Blepharocheilodontic Syndrome Flashcards
(29 cards)
When is cleft lip usually repaired?
Cleft palate?
3-6 months
9-18 months
Term for cleft lip repair?
cheiloplasty
Term for cleft palate repair?
palatoplasty
Describe palatoplasty procedure.
1) incisions along medial margins of cleft soft palate ant -> post toward uvula
2) releasing incisions on soft palate posterior to the maxillary tuberosity, followed by blunt dissection in a plane between the superior constrictor and the velar musculature
3) nasal mucosa is dissected from palatal musculature and then approximated
4) hemiuvulae are approximated
5) incisions along medial margins of cleft hard palate; releasing incisions stop short of the incisive foramen anteriorly an the greater palatine foramen posteriorly to preserve perfusion
6) the above mucoperiosteal flaps are approximated (if they are not mobile enough, the pterygoid hamuli may be fractured to provide movement)
Common long-term complications of palatoplasty
1) fistula between oral and nasal cavities (palatal, nasoalveolar, or oronasal) – Tx is add’l Sx to separate the cavities – this is the most common complication
2) bifid uvula – mucosal and muscular layers must be carefully approximated separately
3) inadequate soft palate (velar) movement – palate should move up and back to close the velopharyngeal port; inadequate movement can be due to scarring, poor tissue management, or compromised healing
4) palatal necrosis – results from injury to the greater palatine artery during palatal flap elevation (may be immediate or delayed)
What would be the cause of palatal necrosis resulting from palatoplasty?
injury to the greater palatine artery
Describe embryologic development of the lip.
1) 4w - first pharyngeal arch forms facial prominences
2) 5w - medial and lateral nasal prominences are formed
3) 6-7w - fusion of bilateral maxillary prominences (lateral portions) with medial nasal prominences (medial portion) to form the upper lip
- further medial growth of the maxillary prominences leads to add’l fusion of deep medial nasal prominences to create the primary palate (four incisors and palate anterior to the incisive foramen)
- medial nasal prominences also form the philtrum, columella, and nasal tip
Disruption of any of these steps leads to cleft lip deformity.
Complete cleft vs. partial cleft
complete cleft - disruption of entire vertical thickness of lip; usually assoc. w/ alveolar defect, since alveolus is part of the primary palate
incomplete cleft - involves only a portion of the upper lip; extent varies from muscular discontinuity with intact skin to wide cleft with a thin segment of skin that crosses the cleft (Simonart band)
What muscle is disrupted by a cleft lip?
orbicularis oris
Why are the anterior maxillary teeth usually protruded in bilateral cleft lip patients?
the medial prolabial segment is usually devoid of muscle tissue, which causes anterior protrusion of the prolabial segment and primary palate relative to the nasal septum
What causes a nasal deformity associated with cleft lip?
traction from the abnormally inserted orbicularis oris muscle and the absent anterior nasal floor
Describe cleft lip repair.
Correction of the cleft lip requires detachment of the orbicularis oris muscle’s abnormal attachments and creating a horizontal bridge across the cleft to form a complete sphincter around the mouth
rotation-advancement technique consists of a rotational flap on the cleft’s medial segment and an advancement flap originating from the cleft’s lateral segment. (better cosmetic outcome but more difficult)
triangular flap technique consists of a triangular flap originating from the cleft side inserted on an incision performed on the noncleft side. (easier but poorer cosmetic outcome)
What are common complications of cleft lip repair?
1) wound dehiscence
2) scar contracture
3) scar hypertrophy
4) infection
5) vermillion notching
6) orbicularis oris discontinuity
7) short or excessive length of the lip
8) short or deviated columella
9) horizontal orientation of the nares
10) abnormalities of nostril size
11) disturbance of alar base position
bridge of tissue separating the nostrils at the bottom of the nose
columella
What causes BCD, and what are the main proteins involved?
mutations in the genes that provide instructions for making epithelial cadherin and p120-catenin
e-cadherin
protein found in epithelial cell membranes involved in cell adhesion
p120-catenin
protein that helps keep e-cadherin in its place in the cell membrane, preventing it from being taken into the cell and prematurely broken down
What is the effect of palatoplasty on skeletal growth in cleft lip and palate patients?
anteroposterior and vertical palatal growth are less in cleft patients
palatoplasty can further limit anteroposterior growth of the maxilla (no effect on vertical development compared to un-repaired cleft)
What is the cause of fistula formation following palatoplasty?
- closure under tension (usually from tissue dificiency)
- flap trauma
- infection
- hematoma
- tissue ischemia
classification of cleft palate
Veau 1 - midline velum cleft, intact hard palate
Veau 2 - midline velum & secondary hard palate cleft, intact primary palate (anterior to incisive foramen)
Veau 3 - unilateral cleft of velum, secondary hard palate, primary hard palate & alveolus; vomer remains attached to palatal shelf on the non-cleft side (aka the greater segment)
Veau 4 - midline cleft of velum & secondary hard palate extending bilaterally through primary hard palate & alveolus; vomer remains in the imdline and is attached to the premaxilla
What is another palate classification besides Veau?
Randall - describes palatal length
- 1 normal -> 4 very short
- correlation between Randall classification & speech quality
- contested as being too subjective and having poor inter-rater reliability
What is LAHSHAL notation?
shorthand phenotypic description system for clefts
L - right lip
A - right alveolus
H - right hard palate
S - midline soft palate
H - left hard palate
A - left alveolus
L - left lip
capital letter = complete cleft
lowercase letter = incomplete cleft
* = minimal clefting
* = normal feature
My patient is most likely ***SHAL
Why does the patient have a scar on her lip?
The patient had a unilateral cleft lip and palate
Clefting is caused by disruption of embryologic formation of the lip and palate. Formation of the lip occurs by fusion of the lateral maxillary prominences with the medial nasal prominence, and in the case of a unilateral cleft, this fusion is disrupted on one side.
What is a NAM, and what are the goals of treatment?
Removable appliance consisting of an intraoral molding plate connected to nasal stents
Pre-surgical treatment for infants with cleft lip and palate to guide maxillary growth and improve the position of the lip and nostrils, with the goal of reducing the need for additional surgical procedures
Tx objectives:
- approximation of lip segments
- approximation of alveolar segments in the transverse and sagittal dimenisons
- increasing columella length
- achieving midline for nasal tip
- achieving equal alar width and height
Tx begins in the first few weeks of life and continues until primary lip repair at around 3-6 months. Appliance is worn 24h/day with adjustments made regularly.
