Ortho

Front 1

1. What is the most common craniofacial birth defect?

What challenges do patients with CL and/or CP face?

How is treatment for these patients?

Back 1

Orofacial defects
**second only to clubfoot in occurence

Face feeding, communicative, aesthetic and pyschosocial challenges

Long-term process (at least 2 decades long) warranting a multidisciplinary team approach

Front 2

2. What is the overall incidence of clefting?

How often is cleft lip associated with cleft palate?

How does ethnicity affect the incidence of CL(P)?

Which is more common unilateral or bilateral clefting?

Back 2

1 in 700 live births

68% to 86% of the time

More common in African descent followed by Caucasians then Native Americans

Unilateral is 66% vs bilateral is 33%

Front 3

3. How are the majority of children with orofacial syndromic clefting?

Which type of clefting is more likely to have syndromes?

Back 3

"Otherwise normal"
*sometimes there are congenital or growth factors problems

42% to 54% CP cases will be associated with multiple anomalies

14% to 30% of CL(P) cases will be syndromic

**CP more likely to have syndromes than CL

Front 4

4. What are 15% of isolated clefts thought to be caused by?

What are some genetic loci associated with clefting?

Back 4

Thought to be caused by single gene disorder

1. TGF
2. FGFr2
(fibroblast growth factor receptor 2)

Front 5

5. What are some epigenetic factors that contribute to isolated clefting as suggested by phenotypic heterogeneity and variable penetrance?

Back 5

1. Maternal smoking
2. Maternal alcohol use
3. Maternal folate deficiency
4. Maternal disordered metabolism
5. Maternal steroid use
6. Maternal statin use
7. Retinoid exposure

Front 6

6. What are the familial risks of subsequent clefting in...

1. Siblings of cleft child if there are no other affected family members

2. Siblings if there are other affected family members

3. Children of siblings if there are no other affected family members

4. Children of siblings if there are other affected family members

Back 6

1. 4% risk

2. 10% - 12% risk

3. 1% risk

4. 5% - 6% risk

Front 7

7. What does the central segment consist of?
(three parts)

What is the central segment derived from?

What is the remainder of the face derived from?

Back 7

1. Forehead
2. Nose
3. Primary palate

Derived from frontonasal process

Derived from branchial arches

Front 8

8. What fuses to form the upper lip?
(two things)

When does this fusion occur?

What does failure of this fusion result in?

Back 8

1. Fronto-nasal process
2. Maxillary process

During 5th and 6th weeks

Cleft lip

Front 9

9. What is a complete cleft palate?

In a cleft palate where does the levator palatini insert?

Consequently hos is the soft palate?

What happens to the function of the velar?

What causes Eustachian tube dysfunction?

Back 9

Extends up to pre-maxilla (hard palate)

Inserts on the cleft edges instead of on the levator aponeurosis

Soft palate is shorter

Velar function is impaired

Malinsertion of the tensor veli palatini

Front 10

10. What can surgical treatment of the clefting contribute to?

Back 10

Contribute to enamel hypoplasia of the upper incisors in both dentitions leading to increased risk of dental caries

Also have scarring under mucosa due to fixing cleft

Front 11

11. What are other dental abnormalities thought to be related to?

Back 11

Thought to be related to the same molecular mechanism causing the cleft

**FGFr2 signaling loop anomaly

Front 12

12. What are four dental abnormalities that are associated with clefting?

Back 12

1. Hypodontia

2. Asymmetric development of teeth

3. Delayed eruption of permanent dentition
(upper & lower, worse if hypodontia is present)

4. Smaller size of permanent teeth

Front 13

13. What is the most commonly abnormal tooth in orofacial clefting?

What anomalies can occur with lateral incisor?

Back 13

Cleft Lateral Incisor

1. May erupt distal to the cleft
2. Frequently missing
3. Often peg shaped
4. Divided into two
(result in supernumerary teeth)

Front 14

14. In what type of clefting and teeth are supernumerary teeth more common?

What is the severity of deformity of the lateral incisor inversely related to?

Back 14

More common in deciduous teeth and CL(P)

Inversely related to severity of clefting

Front 15

15. Where does hypodontia outside of the cleft more common occur mandible or maxilla?

How does the prevalence of hypodontia relate to the severity of clefting?

What is the prevalence of hypodontia in non-cleft population?

Which teeth seem to be most commonly affected (not including 3rd molars)?

Back 15

Maxilla is more commonly affected

Prevalence increases with severity of clefting

3-11%

2nd Pre-molar

Front 16

16. Where is the highestest incidence of hypodontia outside of the cleft seen?

Back 16

Highest incidence is in syndromic clefting

**Pierre-Robin 50%

**Van der Woude 69%

Front 17

17. When is ultrasonographic identification of cleft and associated anomalies possible?

What is done at this time?

Back 17

Between 13 and 16 wks gestational age

Parents are referred to cleft team to discuss surgical interventions necessary for treatment of cleft

Front 18

18. What can be identified from birth to 3 months?
(three things)

What intervention is done at this stage?

Back 18

1. Airway anomalies
2. Apnea
3. Delineation of other anomalies

1. Early intervention to promote near-normal feeding

2. Familial psychosocial intervention

Front 19

19. What dental and/or surgical treatment can be done from birth to 3 months?

Back 19

Application of maxillary orthopedics

First-stage cleft lip repair

Front 20

20. Which orofacial clefting has a greater degree of severity of feeding difficulty?

What are feeding difficulties related to?

Back 20

Greater in cleft palate

Failure to generate sufficient intra-oral suction

Front 21

21. What can failure to generate sufficient intra-oral suction result in in regard to feeding difficulties?

Back 21

1. Poor attachment to breast or artificial nipple and extraction of milk

2. Poor bolus organization

3. Poor retention before swallowing

4. Poor swallowing initiation

Front 22

22. How can disruption and/or problems in oral-nasal communication cause feeding difficulties?

Back 22

1. Aerophagia

2. Nasal regurgitation

3. Coughing, choking, gagging

4. Fatigue and prolonged feeds

Front 23

23. What are four type of feeding interventions for clefts?

Back 23

1. Feeding support by speech pathologist or nurse

2. Feeding techniques

3. Feeding equipment

4. Palatal obturators

Front 24

24. What is the range of incidence of failure to thrive (FTT) in children with clefts?

How can early feeding education affect FTT?

What do the most effective feeding interventions involve?

Back 24

32% to 49%

Reduce FTT incidence to between 9% to 26%

Weekly assessment by speech pathologist or feeding support nurse for first 3 wks of life

Front 25

25. What is the ESSR feeding technique?

Back 25

1. Enlarge (orifice of nipple)

2. Stimulate (rub buccal mucosa)

3. Swallow

4. Rest

Front 26

26. What are some other widely practiced feeding techniques that have little supporting evidence?

Back 26

1. Upright positioning

2. Responding to infant cues to assist with milk delivery

3. Limiting feeding time to 20-30 minutes

Front 27

27. What type of bottle is preferred for assisted feeding?

Which type of feeders limit aerophagia?

Back 27

Squeezable bottle

1. Pigeon nipple
2. Haberman cleft feeder

**others used are cross-cut Playtex nurser and Mead-Johnson cleft nurser

Front 28

28. What are palatal obturators?

Back 28

Passive device designed to separate oral and nasal cavities

Front 29

29. Why are palatal obturators controversial?

Three reasons...

Back 29

1. Multiple procedures are needed to refashion obturator as child grows

2. Burden of care on parents

3. Obturator may have adverse effects on proprioception and normal feeding development

Front 30

30. Do infants with CL (w/o CP) experience breastfeeding difficulties?

Do infants with CP have feeding difficulties?

Back 30

Rarely

Have significant feeding difficulties and require supplemental feeding in addition to breastfeeding

Front 31

31. How is the musculature across the cleft?

On which side is the lip short?

How are the columella and nostrils on the cleft side(s)?

Back 31

Lack of musculature

Lip is short on the lateral side(s) of the cleft

1. Columella is short
2. Nostrils are horizontally oriented

Front 32

32. What two things does the maxillary bone deficiency result in?

Back 32

1. Dental cross bite

2. Inadequate foundation for the nasal structures

Front 33

33. In the bilateral cleft lip what can happen to the lateral alveolar segments?

How is the pre-maxilla?

Back 33

Lateral alveolar segments may be collapsed behind the pre-maxilla

Pre-maxilla is forward and may be torqued

Front 34

34. When is primary cleft lip repair preformed?

What can be done alternatively?

Back 34

At 3 months

**weigh at least 10 pounds so have can have anesthesia

Maxillary orthopedics is begun at 3 months and lip repair is delayed until 5-6 months

Front 35

35. What are the goals of primary cleft lip repair?

Back 35

1. Restoration of function and anatomic balance

2. Reconstruction of the orbicularis oris

3. Lengthening of the lip skin

Front 36

36. What are the goals of primary cleft lip repair?

Back 36

4. Reconstruction of the full height of the labial sulcus

5. Closure of the nasal floor

6. Correction of the nasal tip asymmetry

Front 37

37. What is the most popular operation for cleft lip repair?

Why can simultaneous rhinoplasty be performed?

How is secondary correction with this advancement flap?

What are the advantages of it?

Back 37

Millard Rotation-Advancement Repair

B/c it provides access to the nasal cartilages

Relatively easy

Simple and flexible

Front 38

38. What are the disadvantages of the Millard repair?

Four things...

Back 38

1. Lip asymmetry
**cleft side --> lip/nostril always shorter

2. Vestibular stenosis

3. Extensive undermining required (scarring)

4. Vertical scar contracture is possible resulting in "whistle" deformity

Front 39

39. What are two advantages of geometric repairs?

Back 39

1. Symmetry between the two sides of the lip

2. Decreased risk of scar contracture

Front 40

40. What are disadvantages of geometric repair?

Four things...

Back 40

1. Violation of philtral column creates scar that crosses anatomic boundaries

2. Exacting pre-surgical measurements that lack flexibility

3. Requires experience

4. Difficult to preform primary rhinoplasty

Front 41

41. What are passive molding appliances (maxillary orthopedics) used for?

Back 41

Used after primary lip repair to prevent alveolar arch collapse caused by action of orbicularis oris

Front 42

42. What are active maxillary orthopedic appliances used for?

Back 42

Used to expand the maxillary arch and retract the pre-maxilla prior to lip repair

Front 43

43. What treatment/intervention occurs at age 3 to 12 months?

Six things...

Back 43

1. Early speech & language intervention

2. Evaluate primary dentition and preventive strategies

3. Repair of cleft palate at age 9 to 12 months

4. Placement of myringotomy tubes

5. Auditory assessment

6. Secondary cleft lip repair

Front 44

44. Why is placement of myringotomy tubes done?

Back 44

So baby can hear and develop normal speech

Front 45

45. What two things can early repair (age 9-12 months) result in?

Back 45

1. Prevents delayed speech and language development

2. Results in maxillary mid-facial growth deficiency

Front 46

46. What two things can late repair (age 8-15 years) result in?

Back 46

1. Mid-facial growth deficiency is prevented

2. Normal speech is never achieved

**can't make G, K, L, M, or N so have compensatory articulation

Front 47

47. What are three types of palatoplasty?

Back 47

1. Two-flap palatoplasty

2. V-to-Y pushback

3. Furlow double opposing Z-plasty

Front 48

48. What does the Furlow double opposing Z-plasty do?
(two things)

What does this technique decrease the risk of developing?

Back 48

1. Closes communication between the oral and nasal cavities

2. Lengthens the soft palate and correctly aligns the musculature

Velopharyngeal Insufficiency (VPI)

Front 49

49. When does VPI occur?

What occurs with VPI?

What are two results?

Back 49

When eat or speak

Soft palate elevates but doesn't touch

Regurgitation or air escapes

Front 50

50. What treatment occurs during toddler or pre-school years?

Four things..

Back 50

1. Continued speech/language, audiologic and dental intervention

2. Repair of palatal fistules

3. Secondary repair of cleft lip

4. Tonsilectomy for sleep apnea

Front 51

51. What treatment occurs during the primary school age?

Back 51

1. Surgical management of VPI

2. Neurological evaluation if child presents with oromotor symptoms (problems chewing/swallowing)

3. Repair of alveolar ridge defect w/ bone grafting

4. Transverse maxillary expansion

5. Initiation of orthodontic treatment

Front 52

52. During the primary school age what are then major treatment/interventions?

(four)

Back 52

1. Surgical management of VPI

2. Repair of alveolar ridge defect with bone grafting

3. Transverse maxillary expansion

4. Initiation of orthodontic treatment

Front 53

53. In the correction of VPI what do pharyngeal flaps form a permanent bridge between?

What type of patient is a pharyngeal sphincteroplasty reserved for?

What two things can all surgical methods of VPI correction result in?

Back 53

Nasopharyngeal surface of the soft palate and the posterior pharyngeal wall that limits nasal air escape

Patients with velopharyngeal incompetence

1. Hyponasal speech
2. Obstructive sleep apnea

Front 54

54. What does alveolar bone grafting do?

Back 54

1. Stabilizes maxillary segment

2. Provides alveolar bone support for teeth adjacent to the cleft, reducing tooth loss

3. Facilitates orthodontic movement of teeth

Front 55

55. What does alveolar bone grafting do?

What is an alternative to alveolar bone grafting?

Back 55

4. Improves aesthetic appearance of alveolus

5. Improves nasal support and projection

Maxillary distraction using a transport disc

Front 56

56. What treatment/intervention occurs during teenage years?

Four things...

Back 56

1. Continued dental care
(implants for missing teeth)

2. Prosthodontic care for persistent VPI and oronasal fistulas

3. Continued orthodontic care coordinated with orthognathic surgery

4. Secondary septorhinoplasty

Front 57

57. When is orthognathic surgery performed?

What does orthognathic surgery correct?
(three things)

Back 57

When facial growth is nearly completed

**15-16 in females
**17-18 in males

1. Malocclusion
2. Prognathic profile
3. Speech problems assoc w/ anterolateral open bite

Front 58

58. In terms of psychosocial issues in teenagers with clefts what are they at greater risk for?

Back 58

Social competence problems
-development of friendship
-progress in school
-participation in organizations

**not at greater risk for psychopathology