Embryology and congenital malformations Flashcards
What happens during gastrulation?
Single layered blastula reorganised to multi layered structure
What forms the germ layers>
Epiblast
What are the 3 germ layers, from top to bottom?
Ectoderm
Mesoderm
Endoderm
What germ layer doe the nervous system form from?
Ectoderm
When does the CNS appear?
Beginning of week 3
What initial structure that will form the nervous system?
Neural plate
What is the neural plate?
Thickening of ectoderm anterior to primitive node
How does the neural tube form?
Edges of the neural plate form and move upwards, forming the neural folds
The neural folds migrate towards each other and fuse in the midline, forming the neural tube
What is the initial structure of the neural tube?
Remains open at anterior and posterior ends
When do the ends of the neural tube close?
Cranial neuropore= 25 days
Caudal neuropose= 27 days
Where is neural tube closure initiated?
Several points along the AP axis and proceeds in cranial and caudal directions
What does failure of proper neural tube closure cause?
Neural tube defects- ancephalopathy, encephalocoele, spina bifida
What causes ancephalopathy?
Failure of anterior neuropore to close
What does ancephalopathy cause?
Skull fails to form and brain tissue degenerates
What is craniorachischisis?
Failure of neural tube closure along entire neuroaxis
What is encephalocoele?
Herniation if cerebral tissue through defect in the skull, most frequently in occipital region
What causes encephalocoele?
Failure in closure of rostral neural tube closure
What causes spina bifida?
Defective closure of caudal neural tube
What does spina bifida cause?
Non fusion of vertebral arches
Affects tissues overlying spinal cord
Neural tissue may or may not be affected
What are the types of spina bifida?
Spina bifida occulta Spina bifida cystica Spina bifida with meningocele Spina bifida with meningomyelocele Myloschisis
What causes spina bifida occulta?
Failure of embryonic halves of vertebral arch to grow normally and fuse
What dies spina bifida occulta cause?
Usually no clinical symptoms
Dimple with small tuft of hair on lower back
What does spina bifida cystica cause?
Protrusion of spinal cord and/or meninges through defect in vertebral arches
What does spina bifida with meningocele cause?
Protrusion of meninges and CSF
What does spina bifida with meningomyelocle cause?
Protrusion of meninges, CSF and nerve roots and/or spinal cord
What are the clinical signs of spina bifida with meningomyelocle?
Neurological deficits- loss of sensation and muscle paralysis
Area affected determined by level of lesion
Often associated with hydrocephalus
What causes myloschisis?
Failure of neural folds to fuse
What does myloschisis cause?
Spinal cord in affected area open
What is the prevention of spina bifida?
Folic acid supplements
How is spina bifida diagnosed prenatally?
Maternal blood screening
Amniocentesis
Ultrasound