinated with timing of growth com-

pletion, which is around age 15–16 years for females

and 17–19 years for males. It is important to integrate

the plan for the replacement or substitution of the

absent maxillary lateral incisor and any other missing

teeth into the orthodontic treatment plan.86

Alveolar cleft repair

The primary goal of alveolar cleft repair is to establish

bony continuity of the maxillary alveolar ridge, pro-

vide bone support for the teeth adjacent to the cleft,

and seal the communication between the nose and

oral cavity when there is a patent oronasal fistula. A

successful alveolar bone graft should facilitate erup-

tion and orthodontic movement of teeth in the line of

the cleft (most often the maxillary canine), maintain

the health of the periodontium of teeth adjacent to the

cleft, provide alar base support, and improve nasal

symmetry.87–89 Gingivoperiosteoplasty performed at

Table 45.3 Orthodontic management in cleft individuals

Age Treatment

2–10 weeks Presurgical orthopedics

6–10 years Phase I orthodontics:

Maxillary expansion for alveolar bone grafting

Maxillary protraction with face mask when

indicated

10–14 years Maintain maxillary expansion and alignment of

teeth

Monitor facial growth and eruption of

permanent teeth

Orthodontic treatment with distraction of

maxilla if deficiency is severe

14–18 years Phase II orthodontics:

Orthodontic treatment with full fixed appliances

to align teeth

Prepare for orthognathic surgery when

indicated

Extract teeth if arch is crowded

Decide whether to replace/substitute the absent

maxillary lateral incisor

Fig. 45.17 Orthodontic expansion in a bilateral cleft of lip and

palate. (a) Collapsed alveolar cleft segments with decreased

anterior maxillary arch width. (b) This maxillary expansion

device allows greater increase in arch width in the anterior

region more than the posterior region. (c) Maxillary width being

maintained after expansion.

(a) (b)

(c)

18 Dentofacial Deformities

the time of primary lip repair may seal the oronasal

communication but does not always preclude the

need for a bone graft. Some studies show that at least

40–50% of these patients require bone grafting in the

future.90–92 More recently, Meazzini et al. have shown

that patients who had a gingivoperiosteoplasty at the

time of primary repair did not require bone grafting

at a later date, however, they report a greater need for

surgical correction of maxillary hypoplasia.93

The timing, source of bone, the surgical technique,

perioperative management, and outcome of bone

grafting have all be studied and debated. The terms

primary bone grafting (2–5 years of age) secondary

bone grafting (7–11 years of age), and late secondary

bone grafting (14–18 years of age) have been used to

define the time of alveolar cleft repair.94,95 The major-

ity of the centers use secondary alveolar bone grafting

in the mixed dentition phase between the ages of 7

and 11 years, after maxillary expansion. Typically,

this coincides with one hals to two thirds of root

development of the maxillary canine or lateral incisor

(if present) in the line of the cleft. Completion of the

maxillary expansion prior to grafting provides ade-

quate access to the cleft defect and aligns the cleft

segments better.

The gold standard for the grafting material has

been particulate corticocancellous bone marrow

harvested from the Iliac crest, which was first

described by Boyne and Sands.96 Other sites that have

been described include rib, symphysis, calvarium,

and tibia.97 More recently, the use of bone growth

factors, such as recombinant human bone morpho-

genic protein-2 (rhBMP-2), has been shown to be

effective in grafting the defect. BMP-2 is an attractive

bone substitute that promotes differentiation of pluri-

potential cells into cells that can form new bone in the

defect. This eliminates donor site morbidity and mini-

mizes hospital stay and postoperative pain and

discomfort.98-100

New imaging techniques, such as cone-beam com-

puted tomography, allow more accurate assessment

of the volume of the bony defect before surgery and

radiographic outcome of the grafted alveolus after

surgery when compared to the conventional two-

dimensional periapical and panoramic radiographs

used previously.

The principles of surgical repair are the same for

unilateral and bilateral clefts and include: proper clo-

sure of nasal floor mucosa to seal the communication

between the nose and oral cavity; removal of super-

numerary teeth in the cleft defect; filling the defect

with cancellous bone; and approximation of the oral

mucosa on the labial and palatal aspects to achieve a

watertight closure over the grafted bone, as illustrated

in Fig. 45.18. In the bilateral cleft, the position of the

premaxilla makes the repair more technically chal-

lenging. The premaxilla is often inferiorly positioned,

and retruded with the incisors located below the level

of the occlusal plane. In some instances, the position

of the premaxilla can be corrected by presurgical

orthodontics, however in others it may have to be

corrected by performing a vomerine osteotomy at the

time of surgical alveolar cleft repair. The osteoto-

(a)

Fig. 45.18 Alveolar cleft repair. (a) Schematic diagram of the

alveolar cleft showing closure of the nasal and oral mucosa.

(b) The alveolar cleft deformity is visible and nasal mucosa is

closed to seal the communication between the oral and nasal

cavities. (c) Cancellous bone is packed into the defect after clo-

sure of the palatal oral mucosa.

(a)

(b)

(c)

Cleft Lip and Palate: An Overview 19

mized premaxilla has to be maintained in position

with a splint for 4–6 weeks postoperatively.101,102

The amount of maxillary arch expansion prior to

surgery should be planned and monitored. Too much

presurgical expansion of the lateral segments leaves a

large palatal defect making it difficult to advance the

scarred palatal mucoperiosteal flaps to cover the bone

graft, and too little expansion limits surgical access to

the alveolar cleft. After alveolar cleft repair, orth-

odontic tooth movement, if necessary, can be initiated

in 3–4 months. The maxillary width achieved by pre-

surgical expansion should be maintained after sur-

gery with a palatal retainer until phase II orthodontic

treatment begins at a later date.

Replacement of absent teeth in

the line of the cleft

Congenital absence of the maxillary lateral incisor in

the line of the cleft can be managed appropriately by

closing the space and substituting the adjacent canine

in its position or by opening space and replacing it

with a fixed or removable prosthesis. The experience

at the center for cleft and craniofacial anomalies at

the University of California has shown success of

implants in grafted unilateral clefts is better than in

bilateral clefts. In the bilateral cases the periodontal

health of the central incisors is very often compro-

mised, and the lateral incisors are also absent. The

substitution of the lateral incisor with the adjacent

canine is cost effective; however, it can result in loss

of arch length and decrease in transverse dimension

of the maxillary arch. It can also be unesthetic due to

the asymmetry in tooth size and shape in unilateral

cases. Replacement with an endosseous implant is an

option when there is adequate space and bone quan-

tity. There may be inadequate bone at the time of

placement of the implant even if the alveolar cleft was

repaired previously.103 Alveolar ridge augmentation

with a cortical onlay graft performed about 4 months

prior to placement of the implant will provide ade-

quate bone height and width for placement of the

implant (Fig. 45.19). A removable prosthesis should

also be considered when there is loss of premaxilla,

severe deficiency of bone height and width with scar-

ring of the overlying soft tissues tissue, and lack of lip

support. Use of teeth-supported fixed prosthetic res-

torations, such as a bridge, across the unrepaired

alveolar cleft segments should be avoided, as move-

ment of the cleft segments results in failure of the

prosthesis and loss of abutment teeth.

Surgical correction of maxillary

hypoplasia

Cleft lip and palate patients exhibit varying degrees

of maxillary hypoplasia due to restriction of midfacial

growth that is apparent in the sagittal, vertical, and

transverse dimensions. This midface deficiency can

be attributed partly to the intrinsic reduction in

growth potential due to the congenital malformation

and partly as a result of scar contracture following

primary palate surgery.104–106 The maxillary deficien-

cy caused by the cleft deformity is superimposed

upon the genetically inherited skeletal growth pat-

tern. Hence, an underlying inherited pattern of exces-

sive mandibular growth can present as severe

midfacial deficiency and a class III malocclusion in a

cleft patient (Fig. 45.20). Regardless of the etiology of

maxillary hypoplasia, approximately 25% (reported

range 14–50%) of cleft individuals undergo surgical

correction of maxillary hypoplasia.107–110 This wide

range highlights the differing indications for skeletal

correction by cleft team specialists. Their decisions

are influenced by differing treatment philosophies

(orthodontic management with dental compensation

vs. surgical maxillary advancement to address facial

esthetics and occlusion more comprehensively), the

availability of appropriately trained surgeons, and

hospital/government funding. Good et al. reported

Fig. 45.19 Replacement of missing lateral incisor. (a) Alveolar ridge augmentation with a cortical bone onlay is often necessary to

increase width and height, before placement of an implant in the grafted cleft site. (b) Unilateral cleft with a missing lateral incisor

replaced with an endosseous implant.

(a)

(b)

20 Dentofacial Deformities

that the frequency of a Le Fort I osteotomy for correc-

tion of maxillary hypoplasia correlated with the

severity of clefting.109

Technical considerations for cleft

orthognathic surgery

Surgical correction of maxillary hypoplasia is usually

performed after growth is completed, unless indicat-

ed earlier for psychological reasons. When the skeletal

discrepancy is severe, a combined maxillary advance-

ment and mandibular reduction can be performed as

seen in Fig. 45.21. A staged advancement of the max-

illa can be considered, particularly when the mandible

is in a normal position. Early surgical maxillary

advancement before the completion of growth will

help to minimize the reverse overjet and potentially

avoid or decrease the magnitude of mandibular

reduction at a later date. An example of staged maxil-

lary advancement is shown in Fig. 45.22. In the last

decade, distraction osteogenesis of the facial skeleton

has become popular and facilitated substantial maxil-

lary advancement without the need for mandibular

osteotomies and bone grafting.111

Orthognathic surgery in the cleft patient is much

more challenging than in the non-cleft patient.

Anesthetic management may sometimes be difficult

due to the presence of a deviated nasal septum and/

or a pharyngeal flap. It may be necessary to use a

fiberoptic-assisted technique or pass the endotracheal

tube over a more rigid tube or catheter. The vascular-

ity of the labial and palatal mucoperiosteal tissues is

invariably affected by previous surgical procedures

for lip and palate repair. Drommer and Luhr demon-

strated with the use of angiography that the greater

palatine arteries were significantly smaller in 10 of 24

sides in 12 cleft patients prior to maxillary advance-

ment.112 When making mucosal incisions to provide

access for the osteotomies, care should be taken to

maintain a generous buccal pedicle, preserve the

greater palatine arteries if possible, and to avoid

unnecessary trauma to the palatal and buccal soft tis-

sue pedicles. Failure to do so may result in the loss of

attached gingival tissues, bone and teeth.

The nasal mucosa and palatal mucosa are fused in

the region of the cleft due to the primary palate repair.

This tissue should be incised close to the nasal floor

just prior to the "down-fracture". Attenuation of scar

tissue close to the nasal floor is necessary to free the

maxilla during the down-fracture. Mobilization of

the maxilla after down-fracture is more difficult due

to the palatal scar tissue and/or a pharyngeal flap. In

some cases, release or division of the pharyngeal flap

may be indicated to reposition the maxilla into the

desired position. The primary palatal repair often

results in more bone formation and stronger union at

Fig. 45.20 Maxillary growth in a child with a unilateral cleft lip and palate. (a) Note the signs of maxillary deficiency are apparent in

the profile view at 9 years in this child with a unilateral cleft lip and palate. (b) The severe deficiency of the maxilla in anteroposterior

and vertical dimensions and its relative position to the mandible are seen at 15 years of age.

(a) (b)

Cleft Lip and Palate: An Overview 21

the pterygo-maxillary junction. Use of a curved chisel

to osteotomize directly through the maxillary tuber-

osities rather than the dense bone of the ptergo-

maxillary junction is helpful to complete this posterior

osteotomy. Complete mobilization to achieve suffi-

cient advancement requires progressive, careful

stretching as the tissues are less compliant than nor-

mal tissues.

The maxillary deficiency in the vertical, transverse,

and sagittal dimensions makes bone grafting a neces-

sity for cleft maxillary osteotomies performed by

conventional orthognathic surgery. A persistent oro-

nasal fistula and alveolar cleft defect, when present,

require careful soft tissue closure and bone grafting.

The maxilla can separate into two segments during

the down-fracture and mobilization even when the

Fig. 45.21 Cleft orthognathic surgery. An 18-year-old female with secondary skeletal

and soft tissue deformities of bilateral CLP corrected with maxillary and mandibular

osteotomies, and cheilo-rhinoplasty. (a) Before and (b) after in frontal view. (c) Before

and (d) after in profile view. (e) Preoperative occlusion reveals anterior and posterior

crossbite. (f) Postoperative occlusion after combined orthodontic and surgical

correction.

Fig. 45.22 Cleft orthognathic surgery. Radiographs of 18 year-old male with unilateral CLP and severe maxillary hypoplasia cor-

rected by staged orthognathic surgery. (a) Preoperative lateral cephalometric radiograph. (b) Postoperative view after initial maxil-

lary advancement. (c) Postoperative view after maxillary advancement and mandibular setback.

(a) (b) (c) (d)

(e) (f) (g)

(h)

(a) (b) (c)

22 Dentofacial Deformities

(a)

Fig. 45.23 (a) A schematic diagram illustrating a Le Fort I osteotomy with simultaneous repair of the unilateral alveolar cleft defect.

(b) Corticocancellous bone harvested from the anterior iliac crest can be used to graft the cleft site and osteotomy gap created by

advancement and inferior repositioning. (c) Profile view before maxillary advancement and alveolar cleft repair. (d) Profile view

after maxillary advancement and alveolar cleft repair. (e) Frontal view shows mild deficiency of midface with poor alar base support.

(f) Frontal view in smile shows better midface projection.

(a) (b)

(e) (f)

Cleft Lip and Palate: An Overview 23

alveolar cleft site has been previously grafted.

Corticocancellous bone harvested from the anterior

iliac crest can be used to graft the residual alveolar

cleft defect, to bridge the gap at the osteotomy site

after anterior and inferior repositioning of the maxilla,

and to augment the deficient area at the piriform rim

on the cleft side (Fig. 45.23).

Rigid fixation with plates and screws has to be

performed carefully as the quantity and quality of

bone is often compromised. When there is deficiency

of the midface and malar region, adjunctive proce-

dures, such as zygomatic osteotomies or a modified

Le Fort I osteotomy, can enhance cheek prominence

and facial contours (Fig. 45.24).

The soft tissue of the upper lip may be tight with a

shallow vestibular depth and deficient vermillion

show that may become worse following maxillary

advancement. When closing the incision, incorporat-

ing a V–Y design to minimize lip shortening should

be considered. Alar flaring is a common problem in

the distorted cleft nose following a large maxillary

advancement. An alar base suture passed in a figure-

of-eight fashion through the anterior nasal spine will

help to maintain a satisfactory alar base width.

VPI after maxillary advancement has been well

documented.113,114 The predictability of VPI after con-

ventional orthognathic surgery is based on premorbid

speech. If there is moderate to severe insufficiency

prior to maxillary advancement it is more likely that

individuals will need postsurgical pharyngeal flap or

pharyngoplasty. It is therefore important that these

patients are thoroughly assessed for VPI prior to cleft

maxillary surgery.115

Conventional orthognathic surgery

vs. distraction osteogenesis

Postoperative stability after maxillary osteotomy is

less favorable in the cleft than in the non-cleft patient.

Relapse in the cleft individual is mainly due to the

inability to mobilize the maxilla adequately. The tight

scarred soft tissue envelope of the palate and upper

lip limit the ability to mobilize and achieve the desired

movement. Despite bone grafting and rigid internal

fixation, relapse in the sagittal and vertical dimen-

sions after maxillary osteotomy has been reported in

several studies.116–121 Distraction osteogenesis can

overcome some of the difficulties encountered with

mobilization and stability in conventional orthogna-

thic surgery: it is the technique of gradually reposi-

tioning the osteotomized bone segments while

forming new bone in the gap created by the osteoto-

my. Distraction osteogenesis is technique sensitive,

costly, and requires patient cooperation and surgical

expertise to properly align, place, and activate the

device in order to achieve a good outcome.

Polley and Figueroa first described the use of a

rigid external frame to distract the maxilla in a series

of cleft individuals. They demonstrated a mean

advancement of 11.7 mm.122 Since then, several

centers have reported their experiences in maxillary

distraction in cleft individuals using face masks to

provide the external anchorage123,124 and internal dis-

tractors.125 A meta-analysis of literature from 1996–

2003 by Cheung et al. on cleft maxillary osteotomy

and distraction osteogenesis showed that majority of

patients who underwent conventional maxillary

osteotomies were older (16–20 years) compared to

those who had distraction (11–15 years). The mean

advancement for both groups was similar but larger

maximum advancement was achieved in the distrac-

tion group. The most commonly used system was the

rigid external distractor (RED) device.126 They subse-

quently conducted a randomized, controlled study

comparing maxillary distraction and orthognathic

surgery in 29 non-growing, cleft patients. Intraoral

distractors were used in 15 patients in the distraction

group and routine miniplate fixation for the 14

patients in the orthognathic surgery group. Clinical

(a)

(b)

Fig. 45.24 (a, b) Midface osteotomy including the zygoma and

infraorbital rim for correction of severe midface deficiency.

24 Dentofacial Deformities

morbidity and stability were assessed using a ques-

tionnaire and lateral cephalometric tracings respec-

tively. It was found that there was no significant

difference in the clinical morbidities but the maxillary

movement in the distraction group was more stable

than in orthognathic repositioning group. Skeletal

relapse was evident in the first 3 months following

conventional cleft maxillary advancement.127

Improved stability and speech, and marked improve-

ment in hard and soft tissue profile were also reported

for distraction by other investigators.128–131 In a 2-year

follow-up of 12 cleft patients with moderate to severe

maxillary retrusion who underwent distraction with

internal distractors, a mean maxillary advancement

of 14 mm with good stability was reported by

Rachmiel et al.125 At the Royal Children's Hospital of

Melbourne internal distractors have been used over

the past 10 years in select cases. Unlike external

distractors, the internal distractors lack three-

dimensional control and particularly the ability to

manage the vertical vector. Other disadvantages of

internal distraction include a limitation of distractor

length and the need for a separate procedure for

removal. External frames are bulky and uncomfort-

able, but provide better vector control and changes

can be made to the vector during distraction. Also

there is no need for a separate procedure to remove

the distractor (Fig. 45.25).

For severe maxillary hypoplasia in the cleft patient,

distraction is considered an option as an interim pro-

cedure during growth. The aim is to minimize or

eliminate the need for mandibular reduction where

the mandible is within normal dimensions and rela-

tionship to the facial structure. Distraction obviates

the need for internal fixation and bone grafting but

disadvantages include prolonged facial edema and

the occasional inflammatory response to the trans-

mucosal presence of the device activation arms.

Traditional staged maxillary advancements remain a

predictable option for patients, but the ability to pro-

duce large advancements using distraction is attrac-

tive in those with marked retrusion. Fig. 45.26

illustrates correction of severe maxillary hypoplasia

with distraction. As more clinical studies evaluate the

results of midfacial distraction techniques in compari-

son with conventional osteotomies, it is hoped that

protocols can be developed to reflect the best timing

and indication for each technique.

Surgical correction of secondary

lip and nose deformities

The final phase of treatment for the cleft patient

involves correction of the residual lip and nasal

deformities to achieve balanced facial esthetics.

Typically, the lip and nose revision is performed dur-

ing late adolescence after skeletal correction of maxil-

lary hypoplasia, and after postsurgical orthodontic

treatment is completed to provide a stable dental

occlusion.

Secondary lip deformities

Secondary lip deformities that require correction are

mainly asymmetries or disproportions. For the major-

ity of patients where the primary repair was carefully

planned and executed, secondary lip revisions are

minor. In unilateral cleft lip patients, the residual

deformities include mismatch of the cutaneous–

vermillion line, notching of the vermillion or a

"whistle deformity", vertical shortening of the lateral

lip element, a hypertrophic scar, and poor muscle

function due to a discontinuity of the orbicularis

oris.132

Notching or mismatch at the vermillion– cutaneous

junction can be corrected by realignment, small trian-

gular flaps or a Z-plasty procedure. A poorly defined

tubercle can be corrected by a V–Y advancement. It is

important to differentiate and maintain the zone of

wet and dry mucosa when correcting these deformi-

ties. Vertical shortening of the lip after a Millard

repair is due to underestimation of the vertical height

and inadequate rotation of lip on the non-cleft side at

the time of primary repair. Shortening of the lip can

also result from severe scar contracture. Correction of

a prominent scar with inadequate lip length and com-

promised muscle function requires a full-thickness

revision where all three layers (skin, muscle, and

mucosa) have to be cleanly dissected and meticulous-

ly repaired (Fig. 45.27).

In the case of a poorly repaired bilateral cleft lip,

the deformity can present as a tight upper lip with

poorly defined philtral columns and cupid's bow, a

short and wide prolabium, poor vermillion show in

the center, unsightly scars, exposed wet mucosa, and

a shallow labial sulcus. Deficiency of the labial sulcus

can be corrected by releasing the scar and performing

a Z-plasty. In the case of a tight upper lip, an Abbe

flap can be used successfully for reconstruction of the

upper lip deformity. This is an axial pattern flap con-

sisting of vermillion, mucosa, skin, and muscle from

the lower lip described by Abbe in 1898. The advan-

Fig. 45.25 (a) An internal maxillary distractor (KLS Martin) in

place secured above and below the Le Fort I osteotomy. (b) A

rigid external distractor (RED) provides skeletal anchorage

with the head frame in a young female with maxillary

hypoplasia.

(a) (b)

Cleft Lip and Palate: An Overview 25

Figure 45.26 Maxillary advancement with internal distraction. (a) Preoperative profile view of 19-year-old male with severe maxil-

lary deficiency and large reverse overjet. (b) Lateral cephalometric radiographic after osteotomy and placement of distractor.

(c) Postoperative radiograph 6 months after removal of distractors showing some relapse. (d) Postoperative facial profile view shows

improvement in midface projection and nasal tip support. (e) Bone generated at the osteotomy site by maxillary distraction

(arrow).

(a) (b)

tages of this flap are that it carries the dimple from

the lower lip to form a philtral dimple in the cupid's

bow and the scars give the semblance of philtral

columns.

Cleft nasal deformity

Asymmetry and distortion of the nasal morphology

is often the most obvious feature of an individual

with a cleft. Correction of the nasal deformity in cleft

individuals is a challenge due to soft tissue distor-

tions, scarring, nasal stenosis, and uneven bony foun-

dations. Primary correction of the cleft nose deformity

is performed by most surgeons today. This does not

eliminate the need for a secondary rhinoplasty, but,

better results can be achieved as the deformity may

be less severe.133 The pathological anatomy in the

unilateral cleft nose is an asymmetric nasal pyramid,

hypoplastic maxilla on the cleft side, and hypertro-

phied inferior turbinate with deviation of the anterior

nasal spine and base of columella to the non-cleft

side. There is inadequate nasal tip support, posterior

and inferior displacement of the dome of the alar car-

tilage, and inward buckling of the ala on the cleft

side.134 The bilateral cleft nose is less asymmetrical

and the predominant deformity is that of a short colu-

mella. Other features of the nasal deformity include a

broad downward-rotated nasal tip that lacks support,

lower lateral cartilage domes that are widely separat-

ed, and lateral splaying of the alar rims with nostril

apertures more horizontally oriented.

Cleft septorhinoplasty

There is a wide variation in timing for secondary

nasal revisions, with some authors recommending

26 Dentofacial Deformities

Fig. 45.27 (a) Whistle notch deformity of a unilateral repaired cleft lip. (b) Lip contour following naso-labial revision.

(a) (b)

Fig. 45.28 (a, b) Preoperative nasal deformity in frontal and inferior facial views showing collapsed alar cartilage with poor tip sup-

port. (c, d) Postoperative frontal and inferior views facial views following septorhinoplasty show improved tip support and

symmetry.

(a) (b)

Cleft Lip and Palate: An Overview 27

early rhinoplasty. Unless there is a particularly strong

psychological indication or severe nasal obstruction,

definite secondary septorhinoplasty in the cleft

patient should follow skeletal maxillary reconstruc-

tion. The total correction of the cleft nasal deformity

is best approached by an open rhinoplasty under

direct vision. Wide exposure provides access for

mobilization and repair of the collapsed and deformed

alar cartilages.135,136 In unilateral cleft patients, an

open rhinoplasty approach is performed using a

trans-columellar incision combined with rim inci-

sions. Complete degloving of the lower lateral carti-

lages and dissection to the cartilaginous septum

enables a full septoplasty with relocation of the

caudal septum and the harvesting of cartilage grafts

if indicated. Dorsal reduction is commonly undertak-

en with bony osteotomies to restore the bony vault.

Additionally, stiffening the columella by a cartilage

strut is necessary to provide tip projection and sup-

port for a more symmetrical nasal tip. Reshaping the

deficient lateral crus may require a batten graft, and

alar base repositioning may be required to improve

nostril symmetry (Fig. 45.28). In bilateral clefts, vari-

ous approaches, including a V–Y advancement to

lengthen the columella, have been undertaken, but

undesirable scarring is often the outcome. The entire

prolabium can be used to lengthen the columella and

an Abbe flap can be used to fill the upper lip defect.137

The goal is to stretch the nasal tip and to dissect and

approximate the lower lateral cartilages to achieve a

more triangular inferior nasal shape.

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30 Dentofacial Deformities

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... The cause of these orofacial clefts remains largely unknown [1]. They represent the most common congenital deformities of the orofacial region worldwide [2]. ...

... The embryological formation of the tissues that comprise the craniofacial system is partly derived from the neural crest, which can consequently affect the development of the brain, ocular, and facial structures that develop from this neural crest [2]. Therefore, effects on speech, hearing, vision, appearance, and cognition can lead to long-lasting adverse effects on health and social integration. ...

Camille Catarina Artuso
Ana Kelly Fernandes
Luisa Moreira Hopker
Luis Ricardo Antunes de Castro

Purpose: To evaluate the prevalence of refractive errors and strabismus in patients with orofacial clefts. Methods: This retrospective study analyzed the medical records of 54 patients with orofacial clefts between August 2018 and March 2020. A complete eye examination was performed, including visual acuity assessment on a logMAR scale, anterior biomicroscopy, cycloplegic refraction, eye motility examination, and indirect ophthalmoscopy. Results: The mean age of the patients at presentation was 9.47 years. Twentythree (42.59%) patients had isolated cleft palate (CP), 10 (18.52%) had cleft lip (CL), and 21 (38.89%) had cleft lip and palate (CLP). The mean spherical equivalent was 1.30D (±1.56) in CL, 0.32D (±2.24) in CLP, and 0.62D (±3.76) in CP. The prevalence of refractive error, either spherical or cylinder >0.5 was 88%. The most common refractive error was hyperopia (60%), followed by astigmatism (54%) and myopia (16%). Overall, 52.63% of the patients were prescribed glasses. No statistically significant difference was observed between the groups with respect to the need for prescription of glasses (p=0.6753). There were 15 patients with some type of strabismus, and other ophthalmological changes were observed in 13 patients. Conclusion: In this population with orofacial clefts, the prevalence of refractive errors and strabismus was 88% and 22%, respectively.

... However, the severity of the final skeletal discrepancy depends not only on the hypoplasia intrinsic to the deformity and the iatrogenic effect of cleft palate repair on subsequent growth of the midface, but also on the underlying pattern of overall facial growth. 2,3 For CLP patients with maxillary hypoplasia and for whom orthognathic surgery is indicated, the basic procedure for surgical correction is the conventional Le Fort I maxillary osteotomy, although gradual lengthening of the maxilla by distraction osteogenesis may be considered if the skeletal discrepancy is severe. ...

... In these situations, the option of maxillary distraction osteogenesis or a staged approach to advance the maxilla may be preferable. 3 In cleft patients, vertical relapse after Le Fort I maxillary osteotomies is much more difficult to control than relapse in the horizontal plane, with most patients experiencing vertical change of 21-25% compared to horizontal change of 6-10% in the first 6 months postoperatively. 26 Inferior repositioning, in particular, has historically been considered one of the most unstable maxillary movements, and the amount of vertical maxillary relapse that has been reported in the cleft literature varies from 0% to almost 70%, representing up to 2.3 mm of vertical change on average. ...

The stability of surgical maxillary advancement in a consecutive series of patients with cleft lip and palate who underwent Le Fort I osteotomy with and without simultaneous mandibular setback surgery was evaluated. Preoperative, postoperative, and follow-up lateral cephalograms of 21 patients were assessed to compare differences in surgical movement and postoperative relapse between two groups: those who underwent maxillary surgery alone and those who underwent bimaxillary surgery. Differences in the number of patients who experienced relapse of <2mm, 2-4mm, and >4mm between the groups were also compared. Mean advancement of the cleft maxilla was 5.5mm in the maxilla only group and 3.6mm in the bimaxillary group, with a mean horizontal relapse of 0.8mm and 0.2mm, respectively. Mean surgical movement in the vertical dimension was comparable in the two groups and the magnitude of vertical relapse was less than 0.4mm overall. Approximately 80% of patients in both groups experienced horizontal relapse of less than 2mm. There was no significant difference in the degree of postoperative relapse between those who had single-jaw surgery and those who had two-jaw surgery.

... At first glance, it may seem a simple process; however, the formation of the facial structures is a complex cascade of events during which cells proliferate, differentiate, adhere, and go through apoptosis. An error during this intricate process can result in orofacial clefts or other abnormalities [1,2]. One of the most common congenital anomalies worldwide is the orofacial cleft. ...

Jana Goida
Mara Pilmane

Orofacial clefts are one of the most common congenital anomalies worldwide; however, morphopathogenesis of the clefts is not yet completely understood. Taking the importance of innate immunity into account, the aim of this work was to examine the appearance and distribution of macrophages (M) 1, M2, and TNF-α, as well as to deduce any possible intercorrelations between the three factors in cleft affected lip tissue samples. Twenty samples of soft tissue were collected from children during plastic surgery. Fourteen control tissue samples were obtained during labial frenectomy. Tissues were immunohistochemically stained, analysed by light microscopy using a semi-quantitative method, and the Mann–Whitney U and Spearman's tests were used to evaluate statistical differences and correlations. A statistically significant difference in the distribution was observed only in regard to M1. A weak correlation was observed between M2 and TNF-α but a moderate one between M1 and M2 as well as M1 and TNF-α. However, only the correlation between M1 and M2 was statistically important. The rise in M1, alongside the positive correlation between M1 and TNF-α, suggested a more pro-inflammatory/inflammatory environment in the cleft affected lip tissue. The moderate positive correlation between M1 and M2 indicated an intensification of the protective mechanisms.

Reza Tabrizi
Barbad Zamiri
Hosein Daneste
Hamidreza Arabion

The aim of this study is to evaluate outcome of bone availability after the secondary alveolar bone graft in 2 age groups: group 1 patients were between 9 and 13 years old and group 2 patients were above 14 years old. Acceptance success criteria (ASC) consisted of sufficient bone height (more than 10 mm), bone width (more than 4 mm), and adequate continuity between maxillary segments. The height and width of alveolar grafted bone were measured by using the cone-beam CT scans. We studied 45 patients who underwent a bone graft in their alveolar cleft in 2 groups (25 patients in group 1 and 20 in group 2). The results showed that as the patients' ages increased, the incidence of ASC significantly decreased. In group 1, 23 patients had ACS (92%), and in group 2, only 4 patients (20%) had ASC. Cleft type did not affect the ASC. The critical age for decreasing ASC was 14.5 years. Our study showed successful outcomes of grafted bone were good when done in the mixed dentition period. Additionally, bone availability was more predictable at the mixed dentition stage.

Karin Källén

To investigate a possible association between maternal smoking during pregnancy and oral clefts, a study was conducted using Swedish health registries. Infants with oral clefts (N = 1834) were selected among 1,002,742 infants born between 1983 and 1992 with known smoking exposure in early pregnancy. Confounders such as maternal age and parity were controlled for by using the Mantel-Haenszel technique. A statistically significant association with maternal smoking was found. The odds ratio (OR) for any maternal smoking among cases of cleft lip with or without cleft palate [CL(P)] was 1.16 (95%CI: 1.02–1.32). For cases of cleft palate alone (CP), the corresponding OR was 1.29 (95%CI: 1.08–1.54). The results of the present study, based on the largest series of oral cleft cases published to date, indicate that cigarette smoking during pregnancy is associated with increased risks of CL(P) and CP.

Christian Vacher
B Pavy
Jeffrey Ascherman

Objective Hypoptasia of the maxilla, often described as a classic sequela to surgical repair of the cleft palate, has been rare In our experience. We believe that our surgical technique, which includes dividing the nasal mucosa and the abnormal muscular insertions at the posterior border of the hard palate, is an important factor in preventing this sequela. Method We compared the anatomy of 12 normal palates in cadavers to the anatomy of cleft palates seen at operation and to the anatomy of one cleft palate in a fetus aged 34 weeks. Results In cleft palates, the muscular fibers have an abnormal sagittal orientation, inserting on the posterior border of the hard palate. Conclusion The division of both the nasal mucosa and these abnormal muscular insertions at the posterior border of the hard palate enables the surgeon to eliminate the abnormal posterior pull of these fibers on the maxilla.

Jeffrey Posnick
Michael Taylor

We present a series of 14 consecutive isolated cleft palate patients aged 17 to 25 years (mean 19 years) who underwent Le Fort 1 maxillary advancement fixed with miniplates by the senior author (Posnick) over the period 1987-1991. Ten of the patients underwent autogenous bone grafting; all were stabilized intraoperatively with four miniplates, a prefabricated acrylic splint, and intermaxillary fixation. The patients were analyzed to determine amount and timing of horizontal and anterior and posterior vertical relapse, correlation between advancement and relapse, effect of a pharyngoplasty in place at time of osteotomy, effect of performing multiple jaw procedures, and maintenance of overjet and overbite. Tracings of preoperative and serial postoperative lateral cephalograms (immediate, 6 to 8 weeks, and 1 year) were analyzed to calculate horizontal and vertical maxillary changes and the amount of overjet and overbite maintained. Clinical follow-up ranged from 1.5 to 5.5 years (mean 2.5 years). No significant difference was seen in vertical or horizontal change or relapse between patients who had maxillary surgery alone (n = 10) and those who had operations in both jaws (n = 4), nor did outcome vary significantly for those who had a pharyngoplasty in place (n = 8) at the time of their Le Fort I osteotomy. The mean "effective" maxillary advancement for the group was 6.4 mm, with 5.4 mm maintained 1 year later (mean relapse 1.0 mm). The mean anterior vertical change of the maxilla was 2.0 mm, with 1.4 mm maintained after 1 year (mean relapse 0.6 mm), while the mean posterior vertical change was 2.7 mm, with 1.9 mm maintained 1 year later (mean relapse 0.8 mm). All patients maintained a positive overjet and overbite. The extent of skeletal relapse in this series fell within a range small enough that problems could be managed effectively through a combination of planned surgical overcorrection and orthodontic treatment. (C)1994American Society of Plastic Surgeons

Dean Deluke
Anick Marchand
Elizabeth C Robles
Patricia Fox

Purpose: Controversy still exists regarding the optimal timing and surgical technique for primary cleft lip and palate (CLP) repair, and treatment protocols vary considerably. This study reviews the literature on timing and technique for primary repair and reports on the outcome for a consecutive group of patients treated by a single surgical protocol at the Sunnyview Cleft Palate Clinic. Patients and methods: Twenty-eight patients treated by a standardized clinical protocol from infancy through adolescence were evaluated with respect to the need for orthognathic surgery to correct jaw size discrepancy. For each patient, data was collected regarding type of cleft deformity, total number of surgical procedures from infancy, surgeon performing the primary repair, and the need or indication for orthognathic surgery. Results: Twenty-five percent of patients treated by this protocol required orthognathic surgery because of anteroposterior jaw size discrepancy. The number of prior operations was not a significant factor. The need for orthognathic surgery was seen in all types of CLP deformity. Different primary surgeons varied considerably in the percentage of their patients who ultimately required orthognathic surgery. Conclusion: The results of this study parallel other larger cohort studies with respect to the percentage of patients requiring orthognathic surgery. The number of prior operations does not significantly affect the later need for orthognathic surgery.

Source: https://www.researchgate.net/publication/312125407_Cleft_Lip_and_Palate_An_Overview

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