Introduction Facial Clefting is the Second Most

8/14/2019 Introduction Facial Clefting is the Second Most

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Introduction

Facial clefting is the second most common congenital deformity (afterclubfoot).

Affects 1in 750 births

Problems are cosmetic, dental, speech, swallowing, hearing, facial growth,

emotionalOtolaryngologist holds key role on CP team

Epidemiology

z Isolated cleft palate genetically distinct from isolated cleft lip or CLAP

y same among all ethnic groups (1:2000, M:F 1:2)

z Isolated CL or CLAP

y different among ethnic groups

x American Indians: 3.6:1000 (m:f 2:1)

x Asians 3:1000 (m:f 2:1)

x African American 0.3:1000 (m:f 2:1)

l Cleft Lip +/- Palate- 2 Male: 1 Female

l Cleft Palate - 2 Female: 1 Male

l Cleft Lip +/- Palate- Native Americans > Oriental and Caucasians > Blacks

l Cleft Palate- Same among ethnic groups

l Environmental: Ethanol, Rubella virus, thalidomide, aminopterin

l Increased Clefts with maternal diabetes mellitus and amniotic bandsyndrome

l Increased Clefts with increased paternal age

l Cleft Lip + Palate- 50%

l Cleft Palate- 30%

l Cleft Lip- 20%

l Cleft Lip + Alveolus- 5%

Anatomy


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l Hard Palate

Bones: Maxilla( Palatine Processes) + Palatine Bones(HorizontalLamina)

Blood Supply: Greater Palatine Artery

Nerve Supply: Anterior Palatine Nerve

l Soft Palate

Fibromuscular shelf attached like a shelf to posterior portion of hardpalate

Tenses, elevates, contacts Passavants Ridge

Muscles: Tensor Veli Palatini(CNV), Levator Veli Palatini(PrimaryElevator), Musculus Uvulae, Palatoglossus, Palatopharyngeus(CN IXand X)

l Primary Palate- Triangular area of hard palate anterior to incisive forameto point just lateral to lateral incisor teeth

Includes that portion of alveolar ridge and four incisor teeth.

l Secondary Palate- Remaining hard palate and all of soft palate

l Primary Palate

Forms during 4th to 7th week of Gestation

Two maxillary swellings merge

Two medial nasal swelling fuse

Intermaxillary Segment Forms: LabialComponent(Philtrum) Maxilla Component(Alveolus + 4Incisors) Palatal Component(Triangular Primary Palate)

l Secondary Palate

Forms in 6th to 9th weeks of gestation

Palatal shelves change from vertical to horizontal position and fuse

Tongue must migrate antero-inferiorly

Causes


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During the early stages of pregnancy, the upper lip and palate develop

from tissues lying on either side of the tongue. Normally, as the face andskull are formed, these tissues grow towards each other and join up in thmiddle.

When the tissues that form the upper lip fail to join up in the middle of thface, a gap occurs in the lip. Usually, a single gap occurs below one orother nostril (unilateral cleft lip). Sometimes there are two gaps in theupper lip, each below a nostril (bilateral cleft lip).

When the palate fails to join up, a gap is left in the roof of the mouth up

into the nose. We don't know why the 'joining up' process fails in this waSome evidence suggests there may be a genetic factor, as cleft lip andpalate can run in families.It is thought, however, that certain types ofdrugs may increase the risk.

Classification

z Veau Classification - 1931

y Veau Class I: isolated soft palate cleft

y Veau Class II: isolated hard and soft palate

y Veau Class III: unilateral CLAP

y Veau Class IV: bilateral CLAP

z Iowa Classification - a variation of Veau Classification

z Complete Clefts

y absence of any connection with extension into nose

y vomer exposed

z Incomplete Clefts

y midline attachment (may be only mucosal)

z ex: submucous cleft (midline diasthasis, hard palatal notch,bifid uvula)


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Unilateral Cleft Lip

l Nasal floor communicates with oral cavity

l Maxilla on cleft side is hypoplastic

l Columella is displaced to normal side

l Nasal ala on cleft side is laterally, posteriorly, and inferiorly displaced

l Lower lat on cleft side -lower, more obtuse

l Lip muscles insert into ala and columella

Palatal Clefts

l Soft palate muscles insert on posterior margin of remaining hard palaterather than midline raphe.

l Associated Dental Abnormalities

Supernumery Teeth- 20%

Dystrophic Teeth- 30%

Missing Teeth- 50%

Malocclusion- 100%

bilateral

Genetics


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l Non-syndromic inheritance is multifactorial

Cleft Lip, With or Without Cleft Palate:

One Parent-2%

One Sibling- 4% Two Siblings- 9%

One Parent + One Sibling- 15%

Cleft Palate:

One Parent- 7%

One Sibling- 2% Two Siblings- 1%

One Parent + One Sibling- 17%

l Increased clefts with chromosome aberations

l Clefts a part of a Syndrome 15-60% of timel More than 200 syndromes include clefts

l Cleft Palate- Aperts, Sticklers, Treacher

l Cleft Lip +/- Palate- Van der Woudes, Waardenbergs

Problems it Creates

Difficulty feeding

The most immediate problem caused by a cleft lip or palate is likely to bedifficulty with feeding. Many babies with a cleft lip can breastfeed. However,some have difficulty in forming a vacuum in order to suck properly. Babies witthese problems may need a special teat and bottle that allow milk to bedelivered to the back of the throat where it can be swallowed. Sometimes,special dental plates can be used to seal the roof of the mouth to help the babsuckle milk better.

Babies who find it difficult to feed may gain weight slowly at first, but haveusually caught up by the time they are six months old.

Speech and Hearing

Cleft palate can cause problems with speech. The size of the cleft is not anindicator of how serious such problems are likely to be - even a small cleft canaffect speech quite seriously. Most children go on to speak normally after thepalate is repaired, although some may develop problems such as nasal speec


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Hearing may be affected because the muscles of the palate affect the ear,making the child more likely to develop "glue ear". This is a condition wherethick sticky fluid accumulates behind the eardrum as a result of an infection othe middle ear. Doctors call it otitis media with effusion. It can causetemporary hearing loss but can be treated with antibiotics or, if it is an ongoinproblem, with a minor operation to insert a tiny plastic tube (a grommet) intothe eardrum through which the fluid can drain.

Occasionally, cleft palate may also affect the growth of the jaw and thedevelopment of the teeth

Pictures: Cleft Lip

Pictures: Cleft Palate

Management

l Team Approach

l Otolaryngologist has a pivotal role

l Initial Head and Neck Examination

l Speech Disordersl Ear Disease

l Airway Problems

l Surgical Repair

A TEAM APPROACH IS REQUIRED

l pediatrician


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l surgeon

l OMFS

l dentist

l ENT

l psychiatrist

l speech

l nurse coordinator

Head and Neck Exam

l Head- facial symmetry

lOtologic- auricle and canal development and location, pneumaticotoscopy, forks

l Rhinoscopy- identifies clefting, septal anomalies, masses, choanal atresia

l Oral Exam- cleft, dental, tongue

l Upper airway- phonation, cough, swallow

Speech Disorders

l Errors in Articulation: Fricatives, Affricates

l Velopharyngeal Competence- Most important determinant of speechquality in cleft palate patients-75% achieve competence after initialpalate surgery

l Incompetence- nasal emission or snort

l Evaluation- Direct exam , Fiberoptic Exam

Ear Disease

l Cleft Lip- Incidence similar to normal pop.

l Cleft Palate- Almost all with ETD, CHL

l ETD- Due to abnormal insertion of levator veli palatini and tensor velipalatini into posterior hard palate

l ETD- Returns to normal by mid-adolescent

l Cleft Palate- Increased Cholesteatoma(7%)


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l Otologic Goals For Cleft Palate Patients

l Adequate hearing

l Ossicular chain continuity

l Adequate middle ear space

l Prevent TM deterioration

l Indications for Myringotomy Tubes

l CHL, Persistent/Recurrent effusion, Retraction

l Cleft palate: Multiple BMTs from 3mo. - 12 yrs

Airway Problems

l More common in Cleft Palate patients with concomitant structural orfunctional anomalies.

l e.g. Pierre-Robin Sequence

Micrognathia, Cleft Palate, Glossoptosis

May develop airway distress from tongue becoming lodged in palatadefect

Surgical Repair- Cleft Lip

l Lip Adhesions-

2 weeks of age

Converts complete cleft into incomplete cleft

Serves as temporizing measure for those with feeding problems

May interfere with definitive lip repair

Less often needed in recent years due to wider variety of specialtyfeeding nipples

l Cleft lip repaired at 10 weeks

l Rotation-advancement method- Most common in the U.S.

l Nine Landmarks

l Rotation Flap cuts made first

l Advancement cuts made next

l Cleft side nasal ala cuts made last


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Surgical Repair- Cleft Palate

l Several Techniques- Trend is towards less scarring and less tension onpalate

l Scarring of palate may cause impaired mid-facial growth(alveolar archcollapse, midface retrusion, malocclusion)

l Facial growth may be less affected if surgery is delayed until 18-24months, but feeding, speech, socialization may suffer.

l Bardach Method- Two Flap technique

l Medial incisions made, which separate oral and nasal mucosa

l Lateral incisions made at junction of palate and alveolar ridge

l Elevate flaps, preserve greater palatine artery.

l Detach velar muscles from posterior palate

l Close in 3 layers

Non-Surgical Treatment

l Dental Obturator

For high-risk patients or those that refuse surgery.

Advantage- High rate of closure

Disadvantage- Need to wear a prosthesis, and need to modifyprosthesis as child grows

Multidisciplinary Approachz These are not merely surgical problems

y Requires team approach throughout life

x neonatal period

x toddlerx grade school

x adolescence

x young adulthood

The Neonatal Period

z Pediatrician:


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y directs care

y establishes feeding

x complete clefts preclude feeding

breast feeding not possible

a soft, large bottle with large hole is required

a palatal prosthesis may be required

z Presurgical Orthodontics (Baby Plates)

Molds palate into more anatomically correct position

decreases tension

may improve facial growth

Grayson, presurgical nasal alveolar molding (PSNAM)z Surgical Repair

y Cleft Lip

x In US - the rule of tens - 10 wks, 10 lbs, Hgb 10

x Lip adhesion vs baby plates

y Cleft Palate

x Varies from 6-18 months - most around 10 mo

x Early repair may lead to midface retrusion

x Early repair improves speech

The Toddler Years

z Priority: Speech

y Cleft errors of speech in 30%

x primary defects - due to VPI (hypernasality)

consonants are most difficult sounds (plosives)

x secondary defects - due to attempted correction

glottic stops, nasal grimace

y Velopharyngeal insufficiency

x diagnosed by fiberoptic laryngoscopy or BaSw


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x surgical repair after failed speech therapy - usually around age4

z Growth hormone deficiency

y 40 times more common in CLAP

y suspects when below 5% on growth chart

The Grade School Years

z Three primary issues

y Orthodontics

x poor occlusion

x congenitally absent teeth

y alveolar bone grafting

x fills alveolar defect - around age 12

y psychological growth

x considered standard of care

The Teenage Years

y Midface retrusion

x etiology - ?early palatal repair

x surgical correction around age 18

y Psychological development

x counseling standard of care

y Rhinoplasty

x usually last procedure performed, around age 20

Surgical Techniquesz Cleft Lip Repair

y unilateral


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x rotation-advancement flap developed by Millard

x complications

dehiscence

infection

thin white roll

excess tension

z Cleft Lip Repair

y bilateral

x bilateral rotation advancement with attachment to premaxillamucosa

x complications

dehiscence

thin white roll

Controversies:Timing of Repair

z Early repair

x Advantage: improved speech

Rohrich, et. al; retrospective study. The earlier the repaithe better speech.


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x Disadvantage: worsening midface retrusion

Rohrich, et. al; people with unrepaired palates have lessmidface retrusion

Conclusions

l Cleft Lip and Palate are common congenital deformities that often affect

speech, hearing, and cosmesis; and may at times lead to airwaycompromise.

l The otolaryngologist is a key member of the cleft palate team, and is in aunique position to identify and manage many of these problems .

Documents

Introduction Facial Clefting is the Second Most