Clinical management of cleft lip and palate

Question 1

Cleft description (4)

Answer 1

LAHSAL
Lip Alveolus Hard Soft palate Alveolus Lip
UPPER case denotes complete
lahsal
lower case denotes incomplete

Question 2

Cleft epidemiology (3)

Answer 2

1 in 2000 births
Affects hard/ soft/ both
Associated with syndromes

Question 3

Sub mucous cleft palate (3)

Answer 3

1 in 12000 births
Not obvious and may not be suspected until child is older
Usually a previous history of problems with feeding/failure to thrive

Question 4

Sub mucous cleft palate - diagnostic features (2)

Answer 4

Notch on palatal shelf
Bifid uvula
‘blue translucent zone’
or diagnosis made at surgery

Question 5

Overview of cleft lip and palate (3)

Answer 5

Affects 1 in 600/700 babies
1000 births per year in UK
2 distinct presentations
cleft lip +/- cleft palate
isolated cleft palate

Question 6

Cleft lip and palate: aetiology (3)

Answer 6

Genetic - family history in 40% of cases
Environmental - possible causes include anti convulsant drugs, nutritional deficiency, anaemia, alcohol, low socio-economic status.
Probably a genetic predisposition triggered by environmental factors

Question 7

CL & P: diagnosis (2)

Answer 7

Ante natal scan, around 20/40 weeks

Question 8

CL & P: different diagnoses (4)

Answer 8

20% Cleft lip (uni or bi lateral)
50% CLP (uni or bilateral)
30% Cleft Palate
IN ADDITION
15 – 40% of cleft children have  associated anomalies

Question 9

Van der Woude syndrome (5)

Answer 9

Autosomal dominant
Lip Pits
Cleft Palate
Linked with cardiac anomalies
Hypodontia

Question 10

Pierre Robin Sequence (4)

Answer 10

Cleft palate,Glossoptosis,
Mandibular retrognathia
Around 1/3 of cases are linked to
Stickler syndrome - a connective tissue disorder

Question 11

Foetal alcohol spectrum disorder (4)

Answer 11

A wide range of symptoms:

Small head size, low body weight, learning difficulties, co ordination issues which can require life long care.

Question 12

Organisation of cleft care in UK (5)

Answer 12

Clinical Standards Advisory Group 1998 recommended re organisation of cleft care – fewer centres caring for larger volume of cleft patients.
10 regional cleft units in England and Wales:
Trent regional cleft network set up 2000:
Hub in Nottingham,
Spokes in Sheffield, Doncaster, Chesterfield, Lincoln, Derby, Leicester

Question 13

Neonatal period (5)

Answer 13

Breathing
Feeding
Comorbidity in syndromic babies
Notification of cleft service – Cleft Nurse Specialist review
Paediatrician review

Question 14

Cleft lip and palate repair (3)

Answer 14

When the baby is thriving
Lip from 3 months
Posterior palate from 6 months

Question 15

Unilateral lip and anterior palate repair (2)

Answer 15

Soft tissue mobilisation

Vomerine flap and septal centralisation

Question 16

Flap elevation (1)

Answer 16

Von Langenbeck flaps raised off hard palate and musclesin soft palate –> greater palatine artery

Question 17

Muscle dissection (4)

Answer 17

Nasal mucosal closure
Muscle dissected off nasal mucosa under microscope
Muscle bundles rotated from oblique to transverse
Left and right muscles sutured together across the cleft

Question 18

Orthodontic neo natal intervention (1)

Answer 18

Lip strapping

Question 19

Baby - toddler - school age (5)

Answer 19

Monitor speech development
ENT assessment/ hearing tests
NB : glue ear
Dental health education
Pharyngoplasty or revision surgery to assist speech where indicated

Question 20

Incidence of speech problems (4)

Answer 20

2/3rds of all children with cleft palate repairs receive speech therapy (Sell et al 2000).
Around half of all children with cleft palate will have persistent speech difficulties.
Speech can be nasal or produced too far back in the mouth
but speech difficulty not related to defect size.

Question 21

Speech surgery (2)

Answer 21

Palatal lengthening

Pharyngoplasty

Question 22

6 to 10 years (6)

Answer 22

7 yr combined clinic appointment
Dental development and regular dental care
- hypodontia / supernumeraries / microdontia in cleft site
orthodontic assessment
alveolar bone graft as necessary
speech and language therapy
ENT monitoring

Question 23

Dental and orthodontic problems in cleft lip/ palate (6)

Answer 23

Dental disease, poor oral hygiene
Missing/ malformed/ extra teeth
Delayed dental development
Unusual paths of eruption/ intra oral appearance
Skeletal factors – Adverse effects of facial growth and previous surgery.
Dental occlusion and alignment

Question 24

Dental disease/ poor oral hygiene - cleft children have twice as many bad teeth as their peers. Why? (3)

Answer 24

Despite over 90% of cleft children registered with a dentist

40% of 5 year olds and
20% of 12 year olds had active, untreated caries (decay).

National CSAG report 1998

Question 25

CLP: high caries risk (4)

Answer 25

Diet: Highly cariogenic
Oral health: limited intra oral access, reluctance to brush in areas close to previous surgery, unable to tolerate strong taste e.g mint
Reduced salivary flow
Access to dental care: attendance often difficult - multiple hospital appointments
Social deprivation: Higher prevalence of CP in deprived areas

Question 26

Missing/ malformed/ extra teeth (2)

Answer 26

Missing upper permanent lateral incisor occurs in 30-50% of cleft cases (5%in a non cleft population).
54% of cleft cases have dental anomalies (15% in non cleft population).

Question 27

Adverse effects of previous surgery and facial growth (3)

Answer 27

Scarring of soft tissues
Restriction of maxillary development
Mandibular development

Question 28

Dental / orthodontic problems in cleft lip/ palate (1)

Answer 28

Scarring and collapse of maxillary segment(s)

Question 29

Orthodontic treatment (3)

Answer 29

Preparation for alveolar bone graft age 9-11 yrs
Definitive orthodontics age 13 + yrs
Orthodontic/ orthognathic surgery when growth complete

Question 30

Pre-surgica orthodontic alignment (3)

Answer 30

Orthodontic arch expansion.

• open cleft site to facilitate placement of bone graft.
• align rotated incisors if bone support adequate

Question 31

11-20+ years (5)

Answer 31

Support for transition to secondary school
Definitive orthodontic treatment
Or
Combined orthodontic/ orthognathic surgery
Rhinoplasty as appropriate
Referral to clinical geneticist if appropriate

Question 32

13-15 years: definitive orthodontic treatment (2)

Answer 32

Treatment options totally dependent on quality of primary and secondary cleft surgery.
Examples of favourable/less favourable facial growth

Question 33

Orthognathic surgery in cleft patients (5)

Answer 33

Full speech assessment essential to estimate risk of speech deterioration.
Video fluoroscopy (2 d)
Nasendoscopy (3d)
Psychological assessment recommended by CSAG
Any other procedures eg.rhinoplasty, deferred until orthognathic phase complete