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
How do you classify cleft defects
Kernahan striped Y classification
- shade areas of cleft
- 1st box lip
- 2nd box alveolus
- 3rd box 1’ palate
- has one option of each side for R and L
- 4th and 5th boxes - secondary palate
- last box - soft palate
What is a microform/forme fruste cleft lip?
- has some or all of below features
- notch in vermilion
- fibrous band form vermilion to nostril floor
- notcho r band in ala
What defines a partial/incomplete cleft lip
up to 2/3 of lip is disrupted
- nasal defect is worse than anticipated for lip defect
- bone hypoplasia also worse
What are clinical features of a complete unilateral cleft lip
Maxilla
- Cleft: hypoplasia, posteriorly retropositioned
- Non-Cleft: premaxillaa outwardly rotated +projects
Nose
-
Septum
- Cleft: midseptum bowed into cleft side. short columella
- Non-cleft: caudal septum dislcoated off vomer and sitting in non-cleft side
-
LLC
- Cleft: attenuated, buckled, absent scroll area with no support from ULC. Tip points to cleft side
-
Ala
- Cleft: wide alar base, slumping dome separated from other dome, deficient vestibular lining
Lip
- Lateral lip
- diminshing cutaneous roll & mucosal vermilion height, reduced philtral height
- Medial lip
- short philtral height, 2/3rd of cupids preserved
- Orbicularis
- complete cleft - deep orbicularis disrupted andsuperficial inserts into periosteum of ala/columella
- incomplete cleft - orbicularis continuity if >1/3 rd of lip intact
What is simonarts band
band of fibrous tissue (consisting of muscle, artery, nerve) bridging the cleft between the lateral lip and medial alveolus or lip to lip
Significance - potentially reduces the deformity/narrows cleft
What are procedures that can be done prior to cleft lip repair
- presurgical orthodontia (NAM)
- Lip adhesion
- GingivoPeriosteoPlasty
Describe presurgical orthodontia: definition, use, timing, adv/disadv
- Def: use of applicance to guide growth of maxilalry alveolus to decrease deformity before lip surgery
- indication: wide or misaligned clefts
- Timing 2-5wks until CL repair
- done in conjunciton with GPP to reduce need for BG
- Types:
- Active- Latham device - pinned into palate
- Passive - NAM - formed to roof of mouth and adjusted qwk to guide growth
- Adv - reduces discrepancy b/w alveolus, better correction of lip and nasal deformity, possibiliity of 1 repair of alveolar cleft (w GPP and no BG)
- DisADv - time/cost, delay lip surgery, possibel long term growth disruption
Describe lip adhesion
Def: sturing medial and laeral lip elements to gether prior to lip reapir
- Indication - wide cleft
- Timing 2-5wks
- Adv: may facilitate GPP, narrow cleft, decrease tension across lip for definitive repair
- DsADv - sgnificant scarring, added OR, may worsen or not improve lip repair
Describe GPP
Def: mucoperiosteal flaps are raised along alveolus ot close cleft and promote bone healing
- Indicated: aligned alveolar segemtns w cleft
- Timing - after alignment w NAM/Latham
- Adv - may eliminate need for BG, allow for tooth eruption
- Dsiadv - may require BG anyway, may impede facial skeletal sgrowth
What are goals of Cleft Lip repair speficially
- Symmetry
- White lip - scars hidden, length restored
- Red lip - height restored, central tubercle, red line restored
- Muscle - continuity and function
- reduce cleft nasal deformity
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
Describe the repair of a microform cleft lip
- Mild - repair with excision of abnormal band +/- zplasty
- Moderate/severe - repair w full lip repair as stright line or other technique
What are techniqeus of unilateral cleft lip repair
Categorized by Z-plasty type
- Straight lip
- Rose Thompson
- Lower lip
- Randall-Tennison (inferior traingular flap repair)
- Le Measurier (rectangular flap)
- Upper lip
- Millard
- Upper and Lower lip
- Skoog
- Anatomic Subunit
- Fisher
What are surgical goals of primary cleft nose repair at time of cleft lip repair?
- reposition LLC medially
- reconstuct anterior nasal floor
Describe two techniques for 1’ cleft nose repair
- McComb
- relaease LLC form pyrifomr and skin surrounding LLC
- suspension suture to contralat nasion
- stent
- Fisher
- release LLC from pyriform only
- resocntitue scroll area with horizontal mattress between LLC and ULC
- reconstitue alar cheek jx and vestibular skin against LLC with dermal suture
What are the features of BCL
Maxilla
- premaxilla protruding
- lateral alveolar segments retropositioned and inferior
- maxilla at pyriform hypoplastic
Nose
- septum midline
- alae positioned inferior and lateral
- columella short
- tip flat and wide
Prolabium
- NO muscle, philtrum dimle/column, cupids bow, white roll
- deficient vermilion and gingival labial sulcus
Lateral Lip elements
- O.oris bulges at lateral ends
- aberrantly inserting at alar bases
- vertically short
What are procedures done prior to cleft lip repair
For BCL/P, compared to unilat CL/P
3 elements to reposition
- presurgical orthodontia (latham/NAM)
- lip adhesion
- GPP
What are disadvantages of
- Manchester
- Veau
- Tennison
- Skoog
- Manchester = whistle notch - deficient red lip under philtrum and chapped mucosa with red lip preserved at prolabium
- Veau = cupids bow scar contracture
- Tennison= scar across central tubercle
- Skoog= compromised phitral blood supply (posterior septal artery)
List secondary deformities of cleft lip repair
- Vermilion deficiency (whistle notch)
- White roll misalignment (stair step)
- Short lip
- Long lip
- Tight Lip
- Philtral column absence
- Wide philtrum
- Red lip deficiency
- Buccal sulcus deficiency
- Absence of unilat Cupids bow
What are potential corrections available for vermillion whistle notch deformity
- V-Y advancement of vermilion mucosa
- Zplasty
- Cross lip flap
- Submucosal dermla/fat graft
- revision of entire lip
What are potential corrections for short lip deformity
- Diamond excision to straight line
- Zplasty just above white roll
- Redo lip if
- o.oculi dysfx
- >3mm discrepancy
Short lip caused by scar contracture, o/oris poorly repositioned or 2’ to MIllard underrotation
What are potential corrections for long upper lip?
Problem seen often with Tennison Randall straight lip repair
- correct with excision of lateral lip elements at alar base
- redo lip if major discrepancy
What are potential corrections for tight upper lip?
- lack of tissue vertically and horizontally
- Correction with
- abbe flap
- cross lip flap
What are causes of wide philtrum followign cleft lip repair
- prolabium designed too wide
- no repair of o.oculi across philtrum or ends attached to prolabium
