What percentage of cleft lip & palate are associated with syndromes?

15-60%

What are some environmental factors and teratogens associated with cleft lip or
palate?

Amniotic band syndrome, maternal DM, maternal dietary folate deficiency,

ethanol, tobacco smoke.

Teratogens include ethanol, thalidomide, and phenytoin.

What are 2 genes associated with cleft lip and palate?

Interferon regulatory factor 6 and TGF-alpha.

What are the primary and secondary palates? When do they develop and close?

Primary: anterior to incisive foramen, containing 4 central and lateral incisors.

Develops at 4-5 weeks. Closure during 4th to 7th week.

Secondary: hard and soft palate posterior to incisive foramen. Develops at 8-9
weeks. Closure by 9 weeks.

When does the upper lip develop?

4-5 weeks.

What 3 things are required for closure of the secondary palate?

1. Palatal shelves change from vertical to horizontal position.

2. Migration of the tongue out of the way in anteroinferior direction away from the
shelves.

3. Fusion of the palatal shelves (occurs initially at the incisive foramen and
progresses posteriorly toward uvula).

Where does the secondary palate begin to fuse?

At the incisive formamen. Then progresses posteriorly.

Describe the Veau classification of cleft lip and palate.

I (A) Defect of soft palate only

II (B) Defect of hard palate and soft palate to the incisive foramen

III (C) Defect from soft palate, hard palate, and alveolus, may involve the lip

IV (D) Complete bilateral cleft

Describe the Kernahan and Stark classification of cleft lip and palate.

1 and 4 Lip
2 and 5 Alveolus
3 and 6 Palate between alveolus and incisive foramen
7 and 8 Hard palate
9 Soft palate

With a cleft of the alveolus, what else is found?

Always associated with cleft lip.

What sounds are produced with nasal vs. oral air flow?

only /n/, /m/, and /ng/ use nasal air escape. All others use oral air flow.

How can you detect inappropriate nasal air flow during speech with VPI?

Look for fog of a mirror below the nose with sentences like "Daddy did it",
"Puppy", "Sissy saw it".

What are some surgical options for treatment of persistent VPI after cleft palate
closure?

Intravelar veloplasty and Furlow double-opposing Z-plasty: both reorient the

levator.

Sphincter pharyngoplasty: borrows lateral wall tissue to obturate the lateral and
posterior walls.

Paryngeal flap: borrows tissue from posterior wall to obturate the middle portion
of the velopharynx.

Treatment options for VPI after cleft palate closure?

Speech therapy, surgery, prosthetic obturation.

Describe the lip adhesion procedure. When is it done? What is the technique?
Lip adhesion is performed within the first few days and weeks of life as a
preliminary procedure, with definitive cleft repair delayed to a later date. The
purpose of lip adhesion is to convert a wide complete cleft to an incomplete cleft
and allow tissue growth during the delay phase and to provide additional
reconstructive tissue. In addition, the lip adhesion is thought to act as an
orthodontic appliance that improves the alignment of the maxillary arches and
narrows the alveolar defect. Some surgeons choose not to perform this
preliminary closure as they feel the subsequent scar tissue will compromise the
definitive cleft repair.

Lip adhesion is performed by creating medially and laterally based flaps (typically
rectangular) from the margin of the cleft and suturing them together to create a
bridge over the cleft.

Describe the effect of cleft palate on dentition and how this is treated.

CP pts have missing teeth/malocclusion etc due to alveolar defects. More

specfically, the anterior maxillary/alveolar defects lead to:
1) displaced, tipped, or rotated permanent teeth
2) prevention of permanent teeth from appearing
3)prevention of alveolar ridge formation

Treatment:
alveolar bone grafts so that teeth can be corrected orthodontically
(can use rib/iliac etc for bone grafts)
Orthodontic Tx :
1) first phase AKA "orthopalatal expansion" - begins as permanent teeth start to
come in; expander is placed which causes the upper dental arch to be rounded
out and the widening of the upper jaw. May follow with alveolar bone graft.
2) 2nd phase - once all permanent teeth are in, perform extractions of extra
teeth, putting in implants for missing teeth, or braces to straighten teeth

Describe the effects of cleft lip on the nasal ala.

Ipsilateral ala lies inferior and lateral to the contralateral side. Ala rests on an
underdeveloped premaxilla, which partly accounts for alar base lowering and
horizontal nostril.

-because of the abnormal ala, the columella is foreshortened and lies obliquely,
with its base directed away from the cleft side.
-nasal dorsum to tilt to the cleft side.

Why do kids with cleft palate have eustachian tube dysfunction?

Dysfunction of tensor veli palatini.

Also have swallowing disorders and reflux that contribute to increased incidence
of SOM and AOM.

Discuss the data on prophylactic PE tube placement for cleft palate patients.

Studies have shown 92 to 97% rates of COM in cleft patients. Some studies
found that hearing ability was more important than severity of cleft or surgical
repair in determining language development.

One study (Hubbard 1985) did a case control study looking at 2 different cleft
teams, one that placed prophylactic tubes and one that didn't. The tube group
had slightly better hearing and consonant articulation but there were no
differences in cognitive, language or psychosocial development.

A recent systematic review found contradictory evidence regarding benefit

(Ponduri 2008)