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Head and Neck / CF > Cleft lip, palate & nasal deformity; VPI > Flashcards

Cleft lip, palate & nasal deformity; VPI Flashcards

1
Q

Describe features of unilateral cleft nasal deformity

A

1- Maxillary hypoplasia (posteriorly retruded, inferiorly displaced 2- ANS and nasal septum deviated toward non-cleft side 3- Posterior septum concave in cleft airway causing obstruction 4- LLC: obtuse angle of middle crura 5- LLC: caudal posterior attachment to piriform 6- LLC: lateral crural buckling 7- Tip deviated to non-cleft side 8- Columella shortened 9- Wide nostril floor 10- Horizontal nostril

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2
Q

Describe features unique to bilateral cleft nasal deformity

A

1- Prolabium retracted into shortened columella 2- Flat tip, Wide base 3- Absence of nasal floor 4- Alar flaring 5- ANS and caudal septum as displaced caudal relative to alar bases

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3
Q

What is the theory for the aetiology of cleft nasal deformity?

A

McComb - LLC is center pt of deformity Fisher - posteriolateral displacement of maxilla is centre pt of deformity

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4
Q

What are surgical options for unilateral cleft lip nasal deformity

A
  • Alar mobilization an suspension (Millard - 3 pt suspension with bolsters, Potter - recreate scroll by suspending LLC to ULC) - Alar incision and retroposition - External approach w rotation of cleft nasal lobule - Graft augmentation
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5
Q

What are surgical options for bilateral cleft nasal deformity

A

1- Alar mobilization, fibrofatty removal and lengthen columella with incision down centre columella and along bases (Salyer at 1y) 2- Forked flaps (at 5y) 3- Composite ear lobe graft

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6
Q

Define cleft lip

A
  • Congenital malformation of the lip due to embryological failure of fusion of the median nasal prominence with the maxillary prominence
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7
Q

What is the incidence overall and racial incidence of CL/P and CP?

A
  • CL/P Overall 1/1000 (0.1%)
    • Asian 1/500
    • Caucasian 1/750 - 1/1000
    • Black 1/2000
  • CP Overall 1/2000; no racial differences
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8
Q

In counselling a family regarding risk to the subsequent child

  1. what is the risk with one parent and one child with CL/P for the next to have CL/P?
  2. What is the risk when one child has CP but no parents, for next CP?
  3. What is the risk when 2 children have CL/P for next CL/P?
  4. What is the risk when the husband has CP and they don’t have children yet, for next to have CP?
  5. What is the risk when the wife has CL/P and they don’t have children yet, for next to have CL/P?
A

1: 17%; #2: 4%; #3: 9%; #4: 6%; #5: 4%

Family Members with CP

Prob Next Child w/ CLP(%)

Prob Next Child with CP (%)

Frequency Gen Pop

  1. 1
  2. 04

1 affected child

4

4

1 affected parent

4

6

1 affected child + FH (unaffected parents)

7

7

Unaffected parents + 2 affected children

9

1

1 affected parent + 1 affected child

17

15

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9
Q

What is the etiology of CLP?

A
  • Combination of genetic and environmental factors
    • Modifiable factors: maternal teratogen (phenytoin specifically associated w/ 10x increased risk) - phenytoin/valproate & other anticonvulsants, EtOH, steroids, diazepam; smoking; nutrient deficiency (folate, B6)
    • Non-modifiable factors: infectious (rubella, toxoplasmosis), FHx, incresed parental age
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10
Q

Name 2 chromosomal abnormalieis that are associate wtih CL/P or CP

A
  • trisomy 21
  • 22q11 deletion syndrome
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11
Q

Name the most common syndrome associated specifically w CL/P.

List 5 other syndromes associated w CL/P or CP

A
  • Most common syndrome associated w/ CLP is Van der Woude syndrome
  • Other syndrome associations include: Stickler, Waardenberg, Gorlin, Treacher-Collins, HFM/Goldenhaar
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12
Q

Discuss the embryology of the oropharyngeal/lip/palate region

A
  1. Week 3: NCC migrate into / to form the FNP, Maxillary Prominence and Mandibular prominence
  2. Week 4: FNP divides into medial and lateral nasal prominences; the MNP moves toward the midline
  3. Week 4-6: MxP merges w/ LNP; then MxP merges toward the MNP
  4. Week 6: paired vertical projections form on each side of the tongue from the MxP (lateral palatine processes)
  5. Week 7: tongue withdraws / descends, the lateral palatine processes ascend from vertical to horizontal (right before left)
  6. Week 8: IMO of hard palate, as lateral palatine processes fuse
  7. Week 7-12: growth centres along caudal end of the palatal shelves (lateral palatine processes) fuse to form soft palate
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13
Q

What does each primordial facial prominence give rise to?

A
  • FNP: MNP, LNP –> dorsal nose
  • MNP: nasal tip, septum, premaxilla, primary palate, columella, midline lip (including philtral column)
  • LNP: nares / nostril sill
  • MxP: lateral upper lip, cheek, 2’ palate, zygoma
  • MdP: chin, lower lip, mandible (condyle via EO; rest via IMO; malleus via IMO)
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14
Q

In one sentence, describe the pathophysiology of cleft lip +/- cleft of primary palate

A

Failure of fusion of medial nasal prominence with maxillary prominence results in cleft of the primary LIP & palate (lip, alveolar process, hard palate anterior to incisive foramen); weeks 4 – 7

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15
Q

How would you classify defect of cleft lip and primary palate?

A
  • Side: uni (R vs L) vs bilateral
  • Completeness: incomplete vs. complete
  • Association w/ palate: CL alone vs. CL + primary palate vs. CL + primary palate + 2’ palate
  • Formal classification system: Kernahan striped Y
    • advantage is an embryologic system
    • includes complete/incomplete defects; differentiates between Lip and primary and secondary alveolus
  • Others: LAHSHAL
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16
Q

Describe the findings associated w UNILATERAL CLEFT LIP AND PRIMARY PALATE

A
  • Maxilla
    • Non-Cleft side: lateral segment is retropositioned; the alveolus & piriform margin is hypoplastic and retropositioned
    • Cleft side: premaxilla outwardly rotated & projecting
  • Lip
    • Cleft side:
      • Philtral column / lip height is short
      • Cupid’s bow is angled superiorly toward cleft margin
      • Vermillion is deficient under cleft half of cupids bow
      • Orbicularis oris inserts into cleft margin and superiorly to columella
    • Non-cleft side
      • Cupids bow peak is less well defined (Noordhof’s point)
      • Vertical height at cleft margin is short (complete cleft) or excessive (incomplete cleft)
      • Aberrent insertion of OO - to alar rim and piriform - causes lateral bulge
      • Lateral lip length is short
  • Nose - 7 key features
    • Alar base is retropositioned and inferiorly displaced
    • Shape of nostril is horizontally oriented
    • Tip is deviated to non-cleft side; flat
    • Columella is short and deviated to non-cleft side
    • Septum bulges to cleft side, but can be dislocated onto non-cleft side
    • ULC and LLC do not overlap
    • Oblique angle between middle and lateral crura of LLC
    • Alar rim is distored by a vestibular web
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17
Q

What are the goals of presurgical orthodonia?

A
  • Reduce tension on repair
  • Bring cleft and lateral alveolar segments into closer apposition
  • decrease the cleft lip nasal deformity
  • Permit the use of GPP if desired
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18
Q

Compare the advantages and disadvantages of various pre-operative interventions prior to cleft lip repair

A
  • Orthodontia (active = Latham vs. passive = NAM)
    • advantages: no scar to lip, decreases alveolar gap, decreases tension on lip repair, helps to align the alveolar margins, improves appearance of lip repair and nasal deformity, permits GPP if desired
    • disadvantages: cost, compliance, visits, ? delay lip surgery, ? impair facial growth
  • Lip adhesion (simple suturing of lip margins together)
    • advantages: decreae tension on repair at definitive lip repair, decrease alveolar gap, permits GPP if desired
    • disadvantages: induce scar so definitive lip repair not in virgin field, negative influence of definitive lip repair
  • Gingivoperioplasty (suturing givgeval margins together)
    • advantages: thought to decrease need for 2’ bone graft, may allow normal tooth eruption
    • disadvantages: may not decrease need for 2’ bone graft (or bone made not of sufficient quality); adverse effects on midface growth (?)
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19
Q

Why would you want to repair a cleft of lip and primary palate? (what are the OVERARCHING goals?)

A
  1. separate nasal and oral cavity
  2. faciliate speech and eating
  3. promote facial skeleton growth
  4. cosmesis and social function
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20
Q

What are the goals of CLEFT LIP surgery?

A
  1. Symmetrical outcome
  2. Medial and lateral lip elements are reapproximated without compromise to natural landmarks
  3. Scars fall into natural landmarks or along subunits
  4. Restore/reconstruct muscle continuity
  5. Discerning excision of poor quality tissue
  6. Similar nare circumference and alar base position
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21
Q

What are the goals of surgery for cleft lip?

A
  • Accurate marking and a customized approach / plan for each cleft / patient
  • Augment short/deficient medial lip with lateral lip
  • Precise alignment of natural landmarks between medial and lateral lip (vermillion-cutaneous junction, red line, cupids bow peak, nostril sill, etc)
  • Release of muscle from skin and mucosa and aberrent insertions to allow tension free closure
  • Varied approach to primary rhinoplasty - principle to achieve symmetry of alar base and alar circumference
  • Closure of gingeva and varied approach to GPP
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22
Q

Describe a timeline for comprehensive cleft care

A
  • Prenatal diagnosis - meet with family, SW, genetics
  • Post-natal - first 2-5 wks of life: surgeon, dentist, prosthedontist - start NAM (preoperative orthodontia)
  • 3 months - cleft lip repair
  • Prior to CP repair - meet with ENT
  • 6-12 mos: CP repair; grommet tubes/tympanoplasty
  • 2-5 years: followed by team; specific assessments by SLP; VPI repair if present and not responding to non-operative interventions (5-7yr +)
  • 5-11 years: dentist/orthodontist assessments, orthodontia
  • ~ 9-11 years: at mixed dentition (prior to canine eruption) alveolar bone graft
  • 11 yrs +: orthodontics adjusted
  • skeletal maturity (16F, 18M) - orthognathic surgery
  • after orthognathic surgery: primary rhinoplasty (if not addressed around 5-6 yrs)
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23
Q

What is the Rose-Thompson effect?

A
  • the approximately 1mm length gained by reapproximation of angle lines
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24
Q

What is the Rose-Thompson repair? Advantages and disadvantages

A
  • repair utilizes a semi-straight line repair (2 angles lines) with a vermillion flap from lateral to medial
  • advantages are minimal scar, scar along philtrum, ease of repair; ideal for mild or microform clefts
  • disadvantages are short lip, notch due to short lip
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25
Q

What is the Randall-Tennison repair? Advantages & disadvantages?

A
  • The Randall-Tennisson repair is a triangular flap repair, where the triangular flap / z-plasty component occurs at the cutaneous roll above vermillion cutaneous junction
  • advantages
    • easy to learn bc standardized technique and measurements
    • can be used for wide or narrow clefts
    • preserves cupids bow, lengthens the lip, narrows nasal floor, lengthens the shortened medial lip
    • minimal dissection
  • Disadvantages
    • little flexibility
    • deformity/scar crosses the philtrum, flattens the philtral dimple
    • associated w/ late lengthening
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26
Q

What is the Millard CL repair? Advantages and disadvantages

A
  • Millard repairs is the rotation advancement repair
  • utilizes a C flap to either close the nasal sill or lengthen the columella
  • advantages:
    • preserves cupids bow, philtral dimple, scar mirrors philtrum for inferior 1/2 of philrum, narrows nostril sill
    • little discard of tissue
    • flexible; cut as you go
  • disadvantages
    • not good for wide clefts; tension
    • difficult to learn
    • can result in short medial lip vertical height; short lateral horizontal length; narrow nostril; scar does not mirror philtrum in upper 1/2
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27
Q

What is the fisher repair? advantages and disadvantages

A
  • the fisher repair is the anatomic subunit repair
  • advantages
    • scar at base of nose is minimized
    • nostril sill closure is uninterrupted
    • scars fall along anatomic subunits / philtrum
    • cutaneous roll / white roll / vermillion (ie lip landmarks) are restored/preserved/maintained
    • lateral lip length is not compromised to ahcieve vertical length
  • disadvantages
    • complex, difficult to learn/teach, technically challenging
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28
Q

Describe your cleft lip repair

(below is description of Fisher’s repair)

A
  1. prep/drape/position neck hyperextended/thoat pack/eye tapes
  2. First I will make my skin markings on nose
    1. mark the midline and height of the philtral column
    2. mark the position along the lip columellar crease on non-cleft side; mirror this on cleft side
    3. mark sub-alare
  3. Then I will make markings on medial / cleft side lip
    1. mark midline and cupids bow peak (cleft side mirror non-cleft side)
    2. mark in white role perpendicular to vermillion cutaneous junction, above midline and cupids bow peak
    3. mark approximately 1mm opening cut in line with cleft-side cupids bow peak, along white roll
    4. mark positions along red-line below cupids bow peak; connect witha line the cleft-side and midline marks
    5. connect these points with a line as follows
  4. Then I will make my measurements off medial lip to clarify lateral lip markings
    1. total lip height is non-cleft side philtral height to cupids bow peak mesured at rest
    2. greater lip height is that measurement on cleft side with gentle traction
    3. lesser lip height is total minus greater minus 1mm and should be less than 2mm
  5. Then I will mark the lateral lip elements
    1. noorhof’s point
    2. point perpendicular to noordhof’s above the white role
    3. make the isoscoles triangle of lesser lip height above this point
    4. use the caliper to measure a point that is between the nasal sill closure point and the isocoles triangle that is the distance of the greater lip height
    5. make red lip markings at red line, then at a point between red line and noordhof’s point that is the same height on cleft side
    6. then make the triangle vermillion flap point of equal distance to the cleft side
  6. Then I would infiltrate w/ lidocaine and epinephrine & make my incisions following my markings
    1. on cleft side first, then non-cleft side
    2. then i would separate the muscle fromthe overlying skin and underlying muscosa, and from aberrernt insertions along piriform or alar base
    3. then I would make my relaxing incisions along the gingevobucchal sulcus
    4. then i would make a wedge excision along nasal sill (if required)
    5. then i would use an inferior turbinate flap to close the GBS if required
  7. then i would do a small primary rhinoplasty maneuver to place an interdomal suture to plicate nasal valve and restore overlap to ULC and LLC and place a cince suture in the ala
  8. then i would close in a layered fashion, internal to external, including restoring muscle continuity
  9. then polysporin and arm restraints
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29
Q

what are the clinical features of bilateral cleft lip (+ cleft 1’ palate?)

A
  • Skeleton:
    • premaxilla is projecting ahead of lateral segments, rotated cranially
    • lateral segments are retropositioned and medially collapsed
    • maxilla at alar bases / piriform is hypoplastic
  • Prolabium:
    • Absent defining features: cupids bow/peak, philtral columns, philtral dimple, cutaneous white roll, hypoplastic vermillion
    • Absent muscle
    • Deficient GBS
    • blood supply from posterior septal artery
  • Lateral lip
    • verticaly and horixontally short
    • bulge muscle, OO aberrant insertion (alar base, piriform)
  • Nose
    • flat, wide tip
    • short columella
    • wide ala, alar bases retro and inferiorly positioned
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30
Q

What are the principles of treatment for bilateral cl/p?

A
  • need to align 3 maxillary segments
  • pre-surgical orthopedics (weight passive vs. active orthopedics vs. lip adhesion vs. GPP)
  • consider pre-surgical botox to reduce tension on repair
  • simultaneous repair of bilateral clefts
  • reduce the prolabium
  • recreate the cupids bow and tubercle from lateral lip elements
  • repair muscle in midline
  • use prolabial mucosa to deepen the GBS
  • primary rhinoplasty to address tip projection, alar shap
31
Q

List some characteristic deformities that can occur after bilateral CL/P repair

A
  • whistle notch deformity
  • scar across central tubercle
  • compromise to labial blood supply
  • chapped prolabial mucosa
  • contracture of cupid bow peak
  • wide prolabium
32
Q

Describe your repair for bilateral CL/P

(below is Fisher modification of Mulliken repair)

A
  1. prep/drape, throat back, neck extension, eye tapes
  2. First I will undertake my markings
    1. First I will mark the prolabial segment
      1. Mark the midline and philtral height - approximately 2.5 mm wide
      2. Mark the inferior midline to establish height of the tie, then adjacent points for cupids bow to maximize width of prolabium and achieve ~ 5mm width
      3. Mark points along the colummelar lip crease in prolabium so that they are the medial point of closure
    2. Then I will mark the lateral lip elements bilaterally
      1. Noordhof’s point and point above white roll perpendicular to this
      2. Subalare
      3. Point along nostril sill to meet the medial point of closure
      4. Point lateral too noordhof’s point that equals the distance of the inferior dimension of the prolabial tie, and again along the white roll perpendicular to vermillion
      5. point medial to noordhof’s along the lateral lip skin near cleft margin, such that the distance between the points lateral and medial to noordhof’s point equal the height of the tie on the prolabium
      6. point along red lip, at red line, inferior to Noorhof’s point such that these points are all perpendicular to the vermillion cutaneous junction
  3. infiltrate local with epi and make my incisions following my markings
    1. first I will incise lateral lip
    2. release orbicularis from skin and mucosa and aberrant insertion
    3. incise and inset my turbinate flap
    4. then i will incse the prolabium
    5. elevate the prolabium mucosal flap & tack to periosteum
    6. narrow alar bases with alar base cinch stitch
  4. Layered, tension free closure including muscle repair in midline
33
Q

Describe the post-operative management for a cleft lip repair

A

· Breast or bottle-feeding allowed in the immediate perioperative period (no increase in complications)

· Prophylactic antibiotics - not used by all surgeons

· Arm guards to prevent self-harm of repair

· Continuous monitoring not generally employed

· Discharge as soon as feeding well (often POD#1)

· Suture removal - 1 week

34
Q

what are general complications for cleft lip repair?

A

· Early (Wound infection, dehiscence, bleeding)

· Late (Scarring, secondary deformities)

35
Q

list secondary deformities after cleft lip repair, understand why it happened, and indicate your plan

A
  1. note: for major deformities, approach is to take down and re-do the repair or consider an abbe flap
  • short medial lip - common after Millard, 2’ inadequate advancement or recruitment of lateral lip element - if < 1mm then rose-thompson diamond excision and closure; if 1-3mm then add z-plasty; if > 3mm then redo
  • tight lip - seen in bilateral, after pts/ w no NAM; minor - fat injection, reduce lower lip; major Abbe
  • Wide lip centrally - common after bilateral - excise excess and restore anatomic landmarks
  • Whistle notch - inadequate tubercle or vermillion - common bilateral (also seen unilat) - minor - V-Y, z-plasty in vermillion, fat graft; major - de-ep a medially based mucosal flap vs. Abbe
36
Q

DESCRIBE THE ABNORMAL ANATOMY SEEN IN CLEFT PALATE

A
  • Bone: variable extent of HP hypoplasia
    • UCLP: vomer attached to non-cleft palatal shelf
    • BLCP: vomer not attached to palatal shelf
    • iCP: vomer attached to anterior extent of cleft
    • incomplete CP, soft palate only - vomer attached as usual
  • Muscle
    • LVP: AP orientation, attache to cleft margin and posterior HP; hypertrophy
    • TVP: AP orientation, attach to posterior HP lateral to LVP; hypotrophy (of aponeurosis)
    • PP, PG: abnormal anterior insertions into cleft margin and HP w/ LVP
      • PP hypertrophy, makes Passavant’s ridge; PG hypotrophy
      • LVP/PP/PG abn insertions - “Veau’s muscle”
  • Fascia
    • no palatal aponeurosis from TVP on cleft side
37
Q

List syndromes associated w/ CP

A
  • Stickler
  • VCF/22q
  • TCS/Nager
  • Moebius
  • Van der woude
  • Wardenberg
  • Orofacialdigital
  • Klippel Feil
38
Q

List the members of a CL/P team:

A

Plastic surgeon, ENT, pediatrician, ophthalmologist, orthodontist, dentist, prosthodontist, audiologist, SLP, genetics, social work, clinical psychologist/psychiatrist, nurse, patient, coordinator

39
Q

List the major issues to be addressed for a CP patient, and major considerations for each.

A
  • Airway:
    • routine: infant “used” to larger airway, which is smaller after CP repair; therefore close post-operative monitoring in monitored setting
    • high risk PRS patients - pre-operative interventions/considerations and post-operative monitorong/LOS (monitor, suppl O2, prone, NPT, tongue-lip, DO, trach)
  • Feeding
    • nasal regurgitation and difficulty establishing suck (swallow ok) - use haberman nipple, feed w/ EHOB, frequent feeds, monitor weight gain, nutrition assessment, consideration for PPI/NG/g-tube (esp PRS)
  • Middle ear disease:
    • prone to otitis media and CHL - ENT assessment, myringotomy and tubes for majority at time of CP repair
  • Assessment for another anomalies / syndromes
    • up to 40%+ of iCP are associated w/ syndrome, others w/ other cardiac, CNS, skeletal abn - therefore genetics referral and w/u
  • sequellae of unrepaired/repaired CP
    • Maxillary growht impairment, abnormal tooth growth & occlusion, speech & VPI, other issues discussed (middle ear, feeding, OSA etc)
40
Q

what are the goals of CP repair?

A
  • water tight closure of cleft and re-establish separation of oral and nasal cavities without fistula
  • Recreate velo-pharyngeal sling of sufficient length to promote speech, feeding and prevent VPI
  • Re-establish levator / muscular sling
  • Minimize growth disturbrance (maxillary, alveolar)
  • Minimize and address eustacian tube dysfunction and middle ear disease
41
Q

discuss considerations, advantages, disadvantages of different timing of repair to CP

A
  • Early (3-9 mos)
    • Adv: Improved speech outcome, less VPI surgery
    • Disadv: more maxillary/alveolar growth disturbance, more difficult, ? more fistula
  • Intermediate (9 - 14 mos)
    • aims to maximize advantages of both and minimize disadvantages of both
    • utilized at my institution
  • Late (6 - 14 yrs; occas 2-stage w/ SP closure @ ~ 1 yr & HP closure @ 6-14yr)
    • Adv: ? less Mx/alveolar growth disturbance
    • Disadv: worse speech outcome, chronic OM, fistula
42
Q

Briefly describe the Von Langenback repair, outline advantages & disadvantages

A
  • Procedure involves bipedicle mucoperiosteal flaps developed using lateral relaxing incisions; blood supply dominant off greater palatine a
  • for complete/incomplete clefts of HP
  • Adv: better blood supply, less Mx growth restriction vs. some operations, simple closure
  • Dis: difficult to close 1’ cleft, no lengthening at closure
43
Q

briefly describe the Veau-Wardill-Kilner CP repair

A
  • This repair is a unipedicle repair, with mucoperiosteal repairs based on the greater palatine, and flaps are closed in V to Y fashion
  • For complete or incomplete cleft of hard palate
  • Adv: palatal lengthening
  • Disadv: greatest area of denuded bone & thereofre maxillary growth disturbance; higher fistula rates
44
Q

Describe the hybrid CP repair, advantages & disadvantages

A

hybrid repair as described by Gillett and Clarke, is indicated specifically for unilateral completel cleft lip and palate

it combines on the cleft side, a bardach unipedicle flap, and on the non cleft side a von-langenback bipedicle flap using relaxing incisions

is primary advantage is to close the cleft of the primary palate and reduce the likelihood of fistula formation behind the incisive foramen. there is less periosteal stripping than some methods (for less growth restriction). it also lengthens on cleft side

disadvantages include still some stripping & growth disturbance

45
Q

Describe the Bardach 2-flap CP repair and advantages/disadvantages

A
  • bardach uses 2 unipedicle flaps based on greater palatine arteries, but does not push them back
  • advantages: because no pushback, no tension on flaps, ? less fistula; allows 2 layer closure of fistula behind incisive foramen
  • disadvantages related to amount of periosteal stripping and therefore ? more Mx growth disturbance
46
Q

Describe & compare options for treatment of soft palate cleft

A
  • Furlow double opposing palatoplasty
  • Intravelar veloplasty
  • What
  • Opposing Z-plasties from oral and nasal surfaces
  • Muscular flaps must be based posteriorly to remain fxn’l
  • Divide abnormal insertion of the levator/tensor muscles from back of the hard palate
  • Reposition muscle to recreate the levator sling across the midline
  • Whom
  • Isolated cleft of SP [or combined w/ HP proc]
  • Submucous cleft
  • Correct VPI: small central defect, high closure
  • Any cleft of SP (ie w/ or w/o HP)
  • W/ HP repair
  • Adv
  • Lengthen SP
  • Preservation of vasc to muscle
  • Restore muscle sling
  • Anatomic restoration of muscle sling
  • No lateral tightening
  • Disadv
  • Lateral tightening
  • Less anatomic
  • Asymmetric
  • More difficult if wide
  • More muscle dissection
47
Q

Describe surgical treatment options for the palate, for the following scenarios:

  1. SP only
  2. complete cleft of 2’ palate (posterior to incisive foramen, HP & SP)
  3. complete unilateral CL/P
  4. bilateral complete CL/P
A
  1. Furlow vs IVVP
  2. HP: von langenback, bardach 2-flap, veau pushback PLUS SP: IVVP vs. Furlow
  3. HP: Hybrid (Bardach + von langenback) vs. Bardach vs. Sommerlad PLUS SP: IVVP vs Furlow
  4. HP: Bilateral Veau + anteiror V flap of HP + bilateral vomer flaps PLUS SP: IVVP vs Furlow
48
Q

List complications of cleft palate repair

A
  • Early
    • hemorrhage
    • dehiscence
    • infection
    • aspiration
    • airway obstruction
    • death
    • prolonged hospitalization, dehydration
  • Late
    • oronasal fistula
    • VPI
    • Mx growth disturbance
    • Malocclusion
    • Obstructive sleep apnea
49
Q

What are potential causes for oronasal fistula after CP repair?

A
  • trauma
  • technique
  • infection
  • hematoma
50
Q

How would a patient with oronasal fistula after CP repair present?

A
  • Nasal air emission
  • Nasal regurgitation
  • Chronic crusting

See oronasal fistula most commonly just posterior to incisive fossa

51
Q

What are treatment options for secondary oronasal fistula?

A
  • Non-operative: mechanical obturator
  • Operative: principle is tension free 2-layer closure
    • redo/revision of palate repair (local palatal mucoperiosteal flap)
    • FAMM or Bucchal flap (no teeth)
    • Tongue flap
    • Distant flap - radial forearm, ALT fascia, ? add BG
52
Q
A
53
Q

What are requirements to achieve normal speech?

A
  1. airstream
  2. normal velopharyngeal function
  3. normal hearing and intelligence
  4. normal dentition and skeletal relationship
  5. normal tongue-lip articulation
54
Q

Describe the different types of velopharyngeal dysfunction

A

VPD is any abnormal velopharyngeal fxn

  1. Velopharyngeal insufficiency: structural abnormality of velum
    1. ex: un-repaired/failed CP repair; oronasal fistula, palatopharyngeal disproportion (short velum)
  2. Velopharyngeal incompetence: neuromuscular abnormality of velum
    1. obstructive apea or central paresis
  3. Velopharyngeal mislearning: functional impairment of velum, dx of exclusion
    1. deafness, developmental delay
55
Q

What are primary and secondary characteristics of cleft speech and VPI?

A
  1. Primary: nasal air escape, hypernasality
  2. Secondary: compentations (substitutions, distortions, ommissions), grimacing and abnormal tongue-lip accommodations
  3. Overall: decreased intelligibility of speech
56
Q

What would you want to know on history and physical exam when examining a cleft patient (after repair) for decreased intelligiblity of speech

A
  • History
    • Onset, duration, progression, effect to daily life
      • specific symptoms: speech, swallowing, regurgitation
    • Cleft care received (type of surgery, timing of surgery, SLP assessments, other non-opeartive or operative interventions, response to interventions incl SLP, plans for future surgeries, complications)
    • Consideration of other causes: OSA symptoms, CNS disorder, hearing abnormality & assessments, developmental delay
  • Physical
    • Intra-oral inspection: fistula, velar movement, SMC/bifid uvula, adenoids, carotid pulsations, dentition, occlusion
    • Mirror fog test
    • Perceptual SLP assessment is gold standard
57
Q

List investigations used to measure and diagnose VPI

A
  1. Perceptual evaluation by SLP is gold standard
  2. Direct: naso-endoscopy, videofluoroscopy: give closure pattern and rating
  3. Indirect: TONAR, nasometry (pressure flow pattern): gives nasalance
58
Q

How do your findings of VPI investigations influence your treatment plan for diagnosed / established VPI?

A
  1. If patient has received SLP assessments/treatment and has stagnated, this provides indication
  2. if closure pattern is circular and/or with small central closure defect and high closure rating (> 0.8-0.9) then repeat Furlow is indicated
  3. if closure pattern is coronal then spincter palatoplasty is indicated
  4. if closure pattern is non-coronal and closure rating is low (<0.7-0.8) then posterior pharyngeal flap pharyngoplasty
59
Q

Describe a pharyngeal flap pharyngoplasty

A

· Creates a single subtotal central obstruction w/ openings lateral

· Best for sagittal or circular closure pattern à pre-op lateral wall motion a necessity

· Raising the flap – width should meet lateral excursion of LPW; Height to atlas

· Superiorly based = more tissue, easier hemostasis, higher base (PALPATE for carotids before cutting)

· Raise lateral edges 1st, periosteal elevator off of prevertebral fascia, release V-shape caudally

· Soft palate: fish-mouth incision (submucosal pocket), inset flap (height/width of flap planned pre-op)

· Lateral ports: lateral flap to lateral tonsillar pillar, 1cm2

60
Q

Describe Sphincter pharyngoplasty

A

· Decreases x-sectional area of central port & narrows central VP orifice

· Proposed to be best for coronal type closure

· Rearranges palatopharyngeus myomucosal flaps raised from posterior tonsillar pillars, transposed to post pharyngeal wall & each other (Ortichochea) vs. cross-over (Hynes)

· Advantages

o Soft palate not violated

o Less tethering during later Lefort I advancement

o Secondary adjustments possible

61
Q

Describe complications of a pharyngoplasty procedure

A
  • Early: hematoma, dehiscence, infection, airway obstruction, aspiration
  • Late: sleep apnea, nasal obstruction, hyponasality, need for take-down procedure
62
Q

What are the goals for management of alveolar cleft?

A
  • Functional:
    • close fistula
    • establish continuous dental arch
    • to faciliate tooth growth or osteointegrated implants
    • facilitate orthodontic movement
    • improve hyegeine
  • Aesthetics
    • augment piriform rim
    • establish symmetry
    • support alar base
63
Q

List options for treatment of alveolar cleft

A
  • GPP at time of cleft lip repair
  • primary (early) alveolar bone graft
  • secondary (later) alveolar bone graft - prior to eruption of canine / mixed detnitiaton stage
  • during orthognathic surgery
64
Q

What are the characteristic facial growth disturbances observed in the CLP/CP population?

A
  • Class III malocclusion (or class II malocclusion)
  • Maxillary hypoplasia, short vertical height & decreased arch width
  • Midface retrusion
  • Increased lower facial height
  • Nasal abnormalities
65
Q

List potential operative interventions to address the skeletal / cephalometric sequellae of CLP/CP

A
  • Lefort 1 advancement, can be segmental - >>> lefort II >>> lefort III
  • Distraction osteogenesis (not routinely done any longer)
  • BSSO
  • Genioplasty
  • Principle is to advance the maxilla rather than set back the mandible.
66
Q

What are the findings seen with submucous cleft palate?

A
  • Bifid uvula
  • Zona pellucida (diastasis of velar muscles)
  • Notch posterior hard palate
  • Approximately 50% have VPI
67
Q

What are the characteristic features associated w/ unilateral cleft lip nasal deformity?

A
  1. alar base is posteriorly and inferiorly displaced
  2. nostril is horizontally oriented and wide
  3. angle between middle and lateral crura is obtuse; there is buckling of the lateral crura
  4. LLC and ULC do not overlap
  5. Alar rim distorted by vestibular web
  6. Short columella, with base devitating to non-cleft side
  7. Septum bows into cleft side aperture, but Caudal septum dislocated to the non-cleft side
  8. Nasal tip is flat, wide and deviated to non-cleft side
68
Q

What are the characteristic features of the bilateral cleft lip nasal deformity?

A
  • process marked more by symmetry
  • bilateral alar bases are posteriorly and inferiorly displaced
  • LLC/ULC do not overlap
  • nares are horizontal and wide
  • obtuse angle btween medial and lateral crura w/ buckling of lateral crura
  • short columella
  • caudal septum and spine are displaced inferior to alar base
  • flap, broad nose, sometimes bifid
  • nasal floor absent
69
Q

describe suspected etiologies of the nasal deformity associated w/ clefting

A
  1. agenesis / hypoplasia hypothesis
    1. primary deficiency of nasal structures vs
    2. secondary changes to mechanical distortion from cleft
  2. Cutting: displacement
  3. Fisher: distortion is mechanical in A/P dimension secondary to hypoplastic and retropositioned maxilla on cleft side and protruded maxilla on non-cleft side
70
Q

compare different timing of rhinoplasty for cleft lip nasal deformity

A

Timing

Advantages

Disadvantages

At primary lip repair

(Done routinely, extent and procedure varies)

  • cartilages easily moved
  • no effect on facial growth
  • possibly reduces need for 2⁰ surgery (allows more normal growth)
  • ?reduced psychological stress
  • ? stable long term results (controversial)
  • cartilages too small/fragile
  • scarring (affects future rhino)
  • ?unpredictable results long term

Pre-school age

(only if severe functional/aesthetic issues)

  • cartilages thicker and stronger
  • kids begin to notice facial differences
  • allows time for orthodontic correction of skeletal base

• may result in thick bulbous nose, (especially Asians)

Adolescent

(after orthognathic sx, completion nasal growth)

  • single definitive sx - formal rhinoplasty
  • No growth restriction/progression
  • Predictable result

• psychological effects of living with deformity

71
Q

describe your physical exam for rhinoplasty for a cleft lip nasal deformity:

A
  • Assess oral cavity - alveolar cleft, abnormal occlusion
  • NOSE
    • Skin
    • Nasal bones
    • Midvault – ULC
    • Tip
    • Alar bases, position
    • Nasal sill configuration
    • Profile – radix, hump, tip projection, NL angle, STB
    • Worm’s eye view – tip, infratip lobule, nostril length, columella direction, shape of ala, nostril sill
    • Internal: stenosis of valves, deviated septum, turbinates, etc.
72
Q

What are some things you would consider during rhinoplasty for cleft lip nasal deformity

A

Skeletal Base

·Correction skeletal base / augment the deficient maxilla prior to rhinoplasty (LeFort I, ABG) +/- minor cartilage grafts (conserve cartilage for definitive rhinoplasty)

Nasal Dorsal Bone and Cartilage

·Submucous septal resection (will help to correct the septal deformity)

·Hump reduction, osteotomies, bilateral spreader grafts

·Consider cephalic trip to LLC or even transection of LLC to address wide

Nasal Tip Cartilage

·Columella strut for support, +/- alar rim graft, rarely a tip graft

·Stents (internal and external), suture medial crura to columellar strut, approximate footplates to narrow columellar base

Skin Envelope

·Most difficult part of 2⁰ cleft rhinoplasty

·Skin at tip is deficient and thin, absent soft triangle and short columella

73
Q
A

Head and Neck / CF (27 decks)

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