Cleft Lip/Cleft Palate Flashcards
(108 cards)
1
Q
Cleft lip/palate epidemiology
A
2: 1,000 Asians
1: 1,000 Whites
0.5: 1,000 Black
2:1 Males:Females (CL/P only)
Left unilateral CL/P is the most common
L/R/BL: 6:3:1
Unilateral:Bilateral: 6:1
10% have associated syndrome
2
Q
What is the most common craniofacial abnormality?
A
cleft lip with or without cleft palate
3
Q
Embryologic cause of cleft lip
A
Failure of medial nasal processes to contact maxillary process
Lip formation at 4-7 weeks
4
Q
Muscles, blood supply and innervation of the upper lip
A
Orbicularis oris, Levator labii superioris
Bilateral superior labial arteries
Motor CN VII (facial)
Sensory CN V2
5
Q
Residual skin bridge spanning upper portion of the cleft lip
A
Simonart’s Band
6
Q
What is the most subtle sign of an incomplete cleft?
A
Slight notch at the vermillion
7
Q
Genetic factors:
A
No single gene
Isolated CP is different than CL or CL/P
Maternal age <20 or >39 increases incidence
8
Q
Familial recurrence Risk CL or CL/P with 1 affected parent
A
3-5%
9
Q
Familial recurrence Risk CL or CL/P with 1 affected child
A
4%
10
Q
Familial recurrence Risk CL or CL/P with 2 affected children
A
9%
11
Q
Familial recurrence Risk CL or CL/P with 1 affected parent and 1 affected child
A
17%
12
Q
Familial recurrence Risk CL or CL/P with monozygotic twins
A
40-50%
13
Q
Familial recurrence Risk CL or CL/P with dizygotic twins
A
5%
14
Q
Familial recurrence Risk CL or CL/P with affected niece or nephew
A
1%
15
Q
Familial recurrence Risk CL or CL/P with affected cousin
A
0.5%
16
Q
Environmental risk factors for CL/P
A
Phenytoin (10x) Anticonvulsants increases risk of CL Smoking increases risk for CL/P Folic acid prevents CL/P Maternal corticosteroid use CL/P and CP High altitudes CL/P
17
Q
Timing for intervention
A
CL repair, ear tubes, tip rhino - 3 mo Palate, T tube - 9-18 mo Speech eval - 3-4 yr VPI workup and surgery - 4-6yr Alveolar bone grafting - 9-11yr Nasal recon - 12-18yr Orthognathic surgery - Completion of mandibular growth (>16y)
18
Q
Pre-lip repair intervention techniques for CL
A
Nasoalveolar molding
Lip adhesion
Gingivoperiosteoplasty
19
Q
Whistling deformity
A
central vermillion deformity, more common in B/l lip repair, presents as notching or inadequate vermillion with exposure of the central incisors in repose, tx with V-Y or Abbe
20
Q
Short lip
A
more frequent after Millard repair
Can be corrected with rerotation/advancement or V-Y advancement from nostril sill
21
Q
Long Lip
A
more frequent after LeMesurier or triangular flap repair, requires full thickness excision below nostril sill
22
Q
Widened lip scar
A
May be evidence of inadequate orbicularis continuity
23
Q
Lip landmark abnormalities
A
may be corrected with elliptical excision or Z plasty
24
Q
Timing for correction of secondary deformities
A
After cessation of facial growth
25
Primary Palate
- lip, nostril sill and hard palate ANTERIOR to incisive foramen
- formed by medial and lateral prominences (frontonasal) fusion with maxillary prominence
26
Secondary Palate
- hard palate POSTERIOR to the incisive foramen and the soft palate
- lateral palatal processes rotate from vertical to horizontal positions
27
CL/P thought to be a result of
failure of mesodermal penetration
28
CP only is thought to be the result of
failure of epithelial fusion
29
____lateral process becomes horizontal first, increasing risk of palate on the opposite side
Right; clefts are more common on the left
30
Which muscle of the velum is not innervated by the pharyngeal plexus and what is its innervation?
- Tensor veli palatini; opens eustachian tube
- Innervated by CN V
- Pharyngeal plexus is CN IX, CN X and contributions from CN XI)
31
Epidemiology of CL/P
```
1:750 live births
46% CL/P, 33% CPO, 21% CL
Bifid uvula 2% of the population
M/F CL/P: 2:1
M/F CPO: 1:2
```
32
Familial risk for recurrence for CPO with one affected child
2%
33
Familial risk for recurrence for CPO with family history and one affected child
7%
34
Familial risk for recurrence for CPO with one affected parent
6%
35
Familial risk for recurrence for CPO with affected parent and one affected child
15%
36
Most common associated anomaly with non-syndromic cleft palate
Robin Sequence:
Micrognathia/retrognathia, glossoptosis, and airway obstruction
CP is common, but not essential (usually wide and U shaped)
37
25% of syndromic CP
AD
Mutation for type 2 collagen
RS, ocular malformations, hearing loss and arthropathies
Stickler Syndrome
38
```
15% of syndromic CP
AD with variable expression
22q11 "Catch 22" deletion
Diagnosis with FISH
Cardiovascular abnormalities, abnormal facies, developmental delay, hypocalcemia, hypotonia
Higher risk of VPI
```
DiGeorge/Velocardiofacial/Shprintzen's Syndrome
39
```
19% of syndromic CL/P CPO
AD with 70-100% penetrance
CL/P or CP
Lower lip pits
IRF6 gene
Name the syndrome and risk of CL/P in child
```
Van der Woude syndrome
| 50%
40
Middle ear function in CP
Incidence of otitis media is 97%
Hearing loss 50%
Early tubes improve hearing/speech outcomes
41
Acute Post-op Complications for CP repair
```
Airway compromise
Bleeding (digital pressure and intranasal oxymetazoline, operative exploration)
Prolonged hospitalization
Dehydration
Death (0.5%)
```
42
Chronic post-op complications for CP repair
Palatal fistula (5-60%, most at soft-hard junction)
VPI
Malocclusion
Midface hypoplasia
43
If the greater palatine artery pedicle is accidentally divided when performing a unipedicled palatal flap repair, then the flap usually survives off _____
posteriolateral supply from the lesser palatine, ascending pharyngeal, and ascending palatine arteries
44
Syndrome associated with white forlock in hair and sensorineural hearing loss, cleft
Waardenburg syndrome
45
Anatomic feature that divides the primary and secondary palate?
Incisive foramen
46
Which muscles control the velum?
levator veli palatinia, tensor veli palatini, palatopharyngeus, palatoglossus, musculus uvulae
47
What are the anatomic features of a submucous cleft?
bifid uvula, zona pellucida (muscle diastasis), V notch posterior to palatal ridgeWh
48
What is the probability of a patient with a submucous cleft having VP dysfunction?
Probably 10% (underreported due to lack of symptoms)
49
What is the fundamental argument for the surgical repair of the palate before 18 months of age?
Preservation of speech
50
Which of the paired velar muscles is innervated by CN V, Mandibular division?
tensor veli palatini
51
What is the reason to stage the palate repair?
Delay of elevation of the hard palatal mucoperiosteum may preserve midface growth potential
52
What is the principle blood supply to the palatal mucoperiosteal flap?
Greater palatine artery and vein
53
What is the largest target of innervation of the greater and lesser palatine nerves?
sensory innervation of the palatal mucosa
54
What is the motor innervation of the velar musculature except the tensor veli palatini?
CN X (vagus) via the pharyngeal plexus
55
Which teeth usually originate in the premaxilla?
Canines and lateral incisiors (teeth affected by cleft palate)
56
Which tooth is most likely to be abnormal in a cleft lip?
Lateral incisior
57
What is the significance for lateral incisor agenesis in children with cleft palates?
It is a predictor for the need for maxillary advancement
58
Where is the lesser palatine foramen
posterior to the greater palatine foramen within the palatine bone
59
Which muscles of the palate control eustachian tube function?
tensor veli palatini and lesser extent, levator veli palatini
60
What is the blood supply to the soft palate?
Ascending palatine artery
61
What is the argument against gingivoperiosteoplasty in infancy?
Restricted maxillary growth
62
What are the punitive etiologic factors in clefting?
advanced paternal age, genetic, prenatal exposure to drugs, other environmental agents (multifactorial)
63
Which muscles form the anterior and posterior tonsillar pillars?
palatoglossus and palatopharyngeus (respectively)
64
What is the main function of the levator veli palatini?
Elevates the soft palate
65
Does the tensor veli palatini elevate the soft palate?
No, it primarily controls eustachian tube function and aids in swallowing
66
What is the probability that a parent with nonsyndromic cleft lip/palate will have a child with a cleft?
4% for first child, 17% for second child if first child has cleft
67
What is the probability that parents without clefts who already have a child with a nonsyndromic cleft will have another child with a cleft?
4%
68
What is the probability of a parent with Van der Woude syndrome will have a child with a cleft?
50% (autosomal dominant)
69
What is a Simonart band?
soft tissue at the nasal sill on the affected side, it is a feature that commonly defines and incomplete cleft lip but is abnormal tissue in varying thickness
70
At what point in gestation is the error responsible for cleft lip and palate most likely to occur?
5-6 weeks for lip, 7-8 weeks for palate
71
In embryology, what are the five facial prominences that eventually form the face?
Frontonasal (1), maxillary (2), mandible (2)
72
What is the embryological error in cleft lip/palate?
The frontonasal process derives the medial and lateral nasal processes, failure of fusion of one or both of the medial nasal processes and corresponding maxillary processes results in cleft lip
73
What is the purpose of presurgical orthopedics?
To narrow the cleft and align the alveolar segments, facilitating surgical repair by reducing tension
74
What is the rate of postoperative cleft palate fistula formation?
2-30%
75
What is the essential advantageous feature of the Furlow palatoplasty?
effectively lengthens the palate
76
What is the most pertinent outcome of success in palatoplasty?
speech intelligibly
77
Which cleft palate surgical technique is shown to give superior speech results?
Furlow double-opposing Z plasty > Straight line repair
78
What is the theoretical purpose of the intravelar veloplasty?
to detach from the hard palate and correctly reorient the levator muscle and optimize its function
79
What governs the timing of the alveolar bone grafting?
eruption of permanent canines on the affected side (usually 8-10 years)
80
In cleft patients with large negative overjets (>8-10mm) and normal mandibular projection, what is the most appropriate treatment for correction of their malocclusion?
LeFort I distraction osteogenesis
81
At what age can a LeFort I distraction be considered?
When the developing dentition has descended sufficiently to accommodate the osteotomy
82
How does NAM differ from standard presurgical orthopedics?
It addresses the nasal deformity with passive stretching of the nasal vestibule and alar dome
83
Does presurgical NAM improve outcomes in cleft lip/nose repair?
yes, there is improved nasal symmetry and reduced need for secondary procedures
84
What is the difference between presurgical orthopedics and presurgical orthodontics?
Orthopedics refers to movement of bone (not teeth) and is applicable in the newborn cleft patient when referring to the alignment of the alveolar segments, orthodontics is movement of teeth.
85
What is the purpose of cleft feeding aids?
To compensate for lack of suction in the cleft palate baby, by allowing the manual delivery of formula or breast milk
86
How can speech development be impeded by cleft lip/palate?
Velopharyngeal dysfunction, conductive hearing loss, dental derangement
87
What is the theoretical advantage of gingioperiosteoplasty in infancy?
It may preclude the necessity of alveolar bone grafting in mixed dentition
88
What is the expected rate of growth in cleft infants
0.5 to 1 ounce per day (normal growth)
89
Where is enamel hypoplasia of the permanent dentition most commonly seen in the cleft patient?
anterior dentition on the side of the cleft
90
What reasons could explain poor weight gain in a patient with isolated cleft palate?
Poor feeding technique, neuromotor dysfunction, occult airway obstruction
91
What disciplines are represented in a typical cleft team?
Plastic surgery, ENT, SLP, audiology, pediatric dentistry, orthodontics, prosthodontics, psychology, social work, genetics, nursing
92
What genes have been identified as involved in susceptibility to non syndromic cleft lip/palate?
TGF A, TGFb3, MSX1, IRF6, CYP1A1, GSTM1, NAT2, MTHFR, RARA, PVRL1
93
What is a Latham appliance?
A presurgival orthopedic device that realigns alveolar segments
94
When does middle ear effusion typically become apparent in a patient with a cleft palate?
8 weeks from birth
95
What is the essential feature of the von Langenbeck palatoplasty?
Incomplete elevation of the hard palate mucoperiosteal flaps
96
What are the abnormal anatomic features in a unilateral cleft lip?
Overal deficiency of lip tissue on the cleft side, shortened columella on the cleft side, upwardly rotated Cupid's bow, disruption and aberrant insertion of the orbicularis muscle, nasal asymmetry, anterior nasal spine deviated to the non cleft side, sagittal retrusion of alveolus and piriform on the cleft side
97
What anatomic features distinguish the bilateral cleft lip from unilateral?
Wide nasal tip with splaying of lower lateral cartilages, greater severity of columellar deficiency, greater projection of the premaxilla, effaced sulcus on the prolabium, absent muscle in the prolabium, deficient muscle in the lateral segments of the upper lip
98
What characterizes the nasal deformity in clefts of the lip/palate?
Diminutive lower lateral cartilages, caudally rotated, resulting in depressed alar dome with a widened base on the cleft side as well as severe septal deviationW
99
What are the essential features of the Millard technique of cleft lip repair?
Levels cupid's bow by rotating medial flap downward, advancing lateral segment into the defect, which narrows the alar base and restores symmetry to the nose, its result is to place the scar along the philtral column, preserving the philtral dimple
100
What percentage of cleft lips are incomplete?
10-30%
101
What is the M flap?
medial boundary of the cleft, may be elevated to add Mucosal lining to the sulcus or to reconstruct the nasal floor
102
What is the C flap?
skin on the medial side of the cleft, bounded by medial vermillion, extends into the cleft and creates the Columella
103
What is the L flap?
the lateral extent of the cleft, superior aspect of the abnormal vermillion on the cleft side, it may be elevated based on nasal mucosa to reconstruct the nasal floor and expand the nasal vestibule on the cleft side
104
What is the Mohler modification on the Millard technique?
Medial incision is carried into the columellar skin to add length to the medial lip and allows for downward rotation of the cupids bow while restricting the scar to the philtral column, columella, and nasolabial junction. In the classic Millard repair the scar can be positioned obliquely across the philtrum
105
What is the blood supply to the upper lip?
Superior labial artery from the facial artery
106
What is the primary nerve supply to the upper lip?
Infraorbital nerve )CN V2
107
When is the Abbe-Estlander flap employed in cleft surgery?
Often in bilateral cleft lip the disparity between the upper and lower lip transverse dimension is so great that transfer of the lower lip tissue is indicated to address the short, immobile upper lip that can result in bilateral repair
108
