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Syndromes Flashcards

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1
Q

Define congenital anomalies

A

structural or functional anomalies that occur during intrauterine life and
can be identified prenatally, at birth, or sometimes may only be detected later in infancy

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2
Q

Define deformation

A

Result of a mechanical defect without basic morphogenic defect

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3
Q

Define disruption

A

Result of the destruction of a structure normally formed

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4
Q

Define malformation

A

Intrinsic alteration of the affected tissue

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5
Q

Define dysplasia

A

Result of the differentiation and organization of a tissue

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6
Q

Genetic factors make up ____% of congenital malformations

A

90%

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7
Q

Environmental factors make up ____% of congenital malformations

A

1-%

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8
Q

What types of drugs can contribute to congenital malformations? (5)

A
  • alcohol
  • anticonvulsants
  • antithyroid
  • lithium
  • thalidomine
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9
Q

What congenital infections can contribute to congenital malformations? (5)

A
  • Toxoplasmosis
  • rubeola
  • CMV
  • herpes
  • syphilis
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10
Q

What maternal disorders can contribute to congenital malformations? (3)

A
  • Diabetes
  • Phenylketonuria
  • Maternal hyperthermia
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11
Q

What physical agent can contribute to congenital malformations?

A

Ionizing radiation

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12
Q

The most frequent congenital malformations are? What are they related to? (3)

A

Cleft palate and Craniosynostosis

May or may not be associated to syndromes

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13
Q

Cleft palate makes up ____% of the total craniofacial malformations

A

15

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14
Q

Cleft palate affects ____ of births. Frequently in which ethnicities? (3)

A

1/1000 births.

Frequently in american and asian indians

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15
Q

What causes cleft palate?

A

Lack of coalescence between embryonic facial processes: No

union of nasomedial and maxillary process.

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16
Q

How many times is the palate formed?

A

Twice

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17
Q

When are the different palate formations?

A

• Primary palate: at the 6th week.
• Secondary palate: posterior part of the palate formed at
the 8th week

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18
Q

What is cleft lip?

A

Can range from a little notch in the coloured part of the lip to a
complete separation of the upper lip which can extend up and into the nose

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19
Q

What does complete cleft lip mean?

A

Meaning the cleft goes up into the nose

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20
Q

What is cleft palate?

A

A gap in the roof of the mouth

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21
Q

What divides the primary and secondary palate?

A

Incisive foramen

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22
Q

What was Victor Spina’s classification of cleft palate? (2)

A
  • In function of the anatomical extension of the fissures

- embryonic origin

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23
Q

What are Victor Spina’s fissures ? (3)

A
  • Transforament cleft
  • Post foramen cleft
  • Preforamen cleft
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24
Q

What are the affected areas for the transforamen cleft? (4)

A
  • lip
  • dental arch
  • hard and soft palate
  • up to the uvula
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25
The transforamen cleft promotes...
total communication between nasal and oral cavities
26
The transforamen cleft can be...
unilateral or bilateral
27
What are the affected areas for the post-foramen cleft ?
Posterior palate
28
What are the affected areas for the pre-foramen cleft ?
- lips | - primary teeth up to the incisor foramen
29
Clinical manifestations of preforamen cleft?
Great diversity of clinical manifestations
30
What is keith's scar? (2)
- Cut in cutaneous border of the lip | - seen in preforamen cleft
31
What happens in the pre-foramen cleft?
Rupture of maxilla & primary palate until the incisive foramen
32
Preforamen cleft laterality? (3)
- Unilateral - bilateral - medium (lip filtrum)
33
Extension of fissure in post-foramen cleft?
Variable extension starting at the uvula towards the incisive foramen
34
Post-foramen cleft: alveolar ridge?
Alveolar ridge remains complete even in the complete form
35
Post foramen cleft problems? (3)
* Not so important aesthetic problems: integral perioral musculature. * Functional functional problems: phonation and swallowing.
36
Transforamen cleft fissures?
include primary and seconday palate
37
Transforamen cleft laterality?
Can be uni, bilateral or occupy the midline of the maxilla
38
Transforamen cleft segments?
Compromise from the lip to the uvula, dividing the maxilla into two segments or three (uni or bilateral
39
What dental alterations occur with cleft palate? (4)
* Loss of tooth support in teeth adjacent to the fissure * Deviation in the eruption lines * Slightly concave profile * Posterior crossed bite by the collapse of the maxilla
40
What alterations (nondental) occur with cleft palate?
* Auditory alterations: Otitis media suppurativa, hearing loss * Alterations in phonation * Alterations in swallowing and feeding * Psychological problems: self-esteem, rejection
41
Cleft palate treatment at 2 weeks? (2)
Impression taking for maxillary obturator. Pre-surgical maxillary orthopedics for segment approximation
42
Cleft palate treatment at 3 months? (2)
Cheiloplasty. Take impression for new shutter to follow the growth
43
Cleft palate treatment at 1 year?
palatoplasty
44
Cleft palate treatment at 10-15 years?
Nasal bone surgery
45
What is craniosynostosis?
- premature closure of sutures produces a decrease in the growth in that area - compensatory growth in other sutures
46
Craniosynostosis: head shape?
Depends on the fused suture, the age of the child at the moment of closure and the number of sutures involved
47
Craniosynostosis: premature closure of sagital suture?
It limits the lateral growth—-Dolicocephalic
48
Craniosynostosis: premature closure of cornoal suture?
It limits the sagital growth. It may be unilateral (plagicephalic) or bilateral (braquicephalic)
49
Craniosynostosis: premature closure of metopic suture?
Trigonocephalic
50
The metopic suture usually closes ....
At the 2nd - 6th month
51
The coronal suture usually closes ....
Between the 6th and 12th month
52
The sagittal suture usualy closes ...
Between the 6th and 12th month
53
Craniosynostosis may be associated with...? (2)
Blindness and mental disability
54
What is oxicephalia? (3)
- Craniosyntosis - bilateral affectation of coronal and sagittal sutures - produces cranial hypertension (severest kind of craniosyntosis)
55
Oxicephalia doesnt affect... (2)
Doesn't affect facial and cranial base growth
56
Craniosynostosis treatment? (2)
* The postural plagiocephaly is corrected with helmets. | * If necessary lineal craniotomy
57
What type of inheritance does Van der Woude syndrome have?
Autosomal dominant
58
What type of inheritance does Crouzon syndrome have?
Autosomal dominant
59
Apert syndrome inheritance?
Autosomal dominant
60
Cleidocranial dysostosis inheritance?
Autosomal dominant
61
Nasofrontal dyslasia inheritance?
Autosomal dominant
62
Franceschetti syndrome inheritance?
Autosomal dominant
63
Otomandibular dysostosis inheritance?
Autosomal dominant
64
Pierre-robin syndrome inheritance?
Autosomal dominant
65
Tricho dento osseous symdrome inheritance?
Autosomal dominant
66
Ostogenesis imperfecta inheritance?
Autosomal dominant
67
Acondroplasia inheritance?
Autosomal dominant
68
Van de woude syndrome? (3)
* Abnormal depressions and prominences in the lower lip (88%). * Dental agenesis. * It is the most frequent form of syndromic cleft palate.
69
What are the two types of Van de woude syndrome? (3)
- cleft lip with or without cleft palate | - cleft palate only
70
What is the most frequent form of syndromic cleft palate?
Van de woude syndrome
71
What is another name for crouzon syndrome?
craniofacial dysostosis
72
What is crouzon syndrome? (2)
* Premature cranial sinostosis producing facial malformations. * Hypertelorism and exophthalmos
73
Crouzon syndrome treatment? (2)
• Lefort III osteotomy with complete facial advance (4-5 years). • Progressive bone distraction of the facial middle third
74
At what age do we treat crouzon sydrome?
4-5 years old for the lefort 3 ostectomy
75
What is another name for apert syndrome?
Acrocephalosyndactyly
76
What is apert syndrome? (6)
- Premasture fusion of coronal and sagital sutures - hypoplasia of facial middle third (present at birth) - Wide nose, hypertelorism and exphthalmos - heart alterations and mental disability - syndactyly in hands and feet (fingers 2, 3, 4 fused) - aplasia and ankylosis of articulations
77
Dental manifestations of crouzon syndrome?
``` Nasomaxilar retrusion creating a false prognatism and a class III malocclusion. ```
78
Dental manifestations of apert syndrome? (3)
- Frequent class III | - open or cross bite
79
What is cleidocraneal dysostosis? (2)
- Delayed closure of the anterior fontanella, producing a prominence of the frontal and parietal bones—-> Brachycephalic skull. - Aplasia or hypoplasia of the collarbone
80
What are the dental manifestations of cleidocraneal dysostosis? (4)
- Brachycephalic skull - Class III malocclusion - delayed eruption - supernumerary teeth
81
What is nasofrontal dysplasia? (4)
* Hypertelorism * Non complete formation of the nose tip. * Bifid anterior skull (V shaped) * Cleft in the nose or lip

Peds III (9 decks)

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