Download Cleft palate

CLEFT PALATE
INTRODUCTION
2 important factors
1. Speech production
2. Facial growth
 Early repair of the palate results in normal speech, however may cause abnormalities in facial
growth
 Speech difficult to correct late however facial growth can be corrected
 Type of repair also influences growth and speech
Factors
1. Type of flap
2. Whether the levator is reconstructed
3. Post op moulding of the palatal segments
4. Bone grafting of the alveolar segment
HISTORY
500 ad first recorded operation
1552 Jacob Houlier proposed suturing palatal clefts
Ambroise Pare illustrated obturators
1816 Von Graefe is credicated with the first velar repair
!824 IC Warren pioneered cleft closure
1834 Dieffenbach closed the hard and soft palate with mucoperiosteal relaxing incisions
1926 Davis described the bone flap technique
1845 Fergusson detailed the fx of the palatal muscles
1861 Von Langenbeck described mucoperiosteal flaps in closure
Passavant noticed high incidence of VPI in those undergoing Von Langebeck procedures
Gilles and Fry, Dorrance, and Brown all described push back procedures
Kilner and Wardll independantly developed a push back procedure combining the techniques of
Von Langenbeck and Dorrance
Pharyngeal flaps for pharyngoplasty
1874 Schoenborn performed the first superiorly based flap
1927 Kirkham sutured the superior constrictor at the sides of the pharyngeal cavity
1944 Schweckendiek used velar closure for cleft defects
ANATOMY
Embryology
Blood supply
 Hard palate
1. greater palatine artery
2. sphenopalatine artery through incisive canal
 Soft palate
1. lesser palatine artery through soft palate
2. ascending palatine branch of facial artery
3. palatine branches of ascending pharyngeal artery
Nerve supply
Sensory
1. greater palatine nerve
2. nasopalatine nerve
3. lesser palatine nerve
Parasympathetic
1. pterygopalatine ganglion to greater and lesser petrosal nerves
Muscles of the palate
Levator palati
Tensor palati
Palatoglossus
Palatopharyngeus
Muscularis Uvulae
Nerve Supply
Vagus
Mandibular division of CN V
Vagus
Vagus
Vagus
Tensor palati
 Origin
1. scaphoid fossa at the upper end of medial pterygoid plate
2. lateral aspect of Eustachian tube
3. spine of sphenoid
 Passes above the fibrous arch (origin of buccinators), bends around the hamulus
 Insertion
1. anterior border attached to crest of palatine bone
2. Tensor aponeurosis
 Action
1. tenses the palate to allow other muscles to work
2. opens the auditory tube for equalisation
Levator palati
Origin
1. medial aspect of Eustachian tube
2. quadrate area in front of carotid canal
Insertion
 tensor aponeurosis between 2 heads of palatopharyngeus
Action
 Pulls the palate superior and posteriorly to contact Pasavant’s ridge (level with the
anterior arch of atlas)
Palatoglossus
Origin
 under surface of palatine aponeurosis
Insertion
 Interdigitates with styloglossus
Action
1. Raises the tongue
2. narrows the oropharngeal isthmus
Palatopharyngeus
Origin
1. Anterior head - Posterior border of hard palate
2. Posterior head – upper surface of aponeurosis
3. Both heads embrace insertion of levator palate
Insertion
 Thyroid cartilage
 Inferior constrictor
 Hard palate is static and covered by mucoperiosteum
 Soft palate is mobile and functions as a valve to separate nose from mouth for speech and
swallow - all vowels and consonants except m and n
 Foremost pathological feature of the cleft velum is hypoplasia and atypical insertions of the
muscles of velopharyngeal closure, which correlated with severity of the cleft
 Tensor
o contracted / attenuated / cleft edge
o fails to open auditory tube
 levator
o marked hypoplasia
o inserts anteriorly to posterior edge bony ofpalate & cleft margin > VPI / hypoactive
palatal elevation
 Resulting in problems with
1. Hearing loss
2. Suckling
3. deglutition
4. Speech (hypernasality)
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Alveolar cleft is associated with a cleft lip (nearly always)
Cleft hard palate without cleft of soft palate is extremely rare <1%
Clefts of palate accompanying cleft lips tend to be long and narrow
Clefts of the secondary palates tend to be U-shaped or broadly V-shaped
Clefting severity ranges from bifid uvula (2% of population ) to simple notching of hard
palate to complete clefting through the alveolus
CLASSIFICATION
 unilateral cleft palate = vomer on non-cleft side
 bilateral cleft palate - vomer midline island
Veau Classification
Veau I Cleft of the soft palate
Veau II Cleft of the soft and hard palate
Veau III Unilateral complete cleft
Veau IV Bilateral complete cleft
Kernahans striped Y
Each limb has three boxes
Lip:
Alveolus
Primary palate
Hard palate posterior to incisive foramen
Soft palate
Boxes 1 and 4
Boxes 2 and 5
Boxes 3 and 6
Boxes 7 and 8
Box 9
Epidemiology
Classification based on different putative inheritance patterns
1. Nonsyndromic CL/P
2. Nonsyndromic CP
3. Syndromic CL/P
4. Syndromic CP
Important in providing prognostic infomation and genetic counselling
Cleft Uvula
 Commonest (1 in 50)
 Black least, Asians/native American more
 Associated with submucous cleft / VPI / levator dehiscence / ear problems
CL (P) L unilat
 2nd commonest (1:700-1000); racial variation
 M>F 2:1
 Strong genetic prediposition
 Maternal age - sig
 Threatened abortion
 Influenza virus
 Maternal ingestion drugs
CP
 1:2000; no racial variation
 F>M 2:1
 low heritability
 Higher other anomalies (55% syndrome, 300 syndromes associated with oro-facial clefts)
ANTENATAL DIAGNOSIS
Antenatal diagnosis of cleft lip palate is possible with ultrasound
Able to detect by 12-15th week (20% sensitivity)
Important and developing area with the possibility of in utero repair
HEARING IN CLEFT PALATE
Epidemiology and recommendations
 cleft palate is very often associated with eustachian tube dysfunction and a resulting
conductive hearing loss
 Patients with an isolated cleft lip have an incidence of hearing loss similar to that in the
normal population
 High incidence of otitis media (97%)
 increased incidence of cholesteatoma (7%).
 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.
 All should receive early myringotomy and grommet tube placement
 Less impairment of hearing and better consonant articulation
 require multiple sets of tubes from 3-4 months of age until the beginning of the second
decade of life.

With increasing age, the incidence of eustachian tube dysfunction decreases, and in many
cases normal eustachian tube function develops by mid adolescence.
Pathomechanics
 Cause not known. Theories
1. anomalous structure and function
 milking action of levator
 dilation action of tensor
2. inadequate velopharyngeal valving with disturbed aerodynamic and hydrodynamic
relationships in the nasopharynx and proximal portions of the eustatian tube
 The effect of palatal repair on sensorineural hearing in patients with cleft palate is
controversial with some finding improvement and other no improvemnt
 The effects of VPI and its treatment with pharyngeal flaps on otitis media and hearing are
controversial
PATHOLOGICAL CONSEQUENCES OF SPEECH
Brain responds to inability to close velopharynx with compensatory articulations (maladaptive)
Sibilant consonants s,z,sh and ch,j and g
intraora; consonants p,b,t,d, and k
THE SUBMUCOUS CLEFT PALATE
First described by Roux 1825
Calcan listed 3 diagnostic signs
1. Bifid uvula
2. Notching of the posterior border of the hard palate
3. Septum pellucidum - Muscular diastasis of the soft palate with an intact mucosal layer
all 3 signs do not have to be present, hard palate notching is the most comsistent finding
Incidence 0.05 % - 0.1%
Pathogenesis
 Direct result of malposition of the palatal muscle complex
 The levator is displaced anteriorly and inserts into the hard palate
 musculus uvulae is abnormal
 VPI may be a problem (1 in 9 children with submucous cleft) and may occur after
adenoidectomy
 These children with VPI will tend to show a coronal closure pattern
Surgical options
1. Superiorly based pharyngeal flaps are commonly used in the treatment of VPI for submucous
clefts
a. Classical operation is excising the entire region of the zona pellucida, then using a
posterior wall pharyngeal flap
b. does not restore the single, anatomically normal velopharyngeal sphincter but
creates two sphincters on either side of the pharyngeal flap
c. May need palatal retrodisplacement when the palate is felt to be short
2. Palatal pushback and levator repositioning with levator lengthening
a. intravelar veloplasty and palatal lengthening
3. Furlow palatoplasty
a. Patients <20 years of age with a small velopharyngeal gap (<5 mm)
b. circular or sagittal patterns of closure
c. positive response to biofeedback speech therapy
Age of repair may influence results
early repair has better outcome with respect to speech and reduces the incidence of otitis media
Summary(Gosain PRS 1996)
 VPI is the only indication for surgery and thus these patients should be carefully watched
 present literature does not support "prophylactic" operations on patients who present with the
physical stigmata of submucous cleft palate prior to reaching an age at which it can be
demonstrated by perceptual speech assessment that velopharyngeal inadequacy remained
refractory to speech therapy.
 A significant number of patients will never develop velopharyngeal inadequacy; therefore,
surgery would be unnecessary
CLINICALLY
History and examination
Assoc. abnormalities
Difficulty feeding
Cleft kids more likely to be premature and underweight
regurgutation is common esp. initially
Haberman feeder
Special nipples
Squeeze bottle
nasogastric
gastrostomy
Breast feeding post palate repair not recommended at PMH
Goals of palatal repair
1. Separate the oral and nasal cavities
2. Repositioning of the soft palate musculature to anatomically recreate the palate
3. Minimize the restriction of growth of the maxilla in both sagital and transverse dimensions
PATHOLOGICAL SEQUELAE
1. Feeding and nuturitional difficulties
2. Recurrent ear infections and conduction deficits
3. Abnormal speech production
4. Facial growth distortion
MANAGEMENT
Multidisciplinary approach
Medical/surgical
Plastic surgery
Pediatrician
Geneticists
Nursing
Social work
Dental
paedodontist
prosthodontist
Orthodontics
Maxillofacial
Speech and hearing
ENT
Audiologist
Speech therapy
TIMING OF PALATOPLASTY
Goals are
1. Normal speech
2. Normal palatal and facial growth
3. Normal dental occlusion
4. Normal hearing
Timing controversial - early vs delayed
 Argument regarding speech vs growth
 earlier repairs benefit speech development because the speech process in children begins at 1
year of age
 conversely, delayed repairs theoretically allow for proper maxillo-facial growth because
transverse facial growth is not complete until 5 years of age
1. Effect on facial growth must distinguish between CL/P and isolated cleft palate
2. Repair of CL exerts a restrictive growth pattern on the maxilla
3. There is no convincing evidence that palatal repair in patients with isolated CP
further enhances the inherently limited growth potential of the maxilla
a. Conflicting evidence in the literature
4. Little evidence that dental relationships are affected by early versus delayed palatal repair
5. Speech is profoundly affected by the timing of palatal repair
6. Fistulas rates higher with delayed repair (35%) vs early(5%)
7. Better speech with early repair
8. Reduced VPI and need for pharyngeal flaps with early repair especially those with
unattached vomer
9. Delayed repair is easier due to palatal growth thus less dissection required.
10. Closure must be timed according to the particular anatomy and functional needs of the
patient and not just age alone
a. In children with Pierre-Robin sequence, for instance, the timing of palatoplasty is
dependent upon the child’s airway status; it is often prudent to delay primary
closure until 18 months to 2 years of age to minimize the risk of airway
obstruction.
b. Similar strategies are required for patients with other syndromes in which airway
obstruction is an issue, such as Treacher-Collins, Apert, or Crouzon syndromes
SEQUENCE OF OPERATIONS
Common approaches to palate repair
a. Early complete palate repair at 3-9 months
b. Delayed complete repair at 12-24 months
c. Late complete plalate repair at 2-5 years
d. Staged palate palate
i. Delaire – lip and soft palate at 6-9 months, and hard palate at 18 months
ii. Schweckendiek – lip and soft palate before 12 months, hard palate at 12 years
Currently (PMH) early lip repair at 3 months followed be secondary closure of the palate at 9/12
Note fit an orthopedic device as a neonate
OPERATIONS
Options
Incomplete cleft of soft palate
1. intravelar veloplasty
2. Furlow
3. Sommerlad repair
Incomplete cleft of soft and hard palate
1. Von Langenbeck
2. Veau-Wardill-Kilner (3 flap)
Complete cleft of soft and hard palate
1. Von Langenbeck
2. Veau-Wardill-Kilner (3 flap)
3. 2 flap repair
4. 4 flap repair – for bilateral clefts
Principles
1.
2.
3.
4.
5.
Recreate normal anatomy
Nasal layer (vomer flaps may be required to help this closure)
Muscle layer (divide the abnormal insertion and recreate the levator sling)
Oral layer
Tension free closure with broad apposition.
International trends
 97% perform single stage
1. Furlow Z-plasty (34.8%),
2. pushback palatoplasty (30.3%)
3. intravelar veloplasty (20.4%)
Maneuvers to reduce transverse tension across closure
1. Fracture of hamulus
2. Division of tensor medial to the hamulus - Anatomical dissections of the normal tensor
tendon have shown that it is at least partially attached to the hamulus and that division of the
tensor tendon medial to the hamulus should not affect the role of the muscle in eustachian
function.
3. uvular transposition technique (PRS 1999) - recruits tissue for soft palate lengthening from
the uvula and can be performed in conjunction with the Furlow palatoplasty or intravelar
veloplasty. Transposition of uvula mass to the nasal surface of the soft palate results in a
permanent ridge on the palate to assist with velopharyngeal closure. This functionally
shortens the distance required to close the nasal pharyngeal aperture
VON LANGENBECK'S PROCEDURE
Bilateral bipedicled mucoperiosteal flaps
No attempt to lengthen the palate
Parallel incisions one along the cleft and one along the lingual side of the alveolus
Other issues
Pressure equalization tubes (traditionally placed before intubation)
good oral hygiene
Orthodontic work
Genetic evaluation
Informed consent
Method
 Supine, head at end of bed, head down
 Local with andrenaline
 A Dingman mouth guard
 Incise the cleft and reflect nasal mucosa for closure, undermine and free the greater palatine
artery
 Divide the abnormal insertion of the levator of the posterior part of the hard palate
 Dissection in the space of Ernst (Baker uses the microscope)
 Nasal mucosa is closely adherent to the muscle
 May need vomer flap for the nasal mucosa or alveolar closure
 Close nasal mucosa first (anteriorly to posteriorly)
 Soft palate then proceeds posteriorly and proceeds anteriorly
 Incorporate a portion of the muscle in the anterior part of the repair
 Thus the muscle is reorientated
Sommerlad modification (PRS 2003)
 Similar repair but without the lateral releasing incision
 Division of tensor medial to the hamulus
 20% converted to von Langenback repair due to tension
Disadvantages
 chief criticism of the von Langenbeck palatoplasty has been the concern over inferior speech
results (10-60% have VPI)
Important considerations
a. intravelar veloplasty is important with respect to preventing VPI and speech
b. Increased risk of airway obstruction when combined with a pharygeal flap and consdered
unneccessary in the majority at the initial closure of the palate
V-Y PUSHBACK/3 FLAP REPAIR
 Veau
 Closure of the nasal layer separately
 Fracture of the hamular process
 Suture of the muscles of the soft palate
 Staged palatal repair following primary lip and vomer flap closure
 Creation of palatal flaps based on a vascular pedicle
 Kilner-Wardill 1937 modification
 lateral relaxing incisions
 bilateral unipedicled flaps based on the greater palantine artery
 V-Y palatal lengthening
 Some claim better speech outcomes (10-20% VPI) than von Langeback
 Whether the benefits of the push back is permanent is still in question
V-Y pushback with nasal mucosal flaps (Cronin modification)
 intranasal dissection of the nasal mucosa off the bony floor of the nose
 tissue shifted to the nasal side of the soft palate
 better speech results than traditional VY pushback
 chief criticism is the technical difficultly and the potential for growth disturbances due to the
denuded palatal bone.
 may not be possible in every case
 variations on nasomucoperiosteal flaps include use of SSG and Buccal grafts to line the raw
areas to prevent contracture
TWO FLAP REPAIR
 for complete clefts
 similar to the V-W-K repair, but the dissection extends further anteriorly to encompass the
cleft edges at the alveolus.
 A vomer flap is usually required to close the nasal layer
 The two-flap palatoplasty combined with a vomer flap results in a four-flap palatoplasty.
 Buccal flaps have also been used to cover denuded areas of palate
DOUBLE-OPOSING Z-PLASTIES (FURLOW'S TECHNIQUE)
 Useful only for soft palate clefts only.
 Single stage palatal closure technique consisting of double opposing Z-plasties from the oral
and nasal surfaces
 The anteriorly based flap contains mucosa and the posteriorly based flap contains the levator
muscle complex
 The levator complex is reorientated horizontally
 The palate in lengthened with the Z-plasty with the expense of the transverse diameter
 Advantages of the Furlow repair are:
1. it lengthens the palate
2. restores normal muscular anatomy
3. eliminates the need for lateral relaxing incisions to gain tissue for cleft closure.
 A concern shared by many surgeons, including Millard,, is that the Z-plasties in the soft palate
tend to pull the sides of the velum toward the midline to lengthen the palate; this tightens the
velum in the transverse axis.
 Other cleft surgeons have criticized the Furlow procedure as not obtaining an optimal
anatomic reconstruction of the muscles of the velum.
 Good results with respects to hypernasality and nasal escape and speech production
 Problems is with wide clefts and the increased risk of fistula formation because of the
increased tension in the transverse diameter
 Occasionally a vomer flap is necessary to complete the closure.
PALATOPLASTY WITH PRIMARY PHARNYGEAL FLAP
 using a pharygeal flap either inferiorly or superiorly based with donor closed primarily
 belief that the pharyngeal flap permanently and effectively tethers the velum to the posterior
pharynx
 best reserved for cases with tissue deficiency
 low VPI rates
 higher risk of airway obstruction post operation
 does not always prevent aberrant cleft speech problems
VOMER FLAPS IN PALATOPLASTY
 1932 Veau and Plessier first proposed the technique of vomer flap repair
 Primary concern is effect on facial growth as maxillary growth depends on the sliding
action of the vomer along its maxillary surface
 Scar on the vomer affects the sliding action of the palatine bone on the maxilla
 Use of FTSG or mucosal flaps could reduce the growth disturbance
 Used if tissue deficiency
DELAYED HARD PALATE CLOSURE
 Rohrich and Byrd repair the lip and velum at 3 months with secondary closure of the hard
palate at 15-18 months
1. major benefit is that it narrows the hard palate gap so that less extensive hard palate
surgery is required later
2. early soft palate closure allows the normal fx of phonation, deglutination, tongue
position and skeletal morphogenesis
 Schwechendiek closes the soft palate early and delays hard palate closure until 12-15 years
1. Speech results questionable
2. No growth restriction
SURGICAL COMPLICATIONS
 Complication rate 0.5%
EARLY
1. Bleeding
a. Possibly slightly higher incidence of bleeding with the Veau-Kilner-wardill because of
division of the anterior branch or the greater palatine artery
b. Sit child up
c. Analgesia
d. Adrenaline soaked gauze and pressure
e. Return to operating theatre
2. Airway obstruction is considerably more common after a Von Langenbeck procedure with a
pharygeal flap
3. Dehiscence
4. Respiratory tract infection
5. Oral infection
6. Secondary haemorrhage
7. Otitis media
LATE
1. Fistula formation (10-20%)
a. pushback repairs resulted in higher fistula rates than von Langenbeck repairs, which in
turn were higher than Furlow/intravelar veloplasty repairs – may reflect difficulty of the
cleft the operation was used for.
2. VPI
3. poor mid face growth and crossbite
PALATAL FISTULA FORMATION
 Incidence of fistula formation reported from 0-34% (9-50%) – inflated figures as studies
include fistulas anterior to the alveolus which are intentionally left
 Correct incidence probably 6-10%
 Factors:
1. No significant difference between the procedures
2. Surgeon dependant
3. Extent of cleft
4. Type of cleft is a factor in the future development of a fistula
Bilateral clefts(60%) > unilateral cleft lip and palate(26%) > isolated cleft palate(15%)
 Due to incomplete dissection of the flaps, failure to adequately release the muscles from their
abnormal attachment on the hard palate, poor handling of the tissues, closure under tension,
failure to achieve a layered closure in the soft and in the hard palate, and postoperative
bleeding or infections.
 Some small fistulas might close spontaneously, but the vast majority of fistulas do not.
 Fistulas may become symptomatic, resulting in nasal regurgitation of saliva and food
particles, the collection of food within the fistula, and halitosis
 They might also affect speech, resulting in hypernasality, nasal emission, and articulation
problems.
 The size of the fistula does not necessarily correlate with the effects on speech; even small
fistulas (4.5 mm) can have negative effects on speech
 Most common position is anterior hard palate
Approx. 50% pts with a fistula require treatment
Which fistulas require operative intervention?
 controversial
 Jackson believes all should be repaired
 Isberg and Henningsson showed velopharygeal movements are improved or even normalised
with an obturator
Principles of fistula repair
1. Repair as soon as possible to prevent complications but…
2. orthodontic arch alignment first if maxillary segment collapsed
3. mobilisation of local tissue
a. reraise flaps
b. turn over flap
c. rotation flaps
d. Z plasties
4. recalcitrant fistulas can be closed with tongue flaps
a. Can be anteriorly or posteriorly based depending on the position of the fistula
b. anteriorly it is based the rich vascular plexus at the tip of the tongue (the ranine arch)
2.5-3 cm x 6cm
c. flaps usually contain mucosa and muscle
d. placed in IMF for 3 weeks
5.
6.
7.
8.
Buccal flaps
partial buccinator musculomucosal flap posteriorly based
FAMM flaps (Pribaz)
free radial forearm flap esp with large fistulas
a. cutaneous portion of the flap is oriented toward the oral side while a split thickness skin
graft is placed on the nasal surface of the flap
b. pedicle is passed through the maxillary cleft along a subcutaneous tunnel and is sutured
to the anterior facial vessels
 For closure of postalveolar fistulas, particularly in patients with bilateral clefts, avoid using
labial or tongue flaps across the alveolus as these producessuboptimal results and an inferior
habilitation because teeth cannot erupt through those tissues; the aesthetic outcome is also
inferior.
 soft tissue coverage with gingival mucoperiosteal flaps anteriorly and palatal flaps posteriorly
to the alveolar ridge is preferred