Craniosynostosis and Deformational Plagiocephaly Flashcards
1
Q
Differentiation between positional posterior plagiocephaly
and lambdoid craniosynostosis
A
based on the shape
of the head, position of the ears, the presence of a mastoid
bulge, and the appearance of the forehead on the affected
side.
2
Q
What is the suitable time for surgical intervention for craniosynostosis?
A
most centers intervene during the first year
3
Q
Complications in craniosynostosis surgeries are common
A
f Complications in craniosynostosis surgeries are fortunately rare
4
Q
Most cases are genetic underpinnings
A
F Most cases are idiopathic,
with a small number having genetic underpinnings
5
Q
What Virchow law
A
Virchow law states
that closure of a cranial suture results in compensatory growth parallel to the fused suture, with the arrest of growth in a perpendicular fashion to the suture
6
Q
The modern management of craniosynostosis involves what?
A
The modern management of craniosynostosis involves early
diagnosis with interventions aimed at minimizing constricted brain
growth and craniofacial deformities.
7
Q
All
sutures, with the exception of the metopic suture, are initiated at a
point where neural crest cells and mesoderm are in close approximation
A
T
8
Q
Most common of suture fused ?
A
with the most common suture fused
being sagittal, followed by coronal, metopic, and lambdoid
9
Q
All single sutural forms are considered non genetic inheritance
A
F single sutural forms are more
diverse and generally do not demonstrate as much uniformity. A
very small percentage of these are thought to be familial
10
Q
mutations associated with syndromic craniosynostoses to be FGFR3, FGFR2, and
TWISTl T F
A
F with nonsyndromic craniosynostoses to be FGFR3, FGFR2, and
TWISTl
11
Q
genetic counseling is recommended in all cases of
craniosynostosis
A
T particular importance in the presence of
coronal craniosynostosis or when multiple sutures are involved
12
Q
lambdoid craniosynostosis is more common in male infants and in mothers experiencing preterm labor
A
T
13
Q
to differentiate Deformational Plagiocephaly from the similarly appearing, but extremely rare, lambdoid craniosynostosis.
A
This distinction can almost always be based
on physical examination
-The presence of a mastoid bulge on the involved side is nearly pathognomonic for lambdoid craniosynostosis and is rarely found in cases of positional plagiocephaly.
14
Q
The most suture fusion in female
A
Coronal
15
Q
a computed tomography (CT)
scan is considered the standard for radiological imaging ofcranial
sutures, T F
A
T
16
Q
How much the percentage of increased ICP in craniosynostosis?
A
14% of patients with single sutures and 47% with multisuture craniosynostosis experienced obviously elevated ICP.
17
Q
timing
of surgery, and specifically an earlier age at correction, may correlate
with improved long-term neurological outcomes.
A
t
18
Q
The optimal timing of surgery for craniosynostosis is
A
within the first year of life
19
Q
Open cranial vault remodeling techniques are performed , between 6 and 3 months of age
A
F Open cranial vault remodeling techniques are performed later, between 8 and 11 months of age
20
Q
in sagittal craniosynostosis The posterior vault is traditionally performed first, why?
A
T. as there have
been reports of an improvement in the anterior deformity following
this intervention
21
Q
The posterior vault reconstruction is performed at
9 to 12 months of age
A
T
22
Q
traditional fronto-orbital advancement (FOA) ifneeded at 18 to 24 months of age
A
T
23
Q
What is the harlequin orbital deformity?
A
is seen on anteroposterior radiographs of patients with unicoronal craniosynostosis In Coronal Craniosynostosis
24
Q
What are the characteristics of features of facial deformity in coronal synostosis?
A
characteristic facial features, including nasal radix deviation toward the affected side and chin point deviation toward the unaffected side
25
is desirable to perform an overcorrection of the deformity
to accommodate future growth of the child
T
26
Metopic Craniosynostosis fusion name
a keel-shaped forehead trigonocephaly
27
the metopic suture normally closes
before 12 months of age
F the metopic suture normally closes
before 8 months ofage
28
The decision to intervene surgically is the most
often based on the severity of the suture is closed in metopic suture
F The decision to intervene surgically is most
often based on the severity ofthe trigonocephaly, not the fact that the
suture is closed
29
Lambdoid Craniosynostosis is the most common type. T. F
Premature closure ofthe lambdoid suture is the least common form
ofcraniosynostosis
30
In Lambdoid Craniosynostosis an contralateral frontal flattening occures
F an ipsilateral frontal flattening
31
The surgical treatment oflambdoid craniosynostosis is traditionally a posterior vault remodeling procedure
T
32
Complications of craniosynostosis Surgery?
The types of complications can be separated
into acute perioperative complications (death, hemorrhage, venous
air embolism, cerebrospinal fluid fistula, blindness, infection)
And those occurring months to years following surgery (strabismus, sensory disturbances, contour irregularities
33
Among the acute complications perioperative death is most often due to exsanguination
T
34
Venous air embolism can occur whenever surgery is performed above the level of the heart and noncollapsible veins are exposed to air
T
35
Venous air emboli are uncommon during craniosynostosis T F
Venous air emboli are fairly common during craniosynostosis surgery (up to 82% of patients experience an event) but few are symptomatic
36
If an air embolus is suspected
and the patient is symptomatic what should you do?
The treatment is the placement of a wet sponge over the operative field and putting the patient in the left lateral decubitus and Trendelenburg position to discourage continued air entry into the venous system and position the right ventricular outflow tract inferior to the right ventricular cavity.
37
Treatment of an asymptomatic cerebrospinal fluid leak is conservative with the putting drains
F with the removal of drains from suction
38
Ophthalmologic complications (strabismus, astigmatism, and
arnblyopia) following craniosynostosis surgery are seen in cases of
Picoronal craniosynostosis
F
unicoronal craniosynostosis
39
indications for reoperation in patients with craniosynostosis?
is the presence of bone gaps and contour irregularities
at skeletal maturity
40
One of the most difficult things to measure with regard to surgery
for nonsyndromic craniosynostosis is an subjective outcome
F One of the most difficult things to measure with regard to surgery
for nonsyndromic craniosynostosis is an objective outcome
41
is the Whitaker classification?
A Whitaker class I is a patient in whom no additional procedure is required
class II indicates the need for a soft tissue or bone-contouring procedure
Class III necessitates additional major osteotomies to be performed
IV result is a patient in whom an additional major craniofacial procedure is required, essentially duplicating or exceeding the original
procedure
42
The common syndromic craniosynostoses?
are primarily
autosomal dominant and FGFR-related with bicoronal
synostosis being the most common pattern
43
overall complication
rates in syndromic craniosynostoses are high,
F LOW
44
The metopic suture normally fuses in the second
year of life
F he metopic suture normally fuses in the first
year of life the remaining major sutures typically fuse in the second
or third decade
45
Syndromic craniosynostoses are nearly as common as isolated forms
T
46
What is the Brachycephaly?
. Brachycephaly, a head shape with biparietal widening and anterior-posterior restriction, results from bilateral coronal synostosis
47
What is Turricephaly?
. Turricephaly (also calledoxycephaly}, a steeple-shaped head with a long, high forehead resulting from synostosis of the coronal plus another major suture
48
What is a Cloverleaf-shaped head?
Cloverleaf-shaped head,
known by its German term Kleeblattschadel, is uncommon, resulting from the fusion of multiple major sutures, but is encountered in
severe Pfeiffer syndrome patients
49
What is the Trigonocephaly?
Trigonocephaly is typically seen with fusion of the
metopic suture and leads to a triangular forehead shape with temporal
narrowing and, depending on severity, hypotelorism
50
What is the Scaphocephaly?
Scaphocephaly,
from Greek skaphe meaning boat, results from fusion of the sagittal
suture, elongating the head in the anterior-posterior dimension and
giving it a boatlike appearance.
51
What is the Plagiocephaly?
Plagiocephaly can be seen with either
prolonged positioning or preferential lying on one side of the head
as well as with unilateral fusions of the lambdoid or coronal sutures
52
Both simple and
complex syndactylies are seen in syndromic craniosynostosis
T
53
cases sporadic mutations are more
common than familial forms syndromic craniosynostosis
T
54
Saethre-Chotzen
is the syndrome, which is associated with mutations in the FGFR gene
F Saethre-Chotzen
syndrome, which is associated with mutations in the TWISTI gene
55
Crouzon Syndrome Craniosynostosis, usually uni coronal
F Crouzon Syndrome
Craniosynostosis, usually bicoronal
56
What are the facial features of Crouzon Syndrom?
midface hypoplasia, shallow orbits, and exophthalmos, hypertelorism, parrot-beaked nose, maxillary hypoplasia, and mandibular prognathism
57
Pattern of inheritance of Crouzon Syndrom
When familial, it is inherited in an autosomal dominant fashion via mutations of the FGFR2
58
the percentage of Crouzon Syndrom in all Syndrome Craniosynostosis
The prevalence of Crouzon syndrome at birth
is about 1:60,000, making it responsible for about 4.8% of cases of
craniosynostosis.
59
What is the head shape of Crouzon Syndrom
, brachycephaly is the most common calvarial deformity, although trigonocephaly and scaphocephaly, from fusion of the
metopic and sagittal sutures, respectively, have also been reported
60
The type of mandibular malocclusion in Crouzon ?
Involvement of
the cranial base sutures is responsible for the maxillary hypoplasia
component and when combined with normal mandibular growth
results in class III malocclusion and relative prognathism
61
Exophthalmos in this syndrome can be particularly severe
T
62
Strabismus can occur in Crouzon syndrome
T
63
The hands and feet are usually deformed in crouzon?
The hands and feet are usually normal and intelligence is normal
or near-normal.
64
Apert Syndrome shares cranial and
facial features with Crouzon syndrome
T
65
Type of craniosynostosis in Apert?
brachycephalic or turribrachycephalic head shape
66
How you can distinguish between Apert and Crouzon syndromes.
The presence of syndactyly is a distinguishing feature between Apert and Crouzon syndromes.
67
The pattern of inheritance of Apert Syndrom
inherited in an autosomal dominant fashion
68
the pathognomonic finding of Apert syndrom?
Symmetrical hand syndactyly
69
characteristic exorbitism due to shallow
orbits as well as a negative canthal vector occurs in apert
T
70
Severe acne, responsive to standard treatments, develops as children
with Crouzon syndrome reach adolescence
F Severe acne, responsive to standard treatments, develops as children
with Apert syndrome reach adolescence
71
Children with Apert syndrome have, on average, some degree of intellectual disability
T
72
The overall phenotype of Crouzon is typically more severe than Apert syndrome.
F Apert phenotype is typically more severe than in Crouzon syndrome.
73
What is Saethre-Chotzen Syndrome?
Is defined by craniosynostosis in association with facial dysmorphia and limb anomalies
74
The metopic suture normally fuses in the second
year of life
F the metopic suture normally fuses in the first
year of life the remaining major sutures typically fuse in the second
or third decade
75
In Saethre-Chotzen Syndrome the mutation occurs in FGFR2
F the majority of mutations in SaethreChotzen syndrome occur in the TWISTl gene (although FGFR2 and
FGFR3 mutations have been described
76
Mild phenotype of Saethre-Chotzen may go undiagnosed
T
77
Apert cosidered the most common type ?
in Saethre-Chotzen estimated birth prevalence of 1:25,000 to 1:50,000 makes this the most common of the syndromic craniosynostoses
78
What are the facial findings in Saethre-Chotzen syndrome
include facial
asymmetry, hypertelorism, eyelid ptosis, lacrimal anomalies, and a
low frontal hairline.
79
Exorbitism is a typical finding in Seather -Chotzen
Exorbitism is not typical of the syndrome
80
What are the hand anomaly in Seather - Chotzen?
The
most common limb anomalies are simple (cutaneous) syndactyly,
brachydactyly, clinodactyly, broad great toes, and radioulnar synostosis.
81
Mild to moderate intellectual disability has been
reported in some cases of Seather syndrom
T
82
patients are typically short in stature and have vertebral anomalies
are also common IN Muenek syndrome.
F common Seather syndrom
83
What mutation occurs in Muenke Syndrome?
In the FGFR3 gene
84
Type of craniosynostosis that occurs in Muenk syndrome?
Unicoronal or bicoronal craniosynostosis
85
The shape of the head in Meunk syndrom?
some carriers do not have
premature suture fusion and instead have either macrocephaly or a
normal head size and shape
86
Som cases of Meunk syndrom condition may be underdiagnosed and underreported;
T
87
genetic testing is advisable in cases of apparently isolated
coronal synostosis
T
88
What are the facial feature of Meunek?
include negative canthal
vector, eyelid ptosis, and midface hypoplasia
89
Hearing loss commonly with crouzen syndrom
Sensorineural hearing
loss is very common and is usually bilateral in Meunek
90
Does the developmental abnormality occur with Muenk?
Developmental delay has
been noted in about two-thirds of patients, although intellectual disability is only present in about one-third
91
What are the limp abnormality in Meunek syndrome?
brachydactyly, clinodactyly, or broad thumbs and great toes, but
radiographic findings of the extremities are dispositive
Thimble-like middle phalanges are often seen, although there is no functional significance
92
calcaneal-tarsal fusion and carpal bone fusion considered pathognomic feature for Murnek syndrom ?
radiographic findings include calcaneal-tarsal fusion
and carpal bone fusion which, although not cardinal signs of the syndrome, are nearly pathognomonic when present.
93
hand deformity in Pfeiffer Syndrome?
broad thumbs and great toes with radial
deviation, and partial soft tissue syndactyly of the hands and feet.
94
All type of Pfeiffer syndrome are autosomal dominant T F
One is autosomal dominant and the others are sporadic mutations owing to to heterogeneity of underlying mutations, which occur in either the FGFRl or FGFR2 genes
95
Types of Pfeiffer syndrome?
Type 1 or classic Ffeiffer syndrome is as described. Intelligence is
typically normal, although intellectual disability has been reported.
Other anomalies are uncommon.
Type 2 Pfeiffer syndrome consists
of a Kleeblattschiidel skull shape with severe exophthalmos, broad
thumbs and great toes, brachydactyly, and syndactyly.
Type 3 Pfeiffer syndrome has the
same findings as type 2 save for the cloverleaf skul
96
Elbow ankyloses or synostosis is found commonly in type 1
Elbow ankyloses or synostosis is found commonly in both type 2 and type 3 but rarely in type 1 Ffeiffer syndrome.
97
Both type 2 and type 3
Ffeiffer syndrome result in mortality in adult in the
vast majority of cases.
F Both type 2 and type 3
Ffeiffer syndrome result in mortality in infancy or childhood in the
vast majority of cases.
98
The brain triples in size during the second year of life
and continues to grow throughout childhood
F The brain triples in size during the first year of life
and continues to grow throughout childhood
99
How much the percentage of patients with craniosynostosis developed in crease intracranial pressure ?
Direct recordings have
demonstrated that about one-third of children with craniosynostosis have increased ICP; another third have borderline pressures,
100
papilledema on fundoscopic examination is may not always be present in case of increase Intracranial pressure
T
101
Radiological finding in increased intracranial pressure?
Radiographic findings such as thumb-printing and
the copper-beaten appearance of the skull demonstrates increased ICP but do not appear until later in the development
102
Bicoronal synostosis, the most common form
in the syndromes
T
103
Fronto-orbital advancement and midface advancement preserving vision techniques
T
104
For mild or moderate cranial vault restrictions, such as in single suture synostosis, an posterior vault correction is performed at 6 months
F For mild or moderate cranial vault restrictions, such as in single suture synostosis, an anterior vault correction is performed at 6 months
105
In more severe conditions, such as syndromic craniosynostoses, can perform posterior fossa expansion as the first intervention between 3 and 6 months of age
T
106
At wich age we can perform FOA?
FOA at around age 1 year and midface procedures on an as-needed basis
107
What is the main stay treatment of craniosynostosis?
Fronto-orbital advancement (FOA) has been a mainstay of treatment
for craniosynostosis
108
Fronto-Orbital Advancement the initial treatment of choice for cranial vault expansion in bicoronal craniosynostosis
T
109
the gain in intracranial volume with FOA more than posterior approach
F less gain in intracranial volume than with posterior
procedures, FOA simultaneously provides anterior-posterior expansion and attempts to reproduce the normal forehead appearance
110
At wich age shaping of the mid-face need to do ?
reshaping of the midface.
This is typically done in childhood, usually between the ages of 5 and
8 years,
111
there is essentially no further vertical maxillary growth following midface osteotomy
T
112
Monobloc move the midface in a primarily a horizontal plane
F Monobloc and Le Fort III operations move the
midface in a primarily anterior-posterior plane; facial bipartition controls horizontal and vertical orbital position as well as midface width
113
Monobloc correct exorbitism, midface retrusion, and forehead retrusion and offer a small increase in intracranial volume.
T
114
Complications of Monoblock surgery?
meningitis, persistent cerebrospinal fluid (CSF)
leaks, and nasocranial fistulae
115
In monoblock In skeletally immature patients, overcorrection of the zygoma is critical
F In skeletally immature patients, overcorrection of the malar
eminences is critical
116
Monobloc surgery can be used to treat the dish face deformity
Facial Bipartition used to manage dish face deformity is seen in syndromic craniosynostoses, particularly Apert syndrome
117
Bipartition with monobloc advancement results in differential
movement of the central and lateral face, with greater advancement
centrally
T
118
Bipartition results in malocclusion due to expansion of the maxilla
T
119
with Saethre-Chotzen syndrome often have syndactyly ofthe second and third digits, involvement ofthe fourth digit and complete
syndactyly are less common
T
120
What is the charge syndrom ?
CHARGE syndrome, a craniofacial syndrome that is not typically associated
with craniosynostosis. Findings in CHARGE syndrome include
coloboma, heart defects, choanal atresia, growth retardation, genital anomalies, and ear anomalies
121
What is the stickler syndrome?
Collagen type II alpha I chain mutations are associated with Stickler syndrome. In addition to hypoplasia of the
midface and Pierre Robin sequence, Stickler syndrome is associated with hearing loss, joint problems, and eye abnormalities.
122
FOA is not always required after posterior fossa distraction .
FOA is not always required after posterior fossa distraction owing to spontaneous correction
123
FOA is a more invasive surgery than
posterior fossa distraction with both longer operative times and
higher rates of blood transfusion
T
124
Monoblock does not permit differential correction of frontal and midface deficiencies.
T
125
Maternal smoking and exposure to nitrosatable drugs in utero have been associated with certain forms of craniosynostosis.
T
126
The development of craniosynostosis has been associated with a paternal occupation of mechanic or repairman and in the agriculture and forestry sector
T
127
The anterior restriction in growth is translated to the cranial base through the greater wing of the sphenoid, resulting in the harlequin orbital
T
128
If the patient presents early (2-3 months of age), it may be possible to perform distraction osteogenesis for this deformity in coronal synostosis
T
129
he most widely accepted treatment of trigonocephaly is a bilateral FOA.
T
130
The surgical treatment oflambdoid craniosynostosis is traditionally a posterior vault remodeling procedure
T
131
Recent increasing in the prevalence of which suture?
Metopic
