Slu Sbj Quiz 2

Front 1

Describe Aplasia Cutis Congenita

Back 1

Localized absence of skin, usually on scalp

Front 2

Describe Icthyosis and the subtypes

Back 2

Hyperkeratosis of stratum corneum possibly from increased cell-cell adhesion
Harlequin fetus - Rare, AR, thick ridge-cracked skin
Collodion baby - Thick, taut membrane resembling collodion, cracks with respiration

Front 3

What is a cystic hygroma

Back 3

Benign tumor of lymphatics

Front 4

What is the most common neoplasm in infants

Back 4

Hemangioma

Front 5

Albinism involves what

Back 5

AR mutation in tyrosinase that is involved in the conversion of tyrosine to DOPA and DOPA to melanin

Front 6

Describe piebaldism

Back 6

AD
localized albinism (along midline)
KIT protooncogene mutation (TK receptor)

Front 7

Arthrogryposis multiplex congenita presents with what

Back 7

Fetal akinesia-hypokinesia sequence:
Joint contractures
Polyhydramnios
Pulomonary hypoplasia
Craniofacial anomalies

Front 8

Describe congenital toricollis

Back 8

aka Wry Neck
Injury to sternocleidomastoid muscle leading to fibrosis
Results in fixed rotation and tilting of head

Front 9

Describe the etiologies of limb reduction defects

Back 9

Genetic factors (chromosomal, T18)
Mutant genes (brachydactyly)
Environment/teratogens (Thalidomide)
Multifactorial
Vascular interruption (amniotic band sequence)

Front 10

Describe amelia

Back 10

Complete absence of limb
Suppression of limb bud development in wk 4

Front 11

Describe Meromelia

Back 11

Partial absence of limb
Arrest/disturbance of differentiation/growth in wk 5

Front 12

Describe ectrodactyly

Back 12

Subtype of meromelia
Cleft hand/foot "lobster claw"
Dev. failure of one or more digital ray

Front 13

Describe rickets-osteomalacia

Back 13

Deficient bone matrix calcification from inadequate calcium intake or a vit. D deficiency
Altered endochondral bone formation

Front 14

Describe the bones of a patient that has rickets-osteomalacia

Back 14

Short, deformed, bowed long bones
Frontal bossing
Squared head
Hypertrophic epiphyseal cartilage
Rachitic rosary

Front 15

Describe craniosynostosis and its subtypes

Back 15

Premature closure of cranial sutures
Scaphocephaly - Sagittal suture - long, narrow, wedge-shaped skull
Oxycephaly - Coronal suture - high, tower-like skull

Front 16

Describe the characteristics of benign osteopetrosis

Back 16

AD
Apparent in adolescence
Repeated fractures and anemia

Front 17

Describe the characteristics of malignant osteopetrosis

Back 17

AR
Apparent in utero
Fractures, anemia, hydrocephaly, cranial nerve entrapment

Front 18

Describe the pathogenesis and cure for osteopetrosis

Back 18

Failure of osteoclastic resorption
Possible deficient CA II, so osteoclasts can acidify environment

Front 19

Describe mucopolysaccharidosis

Back 19

Lysosomal storage disease
Deficient acid hydrolase that degrades GAG
Patients have short stature and bone dysplasia
Cartilage at growth plates and articular surfaces are disorganized

Front 20

Describe Gaucher Disease

Back 20

Most common lysosomal storage disease
AR
Deficient glucocerebrosidase (which cleaves glucose from ceramide)
Glucocerebroside accumulates in macrophages giving "crumpled tissue paper" appearance

Front 21

Describe the types of gaucher disease

Back 21

Type I: Chronic non-neuropathic, can be treated with enzyme infusion
Type II: Acute neuropathic (CNS), die in infancy
Type III: Intermediate form

Front 22

Where are Gaucher cells found

Back 22

Spleen
Bone marrow (cause erosions, pathologic fractures, cytokines)
Lymph nodes
Thymus

Front 23

Children usually develop osteomyelitis where? Adults?

Back 23

Children - Metaphysis
Adults - Epiphysis and subchondral areas

Front 24

Describe "sequestrum"

Back 24

Osteomyelitis - necrotic bone in acute inflammation

Front 25

Describe "involucrum"

Back 25

Osteomyelitis - new bone forms sleeve around dead infected bone

Front 26

Describe osteochondroma

Back 26

aka exostosis
Most common benign bone tumor
Young adults
Stop growing when growth plates close

Front 27

Describe osteoid osteoma

Back 27

<2cm painful lesion
Young male, nocturnal pain, releived by NSAIDs
Pain from PGE2 from proliferating osteoblasts
Tumor nidus is well-defined with circumscribed zone of trabeculae rimmed by benign osteoblasts surrounded by halo of sclerotic bone
NIDUS is radiolucent

Front 28

Describe osteoblastoma

Back 28

Young person
Spine and long bones
Dull, achy pain, not responsive to ASA

Front 29

Describe a fibrous cortical defect

Back 29

Developmentally caused eccentric lesion
Usually in distal femur or proximal tibia
Often multiple and bilateral
Most self-resolve

Front 30

Describe a non-ossifying fibroma (NOF)

Back 30

FCD that doesn't regress
Radiolucent with fibroblasts and macrophages

Front 31

Describe the two types to fibrous dysplasia

Back 31

GNAS1 mutation
Monostotic - 70% of cases, adolescence -> ribs, femur, tibia, jaw
Polyostotic - Younger child, any bone, fracture risk, deformity, sarcoma

Front 32

Describe the histologic appearance of fibrous dysplasia

Back 32

Chinese letter bone trabeculae with fibroblasts

Front 33

Describe Aneurysmal Bone Cyst (ABC)

Back 33

Benign, rapidly growing tumor
Young adults and children at the metaphyses and vertebrae
Pain

Front 34

Describe Ewing's Sarcoma (EWS) and Primitive Neuroectodermal Tumor (PNET)

Back 34

Closely related malignant tumors
Neuronal differentiation on LM or EM = PNET
Second most common bone tumor in children
multipotent mesenchymal stem cells
Overexpression of c-myc from fusion gene

Front 35

Describe Langerhans Cell Histiocytosis

Back 35

Proliferation of Langerhans Histiocytes in tissue
Appears as Eosinophilic Granuloma, Hand-Schuller-Christian Disease, and Letterer-Siwe Disease
Large phagocytic cells with **Cytoplasic Birbeck granules** (zipper)

Front 36

Describe Eosinophilic granuloma (of LCH)

Back 36

70% of cases
Unisystem
Self-limited
Asymptomatic or mild pain

Front 37

Describe Hand-Schuller-Christian disease (of LCH)

Back 37

20% of cases
Multifocal
Children
Lytic lesions of skull, ribs, pelvis, and scapulae
Classic triad --> Lytic skull lesions, diabetes insipidus, exophthalmos

Front 38

Describe Letterer-Siwe Disease

Back 38

10% of cases
<2yo
Aggressive, multisystem
Seborrheic rash
Pancytopenia

Front 39

Describe Rhabdomyosarcoma (general)

Back 39

Most common childhood soft tissue sarcoma
Embryonal and alveolar subtypes
65% cure rate

Front 40

Describe Embryonal rhabdomyosarcoma

Back 40

Most common subtype
nasal cavity, orbit, middle ear, prostate, retroperitoneum
Sarcoma botryoid is a subtype

Front 41

Describe Embryonal Rhabdomyosarcoma Sarcoma Botryoid

Back 41

Polypoid "grape-like" tumor in hollow mucosal-lined structures like nose, bile duct, bladder, vagina
Better prognosis

Front 42

Describe Alveolar rhabdomyosarcoma

Back 42

Extremity muscle and embryonal rhabdo sites
**Fibrous septae divide tumor cells in clusters**
Cells express desmin and myoglobine
Worse prognosis

Front 43

A patient with sickle cell disease has osteomyelitis, the most likely causative organism is

Back 43

Salmonella

Front 44

Describe Paget Disease

Back 44

Osteitis Deformans
Osteoclast dysfunction (infection by paramyxovirus increasing RANK expression = IL-6 secretion)
SQSTM1 gene
Pain with microfractures
Overgrowth compresses nerves
**Lamellar bone with mosaic pattern**

Front 45

Describe Osteonecrosis-Avascular Necrosis

Back 45

Ischemia in bone from vessel damage/obstruction
Cortex NOT involved (collaterals)
Predispostion to osteoarthritis

Front 46

Describe enchondroma

Back 46

Asymptomatic benign solitary radiolucent tumor
Common in hands
Medullary cavity
Well-differentiated hyaline cartilage
Small, gray-blue nodules

Front 47

Describe the syndromes with multiple enchondromas

Back 47

Ollier disease - Multiple asymmetric metaphyseal/diaphyseal enchondromas, large, deforming, not inherited, *sarcoma* risk
Maffucci syndrome - Skin and soft tissue hemangiomas, ovarian and brain gliomas, half dev. chondrosarcoma

Front 48

Describe chondrosarcoma

Back 48

Second most common malignant matrix producing bone tumor
40+yo, men>women
Axial skeleton and proximal limbs
Painful bulky tumor
Intramedullary or juxtacortical

Front 49

List the "lytic" metastatic bone tumors

Back 49

Kidney, GI, thyroid, melanoma, breast, lung

Front 50

List the "blastic" metastatic bone tumors

Back 50

prostate, breast, lung

Front 51

Describe/define fibromyalgia

Back 51

A syndrome of chronic, widespread pain which often coincides with symptoms including fatigue, sleep disturbance, cognitive dysfunction, and depression/anxiety

Front 52

What are the psychiatric comorbidities of fibromyalgia

Back 52

SIG-E-CAPS
Sleep changes
loss of Interest
Guilt
lack Energy
difficulty Concentrating
changes in Appetite
Psychomotor agitation
Suicide

Front 53

What is notable about treating catastrophizing associated with fibromyalgia

Back 53

Cognitive-behavioral feedback therapy is as good as/better than pharmacological therapy

Front 54

What are the diagnostic criteria for fibromyalgia

Back 54

Meet widespread pain thresholds
Symptoms present for >3mos
*Patient does not have another disorder to explain pain
*Does not require presence of tender points
*Diagnosis of exclusion and careful evaluation
*No specific lab abnormalities

Front 55

What is the therapy for fibromyalgia

Back 55

*Non-pharmacologic - education, exercise, cognitive behavioral therapy
Pharmacologic - Amitriptyline, SSRI
**Steroids, NSAIDs, and opiods are NOT useful

Front 56

What are the innervations of the rotator cuff

Back 56

Suprascapular
axillary
supper/lower subscapular

Front 57

What are the muscles associated with the rotator cuff

Back 57

Supra/infraspinatus
teres minor
subscapularis
Coracoacromial ligament
Long head of biceps

Front 58

Describe the labrum

Back 58

Peripheral fibrous tissue-fibrocartilage
Attaches to the glenoid capsule and provides increased depth around the rim
Protects the edges of the bone from bone-bone contact

Front 59

What is the more common SC dislocation

Back 59

Anterior dislocation more common than posterior

Front 60

Will reduction be successful with anterior SC dislocation

Back 60

No

Front 61

Will reduction be successful with posterior SC dislocation

Back 61

yes, must be reduced acutely or risk venous congestion/tracheal erosion

Front 62

Describe the glenohumoral ligaments

Back 62

All - proprioceptive function and stabilization
Superior - prevent inferior translation in ADduction
Middle - prevent anterior translation in ABduction
Inferior - hammock like, prevent ant/post translation in ABduction

Front 63

Describe clavicle fractures

Back 63

Caused by fall on shoulder
Normally treat with sling with early gradual ROM exercise
If complicated or open, surgical treatment is indicated --> *dimple sign* - bone can't reform through fascia

Front 64

Describe the acromio-clavicular joint separation types

Back 64

1 - strain AC
2 - tear AC, strain CC
3 - tear AC and CC
4 - bones overlap
5 - >200% separation
6 - clavicle under muscles

Front 65

Differentiate between glenohumoral joint dislocation vs separation

Back 65

Separation - ligaments attaching bone are torn and decrease joint integrity
Dislocation - joint leaves capsule

Front 66

What is observed externally with a glenohumoral joint dislocation

Back 66

prominent acromion process because deltoid is pulled down

Front 67

Briefly describe the Kocher reduction technique

Back 67

Adduct arm
Gradually externally rotate until resistance
Forward flex+adduct

Front 68

Briefly describe the Modified Milsch reduction technique

Back 68

Gradual ABduction and externally rotate (no traction)

Front 69

Briefly describe the Hippocratic reduction technique

Back 69

Don't do it
traction-counter-traction

Front 70

Briefly describe the Stimson's reduction technique

Back 70

Prone w/ towel under clavicle and wrist weights - relaxes spasming muscles with persistent traction

Front 71

Briefly describe the Matsen reduction technique

Back 71

Traction-countertraction on chest wall (not axilla like with hippocratic) with gentle ABduction

Front 72

What are some complications associated with shoulder dislocation

Back 72

Vasculary injury to posterior circumflex artery
Axillary nerve injury - loss of major abduction of arm, loss of adduction and lateral rotation of humerus

Front 73

What causes elbow dislocation typically

Back 73

Fall onto outstretched hand
or sudden violent medial force causing hyperextension

Front 74

What are some complications of elbow dislocation

Back 74

Lose full extension
Soft tissue calcification
Ulnar nerve entrapment
Claw hand due to loss of lumbrical function --> flexion of MTP, extension of PIP

Front 75

What is the major complication of a scaphoid fracture

Back 75

Nonunion

Front 76

What is the most frequent fracture at the elbow

Back 76

Radial head fracture

Front 77

What is a "sail sign"

Back 77

Fat pad showing hemarthrosis on plain film xray

Front 78

What is the typical treatment for an olecranon fracture

Back 78

Operative repair
**see card for non-operative indications

Front 79

What are the indications for non-operative treatment of olecranon fractures

Back 79

Non-displaced fractures
Intact extensor mechanism

Front 80

What is the typical mechanism for a patella fracture

Back 80

Direct impact

Front 81

The two most common carpal fractures are

Back 81

Scaphoid
Triquetrum

Front 82

A scaphoid fracture typically presents with what

Back 82

Pain over anatomic snuffbox, but should be suspected in anyone with wrist pain following an injury

Front 83

How is a scaphoid fracture treated

Back 83

Immobilize with thumb spica unless surgery is indicated

Front 84

What are the types of triquetrum fractures and how is the most common fracture treated

Back 84

Dorsal ridge
Volar fractures
Body fractures
---> Chip/avulsion fractures are the most common and treated symptomatically

Front 85

What injury associated with a distal radial fracture is a surgical emergency and what are its symptoms

Back 85

Acute Carpal Tunnel Syndrome
Pain
Paresthesias
Numbness in median nerve distribution

Front 86

How might a distal radial fracture be treated

Back 86

Non-closed "sugar tong" splint
Ligamentotaxis (pulling on hand to let fragments fall back into place
Hematoma block

Front 87

What are the three key things to document on a humerus fracture

Back 87

Open vs closed
Vascular exam
**Radial nerve function**

Front 88

Most humerus fractures can be treated in what way

Back 88

Non-surgical closed splint tightening around muscles

Front 89

What is the most common weight bearing skeletal injury

Back 89

Ankle fracture

Front 90

What are the two main types of hip fractures

Back 90

Intertrochanteric femure fracture
Femoral neck fracture

Front 91

What are the treatment goals in the case of hip fractures

Back 91

Early mobilization
Weight bearing as tolerated
Restore pre-break function

Front 92

What is the main concern if surgery is delayed >48hrs after a hip fracture

Back 92

Increased mortality

Front 93

What is the main goal of treating a fracture in a lower extremity

Back 93

Restore mechanical axis

Front 94

What is the main goal of treating a fracture in an upper extremity

Back 94

Permit use of hand in space

Front 95

All hip fracture patients should be given some amount of prophylaxis for what

Back 95

DVTs

Front 96

What does the ACL normally do

Back 96

Attaches lateral femoral condyle to anterior tibia
Stabilizes rotary instability of the tibio-femoral joint during pivoting to prevent anterior translation of tibia when planting foot

Front 97

How does an ACL tear typically occur

Back 97

Twisting injury

Front 98

What is the clinical presentation of a torn ACL

Back 98

Large effusion, limited ROM, pain, **lateral joint line tenderness+bruising
Positive Lachman test (Shift shin forward relative to knee)

Front 99

Do torn ACLs heal

Back 99

No

Front 100

When does a PCLtear typically occur

Back 100

"dashboard" injury where tibia is pushed back

Front 101

When does a MCL tear typically occur

Back 101

Lateral hit on knee, ie football

Front 102

Describe the causes a meniscus tear (typical)

Back 102

Caused by acutely by twisting or pivoting in younger patients

or

Chronically with trauma history, pain with twisting and degenerative cartilage changes

Front 103

Describe the clinical presentation of a meniscus tear

Back 103

Tibiofemoral joint line tenderness
Effusion
pain with deep flexion
+ McMurray's test (bend knee, rotate foot out, straighten leg, see pain or click
Often see degen. of quadriceps

Front 104

Describe McMurray's test

Back 104

Bend knee, rotate foot out, straighten leg, get pain and/or click

Front 105

Most tendon pathology is due to...

Back 105

overuse=degeneration

Front 106

Tendon regeneration yields weaker tendons if

Back 106

remodeling does not occur

Front 107

The best therapy for painful tendons is

Back 107

Eccentric strengthening