Cleft Lip Flashcards by Kathryn isaac

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
  *

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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

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What forms the white roll

anterior projection of pars marginalis (o.oris) causing a prominence along jx of white and red lip

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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

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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

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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

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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

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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

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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

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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

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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

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What is the ideal timing of cleft lip repair

10wks of age

10 Hb

10 lbs

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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

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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
Syndromic CLP “VolkSWagon GT22”, and CPG implants 2113

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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

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How do you classify cleft defects

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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?

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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

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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

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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

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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

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presurgical orthodontia (NAM)
Lip adhesion
GingivoPeriosteoPlasty

Describe presurgical orthodontia: definition, use, timing, adv/disadv

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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

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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

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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

Cleft Lip Flashcards

(39 cards)