Cleft Lip and Palate - Presentation.
Cleft Lip and Palate is the 2nd most common Congenital Anomaly after Clubfoot. This presentation goes in depth about the Presentation, eitiology, Genetics, Medical management, Nasoalveolar Moulding, Surgical management of Cleft Lip & Palate
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Cleft Lip and Palate
1. Cleft Lip And Palate
Chairpersons :
Dr. Mohan G
Dr. Vijay Kumar
Dr. Sunil Kumar
Dr. Yogitha
Presenter : Dr. Vikas V
2. Introduction
Cleft lip and palate is the 2nd most common congenital
anamoly after clubfoot
Clefts of the lip, alveolus and hard and soft palate are the MC
Congenital Abnormalities among 15 of the orofacial structure
anomalies.
Frequently occur as isolated deformities but can be a/w other
medical conditions, particularly CHDs.
Associated feature in over 300 recognised syndromes.
3. History
Hippocrates (400 BC) and Galen (150 AD) mention cleft
lip, but not cleft palate in their writings
Cleft palate – Fanco (1556)
Repair of cleft lip – as early as 255-206 BC in China
The first successful closure of a soft palate defect was
reported in 1764 by LeMonnier, a French dentist.
4. Incidence
Cleft Lip + Palate – 1:600 LB
Isolated Cleft Palate – 1:1000 LB
Typical distribution of cleft type:
cleft lip alone: 15%
cleft lip and palate: 45%
isolated cleft palate: 40%.
CL/P – Males ; CP – Females
A child is born with a cleft somewhere in the world every 2 minutes
according to a WHO study published in 2001
In India alone the number of infants born every year with CLP is
28,600
78 affected infants are born every day, or 3 infants with clefts born
every hour
6. Embryology
Development of facial
structures starts at
end of 4th week
5 facial prominences around stomatodeum
1. Unpaired frontonasal process
Nasal Placodes – Invagination – Nasal Pits
Medial Nasal Process
Lateral Nasal Process
2. Paired maxillary prominences
3. Paired mandibular prominences
7. Development of Lips
In following 2 weeks –
2 medial nasal processes fuse in midline – upper lip
Mandibular processes fuse in midline – lower lip
8. Development of Lip and Nose
The maxillary and lateral nasal process separated by nasolacrimal
groove/duct
Frontonasal process – bridge of the nose
Medial nasal process – tip of nose and philtrum of upper lip
Lateral nasal process – ala of the nose
9. Development Of Palate
Primary palate –
maxillary and medial nasal process merge
containing the premaxilla, alveolus,and lip, is anterior to
the incisive foramen
Formation of intermaxillary segment from merged medial
nasal prominences
10. Development Of Palate (Contd)
Secondary palate –
formed from 2 outgrowths from maxillary prominences i.e
palatine shelves
containing the uvula, palatal musculature, maxilla, palatine
bones, and pterygoid plates
Fuse in midline at 7th week
Incisive foramen –
midline landmark between primary and secondary palate
contains the nasopalatine vessels and nerve
14. Aetiology --> 1] Genetic
Family history : First-degree relative affected increases
the risk to 1:25 live births.
Genetic influence ---> more significant in Cleft lip/Palate ;
Environmental factors ---> Isolated cleft palate
Role of TGF-β3 in Palatal Fusion : Expressed in MEE
Expressed by Medial Edge Epithelial cells
Homozygous null TGF-β3 --> Cleft Secondary Palate
Defect in Wnt9b Signaling
Insufficient Growth of Maxillary prominces
Mutation in FOXE1 gene
Expressed at point of Fusion b/w Maxillary & Nasal Process
16. Classification of Cleft Lip
Cleft Lip can be classified by two main characteristics:
Unilateral v/s Bilateral deformity &
Complete v/s Incomplete deformity
Through-and-through /Partial Penetrance involvement of the lip,
alveolus, and nasal sill.
Incomplete cleft lips may be further characterized as
Minor form (defect extending 3 mm or more above the normal
Cupid’s bow peak),
Microform (notch less than 3 mm above the normal peak),
Minimicroform (disrupted vermilion cutaneous junction without
elevation of the bow peak),
19. Classification of Cleft Palate
Cleft palate may be defined as:
(1) Unilateral or Bilateral
depending on laterality of involvement of the primary palate
(2) Complete or Incomplete
Complete:
extends from the Alveolar ridge to the uvula
Involves primary and secondary palates.
Incomplete :
involves the secondary palate only,
Submucous cleft palate
Bifid Uvula
21. Veau’s classification (1931)
CLEFT LIP
Class I : U/L notching of vermillion border, not extending
into the lip.
Class II : U/L cleft extending into the lip, but not including
the floor of the nose.
Class III: U/L extending into the floor of the nose.
Class IV: Any B/L cleft of the lip, whether incomplete or
complete.
23. Veau’s classification (1931)
CLEFT PALATE
Group 1 – Cleft involving soft palate only
Group 2 – Cleft of Hard and Soft palate extending upto
incisive foramen
Group 3 – Complete Unilateral clefts involving soft
palate, hard palate, lips and alveolar ridge
Group 4 – Complete Bilateral clefts affecting the soft
palate, hard palate, lips and alveolar ridge
24.
25. Fogh-Andersen’s classification (1942)
1. Harelip (single or double)
2. Harelip with cleft palate
3. Isolated cleft palate
4. Rare atypical clefts, e.g., median cleft lip
26. Kernahan and Stark’s classification (1958)
Pictorial Representation of Cleft Lip & Palate
Upper Limbs –
Represent – Rt & Lt sides of Primary palate
Lip, alveolus, hard palate anterior to Incisive Foramen
Lower Limb -
Represent – Secondary Palate
Hard & Soft Palate post to Incisive Foramen
Limitation:
Clefts of Secondary Palate cannot be classified to Left or
Right
29. Facial Growth in Cleft Lip & Palate
Prenatal Growth
Various forces which influence the facial growth in utero are:
A] Over maxillary segment on non-cleft side:
Pull of lip and cheek muscles
Tongue pressure
Relatively unstrained nasal septum growth
B] Over maxillary segment on cleft side:
Instrinsic Deficiency
Pressure from alar base due to stretching of the nostrils.
30. Facial Growth in Cleft Lip & Palate
Due to above mentioned forces, deficiency produced in cleft lip and
palate babies are:
A] Incomplete unilateral cleft lip and palate:
Severe deviation of midline away from cleft.
Smaller maxillary segment shows retro-positioning or growth
inhibition and collapse.
Nose is deviated towards normal side.
B] In bilateral cleft lip and palate cases
Premaxilla tilts forward and/or shifts to one side due to tongue
pressure.
31. Prenatal Diagnsois
Prenatal ultrasound – 2D or 3D
Usually made after 16-20 weeks of
Gestation
Prenatal counselling
Prenatal Detection Rates:
22% to 33% rates for detecting facial clefts
73% - fetal cleft lip
1.4% - isolated cleft palate
Color Doppler ultrasonography can also be used
32. Babcock and McGahan (1997)
Starts with coronal plane
Assessment continues in axial view
Bilateral clefts: sagittal view • Isolated clefts: axial view
41. Overall Cleft Treatment Plan
TIMING AND SEQUENCING OF
CONSERVATIVE SURGICAL-
ORTHODONTIC TREATMENT
42. Stages in Management
Management of cleft lip and palate can be divided into
following stages:
Stage I - treatment done from birth to 18 month of age
Stage II - from 18th month to 5th year of life
( primary dentition stage)
Stage III - treatment carried out during mixed dentition stage
from 6th to 11th year of life
Stage IV - treatment done during permanent dentition stage
( 12-18 years )
43. Stages of Management
Stage I treatment Includes:
1] Fabrication of a passive obturator
2] Pre surgical orthopedics
3] Surgical management of cleft lip
4] Surgical management of cleft palate
Passive maxillary obturator:
Intraoral prosthetic device
Fills the palatal clefts
Provides false roofing against which child can suckle
Reduces the feeding difficulties like insufficient suction, choking,
excessive air intake
44. • Obturator is fabricated
using cold cure acrylic
after selective blocking
of all the undesirable
undercuts
• Clasp aid in retention
45. Pre Surgical Orthopaedics
Nasoalveolar Molding
1. Creation of good functioning palate.
2. Normalize tongue position.
3. Help in speech development.
4. Improve symmetry of nose and cleft of maxilla.
5. Psychologically boost patient and parents as the patient get
continued supervision.
46. Presurgical alveolar and nasoalveolar molding
Alveolar molding :
External taping with or without dental plate
Strip of Micropore tape is placed across the cleft to
approximate the upper lips.
Objective :
simulate effects of an adhesion cheiloplasty
reposition the maxillary segments into proper alignment
Dental plate
47. Forces That Exert an Influence on Cleft Alveolus
Central tongue force, pushing the cleft laterally,
Tongue in the cleft alveolus, preventing approximation.
Anteriorly, the Muscle pulls laterally
Cheek pressure pushes posteriorly on the palate.
48. Naso-Alveolar molding
Liou’s method
Molding Bulb attached to a Dental Plate
Mold the nose along with External Taping of the Lip
The force from taping and counterforce from the molding bulb
provide the combined force necessary to bring the Alveolus into
proper position
Nasal & alveolar molding --> done at the same time
Time frame : approximately 3 months
Overstretching of the nasal cartilage on the cleft side
Nasal projection & dental plate adjusted every 1–2 weeks
50. Grayson’s Method -
Nasal molding After alveolar approximation to avoid overstretching the nasal
cartilage
Appliance consists of
An acrylic or Resin plate which fits over Maxillary Dental arch
An acrylic retention arm or button
A Nasal stent
Micropore tape applied to the lips and connected with orthodontic elastics hold the
dental plate to the palate
Gradually adding soft resin on the inner surface of the buccal flange approximates
the alveolar shelves.
Nasal projection is added and adjusted underneath the deformed cartilage
Started within the first 2 weeks after birth
Monitoring is required every 1–2 weeks, for a period of 3–6 months to complete it
54. Surgical Cleft Lip Repair
Primary closure of lip - Age of 3 months or 10 weeks when
child is fit to undergo General Anaesthesia
Millard has suggested “RULE OF TEN”.
10 weeks of age
10 pounds (4.54 kg)
10 gram% - Blood haemoglobin not less than 10g%
Four techniques have been proposed:
a) Straight Line Repair
b) Quadrangular Flap
c) Triangular Flap
d) Rotation Advancement Flap – Millard’s Repair
55. A. Straight-line repair
●
1. Historically, the first cleft lip repairs relied
on freshening the edges of the cleft and
suturing them together. These have been
largely replaced by various Z-plasty-based
techniques.
●
2. Rose-Thompson repair
– a. Modified straight-line repair
used for minor clefts with lip length nearly
equal on both sides of cleft (e.g., forme fruste).
– b. Fusiform excision with straight-line closure
56. B. Quadrangular flap
●
B. Quadrangular flap
– Proposed by LeMesurier
and Hagedorn.
– Cupid's bow is derived
from the lateral lip.
– 90-degree Z-plasty.
– Violates Cupid's bow
and philtral dimple.
– Has a tendency to
produce a long lip
57. C. Triangular flap
●
Initially described in 1952 by Tennison
●
In 1959, Randall described a
mathematical approach
●
Conceptually similar to the rotation
advancement repair.
●
Difference - rotation back- cut in the
noncleft segment is performed more
inferiorly, closer to the vermilion border
58. D. Rotation Advancement- Millard’s Repair
●
The rotation advancement repair
of the unilateral cleft lip deformity as
described by Millard is the most commonly
used method of repair
59. Markings for Millard’s Technique
1 to 2 = 1 to 3 = 2-4 mm
2 to 6 = 8 to 7 = 20 mm
2 to 4 = 8 to 10 = 9 -11mm
3 to 5 + x = 8 to 9
1) Center/Low Point of Cupid’s Bow
2) Peak of Cupid’s Bow on NCS
3) Peak of bow on medial Cleft
4) Alar Base, NCS
5) Columellar Base, NCS
6) Commisure, NCS
7) Commisure , Cleft Side
8) Peak cupid’s bow lateral side of Cleft
9) Superior extent of advancement flap
10) Alar base, Cleft side
Point X – Back Cut Point
61. Rotation advancement: Technique
●
Make markings: from x to 5 to 3 (rotation flap)
– From point 8 to point 9
– Dissect skin off orbicularis on either side
of cleft
– Bilateral gingival sulcus incisions made to
cleft margin
– Cheek soft tissue elevated off maxilla
above periosteum (caution w/ infraorbital N)
– Free orbicularis from attachments to
columella/ alar margin
– Incise along alar margin from 9 to 10
– Elevate c flap
62. Rotation advancement: Closure
●
Close intraoral mucosa
●
Close orbicularis serially
●
Cleft alar base medialized with stitch to
periosteum of nasal spine
●
C- flap rotated into columellar defect
●
Close nasal floor
●
Inset/sew flaps
●
Nasal correction at same time
65. Advantages
●
Main Advantage : Flexibility and application of Surgery
●
Relies on a “cut as you go” strategy that allows continuous
modifications during the design and execution of the repair.
●
It does not adhere to strict geometrical principles or
measurements.
●
The suture line approximates a new philtral column.
●
The aesthetic philtral subunit is not violated, and this tends to
create a scar that is more camouflaged.
●
Minimal tissue is discarded during the rotation advancement
technique, and this tends to put less tension on the closure.
66. Postoperative care
●
A. Orders
– 1. Arm restraints for 3 weeks to prevent disruption of repair.
– 2. Specialized nipple/bottle to decrease sucking effort
when bottle-feeding.
– 3. Breast-feeding is controversial; based on surgeon
preference.
●
B. Leave Steri-Strips in place over the incision for
reinforcement.
●
C. Follow up in 1 week for suture removal if nonabsorbable
skin sutures were used.
67. Complications of cleft lip repair
1) Failure to Reconstitute Competent Oral Sphincter
• Inadequate reapproximation of the orbicularis oris
• Visible muscle bulge - readily apparent under the skin of
the repaired lip on dynamic motion.
2) Inaccurate alignment of Vermilion-Cutaneous Juncn
• Small step-off deformity Visible to the untrained eye
3) Vertical scar contracture or inadequate rotation.
• shortening of the lip segment
• notch in vermilion – Whistle Deformity
4) Scar contracture - narrow nostril sill – Vestibular Stenosis
5) Wound dehiscence & Scar widening
68. B/L Cleft Lip Repair
●
Repair of bilateral cleft lip is essentially the same, except
that the premaxilla which is projecting has to be pushed
back.
●
Steps:
– Labial dissection -Alveolar closure - Labial closure
– Nasal dissection & positioning Lower Lateral cartilages
72. Alveolar Cleft repair
●
There are two main ways in which this can be achieved.
– Multi Staged Approach
●
Secondary Alveolar bone grafting @ Age 9-11 yrs
●
Preceded by primary soft-tissue closure with a
Vomer flap at the time of primary lip repair,
●
Although the alveolar cleft may be left unrepaired at
the time of primary surgery.
– Primary gingivo-periosteoplasty (GPP) at either the
time of palate closure or delayed until the age of 3 to 5
76. MANAGEMENT OF CLEFT PALATE
●
Primary goals
– Separate the nasal cavity from the oral cavity
– Creation of velopharyngeal valve for swallowing and speech
– Preservation of midface growth
●
Timing (Controversial )
– Speech outcomes improved with early closure
– Midface growth maybe hindered by early closure (2 stage palate
repair)
– Most repaired between 8-12 months of age to minimize speech
abnormalities ( Nasal Speech)
– Closure of the hard palate defect can be deferred till the time of
secondary dentition.
77. LANGENBECK’S OPERATION
●
Margins of the cleft are pared, the nasal septum is defined
and separated off the upper surface of the cleft palate.
●
Mucoperiosteal flaps lifted up from the hard palate
●
Two release incisions are made one on each side just
medial to the alveolar margins.
●
Mucoperiosteal flaps mobilized till release incisions reached
●
Cleft repaired in midline by bringing mucoperiosteal flaps &
Nasal mucosa medially.
●
Repair is done in Three layers –
– Nasal mucosa and Soft palate muscles with
interrupted catgut sutures
– Mucoperiosteal flaps with interrupted silk.
78. Von Langenbeck Repair.
A) Relaxing incisions
are made behind the
alveolar ridge,
creating bilateral
bipedicle flaps for
midline closure. The
greater palatine
vessels must be
preserved.
B)The cleft margins are
incised in a manner
to leave adequate
nasal mucosa for
complete closure.
C)Closure of the nasal
mucosa and muscle
repair.
D)Final appearance.
A B
C D
79. WARDILL’S OPERATION ( Four flap operation)
●
This operation differs from the previous one in three aspects.
– i) The two flaps of the cleft palate are divided obliquely up to the releasing
incisions.
●
Four flaps are available.
●
Anterior two flaps brought to the midline and sutured.
●
Posterior flaps brought to the midline, Also pulled posteriorly to lengthen
Palate.
●
Lengthens the palate, Space between Oropharynx & Nasopharynx is
diminished.
– ii) Tension at the suture line is reduced further by
●
breaking Hamulus process of pterygoid bone on each side
●
thus relaxing the tensor palati muscle.
– iii) Pharyngoplasty is added.
●
Aimed at reduction of the diameter of the nasopharynx
●
Makes Ridge of Passavant prominent.
80. V-Y pushback (Veau–Wardill–Kilner)
A) Design of anterior “W”
incision.
(B) Elevation of bilateral
mucoperiosteal flaps
based on palatine
vessels. The levator veli
palatini muscles are freed
from the posterior border
of the hard palate.
(C) The muscles are
repaired across the
midline of the soft
palate.
(D) The “Y” closure
creates additional length
but also leaves large raw
areas bilaterally.
81. Pharyngoplasty
●
Children with repaired cleft palate may
have a resulting condition referred to as
“VPI” (Velopharyngeal Incompletence).
●
This means that too much air escapes through the nose
during speech,resulting in nasal speech.
●
This occurs because the repaired soft palate is too short or
does not move adequately.
●
In this operations,some of the tissue from palate and back
of throat are repositioned to help close off the escape of air
through the nose
82. RECENT ADVANCES
●
Fetal surgery – done in intrauterine life (prior to 20 weeks)
●
Non-life threatening defects like cleft lip, cleft palate, Pierre
Robin syndrome, Treacher-Collins syndrome, craniofacial
microsomia
●
Open fetal surgery
●
Feto-endoscopic approach
83. CONCLUSION
●
Oral clefts are the second most common congenital anamoly, having
multifactorial origin.
●
A considerable knowledge about the etiology and embryology is
required for proper diagnosis and treatment planning of such patients
●
Treatment begins soon after birth and continues till adulthood
requiring a team approach.
●
Multiple problems and syndromes with clefts
●
Multidisciplinary approach for management, patient-centered
●
Variations in treatment sequence
●
Role of orthodontists.