Transcript CLEFT LIP and palate

‫بسم هللا الرحمن الرحيم‬
‫”وقل رب زدنى علما “‬
Dr: IMRANA ZULFIKAR
ASSISSTANT PROFESSOR
Surgical dept:
Cleft:
Clefting is deformity, in fusion of
mid face skeleton with soft
tissue, occurs in second month
of pregnancy.
Cleft lip and palate(1:600 live birth).
Cleft palate alone (1:1000 live birth)
• Incidence:
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Cleft lip and palate------45%, F > M
Cleft lip alone-------------15%
Cleft palate alone-------40% M>F
Cleft lip and palate is more common on
the left side
• Inc: in oriental groups (1:500)
• Dec: in negro population (1:2000)
• ETIOLOGY:
Genetic :
• Family history: 1st degree affacted have risk
of(1:25 live birth).
• Isolated:
• Syndrome: over 150 syndroms are associated
Pierre robin syndrome, Syickler syndrome
Shprintzen, Down, apert, treacher collins.
Environment:
• Maternal epilepsy
• Drug ( steroid, phenytion, diazepam)
Genetic factors
• Syndrome vs. sequence
• Pierre Robin sequence
– Small mandible (micrognathia) causes failure of tongue to
descend
– Failure of the tongue to descend causes cleft
– Heart problems, conductive hearing loss, developmental deficits
– about 40% of infants with Pierre Robin have Stickler Syndrome
and about 15% have Velocardiofacial Syndrome
• Treacher Collins syndrome
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Under-developed cheekbones with scalp hair
Malformed ears
Conductive hearing loss
Normal cognitive functioning
Chromosomal abnormalities
• Trisomy 13
– Rare chromosomal
abnormality
– Multiple congenital
malformations
– Mean life expectancy is
130 days
Environmental factors
• Recognized teratogenic agents
– Drugs
– Aspirin overdose
– Acne medications
– Alcohol overdose
– EPILEPTIC DRUGS
– PHENYTOIN
Mechanical factors
• Intrauterine crowding (tumors, multiple
births, placenta abnormalities)
• AMNIOTIC BAND SYDROME
EMBRYOLOGY
• When the fetus is developing, the face forms by
the fusion of five different facial processes, that
must fit together perfectly to create a complete
face.
• Frontonasal process.
• Maxillary process
• Mandibular process.
• Medial nasal process.
• Lateral nasal process.
When two of these processes fail to come
together, for whatever reason, a cleft is formed
Physiology of palate:
• Separate the nasal cavity from oral cavity.
• Soft palate cuts off oropharynx from
nasopharynx during feeding.
• Palate generating suction with their
mouths, during feeding the infant.
• During speech, closure of soft palate is
essential.
• an intact palate with normal muscle
function is necessary for proper drainage
of the middle ear.
classification
• Cleft lip and palate
• Cleft lip alone
This separation can vary from a subtle
notch or groove to a wide gap.
• Cleft palate alone
Cleft palate occur in midline
(isolated) extend to uvula, soft
palate, hard palate, incisive foramen
LAHSHAL
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L CLEFT LIP
A ALVEOLUS
H HARD PALATE
S SOFT PALATE
lahsl
Cleft lip and palate
Classification systems
Numerous classification systems:
• Veau
• American Cleft Palate-Craniofacial Assn (ACPA)
• Kernahan and Stark
Veau’s system:
I Cleft of soft palate only
II Cleft of hard and soft palate to incisive foramen
III Complete unilateral cleft of soft and hard palate and lip
and alveolar ridge on one side
IV Complete bilateral cleft of soft and hard palate and lip
and alveolar ridge on both sides
• Anatomical defects:
Cleft lip:
Nasolabial and bilabial muscle rings
disruption results deformities involving
mucocutanous tissue, muscular and
bony deformity.
Unilateral:
Muscle rings disrupt on one side results
asymmetrical deformity.
Bilateral :
Bilateral disruption produce symmetrical
deformity.
Complete CL:
A gap goes all the way from the lip, mouth
into nose, called Complete cleft.
Incomplete CL:
A gap does not extend from mouth all the
way into nose, called an Incomplete cleft.
Microform CL:
Incomplete cleft is very minor (a groove or
a notch), called a Microform cleft.
Cleft palate:
• Failure of fusion of two palatine process.it may
involve:
• Soft palate
• Hard and soft palate.
Hard palate may be:
Complete:
nasal septum and vomer bone completely
separated from palatine process.
Incomplete:
cleft of hard palate remains attached to nasal
septum and vomer.
Antenatal diagnosis:
Only possible for cleft lip by US after
18wk of gestation.
Postnatal diagnosis:
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Immediate problems:
feeding problem
breathing problem
Associated anamolies.
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Late problems:
 Recurrent serous otitis media
 Hearing
 language delays
 speech problem
(hypernasality, articulation error )
 Orthodentic complication
(delayed development. Delayed eruption,
morphological abnormalities, hypodontia,
hyperdontia)
 Cosmetic disfigurement.
 psychological problems.
Primary management
Feeding problem manage :
Breathing problems manag
Primary surgical procedure
• Feeding problem management:
Mother advised:
• Breastfeeding is unlikely to be
unsuccessful.
• Bottle feeding with soft plastic bottle
(squeezed rhythmically to squirt milk into
oral cavity).
• Position of baby should upright, prevent
nasal regurgitation.
• Breathing problems management:
• Nursing the in prone position.
• Persistent airway compromise managed
by “retained nasopharyngeal intubation”.
• Labioglossopexy in first few days is
alternative.
Primary surgical
procedure
Goals of surgery:
• Normal appearance of lip, nose, face.
• Normal speech
• Dentition and facial growth within normal
range of development.
Principals of surgery:
• To restore the normal anatomy by
muscular reconstruction.
Timing of primary
surgical procedure
CL repair is performed B/W 3-6
month of age.
CP repair is performed B/W 618month of age.
• Cleft lip alone: one operation
• Unilateral:
• Bilateral:
5-6 month.
4-5 month.
• Cleft palate alone:
• Soft palate only: 6month
• Soft and hard palate: Two operation
Soft palate at 6month
Hard palate at 15-18 month.
Cleft lip and palate:
Unilateral and Bilateral: Two operation
- Cleft lip and Soft palate at 5-6 month.
- Hard palate and gum pad with or
- without lip revision at 15- 18 month
Secondary
management
Hearing problems
Speech problems
Dental development
Facial growth
Hearing management:
Sensoneuranal hearing loss:

with hearing aid
Conductive loss due to serious otitis media

Prophylactic myringotomy

grommet insertion.

Regular autological testing.
• Speech problem management:
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Speech and language therapy
Secondary palatal surgery
Intralveolar veloplasty
Pharyngoplasty
Speech training devices.
• Orthodontic management:
• In two phases
1. mixed dentition (8-10 yrs) to expend
maxillary arches by alveolar bone graft.
2. Parmanent dentition (14-18 yrs) to
aligndentition by maxillar osteotomy.
• Cleft lip revision surgery:
• Indication:
• Lip deformity:
-Misaligned vermilion
-Asymmetrical or muscle discontinuity.
• Nasal deformity:
-Lateral drift of alar base
-Poor nasal tip projection
-Deviation of cartilaginous nasal
septum.