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
2
Q
Perkins line is drawn
A
Perpendicular to the lateral acetabular rim
3
Q
What part of the aorta affected in Marfans
A
Ascending
4
Q
What valve is insufficient in Marfans
A
Aortic and mitral
5
Q
5th digit change in Marfans
A
Flexion deformity of the finger
6
Q
Homocystinuria vs Marfans IQ retardation
A
Homocystinuria
7
Q
Def dolicostenomelia
A
Narrow body habitus/Long limbs
8
Q
What kills pts with homocysteinuria
A
spontaneous venous and arterial thromboses
9
Q
Malar rash is seen in homocysteinuria or Marfan
A
Homocysteinuria
10
Q
Change in bone density in homocysteinuria or Marfan
A
Homocysteinuria has osteopenia, normal in marfan
11
Q
3 clinical findings of Ehlers-Danlos
A
- Joint hypermobility 2. skin bruising and elasticity 3. vascular abN that can be life threatening
12
Q
2 rad findings in Type 4 Ehlers-Danlos
A
Occipital horns and deformed clavicles
13
Q
4 clinical criteria for OI
A
Osteoporosis, blue sclera, dentinogenesis imperfecta and premature otosclerosis
14
Q
Severe OI forms die from
A
Respiratory complications and/or intracerebral hemorrhage
15
Q
type of hearing loss in OI
A
Nerve conduction seen in 10%
16
Q
Severe GI bleeding in Child
A
Pseudoxanthoma elasticum
17
Q
Multiple Epiphyseal dysplasia AKA
A
Fairbank disease
18
Q
Fairbank dz/Multiple epiphyseal dysplasia is found
A
Bi/Sym at hips, knees, wrists and ankles
19
Q
Fairbank dz/Multiple epiphyseal dysplasia spine changes look like
A
Scheurmann dz
20
Q
What is Conradi-Hunermann syndrome
A
type of chondrodysplasia punctata
21
Q
Zellwegers cerebrohepatorenal syndrome has stippled calcifications where?
A
Patella
22
Q
Dysplasia epiphysealis capitis AKA
A
Meyer’s dysplasia
23
Q
MC digits involved in macrodystrophy lipomatosa
A
2/3rd in UE or LE
24
Q
MC nerve involved in macrodystrophy lipomatosa
A
Medial followed by plantar
25
Def nevus flammeus
Port-wine cutaneous hemangiomas
26
Achondroplasia bone shapes in spine and pelvis
Bullet-nosed, flattened vertebral bodies, squared ilia with small sacrosciativ notches
27
rad appearance in Jeune's syndrome
narrow thorax, short tubular bones with coned epiphysis, High handlebar appearance
28
Chondroectodermal dysplasia AKA
Ellis-Van-Creveld
29
Chondroectodermal dysplasia/Ellis-Van-Creveld general appearance is
Ectodermal dysplasia, polydactyly, congenital heart disease
30
Spondyloepiphyseal dysplasia tarda's classic vertebral body appearance
Heaped/hump shaped vertebra along the middle/posterior aspect
31
Clubfoot is AKA and how often are they bilateral
Talipes Equinovarus, bilateral 50%
32
Clubfoot/Talipes Equinovarus components are (4 parts)
1. Hindfoot varus 2. Equinus heel deformity 3.adduction and 4 talonavicular subluxation
33
Congenital vertical talus deformity
Dorsal dislocation of navicular (Rockerbottom foot)
34
OI Type 1
Tarda; 90%, Blue sclera, lax ligaments, dentin dysplasia, otosclerosis, osseous abnormalities
35
OI Type 2
Congenita; 10% die in utero/neonate
36
OI Type 3
Fx at birth, progressive limb deformity, normal sclera and hearing
37
OI type 4
Fragile bones, discolored teeth, Normal sclera and hearing
38
Achondroplasia buzz words
Champagne pelvis, ping-pong paddle pelvis
39
Def Crouzon syndrome
Craniosynostosis, exopthalmos, midface retrusion
40
Spinal fusion level in Crouzon syndrome
C2/3
41
Spinal fusion level in Apert syndrome
C5/6
42
Apert syndrome findings
Acrocephalosyndactyly; sutural closure, midface hypoplasia, sym syndactyly of hands/feet
43
Mandibulofacial dysostosis AKA
Treacher-Collins syndrome
44
Rad findings of Tracher-Collins/Mandibulofacial dysostosis
Egg-shaped orbits, hypoplasia of zygomatic arch, maxill sinus, ext aud canal may be absent
45
Trisomy 18 is known as and clinical features are
Edwards syndrome, cardiac abN, omphacele and renal abN
46
Hypoplasia of the 1st metacarpal is seen in trisomy
18
47
GI abN in Trisomy 21 (Down)
Duodenal atresia, Hirschsprung dz, tracheoesophageal and anorectal abN
48
Shortened 4th Metacarpal with delayed epiphyseal fusion is
Turner Syndrome
49
MC location of diastatomyelia is and other 2 associations
L1-L4, congenital scoliosis in 15% and 80% with skin changes
50
Causes of tethered cord
Intraspinal dermoid, lipomas, congenital bands and myelomeningoceles
51
Level of the conus at 2 months
L2-3
52
Associated with infantile idiopathic scoliosis
Plagiocephaly (flat-head)
53
MC type of idiopathic scoliosis in US is
Adolescent idiopathic scoliosis
54
What type of scoliosis shap is seen in neuromuscular scoliosis
Long C-shaped
55
MC cause of neuromuscular scoliosis is
Cerebral Palsy
56
What is TAR syndrome
Thrombocytopenia with absent radius
57
Ass'd with syndactyly
Poland syndrome
58
What parts are fused with talocalcneal coalition
Middle facet btw talus and sustentaculum tali of calcaneus
59
Foot deformity in neuromuscular disorder
Cavus foot deformity
60
Osteodysplasty is AKA
Melnick-Needle syndrome
61
Type of osteopetrosis with early death
Precocious
62
Alber-Schonberge dz is AKA
Delayed type osteopetrosis
63
MPS VII, Tubular acidosis type of osteopetrosis
Sly disease
64
Congenital block vertebra are found where in pyknodysostosis
Upper cervical spine
65
Diaphyseal dysplasia is AKA
Comurati-Engelmann dz
66
Engelmann dz rad findings
Bi/Sym cortical thickening with narrowed medullary spaces, spares epiphysis
67
3 types of endosteal hyperostosis
Van Buchem, Sclerosteosis and Worths
68
Cranial changes in frontometaphyseal dysplasia
prominent supraorbital ridge, absent frontal sinus, calvarial hyperostosis
69
Which sinus is obliterated in craniometaphyseal dysplasia
Paranasal
70
MC craniosynostosis
Dolichocephaly
71
Def Brachycephaly
Bilateral corontal sutural closure leading to small AP diameter
72
What is almost always present in Ellis-Van-Creveld/Chondroectodermal dysplasia
Polydactyly (post-axial)
73
Central vertebral beaking
Morquio (MPS IV)
74
Inferior vertebral beaking
Hurler (MPS I-H) and Hunter (MPS II)
75
Def Still disease
JRA/JCA +Lymphadenopathy + Splenomegaly
76
MC tumor in newborn
Sacrococcygeal teratoma
77
Closure of Metopic suture is
Trigonocephaly
78
Harlequin eye is seen in what sutural closure
Coronal unilateral
79
Cloverleaf or Kleeblattschadel is seen with which sutural closure
All except metopic and squamosal
80
% of scoliosis are what type
idiopathic
81
3 findings in Gardner syndrome
Osteomas, Colonic polyps and ST tumors
82
Osteoma age and location
30-50 YO, Frontal/ethmoid bones
83
2 MC locations of osteoid osteoma
1. femoral diaphysis
| 2. Tibia
84
Which bone layer are osteoid osteomas found
Periosteal
85
Can bone islands occur in the skull
No
86
Osteoid osteomas in long bones %
70%
87
MC location of osteoblastoma
Flat bones and spine
88
MC loc for ossifying fibroma
Ant tibia (90%)
89
Ossifying fibroma is AKA
Osteofibrous dysplasia
90
MR findings of telangiectatic osteosarcoma
T1 high with fluid-fluid level
91
Osteosarcoma with cleavage plane
Parosteal
92
Skip lesions are found in ewing vs osteosarcoma
Ewing
93
How common is osteosarcoma mets
60%
94
Osseous expansion of enchondroma is known as
Enchondroma protuberans
95
Chondrosarc>Enchondroma is over what size
5cm
96
MC location of periosteal chondrosarcoma
Humerus and femur 70%, 25% feet
97
Chondroblastoma age range
5-25 YO
98
MR appearance of chondroblastoma
low T1, high T2, lots of periosteal reaction
99
Type of cartilage in enchondroma
Hyaline
100
Radial or ulnar sided enchondroma formation
ulnar
101
+C pattern of Enchondroma vs Chrondrosarcoma
Enchondroma = peripheral, Chondrosarcoma is diffuse
102
MC location of Ollier
Femur and tibia
103
MC location of Maffucci
metacarpal and phalanges
104
MC location of chondromyxoid fibroma
Tibia/femur - 55%
105
Codman tumor is AKA
Chondroblastoma
106
In feet chondroblastoma is found where
talus and calcaneus
107
MC benign tumor of the patella
GCT (MC) and chondroblastoma (2nd MC)
108
least common benign cartilage neoplasm is
chondromyxoid fibroma
109
Chondromyxoid fibroma is found in what age
<30 or 50-70
110
MC location of Trevor disease is
Talus, followed by distal femur> prox tibia
111
MC location of subungual exostosis
Great toe (70-80%)
112
Def Turret exostosis
Broad based protuberance on dorsal surface of a finger folloing trauma, believed to be ossified subperiosteal hematoma
113
HME is AKA
Diaphyseal aclasia
114
Medial or lateral epiphysis is involved in Trevors
Medial
115
Def Metachondromatosis
Multiple osteochondromas and enchondromas
116
MC location of conventional chondrosarcoma
Long tubular bone metaphysis, common in femu, innominate and ribs
117
DDx feature between juxtacortical chondrosarcoma and juxtacortical chondroma
Chondrosarcoma has an aggressive periosteal pattern
118
MC loc of clear cell chondrosarcoma
Epiphyseal, femur and humerus
119
MC location of fibroxantoma
Tibia followed by femur
120
Def jaffe-Campanacci syndrome
Multiple NOF, with extraskeletal congenital anomalies of cafe au lait spots, mental retardation
121
MC loc and age of desmoplastic fibroma
Mandible, central metaphysis of femur/hum/tib and found in <30 YO
122
ST involvement of fibrosarcoma
Large involvement with central necrosis=calcifications/sequestrum
123
MC location of GCT in the spine
Sacrum
124
GCT can be found with which other dzs
Pagets or HPT
125
Def Goltz syndrome
Focal dermal hypoplasia, ocular defects, skeletal anomalies
126
MC loc for a intraosseous ganglion
Medial malleoli, carpal bones, periarticular hip
127
MC malignant ST tumor in adults
MFH
128
MFH loc in skeletal
75% ends of long bones, femoral metaphysis in particular
129
Age of MFH
40-60
130
MC loc of intraosseous lipoma
Fibula>fem>tib>calc
131
MC loc of liposclerosing myxofibroma
80% in intertrochanteric
132
Desc cystic angiomatosis
widespread angiomatosis/hemangiomatosis of bone with visceral involvement (65%)
133
MC loc of spinal hemangioma
Thoracic spine
134
MC loc of skull hemangioma
Frontal bone
135
Histologic hemangioma type in skull vs axial skel
Skull = Cavernous, Axial = capillary
136
Angioglomoid tumor is AKA
Glomus tumor
137
MC loc of glomus tumor and age is
Extraosseous, fingertips/nails bed in 30-50 YO
138
Origin of a glomus tumor
neuromyoarterial glomus
139
MC bone tumor to cause Vit D resistance is
Hemangiopericytoma (can also be seen in NOF and GCT)
140
Angiosarcomas can be found at sites of what other lesions/dz
Infarction, chronic OM, Paget and other neoplasms
141
Loc of angiosarcoma
Met/dia of Tib>Fem
142
Synchronous or metachronous angiosarcoma presentation %
20-50%
143
MC flat bones in angiosarcoma
Pelvis and skull
144
Tissue type in Epidermoid cyst
Ectodermal
145
Loc for intraaosseous epidermoid cyst
Skull (Frontal), phalanges
146
2nd MC malignant ST neoplasm in adults
Liposarcoma
147
Def Stewart-Treves syndrome
Bursitits about the Achilles tendon
148
Loc of elastofibroma
Shoulder
149
MC fibromatosis
Palmer fibromatosis (Duputtren contracture)
150
MC source of mets in kids
Neuroblastoma, Ewing, Osteosarcoma, retinoblastoma
151
Solitary mets occurs from
Kidney and thyroid
152
Most likely cranial tumor to spread outside the CNS
Medulloblastoma
153
MC mets to synovium
Breast and lung
154
MC tumor in kids with subarachnoid spread
Medulloblastoma and ependymoma
155
Cookie-bite sign is seen in what mets
Lung CA
156
MC solid abdominal malignancy in kids
Wilms (Nephroblastoma)
157
MC solid intracranial malignancy in kids
Neuroblastoma
158
Wermer syndrome is AKA
Familial multiple endocrine neoplasia syndrome (MEN1)
159
Sipple syndrome is AKA
MEN2
160
Def Beckwith-Wiedemann syndrome
Exomphalos, macroglossia, gigantism syndrome, nephroblastoma
161
Def Diabetic cheiroarthropathy
Joint contractures of 4/5 seen in DM
162
Friedreichs dz
AVN of medial clavicular epiphysis
163
% of Ra patients with nodules
35%
164
Ribs ass'd with RA
Sup margin of post 3-5th ribs
165
% of pts with psoriatic that have psoriatic arthritis
6%
166
Leading cause of psoas abscess
Crohns
167
Intestinal lipodystrophy is AKA
Whipples dz
168
Syndrome with HLA-B5
Behcet disease
169
Def Thiberge Weissenbach syndrome
Calcinosis, digital ischemia
170
Schulman syndrome is AKA
Eosinophilic fasciitis
171
Schulman syndrome/Eosinophic fasciitis shows
Eosinophili, hypergammaglobulinemia with systemic vascular involvement
172
Def Churg-Strauss
Asthma, esoinophilia, vasculities and extravascular granulomas it is AKA Allergic granulomatosis, angiitis
173
Henoch-Schonlein triad
Purpura, abd pain and arthritis (<20 YO)
174
Normal interpediculate distance in Lumbar spine is
20mm
175
Stages of Arachnoiditis
1. Radiculitis 2. Arachnoiditis 3. adhesive arachnoiditis
176
Def Lesch Nyhan syndrome
Deficient HPRT, gouty erosions, seen in boys, delayed skeletal maturation, hip deformities
177
MC loc for HADD in wrist
FCU
178
Triad of hemochromatosis
Cirrhosis, bronze pigmentation and DM
179
Loc of adult cystinosis deposition
Cornea
180
% of Barretts esophagus that go malignant
10%
181
Gallstones on XR seen %
10-15%
182
Def Meigs-Salmon syndrome
Serous cyst adenoma with ascites or pleural effusion
183
MC cause of mechanical colon obstruction
Primary colon CA
184
MC cause of gastric outlet obstruction
Peptic ulcer disease
185
MC cause of spontaneous pneumoperitoneum
Perforated duodenal/gastric ulcer
186
Loc of Zenkers
Posterior esophagus above the cricoharyngeus mm @ Killians dehissence
187
Plummer Vinson Triad
Dysphagia, Esophageal webs, and Fe deficiency
188
MC malignant tumor of pharynx
SCC
189
MC diaphragmatic hernia
Hiatal - Sliding is MC subtype
190
% of pts with GERD and hiatal hernia
90%
191
MC cause of epiglottis
GERD
192
MC cause of esophageal ulceration
Reflux esophagitis
193
Barretts esophagus degenerates to
Adenocarcinoma
194
MC infectious agent of esophagus
candida albicans
195
Def Shaggy esophagus
Worse form of candida ifnection in IC patients (AIDS)
196
rare location of Crohns
Esophagus
197
Manimetric triad of achalasia
1. lack of peristalsis of esophageal body 2. incomplete relaxation and 3. hypertensive resting phase
198
Scleroderma affects the GI tract most where
lower 2/3rd of esophagus
199
Gastric ulcer location
Posterior antrum/body junction
200
Hamptons line indicates benign or malignant ulcer
benign
201
MC type of gastric polyp
Hyperplastic (90%)
202
MC extranodal site of NHL
Stomach
203
Def Cloverleaf duodenum
MM spasm d/t ulcer extending into muscular layer
204
MC complication of peptic ulcer disease is
hemorrhage
205
Congenital duodenal condition seen in Downs
Congenital webs
206
What bug mimicks Whipples disease
Myobacterium avium intercellularis
207
Peutz-Jegher syndrome
GI polyps, cutaneous pigmentation. MC to affect small bowel
208
Poor prognosis of Crohns if found where
Ilium
209
MC organ affected by Chaga disease and what bug
Heart, Trypanosoma Cruzi, reduviid bug bite
210
MC TB location in GIT
Ileocecal
211
MC Shigella ulcer location
Rectosigmoid
212
MC non GI malignancy ass'd with FAPS
GBM
213
MC hamartomatous polyposis syndrome
Peutz-Jegher
214
Almost all Peutz-Jegher pts have this tumor
Gonadal stromal tumor
215
Only polyposis syndrome to affect the esophagus
Cowdens syndrome
216
HCC is AKA
Hepatoma
217
Liver tumor ass'd with PVC
Angiosarcoma (MC primary malignant mesenchymal tumor)
218
MC cause of hepatic granulomas
Sarcoidosis
219
Normal diameter of splenic artery
<1cm
220
MC splenic benign tumor
Hemangioma
221
MC splenic malignant tumor
Lymphoma
222
Iron deposits into spleen from chronic portal HTN
Gamna Gandy Bodies
223
What is a sentinal clot
Perisplenic clotted blood
224
Def Hartmanns pouch
Area of GB proximal to junctional fold (phrygian cap)
225
Def hydrops or courvoissieur GB
Distended GB
226
Def Adenomyosis
Proliferation of GB mucosa with increased thickness
227
Def Rokitansky-Aschoff sinus
Diverticular outpouching in mucosa that forms in adenomyosis
228
MC tumor to spread to GB
Melanoma
229
3 things ass'd with Caroli disease
Congenital hepatic fibrosis, renal cystic dz in kids and renal tubual ectasia in adults
230
Def medullary sponge kidney
Cystic dilation of collecting tubules in kidnets
231
Def primary sclerosing cholangitis
idiopathic dz of liver with diffuse inflammatory fibrosis of biliary tree
232
ERCP can falsely elevate what markers
Lipase and amylase
233
2 physical examination signs of pancreatitis
1. Grey turner sign (flank echymosis) 2. Cullin sign (periumbilical echymosis)
234
Anomaly that predisposes to biliary tree disease
GB agenesis
235
Def Accessory duct of Santorini
Ventral duct is secondary duct that failed to fuse with the main dorsal duct. Inserts proximal to the duct of Wirshung
236
Renal tumor in pts with tuberous sclerosis
AML
237
MC tumor of renal pelvis
TCC
238
Squamous metaplasia of renal pelvis
Leukoplakia
239
Renal issues in Sickle cell anemia
Renal papillary necrosis with Bi renal enlargement
240
Bladder ears
herniation of bladder into Inguinal canal
241
Prune-Belly Syndrome is AKA and Def as
Eagle-barrett syndrome, Anterior abdominal agenesis, cryptorchidism and renal system dilation
242
Changes with radiation nephritis
6-12 mo post, atrophy, glomerulonephritis, pyelonephritis, malignant HTN
243
% of DM pts with emphysematous cystitis
50%
244
MC primary tumor to mets to bladder
melanoma
245
MC benign bladder tumor
Leiomyoma or fibroepitheloid fibroma
246
MC malignant bladder tumor
TCC
247
What tumor is seen in 75-80% of pts with primary aldosteronism
adrenal Adenoma
248
Def Hydrocele
Fluid between layers of tunica vaginalis
