Radial Club Hand

Question 1

Radial clubhand genetic issue is with:

Answer 1

sonic hedgehog embryology

Question 2

Hematologic associations of radial clubhand

Answer 2

• TAR

- Fanconi’s Anemia

Question 3

Physical Exam of Radial clubhand

Answer 3

perpendicular relationship between the wrist and the forearm; absent thumb; possibly complete absence of the radius

Question 4

Additional tests needed in radial clubhand

Answer 4

• CBC (thrombocytopenia, aplastic anemia)
• renal ultrasound
• echo

Question 5

Indications for Hand Centralization with Radial Clubhand?

Answer 5

• good elbow motion and biceps function
• age 6-12 months
• follow with tendon transfers

Question 6

Contraindications for Hand Centralization with Radial Clubhand

Answer 6

elbow extension that forced the child to rely on radial deviation of the wrist

Question 7

Thrombocytopenia Absent Radius Syndrome

Answer 7

autosomal recessive condition

• absent radius
• thrombocytopenia
• thumb usually present

Question 8

Fanconi’s Anemia

Answer 8

Autosomal recessive

• Fanconi screen needed
• Chromosomal breakage test needed
• Treatment is bone marrow transplant, otherwise patient will die from aplastic anemia
• presents between 6-9 years of age