Cleft Lip and Palate

1
Cleft Lip and Palate

• Christian El Amm, MD
• Plastic and Reconstructive Surgery

2
Normal Anatomy
Columella
Philtral columns
Cupids bow
Vermillion roll
Wet vermillion
Dry vermillion
3
Classification

• Unilateral / Bilateral
• Complete / Incomplete
• Cleft palate / complete / incomplete / Isolated /
  syndromic
• Cleft lip and palate

4
Cleft Lip
Simonarts band
Complete
Incomplete
5
Complete Cleft Lip
Severe nasal deformity
Cleft anterior palate primary palate up to
incisor foramen
Cleft alveolus and gingiva
Complete cleft lip
6
Incomplete cleft lip
Milder nasal deformity
May have a notch in the alveolus
Simonarts band
Incomplete cleft lip
7
Cleft Palate
8
Cleft palate
Incisor Foramen
R
L
Primary palate
Secondary palate
Hard
Soft
Uvula
9
Cleft Palate
VOMER
10
Bilateral Cleft Lip
Prolabium
Premaxilla
11
Bilateral Cleft Lip and Palate
Absent columella
Prolabium
Premaxilla
Vomer
Palatal Shelf
12
Collapse of the lateral segment due to cheek
pressure
13
Cleft palate
Incomplete cleft palate
Unilateral complete cleft lip and palate
Complete Cleft Palate
Bilateral complete CLP
Collapse of both lateral segments of the palate
due to cheek pressure The premaxilla is unable
to move into its right position Premaxillary
lockout
14
Embryology
6 weeks gestation (human) 13 somite stage
(mouse)
15
Embryology
Sperber Clefting occurs because of failure of
fusion of MNP (medial nasal process) and
maxillary process (MxP)
16
Embryology
Carstens and Walters Clefting occurs because of
failure of Rhombomere r2 to migrate. This better
explains the clinical observation that the most
severe deficiency is in the lateral nasal area
17
Embryology
Proposed migration path of r2 the rhombomere
process migrates towards the free margin of the
lip and gingiva before continuing cephalad
towards the lateral nose
18
Embryology
Gene activation during differentiation and
migration
19
Embryology and Genetics
20
Embryology and genetics
21
Treatment
Priorityrestore the Levator Veli Palatini
muscle sling
22
(No Transcript)
23
Furlow double opposing Z-plasty
Z-plasty results in longer palate
24
Treatment
Treatment sequence of complete cleft lip and
palate First, get the segments in alignment by
pre-surgical orthodontics, then perform lip and
gingiva repair. Typically this occurs during the
first months of life (3 months)
25
Palate repair is a separate stage Typically
before the age of one year. The levator muscle
should be ready and mobile (free of scarring) for
the phase of speech acquisition 15-18 months
26
Surgical aims in cleft lip repair

• Reposition ala
• Restore nasal floor
• Lengthen columella on cleft side
• Lengthen medial lip segment (typically, lateral
  lip segment has enough length)
• Reconstitute symmetrical vermillion roll
• Restore dry vermillion medially (typically,
  lateral segment has enough dry vermillion)
• Align wet vermillion to dry vermillion line (wet
  to dry line
• Realign and correct abnormal insertion of
  orbicularis oris muscle
• Reconstitute philtral column (typical by placing
  the scar at the philtral column site)

27
(No Transcript)
28
Millard
Randall-Tennysson
29
Techniques of cleft palate repair

• Von-Langenbeck with Intravelar Veloplasty linear
  scar with muscle alignement (see previous slides)
• Two-Flap palatoplasty with IVV
• Furlow double opposing Z-plasty (previous
  slides)

30
Secondary deformities

• Velopharyngeal incompetence

31
Velopharyngeal incompetence
32
VPI pharyngeal flap
33
Dental eruption

• Lateral incisor absent in 70 cases
• Canine tooth absent or abnormal in 15 (?) of
  cases
• Canine tooth can be successfully erupted through
  cleft once the cleft alveolus is bone-grafted
• Implants-Orthodontics

34
Growth Maxilla and mandible

• Higher prevalence of class III occlusion
  (maxillary retrognathia) The maxilla is
  underdeveloped, due to surgical and/or congenital
  etiology
• Growth restriction highly correlated with
  surgical technique scarring, incisions and
  denuded bone.
• Can be corrected by maxillary advancement (lefort
  I or lefort III)

35
(No Transcript)
36
Technique used at OU
Immediate results scar will fade
37
Long term
38
Craniofacial Malformations

• Craniosynostosis
• Craniosynostosis syndromes
• Mandibulo-Facial Dysostosis
• Hemifacial Microsomia and Oculo-Auriculo-vertebral
  Syndrome

39
Craniosynostosis
40
Craniosynostosis

• Sagittal Scaphocephlay (boat)
• Unicoronal Plagiocephaly (twisted)
• Bicoronal brachycephaly (short)
• Metopic trigonocephaly (keel)
• Most commonsagital
• Biggest differential deformational
  plagiocephaly or positional plagiocephaly
  non-synostotic

41
Trigonocephaly
42
Scaphocephaly
43
(No Transcript)
44
Craniosynostosis syndromes

• Associated with midface abnormalities Base of
  skull synchondrosis
• Associated with finger/toe malformation often
  diagnostic
• Other associated malformations

45
Major Craniosynostosis syndromes

• Crouzon normal hand
• Aperts Major syndactyly
• Pfeifer broad thumbs
• Muenkes syndrome First genetic-based diagnosis
  FGFr-3 mutation predictably causing bicoronal (or
  unicoronal) synostosis

46
Crouzon
47
Aperts
48
Pfeiffers
49
Madibulo-facial dysostosis

• Treacher-collins syndrome
• Nagers

50
Treacher Collins
Autosomal dominant Variable expression Zygomatic
arch, masseter, mandible, side of mouth variably
affected