Dr V.RAMKUMARCONSULTANT DENTAL&FACIOMAXILLARY SURGEONREG:NO -4118 TAMILNADU- INDAI(ASIA)

Pierre Robin Sequence;– Micrognathia– U or V-shaped palatal cleft– Glossoptosis / airway obstruction

Causes ofIsolated Cleft Lip +/- palateMultifactorialenvironmental + genetic factors -

positive family history in 26% cases

Known Teratogens-specific drugs, i.e. phenytoin, methotrexate, sodium valproate, alcohol, cigarette smoking, pesticides(dioxin)

Syndromic Cleft Lip +/- Palate400 syndromes associated with CLPChromosomal anomalies

-trisomy 13(Patau), 18(Edwards), 21(Downs), velocardiofacial (22q11)

Inherited Syndromes -Sticklers(AD) -Treacher Collins(AD) -Van der Woude(AD)

Non inherited syndromes-Pierre Robin Sequence (50%have a syndrome-Sticklers/22q11)

Development of the Embryo

Lip and facial development occurs between 5- 10 weeks

Palatal development occurs between 6 – 11 weeks

Oral Anatomy

Orbicularis oris

Orbicularis oris - closes lips, compresses lips against teeth, protrudes lips, shapes lips during speech

Orbicularis oris

The orbicularis oris muscles run parallel to the edge of the cleft and inserts into the alar margin. . There is no muscle in the prolabium in bilateral cleft

Note transverse orientationof levator muscle in middle portion ofthe soft palate

The levator muscles are orientated more longitudinallyand insert on posterior edge ofpalatal bone and along bony cleft

margins

The family’s journey

Antenatal diagnosis

Nurse will contact within 24hoursProvide information / supportPre/post repair photographs Develop feeding planOn going support for family

CHILD & FAMILY

PSYCHOLOGIST

SPEECH THERAPIST

SPECIALIST NURSE

DENTIST

ORTHODONTIST

GENETICIST

ENT/AUDIOLOGY

SURGEON

BirthNurse will visit within 24hoursProvide information / supportFeeding assessment and advicePre/post repair photographs On going support for family

Pierre Robin Sequence;– Micrognathia– U or V-shaped palatal cleft– Glossoptosis / airway obstruction

Pierre Robin Sequence;– Micrognathia– U or V-shaped palatal cleft– Glossoptosis / airway obstruction

Feeding Assessments – tongue position/ oral skillsStabilise airway

PositioningNPA

Oral feeding gradually introduced as toleratedOral stimulation/ NBMRestrict suckling time, lateral position

NGT or gastrostomy support

Why does a cleft cause feeding problemsReduced negative intra-oral pressureCleft lip

leads to poor stabilization of nipple Cleft Palate- reduced area of intact palate

tongue position may be posteriorPierre Robin sequence-

micrognathia, glossoptossis,airway difficulties.

Potential outcomeCleft infant suckles↑ effort = infant tiredSmall volumes taken poor demand = BF ↓ milk producedpoor weight gain

Overcoming Feeding DifficultiesAll infants individualsArtificial feedingCleft lip (including alveolus/gum)

assist lip seal by positioning to underside (rugby ball hold)

maternal finger across lip (reduces swallowed air)

Hold nipple in the mouth

Exaggerated latch techniqueas much breast in mouth as possiblepractice when breast is softVary positions to empty all lobes

Encourage milk flowBreast compression

Assisted feeding

Soft squeezable bottleOrthodontic teatUpright positionPosition teat into non-cleft side

RULE OF TEN(10)IO –WEEKS OLDIO – POUNDS OF WEIGHTIO- GRAMS OF HAEMOGLOBIN

Primary Surgical RepairIsolated Cleft Lip

3-4 months

Isolated Cleft Palate Hard +/or Soft Palate8-9 months

Cleft Lip and Palate Lip and

vomer flap 3-4 months

Soft palate8-9 months

Secondary SurgerySpeech surgery - (Pre school)

Velopharyngeal insufficiency

Alveolar bone graft - (8-10 years)Boney union of alveolus

Orthognathic surgery - (Adult)Malocclusion / aesthetic

Speech

Speech soundsArticulation – production of soundsIntelligibility Resonance – balance of air in oral/nasal cavity- hyper

or hyponasalityOften structural problemsRaised soft palate for ‘p f t s/sh k b v d z g’Lowered soft palate for ‘m n ng’

Why are children prone to Otitis Media?Eustachian tube half adult length

Allows reflux from nasopharynxSupine feeding position aggravates reflux

Otitis Media with effusion (OME)poor middle ear ventilation Negative pressure causes fluid builds up unilateral or

bilateralgrommets

Otitis media in the child with a cleft palate High incidence OMEFailure of the opening mechanism of Eustachian

tubeEustachian tube is shorter than other childrenDeficient attachment of tensor veli palatini

muscleAngle of entry into nasopharynx allows increased

reflux of liquids(Bluestone 1999)

Life with a Cleft Lip and PalateAntenatal diagnosis:- 20 week scanBirthLip Repair:- 3-4 monthsPalate Repair:- 8-9 monthsEarly speech input:- 1yrSpeech Assessment:- 18months / 3yrs / 5yrsMDT Clinic:- 3 / 5 /8 /10 /15 /20yrsAlveolar Bone Graft:- assessment 8yrs

orthodontic preparationAlveolar Bone Graft :- around 10yrsSecondary surgery post adolescent growth

spurtDischarge from service :- 20yrs

Thank you