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Flashcards in Developmental Disorders Deck (26):

What is the most common congenital malformation of the limbs?



What is syndactyly?

Fusion of two digits (fingers or toes)


What is the treatment for syndactyly?

Surgical separation of digits

(age 3-4)


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



How is polydactyly treated?

Amputation of the least useful digit


What is fibular hemimelia?

Partial or complete abscence of the fibula

This is often combined with absence of the lateral foot rays



What are the three key deformities experienced with fibular hemimelia?

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

There is also often abscence of the lateral foot rays


How are mild cases of fibular hemimelia treated?

Limb lengthening with a circular frame external fixator


How are severe cases of fibular hemimelia treated?

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


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

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


How can abscence or hypoplasia of the radius be treated?

Surgical reconstruction

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


What isthe most common congenital bone fusion?

Tarsal coalition

Fusion of the tarsal bones


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

Painful flat feet

Surgical division


How does brachial plexus injury commonly occur?

Vaginal delivery which involves:

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


What is shoulder dystocia?

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


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

Erb's palsy


Which nerve roots are injured in Erb's palsy?

C5 and C6


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

Motor innervation is lost to:

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


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

Waiter's tip posture

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


What is the treatment for Erb's palsy?

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


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

Klumpke's palsy


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

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


How does Klumpke's palsy present?

Fingers which are flexed

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


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

There is disruption of the first sympathetic ganglion from T1


What is Horner's syndrome?


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).


What is the treatment for Klumpke's palsy?

There is no specific treatment

It has a 50% recovery rate

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