Kliegman: Nelson Textbook of Pediatrics,

18th ed.
Copyright © 2007 Saunders, An Imprint of Elsevier

Chapter 307 – Cleft Lip and Palate

Clefts of the lip and palate are distinct entities closely related embryologically, functionally, and
genetically. Although there are a variety of theories, it is commonly thought that cleft of the lip
appears because of hypoplasia of the mesenchymal layer, resulting in a failure of the medial
nasal and maxillary processes to join. Cleft of the palate appears to represent failure of the
palatal shelves to approximate or fuse.

INCIDENCE AND EPIDEMIOLOGY.

The incidence of cleft lip with or without cleft palate is ≈1/750 white births; the incidence of
cleft palate alone is ≈1/2,500 white births. Clefts of the lip are more common in males. Possible
causes include maternal drug exposure, a syndrome-malformation complex, or genetic factors.
Although both may appear to occur sporadically, the presence of susceptibility genes appears
important. There are families in which a cleft lip or palate, or both, is inherited in a dominant
fashion (van der Woude syndrome), and careful examination of parents is important to
distinguish this type from others, because the recurrence risk is 50%. Ethnic factors also affect
the incidence of cleft lip and palate; the incidence is highest among Asians and Native
Americans, and lowest among blacks. The incidence of associated congenital malformations
(chromosomal aneuploidy, holoprosencephaly) and of impairment in development is increased in
children with cleft defects, especially in those with cleft palate alone. The risks of recurrence of
cleft defects within families are discussed in Chapters 80 and 83 .

CLINICAL MANIFESTATIONS.

Cleft lip may vary from a small notch in the vermilion border to a complete separation involving
skin, muscle, mucosa, tooth, and bone. Clefts may be unilateral (more often on the left side) or
bilateral and may involve the alveolar ridge. Deformed, supernumerary, or absent teeth are
associated findings.

Isolated cleft palate occurs in the midline and may involve only the uvula or may extend into or
through the soft and hard palates to the incisive foramen. When associated with cleft lip, the
defect may involve the midline of the soft palate and extend into the hard palate on one or both
sides, exposing one or both of the nasal cavities as a unilateral or bilateral cleft palate. The palate
may also present with a submucosal cleft indicated by a bifid uvula, partial separation of muscle
with intact mucosa, or a palpable notch at the posterior of the palate.

TREATMENT.
A complete program of habilitation for the child with a cleft lip or palate may require years of
special treatment by a team consisting of a pediatrician, plastic surgeon, otolaryngologist, oral
and maxillofacial surgeon, pediatric dentist, prosthodontist, orthodontist, speech therapist,
geneticist, medical social worker, psychologist, and public health nurse. The child's physician
should be responsible for seeking the coordinated use of specialists and for parental counseling
and guidance.

The immediate problem in an infant born with a cleft lip or palate is feeding. Although some
advocate the construction of a plastic obturator to assist in feedings, most believe that with the
use of soft artificial nipples with large openings, a squeezable bottle, and proper instruction,
feeding of infants with clefts can be achieved with relative ease and effectiveness.

Surgical closure of a cleft lip is usually performed by 3 mo of age, when the infant has shown
satisfactory weight gain and is free of any oral, respiratory, or systemic infection. Modification
of the Millard rotation-advancement technique is the most commonly used technique; a
staggered suture line minimizes notching of the lip from retraction of scar tissue. The initial
repair may be revised at 4 or 5 yr of age. Corrective surgery on the nose may be delayed until
adolescence. Nasal surgery can also be performed at the time of the lip repair. Cosmetic results
depend on the extent of the original deformity, healing potential of the individual, absence of
infection, and the skill of the surgeon.

Because clefts of the palate vary considerably in size, shape, and degree of deformity, the timing
of surgical correction should be individualized. Criteria such as width of the cleft, adequacy of
the existing palatal segments, morphology of the surrounding areas (width of the oropharynx),
and neuromuscular function of the soft palate and pharyngeal walls affect the decision. The goals
of surgery are the union of the cleft segments, intelligible and pleasant speech, reduction of nasal
regurgitation, and avoidance of injury to the growing maxilla.

In an otherwise healthy child, closure of the palate is usually done before 1 yr of age to enhance
normal speech development. When surgical correction is delayed beyond the 3rd yr, a contoured
speech bulb can be attached to the posterior of a maxillary denture so that contraction of the
pharyngeal and velopharyngeal muscles can bring tissues into contact with the bulb to
accomplish occlusion of the nasopharynx and help the child develop intelligible speech.

A cleft palate usually crosses the alveolar ridge and interferes with the formation of teeth in the
maxillary anterior region. Teeth in the cleft area may be displaced, malformed, or missing.
Missing teeth or teeth that are nonfunctional are replaced by prosthetic devices.

POSTOPERATIVE MANAGEMENT.

During the immediate postoperative period, special nursing care is essential. Gentle aspiration of
the nasopharynx minimizes the chances of the common complications of atelectasis or
pneumonia. The primary considerations in postoperative care are maintenance of a clean suture
line and avoidance of tension on the sutures. The infant is fed with a Mead Johnson bottle and
the arms are restrained with elbow cuffs. A fluid or semifluid diet is maintained for 3 wk;
feeding is continued with a Mead Johnson bottle or a cup. The patient's hands, toys, and other
foreign bodies must be kept away from the surgical site.
SEQUELAE OF CLEFT LIP AND PALATE.

Recurrent otitis media and hearing loss are frequent with cleft palate. Displacement of the
maxillary arches and malposition of the teeth usually require orthodontic correction.

Speech defects are often associated with cleft lip and palate and may be present or persist
because of inadequate surgical closure of the palate. Such speech is characterized by the
emission of air from the nose and by a hypernasal quality with certain sounds. Both before and
sometimes after palatal surgery, the speech defect is caused by inadequacies in function of the
palatal and pharyngeal muscles. The muscles of the soft palate and the lateral and posterior walls
of the nasopharynx constitute a valve that separates the nasopharynx from the oropharynx during
swallowing and in the production of certain sounds. If the valve does not function adequately, it
is difficult to build up enough pressure in the mouth to make such explosive sounds as p, b, d, t,
h, y, or the sibilants s, sh, and ch, and such words as “cats,” “boats,” and “sisters” are not
intelligible. After operation or the insertion of a speech appliance, speech therapy is necessary.

VELOPHARYNGEAL INCOMPETENCE.

The speech disturbance characteristic of the child with a cleft palate can also be produced by
other osseous or neuromuscular abnormalities where there is an inability to form an effective seal
between oropharynx and nasopharynx during swallowing or phonation. The abnormality may be
in the structure of the palate or pharynx or in the muscles attached to these structures. In a child
who has the potential for abnormal speech, adenoidectomy may precipitate overt hypernasality.
A submucous cleft palate may also cause this problem. In such cases, the adenoid mass may have
facilitated velopharyngeal closure when contacted by the elevated soft palate. If the
neuromuscular function is adequate, compensation in palatopharyngeal movement may take
place and the speech defect may improve, although speech therapy is necessary. In other cases,
slow involution of the adenoids may allow for gradual compensation in palatal and pharyngeal
muscular function. This may explain why a speech defect does not become apparent in some
children who have a submucous cleft palate or similar anomaly predisposing to palatopharyngeal
incompetence. Velopharyngeal incompetence (VPI) can also occur in children with an inherent
palatal abnormality (velocardiofacial syndrome). A craniofacial disorders team and a geneticist
should evaluate VPI.

Clinical Manifestation.

Although clinical signs vary, the symptoms of VPI are similar to those of a cleft palate. There
may be hypernasal speech (especially noted in the articulation of pressure consonants such as p,
b, d, t, h, v, f, and s); conspicuous constricting movement of the nares during speech; inability to
whistle, gargle, blow out a candle, or inflate a balloon; loss of liquid through the nose when
drinking with the head down; otitis media; and hearing loss. Oral inspection may reveal a cleft
palate or a relatively short palate with a large oropharynx; absent, grossly asymmetric, or
minimal muscular activity of the soft palate and pharynx during phonation or gagging; or a
submucous cleft. The latter is suggested by a bifid uvula, by a translucent membrane in the
midline of the soft palate (revealing lack of continuity of muscles), by palpable notching in the
posterior border of the hard palate instead of a posterior nasal spinous process, or by forward or
V-shaped displacement or grooving on the soft palate during phonation or gagging.
Velopharyngeal incompetence may also be demonstrated radiographically. The head should be
carefully positioned to obtain a true lateral view; one film is obtained with the patient at rest and
another during continuous phonation of the vowel u as in “boom.” The soft palate contacts the
posterior pharyngeal wall in normal function, whereas in velopharyngeal incompetence such
contact is absent. Most accurate evaluations of VPI are accomplished by the use of
nasoendoscopy.

In selected cases, the palate may be retropositioned or pharyngoplasty performed using a flap of
tissue from the posterior pharyngeal wall. Dental speech appliances have also been used
successfully. The type of surgery used is best tailored to the findings on nasoendoscopy.

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Mccance

DISORDERS OF THE GASTROINTESTINAL

TRACT
Congenital Impairment of Motility
Cleft Lip and Cleft Palate
Cleft lip (harelip) and cleft palate (CLP) are developmental
anomalies of the first branchial arch (Figure 42-1). The incidence
of CLP is estimated at 1 in 1000 live births in the United
States.1 Incidence is lower in black populations and higher in
Asian populations.2 Cleft lip, with or without cleft palate, is
more common in males and isolated cleft palate is more common
in females. Both anomalies can be unilateral or bilateral,
partial or complete and may also be associated with other malformations.
Nonsyndromic (isolated) CLP is a malformation
with an incomplete separation between nasal and oral cavities
without any associated anomaly. Syndromic CLP is associated
with other malformations (e.g., Crouzon syndrome [craniofacial
dysostosis], Treacher Collins syndrome [mandibulofacial
dysostosis], hemifacial microsomia).3
In most cases, cleft lip and cleft palate are caused by multiple
gene-environmental interactions, including maternal deficiency
of B vitamins (B6, folic acid, and B12), maternal smoking
and alcohol use, maternal hyperhomocysteinemia, and maternal
diabetes mellitus as well as genetic variations of interferon
regulatory factor-6, fibroblast growth factor, tumor growth
factor-alpha, and other growth factors.4 (This phenomenon,
called multifactorial inheritance, is discussed in Chapter 4.)
Together these factors reduce the amount of neural crest mesenchyme
that migrates into the area that will develop into the
face of the embryo.5
PATHOPHYSIOLOGY. Cleft lip (harelip) is caused by the incomplete
fusion of the nasomedial and intermaxillary processes
beginning during the fourth week of embryonic development,6
a period of rapid fetal growth. The cleft causes structures of
the face and mouth to develop without the normal restraints
of encircling lip muscles. The facial cleft may affect not onlythe lip but also the external nose, the nasal
cartilages, the nasal
septum, and the alveolar processes (bony ridge of maxilla that
contains the tooth sockets).
The cleft is usually just beneath the center of one nostril. The
defect may occur bilaterally and may be symmetric or asymmetric.
The cleft can range in severity from a slight indentation
of the lip to a fissure that extends to the nostril, causing a sagging
and flattening of the nose. The failure of lip fusion by 35
days of gestation may impair closure of the palatal shelves. The
more complete the cleft lip, the greater the chance that teeth in
the line of the cleft will be missing or malformed.
Cleft palate is often associated with cleft lip but may occur
without it. Cleft palate results from the failure of the primary
palatal shelves, or processes, to fuse during the third month of
gestation. The fissure may affect only the uvula and soft palate,
or it may extend forward to the nostril and involve the hard
palate and the maxillary alveolar ridge. It may be unilateral or
bilateral, with the cleft occupying the midline posteriorly and
as far forward as the alveolar process, where it deviates to the
involved side. Clefts involving the palate only are usually but
not necessarily in the midline. In some cases the vomer and
nasal septum are partly or completely undeveloped. When these
facial bones are involved, the nasal cavity may freely communicate
with the oral cavity. Teeth in the cleft palate area may be
missing or deformed.7
CLINICAL MANIFESTATIONS. Feeding the infant with cleft lip usually
presents no difficulty if the cleft lip is simple and the palate
intact. Nursing at breast or bottle depends on suction developed
by pressing the nipple against the hard palate with the tongue.
Closure of the lips is not necessary, but the tongue must work
harder if the lips cannot be pursed. A baby with cleft palate
usually requires large, soft nipples with cross-cut openings and
better tolerates feeding when in an upright position to prevent
milk escaping through the nose. Although most infants with
cleft palate can be successfully breast-fed, it may be impossible
for some because of an unproductive suck and difficulty swallowing.
8 An orthodontic prosthesis for the roof of the mouth
may facilitate sucking for some infants.9
EVALUATION AND TREATMENT. Prenatal diagnosis is made
by ultrasound; facial x-ray films confirm the extent of bone
deformity. Soft tissue alterations are evaluated by history and
physical examination.10 The nature and extent of the cleft, the
infant’s condition, and the method of surgical correction proposed
determine the course of treatment. Surgical correction is
planned as soon as possible and may be in stages.11 The aim of
surgery is to obtain an airtight closure of the palatal cleft and to
preserve the mobility and length of the soft palate without compromising
mid-facial growth.12 Children with cleft palate tend
to have repeated infections of the paranasal sinuses and middle
ear both before and after surgery, which require treatment. The
child should be evaluated for hearing loss.13 Excessive dental
decay is not unusual. Speech training and special attention by a
prosthodontist and orthodontist are almost always required. 14
Periconceptual B vitamins, folate, and folic acid intake and
reduced tobacco and alcohol use may prevent orofacial clefts.5
Surgical correction facilitates feeding and normal growth and
development.15
Schwartz

PEDIATRIC PLASTIC SURGERY

Cleft Lip and Palate
Orofacial clefting is the most common congenital anomaly and
is known to occur in 1 in 500 live white births.20 The incidence
is lower in African Americans and higher in Native Americans
and Asians. Clefting of the lip and/or palate is felt to occur
around the eighth week of embryogenesis, either by failure of
fusion of the medial nasal process and the maxillary prominence
or by failure of mesodermal migration and penetration between
the epithelial bilayer of the face. The cause of orofacial clefting
is felt to be multifactorial. Factors that likely increase the incidence
of clefting include increased parental age, drug use and
infections during pregnancy, smoking during
pregnancy, and a family history of orofacial clefting. The increased chance of
clefting when there is an affected parent is approximately 4%.
The primary palate is defined as all tissue anterior to the
incisive foramen, including the anterior hard palate (premaxilla),
alveolus, lip, and nose. The secondary palate includes everything
posterior to the incisive foramen, including the majority of
the hard palate and the soft palate (velum). Clefting can involve
the lip and nose, with or without a palatal cleft. Clefts of the lip
and/or palate are first classified as unilateral or bilateral and then
as complete or incomplete (Fig. 45-12). Complete clefts of the
lip affect the entire lip and extend up into the nose. Incomplete
clefts affect only a portion of the lip and contain a bridge of tissue
connecting the central and lateral lip elements, referred to
as Simonart’s band.
Treatment Protocol. Considerable controversy remains over
the details of the timing, technique, and protocol for treating children
with orofacial clefting. The treatment protocol described in
this chapter is accepted at many large cleft centers around the
United States. All infants born with cleft-craniofacial anomalies
benefit from care by a specialized team dedicated to the treatment
of congenital anomalies. Today, this is widely accepted as
the standard of care. Often, patients are seen prenatally
after a diagnosis is made using sophisticated antenatal ultrasonography.
The prenatal consultation has proven to be beneficial
to parents, serving to dispel fears and uncertainties, and assuring
them that treatment exists. After the infant’s birth, a team evaluation
occurs, and input is obtained from the surgeon, speech and
language pathologist, social worker, craniofacial orthodontist,
geneticist, otorhinolaryngologist, and pediatrician. For infants
born with orofacial clefting, initial concerns relate to successful
feeding and breathing. Infants with palatal clefts cannot generate
negative pressure when suckling and therefore need milk dispensed
into their mouths from a specialized nurser when they
make suckling motions.
Once adequate nutrition and a safe airway are ensured,
attention is turned to the cleft anomaly. Attempts to lessen the
deformity and set the stage for the surgical repair of the lip and
nose begin with a process known as presurgical infant orthopedics
(PSIO), which includes procedures such as nasoalveolar
molding (NAM) (Fig. 45-13). NAM repositions the neonatal
alveolar segments, brings the lip elements into close approximation,
stretches the deficient nasal components, and turns wide
complete clefts into the morphology of narrow “incomplete”
clefts. After PSIO with NAM, the definitive single-stage cleft
lip and nose repair is performed at 3 to 6 months of age. With
this initial operation, the lip deformity is repaired and a primary
nasoplasty reconstructs the cleft lip nasal deformity. If the family
does not have access to PSIO or have the resources for this
time-intensive therapy, a cleft lip adhesion can be performed as
an initial stage in the repair. The preliminary cleft lip adhesion
unites the upper lip and nasal sill, truly converting complete
clefts into incomplete clefts. A cleft lip adhesion is performed in
the first or second month of life, and the definitive cleft lip and
nose repair follows at 4 to 6 months. After the definitive cleft
lip and nose repair, the cleft palate is repaired in a single stage
at 9 to 12 months of age.
Unilateral Cleft Lip. The unilateral cleft lip is classically associated
with a cleft lip nasal deformity. The cleft lip nasal deformity
includes lateral, inferior, and posterior displacement of the alar
cartilage. This results from the deficient and clefted underlying
skeleton as well as the unopposed pull of the clefted orbicularis
Figure 45-12. A. Unilateral cleft lip and palate. B. Bilateral cleft
lip and palate. C. Incomplete unilateral cleft lip.
oris muscle abnormally inserted on the alar base (Fig. 45-14A).
The maxillary minor segment (the smaller alveolar/maxillary
segment
on the clefted side) is collapsed medially. The process
of unilateral cleft lip repair can be thought of as “philtral subunit
reconstruction.” The goal of the operation is to level Cupid’s bow
and reconstruct the central philtrum of the lip, ideally placing
the incision and subsequent scar as close to the normal philtralcolumn as possible. The surgical
repair is performed under general
anesthesia, and local anesthesia containing epinephrine is used.
Many different techniques of cleft lip and nose repair have been
proposed; however, most of the commonly used procedures are
variations of a “rotation-advancement” procedure.21 The rotationadvancement
procedure, as championed by Millard (Fig. 45-14B),
rotates the philtral subunit of the central lip downward to level
Cupid’s bow as the lateral lip element is advanced into the defect
created by the downward rotation of the philtrum. Some surgeons
choose to perform primary closure of the alveolar cleft at the time
of primary lip and nose repair, called a gingivoperiosteoplasty. If
the alveolar cleft is to be repaired, the gingivoperiosteoplasty is
performed by raising mucoperiosteal flaps within the alveolar cleft
margin and reapproximating them across the alveolar cleft defect.
This creates a bony tunnel closed with periosteal flaps and facilitates
the generation of bone in the alveolar defect. It is accepted
today that some form of primary nasoplasty should be performed
at the time of primary definitive lip repair. Techniques to release
and reposition the nasal tip cartilages, as well as the ala, are performed
with variations of tip rhinoplasties using suture methods.
Some surgeons choose to use postoperative internal and/or external
splints to maintain the nasal correction achieved at surgery during
the healing process.
Bilateral Cleft Lip. In the complete bilateral cleft lip and
nose deformity, the central lip element, called the prolabium, is
entirely separate from the rest of the upper lip. The prolabium
is displaced on top of the central alveolar segment, called the
premaxilla, containing the unerupted four central incisors. Often,
the premaxilla and prolabium are outwardly displaced. This is
referred to as a flyaway premaxilla. For the child with a complete
bilateral cleft lip and nose, PSIO is a very important step in preparing
the child for definitive lip and nose surgery by retracting
the premaxilla into the maxillary arch, repositioning the lip segments,
and stretching the rudimentary columella. Bilateral cleft
lip and nose repairs often are versions of straight-line repairs, with
the Mulliken technique being the more commonly performed
(Fig. 45-15). In the bilateral cleft lip deformity, the new philtrum
is made from the prolabium and is united to the lateral lip elements
on top of the repaired orbicularis oris muscle.22
Cleft Palate. During the eighth to twelfth weeks of gestation,
the mandible becomes more prognathic, the tongue drops from
beneath the clefted lateral palatine processes, and the palatalshelves migrate upward into a more
horizontal position and
fusion occurs. A cleft palate results from the failure of fusion of
the two palatal processes. As with labial clefting, isolated clefts
of the palate are multifactorial in etiology, and isolated clefts of
the palate are more likely to be associated with other anomalies.
Between 8% and 10% of isolated clefts of the palate are associated
with the 22q deletion of velocardiofacial syndrome.23
The main goal of cleft palate surgery is to help the patient
attain normal speech, which results from velopharyngeal competence.
During speech, the soft palate, or velum, is moved posteriorly
and superiorly, primarily by the levator palatini muscle
sling that suspends the velum from the skull base. Velopharyngeal
competence is obtained during attempted speech when the
velum approximates the posterior pharyngeal wall, preventing
air and liquid from regurgitating into the nasal cavity. Velopharyngeal
competence allows intraoral pressure to be built up
for speech sounds. A cleft palate precludes this from occurring
and results in velopharyngeal incompetence (VPI). Because it
is impossible for the oral and nasal cavities to be partitioned in
the patient with a cleft palate, it is also difficult for the patient
to develop negative intraoral pressure for an effective suck.
Therefore, specialized nursers are used to dispense liquid into
the infant’s mouth during the suckling motions. Children with
clefts of the palate have an increased incidence of otitis media;
this may be related to the abnormality of the velar musculature
and ineffective function of the eustachian tube. The increased
incidence of otitis media can result in hearing loss if not treated
appropriately. In addition, VPI and nasal air escape during
speech results in hypernasal speech.
As with the repair of cleft lip and nose, the timing, technique,
and protocols for cleft palate repair are controversial. Most
agree that palate repair should be performed before the development
of speech. The cleft palate usually is repaired when the
infant is between 6 and 18 months of age. Cleft palate repair also
is performed under general anesthesia, with the head slightly
hyperextended and a retractor, such as the Dingman mouth gag,
placed intraorally to retract the tongue and endotracheal tube. An
epinephrine solution is injected into the palate. Techniques of hard
palate closure include the use of unipedicled hard palate mucoperiosteal
flaps as in the Wardill-Veau-Kilner repair or bipedicled
hard palate mucoperiosteal flaps as in the von Langenbeck repair.
Both the unipedicled and bipedicled hard palate palatoplasty techniques
rely on the greater palatine neurovascular pedicle. Soft
palate or velar closure techniques are divided into straight-line
and Z-plasty procedures. With either a straight-line or Z-plasty
velar repair, the levator palatini muscle should be independently
repaired; this is called an intravelar veloplasty. The clefted levator
is identified coursing sagittally in an anterior-posterior direction,
abnormally inserted onto the posterior edge of the hard palate.
In intravelar veloplasty, it is released from the posterior edge of
the hard palate in the midline and dissected free from abnormal
attachments to the aponeurosis of the tensor veli palatini muscle
and superior constrictor laterally. After its complete release, the
levator palatini muscle is united in the midline, with reconstruction
of the levator muscle sling that suspends the velum from the
skull base and aids in velopharyngeal competence.
The authors prefer the double opposing Z-plasty technique
of soft palate or velar reconstruction known as the Furlow palatoplasty.
24 The procedure uses four triangular flaps, two oral
and two nasal, with the posteriorly based flaps containing the
released levator muscles. The Z-plasty lengthens the soft palate,
prevents longitudinal scarring from a straight-line repair, and
produces a secondary pharyngoplasty effect by narrowing the
velopharyngeal port (Fig. 45-16).
Complications of palatoplasty include wound healing problems
resulting in a breakdown of the suture line and the development
of a fistula. The literature reports fistula rates ranging
from approximately 1% to 20%. Treatment of palatal fistulae is
particularly challenging, because the recurrence rates have been
noted to approach 96%. The second most common complication
of palatoplasty is the incomplete correction of speech and the
development of postoperative VPI. The literature reports postoperative
VPI rates ranging from 10% to 40%. Some of the best
rates of velopharyngeal competence have been reported with the
Furlow double opposing Z-plasty palatoplasty. Postoperative VPI
is treated with pharyngoplasty—either a posterior pharyngeal flap
pharyngoplasty or a sphincter pharyngoplasty. A posterior pharyngeal
flap is a static flap formed from the posterior pharyngeal wall
including mucosa and a portion of the superior constrictor muscle.
The midline superiorly based pharyngeal flap is inset into the posterior
free edge of the soft palate, permanently attaching it to the
posterior pharyngeal wall. The sphincter pharyngoplasty has been
reported to involve creation of a dynamic sphincter made with the
bilateral posterior tonsillar pillars containing the palatopharyngeus
muscle. The superiorly based tonsillar pillars are elevated from the
lateral pharynx and inset into a horizontal incision on the posterior
pharyngeal wall at the level of the adenoid pad.
Table 24. Pediatric Craniofacial Anomalies
Definitionı Epidemiology Clinical Features Treatment
OeftUpı failure of fusion of maxillary and medial I in 1000 live lirthsll in BOO Caucasians,
Classified as incomplete/complete Cleft lip team; Surgery 13 monthsl:
nasatprocessesı tAsians, '8~O:s) &unilbllateral Milliard or Tennison·Randall; corrections
M:h2:1 213cases:unilateral,left·sided,male usually required later on lesp. for nasal
Cleft of left lip/palate in boys has deformityl
hereditary component A - Defects of soft palate only
C~ftPalate failure of fusion of lateral palatine/mlldlan Isolated Cleft Palale: 0.5 per 1000
Classified JS ,ncom~etelcomplete Special bottles for feeding
palaline processes and nasal septumı Ino racial variatlOnl &un~lateral Speech pathologi~ B
f>Mı Isolated [common in femalesl or Surgery 16-9 months): Von LangenbelJ( or
In conjunclronwith cleft lip furlowZ·Pfasty
[common in males) E~T consult - often r~urrent OM,
requiring myringotomy tubes
 Genetic Lingkungan

IRFG MSXI FGFR1 TGFA TGFB Maternal Nutrien Obat- faktor

RARA P450 merokok obatan lainnya
GST EPHX

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Valproate hydrocanna- stress,
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mengalami interaksi dengan tdk tjdi ekspresi teratogen

paparan asap rokok dr faktor transkripsi
perkembangan
homebox gen

terjadinya interaksi antara gen & lingkungan

atau faktor yang lainnya

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jaringan ikat mesenkim hipoplasia

terjadinya proses kegagalan fusi