Developmental Disorders Flashcards
What is the most common congenital malformation of the limbs?
Syndactyly
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?
Polydactyly
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?
- Shortened limb
- Bowing of the tibia
- 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?
- Underdevelopment, usually with abscence of the thumb
- 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:
- Large babies
- Twin deliveries
- 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:
- Deltoid
- Supraspinatus
- Infraspinatus
- Biceps
- 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:
- Miosis (a constricted pupil)
- Partial ptosis (a weak, droopy eyelid)
- 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
