This lecture is really confusing. I emailed him to ask for his lecture.
Results from perturbations in signaling centers producing alterations in:
Of facial primordial growth
Cleft lip and palate is characterized by a vertical defect in the oro-nasal complex, resulting in direct communication between the nose and the mouth. It is one of the most common birth defects in US
Af am: 1:2000
Cac 1: 1000
Cleft lip .29:1000
Cleft palate .39:1000
More males have cleft lip than females
More females have cleft palate
Left side: right: bilateral 6:3:1
genetic contribution to facial morphogenesis plays a role in syndromes asso with CLP-poorly understood. Transforming growth factor-alpha. Retinoic acid receptor – alpha
envi modifying role may effect genetic activation/repression in non-syndromic CLP. Tobacco, anti-epileptic drugs, etc.
Clefting of lip can interfere with palate closure. Isolated clefting of palate is etiologically independent entity from CLP
multi factorial threshold model: major genes, minor genes, environment, developmental
single major gene with reduced penetrance (less than 40% CLP are directly genetic in origin
· both parents unaffected : 0.1% chance for first child
· first child affected: 4% chance for 2nd child
· two affected kids: 9% first child CLP
· one parent: 4% first child
· 1st child: 17% second child
· Both parents affected: 60% chance for all kids
Meckel 1808-failure of the facial primordial to fuse between the fifth and eighth week: FUSION.
Primordial come together by forces of growth. As move together, they touch. And epi degenerates and mesothelium flow thru area and have normal tissues and then u have fusion complete. If epithelium does not degenerate, you get cleft.
Stark: pieces actually do come together, but not strong enough to stay together (FISSION)
Four mx movements
· the membranous sutures of the face must be responsive:
· the Vomerine-palatal suture is vital for adequate forces to be transmitted to the developing mx. Scarring of the suture will inhibit all aspects of growth.
· (if upset suture of vomer, mx won’t growth properly)
Stage I: incomplete CP
Stage II: more
The "Y" (Kernahan)
Microform: full thickenus off mucosa, muscle never got across. Must open area up and close cleft lip together
Minimal: doesn’t go half way up lip
Incomplete: goes half way up or more
orbicularis oris: eighth muscle components, arising from modioli at either end of mouth
superior and inferior horizontal band
oblique bands: allow to take lips up and out. (cleft pts can’t bring out but can bring up.) horizontal fibers can bring lips together
normal anat is askew with deviation of philtrum, cupsids bow, tubercle
lateral lip element exhibits vertical discrepancy
mesodermal deficiency: muscle never grew across so can’t fn properly
the muscles remain underdeveloped
submucous cleft: cleft of hard palate under mucosa.
soft and hard palate
· tensor palatine
· palatal-glossus, levator, uvular, etc:
· all are imp in speech.
Cleft soft palate demonstrates abnormal muscle insertion a the post edge of the hard palate, leading to dysfunction of muscles. CAN"T SPEAK.
The valvular system is a continuum of movement that allows sound to be modified thru a change in pressure. Get Otitis media. Can’t equalize pressure. So lose hearing
Goals of palatal surgery
Release abnormal muscle insertion
Establishment of muscle continuity
Correct orientation of the velum to serve as a dynamic sling
Establish functional velo-pharyngeal valve mechanism to allow closure between oral pharynx and nasal pharynx.
Vertical deficiency, retrodisplaced
Mx alveolus is frequently involved in the cleft lip and palate deformity. It routinely presents as a subtle fistula in the labial vestibule following the repair of the lip.
Food and fluid come out of nose
Inability to suck or blow
Poor ability to keep teeth clean
Decayed or deformed front teeth
Missing or extra teeth in the cleft site
Lack of boney support of adjacent teeth
Mobility and deformity of primary palate
Lack of support for nose and lip
stabilitze mx arch-consolidat to one jaw
establish functional nasal airway
close oro-nsal fistula
get osseous volume to support teeth
eliminate depressed alar base
allow for dental rehab
· primary gingivoperiosteoplasty- should not be done
· primary alveolar bone grafting- do at time of lip repair.
· secondary gingivoperiosteoplaty-should not be done
· secondary alveolar bone grafting- done at a later time.
Depends of type of cleft
To understand the facial patterns of CP: questions
does the unoperated cleft ind have same faial growth potential as non cleft ind
do all unoperated cleft types have same growth potential?
· Skeletal: mx protrusion. No md. Diff.
· Dental- cleft segment has tendency to rotate medially with cuspid crowss bite occurrence.
Effect of lip repair
skeL: ant mx is molded with reduction of protrusion. No md. Diff. overall appear is like non-cleft ind.
Dental;mx and md incisors became more up right.. get post crossbite.
Skel: mx and md retrusion. Md has steep plane angle
Dent: no effect
Skel: no effect on ant postion, however decreased vert ht
Decrease md palne due to rotation. Get more of class III relationship
Dental: see much greater post crossbite. (transverse growth of mx is restricted)
Skel: mx is normal but md is rotated backwards
Dental: collapse of segments, get post crossbite
Mx retruded compared to unodperated cleft lips. Md unaffected. ANB is smaller than unoperated clefts but still pos
Dental: no incr in ant crossbite. But yes post crossbite
Lip and palate repair
Skel: class III
dentL ant crossbite and post cross bite
mx and md with repaired clefts are related to the presence of the cleft itself. Means: these pts with class III: thought it was result of surgical interference. They have class III because that’s what they have, not what the surgeons are doing.
Plastic reconstructive surgieon
Oral and mx facial surgeon
Speech and language path
Immed after birth: feeding,
Alv bone grafting repair: usually done at age 8 and 9 since canines haven’t erupted yet. so now, do cleft repair at 5-6