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Projections > MSK - Congenital, Mix of GI/GU/Neuro/other MSK > Flashcards

MSK - Congenital, Mix of GI/GU/Neuro/other MSK Flashcards

1
Q

What condition is 10x more likely to get DDH

A

Infantile idiopathic soliosis

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2
Q

Perkins line is drawn

A

Perpendicular to the lateral acetabular rim

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3
Q

What part of the aorta affected in Marfans

A

Ascending

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4
Q

What valve is insufficient in Marfans

A

Aortic and mitral

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5
Q

5th digit change in Marfans

A

Flexion deformity of the finger

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6
Q

Homocystinuria vs Marfans IQ retardation

A

Homocystinuria

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7
Q

Def dolicostenomelia

A

Narrow body habitus/Long limbs

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8
Q

What kills pts with homocysteinuria

A

spontaneous venous and arterial thromboses

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9
Q

Malar rash is seen in homocysteinuria or Marfan

A

Homocysteinuria

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10
Q

Change in bone density in homocysteinuria or Marfan

A

Homocysteinuria has osteopenia, normal in marfan

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11
Q

3 clinical findings of Ehlers-Danlos

A
  1. Joint hypermobility 2. skin bruising and elasticity 3. vascular abN that can be life threatening
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12
Q

2 rad findings in Type 4 Ehlers-Danlos

A

Occipital horns and deformed clavicles

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13
Q

4 clinical criteria for OI

A

Osteoporosis, blue sclera, dentinogenesis imperfecta and premature otosclerosis

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14
Q

Severe OI forms die from

A

Respiratory complications and/or intracerebral hemorrhage

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15
Q

type of hearing loss in OI

A

Nerve conduction seen in 10%

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16
Q

Severe GI bleeding in Child

A

Pseudoxanthoma elasticum

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17
Q

Multiple Epiphyseal dysplasia AKA

A

Fairbank disease

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18
Q

Fairbank dz/Multiple epiphyseal dysplasia is found

A

Bi/Sym at hips, knees, wrists and ankles

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19
Q

Fairbank dz/Multiple epiphyseal dysplasia spine changes look like

A

Scheurmann dz

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20
Q

What is Conradi-Hunermann syndrome

A

type of chondrodysplasia punctata

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21
Q

Zellwegers cerebrohepatorenal syndrome has stippled calcifications where?

A

Patella

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22
Q

Dysplasia epiphysealis capitis AKA

A

Meyer’s dysplasia

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23
Q

MC digits involved in macrodystrophy lipomatosa

A

2/3rd in UE or LE

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24
Q

MC nerve involved in macrodystrophy lipomatosa

A

Medial followed by plantar

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25
Q

Def nevus flammeus

A

Port-wine cutaneous hemangiomas

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26
Q

Achondroplasia bone shapes in spine and pelvis

A

Bullet-nosed, flattened vertebral bodies, squared ilia with small sacrosciativ notches

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27
Q

rad appearance in Jeune’s syndrome

A

narrow thorax, short tubular bones with coned epiphysis, High handlebar appearance

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28
Q

Chondroectodermal dysplasia AKA

A

Ellis-Van-Creveld

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29
Q

Chondroectodermal dysplasia/Ellis-Van-Creveld general appearance is

A

Ectodermal dysplasia, polydactyly, congenital heart disease

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30
Q

Spondyloepiphyseal dysplasia tarda’s classic vertebral body appearance

A

Heaped/hump shaped vertebra along the middle/posterior aspect

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31
Q

Clubfoot is AKA and how often are they bilateral

A

Talipes Equinovarus, bilateral 50%

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32
Q

Clubfoot/Talipes Equinovarus components are (4 parts)

A
  1. Hindfoot varus 2. Equinus heel deformity 3.adduction and 4 talonavicular subluxation
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33
Q

Congenital vertical talus deformity

A

Dorsal dislocation of navicular (Rockerbottom foot)

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34
Q

OI Type 1

A

Tarda; 90%, Blue sclera, lax ligaments, dentin dysplasia, otosclerosis, osseous abnormalities

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35
Q

OI Type 2

A

Congenita; 10% die in utero/neonate

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36
Q

OI Type 3

A

Fx at birth, progressive limb deformity, normal sclera and hearing

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37
Q

OI type 4

A

Fragile bones, discolored teeth, Normal sclera and hearing

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38
Q

Achondroplasia buzz words

A

Champagne pelvis, ping-pong paddle pelvis

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39
Q

Def Crouzon syndrome

A

Craniosynostosis, exopthalmos, midface retrusion

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40
Q

Spinal fusion level in Crouzon syndrome

A

C2/3

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41
Q

Spinal fusion level in Apert syndrome

A

C5/6

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42
Q

Apert syndrome findings

A

Acrocephalosyndactyly; sutural closure, midface hypoplasia, sym syndactyly of hands/feet

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43
Q

Mandibulofacial dysostosis AKA

A

Treacher-Collins syndrome

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44
Q

Rad findings of Tracher-Collins/Mandibulofacial dysostosis

A

Egg-shaped orbits, hypoplasia of zygomatic arch, maxill sinus, ext aud canal may be absent

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45
Q

Trisomy 18 is known as and clinical features are

A

Edwards syndrome, cardiac abN, omphacele and renal abN

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46
Q

Hypoplasia of the 1st metacarpal is seen in trisomy

A

18

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47
Q

GI abN in Trisomy 21 (Down)

A

Duodenal atresia, Hirschsprung dz, tracheoesophageal and anorectal abN

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48
Q

Shortened 4th Metacarpal with delayed epiphyseal fusion is

A

Turner Syndrome

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49
Q

MC location of diastatomyelia is and other 2 associations

A

L1-L4, congenital scoliosis in 15% and 80% with skin changes

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50
Q

Causes of tethered cord

A

Intraspinal dermoid, lipomas, congenital bands and myelomeningoceles

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51
Q

Level of the conus at 2 months

A

L2-3

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52
Q

Associated with infantile idiopathic scoliosis

A

Plagiocephaly (flat-head)

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53
Q

MC type of idiopathic scoliosis in US is

A

Adolescent idiopathic scoliosis

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54
Q

What type of scoliosis shap is seen in neuromuscular scoliosis

A

Long C-shaped

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55
Q

MC cause of neuromuscular scoliosis is

A

Cerebral Palsy

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56
Q

What is TAR syndrome

A

Thrombocytopenia with absent radius

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57
Q

Ass’d with syndactyly

A

Poland syndrome

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58
Q

What parts are fused with talocalcneal coalition

A

Middle facet btw talus and sustentaculum tali of calcaneus

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59
Q

Foot deformity in neuromuscular disorder

A

Cavus foot deformity

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60
Q

Osteodysplasty is AKA

A

Melnick-Needle syndrome

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61
Q

Type of osteopetrosis with early death

A

Precocious

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62
Q

Alber-Schonberge dz is AKA

A

Delayed type osteopetrosis

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63
Q

MPS VII, Tubular acidosis type of osteopetrosis

A

Sly disease

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64
Q

Congenital block vertebra are found where in pyknodysostosis

A

Upper cervical spine

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65
Q

Diaphyseal dysplasia is AKA

A

Comurati-Engelmann dz

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66
Q

Engelmann dz rad findings

A

Bi/Sym cortical thickening with narrowed medullary spaces, spares epiphysis

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67
Q

3 types of endosteal hyperostosis

A

Van Buchem, Sclerosteosis and Worths

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68
Q

Cranial changes in frontometaphyseal dysplasia

A

prominent supraorbital ridge, absent frontal sinus, calvarial hyperostosis

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69
Q

Which sinus is obliterated in craniometaphyseal dysplasia

A

Paranasal

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70
Q

MC craniosynostosis

A

Dolichocephaly

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71
Q

Def Brachycephaly

A

Bilateral corontal sutural closure leading to small AP diameter

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72
Q

What is almost always present in Ellis-Van-Creveld/Chondroectodermal dysplasia

A

Polydactyly (post-axial)

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73
Q

Central vertebral beaking

A

Morquio (MPS IV)

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74
Q

Inferior vertebral beaking

A

Hurler (MPS I-H) and Hunter (MPS II)

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75
Q

Def Still disease

A

JRA/JCA +Lymphadenopathy + Splenomegaly

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76
Q

MC tumor in newborn

A

Sacrococcygeal teratoma

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77
Q

Closure of Metopic suture is

A

Trigonocephaly

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78
Q

Harlequin eye is seen in what sutural closure

A

Coronal unilateral

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79
Q

Cloverleaf or Kleeblattschadel is seen with which sutural closure

A

All except metopic and squamosal

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80
Q

% of scoliosis are what type

A

idiopathic

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81
Q

3 findings in Gardner syndrome

A

Osteomas, Colonic polyps and ST tumors

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82
Q

Osteoma age and location

A

30-50 YO, Frontal/ethmoid bones

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83
Q

2 MC locations of osteoid osteoma

A
  1. femoral diaphysis

2. Tibia

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84
Q

Which bone layer are osteoid osteomas found

A

Periosteal

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85
Q

Can bone islands occur in the skull

A

No

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86
Q

Osteoid osteomas in long bones %

A

70%

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87
Q

MC location of osteoblastoma

A

Flat bones and spine

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88
Q

MC loc for ossifying fibroma

A

Ant tibia (90%)

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89
Q

Ossifying fibroma is AKA

A

Osteofibrous dysplasia

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90
Q

MR findings of telangiectatic osteosarcoma

A

T1 high with fluid-fluid level

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91
Q

Osteosarcoma with cleavage plane

A

Parosteal

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92
Q

Skip lesions are found in ewing vs osteosarcoma

A

Ewing

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93
Q

How common is osteosarcoma mets

A

60%

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94
Q

Osseous expansion of enchondroma is known as

A

Enchondroma protuberans

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95
Q

Chondrosarc>Enchondroma is over what size

A

5cm

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96
Q

MC location of periosteal chondrosarcoma

A

Humerus and femur 70%, 25% feet

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97
Q

Chondroblastoma age range

A

5-25 YO

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98
Q

MR appearance of chondroblastoma

A

low T1, high T2, lots of periosteal reaction

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99
Q

Type of cartilage in enchondroma

A

Hyaline

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100
Q

Radial or ulnar sided enchondroma formation

A

ulnar

101
Q

+C pattern of Enchondroma vs Chrondrosarcoma

A

Enchondroma = peripheral, Chondrosarcoma is diffuse

102
Q

MC location of Ollier

A

Femur and tibia

103
Q

MC location of Maffucci

A

metacarpal and phalanges

104
Q

MC location of chondromyxoid fibroma

A

Tibia/femur - 55%

105
Q

Codman tumor is AKA

A

Chondroblastoma

106
Q

In feet chondroblastoma is found where

A

talus and calcaneus

107
Q

MC benign tumor of the patella

A

GCT (MC) and chondroblastoma (2nd MC)

108
Q

least common benign cartilage neoplasm is

A

chondromyxoid fibroma

109
Q

Chondromyxoid fibroma is found in what age

A

<30 or 50-70

110
Q

MC location of Trevor disease is

A

Talus, followed by distal femur> prox tibia

111
Q

MC location of subungual exostosis

A

Great toe (70-80%)

112
Q

Def Turret exostosis

A

Broad based protuberance on dorsal surface of a finger folloing trauma, believed to be ossified subperiosteal hematoma

113
Q

HME is AKA

A

Diaphyseal aclasia

114
Q

Medial or lateral epiphysis is involved in Trevors

A

Medial

115
Q

Def Metachondromatosis

A

Multiple osteochondromas and enchondromas

116
Q

MC location of conventional chondrosarcoma

A

Long tubular bone metaphysis, common in femu, innominate and ribs

117
Q

DDx feature between juxtacortical chondrosarcoma and juxtacortical chondroma

A

Chondrosarcoma has an aggressive periosteal pattern

118
Q

MC loc of clear cell chondrosarcoma

A

Epiphyseal, femur and humerus

119
Q

MC location of fibroxantoma

A

Tibia followed by femur

120
Q

Def jaffe-Campanacci syndrome

A

Multiple NOF, with extraskeletal congenital anomalies of cafe au lait spots, mental retardation

121
Q

MC loc and age of desmoplastic fibroma

A

Mandible, central metaphysis of femur/hum/tib and found in <30 YO

122
Q

ST involvement of fibrosarcoma

A

Large involvement with central necrosis=calcifications/sequestrum

123
Q

MC location of GCT in the spine

A

Sacrum

124
Q

GCT can be found with which other dzs

A

Pagets or HPT

125
Q

Def Goltz syndrome

A

Focal dermal hypoplasia, ocular defects, skeletal anomalies

126
Q

MC loc for a intraosseous ganglion

A

Medial malleoli, carpal bones, periarticular hip

127
Q

MC malignant ST tumor in adults

A

MFH

128
Q

MFH loc in skeletal

A

75% ends of long bones, femoral metaphysis in particular

129
Q

Age of MFH

A

40-60

130
Q

MC loc of intraosseous lipoma

A

Fibula>fem>tib>calc

131
Q

MC loc of liposclerosing myxofibroma

A

80% in intertrochanteric

132
Q

Desc cystic angiomatosis

A

widespread angiomatosis/hemangiomatosis of bone with visceral involvement (65%)

133
Q

MC loc of spinal hemangioma

A

Thoracic spine

134
Q

MC loc of skull hemangioma

A

Frontal bone

135
Q

Histologic hemangioma type in skull vs axial skel

A

Skull = Cavernous, Axial = capillary

136
Q

Angioglomoid tumor is AKA

A

Glomus tumor

137
Q

MC loc of glomus tumor and age is

A

Extraosseous, fingertips/nails bed in 30-50 YO

138
Q

Origin of a glomus tumor

A

neuromyoarterial glomus

139
Q

MC bone tumor to cause Vit D resistance is

A

Hemangiopericytoma (can also be seen in NOF and GCT)

140
Q

Angiosarcomas can be found at sites of what other lesions/dz

A

Infarction, chronic OM, Paget and other neoplasms

141
Q

Loc of angiosarcoma

A

Met/dia of Tib>Fem

142
Q

Synchronous or metachronous angiosarcoma presentation %

A

20-50%

143
Q

MC flat bones in angiosarcoma

A

Pelvis and skull

144
Q

Tissue type in Epidermoid cyst

A

Ectodermal

145
Q

Loc for intraaosseous epidermoid cyst

A

Skull (Frontal), phalanges

146
Q

2nd MC malignant ST neoplasm in adults

A

Liposarcoma

147
Q

Def Stewart-Treves syndrome

A

Bursitits about the Achilles tendon

148
Q

Loc of elastofibroma

A

Shoulder

149
Q

MC fibromatosis

A

Palmer fibromatosis (Duputtren contracture)

150
Q

MC source of mets in kids

A

Neuroblastoma, Ewing, Osteosarcoma, retinoblastoma

151
Q

Solitary mets occurs from

A

Kidney and thyroid

152
Q

Most likely cranial tumor to spread outside the CNS

A

Medulloblastoma

153
Q

MC mets to synovium

A

Breast and lung

154
Q

MC tumor in kids with subarachnoid spread

A

Medulloblastoma and ependymoma

155
Q

Cookie-bite sign is seen in what mets

A

Lung CA

156
Q

MC solid abdominal malignancy in kids

A

Wilms (Nephroblastoma)

157
Q

MC solid intracranial malignancy in kids

A

Neuroblastoma

158
Q

Wermer syndrome is AKA

A

Familial multiple endocrine neoplasia syndrome (MEN1)

159
Q

Sipple syndrome is AKA

A

MEN2

160
Q

Def Beckwith-Wiedemann syndrome

A

Exomphalos, macroglossia, gigantism syndrome, nephroblastoma

161
Q

Def Diabetic cheiroarthropathy

A

Joint contractures of 4/5 seen in DM

162
Q

Friedreichs dz

A

AVN of medial clavicular epiphysis

163
Q

% of Ra patients with nodules

A

35%

164
Q

Ribs ass’d with RA

A

Sup margin of post 3-5th ribs

165
Q

% of pts with psoriatic that have psoriatic arthritis

A

6%

166
Q

Leading cause of psoas abscess

A

Crohns

167
Q

Intestinal lipodystrophy is AKA

A

Whipples dz

168
Q

Syndrome with HLA-B5

A

Behcet disease

169
Q

Def Thiberge Weissenbach syndrome

A

Calcinosis, digital ischemia

170
Q

Schulman syndrome is AKA

A

Eosinophilic fasciitis

171
Q

Schulman syndrome/Eosinophic fasciitis shows

A

Eosinophili, hypergammaglobulinemia with systemic vascular involvement

172
Q

Def Churg-Strauss

A

Asthma, esoinophilia, vasculities and extravascular granulomas it is AKA Allergic granulomatosis, angiitis

173
Q

Henoch-Schonlein triad

A

Purpura, abd pain and arthritis (<20 YO)

174
Q

Normal interpediculate distance in Lumbar spine is

A

20mm

175
Q

Stages of Arachnoiditis

A
  1. Radiculitis 2. Arachnoiditis 3. adhesive arachnoiditis
176
Q

Def Lesch Nyhan syndrome

A

Deficient HPRT, gouty erosions, seen in boys, delayed skeletal maturation, hip deformities

177
Q

MC loc for HADD in wrist

A

FCU

178
Q

Triad of hemochromatosis

A

Cirrhosis, bronze pigmentation and DM

179
Q

Loc of adult cystinosis deposition

A

Cornea

180
Q

% of Barretts esophagus that go malignant

A

10%

181
Q

Gallstones on XR seen %

A

10-15%

182
Q

Def Meigs-Salmon syndrome

A

Serous cyst adenoma with ascites or pleural effusion

183
Q

MC cause of mechanical colon obstruction

A

Primary colon CA

184
Q

MC cause of gastric outlet obstruction

A

Peptic ulcer disease

185
Q

MC cause of spontaneous pneumoperitoneum

A

Perforated duodenal/gastric ulcer

186
Q

Loc of Zenkers

A

Posterior esophagus above the cricoharyngeus mm @ Killians dehissence

187
Q

Plummer Vinson Triad

A

Dysphagia, Esophageal webs, and Fe deficiency

188
Q

MC malignant tumor of pharynx

A

SCC

189
Q

MC diaphragmatic hernia

A

Hiatal - Sliding is MC subtype

190
Q

% of pts with GERD and hiatal hernia

A

90%

191
Q

MC cause of epiglottis

A

GERD

192
Q

MC cause of esophageal ulceration

A

Reflux esophagitis

193
Q

Barretts esophagus degenerates to

A

Adenocarcinoma

194
Q

MC infectious agent of esophagus

A

candida albicans

195
Q

Def Shaggy esophagus

A

Worse form of candida ifnection in IC patients (AIDS)

196
Q

rare location of Crohns

A

Esophagus

197
Q

Manimetric triad of achalasia

A
  1. lack of peristalsis of esophageal body 2. incomplete relaxation and 3. hypertensive resting phase
198
Q

Scleroderma affects the GI tract most where

A

lower 2/3rd of esophagus

199
Q

Gastric ulcer location

A

Posterior antrum/body junction

200
Q

Hamptons line indicates benign or malignant ulcer

A

benign

201
Q

MC type of gastric polyp

A

Hyperplastic (90%)

202
Q

MC extranodal site of NHL

A

Stomach

203
Q

Def Cloverleaf duodenum

A

MM spasm d/t ulcer extending into muscular layer

204
Q

MC complication of peptic ulcer disease is

A

hemorrhage

205
Q

Congenital duodenal condition seen in Downs

A

Congenital webs

206
Q

What bug mimicks Whipples disease

A

Myobacterium avium intercellularis

207
Q

Peutz-Jegher syndrome

A

GI polyps, cutaneous pigmentation. MC to affect small bowel

208
Q

Poor prognosis of Crohns if found where

A

Ilium

209
Q

MC organ affected by Chaga disease and what bug

A

Heart, Trypanosoma Cruzi, reduviid bug bite

210
Q

MC TB location in GIT

A

Ileocecal

211
Q

MC Shigella ulcer location

A

Rectosigmoid

212
Q

MC non GI malignancy ass’d with FAPS

A

GBM

213
Q

MC hamartomatous polyposis syndrome

A

Peutz-Jegher

214
Q

Almost all Peutz-Jegher pts have this tumor

A

Gonadal stromal tumor

215
Q

Only polyposis syndrome to affect the esophagus

A

Cowdens syndrome

216
Q

HCC is AKA

A

Hepatoma

217
Q

Liver tumor ass’d with PVC

A

Angiosarcoma (MC primary malignant mesenchymal tumor)

218
Q

MC cause of hepatic granulomas

A

Sarcoidosis

219
Q

Normal diameter of splenic artery

A

<1cm

220
Q

MC splenic benign tumor

A

Hemangioma

221
Q

MC splenic malignant tumor

A

Lymphoma

222
Q

Iron deposits into spleen from chronic portal HTN

A

Gamna Gandy Bodies

223
Q

What is a sentinal clot

A

Perisplenic clotted blood

224
Q

Def Hartmanns pouch

A

Area of GB proximal to junctional fold (phrygian cap)

225
Q

Def hydrops or courvoissieur GB

A

Distended GB

226
Q

Def Adenomyosis

A

Proliferation of GB mucosa with increased thickness

227
Q

Def Rokitansky-Aschoff sinus

A

Diverticular outpouching in mucosa that forms in adenomyosis

228
Q

MC tumor to spread to GB

A

Melanoma

229
Q

3 things ass’d with Caroli disease

A

Congenital hepatic fibrosis, renal cystic dz in kids and renal tubual ectasia in adults

230
Q

Def medullary sponge kidney

A

Cystic dilation of collecting tubules in kidnets

231
Q

Def primary sclerosing cholangitis

A

idiopathic dz of liver with diffuse inflammatory fibrosis of biliary tree

232
Q

ERCP can falsely elevate what markers

A

Lipase and amylase

233
Q

2 physical examination signs of pancreatitis

A
  1. Grey turner sign (flank echymosis) 2. Cullin sign (periumbilical echymosis)
234
Q

Anomaly that predisposes to biliary tree disease

A

GB agenesis

235
Q

Def Accessory duct of Santorini

A

Ventral duct is secondary duct that failed to fuse with the main dorsal duct. Inserts proximal to the duct of Wirshung

236
Q

Renal tumor in pts with tuberous sclerosis

A

AML

237
Q

MC tumor of renal pelvis

A

TCC

238
Q

Squamous metaplasia of renal pelvis

A

Leukoplakia

239
Q

Renal issues in Sickle cell anemia

A

Renal papillary necrosis with Bi renal enlargement

240
Q

Bladder ears

A

herniation of bladder into Inguinal canal

241
Q

Prune-Belly Syndrome is AKA and Def as

A

Eagle-barrett syndrome, Anterior abdominal agenesis, cryptorchidism and renal system dilation

242
Q

Changes with radiation nephritis

A

6-12 mo post, atrophy, glomerulonephritis, pyelonephritis, malignant HTN

243
Q

% of DM pts with emphysematous cystitis

A

50%

244
Q

MC primary tumor to mets to bladder

A

melanoma

245
Q

MC benign bladder tumor

A

Leiomyoma or fibroepitheloid fibroma

246
Q

MC malignant bladder tumor

A

TCC

247
Q

What tumor is seen in 75-80% of pts with primary aldosteronism

A

adrenal Adenoma

248
Q

Def Hydrocele

A

Fluid between layers of tunica vaginalis

Projections (9 decks)

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