Dr V.RAMKUMAR
CONSULTANT
DENTAL&FACIOMAXILLARY SURGEON
REG:NO -4118 TAMILNADU- INDAI(ASIA)
Pierre Robin Sequence;
– Micrognathia
– U or V-shaped palatal cleft
– Glossoptosis / airway obstruction
Causes of
Isolated Cleft Lip +/- palate
Multifactorial
environmental + 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 +/- Palate
400 syndromes associated with CLP
Chromosomal 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 orientation
of levator muscle in
middle portion of
the soft palate
The levator muscles are
orientated more longitudinally
and insert on posterior edge of
palatal bone and along bony cleft
margins
The family’s journey
Antenatal diagnosis
Nurse will contact within 24hours
Provide information / support
Pre/post repair photographs
Develop feeding plan
On going support for family
Birth
Nurse will visit within 24hours
Provide information / support
Feeding assessment and advice
Pre/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 skills
Stabilise airway
Positioning
NPA
Oral feeding gradually introduced as tolerated
Oral stimulation/ NBM
Restrict suckling time, lateral position
NGT or gastrostomy support
Why does a cleft cause feeding
problems
Reduced negative intra-oral pressure
Cleft lip
leads to poor stabilization of nipple
Cleft Palate- reduced area of intact palate
tongue position may be posterior
Pierre Robin sequence-
micrognathia, glossoptossis,airway difficulties.
Potential outcome
Cleft infant suckles
effort = infant tired
Small volumes taken
poor demand = BF ¯ milk produced
poor weight gain
Overcoming Feeding Difficulties
All infants individuals
Artificial feeding
Cleft 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 technique
as much breast in mouth as possible
practice when breast is soft
Vary positions to empty all lobes
Encourage milk flow
Breast compression
Assisted feeding
Soft squeezable bottle
Orthodontic teat
Upright position
Position teat into non-cleft side
RULE OF TEN(10)
IO –WEEKS OLD
IO – POUNDS OF WEIGHT
IO- GRAMS OF HAEMOGLOBIN
Primary
Surgical Repair
Isolated Cleft Lip
3-4 months
Isolated Cleft Palate
Hard +/or Soft Palate
8-9 months
Cleft Lip and Palate
Lip and
vomer flap 3-4 months
Soft palate
8-9 months
Secondary Surgery
Speech surgery - (Pre school)
Velopharyngeal insufficiency
Alveolar bone graft - (8-10 years)
Boney union of alveolus
Orthognathic surgery - (Adult)
Malocclusion / aesthetic
Speech
Speech sounds
Articulation – production of sounds
Intelligibility
Resonance – balance of air in oral/nasal cavity- hyper
or hyponasality
Often structural problems
Raised 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 nasopharynx
Supine feeding position aggravates reflux
Otitis Media with effusion (OME)
poor middle ear ventilation
Negative pressure causes fluid builds up unilateral or
bilateral
grommets
Otitis media in the child with a
cleft palate
High incidence OME
Failure of the opening mechanism of Eustachian
tube
Eustachian tube is shorter than other children
Deficient attachment of tensor veli palatini
muscle
Angle of entry into nasopharynx allows increased
reflux of liquids
(Bluestone 1999)
Life with a Cleft Lip and Palate
Antenatal diagnosis:- 20 week scan
Birth
Lip Repair:- 3-4 months
Palate Repair:- 8-9 months
Early speech input:- 1yr
Speech Assessment:- 18months / 3yrs / 5yrs
MDT Clinic:- 3 / 5 /8 /10 /15 /20yrs
Alveolar Bone Graft:- assessment 8yrs
orthodontic preparation
Alveolar Bone Graft :- around 10yrs
Secondary surgery post adolescent growth
spurt
Discharge from service :- 20yrs