Cleft Lip and Palate

Christian El Amm, MD
Plastic and Reconstructive Surgery
Normal Anatomy
Columella

Philtral columns

Cupid’s bow

Vermillion roll

Wet vermillion

Dry vermillion
Classification

 Unilateral / Bilateral
 Complete / Incomplete

 Cleft palate / complete / incomplete /

Isolated / syndromic
 Cleft lip and palate
 Cleft Lip

Simonart’s
band

Complete Incomplete
Complete Cleft Lip

Severe nasal deformity

Cleft anterior palate = “primary

palate up to incisor foramen

Cleft alveolus and gingiva

Complete cleft lip

 Incomplete cleft lip

Milder nasal deformity

May have a notch in the alveolus
Simonart’s band
Incomplete cleft lip
Cleft Palate
Cleft palate
Incisor Foramen
R L

Primary palate

Hard Secondary palate

Soft

Uvula
Cleft Palate

VOMER
Bilateral Cleft Lip

Prolabium

Premaxilla
 Bilateral Cleft Lip and Palate
Absent columella

Prolabium

Premaxilla
Palatal Shelf Vomer
Collapse of the
lateral segment due
to cheek pressure
 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”
Embryology

6 weeks gestation
(human) ~ 13
somite stage
(mouse)
Embryology

Sperber:
Clefting
occurs
because of
failure of
fusion of MNP
(medial nasal
process) and
maxillary
process (MxP)
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
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
Embryology
Gene
activation
during
differentiation
and migration
Embryology and Genetics
Embryology and genetics
 Treatment

Priority=restore the Levator Veli Palatini muscle sling

Furlow double opposing Z-
plasty

Z-plasty results in longer palate

 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)
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
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)
 Millard

Randall-Tennysson
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)
Secondary deformities

 Velopharyngeal incompetence
Velopharyngeal
incompetence
VPI: pharyngeal flap
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
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)
Technique used at OU

Immediate results: scar

will fade
Long term
Craniofacial Malformations

 Craniosynostosis
 Craniosynostosis syndromes

 Mandibulo-Facial Dysostosis

 Hemifacial Microsomia and Oculo-

Auriculo-vertebral Syndrome
Craniosynostosis
Craniosynostosis

 Sagittal: Scaphocephlay (boat)

 Unicoronal: Plagiocephaly (twisted)
 Bicoronal: brachycephaly (short)
 Metopic: trigonocephaly (keel)
 Most common=sagital
 Biggest differential: “deformational
plagiocephaly” or “positional
plagiocephaly”: non-synostotic
Trigonocephaly
Scaphocephaly
Craniosynostosis syndromes

 Associated with midface

abnormalities: Base of skull
synchondrosis
 Associated with finger/toe
malformation: often diagnostic
 Other associated malformations
Major Craniosynostosis
syndromes
 Crouzon: normal hand
 Apert’s: Major syndactyly

 Pfeifer: broad thumbs

 Muenke’s syndrome: First genetic-

based diagnosis: FGFr-3 mutation
predictably causing bicoronal (or
unicoronal) synostosis
Crouzon
Apert’s
Pfeiffer’s
Madibulo-facial dysostosis

 Treacher-collins syndrome
 Nager’s
Treacher Collins

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