Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

FESSH exam 2022 > Children > Flashcards

Children Flashcards

1
Q

Neurofibromatosis typ 1 characteristics

A

Von Recklinghousen

NF1 gene 50% spontanous, dominant pattern

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q
A

Clinodactyly of the thumb

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Web deepening double oppositionen z plast

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Standard four-flap Z-plasty for thumb web

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q
A

Apert syndrome

FGFR2 mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Acrosyndactyli

A

Syndacryli with febestrations prox to distal fusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q
A

Polanski syndrome

Disruption of subclavian artery blood flow in embryo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q
A

Ulnar polydactyly
Left syndactyly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q
A

Postaxial polydactyly in chondroectodermal dysplasia (Ellis van Creveld syndrome) with characteristic nail dysplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q
A

Postaxial (ulnar) polydactyly
Type B

(Type A = well developed digit)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q
A

Grebe condrodysplasia and severe brachydactylia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q
A

Familial synpolydactyly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q
A

Central polydactyly - all finger full motion and function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q
A

Mirror hand

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Most common in mirror hand?:

2 radius or 2 ulnas

A

2 ulnas

Type 1 (of 5)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Brachydactyly

A

Short fingers

Can be generalized or on P1,P2,P3,MC…

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q
A

Central hand deficiency - cleft hand

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q
A

Constriction ring syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q
A

Symbrachydactyly

Fused short fingers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Classification of symbrachydactyly

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q
A

Clinodactyly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Treatment of Clinodactyly caused by a delta phalanx

A

Excision of malformed phalanx
Ligament reconstruction
Arthrodesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

4 types of Macrodactyly

A

1 lipofibromatosis
2 neurofibromatosis
3 digital hyperostosis - bone/joints
4 hemihypertrophy - rare - all digits

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q
A

Macrodactyly with lipofibromatous of ulnar digital nerve

Most common type (próx nerve involvement) often 1 digit or nerve distribution

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q
A

Macrodactyly with syndactyly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q
A

Hyperostotic macrodactyly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Most reliable method for stopping growth in Macrodactyly

A

Epiphysiodesis

(Other: digital nerve stripping)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Tsuge method for reducing length on a macrodactylous finger

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

God salvage for macrodacytly

A

Ray amputation

Consider early

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q
A

Type II thumb hypoplasia

(Absense intrinsic thenar musles, first space narrowing, UCL insufficiency)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Type IV thumb hypoplasia

A

Floating thumb

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Type 3 thumb hypoplasia

A

Type 2 plus extrinsic muscle abnormalities
Skeletal defisiency
3a: stable - reconstructable
3b: unstable CMC1 - pollicisation/toe to hand

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Syndromes associated with radial deficiency?

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Classification of thumb duplication

A

Wassel

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q
A

Wassel IV : duplicated thumb (p1 and p2)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q
A

Assymetric typ 2 (Wassel) duplication, dominant side is determinerat prior surgery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Wassel 2 surgery Bilhaut-Cloquet procedure

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Wassel 2 surgery of assymetric thumb

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Wassel 4 skin insicion

A

If ni dominant component save the ulnar for good UCL function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

2 types of 3 phalangeal thumb

A
  1. Relatively normal-appearing thumb
    -> fusion of one bad joint
  2. 5 fingered hand (in plane of fingers)
    -> pollicisation

Genetic counseling (autosomal dominant pattern)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Trigger thumb etiology

A

Not congenital
Acquired of thight A1 big FPL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Indication for surgery trigger thumb

A

Stage IV = locked in flexión and >1y

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

When conservative treatment of trigger thumb for

A

<1 y age

Obs/splinting 50-70% success 48m time

Can wait up to 3y age with no contracture

Type 1-3
1 only Notta nodule
2 Triggering
3 Actively locked but not passive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Trigger finger surgery

A

A1 release
And FDS slip excision. Open A3 also and cut from there.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Multiple trigger finger think of

A

Inflammatory arthritis
Juvenile diabetes
Mucopolysaccharide disorders

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q
A

Clasped thumb
- no EPB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Clasped thumb types

A
  1. Supple and bad/no extensors
  2. Complex with joint involvement
  3. With syndrome ex Arthrogryposis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q
A

Radial longitudinal deficiency

(Not radial club hand)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

VACTERL

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Holt-Oran syndrome

A

“Hand heart syndrome”
RLD + ASD/VSD septal defects

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

TAR

A

Trombocytopenic Absent Radius

Recessive RBM8A gene

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Fanconi anemia

A

Autosomal recessive

Peculiar face
Need bone marrow transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

RLD type 4

A

All types hypoplastic or absent thumb
N. Only thumb
0. carpus
1. Radius 2mm shorter than ulna
2. Próx radius hypoplastic
3. No distal radius physis
4. Absent ulna radius

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Surgery for RLD

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Treatment timing RLD

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

RLD

Lamb method

Buck-Gramcko method

A

Lamb almost like arthrodesis

In dynamic the muscles seldom possible to transposable

Full release of soft soft tissues

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Treatment RLD type III-IV

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

NF2

A

Vestibular schwannoma
Meningeoma..

Less common than NF1 (von Recklinghousen)

50% spontanous mutation
Dominant pattern, NF2 gene

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Whats most important for hand function in RLD

A

Conditioins of fingers not radial deviation
Complex syndrome
Variable combinations of hand malformations

62
Q

How preserve forearm growth before wrist positioning

A

Soft tissue distraction

63
Q
A

Ulnar deficiency only in hand

64
Q
A

Ulnar deficiency and classic hand on flank position

65
Q

Is ulnar deficiency associated with toher systemic conditions

A

No
But association with other musculoskeletal abnormalities (syndactuli, lower extremity, scolios, phocomelia)
Sporadic cases

RLD is associated with many

66
Q

Phocomelia

A

“Seal extremities”

if from drugs “Thalidomide syndrome”

67
Q

Surgery in ulnar deficiency

A

And treatment for possible thumb deficiency like thumb hypoplasia

68
Q

Madulung deformity causes

A

Mainly unknown; maybe:
- Vickers ligament
- injury to radius growth plate, in ulnar part
- Leri-Weill dyschondrosteosis (dominant pattern, SHOX-gene)
- Repeated loading

69
Q

Treatment Madelung deformity in skeletall immature

A

If painless: no treatment but consider physiolys if skeletally immature
Pain: Physiolys + Vickers lig release (often pain too late in adolecense - combine ulnar epiphysiodesis + dome osteotomy radius)

70
Q

Treatment Madelung deformity in skeletall mature

A
  1. Lig resection + radius dome osteomy
  2. radial wedge ostetomy + ulnar shortening
  3. radial opening wedge ostetomy
  4. radial osteotomy and distal ulnar resection
  5. radial ostetomy and Suvé-Kandju procedures
  6. 3D reconstruction
71
Q
A

Transverse deficiency

72
Q

Congenital radial head dislocation vs traumatic

A

In congenital:
dome shaped radial headwith no central concavity, elongated and narrow in posterior dislocation. Often bilateral and other anomalies is common. Shortened ulna with palmar bow with anterior dislocation och dorsal bow with posterior. Hypoplastic och absent capitellum.

73
Q

PRUS

A

Proximal radioulnar synostosis

Resection unsuccessfull
+interposition free vasc fascial flap not much

Derotation osteotomy

74
Q
A

Congenital pseudo arthritis of radius and ulna - neurofibromatosis

Portion of ulna replaced by fibrous tissue
Resection- Vasc fibula graft

75
Q

MHE

A

Multiple hereditary exostosis

Cartilaginous exostosis (osteochorndromas) grow fr physiatrist of long bones and pelvis, scapula, ribs, vertebrae

Malignant transformation uncommon

76
Q

Artrogryposis

A

Congenital disorder with 2 or more stiff joints

77
Q

Common hand surgeries in Artrogryposis

A

Humerus external rotation osteotomy
Elbow relese
LD dorsi for biceps
Carpal wedge osteotomy (for flexion deforimty not wrist arthrodesis
Camptodactyly release
Thumb reorientation osteotomy

78
Q

Salter-Harris 3

A
79
Q

Salter-Harris 3

A
80
Q

When is the formation of the limb bud?
The differentiation?
Growth?

A
81
Q

What initiates limb bud growth

A

HOS, TBOX genes

82
Q

What signalling centre and protien is responsible for:
1. Proximal/distal growth?
2.Anterior(ulnar)/Posterior (radial)?
3. Dorso/ventral?

A
  1. AER (Apical ectodermal ridge, prox), FGF (fibrobl GF, distalises))
  2. ZPA (Zone of Polarazing activity, ulnar), SHH (Sonic Hedgehog, ulnarises)
  3. Dorsal ectoderm, WnT (dorsalises)
83
Q

Resonsible transcription factors for each signalling centre/responsible proteins?

A

AER/FGF - HOXA10,11,13, TBX4,5
ZPA/SHH - HOXD10-13 GLI3
Wnt - LMX1, EN1

84
Q

Experiments done to understand each signalling centre/responsible proteins experiments.

A

Truncated limb (-) ectopic limb (+) AER/FGF - HOXA10,11,13, TBX4,5

Duplication (+), loss of digit and ulnar (-) ZPA/SHH - HOXD10-13 GLI3

Biventral (-) Bidorsal mice (+) Wnt - LMX1, EN1

85
Q

OMT classification 4 parts

A
  1. Malformation
  2. Deformation
  3. Dysplasia
  4. Syndromes
86
Q

OMT difference from previous Swnason classification

A

1-3 is the same but grouped under Malformation.

Constriction band is nr VI but in OMT nr 2 Deformation.

Over/undergrowth IV-V is Dysplasia

87
Q

Makrodaktyli mutation/Pathway?

A
88
Q

Responsible protein for syndactyly (controlled cell apoptosis)

A

BMP

89
Q

Apert syndrome mutation?

A

FGFR2 receptor -> upregulation of FGF -> overwhelmses BMP ->syndactyly

90
Q

Synpolydactyly gene

A

HOXD13 , autosomal dominant

91
Q

Synpolydactyly with cranial deformities

A

Cephalosynpolydatyly HOXD13, autosomalt dominant

92
Q

Where does the thumb come from?

A
93
Q

What elese is deformed in thumb hypoplasia?

A
94
Q

Effects of ectopic sonic hedgehog

A
95
Q

How do thumb variations happen?

A
96
Q

When to contact a geneticist?

A
97
Q

When to think it´s a syndrome?

A
98
Q

4 ex of Malformations OMT 1A (entrie upper limb) - and what axis are mentioned

A
99
Q

When are fingers separated?

A
100
Q

3 ex of Malformations OMT 1B (Axis of formation/Handplate) type 4 unspecific axis

A
101
Q

3 ex of Malformations OMT 1B (Axis of formation/Handplate) type 4 unspecific axis

A
102
Q

2 types of Hypertrophic Dysplasia and 1 type of tumerous Dysplasia?

A
103
Q

One type of deformation OMT 2

A
104
Q

5 mist common syndactyly types?

A
105
Q

Syndrome syndactyly

A

Poland 1:30.000 (sporadic not true genetic syndrome, 6th week pregnancy vascular problem)
Apert 1:100.000
Grieg cephalopolysyndactyly
Oculodentodigital syndrome
Timothy syndrome (Lqt heart)

106
Q

5 types of syndactyly

A
107
Q

Modern technique for syndactyly release

A

Much larger dorsal flap

108
Q

Why full thickness skin graft in syndactyly release

A

Prevent web creep if tight

109
Q

Web anatomy

A
110
Q

Nail fold reconstruction in syndactyly

Sometimes after splitting and full thickness stage one

A

Before fingers are separated
Buck Gramko

111
Q

Incomplete syndactyly surgery

A
112
Q

3 types of Apert syndrome

A
113
Q

Acrosyndactyly

A

Fusion of distal part of digit in the presence of a formed web space proximal to this fusion

Often constriction ring syndrome

114
Q

Are trigger digits congenital

A

No

115
Q

Difference between trigger thumb and thumb in palm

A

IPj vs MCPj

116
Q

Trigger thumb history

A

100% spontaneous
Weak association with trauma

117
Q

Trigger thumb classification

A
118
Q

What is Nottas nodule

A

Thickening of FPL
Differentiate from sésamo ida that doesn’t move

119
Q

Management of trigger thumb

A

2-5y
grade 1 not painful disharge
Painful observe/splint 6m

Grade 2-3 consider surgery

120
Q

Trigger finger what to think?

A

Very uncommon 1/10 of trigger thumb

MUST consider
Mucopokysaccaroidosis
Hurlers disease
Juvenile RA

More room for observation

121
Q

Trigger finger release

A

Much more extensive than thumb
Anatomical variations common

Brunner
A1 if not enough
A3 adhesion if not enough
Excise one slip of FDS

122
Q

Camptodactyly

A

Description not diagnosis
Bent finger
Congenital or traumatic

123
Q

Camptodactyly history

A
124
Q

Camptodactyly examination

A

Bouvier test testar PIP kontraktur I’m flex MCP
FDS kontraktur?…
RTG
Tenting of skin Jeff not tenting after trauma

125
Q

Camptodactyly treatment

A

Splint until skeletal maturity

No need surgery unless >60 degrees

Surgery
Remove all fibrous tissues and inspect intrinsically. If lumbrical flex pip when tighten respect it

Always cut FDS and suture on to the other lengthened

126
Q

Clinodactyly management

A

Many syndromes!
X-ray exclude trauma (osteotomy)
And confirm cause of deformity
(Delta phalanx, trapezoidal improve with age, irregular physis)

127
Q

Surgery of Delta phalanx

A

Piece of fat for forearm put inside sutures after break up the fysis

Be sure it is not irregular physis (UL or MRI) treated with corrective osteotomy

128
Q

Wassel classification

A
129
Q

Classification of hypoplastic thumb

A

Blauth

130
Q

Ulnar polydactyly classification

A
131
Q

IIIA Thumb hypoplasia treatment?
II thumb hypoplasia treatment

A

FDS opponensplasty prefered if available. Use a distal FCU loop. Drill hole trough MC head and use rest to reconstruct UCL.

132
Q

Pollex abductus

A

Connection between FPL and EPL in thumb hypoplasia
Makes secondary lax UCL

133
Q

what does lateral plate mesoderm form?

A

Bone, cartilage and thendon

Somatic mesoderm forms muscle, nerve and vascular elements

134
Q

Classification of RLD

A

N: hypoplastic thumb or absent
0: Carpus involvement
1: >2cm shorter radius
2: whole radius hypoplastic
3: absent distal physis radius
4: absent radius

135
Q

At what age centralization?

A

Before 1y of age

Serial casting and/or soft tissue distraction

transfer abberant wrist extensors, thumb hypoplasia at second stage

136
Q

How much ulna lengthening in RLD

A

30-50%

Rarely indicated, risk damage growth - need repeted lengthening

137
Q

Risk of centralization in RLD?

A

Stiff and straight of flexible and deviated
Recurrence

Some have abandoned in favor of soft tissue reconstruction: bilobed skin flap + musculotendinous release + tendon transfers

138
Q

What the hereditery pattern of ulnar deficiency

A

Sporadic

And not associated with systemic conditions like radial deficiency

139
Q

Ulnar deficiency presentation
Surgical options

A

Whole upper extremity hypoplastic
Often radiohumer synostosis
Ulna absent or partial + anlage
Carpus alway affected; 90% missing digits, 30% syndactyly, 70 thumb abnormalities - surgery to increase function (syndactyly, first web deepening, opponensplasty, pollicization)

Excision anlage if progressive deviation

140
Q

Madelung causes

A

Disturbance of distal radial growth in ulnar palmar side. Often bilat.

Vickers ligament
Leri-Weill dyschondrosteosis - SHOX mutation (short-status homeobox-containging gene (dominant 50% penetrance)
Repetetiv loading in growing child
Lesion in physis

141
Q

Epiphysiolysis vs epiphysiodesis in Madelung

A

lys - Open up closes bone physis to promote growth in radius

des - close physis to stop growth of ulna when close to skeletalat maturity

142
Q

Madelung surgery

A

Skeletal immature - often no pain - surgery if progression
Epiphysiolys radius + vickers excision (if close to maturity epiphysiodes of ulna also)

Mature - more pain problems
Dom osteotomy + lig resection, radial closing wege + ulnar shortening, radial opening wedge, radial osteotomy + Darrach, Radial osteotomy + Sauvé-Kapandji

143
Q

How to distinguish traumatic from congenital dislocation of the radial head

A

Radiography of other side - often bilateral
MRI show intracapsular radial head in congenital cases but protrusion through capsule in trauma
MRI also show convex instead of concave head + hypoplastic capitellum

No surgery needed

Association congen radioulnar synostosis + syndromes - (only excision)

144
Q

PRUS

A

Proximal Radioulnas synostosis

Surgery if extreme pro/sup, corrective osteotomy

145
Q

Congenital pseudoarthrosis of the ulna or radius is associated with

A

Neurofibromatosis

excsision + free vascular fibular graft

146
Q

MHE

A

Multiple Hereditery Exostosis
Autosomal dominant high penetrance
Osteochondromas (cartilaginous exostoses)

Compare Olliers and Mafuccis that have multiple enchondromas.

147
Q

Where are the physises of the hand

A

Phalanges, tumb and MC1 proximal
Other Metacarpals distal

148
Q

Fractors affecting bone remodelling after fractures

A

+ same axis as adjacent joint motion
+ proximity to physis
+ young

  • Rotational
  • radioulnar

Preserve physeal growth

149
Q

Wrinkle test

A

Warm water immersion for several minutes - if skin wrinkling appears implies innervation (no nerve injury)

150
Q

Pediatrik Monteggia fx classification and treatment

Galeazzi treatment

A

Galeazzi is radius + DRUj - in kids surgery seldom needed

FESSH exam 2022 (12 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions