Cleft Lip and Palate (Jan.1998)
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TITLE: Cleft Lip and Palate
SOURCE: UTMB Dept. of Otolaryngology Grand Rounds
DATE: January 28, 1998
RESIDENT PHYSICIAN: Greg Young, M.D.
FACULTY: Ronald Deskin, M.D.
SERIES EDITOR: Francis B. Quinn, Jr., M.D., F.A.C.S.
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INTRODUCTION
Cleft lip and palate represents the second most frequently occurring congenital deformity (after clubfoot
deformity). Cleft lip, cleft palate or both affects approximately 1 in 750 births. Clefting is associated
with many problems including cosmetic and dental abnormalities, as well as speech, , hearing and facial
growth difficulties. The otolaryngologist is uniquely qualified to identify and manage many of these
problems, and holds a key role on the cleft palate team.
ANATOMY
The palate consists of the hard palate and soft palate, which together form the roof of the mouth and the
floor of the nose. The palatine processes of the maxilla and horizontal lamina of the palatine bones form
the hard palate. Its blood supply is mainly from the greater palatine artery, which passes through the
greater palatine foramen. The nerve supply is via the anterior palatine and nasopalatine nerves. The soft
palate is a fibromuscular shelf made up of several muscles attached like a sling to the posterior portion
of the hard palate. It closes off the nasopharynx by tensing and elevating, thereby contacting Passavants
ridge posteriorly. The soft palate consists of the tensor veli palatini, the levator veli palatini, the
musculus uvulae, the palatoglossus, and palatopharyngeus muscles. CN V supplies the tensor veli
palatini, while CN IX and CN X innervate the others. The levator veli palatini is the primary elevator of
the palate.
EMBRYOLOGY
The primary and secondary palates are delineated according to embryological development. The primary
palate or premaxilla is a triangular area of the anterior hard palate extending from anterior to the incisive
foramen to a point just lateral to the lateral incisor teeth. It includes that portion of the alveolar ridge
containing the four incisor teeth. The secondary palate consists of the remaining hard palate and all of
the soft palate. The primary palate forms during the 4th to 7th weeks of gestation as the two maxillary
swellings merge and the two medial nasal swellings fuse to form the intermaxillary segment. The
intermaxillary segment is composed of a labial component (forms the philtrum), a maxilla component
(forms alveolus and 4 incisors), and palatal component (forms the triangular primary palate). Normally
during development of the primary palate, a cleft does not exist (unlike the secondary palate in which
cleft formation occurs as a natural stage of development). The secondary palate forms during the 6th to
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9th weeks of gestation, as the palatal shelves change from a vertical to horizontal position and fuse. The
tongue must migrate away from the shelves in an antero-inferior direction for palatal fusion to occur.
CLEFT FORMATION
In general, patients with clefts have a deficiency of tissue and not merely a displacement of normal
tissue. A cleft lip occurs when an epithelial bridge fails, due to lack of mesodermal delivery and
proliferation from the maxillary and nasal processes. Clefts of the primary palate occur anterior to the
incisive foramen. Clefts of the secondary palate are due to lack of fusion of the palatal shelves, and
always occur posterior to the incisive foramen. The secondary palate closes 1 week later in females,
which may explain why isolated clefts of the secondary palate are more common in females. A cleft of
the lip increases in probability of a cleft palate developing. The cleft of the lip occurs earlier and inhibits
tongue migration, which may then prevent horizontal alignment and fusion of the palatal shelves. In the
unilateral cleft lip, the floor of the nose communicates freely with the oral cavity, the maxilla on the cleft
side is hypoplastic, the columella is displaced to the normal side, and the nasal ala on the cleft side is
laterally, posteriorly, and inferiorly displaced. The lower lateral cartilage of the nose is lower on the cleft
side, its lateral cruz is longer, and the angle between the medial and lateral cruz in more obtuse. The
muscles of the orbicularis oris do not form a complete sphincter but instead are directed superiorly to the
ala nasi laterally and the base of the columella medially. In the bilateral cleft lip, the central portion of
the alveolar arch is rotated anteriorly and superiorly. The medial or prolabial segment of skin contains
no muscle or vermillion. In palatal clefts, the muscles of the soft palate are hypoplastic and insert in the
posterior margin of the remaining hard palate rather than the midline raphe. Associated dentition
abnormalities include supernumerary teeth (20%), dystrophic teeth (30%), congenitally missing teeth
(50%), and malocclusion (almost 100%).
GENETICS
Nonsyndromic inheritance of facial clefting is multifactorial. Familial inheritance of both cleft lip and
palate occurs with varying frequency, depending on whether a parent or sibling is affected. For cleft lip
with or without cleft palate, the risk rate for future offspring is 2% with only one parent affected, 4%
with only one sibling affected, 9% with two siblings affected, and 10-17% with one parent and one
sibling affected. For cleft palate alone, the risk rate for future offspring is 7% with only one parent
affected, 2% with only one sibling affected, 1% with two siblings affected, and 17% with one parent and
one sibling affected. Chromosome aberrations such as trisomy D and E have increased incidence of
clefts. Facial clefts are associated with a syndrome 15-60% of the time. More than 200 recognized
syndromes may include a facial cleft as a manifestation. Common syndromes with cleft palates include
Apert's, Stickler's and Treacher Collins. Van der Woude's and Waardenberg's syndromes are associated
with cleft lip with or without cleft palate.
EPIDEMIOLOGY
Clefts of the lip and combined lip and palate are twice as common in males. Isolated cleft palates are
twice as common in females. Cleft lips, with or without cleft palate, are most common in Native
Americans, then Orientals and Caucasians, and least common in Blacks. Conversely, the rate of isolated
cleft palate is constant among ethnic groups. Environmental factors found to cause clefts in humans are
ethanol, rubella virus, thalidomide, and aminopterin. Maternal diabetes mellitus and amniotic band
syndrome are associated with clefts. Increased paternal, but not maternal age is also associated with
clefts. Combined cleft lip and palate is the most common presentation (50%), followed by isolated cleft
palate (30%), isolated cleft lip (20%) and least common is cleft lip and alveolus (5%).
MANAGEMENT
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A team approach is needed to manage the wide variety of problems common to the patient with cleft lip
and palate. In addition to the reconstructive surgeon, a typical team consists of an otolaryngologist,
dentist, speech pathologist, audiologist, geneticist, nurse, psychiatrist, social worker, and prosthodontist.
Teams may vary depending on available resources and individual interest. The otolaryngologist has a
pivotal role in the diagnosis and management of all disorders relating to the head and neck. Cleft lip and
palate patients are particularly prone to speech disorders and ear disease, and may have significant
airway abnormalities. The otolaryngologist is uniquely qualified to oversee the management of these
disorders, and at times is the primary reconstructive surgeon as well.
INITIA L H EA D A ND NEC=
EXA MINA TION
The otolaryngologist performs a complete head and neck examination on new patients in the cleft palate
clinic. The head is inspected for symmetry, the auricle and external canal for development and location.
A facial analysis is helpful to identify abnormalities of facial symmetry and harmony. Otologic
examination includes pneumatic otoscopy and tuning forks. Anterior and posterior rhinoscopy will
identify clefting, septal abnormalities, intranasal masses, and choanal atresia. Oral cavity examination
will identify any cleft, dental arch abnormalities and tongue anomalies such as bifid tongue,
macroglossia, glossoptosis, or lingual thyroid. In addition, malocclusion, hemifacial hypertrophy or
atrophy, and facial clefting are documented. The upper airway tract is evaluated by assessing the
adequacy of phonation, cough, and deglutition, and by auscultating and palpating the neck.
SPEECH
DISOR DER S
Errors in articulation are common in cleft palate patients, especially those involving affricates and
fricatives. Other errors include stop, glides, and nasal semivowels. Velopharyngeal incompetence is
associated with an audible escape of air from the nose during production of pressure sounds and is
termed nasal emission or snort. It is estimated that 75% of patients have velopharyngeal competence
following primary cleft palate surgery, and this can be increased to 90-95% with directed secondary
procedures. Velopharyngeal competence is the most important determinant of articulation performance
and listener understanding of speech in cleft palate patients. Others factors include dentition, associated
hearing loss, and muscular and neurologic deficits. Velopharyngeal competence can be estimated by
direct examination of the nasopharyngeal depth, palatal length, and palate movement during phonation.
Flexible fiberoptic nasopharyngoscopy has the added advantage of direct visualization of palatal motion
and pharyngeal wall motion with both single sounds and connected speech.
EA R DISEA SE
Patients with an isolated cleft lip have an incidence of hearing loss similar to that in the normal
population. In contrast, cleft palate is very often associated with eustachian tube dysfunction and a
resulting conductive hearing loss. Eustachian tube dysfunction in these patients is due to an abnormal
insertion of the levator and tensor veli palatini muscles into the posterior margin of the hard palate. In
addition to middle ear effusion, the patients also appear to have an increased incidence of cholesteatoma
(7%). With increasing age, the incidence of eustachian tube dysfunction decreases, and in many cases
normal eustachian tube function develops by mid adolescence. Otologic goals in the cleft palate patient
are to provide adequate hearing, maintain ossicular continuity and adequate middle ear space, and
prevent deterioration of the tympanic membrane. Patients with eustachian tube dysfunction are
evaluated every 3-4 months until it resolves. Indications for myringotomy and tube insertion include a
significant conductive hearing loss or persistent middle ear effusion, recurrent otitis media, or tympanic
membrane retraction. In practice, almost all patients with cleft palate will require multiple sets of tubes
from 3-4 months of age until the beginning of the second decade of life. In contrast, patients with an
isolated cleft lip do not have a significantly increased rate of myringotomy and tubes.
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AIRWAY PROBLEMS
Airway problems may arise in children with cleft palates, especially those with concomitant structural or
functional anomalies. For example, Pierre-Robin sequence is the combination of micrognathia, cleft
palate, and glossoptosis. Affected patients may develop airway distress from their tongue becoming
lodged in the palatal defect.
SURGICAL REPAIR
Cleft Lip
Cleft lips are repaired at about 10 weeks of age at most institutions. Lip adhesions can be performed at 2
weeks of age. They convert a complete cleft to an incomplete cleft, and serve as a temporizing measure
for those infants with certain feeding problems. Adhesions may interfere with definitive lip repair and
are less often needed in recent years due to the wider variety of specialty feeding nipples available. The
rotation advancement method of cleft lip repair is the most commonly used in The United States.
Initially, nine landmarks on the lip are identified with a vital dye. These are
1) the non-cleft side nasal ala base,
2) The non-cleft side high point of lateral cupid's bow,
3) The low point of cupid's bow,
4) The non-cleft side high point of medial cupid's bow (equal to distance between #2 and #3),
5) the cleft side high point of cupid's bow (placed where white roll attenuates along vermillioncutaneous junction),
6) superior extent of advancement flap(length of #5 to #6 equal height of non-cleft lip(#4 to #8),
7) point on cleft side alar crease such that length of #5 to #7 equals length of #1 to #2,
8) And 9) the superior extent of the rotation incision.
Through and through cuts are made for the non-cleft side rotation flap, allowing # 4 to drop down with #
2. (The small remaining triangular tissue attached to the columella is Millard's C flap). The cleft side
advancement flap is now made, with distance #5 to #6 corresponding to distance #8 to #4 in its newly
rotated position. Next, the cleft and non- cleft lip segments are separated in a supraperiosteal plane from
the maxilla. The cleft side nasal ala incision is then made (#6 to #7), and the external and vestibular skin
is separated from the cleft side lower lateral cartilage. Next, the tip of the advancement flap (#6) is
brought to point #9(base of the columella). The remaining sutures are then placed to complete the repair.
Bilateral cleft repair, although more complex, is performed using similar principles.
Cleft Palate
Several techniques for cleft palate repair have been developed over the past 3 decades. The current trend
is toward procedures that involve less denuding and scarring of the hard palate, and less tension on the
soft palate. Although controversial, many authors believe there is a relationship between scar formation
of the palatal repair and the impaired mid-facial growth that is observed in most patients with cleft
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palate. Bony abnormalities that develop include collapse of the alveolar arches, midface retrusion, and
malocclusion. Facial growth may also be affected by the age when the repair is performed. Some
surgeons advocate later repair, because facial growth is less affected when surgery is delayed until 18-24
months of age. Others advocate an earlier repair, stating that feeding, speech and socialization are
improved if the surgery is performed by the 1st year of life, and that facial growth problems can be
minimized with less traumatic palate repairs. Several methods of cleft palate repair have been described.
The two-flap techniques, such as those described by Bardach or Furlow are used commonly in the
U nited States today. In the Bardach repair, a single posteriorly based mucoperiosteal flap is developed
over each palatal shelf. In this repair, medial incisions are made which separate the oral and nasal
mucosa, and lateral incisions are made at the junction of the palate and alveolar ridge. The
mucoperiosteal flaps are elevated, taking care to identify and preserve the neurovascular bundle
containing the greater palatine artery. Next, the velar muscle is detached from its attachment to the
posterior border of the palatal shelves. The palate is then closed in three layers, 1st nasal mucosa, then
velar muscle, and finally oral mucosa. The lateral palate incisions are closed loosely. Bilateral cleft
palate repairs are performed in similar fashion. Cleft palate patients at high surgical risk or who refuse
surgical treatment may use a dental obturator to obtain velopharyngeal competence. Disadvantages of
dental obturators include the necessity of wearing a prosthesis and the need for modification of the
prosthesis as the patient grows.
BIBLIOGRAPHY
1. Bardach J, Morris H L, eds. Multidisciplinary management of cleft lip and palate. Philadephia: WB
Saunders, 1990.
2. Bumsted R M. Cleft lip and palate. In: Cummings CW, et al.,eds. Otolaryngology-H ead and Neck
Surgery. St. Louis: CV Mosby, 1988.
3. Crockett DM, Seibert R W, and Bumsted R M. Cleft lip and palate: the primary deformity. In: Bailey
BJ, et al, eds. H ead and Neck Surgery- Otolaryngology. Philadelphia: Lippincott Co., 1993.
4. Crockett DM. Velopharyngeal incompetence. In: H ealy G B, ed. Common problems in pediatric
otolaryngology. Chicago: Y ear Book, 1990.
5. H olt G R and Watson MJ. The otolaryngologist's role in the craniofacial anomalies team. Otolaryngol
H ead Neck Surg 92:406, 1984.
6. Seibert R W. Lip adhesion in bilateral cleft lip. Arch Otol H ead Neck Surg 109:434, 1983.
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