ENT - Cleft Lip and Palate - 05162024

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Cleft Lip and Palate

• Cleft lip and cleft palate are among the more common congenital
deformities
• may often have associated problems:
• otologic disease
• speech and language problems, such as delayed onset of speech, articulation
disorders velopharyngeal dysfunction
• Dental deformities that include malocclusion and missing, malformed, or
supernumerary teeth
• facial growth deficiencies
• Psychosocial issues
CLASSIFICATION OF CLEFT LIP AND PALATE
• incisive foramen divides the palate into the primary palate and the
secondary palate

• primary palate- has the incisive foramen as its posterior border,


consists of the premaxilla, lip, nasal tip, and columella
• secondary palate- develops after completion of development of the
primary palate and extends from the incisive foramen anteriorly to
the uvula posteriorly
• Cleft lip is classified as unilateral or bilateral, and its extent may be
classified as complete or incomplete
• complete cleft involves the entire vertical thickness of the upper lip
and is often associated with an alveolar cleft because the lip and
primary palate share the same embryologic origin
• incomplete cleft lip involves only a portion of the vertical height of
the lip, with a variable segment of continuity across the cleft region
EMBRYOLOGY
end of the 4th embryonic week
• the neural crest–derived facial
prominences appear from the first pair of
pharyngeal arches
• maxillary prominences are found laterally
• frontonasal prominences, formed by the
proliferation of mesenchyme ventral to the
forebrain, form the upper border of the
stomodeum
• On either side of the frontonasal
prominences are local thickenings of
surface ectoderm that form the nasal
placodes
5th week of embryonic
development
• the nasal placodes invaginate
to form the nasal pits
• This invagination process
creates a ridge of tissue
around the pit, called the
lateral nasal prominence
laterally and the medial nasal
prominence medially
Over the 6th and 7th weeks of embryonic development
• The paired maxillary prominences grow medially toward the paired
medial nasal prominences
• paired medial nasal prominences and paired maxillary prominences🡪
upper lip
• Fusion of medial nasal prominences🡪 philtrum, medial upper lip,
nasal tip, and columella
• maxillary prominences🡪 lateral aspects of the upper lip
• lateral nasal prominences🡪nose
• Palatogenesis begins at the end of the 5th week, and complete fusion
occurs by 12 weeks of development.
DIANOSIS
Prenatal UTZ
• Diagnosis as early as 18 weeks or early 2nd trimester (accuracy
improves with age)
EPIDEMIOLOGY
• PH: 2 per 1000 live births
• 3rd MC birth defect in PH (1st is multiple congenital deformity while
2nd is ankyloglossia)
• Cleft Lip – boys
• Cleft Palate - girls
Two unaffected parents with one child affected with a cleft
• 4.4% chance of having another child with a cleft lip and palate
• 2.5% chance of having a child with isolated cleft palate
One parent affected with a cleft
• 3.2% chance of having a child with cleft lip palate
• 6.8% chance of having a child with isolated cleft palate
Presence of a cleft in one parent and in one sibling
• 15.8% chance that the next child will have a cleft lip or palate
• 14.9% chance that the next child will have a cleft palate
ANATOMY
• normal upper lip is divided into red and white components
• red lip is a mucous membrane, whereas the white lip is a cutaneous
structure
• mucocutaneous junction at the vermilion border between the red and
white lips is an important anatomic boundary that must be
reconstructed meticulously in cleft lip repair for a natural-looking
result
• All patients with cleft lip deformities have muscular deficiencies and
irregularities of varying degrees that lead to the abnormal appearance
and function of the lip and mouth
• muscle fibers in cleft lip deformities run in an inferior-to-superior
direction along the margins of the cleft.
• They insert into the columella medially and along the nasal alae
laterally
• These fibers must be detached from their insertions and reoriented in
a horizontal direction to bridge the cleft and create a complete
muscular sling around the entire circumference of the oral cavity
Cleft Palate Deformity
• Five muscle pairs contribute to the soft palate
• levator veli palatini (LVP) muscle
• has a transverse orientation and occupies the midportion of the soft palate,
thereby creating a muscular sling for the velum
• This muscular sling is the principal structural component in closure of the
nasopharynx during speech and swallowing
Other muscles that contribute to the velopharyngeal sphincter
• palatopharyngeus
• superior constrictor
• Tensor veli palatine
• dilation of the eustachian tube and support for the soft palate during
contraction
• musculus uvulae
• seen in SMCP
• may lead to velopharyngeal insufficiency and speech disorders
• In patients with cleft palate,
the muscles of the soft
palate may be hypoplastic in
addition and misdirected,
with abnormal insertions
into the posterior hard
palate
• If the cleft involves the hard
palate, a midline bony
deficiency of variable degree
will extend toward the
incisive foramen
PRESURGICAL CONSIDERATION - narrow gap
• Lip taping - most effective maneuver to improve cleft lip taping
• Nasoalveolar molding - a presurgical option - fabrication of palatal
appliance by a trained orthodontist
TREATMENT
• Cheiloplasty can be done as early as 3 months
• Early cheiloplasty is not done - it may cause maxillary retrusion and reduced
maxillary length
• Alveoloplasty (soft tissue) can be done with primary cheiloplasty
• Alveolar bone grafting postponed until 7-9 years (root of permanent
canine 1/3 to 2/3)
• Primary rhinoplasty done with primary cheiloplasty or 14 years for
females and 16 years for males - definitive age
• Palatoplasty can be done 12 - 18 months
Complications
• Postoperative bleeding- rare
• Perioperative adverse airway events have been reported as high as
23%
• oronasal fistula formation- anterior hard palate and the junction of
the hard and soft palate
• Velopharyngeal dysfunction- most common complication after
palatoplasty
Thank you

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