` Facial clefting is the second most common

congenital deformity (after clubfoot).

` Affects 1in 750 births
` Problems are cosmetic, dental, speech, swallowing,
hearing, facial growth, emotional
` Cleft Lip +/- Palate- 2 Male: 1 Female
` Cleft Palate - 2 Female: 1 Male
` Cleft Lip +/- Palate- Native Americans > Oriental
and Caucasians > Blacks
` Cleft Palate- Same among ethnic groups
` Environmental: Ethanol, Rubella virus, thalidomide,
aminopterin
` Increased Clefts with maternal diabetes mellitus and
amniotic band syndrome
` Increased Clefts with increased paternal age
` Cleft Lip + Palate- 50%
` Cleft Palate- 30%
` Cleft Lip- 20%
` Cleft Lip + Alveolus- 5%
` Primary Palate- Triangular area of hard palate
anterior to incisive foramen to point just lateral to
lateral incisor teeth
◦ Includes that portion of alveolar ridge and four incisor
teeth.
` Secondary Palate- Remaining hard palate and all of
soft palate
` Primary Palate
◦ Forms during 4th to 7th week of Gestation
◦ Two maxillary swellings merge
◦ Two medial nasal swelling fuse
◦ Intermaxillary Segment Forms: Labial
Component(Philtrum) Maxilla
Component(Alveolus + 4 Incisors) Palatal
Component(Triangular Primary Palate)
` Secondary Palate
◦ Forms in 6th to 9th weeks of gestation
◦ Palatal shelves change from vertical to horizontal position
and fuse
◦ Tongue must migrate antero-inferiorly
` Hard Palate
◦ Bones: Maxilla( Palatine Processes) + Palatine
Bones(Horizontal Lamina)
◦ Blood Supply: Greater Palatine Artery
◦ Nerve Supply: Anterior Palatine Nerve
` Soft Palate
◦ Fibromuscular shelf attached like a shelf to posterior
portion of hard palate
◦ Tenses, elevates, contacts Passavant’s Ridge
◦ Muscles: Tensor Veli Palatini(CNV), Levator Veli
Palatini(Primary Elevator), Musculus Uvulae, Palatoglossus,
Palatopharyngeus(CN IX and X)
` Non-syndromic inheritance is multifactorial
◦ Cleft Lip, With or Without Cleft Palate:
x One Parent-2%
x One Sibling- 4% Two Siblings- 9%
x One Parent + One Sibling- 15%
◦ Cleft Palate:
x One Parent- 7%
x One Sibling- 2% Two Siblings- 1%
x One Parent + One Sibling- 17%
` Increased clefts with chromosome aberations
` Clefts a part of a Syndrome 15-60% of time
` More than 200 syndromes include clefts
` Cleft Palate- Apert’s, Stickler’s, Treacher
` Cleft Lip +/- Palate- Van der Woude’s,
Waardenberg’s
` van der Woude syndrome - Autosomal
dominant disorder is on chromosome
Robin Sequence
` Micrognathia
` Glossoptosis
` Cleft palate
` Associated
abnormalities - 52%
to 83%
Velocardiofacial syndrome

` Overlaps with DiGeorge syndrome and

conotruncal anomaly face syndrome
` CATCH 22: cardiac anomalies (usually
conotruncal), abnormal facies, thymic
disorders, cleft palate, and hypocalcemia
caused a deletion of chromosome 22 at the
q11.2 region.
Stickler syndrome
` Most common syndrome found in children with
Robin Sequence - 14% to 34% of cases
` Sensorineural hearing loss

` Myopia - Risk of retinal detachment and early

cataracts
` Hypermobilityof joints
` Stiffness and premature osteoarthritis
` Cleft result in a deficiency of tissue
` Cleft lip occurs when an epithelial bridge fails
` Clefts of primary palate occur anterior to incisive
foramen
` Clefts of secondary palate occur posterior to
incisive foramen
` Secondary Palate closes 1 week later in females
` Cleft of lip increases liklihood of cleft of palate
because tongue gets trapped.
UNILATERAL INCOMPLETE UNILATERAL COMPLETE BILATERAL COMPLETE

INCOMPLETE CLEFT PALATE UNILATERAL COMPLETE LIP AND BILATERAL COMPLETE

PALATE
` Clefts of soft palate only
` Clefts of soft and hard
palate reaching anteriorly
to incisive foramen
` Complete unilateral
alveolar clefts - generally
involve the lip as well
` Bilateral alveolar clefts -
associated with bilateral
clefts of the lip
` A - Incomplete cleft of the
secondary palate
` B - Complete cleft of the
secondary palate
` C- Incomplete cleft of the
primary and secondary
palates
` D - Unilateral complete
cleft of the primary and
secondary palates
` E - Bilateral complete cleft
of the primary and
secondary palates
` Nasal floor communicates with oral cavity
` Maxilla on cleft side is hypoplastic
` Columella is displaced to normal side
` Nasal ala on cleft side is laterally, posteriorly, and
inferiorly displaced
` Lower lat on cleft side -lower, more obtuse
` Lip muscles insert into ala and columella
` Soft palate muscles insert on posterior margin of
remaining hard palate rather than midline raphe.
` Associated Dental Abnormalities
◦ Supernumery Teeth- 20%
◦ Dystrophic Teeth- 30%
◦ Missing Teeth- 50%
◦ Malocclusion- 100%
` Team Approach
` Initial Head and Neck Examination
` Speech Disorders
` Ear Disease
` Airway Problems
` Surgical Repair
` Head- facial symmetry
` Otologic- auricle and canal development and
location, pneumatic otoscopy, forks
` Rhinoscopy- identifies clefting, septal anomalies,
masses, choanal atresia
` Oral Exam- cleft, dental, tongue
` Upper airway- phonation, cough, swallow
` Errors in Articulation: Fricatives, Affricates
` Velopharyngeal Competence- Most important
determinant of speech quality in cleft palate
patients-75% achieve competence after initial palate
surgery
` Incompetence- nasal emission or snort
` Evaluation- Direct exam , Fiberoptic Exam
` Cleft Lip- Incidence similar to normal pop.
` Cleft Palate- Almost all with ETD, CHL
` ETD- Due to abnormal insertion of levator veli
palatini and tensor veli palatini into posterior hard
palate
` ETD- Returns to normal by mid-adolescent
` Cleft Palate- Increased Cholesteatoma(7%)
` Otologic Goals For Cleft Palate Patients
◦ Adequate hearing
◦ Ossicular chain continuity
◦ Adequate middle ear space
◦ Prevent TM deterioration
` Indications for Myringotomy Tubes
◦ CHL, Persistent/Recurrent effusion, Retraction
◦ Cleft palate: Multiple BMTs from 3mo. - 12 yrs
` More common in Cleft Palate patients with
concomitant structural or functional anomalies.
` e.g. Pierre-Robin Sequence
◦ Micrognathia, Cleft Palate, Glossoptosis
◦ May develop airway distress from tongue becoming lodged
in palatal defect
` Lip Adhesions-
◦ 2 weeks of age
◦ Converts complete cleft into incomplete cleft
◦ Serves as temporizing measure for those with feeding
problems
◦ May interfere with definitive lip repair
◦ Less often needed in recent years due to wider variety of
specialty feeding nipples
` Cleft lip repaired at 10 weeks
` Rotation-advancement method- Most common in
the U.S.
` Nine Landmarks
` Rotation Flap cuts made first
` Advancement cuts made next
` Cleft side nasal ala cuts made last
` Surgery > 10 weeks old
` Weight > 10 pounds
` Hemoglobin level > 10 g/dL
` Bridge the cleft
` Create a complete muscular sling around the
entire circumference of the oral cavity
` Approximate cleft edges
` Maintain Cupid's bow and philtral dimple
` Align vermillion border
` Create an intact nasal floor and sill
` Produce symmetry of the alar base and columella
` Reconstituting the circumferential integrity of the
orbicularis oris muscle
` Millard Repair - Rotation advancement technique
` Randall - Graham - Triangular flap interposition
` Rose - Thompson - Straight line repair, Risk of
vertical contracture
` Downward and lateral rotation of the medial segment of the
cleft lip combined with the medial advancement of the
lateral cleft segment into the defect
` Bridges the gap
` Maintains the rotation flap in position
` Maintains the amount of vertical height gained by the
rotation flap and back cut
` Medially and laterally based rectangular flaps from the margin of
the cleft
` Tension-bearing suture through the membranous septum.
` Unilateral lip adhesion: A, landmarks and incisions; B, flaps
elevated and undermined; C, placement of 3-0 nylon retention
suture; D, final suturing.
Cleft lip repair. The edges of the cleft between the lip and nose are cut (A
and B). The bottom of the nostril is formed with suture (C). The upper part of
the lip tissue is closed (D), and the stitches are extended down to close the
opening entirely (E).
` Dorf and Curtin
◦ 10% occurrence of articulation errors when
palatoplasty was completed by 1 year
◦ 86% incidence of articulation errors when repair
was complete after 1 year
` Haapanen and Rantala - Significantly fewer
children in the groups repaired before 18 months
had hypernasal speech, articulation errors, or
required secondary surgery to correct speech
` V-Y Pushback
` Two Flap Palatoplasty
` Four Flap Palatoplasty
` Schweckendick’s Primary Veloplasty
` Furlow Palatoplasty
` Two uni-pedicled flaps (greater palatine artery) and one or
two anteriorly based pedicled flaps
` Posterior flaps rotated in a V-Y advancement technique -
increasing the length of the palate
` Nasal mucosa not closed
` Improved speech results compared with bipedicled
techniques
` Indicated for incomplete clefts
` Incisions made in soft palate
` Muscle bundles released from the posterior
hard palate and rotated
` Reconstruction of levator sling
` Closure of mucosal layers separately
` Lengthens the soft palate
` Reconstructs the muscle
sling.
` Also commonly used to
correct velopharyngeal
insufficiency in patients
with submucous cleft
palate
` Speech outcomes are
improved compared with
other palatoplasty
techniques.
` Oronasal fistula - 8.7% to 23%
◦ Sites of fistulization are typically the anterior hard palate
and the junction of the hard and soft palate.

` Velopharyngeal insufficiency
` Dental Obturator
◦ For high-risk patients or those that refuse surgery.
◦ Advantage- High rate of closure
◦ Disadvantage- Need to wear a prosthesis, and need to
modify prosthesis as child grows.
` The Care will entail attention, not only to surgical
repair, but also more immediate needs such as
feeding.
` Primary lip repairs can often be undertaken at
three months of age with palatal repairs around
six months.
` Additional surgeries as well as speech and
orthodontic therapies are often needed.
` The cleft Lip and Palate Association
(www.clapa.com/) provides support and
information for parents.
` Infants with CL/P have few feeding problems.
` If the cleft involves the hard palate, the infant is
usually not able to suck efficiently.
◦ Experiment (special nipples or alternate feeding positions)
` The infant should be held in a nearly sitting
position during feeding
◦ Prevents flowing to the back into the nose.
` Should be burped frequently, (q 3-4min).
` It is important to keep the
cleft clean

` Breastfeeding is extremely
challenging.
` Activated by tongue and
gum pressure.
` Milk cannot flow back.
` Replenished continuously
as the baby feeds.
` Prevents the baby from
being overwhelmed with
milk.
` A gentle pumping action
to the body of the nipple
will increase flow.
` Have a family meeting with both parents present.
` Infant should be brought to the parents as soon
the mother and the infant are in satisfactory
condition.
` Allow the parents to observe, react and ask
questions about the infant.
` Explained the defect and the how the surgeon will
most likely correct the clefts.
` Before and after pictures are helpful.
` Emphasize as possible to the parents the normal
healthy features of the baby.
` The baby should be present when the defect is
explained, as ugly as the cleft might be.
` Training the mother about feeding techniques and
avoiding complications.
` Monitor feeding, growth, and development
` Repair cleft palate
` Monitor ears and hearing
` Instructions in oral hygiene
` Monitor speech and language development
` Manage velopharyngeal insufficiency
` Monitor ears and hearing; ear tubes if indicated
` Assess development and psychosocial needs
` Consider lip/nose revision before school
` Monitor speech and language; manage
velopharyngeal insufficiency
` Orthodontic evaluation and treatment
` Alveolar bone graft
` Monitor school and psychosocial needs
` Monitor school and psychosocial needs
` Orthodontics and restorative dentistry
` Genetic counseling
` Rhinoplasty (if needed)
` Orthognathic surgery (if needed)
 Tom Brokaw American television journalist.

Jesse Jackson Politician, professional civil rights

activist and Baptist minister

Peyton Manning NFL quarterback

Annie Lennox Scottish pop musician and vocalist

Mark Hamill Actor

Tutankhamun Egyptian Pharaoh who may have had a

cleft lip according to diagnostic
imaging
` Klaus & Fanaroff. “Care of the high-risk neonate”.
Saunders”. 5th edition. 2001 USA.
` Colin D. Rudolph “Rudolph’s Pediatrics” McGraw-Hill. 21st
edition. 2002. USA.
` Christensen, K, et al. “Cleft-twin sets in Finland 1948-
1987”. Cleft Palate Craniofac J 1996; 33:530.
` Clementi, M, et al. “Evaluation of prenatal diagnosis of
cleft lip with or without cleft palate and cleft palate by
ultrasound: experience from 20 European registries”.
EUROSCAN study group. Prenat Diagn 2000; 20:870.
` Milerad, J, et al. “Associated malformations in infants with
cleft lip and palate: a prospective, population-based
study”. Pediatrics 1997; 100:180.
` Cockell, A, Lees, M. “Prenatal diagnosis and management
of orofacial clefts”. Prenat Diagn 2000; 20:149.