Developmental Disorders

Question 1

What is the most common congenital malformation of the limbs?

Answer 1

Syndactyly

Question 2

What is syndactyly?

Answer 2

Fusion of two digits (fingers or toes)

Question 3

What is the treatment for syndactyly?

Answer 3

Surgical separation of digits

(age 3-4)

Question 4

What is the term given to the condition where an extra digit is formed?

Answer 4

Polydactyly

Question 5

How is polydactyly treated?

Answer 5

Amputation of the least useful digit

Question 6

What is fibular hemimelia?

Answer 6

Partial or complete abscence of the fibula

This is often combined with absence of the lateral foot rays

Question 7

What are the three key deformities experienced with fibular hemimelia?

Answer 7

1. Shortened limb
2. Bowing of the tibia
3. Ankle deformity

There is also often abscence of the lateral foot rays

Question 8

How are mild cases of fibular hemimelia treated?

Answer 8

Limb lengthening with a circular frame external fixator

Question 9

How are severe cases of fibular hemimelia treated?

Answer 9

Ankle amputation (10 months - 2 years) and use of below knee prosthetic

Question 10

Abscence or hypoplasia of the radius may lea to which differences in the hand?

Answer 10

1. Underdevelopment, usually with abscence of the thumb
2. Marked deformity known as radial club hand

Question 11

How can abscence or hypoplasia of the radius be treated?

Answer 11

Surgical reconstruction

If the thumb is absent, it can be reconstructed from the index finger (pollicisation) and deformity correction

Question 12

What isthe most common congenital bone fusion?

Answer 12

Tarsal coalition

Fusion of the tarsal bones

Question 13

What may tarsal coalition lead to and what is the appropriate treatment?

Answer 13

Painful flat feet

Surgical division

Question 14

How does brachial plexus injury commonly occur?

Answer 14

Vaginal delivery which involves:

1. Large babies
2. Twin deliveries
3. Shoulder dystocia

Question 15

What is shoulder dystocia?

Answer 15

Difficult of delivery of a baby which presents after the head is delivered fine.

The problem arises with the shoulder which becomes compressed against the mother’s pubic symphysis and requires much manipulation to be released

This may cause damage to the brachial plexus

Question 16

What is the most common type of obstetric brachial plexus palsy?

Answer 16

Erb’s palsy

Question 17

Which nerve roots are injured in Erb’s palsy?

Answer 17

C5 and C6

Question 18

In Erb’s palsy, there is damage to the C5 and C6 nerve roots, what effect does this have?

Answer 18

Motor innervation is lost to:

1. Deltoid
2. Supraspinatus
3. Infraspinatus
4. Biceps
5. Brachialis

Question 19

What is the classical sign in infants with Erb’s palsy?

Answer 19

Waiter’s tip posture

(caused due to internal rotation of the humerus - subscapularis functions and in unopposed as other muscles have no motor innervation)

Question 20

What is the treatment for Erb’s palsy?

Answer 20

Physiotherapy (to prevent contractures) - recovery begins after arund 6 months

If there is failure of recovery, surgical release of contractures and tendon transfers may be required

Question 21

What is the term given to a much rarer form of obstetric brachial plexus palsy?

Answer 21

Klumpke’s palsy

Question 22

What is Klumpke’s palsy and what does it involve?

Answer 22

It is a lower brachial plexus injury affecting the C8 and T1 nerve roots

It is generally caused by forceful adduction

It causes paralysis of the instrinsic muscles of the hand and potentially also finger and wrist flexors as well as Horner’s syndrome

Question 23

How does Klumpke’s palsy present?

Answer 23

Fingers which are flexed

(due to paralysis of interossei and lumbricals which assist with extension at PIP joints)

Question 24

Why does Horner’s syndrome occur with Klumpke’s palsy?

Answer 24

There is disruption of the first sympathetic ganglion from T1

Question 25

What is Horner’s syndrome?

Answer 25

A condition of the eye caused by disruption of the first ganglion of the sympathetic trunk which may be due to Klumpke’s palsy

It is characterised by a triad of condition:

1. Miosis (a constricted pupil)
2. Partial ptosis (a weak, droopy eyelid)
3. Anhidrosis (decreased sweating)

It may occur with enophthalmos (inset eyeball).

Question 26

What is the treatment for Klumpke’s palsy?

Answer 26

There is no specific treatment

It has a 50% recovery rate