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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
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