Clefts

Question 1

How does Cldft lip develop?

Answer 1

Failure of fusion of the medial nasal process and maxillary process.

Question 2

What time during gestation does cleft lip and palate occur?

Answer 2

Upper lip and premaxilla form approximately 7 weeks gestation.

Palatal shelves fuse approximately 12 weeks.

Disruption at these times leads to cleft.

Question 3

What muscles are anatomically abnormal in cleft lip?

Answer 3

Orbicularis oris muscle.

Question 4

What muscles are anatomically abnormal in cleft palate?

Answer 4

In complete cleft palate the levator veli palatini, tensor veli palatini, uvular, palatopharybgeus, abd palatoglossus.

Question 5

Where does the levator veli palatine insert in cleft palate patients?

Answer 5

It inserts abnormally onto the posterior hard palate.

Question 6

What is Passavant’s ridge?

Answer 6

Transverse ridge or bulge produced b forceful contractio of the superior pharyngeal constrictor on the posterior pharynx oppisite the arch of the atlas. This ridge is observed during gagging and pronunciation of vowels. It is important in velopharyngeal closure.

Question 7

What is the blood supply to the palate?

Answer 7

Greater and lesser palatine branches from the Descending palatine artery. The ascending pharyngeal branch of external carotid via the maxillary artery and the ascending palatine branch of facial artery.

Question 8

What factors are known to cause clefts?

Answer 8

Corticosteroids and Diazepam taken during the first 8 wks of pregnancy.
Less than 40% of cleft lips and palate are of genetic origin. Less than 20% of isolated cleft palates are of genetic origin.

Question 9

What is the incidence of cleft lip with or without cleft palate in general population?

Answer 9

1 in 700 births.
The incidence of isolated cleft palate is 1:2000.

Question 10

Unilateral cleft lip and palate is typically seen in?

Answer 10

Males, typically on the left side.

Question 11

Isolated cleft palate more frequently seen in?

Answer 11

Girls.

Question 12

When can prenatal ultrasound can diagnose cleft lip?

Answer 12

15 wks gestation in the coronal plane,
palate in the axial plane.

Question 13

What are the currently available feeding aids for cleft patients?

Answer 13

Haberman feeder- expensive- has a one-way valve
Mead Johnson- low cost squeeze bottle, Pigeon Nipple- faster flow.

Question 14

What angle must cleft patients be fed?

Answer 14

45 degree angle and burped frequently as they swallow more air when sucking.

Question 15

How are clefts classified?

Answer 15

Complete/Incomplete
Prepalatal: Uni/bilateral
Submucosal cleft palate or bifid uvual

Question 16

Complete vs Incomplete cleft lip?

Answer 16

Complete cleft lip is a cleft of the entire lip and underlying premaxilla or alveolar arch.

An incomplete only involves the lip.

Question 17

What is a submucous cleft?

Answer 17

Deficienchy in the musculature of the palate due to failure of the levator muscle fibers to fuse completely in the midline. Palate looks intact because oral and nasal mucous membranse are present. Patients have a bifid uvula, loss of the posterior nasal spine, and a bluish midline streak on the soft palate due to mscular diastases. A notch may be present in the posterior hard palate. Usually associated with VPI and palatal incompetence.

Question 18

What is bifid uvula?

Answer 18

A variation of cleft palate seen in 2% of normal US population. May be associated with palatal incompetence and speech problems.

Question 19

What are the criteria for timing of cleft lip repair?

Answer 19

Rule of Tens:
10-14 weeks of age, >10g/dl hemoglobin,
>10lbs weight

Question 20

What is the Millard rotation advancement flap?

Answer 20

modified Z-plastty technique- placed at top of the cleft so that the point of greatest tension is at the base of the nares. Most popular method of lip repair. Involves downward rotation of the philtrum of the lip as a flap into normal symmetric position whiule the latarl lip segment is advanced across the cleft and into the space behind the central lip. The final scar from the suture line closely recreates the philtrum of the lip.

Question 21

What is the timing for cleft palate repair?

Answer 21

10-18 months of age. Must balance needs for normal speech vs normal palate growth and occlusion.

Question 22

What are some techniques for cleft palate repair?

Answer 22

von Lagenbeck
Furlow palatoplasty
V-Y pushback
Wardhill-Kilner

Question 23

What is the von Lagenbeck operation?

Answer 23

long relaxing incisions laerally with elevation of large mucoperiosteal flaps from the hard palate which is bipedicled anteriorly and posteriorly. The cleft margins of both the hard and soft palates are approximated at the midline. the levator muscles are completely detached from their abrnomral bony insertion and the soft palate musculature is repaired in the midline.

Question 24

What is a vomer flap?

Answer 24

Superiorly based flap of nasal mucosa from the vomer is used to close the hard palate.

Question 25

Most common postoperative complications of cleft palate repair?

Answer 25

Hypernasal speech= 30% of patients.

Oral-nasal fistulas=10-21%.

Question 26

What is VPI?

Answer 26

Velopharyngeal Insufficiency resulting in hypernasality, nasal emissions, or compensatory mechanims.

Question 27

How is VPI diagnosed?

Answer 27

Speech language pathologist visualization of the mechanism during function via direct techniques such as nasopharybgoscopy and videofluoroscopy.

Question 28

Which muscles are the most important for achieving VP closure?

Answer 28

Levator palatini muscles contribute the most by pulling the middle third of the soft palate superiorly and posteriorly to produce frim contact with the posterior pharyngeal wall at about the level of the adenoidal pad.

Other muscles that contribute to VP closure are the paired palatopharyngeus muscles which pull the soft palate posteriorly, the muscularis uvulae which causes the uvula to thicken centrally with contraction; and the superior pharyngeal constrictors which move the lateral pharyngeal walls medially or the posterior pharyngeal wall anteriorly with contraction.

Question 29

How is VPI managed?

Answer 29

Speech therapy beginning with parental counseling at 6 months, child therapy should begin when the child is about age 4 or when the definitive diagnosis is made.

Question 30

What percentage of VPI require surgery?

Answer 30

20-25%.

Question 31

When should alveolar bone graft for cleft repair be placed?

Answer 31

Prior to the eruption of the permanet canine with the canine root is 1/4th to 2/3rds developed. age 9-11 yrs.

Question 32

Orthodontic treatment to stimulate growth and tooth eruption should be instituted when?

Answer 32

3 months before bone grafting .

Question 33

WHen should the cleft site be orthodontically expanded prior to grafting?

Answer 33

To correct posterior and/or anterior cross-bites.

Question 34

What is the ideal bone for alveolar cleft repair?

Answer 34

Particulate bone with cancellous marrow is the best choice for grafting because of osteoinduction and osteoconduction qualities are most predictable.

Question 35

What are the most common skeletal jaw deformities in cleft palate patients?

Answer 35

Midface deficiency
Maxillary transverse deficiency
Class III skeletal and occlusal deformity
Prognathic mandible.Wha

Question 36

What is the ideal age for cleft orthognathic surgery?

Answer 36

16-18 yrs in boys
14-16 yrs in girls.

Question 37

Your patient develops VPI after orthognathic cleft repair. What should you do?

Answer 37

In most cases this resolves within 6-12 months without intervention- speech will return to its preoperative quality. If after 12 months it does not resolve- pharyngeal flap procedure may be necessary.