Cleft Lip and Palate

Question 1

What is the epidemiology of cleft lip/palate?

Answer 1

-Cleft lip w/ or w/o palate: 1/940
-Isolated cleft palate: 1/1574
-Cleft lip more likely in males on left side
-Isolated cleft palate more likely in females on left side (delay in rotation)
-50% of isolated cleft palate is syndrome related (15% of CL/P associated with syndrome)

Question 2

What is the embryology of cleft lip/palate?

Answer 2

-6 weeks gestation: Median nasal process fuses with maxillary process. Failure results in clefting of lip/alveolus
-8-12 weeks gestation: Palatine shelves of maxillary process merge at midline.

Question 3

What are risk factors in cleft lip/palate?

Answer 3

-Family history
-Maternal smoking/alcohol use
-Maternal zinc deficiency
-Advanced parental age
-Folate deficiency in periconception period
-Medications: Retinoids, corticosteroids, anticonvulsants

Question 4

What is the classification system for CL/P?

Answer 4

Veau system
-Group 1: Soft palate only
-Group 2: Cleft of soft and hard palate
-Group 3: Complete unilateral lip/palate
-Group 4: Bilateral cleft lip and palate

Question 5

Who are the members of the cleft team?

Answer 5

-Surgeon (OMFS, ENT, Plastic)
-Pediatric dentist
-Orthodontist
-SLP
-Geneticists
-Audiologists
-Social Worker
-Psychologist
-Pediatrician

Question 6

How early can CL/P be diagnosed?

Answer 6

-Ultrasound can identify clefting as early as 13-14 weeks gestation
-More sensitivity after 20 weeks

Question 7

Describe the sequence of surgical management for CL/P

Answer 7

-At birth: Adjustments to feeding with special bottles, lip/nasal taping (goal is 5 mm defect)
-Age 3-4 months: Lip adhesion (separate procedure of a staged lip repair).
-10 weeks of age: Primary lip repair
-6 months: Audiology screening (eustachian tube dysfunction)
-9-18 months: Primary cleft repair (Earlier restricts maxillary growth more, but want to do it before child starts speech development)
-3-5 years: Correction of velopharyngeal insufficiency
-6-9 years: Alveolar grafting (based on canine root 1/2-2/3 formed)
-Early orthodontics for transverse growth
-14-16 in females, 16-18 in males: Orthognathic surgery
-6-12 months after orthognathic surgery: Rhinoplasty

Question 8

How is the primary lip repair typically completed?

Answer 8

Millard Rotational-Advancement flap

-Three layered closure: Skin, orbicularis oris and mucosa. Excise hypoplastic tissue

Question 9

Why do vermilion deformities occur in cleft repair, how is this managed?

Answer 9

-Inadequate approximation of the marginal portion of the deep orbicularis or with medial/lateral white roll, excessive resection of vermilion.

-Most common in bilateral clefts

-May require revision surgery

Question 10

What is whistle lip deformity and how is it treated?

Answer 10

-Indentation at the junction of the vermilion results in inadequate bulk of the lip with excessive show of incisors
-Caused by inadequate release and advancement of mucosa and the vemilion

-Treat included non-keratinized epithelium mucosal-submucosal flap procedures or fat grafting. May require an Abbe flap

Question 11

How is a hypertrophic scar treated s/p cleft lip repair?

Answer 11

-Watchful waiting
-Scar revision
-Steroid injection

Question 12

How is nasal asymmetry treated s/p cleft lip repair?

Answer 12

-Can be done at time of nasolabial revisions
-May require definitive rhinoplasty for management of asymmetry after orthognathic surgery

Question 13

What are the goals of cleft palate repair?

Answer 13

-Watertight closure of the oronasal communication
-Repair the musculature within the soft palate which is critical for normal creation of speech

-Tensor and levator veli palatini, palatoglossal and palatopharyngeal muscles abnormal insert into the posterior hard palate in an AP orientation and have to be reoriented into the normal transverse alignment

Question 14

Describe the technique for cleft palate repair.

Answer 14

-Furlow Z-Plasty

-Allows for lenghtening of the hard palate
-Myomucosal on one side, mucosal on other. Technically difficult and higher rate of oronasal fistula

-Bardach Two flap technique: Two flaps on greater palatine artery. Either two layers (hard palate-nasal and oral mucosa) or three layers (soft palate, nasal mucosa, muscle, and oral mucosa)

Question 15

How is oronasal fistula s/p palate repair managed?

Answer 15

-Most common sites are junction of soft/hard palate and incisive foramen
-Small fistula can be repaired with a palatal flap repair
-Large fistula may need larger flaps
-May delay repair until after growth to prevent maxillary restriction

Question 16

How is velopharyngeal incompetence managed s/p palate repair?

Answer 16

-Hypernasality and nasal emission
-Confirmed with nasoendoscopy with or without videofluroscopy
-Treatd with pharyngeal flap, pharygoplasty, posterior pharyngeal wall augmentation, palatal revision

Question 17

What is VPI?

Answer 17

-Velopharyngeal incompetence
-Incomplete closure of the velopharyngeal sphincter, incomplete separation of oral and nasal cavity during speech

Velopharyngeal dysfunction is when there is some sort of malfunction but an unknown cause

Question 18

What are signs/symptoms of VPI?

Answer 18

-Hypernasal speech: Nasal resonance during production of vowels
-Nasal emission: Nasal air escape during production of consonants
-Nasal substitution: B becomes M, D becomes N
-Compensatory misarticulations: Can’t produce P and S sounds (fricative sound), try to create pressure at other levels

Question 19

What is the role of SLP in diagnosis of VPI, when is it diagnosed?

Answer 19

-Determining if VPI is a true physical limitation (surgery) vs habituation of patterns that would be treated with speech therapy
-Usually can diagnose at age 3-5 as child’s speech increases

Question 20

When is surgical timing of VPI, what are the contraindications to surgery?

Answer 20

-Around age 5

-Contraindications: Patient declines surgery, suspected airway obstruction, responds well to speech therapy
-Velocardiofacial syndrome: Anomalous internal carotid arteries. Need pre-op cervical vascular imaging

Question 21

What is Passavant’s ridge?

Answer 21

-Bulge on the posterior pharynx above the arch of the atlas produced by forceful contraction of the superior pharyngeal constrictor

Question 22

What is the goal of Pharyngeal flap surgery, how is this accomplished?

Answer 22

-Develop a functional seal between the nasal cavity and oral cavity
-This is accomplished by creating a velopharyngeal port (taking tissue from posterior pharynx and attaching to soft palate (decreasing opening between nasal/oral cavity). Via superiorly based pharyngeal flap

Question 23

What are the goals of alveolar cleft grafting?

Answer 23

-Allow eruption of teeth
-Provide support of adjacent periodontium
-Stabilize maxillary segments
-Closure of oronasal fistulae
-Improve speech/language
-Reconstruct nasal floor

Question 24

What are the different ages (and nomenclature) for timing of alveolar bone grafting?

Answer 24

-Primary (at or before the time of cleft lip or palate repair). Typically not done because it restricts growth too much

Secondary:
-Early: 3-5
-Early mixed dentition: 6-8
-Late mixed dentition: 9-12
-Late: 13 and older

Question 25

What is the most common age of alveolar bone grafting?

Answer 25

-6-8 years old
-When canine root is 1/2 to 2/3 formed

Question 26

What are the overall concepts of alveolar bone grafting?

Answer 26

-Fill pyramidal bone defect caused by the cleft
-Anterior flaps raised, nasal floor is then recreated, palatal (posterior) is recreated
-Split thickness component: As incision carried superiorly, go split thickness for nasal floor
-Full thickness component: Anterior flaps (around teeth/papilla)

Question 27

What is the blood supply to a bilateral cleft premaxilla and how does that affect surgery?

Answer 27

-Posterior septal artery (mostly), lateral nasal artery, anterior ethmoid artery

-Run superior to inferior, so limit superior dissection

Question 28

What bone graft is used in alveolar cleft grafting?

Answer 28

-Anterior iliac crest (cancellous)

Question 29

How is graft exposure treated s/p alveolar grafing?

Answer 29

-Conservative therapy
-Antibiotics, chlorhexidine, removal of sequestrate

-Allow 3 months between surgery prior to attempting closure to allow for scar maturation

Question 30

How is oronasal fistulae treated s/p alveolar grafting?

Answer 30

-Local vs larger flaps for closure
-May wait to repair to prevent growth restriction

Question 31

What is BMP?

Answer 31

-Bone morphogenic protein
-Protein complex belonging to a family of growth factors that promote osteoinduction.
-Usually placed in a carrier like a collagen sponge