Pierre Robin sequence involves micrognathia, a cleft palate, and airway obstruction. Cleft lip and palate can be caused by genetic and environmental factors. Feeding difficulties are common in infants with clefts due to issues creating suction and maintaining nipple contact. A multidisciplinary team supports families throughout treatment including surgery, speech therapy, and orthodontics from infancy through adulthood.

1. Dr V.RAMKUMAR
CONSULTANT
DENTAL&FACIOMAXILLARY SURGEON
REG:NO -4118 TAMILNADU- INDAI(ASIA)

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

4. 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)

5. 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)

6. Development of the Embryo
Lip and facial development occurs between 5- 10
weeks
Palatal development occurs between 6 – 11 weeks

7. Oral Anatomy

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

9. 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

10. 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

11. The family’s journey

12. Antenatal diagnosis

13. Nurse will contact within 24hours
Provide information / support
Pre/post repair photographs
Develop feeding plan
On going support for family

14. PSYCHOLOGIST
CHILD & FAMILY
SPEECH
THERAPIST
SPECIALIST
NURSE
ORTHODONTIST
DENTIST
GENETICIST
ENT/AUDIOLOGY
SURGEON

15. Birth
Nurse will visit within 24hours
Provide information / support
Feeding assessment and advice
Pre/post repair photographs
On going support for family

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

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

18. 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

19. 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.

20. Potential outcome
Cleft infant suckles
­ effort = infant tired
Small volumes taken
poor demand = BF ¯ milk produced
poor weight gain

21. 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

22. 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

23. Assisted feeding
Soft squeezable bottle
Orthodontic teat
Upright position
Position teat into non-cleft side

24. RULE OF TEN(10)
IO –WEEKS OLD
IO – POUNDS OF WEIGHT
IO- GRAMS OF HAEMOGLOBIN

25. 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

26. Secondary Surgery
Speech surgery - (Pre school)
Velopharyngeal insufficiency
Alveolar bone graft - (8-10 years)
Boney union of alveolus
Orthognathic surgery - (Adult)
Malocclusion / aesthetic

27. Speech

28. 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’

29. 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

30. 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)

31. 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

32.  Thank you