Cleft Lip/Palate

Question 1

What are the different types of clefts?

Answer 1

Cleft lip with palate
Cleft lip without palate
Cleft palate

Question 2

Cleft lip and palate is more common in ________ and more common to have ___________ on the ________side

Answer 2

More common in males and unilateral and left side

Question 3

Isolated cleft palate is more common in _________ and has more _________associations

Answer 3

Females, syndrome

Question 4

What is the etiology of cleft lip and palate?

Answer 4

1. Genetic (polygenic mode of inheritance, single gene locus)
2. Environmental factors (alcohol, vitamin deficiencies – retinoic acid, medications – anti-convulsants, nutritional deficiencies, hypoxia, viruses)

Question 5

When do you see induction of cleft lip and palate? And how does it happen?

Answer 5

It is induced 4-10 weeks gestation

There is a failure of fusion of medial nasal process and the maxillary process (or it could be a breakdown of fusion)

There is an influence of growth factors, migration of neural crest cells, and deficient vascularization

Question 6

What are the associated abnormalities of cleft lip, cleft lip + palate, and cleft palate?

Answer 6

1. Feeding difficulties (no suction because there is no physical connection between oral cavity and nasal cavity) – no negative pressure
2. Recurrent otitis media - Eustachian tube important for equalizing pressure from middle ear to oral cavity. Can’t clear it properly –> middle ear infection occurs in 20-30% of kids with clefts, can lose hearing left alone
3. Abnormal speech - not able to separate oral cavity from nasal cavity so you sound very nasal. Not able to close and creat seal anteriorly
4. Nasal deformities - cleft goes through base of nose, no support for inferior part of nose, becomes flat
5. Mid face growth disturbances - classical appearance and big jaw.
6. Multiple dental anomalies
7. Other syndromes and anomalies (especially isolated cleft palate)

Question 7

What is the general overview of the treatment sequence?

Answer 7

1. Maxillary orthopedics vs. lip adhesion (4-6 weeks)
2. Definitive lip repair (rule of 10’s)
3. Repair of soft and hard palate (12-18 months)
4. Ear tubes
5. Speech therapy
6. Orthodontic therapy
7. Correction of velopharyngeal incompetence
8. Dentoalveolar cleft repair (canine eruption I)
9. Orthognathic surgery
10. Nasal surgery
11. Lip revisions

Question 8

What is the first thing that you do?

Answer 8

1. Maxillary orthopedics vs. lip adhesion
   - close the lip within first month child is born
   - don’t want psychosocial effects
   - put a reverse palatial expander or other instruments to help

Question 9

What is the Latham appliance

Answer 9

It is used in the first 2-3 weeks in order to bring the lips closer together by moving the alveolus together.

A screw it at the base of the nose and mom can adjust screw to pull the alveolus closer

Question 10

What is the rule of 10s?

Answer 10

Want kids to be at least 10lbs, 10g of hemoglobin, 10 weeks after they are born to start lip repair

Question 11

When do you start repair of the soft and hard palate (step 3)

Answer 11

12-18 months. We do this early on because we want to get the hole in the palate closed and fixed before the child starts to speak so he will not develop bad speech habits.

We want the back of the palate to be mobile enough to hit posterior part of the pharynx and seal so that there is no escape into the back of the pharynx.

Question 12

How do you fix ear tubes/problems (step 4)?

Answer 12

Around the same time as repair of the soft and hard palate we put a slit in the middle ear of the membrane and put in a tube to equalize pressure from outside to inside. Tube must be replaced several times

Question 13

What do you do in orthodontic therapy (step 6)

Answer 13

So there is no bone in the alveolus on one or both sides. Minor segment is behind the cleft and does not have good support. It is often in crossbite because lateral force of buccinator is squishing everything.

***you must be careful because if you try to put in Hawley or quad helix too soon, you fix the cross bite but you expand the hole

Question 14

What occurs in the correction of velopharyngeal incompetence (step 7)

Answer 14

You correct the soft palate for speech at around 1 years old but you could still have speech problems.

You want to reduce loss of air through posterior palate by sewing the palate (uvula to back of pharynx). You want to leave two openings on either side so they can breath through nose

Question 15

What occurs in dentoalveolar cleft repair (canine eruption) (step 8)

Answer 15

Now you need to close the hole in the alveolus and nasal cavity

There are 2 objectives:

1. Close the hole between nose and oral cavity
2. Close the cap and provide bone so that when canine erupts, there is something for canines to erupt to.

You must wait until canines about 2/3 developed, then consider doing surgery. This is usually around 6-9 years old

Question 16

What are the goals of alveolar cleft bone grafting?

Answer 16

1. Closure of the oronasal and palatial fistula
2. Stabilization of the expanded arch and the pre maxilla
3. Provide bone support for erupting canine
4. Improve the bone support and periodontal status of the incisors
5. Provide bone support for the alar base of the nose

Question 17

What tooth is often unsalvagable in cleft lip/palate?

Answer 17

Lateral incisor because it is usually missing or mail posed.

Question 18

What are patients with cleft usually missing other than lateral incisors?

Answer 18

Piriform rim, so you want to graft the foundation for the piriform rim and floor of nose

Question 19

What is the procedure for alveolar cleft grafting?

Answer 19

So to begin alveolar cleft grafting you incise the mucosal covering you did when repairing the soft and hard palate in step 3.

Incise –> elevate mucosa off bone and into the floor of the nose and sew the flaps. This means the nasal floor is now closed. Now we fill the space using BMP + inorganic material (hydroxyapatite) –> turns into bone.

Question 20

Before BMP + inorganic materials, what was used? What is the advantage of BMP?

Answer 20

Surgeons used to use marrow from patients hips. The benefits of BMP are that you don’t need to do hip surgery.

BMP is only FDA approved for sinus lifts and extraction sites

Question 21

Recall that mid face deformities lead to huge class III why?

Answer 21

This is because the amount of surgery leads to maxillary deformities. All the treatments leads to scar tissue –> immobile tissue and this stops maxilla from growing forward and downward –> class III malocclusion.

Question 22

How do you fix the maxillary deformities?

Answer 22

We must bring the lower jaw back and maxilla forward.

Distraction osteogenesis is the surgical technique in which new bone formation is stimulated by gradual separation of bony segments after an ostectomy.

Distraction:

• allows us to bring maxilla forward slowly
• decrease in relapse
• not that precise
• 3-4 months after distraction, you take hardware off
• cut the maxilla (Lefort I)

Question 23

What occurs in nasal surgery and lip revisions (step 10 and 11)?

Answer 23

If you do nasal surgery too early –> everything will be screwed up because if we do nasal then fix maxilla, the nose will not be adjusted properly.

Plastic surgeon will often not operate until after orthognathic surgery finishes –> late teens, early 20s (rhinoplasty)

Lip revision can be done before or after nasal surgery.

Question 24

What are some ways to manage and prevent cleft?

Answer 24

1. Identification of specific gene defect
2. Prenatal care (vitamin/nutritional deficiency is a cause)
3. Prenatal diagnosis (we know sometimes during development if kids will be born with cleft)
4. Parent education and counseling
5. Fetal surgery (dangerous, high chance of death)