Cleft Palate

Joy Samanich
Pediatrics in Review 2009;30;230
DOI: 10.1542/pir.30-6-230

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pedsinreview.aappublications.org/content/30/6/230

Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly
publication, it has been published continuously since 1979. Pediatrics in Review is owned,
published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point
Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 2009 by the American Academy of
Pediatrics. All rights reserved. Print ISSN: 0191-9601.

Downloaded from http://pedsinreview.aappublications.org/ at University of Connecticut on October 11, 2014

in brief
In Brief
Cleft Palate
Joy Samanich, MD
Children’s Hospital at Montefiore
Bronx, NY
Author Disclosure
Drs Samanich and Adam have
disclosed no financial relationships
relevant to this In Brief. This
commentary does not contain a
discussion of an unapproved/
investigative use of a commercial
product/device.
Development of the Palate. Moore K,
Persaud T. In: The Developing Hu-
man. 5th ed. Philadelphia, Pa: WB
Saunders Co; 1993
The Oral Cavity: Cleft Lip and Palate.
Tinanoff N. In: Behrman R, Kliegman
R, Jenson HB, Stanton BF, eds. Nel-
son Textbook of Pediatrics. 18th ed.
Philadelphia, Pa: Saunders Elsevier;
2007:1532
The Multidisciplinary Evaluation and
Management of Cleft Lip and Pal-
ate. Robin N, Baty H, Franklin J, et
al. South Med J. 2006;99:1111–1120
Craniofacial, Cleft Palate. Witt PF.
eMedicine Specialties, Plastic Sur-
gery, Craniofacial. 2006. Available at:
http://www.emedicine.com/plastic/
TOPIC519.HTM
Cleft Lip/Cleft Palate. Healthinfo. Chil-
dren’s Hospital, St. Louis. 2007.
Available at: http://www.stlouis
childrens.org/tabid/88/itemid/2526/
Cleft-Lip–Cleft-Palate.aspx
Cleft Palate Foundation Website. Avail-
able at: http://www.cleftline.org/
Feeding Your Baby With a Cleft Palate
Video. Moses M, Alexander ME. New
Orleans, La: Cleft & Craniofacial
Team of New Orleans. Available at:
230 Pediatrics in Review Vol.30 No.6 June 2009
Downloaded from http://pedsinreview.aappublications.org/ at University of Connecticut on October 11, 2014
http://www.neworleanscleftteam.org/
videos.html
Cleft palate (CP) is a relatively common
birth defect throughout the world.
However, it is important to distinguish
between cleft palate alone (CPA) and
cleft lip with or without cleft palate
(CLP), two distinct birth defects that
have different causes, incidences, and
sex and racial predilections. For exam-
ple, in the Caucasian population, the
incidence of CLP is about 1 in 750
births and that of CPA is about 1 in
2,500. CLP is more common in males;
CPA is more common in females. The
distribution of CPA varies slightly in
different ethnic groups, but is signifi-
cantly different for CLP, which is most
common in Native Americans (1 in
230 to 1,000) and Asians (1 in 400 to
850) and least common in African
Americans (1 in 1,300 to 5,000).
The incidence of concurrent genetic
disorders is increased for children who
have CLP and even higher in those born
having CPA. More than 200 genetic
syndromes have CLP as a feature; a
larger number of syndromes feature
CPA. Any newborn who has CLP or CPA
should be evaluated by a geneticist for
the presence of additional congenital
anomalies; complete pregnancy, birth,
and family histories should be obtained;
and a physical examination should be
performed. Identifying whether a cleft
is isolated or part of a syndrome is
essential in guiding the evaluation for
additional associated anomalies, pro-
viding anticipatory guidance for the
family, and performing genetic coun-
seling. The recurrence risk for the family
of a child who has isolated CLP is about
4% and for the family of a child who
has isolated CPA is about 2%. These
numbers are increased if more than one
child is born with a clefting defect, if a
parent also is affected, and as the
severity of the clefting defect increases.
If the CLP or CPA is part of a genetic
syndrome, the recurrence risk depends
on the inheritance pattern of the syn-
drome.
Clefting defects occur early in em-
bryologic life, generally between the
6th and 9th weeks of gestation. The
primary palate begins to form at about
35 days, complete lip development oc-
curs by the 6th week, and palatal fusion
follows. Cleft lip occurs when there is
interruption or hypoplasia of the mes-
enchymal layer, which causes a failure
of fusion of the medial nasal process,
maxillary process, and lateral nasal pro-
cess. Clefts of the lip can be unilateral
or bilateral and can vary from a small
notch in the border of the lip to com-
plete separation at the philtral column
and extension to the border of the nose.
Cleft palate occurs when the palatal
shelves fail to fuse. Clefts of the palate
also can be unilateral or bilateral and
can vary in severity from a submucous
cleft to clefts involving both the pri-
mary (anterior to the incisive fossa) and
secondary (posterior to the incisive
fossa) palates. Although clefting de-
fects generally are multifactorial traits
caused by an interplay between genetic
and environmental factors, some clefts
result from mutations in single genes
(TBX22, IRF6, MSX1), some are part of
chromosomal aneuploidy or deletion
syndromes (trisomy 13, velocardiofacial
syndrome), and others result from ter-
atogens (anticonvulsants).
An important type of clefting defect
to recognize in the neonatal period is
Pierre Robin sequence (PRS), which in-
cludes micrognathia (small mandible), a

retropositioned tongue, and a U-shaped
cleft palate. The initial embryologic
event is believed to be failure of the
mandible to grow properly, leading to
positioning of the tongue in the back of
the pharynx, which blocks the ability of
the palatal shelves to fuse properly.
Affected children often experience se-
vere respiratory distress, leading to a
mortality rate as high as 30% and
requiring interventions ranging from
prone positioning to allow the tongue
to fall forward out of the airway to
intubation and tracheostomy. Children
who have PRS require careful monitor-
ing, particularly in the first 1 to 4 weeks
after birth. Over time, the lower jaw
generally “catches up” in growth, but
sometimes surgical intervention is re-
quired for jaw expansion. PRS generally
occurs as an isolated birth defect, but
may be part of syndromes such as
trisomy 18 or Stickler syndrome.
Regardless of the cause, the child
who has a cleft and his or her family
face many challenges. Initially, diffi-
culty in feeding and growth may be
encountered, followed by recurrent ear
infections and sometimes hearing loss,
dysfunctional speech and communica-
tion, and social struggles because of the
child’s appearance, not to mention the
physical and emotional strain encoun-
tered because of the medical and sur-
gical interventions needed to treat
these birth defects.
Because of the complexity of the
sequelae of CP, comprehensive multi-
disciplinary care for affected children is
necessary and generally should be pro-
vided by a CP team, consisting of a
pediatric geneticist, plastic surgeon,
otolaryngologist, dentist, oromaxillofa-
cial surgeon, orthodontist, prosthodon-
tist, audiologist, speech and language
pathologist, and social worker. It is vital
for this team to work together to bal-
ance the many factors important in
managing the care of a child who has a
cleft.
An important initial concern to par-
Downloaded from http://pedsinreview.aappublications.org/ at University of Connecticut on October 11, 2014
ents of a newborn who has a cleft is
how to feed their baby. Although many
new parents find feeding their babies
challenging, feeding can be particularly
difficult for the baby who has a cleft.
Parents have the added concern that
the baby must gain weight before the
cleft can be repaired.
Several techniques can help in feed-
ing a baby who has a cleft. Babies who
have a cleft lip but an intact palate
generally are able to breast- or bottle-
feed. There may be some difficulty in
creating a seal from the lip around the
breast or bottle, but holding the baby in
an upright “football” position or adjust-
ing the breast to help tuck the cleft into
the breast tissue usually can help the
baby feed successfully.
Infants who have a CP have a con-
nection between the mouth and the
nose and, therefore, have difficulty cre-
ating sufficient suction in the mouth to
complete a feeding without tiring. Of-
ten they are unable to obtain enough
nutrition by breastfeeding alone. Moth-
ers should be encouraged to try breast-
feeding, but if weight gain is poor,
supplementation with a bottle, ideally
using pumped human milk, will be
needed. However, bottle-feeding also
may present a challenge. Infants who
have CP often need small, frequent
feedings, particularly in the beginning.
Babies should be positioned relatively
upright, so a limited amount of milk
escapes through the nose, although this
occurrence generally is not harmful to
the baby.
Because babies who have CP often
swallow more air than do other babies,
they should be burped two to three
times during a feeding, with the bottle
positioned as upright as possible to
avoid air in the nipple, or fed with an
angled bottle. However, parents should
be cautioned not to stop for too long in
the middle of the feeding to burp the
baby or the infant may lose interest in
completing the feeding.
Several special types of nipples and
in brief
feeders can help babies who have CP.
Using a cross-cut nipple and cutting
the cross-cut hole at the tip of the
nipple larger with a scalpel or small
scissor allows the baby to get more milk
without having to increase suction.
Feedings generally should be limited to
about 30 minutes, and if the baby takes
longer to feed, the size of the hole
should be increased to allow greater
milk flow. The hole can be enlarged
slightly after each feeding until the
baby coughs or chokes during the feed-
ing, indicating that the hole is too
large. The parent should dispose of this
nipple and use a new one, cross-cutting
the hole to the largest size that did not
cause coughing.
Many parents find it helpful to use
soft, squeezable bottles, which allow
them to provide the force for giving a
mouthful of milk to the baby, rather
than having the baby suck to provide
the force. Several companies make
feeders specifically for infants who
have CP, using specially cut nipples and
compressible bottles. Pediatricians
should consult the CP team for recom-
mendations of specific types and
brands of nipples and bottles suited
best to the needs of a particular pa-
tient.
A cleft lip generally is repaired sur-
gically between the ages of 10 and
12 weeks, according to the “rule of
tens”– 10 pounds, 10 weeks old, and
hemoglobin of 10.0 g/dL (100.0 g/L).
A cleft palate usually is repaired be-
tween 6 and 12 months of age. Such
early repair provides the benefit of
better speech outcome, but poses the
risk of impairing maxillary growth. Sev-
eral techniques are used in CP repair,
depending on the characteristics of the
cleft and experience of the surgeon,
with the Furlow double-opposing
Z-plasty being the most common. As
they get older, children may need revi-
sions of a cleft lip or palate repair,
along with alveolar grafting or rhino-
Pediatrics in Review Vol.30 No.6 June 2009 231
in brief
plasty to repair a nasal deformity asso-
ciated with the cleft lip.
Most children who have CP experi-
ence problems with middle ear disease.
Probably because of anomalies of the
insertion of the tensor and levator veli
palatini muscles into the eustachian
tube, affected children are unable to
open and ventilate the middle ear ad-
equately. If left untreated, chronic se-
rous otitis media can lead to hearing
impairment, which might impair speech
and learning, so myringotomy tubes
often are inserted at the time of pala-
toplasty. Children who have CP must
have frequent hearing evaluations
throughout childhood.
After CP repair, about 10% to 20%
of patients experience velopharyngeal
insufficiency, a phenomenon in which
the soft palate cannot form a tight
closure with the pharynx, causing air to
escape through the nose rather than
being directed through the mouth dur-
ing speech. This effect manifests as
audible nasal air escape and as hyper-
nasal resonance during speech, partic-
ularly when making the s, z, sh, f, p, and
b sounds. Affected children may de-
velop maladaptive compensatory artic-
ulation errors for these sounds, making
their speech more difficult to under-
232 Pediatrics in Review Vol.30 No.6 June 2009
Downloaded from http://pedsinreview.aappublications.org/ at University of Connecticut on October 11, 2014
stand. Children who have CP also may
have speech problems caused by dental
malocclusion from the cleft or from
fistula formation after surgery. Fistulas
can develop between the oral and nasal
cavities, leading to air escape during
speech and to regurgitation of food or
liquids through the nose. Surgical cor-
rection of velopharyngeal insufficiency,
fistula, or other structural anomalies
generally is necessary before speech
therapy, an integral part of treatment
for the child who has CP, can be suc-
cessful.
Many patients have an osseous de-
fect of the maxillary alveolar bone,
requiring a bone graft, which generally
is performed between 6 and 8 years of
age. About 25% of children who have
CLP have residual jaw deformities after
their cleft repairs and may need a
maxillary advancement (LeFort I proce-
dure), orthodontic treatment, or palatal
expanders. Children who have CLP may
be missing their lateral incisors in the
area of the alveolar cleft and often have
poorly formed teeth or teeth that lack
enamel. Good dental hygiene is impor-
tant from the time the first tooth erupts
because healthy teeth stimulate and
retain alveolar bone, which is important
for lip support and function.
Clefting deformities are relatively
common birth defects and have a mul-
tifactorial inheritance pattern. Children
who have clefts and their families can
anticipate difficulties with feeding,
speech and hearing, dentition, and cos-
mesis. With care from a multidisci-
plinary CP team, however, they can
receive appropriate help and compas-
sion to get through the challenging
early years.
Comment: Centers that provide co-
ordinated team care for children who
have clefting and other craniofacial
deformities are ideal for meeting the
multitude of issues confronting the af-
fected child and family. Not all of us
have ready access to such centers for
our patients, and our responsibility be-
comes appreciating the following is-
sues: feeding, hearing and speech, den-
tal and orthodontal problems, the need
for genetic counseling, and emotional
needs. We must do our best to provide,
beyond surgical repair, the full spec-
trum of services needed.
Henry M. Adam, MD
Editor, In Brief
 Cleft Palate
Joy Samanich
Pediatrics in Review 2009;30;230
DOI: 10.1542/pir.30-6-230

Updated Information & including high resolution figures, can be found at:
Services http://pedsinreview.aappublications.org/content/30/6/230
References This article cites 1 articles, 0 of which you can access for free at:
http://pedsinreview.aappublications.org/content/30/6/230#BIBL
Subspecialty Collections This article, along with others on similar topics, appears in the
following collection(s):
Ear, Nose & Throat Disorders
http://pedsinreview.aappublications.org/cgi/collection/ear_nose_-_thr
oat_disorders_sub
Fetus/Newborn Infant
http://pedsinreview.aappublications.org/cgi/collection/fetus:newborn
_infant_sub
Permissions & Licensing Information about reproducing this article in parts (figures, tables) or
in its entirety can be found online at:
http://pedsinreview.aappublications.org/site/misc/Permissions.xhtml
Reprints Information about ordering reprints can be found online:
http://pedsinreview.aappublications.org/site/misc/reprints.xhtml

Downloaded from http://pedsinreview.aappublications.org/ at University of Connecticut on October 11, 2014