Dental management downs syndrome, fetal alcohol syndrome

1. DENTAL MANAGEMENT OF PATIENTS WITH DOWNS
SYNDROME, FETAL ALCOHOL SYNDROME &
CEREBRAL PALSY

2. DOWN SYNDROME (TRISOMY
21 SYNDROME)

3. John Langdon Down (1862) first characterized
Down syndrome as a distinct form of mental
disability
Earlier literature refers to DS as "mongolism,“
 inappropriate; Schreiner (1992).
Thompson and Thompson (1973)  most
frequent chromosomal abnormality in DS is
trisomy 21.

4. • In 1959, the French physician Jérôme Lejeune
identified Down syndrome as a chromosomal
condition.
• Instead of the usual 46 chromosomes present in
each cell, Lejeune observed 47 in the cells of
individuals with Down syndrome.
• It was later determined that an extra partial or
whole copy of chromosome 21 results in the
characteristics associated with Down syndrome.

5. In the year 2000, an
international team of
scientists successfully
identified and catalogued
each of the approximately
329 genes on chromosome
21. This accomplishment
opened the door to great
advances in Down
syndrome research.

6.  4% of DS patients have a
translocation of chromosome 21
onto either chromosome 14 or
another chromosome 22.
 Approx. 1% of the patients with DS
are mosaics, which means that
there is a population of cells with
both normal & trisomic
chromosome numbers.

7. -NEWBERGER D. Down Syndrome: Prenatal Risk Assessment
and Diagnosis. Am Fam Physician. 2000;62(4):825-32.
Chances of Diagnosis of Trisomy 21

8. DIAGNOSIS OF DOWN SYNDROME
 Triple test
 Aminocentesis
 Chorionic villus sampling

9. ULTRASOUND
CHANGES

10. THE CENTERS FOR DISEASE CONTROL (2007)
STATES :
The age of the mother is the only factor
that has been shown to increase the
risk of having a baby with Down
syndrome.
This risk increases with every year, especially
after the mother is 35 years of age. However,
because younger women are more likely to have
babies than older women, 80% of babies with
Down syndrome are born to women younger than
35 years of age.

11. In response to the articles arguing for
dropping the age cut-off, Robert Resta, a
genetic counselor in Seattle, Washington
and former editor of the Journal of Genetic
Counseling, wrote a letter making a key
point.

12. RESTA, HOWEVER, COUNTERED: THIS STATEMENT IS
INCORRECT
The percentage of pregnancies in women
aged 35 years or older has tripled since 1975
and currently about 14% of births in the
United States are to women aged 35 and
older.
The net result is that since about the year
2000, women aged 35 and older have
accounted for slightly more than 50% of
pregnancies with Down syndrome.

13. DOWN SYNDROME; PHYSICAL FEATURES

14. DOWN SYNDROME; PHYSICAL FEATURES

15. APPEARANCE OF CHILD SUFFERING FROM
DOWN SYNDROME & MONGOLIC CHILD

16. CLINICAL FINDINGS IN DS
 Nervous system: Mental retardation  the mild
to moderate range of disability.
 Medical conditions include cardiac defects,
leukemia, and upper respiratory infections.
 Generalized muscular hypotonia is characteristic of
neonates and infants but is less pronounced with
increasing age. General movements are slow,
clumsy and poorly co-ordinated.

17. ORAL FINDINGS IN DS
Mouth breathing
Open bite
 Appearance of macroglossia,
 Fissured lips and tongue,
Angular cheilitis,
Delayed eruption times,

18. Missing & malformed teeth,
Oligodontia, small roots, microdontia,
crowding, and low level of caries.
Malocclusion
Narrow palate and hypotonic tongue
Relative macroglossia.

19. Dental Caries:
Dental caries susceptibility is usually low in those
with DS. This finding has been reported by
Johnson, Young, and Gallios, (1961) who noted a
much lower dental caries incidence in both the
primary and the permanent dentition.
Contrary to the above findings, a higher caries
susceptibility has been reported by
 Rosenstein et al., (1971),
 Gupta et al., (1993) and
 Jain et al., (2009).

20. There is no scientific evidence to support
the hypothesis that people with Down
syndrome have a lower experience of
caries than non-syndromic individuals.
-Int J padetr dent. 2016;26(1):3–12

21. MANAGEMENT IN THE DENTAL OFFICE
 Pleasant, cheerful, affectionate and cooperative, and
dental procedures can be provided without compromise.
 Work at a slower pace.
 Emphasis should be placed on preventive dental care
with frequent follow-up visits to monitor oral hygiene.
 Light sedation and immobilization may be indicated in
those children who are moderately apprehensive.
 Severely resistive patients may require GA.

22. Adequate analgesia should be prescribed
to keep the child comfortable as they may
not be able to express pain or discomfort
due to their learning disability.
If the surgery requires that the neck is placed
in a non-neutral position for a long time
intraoperatively, then cervical spine
radiography should be performed before an
elective case.

23. Routine monitoring should be used as for any
other case. As a minimum, this should include
ECG, pulse oximetry, blood pressure and
temperature monitor.
The National Institute for Health and Clinical
Excellence (NICE) recommends that antibiotic
prophylaxis should only be given if there is
evidence of active infection, as there are
concerns about increasing antibiotic resistance

24. FETAL ALCOHOL SYNDROME

25.  The consumption of one to three drinks a day during
the first 2 months of pregnancy can result in
significant damage to the developing baby.
 Alcohol consumption can cause  neurologic
abnormalities, cardiac defects, growth retardation,
and cognitive defects.
 Leading cause of mental retardation worldwide.
 In the US the FAS prevalence rate is estimated to
be between 0.2 to 2 cases per 1000 live births.

26. FETAL ALCOHOL SYNDROME ; CLINICAL FEATURES

27. HOW DOES FETAL ALCOHOL SYNDROME
AFFECT DENTAL HEALTH?
High incidence of dental and skeletal
malocclusions.
As young children (younger than age 5),
these individuals tend to be hyperactive. As
they become older, these children are
usually cooperative and relatively easy to
treat.

28.  Cleft palate
 Hypo and hyperplasia of the maxilla and mandible,
malformed, missing and/or misaligned teeth.
 Weak oris obicularis muscles
 Late loss of the primary dentition; late eruption of
permanent teeth
 Unusual behavioral patterns and tissue and physical
sensitivities that make dental treatment difficult.

29. SUGGESTIONS FOR THOSE CARING FOR
CHILDREN WITH FETAL ALCOHOL SYNDROME
 Toothbrushing may be a challenge due to misaligned
teeth, small jaw, and weak oral muscles. The
smallest toothbrush size possible should be used..
 Children suffering from FAS are often sensitive to
various flavors and tastes. Dipping the brush bristles
in water and thoroughly removing plaque around the
gums may be the best idea.

30. Routine in his environment. Unfortunately,
dental visits are breaks in the normal routine
and may be upsetting to the patient.
To avoid the suddenness of a dental visit, take
photos of the dental office and staff before
hand to remind the patient of previous visits.
Review the photographs each day for about
two weeks prior to the appointment.

31.  the appointment at the quietest time of the day or
even after regular hours.
 Show them the exact instruments and materials
you will be using to avoid confusion and
misunderstanding.
 FAS have extreme reactions to medications and it
is best to consult with the parent and pediatrician
when administering anesthetics.
 Visually, objects on the wall or hanging from the
treatment lamp may be disturbingDark glasses will
reduce the glare from overhead lights.

32.  Patients with FAS may experience more "loud" sounds
than the average person. Music headphones can be a
great help.

33. CEREBRAL PALSY

34. Nelson et al .,(1969) used the term cerebral
palsy to describe is a group of non-
progressive disorders of the neuromuscular
system in which the motor centres of the
brain have been damaged.Cerebral palsy (CP) is an “umbrella term”
covering a group of nonprogressive, but often
changing motor disorders that occur during
early stages of life due to damage to the
brain.
(National Institute of Neurological Disorders and Stroke, 2006)

35. CEREBRAL PALSY; INCIDENCE
Cerebral palsy in the US, for all ages, is
1.5 to 3 per 1000 individuals.
One newborn in approximately 200 live
births will be affected by this condition.

36. Prenatal Postnatal
- Developmental defects
- Maternal infections like rubella
- Rh incompatibilities
- Premature birth
- Birth injuries
- Anoxia during delivery
- Toxaemias of pregnancy
- Infections of the brain such as
meningitis and encephalitis
- Trauma
- Tumors
- Congenital defects of brain
- Kernicterus
- Poisoning with certain drugs and
heavy metals
Etiology

37. HOW IS CEREBRAL PALSY DIAGNOSED?
After birth, ultrasonic examination of the brain of
a premature infant may reveal cerebral
abnormalities, such as hyperechoic and
hypoechoic lesions.
After birth, hypoechoic areas which appear on
ultrasonic images of a baby’s brain can predict
future problems, such as motor dysfunction
related to movement and coordination.

38. A physical examination of an infant with cerebral
palsy may reveal: spasticity of the limbs, arms or
legs which appear to be locked in an abnormal
position, lack of normal balance, or an abnormal
walking pattern in older children.
A large portion of children with cerebral palsy
experience significant feeding and swallowing
problems during the first 12 months of life. This
finding often proceeds the diagnosis of cerebral
palsy.

39. CEREBRAL PALSY CLINICAL FEATURES
Early symptoms manifest during the
perinatal period.
Difficulties with fine motor tasks,
involuntary movements, imbalance and
walking ability.
Varying degrees of paralysis as well as
muscular weakness and dysfunction.

40.  Monoplegia- involvement of one limb only
 Hemiplegia- involvement of one side of the body
 Paraplegia- involvement of both legs only
 Diplegia- involvement of both legs with minimum
involvement of both arms
 Quadriplegia- involvement of all four limbs

42. CEREBRAL PALSY SPASTIC
 > 70%
 Caused by lesion in the cerebral cortex
 Hyperirritability of involved muscles, resulting in
exaggerated contraction when stimulated.
 Limited control of neck muscles, which results in head
roll

43.  Lack of control of muscles supporting the trunk, which
results in difficulty in maintaining upright posture.
 Lack of co-ordination of intraoral, perioral, and
masticatory musculature; possibility of impaired chewing
and swallowing, excessive drooling, persistent spastic
tongue thrust, and speech impairments.

44. OROFACIAL FINDINGS IN SPASTIC CEREBRAL PALSY:
The head is tensely reclined.
The mouth is open, and facial movements
are tense.
The tongue is hypertonic and cigar-shaped.
There is tongue thrust during swallowing and
speaking.
Since the upper lip is underdeveloped, it
does not produce enough pressure on the
front teeth to align them correctly.

45. CEREBRAL PALSY ATHETOSIS (DYSKINETIC)
 15% of the patients with cerebral palsy
 Caused by lesion in the basal ganglion
 Characterized by constant and uncontrolled motion
of involved muscles.
 Succession of slow, twisting, or writhing involuntary
movements (athetosis) or quick, jerky movements
(chorioathetosis).

46.  Frequent involvement of neck musculature,
which results in excessive movement of the
head.
 Frequent, uncontrolled jaw movements causing
abrupt closure of the jaws or severe bruxism.
 Hypotonicity of the perioral musculature
 Facial grimacing

47. OROFACIAL FINDINGS IN ATHETOTIC CEREBRAL PALSY:
The tongue shows spontaneous wave-
like movements.
There may be an abrupt and wide
opening of the mouth, which can lead to
jaw dislocation.
There is an uncoordinated movement of
tongue, jaw, and face muscles.

48. CEREBRAL PALSY ATAXIA
 5% of cerebral palsy patients
 Caused by lesion in the cerebellum
 Inability of involved muscles to contract
completely so that voluntary movements can be
only partially performed

49. Poor sense of balance and uncoordinated
voluntary movements
Possibility of tremors and an
uncontrollable trembling or quivering on
attempting voluntary tasks.

50. OROFACIAL FINDINGS IN PALSY ATAXIA :
The tongue is large, flat, and protruded.
Facial movements are weak, and the upper
lip is inactive.

51. MIXED TYPE CEREBRAL PALSY
Approximated 10% of cerebral palsy
patients
Combination of characteristics of more
than one type of cerebral palsy (eg. mixed
spastic athetoid quadriplegia)

52. NEONATAL REFLEXES PERSIST
 Asymmetric tonic neck reflex- if the patients
head is suddenly turned to one side, the arm and
leg on the side to which the face is turned will
extend and stiffen. The limbs on the opposite
side will flex.
 Tonic labyrinthine reflex- if the patients head
falls backwards while the patient is supine, the
back may assume the position known as postural
extension; the arms and legs will straighten out
and the neck and back with arc.

53. Startle reflex- this reflex consists of sudden,
involuntary, often forceful body movements.
This reaction is produced when the patient is
surprised by stimuli, such as sudden noises
or unexpected movements by other people.

54.  These patients may give a history of seizure
disorders (30-50%),
 mental retardation (60%),
 behavioural or emotional problems, and
 visual or hearing impairments, which may
influence adversely the development of speech
and learning skills.

55. ORAL FINDINGS
 Enamel hypoplasia in the primary dentition.
 Neuromuscular problems  bruxism, attrition, TMD,
malocclusions
 Presence of dental caries, missing teeth and periodontal
disease occur frequently.
 Santos et al (2002) Children with CP had a higher mean
DMFS and higher plaque index scores. Microbiological
examination revealed higher levels of mutans streptococci
and lactobacilli.
 Camargo et al. (2008) Children and adolescents with CP
suffer a higher burden of untreated dental caries

56. The oral clearance of CP patients is
poor  To compensate, the diet is
usually soft and high in carbohydrates.

57. CAUSE OF DROOLING
 Drooling is an indication of an upset in the
coordinated control mechanism of orofacial
musculature leading to excessive pooling of
saliva in the anterior mouth and resulting in
unintentional loss of saliva from the mouth.
Blasco et al., 1992

58.  During the oral stage of swallowing, marked
dysfunction of the coordinated muscle activity of the
oral musculature that hampered initial swallowing was
identified in the CP patients.
 To determine whether or not drooling is due to
hypersalivation, Curzon et al (2003) carried out a clinical
study to compare CP patients with a matched control group.
 CP who drool did not appear to have excessive saliva
secretion.

59. SPECIAL EQUIPMENTS FOR CP CHILDREN
Standers and Gait Trainers
Modular Chairs

60. RIFTON TRAM

61. MANAGEMENT IN THE DENTAL CLINIC
 Considering treating a patient who uses a wheelchair in a
wheelchair.
 If a patient is to be transferred to the dental chair, ask
about a preference for the mode of transfer.
 Stabilize the patients head throughout all phases of
dental treatment.
 Try to place and maintain the patient in the midline of the
dental chair, with arms and legs as close to the body as
feasible.

62. Keep the patients back slightly elevated.
On placing the patient in the dental chair,
determine the patient’s degree of comfort and
assess the position of the extremities.
Do not force the limbs into unnatural positions.

63.  Avoid presenting stimuli such as abrupt
movements, noises, and lights without
forewarning the patient.
 Introduce intraoral stimuli slowly to avoid eliciting
a gag reflex or to make it less severe.
 Work efficiently and minimize patient time in the
chair

64. Consider the use of pillows, towels, and
other measure for trunk and limb support.
Use immobilization judiciously to control
movements of the extremities.
For control of involuntary jaw movements
 mouth props and finger splints.

65.  Early and vigorous program of prevention is
essential.
 Modification to standard toothbrushes may be
required.
 Electric toothbrushes may also be utilized.
 The parents, care takers from group homes or
nursing personnel from institutions must be
instructed thoroughly in the oral hygiene
techniques essential for the patients.

66. CONCLUSION
It is a challenge to work with patients with
neurological impairments but armed with
the proper knowledge and dedication,
dental personnel can be effective in
providing the very best of care to a
segment of the population in great need.

67. REFERENCES
1. Donald. Dentistry for the Child and Adolescent.
2. Stephen Wei. Pediatric Dentistry: Total Patient
Care. Pg 554-574
3. Kenneth Troutman. Patient Management. Section
7. Pg 833-854.

68. THANK
YOU