Non-adjacent spondylolisthesis in Ehlers-Danlos syndrome

| To report a case of non-adjacent spondylolisthesis in a patient with Ehlers-Danlos syndrome

Non-adjacent spondylolisthesis in ehlers-danlos ..

Confounding Variables :end of season injury and hence no follow up from the academysuffers from Hypermobile Joint Syndrome (HJS) and possibly Ehlers Danlos Syndrome (EDS), however Beighton score 4/9.

Bilateral hip dislocations in a father and son with Ehlers-Danlos syndrome

Ehlers-Danlos syndromes and Marfan syndrome

Rare
Age at diagnosis is variable
Congenital form baby still-born or dies soon after birth
Tarda form becomes evident around 6 months old
Mild adult form results in osteomalacia
(Congenita)
Usually dies of respiratory failure in neonatal period or stillborn
(Tarda)
Failure to thrive
Bossing of the skull
Bowing of the ribs
Flaring of the ends of long bones
Fractures and angular deformities
Short stature
Lose deciduous teeth early due to caries from poor enamel
(Mild adult form)
Presents with osteomalacia or # with delayed healing
Demineralisation of the entire skeleton (severity dependant on type)
Widening of the physis with irregularities extending into the metaphysis
Fractures and deformities of long bones
Renal calcinosis may be evident
Deficiency of alkaline phosphatase
Disturbance of mineralisation and defective ossification of cartilage
Histology resembles Rickets with abundant un-mineralised osteoid
Hypercalcaemia (may renal calculi)
Serum Phosphorus is normal
Increased excretion of phosphorylethanolamine and inorganic pyrophosphate
Decreased excretion of hydroxy proline
Various types of Rickets
Severe congenital form differentiate from osteogenesis imperfecta
Correction of deformity
Enzyme replacement of no long term benefit
Do not give Vit D as increased hypercalcaemia
If survives childhood condition usually improves with increasing maturation of the skeleton
Characterised by failure of bone resorption due to a deficiency of osteoclasts.

2 citations Non-adjacent spondylolisthesis in ehlers-danlos syndrome ..


Joint laxity is associated with connective tissue dysplasias such as Ehlers-Danlos syndrome, Marfans syndrome, Morquios disease and osteogenesis imperfecta.

Karina's Cervical Spine Instability and Ehlers-Danlos Syndrome