PEDIATRIC Section 13: MSK (Misc Conditions)

Question 1

This is the most common cause of an anterior wall “mass.”

Answer 1

Bifid rib

Question 2

If you have 1 bifid rib, then its a variant, when you have multiple, think of what?

Answer 2

Gorlin Syndrome

Question 3

What is Gorlin Syndrome?

Answer 3

Bifid Ribs
Calcifications of the Falx
basal cell cancers
odontogenic keratocysts (lytic jaw lesions).

Question 4

They have a collagen defect and make brittle bones

Answer 4

Osteogenesis Imperfecta

Question 5

totally lucent skull,
or multiple fractures with hyperplastic callus.

Answer 5

Osteogenesis imperfect

Question 6

Answer 6

totally lucent skull,
or multiple fractures with hyperplastic callus.

fibula longer than the tibia.

Question 7

Wormian bones + flat/beaked vertebral bodies + blue sclera + Hearing impairment (otoslcerosis)

Answer 7

Osteogeneis imperfecta

Question 8

Osteopetrosis + Wormian Bones + Acro-Osteolysis

Answer 8

Pyknodysostosis

Question 9

“wide (or obtuse) angled mandible”

Answer 9

Pyknodysostosis

Question 10

congenital fusion of the cervical spine (sorta like JRA).

Answer 10

Kippel Feil

Question 11

Answer 11

Klippel Feil

The cervical vertebral bodies will be tall and skinny

Question 12

Answer 12

Sprengel deformity (high riding scapula) seen in Klippel Feil

Question 13

Answer 13

Omovertebral bone

• which is just some big stupid looking vertebral body.

Question 14

Answer 14

Hunters I Hurlers I Morquio - All three of these are mucopolysaccharidoses.

oval shaped vertebral bodies with anterior beak.

Mid in Morquio
Inferior in Hurlers

Question 15

Clavicles and ribs are often thick (narrow more medially) - like a canoe- paddle.

Answer 15

Hunters I Hurlers I Morquio

Question 16

Answer 16

Hunters I Hurlers I Morquio

The hand x-ray is the most commonly shown in case books and gives you wide metacarpal bones with proximal tapering.

Question 17

Answer 17

Anterior tibial bowing and pseudoarthrosis at the distal fibula. + scoliosis

Neurofibromatosis TYPE 1

Question 18

This is the most common lysosomal storage disease.

Answer 18

Gauchers

Question 19

Answer 19

Gauchers

Big liver and spleen +
*AVN o f the Femoral Heads
*H-Shaped Vertebra
*Bone Infarcts (lots o f them)
*Erlenmeyer Flask Shaped Femurs

Question 20

This is a spectrum that includes
Sacral and/or Coccyx AGENESIS. You see it with VACTERL and Currarino Triads Syndromes.
NF1

Answer 20

Caudal regression syndrom

Question 21

Radial Dysplasia

Answer 21

Absence or hypoplasia of the radius (usually with a missing thumb) is a differential case (VACTERL, Holt-Oram, Fanconi Anemia, Throbocytopenia Absent Radius).

Question 22

Hand or foot pain/swelling + Infant + Sickle cell

Answer 22

Hand Foot Syndrome

Question 23

Varus angulation occurring at the medial aspect of the proximal tibia (varus bowing occurs at the metaphysis not the knee).

Answer 23

Tibia Vara

This is often bilateral, and NOT often seen before age 2 (two sides, not before two). Later in the disease progression the medial metaphysis will be depressed and an osseous outgrowth classically develops. You can see it in two different age groups; (a) early - which is around age 2 and (b) late - which is around age 12.
* Two Sides - Not Before Two * Two Different Ages (2-3, 12)