Cleft Lip Flashcards
Describe the embryological development of the face as it pertains to cleft lip
Wk 3 - neuroectoderm migrates antriorly from the fore/mid/hind brain
Wk4 - 5facial processes form
- FNP
- Maxillary P
- Mandibular P
Wk 5 - on FNP, nasal placodes form and LNP and MNP form
Wk 6/7
- migration of paired MNP to fuse together
- = philtrum, columella, nasal tip, premaxilla, primary palate
- MNP fusion with Maxillary P
- = upper lip laterally, secondary palate, alveolus
- **failure of MxP and MNP ot fuse = cleft lip
- LNP fusion with Max
- Nasolacrimal duct/system
*
- Nasolacrimal duct/system
Describe two theories of why facial clefts occur
- Failure of fusion
- failure of MxP and LNP to fuse with MNP
- Failure of mesodermal penetration
- fialur eof mesoderm to migrate between two layer of epithelium for reinforcement, leading to epithelium breakdown
What forms the white roll
- anterior projection of pars marginalis (o.oris) causing a prominence along jx of white and red lip
Where do the o.oris muscles aberrant insert in a cleft lip
- On medial lip element - insert at periosteum, below columella
- On lateral lip element - insert at periosteum, below alar base
What are risk factors for cleft lip
- P - Paternal age <30, mom+dad>30
- M - Medications - isotretinoin, phenytoin, diazepam, steroids,
- S - Smoking
- F - Family Hx
- I - Infections - rubella, toxoplasmosis
- N - nutritional deficiency - folate, vit b6
What syndormes are associated with CLP
- VolkSWagon GT 22, CPG 2113
MENDELIAN:
- Van der Woude
- AD, mutation 1q
- features: lower lip pits/sinuses, CLP, hypodontia, bifid uvula
- Stickler
- CP, Ocular abN
- joitn arthropathy
- Waardenberg
- AD, defect in NCC migration and melanin synthetis
- features: no melanocytes in hair, skin, SNHL, CLP
- Gorlins
- multiple BCC, palmar pits, calcification falx
- Treacher collins
- tssier cleft 6,7,8, defined by bilateral symmetric absenceof zygoma
Non-mendelian inheritance
- C - CHARGE
- Coloboma/CNS, Heart defect, Atresia chonal, retardation, Genital abn, Ear/HL
- P - PRS
- G - Goldenhar
- TC + vertebral + epibulbar dermoids
Chromosomal
- Downs T21
- T13
teratogenic
- FAS
- Fetal valproate/phenytoin syndrome
What is noordhoffs point
- point on th elateal lip element where the cutaneous white roll and the vermilion muscosal junction line begin to converge medailly
- Medial to noordhoff point, the height of the vermilion and cutaneous roll diminish
Describe anatomical features of the medial and lateral lip elements in a cleft lip
Medial lip
- decreased height of lip
- decreased height of vermilion
- cupids bow preserved w cutaneous role
- aberrant insertion of o.oris
Lateral lip
- Nordhoff point
- if incomplete, length of lip from subnasale to nordhoff is longer
- if complete, length of lip from subnasale to nordhoff is shorter
- decreased trasnverse length of lip
- aberrant insertion of o.oris
What are sugical goals of CL/P repair
- Lip continuity, competance
- Palate - division of oral and nasal cavities
- Functional speech
- Maximize aesthetics
- maximize potential facial skeletal growth
What is the millard repair and the adv/disadv
- Millard - Rotation Advancement
- medial lip is rotated, lateral lip advanced
- upper lip Zplasty
- Advantages
- C-flap - flexibility tailored to cleft
- Scar - hidden in nostril/alae/columella, along philtral colums
- Preservation - philtral dimple, cupids bow, tissue
- Disadvantages
- Lip - SHORT due to underrotation
- Scar - wide if wide cleft
- Nostril - constricted on cleft side
What is the Randall-Tennison repair, adv/disadv
- Lower lip Zplasty
Advantages
- Lip - length achieved
- Cleft - acceptable for wide/narrow clefts
- Nasal deformity - corrected
Disadvantages
- Scar: crosses philtrum
- Presevation: phitral dimple obscured
- lip:lengthning may occur in excess
What is the ideal timing of cleft lip repair
10wks of age
10 Hb
10 lbs
What is the incidence of CL/P
CL/P incidence
- Overall, 1:1000
- Asian 1:500
- Africain 1:2000
- Caucasian 1:750
CL alone- incidence constant across races 1:2000
What are the demographics of CL/P
for CL/P
- M:F 2:1
- L:R:bilat 6:3:1
- CL/P> CP>CL; 50%:30%:;20%
- CL/P twice as common as CL alone
-
3% of CL/P are syndromic
- Mendelian AD
- Van Der Woude
- Stickler
- Waardenberg
- Gorlins
- Treacher collins
- 22qdeletio
- Non mendelian
- PRS
- CHARGE
- Goldenhar
- Chromosomal
- Downs 21, trisomy 13
- teratogenic
- FAS
- Fetal pheyntoin/valproate syndrome
- Mendelian AD
- Syndromic CLP “VolkSWagon GT22”, and CPG implants 2113
What is probablility of having child with CP or CLP
note: CL and CLP are on spectrum whereas CP is a separate entity w/r/t demographics and probabilities
Probability of new child having CP
- general 0.01
- if one child affected = 2
- if one parent affected = 2-4
- If one child, +FMx = 7
- if two children = 1
- if one child and 1 parent affected = 15
Probability of new child having CLP
- general 0.04
- if one child affected = 4
- if one parent affected = 2-4
- If one child, +FMx = 7
- if two children = 9
- if one child and 1 parent affected = 15
Outline the timing of procedures for the cleft patient
- In Utero -3wks - first consult
- 2-5wks - Presurgical Orthodontia
- 3m-6m - Lip repair
- 9m-14m - Palate repair + myringotomy tubes
- 6wks post palate - Speech Asx + Tx
- 5-7yo - VPI surgery
- 7-14yo - Additional Orthodontia pre/post ABG
- 9-12yo - ABG
- 5-6yo 2’ nasal repair
- 16F, 18M - Orthognathic surgery
- 18+ - genioplasty, rhinoplasty
