Cleft Lip and Palate (Abubaker) Flashcards
What approach is indicated for the management of a pt with Cleft Lip and Palate?
Team approach
What do Tessier classifications center on when describing clefting?
The eye
What single genetic defect leading to one phenotype, which causes another genetic defect?
Sequence
What is a multiple or single genetic defect that leads to multiple phenotypes?
Syndrome
A pt that has a genetically malformed small jaw that results in an inability for the tongue to descend to the floor of themouth which results in a cleft palate has a syndrome or a sequence
Sequence (Pierre Robin sequence is described here)
What is the Pierre Robin sequence triad?
- Celft Palate
- Macroglossia
- Micrognathia
What percentage of cleft lip and palate patients have an associated anomaly?
30%
What is the recurrence rate of CLP in families if the first sibling has CLP?
Low, about 5%
What are the 2 populations with the highest CLP rate?
- Asians
2. Native Americans
What is the population with the lowest CLP rate?
Africans
Which has a higher chance of being associated with a syndrome: isolated cleft lip or isolated cleft lip or isolated cleft palate?
Isolated cleft palate
What percentage of patient with clefting have either an isolated cleft lip or an isolated cleft palate?
70% (this is contraindicated by info presented in the same lecture)
What are 2 things that increase the risk for clefting while pregnant?
- Maternal diabetes mellitus
2. Amniotic band syndrome
What parental characteristic increases the risk for clefting?
Increased paternal age
What percentage of clefting cases are combined Cleft Lip and Palate?
50%
What percentage of clefting cases are isolated cleft palate?
30%
What percentage of clefting cases are isolated cleft lip?
20%
What percentage of clefting cases are cleft lip and alveolus?
5%
What are the bones that make up the hard palate?
Palatine process of maxilla and palatine bones
What is the blood supply of the hard palate?
Greater palatine artery
What is the nerve supply to the hard palate?
Anterior palatine nerve
What is the fibromuscular shelf attached to the posterior portion of the hard palate, which etenses, elevates using what muscles?
This describes the soft palate which uses the tensor veli palatini, legator veli palatini, muscular is uvulae, palatoglossus and palatopharyngeus muscles to elevate.
The soft palate comes in contact with what structure on swallowing to keep food from going up into the nasopharynx?
Passavant’s Ridge (bulge produced during contraction of pharyngeal wall)
Who are the members of the cleft lip and palate team?
- Reconstructive Surgeon
- Otolaryngologist
- Oral and Maxillofacial Surgeon
- Pedodontist / general dentist
- Orthodontist
- Prosthodontist
- Audiologist
- Psychologist
- Speech pathologist
- Social worker
- Geneticist
True or false: with a cleft, all parts of the lip or palate are present, they simply failed to fuse in a normal way?
True
The maxillary process, lateral nasal process, and the median nasal process join and fuse to form what?
The primary palate
When is the primary palate formed?
Six weeks in utero
When does the tongue moves inferiorly allowing the lateral palatal shelves to grow towards the midline and fuse?
Eight weeks in utero
The disappearance of Medial Edge Epithelial Cells (MEEC) during embryonal prominences fusion may be due to what?
- Cell migration
- Cell death
- Epithelial to Mesenchymal transformation
Why does fusion not take place in a cleft?
M E E Cells are not eliminated by the macrophages like they are supposed to be.
What percentage of deformities is the isolated cleft lip?
32%
What is the male to female ratio for clefting?
Males 2:1
What percentage of cleft lip and palate are of genetic origin?
Less than 40%
What percentage of isolated cleft palates are genetically derived?
Less than 20%
What chance will unaffected parents with a child with cleft lip and palate have of a second child with cleft lip OR palate?
4.4%
What chance will unaffected parents with a child with cleft lip and palate have of a second child with |SOLATED cleft palate?
2.5%
If one parents has a cleft deformity, what is the percent chance that their first born will have a cleft lip or palate?
3.2%
What are some exogenous factors implicated in clefting if taken during the first 8 weeks of pregnancy?
- Corticosteroids
2. Other drugs (Valium, Dilantin)
When should the team evaluate the child?
At birth
When does repair of the lip take place?
10 weeks to 3 months
When is hearing tested?
6 months
When is re-evaluation done?
1 year
When is the palate repaired and myringotomy tubes done?
12-18 months
When is a recall by the team performed?
2 years
When are repeated hearing evaluations done?
2-6 years
During what age range are interceptive orthodontics begun and erupted supernumerary teeth in the region of the cleft removed?
Ages 5-8
When is a pharyngeal flap done?
5-7 years
When is maxillary arch expansion done?
7-8 years
When is an alveolar bone graft done?
9-11 years
When is comprehensive orthodontics done?
12-13 years
When is orthognathic surgery done if necessary?
14-16 years
When is dental reconstruction and rhinoplasty done?
16-21 years
What is the “rule of 10s” with lip repair?
- 10 weeks
- 10 grams of hemoglobin per dL
- 10 pounds
What is the main goal of lip surgery?
Recreate normal anatomy
For lip surgery to recreate normal anatomy, what three things must be accomplished?
- Preservation of “Cupid’s Bow”
- Reapproximation of Orbicularis Oris
- Minimize scarring / placement of scars in normal anatomic contours
When do children begin to speak?
18 months
When is palatal closure done?
12-18 months
What is the main benefit of palatal closure?
Provides a normal anatomic environment for the development of normal speech
What are two risks of palatal closure?
- Lateral crossbite due to formation of the palatal scar
2. Denuding of the periosteum
How can the transverse problem of palatal closure usually be managed?
Orthodontics
What percentage of children who undergo palatal repair at age 12-18 months develop normal speech?
70-80%
For the 20-30% of children who undergo palatal repair at age 12-18 months and do not develop normal speech when will speech be assessed?
At age 4 or 5, before the child begins in school
When might hyper nasal speech occur?
When the repaired palate is too short, and inadequate closure from the oral to nasal pharynx is present
What is velopharyngeal insufficiency?
When there is inadequate closure of nasopharyngeal airway port during speech
VPI treatment includes what?
- Speech therapy
- Surgical treatment (pharyngeal flaps)
- Prosthetic appliances
What are the goals for alveolar cleft grafting?
- Stabilize dento-osseous segments
- Provide bone for eruption of the teeth
- Improve alveolar continuity
- Closure of oronasal fistula
- Alar base support
- Prevent tooth loss
What age should the alveolar cleft grafting take place?
- Between ages 9-11, prior to the eruption of the permanent canine and after the crossbite is fixed and the incisors rotated
- The canine root is 2/3 formed
- Tooth not yet erupted
What are 3 indications for orthognathic surgery?
- Hypoplastic maxilla and deficient face
- Mandibular prognathism
- Facial asymmetry in lower face
What are two surgeries that are indicated in a patient with a cleft?
- Le Fort Osteotomy with advancement
2. Mandibular osteotomy (BSSO) and setback
What are some restorative options to replace missing teeth?
- Multiple unit bridges
- Removable appliances
- Endosseous implants
What are indications of cleft lip and palate to the GP dentist?
- Understanding the timing of procedures for explaining to a family of cleft patients
- Working as a cleft team member when needed
- Timing of restorative and prosthetic management of these patients
- Early referrals to specialist when needed