Congenital maxilofacial anomalies

1. Cleft lip and palate
DR.OKUKU

2. Introduction
• Most common craniofacial malformation in the newborn.
• 3 main types of oral clefts
 Cleft lip alone
 Cleft palate alone,
 Cleft lip with cleft palate
• Cleft lip with or without a cleft palate (CL/P) and cleft palate alone
(CP) differ with respect to
 embryology,
 etiology,
 candidate genes,
 associated abnormalities, and recurrence risk.

3. Epidemiology
• Race/ethnicity affects the prevalence of CL:
• The First Nations population of British Columbia, Canada, has the highest
reported birth prevalence of CL/P in the world: nearly 30 per 10,000 births
• In the US, the prevalence of CL alone and CL with CP is highest in
American Indian or Alaska Natives (4.2 and 10.1 in 10,000 live births),
lowest in Black Americans (2.1 and 3.7 in 10,000 live births)
• By contrast, the prevalence of CP is relatively constant across races and
ethnicities.
• The male to female ratio of CL/P is 2:1, while the male to female ratio of
CP is 1:2
• Maternal age ≥35 years has been associated with an increased risk for oral
clefts in offspring in several different populations

4. Embryology of cleft lip
• Complete closure of the lip is accomplished by 35 days
postconception as the lateral nasal, median nasal, and
maxillary mesodermal processes merge
• Failure of closure of any one of the three normal sites
of fusion can produce unilateral (most common),
bilateral (less common), or median (rare) lip clefting
• Incomplete when only the upper lip is affected and
complete when the defect extends to the nose.

6. Embryology of Cleft palate
• CP occurs when midline fusion of the palatal shelves fails to
occur.
• Abnormalities in programmed cell death may contribute
to lack of palatal fusion, although this mechanism remains
debated
• CP can occur with CL or alone;
• The latter is possible because palatal closure is not
completed until 56 to 58 days postconception, well after
closure of the lip, and because the etiology may differ.

7. Etiology
• Most oral clefts are nonsyndromic.
• Syndromes account for approximately 30% of
cases of CL/P and 50%of cases of CP
• Nonsyndromic clefting is a genetically
complex event with gene-gene and gene-
environment interactions

8. Etiology
• Genetic factors-several genes(over 30)
• Environmental factors
 Drugs e.g. antiepileptics, ondansetron, nitrofurantoin etc
 Cigarette smoking
 Folate deficiency
 Maternal obesity
 Amniotic band effect
 Syndromic cases e.g. Van der Woude syndrome, Treacher
Collins syndrome

9. Diagnosis
• Prenatal USS done in 2nd trimester
• Investigation to look for associated anomalies
• Complications
• Assessment for fitness for surgery

10. Management
• Multidisciplinary
• Newborn
 Diagnostic examination, general counseling of parents,
feeding instructions, palatal obturator (if necessary);
 genetic evaluation and specification of diagnosis;
empiric risk of recurrence of cleft calculated;
 recommendation of a protocol for the prevention of a
cleft recurrence in the family

11. Continuation of management
protocols
• Age 3 months - Repair of CL (and placement of ventilation tubes)
• Age 6 months - Presurgical orthodontics, if necessary; first speech
evaluation
• Age 9 months - Speech therapy begins
• Age 9-12 months - Repair of CP (placement of ventilation tubes if not done
at the time of CL repair)
• Age 1-7 years - Orthodontic treatment
• Age 7-8 years - Alveolar bone graft
• Older than 8 years - Orthodontic treatment continues

12. Concerns
• Risk of aspiration because of communication
between oral and nasal cavities
• Airway obstruction (especially in Pierre Robin
sequence, where the CP is combined with
micrognathia and the tongue has a normal size)
• Difficulties with feeding of a child with a cleft
and nasal regurgitation

13. Surgical therapy
• Closure of the CL is the first major procedure
that tremendously changes children's future
development and ability to thrive
• The most usual time occurs at approximately
age 3 months
• Rule of 10s ((i.e, 10 lb [4.5 kg], 10 g/dL of
hemoglobin, and age 10 weeks)

14. Protocols for closure
• Watertight closure of the entire cleft can only be achieved by perfect closure of the
lip, anterior nasal floor, area between the dentoalveolar arches, from the anterior
nasal floor to the hard palate and from the hard palate to the soft palate, with the
surgical closure starting with the soft palate and working towards the lip. On the
contrary, primary closure of cleft lip preceding the hard palate cleft closure will
always lead to an anterior or a buccal oronasal fistula.
• Closure of the soft palate should be considered between 5 and 7 months of age, as
the phonetic age of babies begins around this time.
• Closure of the hard palate should be done after the soft palate closure. This avoids
babies experiencing compromised breathing during the recovery period. During the
reconstructed soft pal-ate healing period, the hard palate is still open toward the
nasal cavity i.e. breathing is not restricted. This is of utmost importance particularly
in combined hard and soft palate clefts and especially in patients suffering from a
FairbairnRobin triad, a subdivision of a Pierre Robin sequence.
• Reasons for a surgical protocol
• Achieve optimal closure and avoid oronasal fistula
• Close from inside-to-out-side
• Use mirror-image protocol

15. Techniques