Cleft Lip & Palate

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Cleft Lip & Palate

Risk factors, management and team disciplinary work

Dr. Mohammad Thabet


Tutankhamun
On March, 2005, Egyptian
archaeologist Zahi Hawass
revealed that ,
the results of a CT scan performed
on the pharaoh's mummy.
The pharaoh had :
•cleft palate
•An impacted wisdom tooth

King’s Mask
• Cleft lip and palate is the second most common congenital
anamoly after clubfoot

A considerable knowledge about the etiology and embryology is


required for proper diagnosis and treatment planning of such
patients

Treatment begins soon after birth and continues till adulthood


requiring a team approach.
Definition in simple
Cleft lip: congenital deformity of the upper lip that varies from a notching to a
complete division of lip,any degree of clefting can occur.
Cleft palate: a congentital split of palate that may extend to uvula,soft palate
and into hard palate,the lip may or maynot involve in the cleft of the palate
 Joaquin Phoenix – Actor
Incidence
•A-Genetic risks
• One affected parent, risk of the first child 2%
• One affected child, risk of next child with is (4%).
• One affected parent and one affected child risk of next child with is 10%
• Two affected parents, risk of first child 60%
Prevalence around 1:2000 live births.

55% Associated with syndromes such as Down, Treacher-Collin, Pierre-Robin sequence.

CP the incidence is higher in females overall (3:2).


Gender distribution:
CLP has greater incidence in males 3:1

CP the incidence is higher in females overall (3:2).

There is a male predominance of submucus clefts.

There is equal gender incidence of isolated soft palate clefts and cleft lip alone
Syndromic and non-Syndromic clefting

•15% of cleft children have additional malformations especially BCLP and CP to have additional malformations (400 syndrome) ,
example:

• 1- Van der Woude


• 2- Pierre Robin sequence. Triad of: cleft palate, micrognathia, macroglossia
• 3- Treacher Collins
4. Van der Woude syndrome
2. Pierre robin syndrome
3. Apert’s syndrome
1. Treacher collins syndrome
Etiological Factors:
• Multifactorial etiology

1) Chances increases if more than one family member if affected

2)  More the severity, greater the chances of recurrence in sibling

3) Higher risk if affected individual is of less affected sex


4) Risk decreases in remotely related individuals
5) Consanguinity increases the rate because of sharing of genes
Etiological Factors:

I- Genetic problems: Chromosomal OR Mutant genes


II. Environmental risk factors:
1-Maternal exposure to:
Tobacco smoke (hypoxia)

Alcohol (alteration cell fluidity)

2- poor nutrition: Folic acid


(vitamin B2 & B6)

Vitamin A

low zinc concentrations (Bufalino et al , 2010)


3- viral infection e.g.: Rubella virus

4-Medications: Anticonvulsant drugs (Dilantin- diazepam)

5-Stress

7-Obesity

8-Fever during pregnancy (Mossey et al ,2009)


PREDISPOSING FACTORS
➢High maternal age
➢ Diabetes

➢ Toxemia

➢ Reduced blood supply


➢ Folic acid deficiency

➢ Racial – mongoloids

➢ Radiations
Current concepts on the effect of Meta-Analysis
environmental factors on cleft lip and
palate

1. Smoke .
2. Alcohol .
3. Folic acid deficiency

Solana et al, 2013


4.Stress ,Obesity or Fever,
required more research in the
future
Advantages of prenatal cleft diagnosis
-  Psychological preparation for parents to have realistic

expectations
Parent education for cleft management

-  Preparation for neonatal care and feeding


Opportunity to investigate other abnormalities

Possibility of fetal surgery



Disadvantages of prenatal cleft diagnosis
-  Emotional disturbance

-  High maternal anxiety and dysfunction

-  Termination of pregnancy
The goals of treatment for the child with a cleft lip/ palate:

• Repair the birth defect (lip, palate, nose)


• Achieve normal speech, language and hearing
• Achieve functional dental occlusion and good dental health

The Center for Children with Special Needs


Seattle Children’s Hospital, Seattle, WA
Plastic
Pediatric surgeon
Pediatrician
dentist

Orthodontis ENT surgeon


A team
coordinator

Maxillofacial
Nutritionist
surgeon
Speech
Social workers
pathologist
Team-Oriented Care for
Orofacial Clefts
A Review of the Literature

McGrattan & Ellis 2013

Team-oriented care results


in better outcomes in individuals
with orofacial clefts
DENTAL
➢ Tooth agenesis, hypodontia (most common)

➢ Supernumerary teeth (2nd most common)


➢ Enamel hypoplasia (CI)
➢ Crossbites
➢ Ectopic eruption, transposition
➢ Taurodontism, dilacerations
Clinical features

• Common finding-natal neonatal teeth (cleft palate)

• absent lateral incisor

• missing premolar

• supernumerary teeth ectopic lateral incisor

• Palatal eruption of permanent Canine

• Anomalies like enamel hypoplasia, microdontia,macrodontia,fused teeth

• over bite

• Lateral facial profile


Clinical features

• protruberance and mobile premaxilla in infants


• Bilateral cleft
• Posterior cross bite
• Rotated permanet incisor
• Premature loss (deficiency) of alveolar bone
Speech difficulties :

Retardation of consonant sounds (p, b , t, d, k, g)

Hindrance to early vocabulary development.

Hyper nasality – cleft of soft Palate

Hearing problems contribute


Hearing loss

Hearing loss and middle ear disease

Otitis media- early , in 1st month of life

Prolonged middle ear effusion →permanent damage to the middle ear


Nasal deformities :

Cleft extending to floor of nose- alar cartilage flared,

Columella pulled to non cleft side

Lack of underlying bone support - ↑ problem

Surgical corrections- deferred until all other procedures are completed -as correction of alveolar
clefts, maxillary skeletal retrusion - alter osseous foundation of nose.
SKELETAL

➢Maxillary deficiency
➢ Mandibular prognathism

➢ ➢ Class III malocclusion

➢ Concave profile
“Feed the child and treat the mother”
Feeding :

• Normally the infant creates a negative oral pressure while


sucking the milk.

• use special bottles and nipples.

• With breast feeding use Breast pumper


The Haberman Feeder

The Mead Johnson Cleft


Palate Nurser
The Pigeon nipple
• Nasal regurgitation:

food comes back out of baby’s


nose during a feeding.
Baby tired easily during feeding

Feeding sessions 20-30 min.


• Swallow air during feeding

Burp the baby every 15 minutes


during and after feedings
Feeding interventions for growth and development in
infants with cleft lip, cleft palate or cleft lip and palate
(Intervention Review)

Squeezable bottles easier to use


but there is no evidence of a difference
in growth outcomes .
Glenny et al , 2008
speech
• Air escapes through the gap in the
roof of the mouth and out the nose.

• Hypernasality

speech and language pathologist


Speech
Hearing and ear infections

Eustachian tube malfunction

frequent Ear Infections

Hearing loss

ENT
Orofacial problems

• Altered facial appearance

• Early or late and ectopic eruption of teeth

• Missing, supernumerary, neonatal and hypoplasied teeth

• Poor occlusion (cross bite, overbite)


Orofacial Management of CLP
At BIRTH - 18 months

• Initial contact and interview with parents

• Registration with Cleft Palate Scheme

• Arrange contact with parental Support groups


Pre-surgical appliance

• An impression of the maxillary arch is taken


as early as 24 hours after birth.
24 hours after birth

Obturator
Feeding Plate
Premaxillary retraction using soft elastic tape
(Microfoam Tape, 3M).
Nasoalveolar molding of the bilateral cleft deformity
Bonnet appliance
Premaxillary Retraction.

In cases of bilateral cleft lip and palate, premaxillary


segment may be positioned severely anterior to the
maxillary arch if lip surgery is undertaken with the
premaxilla in such an abnormal position, the chances
of lip dehiscence by increased pressure at the suture
lines are increased.
Segments may be deviated laterally to one side of the cleft
defect. In such cases a straight extra oral force would
not
place the pre maxilla in the facial midline, it must be
positioned before pre maxillary retraction. By the
application of sequential differential force to the
premaxilla with elastic straps attached to the bulb
prosthesis.
An impression is made of the infant's premaxilla for
construction of an external acrylic "bulb" prosthesis.
This appliance is fitted over the protruding and laterally displaced premaxilla and anchored to
the infant's head with a bonnet appliance.
Advantages
1. Provides a false palate which help in feeding .
2. Provides maxillary cross-arch stability and prevents arch
collapse .
3. Provides maxillary orthopedic moving of the cleft segments
to the correct place before surgery .
Effectiveness of pre-surgical infant
orthopedic treatment for cleft lip
and palate patients: a systematic
review and meta-analysis

Papadopoulos et al, 2012


cheiloplasty (surgical closure of the lip).

• Initial lip closure at


approximately 3 months of age.

Peter Hodgkinson et al, 2005


Bone Grafting of Alveolar Cleft Defects
• Primary bone grafting refers to bone-grafting procedures in children younger than 2

years of age.

• Primary alveolar cleft bone grafting is controversial.

• A recent survey from American Cleft Palate–Craniofacial Association reported that

only 3% 1ry graft provides excellent results.


Palatoplasty

• Closure of the palate between (12m-2Y)

The purpose of palate closure


• Normal speech

• hearing

• swallowing
But!!

• The extent and timing of palatal surgery is one of the major and continuing

controversies in cleft management.

• It is likely to interfere with growth of the midface.


Hard Palate Repair Timing and
Facial Growth in Cleft Lip and
Palate:
A Systematic Review

The review showed inconclusive evidence about the


effect of timing of hard palate repair on facial growth
in patients with cleft lip and palate.
Liao et al, 2006
(PRIMARY DENTITION STAGE)
• Maintaining oral health of child

• Keep teeth free from caries

• Periodic recall examinations, possibly to 3- to 4-month intervals.

• Topical fluoride applications and fissure sealing

• Possible revision of lip repair


Interciptive orthodontics
MIXED DENTITION STAGE

• Ectopic eruption of permanent central and lateral incisors >>>>>

teeth alignment .

• Cross bites of >>>>>> Palatal expansion


• Secondary bone grafting

• Providing bony support to teeth


adjacent to the cleft site .

• Time :12 months before surgery


(9-12y)
Adolescence and early adulthood
• Final tooth alignment

• Fixed appliance orthodontic treatment.

• Pre-surgical and postsurgical orthodontics

• Orthognathic surgery

• Cosmetic surgery
Pediatric dentist Role
• Maintaining the highest standard of oral health

The presence of dental disease can severely


compromise both surgical and orthodontic success.
Pediatric dentist Role

• The parent interview

• Initial Examination of baby

• Medical history

• Dental records

• preventive care

• Dental care
Thank You

H.A

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