cleft lip and Cleft palate is one of the most common congenital anomalies encountered in ENT and Pediatrics practice. It is important to be familiar with the clinical features and complications, Surgical procedures, timing of surgery and complications associated with the surgeries. this presentation will give you a simple approach towards the same.
7. All these beliefs came to an end
Fabricus ab Aquapendente
(1537–1619)
Anatomist and surgeon
“The Father of Embryology”
William Harvey was his student
8. Introduction
non syndromic clefts
Majority of orofacial clefts are nonsyndromic
70% of Cleft lip +/- palate 50% of CP only
Multifactorial
Clusters in families but not Mendelian
Palate development complex process with
Several proteins, growth factors, and transcription
factors involved
IRF-6, TGF –B2, TGF-alpha
Any disturbance in the process can result in clefting
9. Syndromic clefts
Associated with over 300
syndromes
Van der Woude syndrome – the
most common Autosomal
dominant Lower lip pits
Teratogen exposure
Ethanol, thalidomide, phenytoin
Environmental factors
Amniotic banding, maternal
diabetes, maternal folate
14. Embryology
Face appears to develop at the end of the fourth
week
is completed by the eighth week
The palate is formed by the end of the tenth
week.
15. 4th week
A series of swellings appear around the stomodeum
the frontonasal process,
the bilateral maxillary process
the mandibular process.
The developing nasal placodes arise as thickenings on
either side of the frontonasal process.
16.
17. 5th week
nasal placode is surrounded by a horseshoe-shaped
ridge consisting of lateral and medial nasal processes
with nasal pit in the middle.
The nasal pits deepen and rupture, forming
communications between the nasal and oral cavities,
called primary choanae.
18.
19. 7th week
maxillary processes have advanced medially
underneath the lateral nasal swellings and
fused with the medial nasal swellings.
This complex comprising the fused medial
nasal swellings and maxillary swellings forms
the primary palate
complete by the end of the seventh week
20. Fusion of the paired maxillary prominences
with the paired medial nasal prominences
forms the complete upper lip
(maxillary prominences form lateral lip)
The lateral nasal prominences form the
bilateral nasal ala
24. Primary palate
Medial nasal prominences fuse to form the
primary palate
Consists of maxillary alveolar arch with 4
incisors and the hard palate anterior to the
incisive foramen
Primary palate forms before the secondary
25.
26. Secondary palate
During the 6th week
Shelf-like outgrowths from the
bilateral maxillary processes,
grow vertically down on both
sides of the tongue
During the 7th week
The tongue moves inferiorly and
the palatal shelves migrate to a
horizontal position above the
tongue
Palatal fusion occurs in an
anterior to posterior direction and
completes with uvular fusion (1
week later in females)
27.
28. Cleft lip and palate
Disruptions at any stage of the developmental
process can result in clefts
Timing
Positioning
29. Cleft lip and palate formation
Failure of fusion of the maxillary and medial
nasal prominences unilaterally or bilaterally
result in unilateral or bilateral cleft lip
with/without primary palate
Failure of fusion of the palatal shelves result
in clefts of the secondary palate
31. Davis and Ritchie Classification
divides cleft lip and palate into
2 groups, which subdivided
into the extent of the cleft (eg,
1/3, 1/2), as follows:
Group I - Clefts anterior to the
alveolus (unilateral, median, or
bilateral cleft lip)
Group II - Postalveolar clefts
(cleft palate alone, soft palate
alone, soft palate and hard
palate, or submucous cleft)
32. Veau classification
Class I – incomplete cleft
involving only the soft palate
Class II – cleft involving the
hard and soft palate
Class III – complete unilateral
cleft involving the lip and
palate
Class IV – complete bilateral
33. Unilateral cleft lip
Incomplete
Muscle fibers of the
orbicularis oris are
often intact but
hypoplastic
Varying degrees of
clefting
Complete
Orbicularis oris inserts at the
columella medially and ala
laterally on the cleft side
Columella is displaced to the
normal side
Nasal ala on the side of the cleft
is displaced laterally, inferiorly,
and posteriorly
Nasal tip is deflected towards
the noncleft side
34.
35. Bilateral cleft lip
Orbicularis oris attaches at the lateral cleft margins
bilaterally at the nasal ala
Premaxilla protrusion
Symmetrical nasal deformities
Laterally displaced ala – widely flared
Extremely short columella
36.
37. Incomplete to complete hard palate
Primary palate
Clefts anterior to the incisive foramen
Secondary palate
Posterior to the incisive foramen
Develops due to failure of the palatal shelves to
fuse
Abnormal insertion of the muscles into the medial
edges of the cleft
Vomer attachment is variable
38.
39. Management
Multidisciplinary approach
Cleft care team
Plastic surgery
Audiology
Speech pathology
Otolaryngology
Orthodontist
Oral maxillofacial surgery
Psychologist
Geneticist
Pediatrician
41. Initial management
Airway Management
Isolated cleft palate rarely results in
airway issues usually coexisting
structural abnormalities
Pierre Robin sequence (most
documented)
Micrognathia, glossoptosis, and cleft
palate
Associated with several syndromes
(stickler, velocardiofacial syndrome,
etc.)
42. Feeding difficulties
Critical aspect in management
Nutrition and feeding
Cleft palate limits the ability to suck due
to the common cavity
Cleft lip alone does not cause much
problem
Special bottles
Frequent burping
Premaxillary orthopedics
43.
44. Otological issues
Abnormal insertion of tensor veli palitini
Persistent OME has been estimated to be
between 80-95% in children with cleft palate
45. Speech development
Unrepaired cleft palate – speech abnormality
Primary goal of palate repair is to restore function of
the velopharyngeal valve
normal speech
10-20% will manifest VPI following surgical closure of
the palate
Hypernasality and articulation errors (glottal stops and
pharyngeal fricatives)
Speech pathologist – important role
46. Surgical correction
Age 1-3 months – Lip taping and nasoalveolar molding
Age 3 months - Repair of cleft lip (and placement of
ventilation tubes)
Age 9-12 months - Repair of cleft palate
Age 1-7 years - Orthodontic treatment
Age 7-8 years - Alveolar bone graft
18 years old or skeletal maturity– Midface advancement
47.
48. Pre surgical
Wide cleft lip or premaxilla protrusion
Advantageous to narrow the cleft and
mold the
premaxilla before proceeding with
surgery
Taping
Effective in reducing the width of the
cleft
it is placed with tension across the
49. Pre surgical
Nasoalveolar molding devices
Custom made devices which
utilize wiring and nasal stenting
to mold the nasal cartilage,
premaxilla, and alveolar ridge
Nasal stenting can be elongated
and adjusted to lengthen the
columella and mold the nasal
cartilage
Takes advantage of the
50. Pre surgical
Lip adhesion
Considered when adhesive tapes
and moulds are ineffective
Surgically convert a complete cleft
to an incomplete cleft
Performed at 2-4 weeks with
definitive repair at
5-6 months
Indications
Wide unilateral cleft where
conventional repair
might produce excessive tension
51. Cleft lip repair
Typically performed at 3 months of age
“Rule of Tens”
10 weeks old, 10 lbs, and hemoglobin of 10
Wide clefts or clefts with premaxilla protrusion
that require lip adhesions will have definitive lip
repair at 5-6 months of age
52. Cleft lip repair
Milliard rotation-advancement
technique
widely used
Advantages
Places scar along philtral borders
Allows complete muscular repair
and primary cleft rhinoplasty
Minimizes wasting of normal
tissue
Disadvantage
53. Millard rotation and
advancement
9 landmarks are marked using
blue dye
Rotation flaps cut
first(medially), followed by
advancement flaps(laterally)
Downward and lateral rotation
of medial segment and medial
advancement of the lateral
segment
54. Millards rotation and advancement
Goals
Tensionless closure
Reapproximation of
orbicularis oris
Formation of Cupid’s bow
Creation of a philtrum
Repositioning of nasal ala
Complications
Hypertrophic scarring
Aggressive massage
Nostril stenosis on cleft side
55. Modified delaire procedure
Avoids crossing aesthetically
sensitive areas, such as the
columella base and alar rim
unlike Millards procedure
Incorporation of wavy lines
and small triangular flaps
above the vermillion allow
some degree of lengthening.
important contribution that the
restoration of labiomaxillary
muscle fnction makes towards
56. Bilateral cleft lip repair
Technically challenging
Goals
Symmetry
Orbicularis oris closure
Proper philtral size and shape
Tubercle formation
Positioning of alar cartilages to construct the
nasal tipand columella
57.
58. Bilateral cleft lip
more severe deformity than unilateral.
Signifcant degree of hypoplasia in the midline
prolabium
characteristic feature of complete bilateral clefs is
the marked projection of the prolabium and
premaxilla,
59. Alveolar cleft repair
There are two main ways in which this can be
achieved.
Secondary alveolar bone grafting
at age 9 to 11 years 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
61. Cleft palate repair
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
62. Earlier the detrimental effect that palatal surgery has
on maxillary growth.
Old Traditional methods
Langenbeck flap
Veau flap, Veau-Wardill-Kilner
Not practised now
Scars with exposed bone in the hard palate, have
the potential to inhibit both anterior and transverse
growth of the maxilla.
Modifcations of the Langenbeck flap have been
introduced whereby the flap is designed inside the
greater palatine pedicle in order to move the area of
64. Bardach Palatoplasty
Margins of the cleft are cut
separating
the nasal and oral mucosa
Lateral incisions are made to
the bone
and carried posteriorly
Mucoperiosteal flaps are
elevated
Care must be taken to avoid
damage to
the neurovascular bundle
66. Bardach Palatoplasty outcomes
Keys to optimizing outcomes
Aggressive repositioning of the soft palate muscles
Adequate mobilization of flaps to create a tensionless
closure
Complete palatal closure in one setting
Complications
Fistula3.9 – 29%
(anterior hard palate and the soft/hard palate junction)
Maxillary growth disturbance
VPI
67. Furlow Double Opposing Z-
plasty
Oral mucosa with muscle is raised on the left from the
nasal mucosa and elevated to the posterior
Oral mucosal flap is raised on the right (oral mucosa
and submucosa is raised from the muscle) and elevated
anteriorly
Nasal mucosa/muscle flap is raised on the right and
elevated posteriorly
Nasal mucosal flap is raised on the left and raised
anteriorly
68. Furlow Double Opposing Z-
plasty
Lower flaps with nasal
mucosa are transposed
Upper flaps with oral
mucosa are transposed
Modified for clefts
involving the hard
palate
69. Furlow Double Opposing Z-plasty
Closure of soft palate
and reconstruction of
levator sling
Advantages
Repositions
musculature into a
more anatomical
location
Lengthens the palate
with potential for
improved speech
outcomes
Disadvantages
70. Take home message
One of the commonest congenital treatable
anomaly
Current operative techniques offer excellent
outcome
Multi disciplinary approach required