Cleft Palate Lip Bifid uvula submucosal cleft modified by Dr Nayyar.ppt

Ent

1. 1
Cleft Lip & Palate
By :-Dr Supreet Singh Nayyar, AFMC
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2. Bifid uvula
A bifid orbifurcateduvula is a split or cleft uvula.
Newbornswithcleft palatealso have a split uvula. The
bifid uvula results from incomplete fusion of the palatine
shelves. Bifid uvulas have less muscle in them than a
normal uvula, which may cause recurring problems with
middle ear infections. While swallowing, the soft palate is
pushed backwards, preventing food and drink from
entering the nasal cavity. If the soft palate cannot touch
the back of the throat while swallowing, food and drink
can enter the nasal cavity.
[6]
Splitting of the uvula occurs
infrequently but is the most common form of mouth and
nose area cleavage among newborns. Bifid uvula occurs
in about 2% of the general population,
[7]
although some
populations may have a high incidence, such asNative
Americanswho have a 10% rate.
[8]
Bifid uvula is a common symptom of the rare genetic
syndromeLoeys-Dietz syndrome,
[9]
which is associated
with an increased risk of aortic aneurysm.
[10]
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3. •SubmucosalCleft: A submucosalcleft is a cleft
that is under the mucosa that lines the roof of
the mouth, hence the term “sub.” Because a
submucosalcleft is under the mucosa, the only
physical indicator of its presence may be a bifid
uvula. Even though not seen from the surface,
the muscles of the palate are not joined at the
midline in a submucosalcleft. This creates an
inability to move the palate for some speech
sounds. Hence, a submucosalcleft is usually
diagnosed when a child has abnormal speech
development and a bifid uvula is present.
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Scope
Introduction
Epidemiology
Embryology
Relevant Anatomy
Causes
Classification of Cleft Palate
Management Principles
Evaluation & Initial care
Cleft palate repair
Complications
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Introduction
Orofacial clefting is the second most common
congenital deformity (after clubfoot).
Cleft has detrimental effects on speech,
appearance, swallowing, dental, hearing,
facial growth, emotions
Multidisciplinary team approach
Time consuming, multistage treatment
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Epidemiology
Incidence of Orofacial clefting: 1:700 live births
68% to 86% of cleft palate is associated with
cleft lip
Isolated cleft palate-more in females
Cleft lip (with or without cleft palate)-more in
males
Unilateral CL(P) is more common, more on left
side
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Embryology
palate forms through the fusion of two paired
outgrowths of the maxillary prominences, the
palatal shelves.
palatal shelves appear during the 6
th
week of
development
during the 7
th
week, the shelves elevate,
assume a horizontal orientation, and fuse.
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Embryology
fusion begins at the incisive
foramen, progresses toward the
posterior palate, and is complete at
about the 12th week of intrauterine
life.
Cleft palate –failure of fusion of
palatal shelves
Intrauterine development of secondary palate, 6 weeks to 12 weeks.
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Anatomy-Bones of the Palate
Premaxilla
Paired maxilla
Paired palatal bones
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Anatomy-Muscles of the Palate
Extrinsic muscles
Levator veli palatini
Tensor veli palatini
Palatopharyngeus
Palatoglossus
Salpingopharyngeus
Superior constrictor
Intrinsic muscle
Musculus uvulae
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Levator veli palatini
Origin: posteromedial
Eustachian Tube
Insertion: palatal
midline
Function: elevates soft
palate: ET dilatation
In cleft palate:
hypoplastic and thin.
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Tensor veli palatini
Origin: scaphoid fossa of sphenoid
Insertion: aponeurosis on the posterior hard palate
Function: Eustachian Tube dilatation
In cleft palate: lesser numbers of fibres
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Palatopharyngeus
Origin: posterior border of aponeurosis and
levator
Insertion: lateral pharynx and larynx
Function: antagonistic to levator: helps in
velopharyngeal closure
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Musculus Uvulae
Origin: anterior to the
aponeurosis in the midline
Insertion: posterior
connective tissue of the
midline
Function: velopharyngeal
closure
In cleft palate:
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Causes -Mechanical Causes
i) palatal shelves hypoplasia
ii) increased resistance to palatal
shelves
iii) excessive head width
iv) failure of shelf fusion
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Causes -Genetic causes
defect in genetic loci
TGF alfa,
TGF beta3,
T-BOX 22,
P-63,
procollagen type XI
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Causes
Malformation syndrome association Teratogens
* Down syndrome * Anticonvulsant
•Stickler syndrome * Thalidomide
•Treacher-Collin syndrome * Maternal smoking
•Alpert syndrome * Fetal alcohol synd
•Marfan syndrome
•Turner syndrome
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Symptoms
Separation of the lip
Separation of the
palate
Nasal distortion
Recurring ear infections
Hearing loss
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Symptoms (cont.)
Failure to gain weight
Nasal regurgitation
when bottle feeding
Poor speech
Misaligned teeth
Growth retardation
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Classification of Cleft Palate
Several classification system based on
morphology, anatomy & embryologic
development
Davis & Ritchie(morphological classification)
Basis of involvement of alveolar process
Gr I: Prealveolar cleft
Gr II: Postalveolar clefts
Gr III: Alveolar clefts
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Classification of Cleft Palate
Veau’s classifications(1931):
Gr I: Cleft of soft palate
Gr II: Cleft of soft & hard palate
Gr III: Complete unilateral cleft
Gr IV: Complete bilateral cleft
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Classification of Cleft Palate
Kernahan & Stark classification(1958):
Based on embryologic development
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Classification of Cleft Palate
Kernahan symbolic (Y) classification:
Elsahy modification of Y classification:
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Classification of Cleft Palate
Kriens classification:
‘LAHSHAL’ code based on anatomy
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Management principles
Assessment & identification of associated
anomalies
* Birth history
* Examination of Head & Neck
* Nasal endoscopy
* Otolaryngologic assessment
Associated problems of feeding, speech
Parental counseling
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Management principles
Feeding:
Immediate concern
Special feeding bottles
* Squeeze bottles
* Haberman bottle
Care needed to burp
the child after each
feed
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Orthodontic manipulation
Required for cleft of primary palate
Alignment of alveolar arch required before
surgical intervention
eg McNeil, Burston method
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Cleft palate repair -History
Chin (Tsin) Dynasty, Circa 390AD
Cleft palate confused with oronasal fistula
of tertiary syphilis
Philibert Roux (1819)
Dieffenbach’s (1840)
Von Langenbeck
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Cleft palate repair -rationale
Growth
Speech
Feeding and swallowing
Eustachian tube
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Cleft palate repair
Goals of cleft palate repair
* separate oral & nasal cavity
* normalize swallowing
* normalize speech-help of speech therapy
* limit side effect of cleft palate repair-
defective midfacial growth
* prevent tooth loss by alveolar bone graft
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Timing of cleft palate repair
Speech outcome
Maxillary growth
Syndromic child with cleft palate
Lip repair –6 wks to 12 wks
Palate repair –10 to 12 mths
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Techniques of Palatal repair
Von Langenbeck Technique
* uses mucoperiosteal flap
to close defect in sec palate
* old method still used
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Von Langenbeck (cont..)
Adv:
•Can be used for ant palatal repair
Disadv:
•Not suitable for wide defect
•Does not increase velar length
•Shortens growing maxilla
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Push back Technique
Started by Veau, Wardill, Kilner
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35. Surgical Repair-Cleft Palate
Langenback flap
Veau flap
Veau-Wardill –Kilner
flap
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36. Surgical Repair-Cleft Lip
Cleft lip repaired at 10
weeks
Rotation-advancement
method-Most common
Nine Landmarks
Rotation Flap cuts
made first
Advancement cuts
made next
Cleft side nasal ala cuts
made last
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Push back Technique
Adv:
* Can be used for wide cleft defect
* Address the velopharyngeal incompetence
Disadv:
•Cannot address the anterior cleft defect
•Narrow dev of ant alveolar arch
•Limits palatal lengthening
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Furlow Z-plasty
Described in 1980 for palatal closure
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Furlow Z-plasty
Adv:
•Lengthening of velum
•Recreates levator sling
•Hard palate can also be addressed
Disadv:
•Increased operative time
•Technically difficult
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Complications
Hemorrhage
Respiratory problems
Dehiscence
Fistula formation
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Patient support -Speech therapy
Because of surgery
being done so early
hopefully speech will
form correctly but
because the child has
to adapt, therapy is
advised.
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Patient support -orthodontics
as the child gets older
teeth are not straight -
orthodontist for
treatment (braces).
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Conclusions
Cleft Palate are common congenital
deformities that often affect speech, hearing,
and cosmesis; and may at times lead to
airway compromise.
The otolaryngologist is a key member of the
cleft palate team, and is in a unique position
to identify and manage many of these
problems .
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References
Pediatric Plastic Surgery,Mathes,2
nd
edition,
Vol IV.
Otolaryngologic Clinics of North America,
2007
Scott-Brown’s Otolaryngology, 6
th
& 7
th
edition.
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Further Information
??????Cleft Palate Foundation
www.cleftline.org
??????Cleft Lip and Palate Association CLAPA
www.clapa.com
??????Online support group
www.cleftclub.com
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46. Surgical treatment mnemonic
LVF (Left Ventricular Failure)
Langenbeck
Veau
Furlow
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