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.

1. CLEFT LIP AND CLEFT PALATE
Dr. Ch. B. Prathyusha,
PG ENT
Narayana Medical College
Nellore

2. Introduction
Prevalence
Etiology
Embryology
Classification
Management
Defects and Reconstruction

3. INTRODUCTION
 History of cleft lip is based on a combination of
 religion,
 superstition,
 invention and
 charlatanism.

5. Aztecs of Central Mexico

6. Chinese belief
Eating hare causes “hare lip”

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

10. Anatomy of lip and palate

11. Anatomy of lip

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.

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.

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

22. Primary palate
secondary palate

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

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)

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

30. Classification
 Clefts
 Unilateral or bilateral
 Complete or incomplete
 Davis and Ritchie Classification
 Veau’s Classification

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

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

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

39. Management
 Multidisciplinary approach
 Cleft care team
 Plastic surgery
 Audiology
 Speech pathology
 Otolaryngology
 Orthodontist
 Oral maxillofacial surgery
 Psychologist
 Geneticist
 Pediatrician

40. Management
 Birth
 Airway concerns
 Feeding problems
 Otological disease
 Speech and language problems
 Surgical Repair

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

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

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

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

60. Incision design for alveolar bone grafting

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

63. Cleft palate repair
 Surgical techniques
Bardach two flap palatoplasty
Furlow double opposing z-plasty

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

65. Bardach Palatoplasty
 Closure occurs in 3 layers
 Nasal mucosal
 Muscular layer
 Oral Mucosal

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

71. Joaquin Cannes (1974-
American Actor

72. Thank you