part 2.1 clinical manifestations

What are the clinical manifestations of Spina Bifida?

SENSORY IMPAIRMENT

MUSCULOSKELETAL IMPAIRMENT

NEUROLOGICAL IMPAIRMENT

INTEGUMENTARY IMPAIRMENT

What clinical manifestation is this?

● Impaired sensation below level of lesion.

● Loss of : kinesthetic, proprioceptive, and somatosensory information

● Rely heavily on vision and other sensory systems

SENSORY IMPAIRMENT

What clinical manifestation is this?

● Impaired sensation below level of lesion.

SENSORY IMPAIRMENT

What clinical manifestation is this?

● Loss of : kinesthetic, proprioceptive, and somatosensory information

SENSORY IMPAIRMENT

What clinical manifestation is this?

● Rely heavily on vision and other sensory systems

SENSORY IMPAIRMENT

CLINICAL MANIFESTATION

Sensory impairment has a loss of?

kinesthetic, proprioceptive, and somatosensory information

What clinical manifestation is this?

• Weakness & Paralysis

• Orthopedic Deformities

  • Lumbar Kyphosis; Scoliosis; Lordosis/ Lordoscoliosis; Equinovarus; Contractures

• Osteoporosis

MUSCULOSKELETAL IMPAIRMENT

What clinical manifestation is this?

• Weakness & Paralysis

MUSCULOSKELETAL IMPAIRMENT

What clinical manifestation is this?

• Orthopedic Deformities

  • Lumbar Kyphosis; Scoliosis; Lordosis/ Lordoscoliosis; Equinovarus; Contractures

MUSCULOSKELETAL IMPAIRMENT

What clinical manifestation is this?

• Osteoporosis

MUSCULOSKELETAL IMPAIRMENT

What are the orthopedic deformities in Musculoskeletal Impairment?

Orthopedic Deformities

• Lumbar Kyphosis; Scoliosis; Lordosis/ Lordoscoliosis; Equinovarus; Contractures

Enumerate the neurological impairments?

Hydrocephalus

Arnold-Chiari II Malformation

Tethered Spinal Cord

Hydromyelia

What neurological impairment is this

• 80% to 90% of children with myelomeningocele

• blockage of the normal flow of CSF between the ventricles and spinal canal ○ abnormal accumulation of CSF in the cranial vault

• S/sx:

  • Abn inc of head size

  • Bulging of fontanelles

  • Irritability

• Clinical signs

  i. Bulging Fontanelles

  ii. Rapid inc of Head Circ.

  iii. Palpable separation of coronal and sagittal sutures

  iv. Sunsetting Sign

  v. Irritable/ Lethargy

● Hydrocephalus

What neurological impairment is this

• 80% to 90% of children with myelomeningocele

● Hydrocephalus

What neurological impairment is this?

• blockage of the normal flow of CSF between the ventricles and spinal canal ○ abnormal accumulation of CSF in the cranial vault

● Hydrocephalus

What neurological impairment has these signs and symptoms?

• S/sx:

  • Abn inc of head size

  • Bulging of fontanelles

  • Irritability

● Hydrocephalus

What neurological impairment has these clinical signs?

• Clinical signs

  i. Bulging Fontanelles

  ii. Rapid inc of Head Circ.

  iii. Palpable separation of coronal and sagittal sutures

  iv. Sunsetting Sign

  v. Irritable/ Lethargy

● Hydrocephalus

What are the signs and symptoms of Hydrocephalus?

■ Abn inc of head size

■ Bulging of fontanelles

■ Irritability

What are the clinical signs of Hydrocephalus?

• Clinical signs

  i. Bulging Fontanelles

  ii. Rapid inc of Head Circ.

  iii. Palpable separation of coronal and sagittal sutures

  iv. Sunsetting Sign

  v. Irritable/ Lethargy

NEUROOLOGICAL IMPAIRMENT

Arnold-Chiari II Malformation

• primary cause of hydrocephalus in most children c SB

• deformity of the ___, ___, and ___

  • variable displacement of ___ into the___

  • caudal dislocation of the ___ and ___

• herniation of the ___ and (at times) ___, ___, and ___ into the ___.

• Occurs bet ____ (may extend to T1)

• ___: series of interrelated, time-dependent defects occur during the embryonic development of the primitive ventricular system → Chiari II Malformation 1st before hydrocephalus

• Small posterior fossa

  • unable to accommodate the ___ and ___ structures → abn positioning

  • 25% of neonates with spina bifida - head circumferences measuring below the ___

• Therefore, neither downward ___ from the spinal defect nor pressure from hydrocephalus caused the malformation.

• Cardinal Features

  • __

  • __

  • __

  • __

  • __

NEUROOLOGICAL IMPAIRMENT

Arnold-Chiari II Malformation

• primary cause of hydrocephalus in most children c SB

• deformity of the cerebellum, medulla, and cervical spinal cord

  • variable displacement of cerebellar tissue into the spinal canal

  • caudal dislocation of the lower brainstem and fourth ventricle

• herniation of the medulla and (at times) pons, fourth ventricle, and inferior aspect of the cerebellum into the upper cervical canal.

• Occurs bet C1-C4 (may extend to T1)

• Widely accepted theory: series of interrelated, time-dependent defects occur during the embryonic development of the primitive ventricular system → Chiari II Malformation 1st before hydrocephalus

• Small posterior fossa

  • unable to accommodate the hindbrain and brainstem structures → abn positioning

  • 25% of neonates with spina bifida - head circumferences measuring below the fifth percentile

• Therefore, neither downward traction from the spinal defect nor pressure from hydrocephalus caused the malformation.

• Cardinal Features

  • Myelomeningocele

  • venting of the intracranial CSF

  • hypoplasia of the posterior fossa

  • herniation of the hindbrain

  • compressive damage to cranial nerve

What neurological impairment is this?

• primary cause of hydrocephalus in most children c SB

• deformity of the cerebellum, medulla, and cervical spinal cord

  • variable displacement of cerebellar tissue into the spinal canal

  • caudal dislocation of the lower brainstem and fourth ventricle

• herniation of the medulla and (at times) pons, fourth ventricle, and inferior aspect of the cerebellum into the upper cervical canal.

• Occurs bet C1-C4 (may extend to T1)

• Widely accepted theory: series of interrelated, time-dependent defects occur during the embryonic development of the primitive ventricular system → Chiari II Malformation 1st before hydrocephalus

• Small posterior fossa

  • unable to accommodate the hindbrain and brainstem structures → abn positioning

  • 25% of neonates with spina bifida - head circumferences measuring below the fifth percentile

• Therefore, neither downward traction from the spinal defect nor pressure from hydrocephalus caused the malformation.

• Cardinal Features

  • Myelomeningocele

  • venting of the intracranial CSF

  • hypoplasia of the posterior fossa

  • herniation of the hindbrain

  • compressive damage to cranial nerve

Arnold-Chiari II Malformation

What neurological impairment is this?

• primary cause of hydrocephalus in most children c SB

Arnold-Chiari II Malformation

What neurological impairment is this?

• deformity of the cerebellum, medulla, and cervical spinal cord

  • variable displacement of cerebellar tissue into the spinal canal

  • caudal dislocation of the lower brainstem and fourth ventricle

Arnold-Chiari II Malformation

What neurological impairment is this?

• herniation of the medulla and (at times) pons, fourth ventricle, and inferior aspect of the cerebellum into the upper cervical canal.

Arnold-Chiari II Malformation

What neurological impairment is this?

• Occurs bet C1-C4 (may extend to T1)

Arnold-Chiari II Malformation

What neurological impairment is this?

• Widely accepted theory: series of interrelated, time-dependent defects occur during the embryonic development of the primitive ventricular system → Chiari II Malformation 1st before hydrocephalus

Arnold-Chiari II Malformation

What neurological impairment is this?

• Small posterior fossa

  • unable to accommodate the hindbrain and brainstem structures → abn positioning

  • 25% of neonates with spina bifida - head circumferences measuring below the fifth percentile

Arnold-Chiari II Malformation

What neurological impairment is this?

• Therefore, neither downward traction from the spinal defect nor pressure from hydrocephalus caused the malformation.

Arnold-Chiari II Malformation

What neurological impairment is this?

• Cardinal Features

  • Myelomeningocele

  • venting of the intracranial CSF

  • hypoplasia of the posterior fossa

  • herniation of the hindbrain

  • compressive damage to cranial nerve

Arnold-Chiari II Malformation

Arnold-Chiari II Malformation has a deformity of what neurological structures?

deformity of the cerebellum, medulla, and cervical spinal cord

• variable displacement of cerebellar tissue into the spinal canal

• caudal dislocation of the lower brainstem and fourth ventricle

Arnold-Chiari II Malformation has a herination of what neurological structures?

○ herniation of the medulla and (at times) pons, fourth ventricle, and inferior aspect of the cerebellum into the upper cervical canal.

Where does the Arnold-Chiari II Malformation occur?

Occurs bet C1-C4 (may extend to T1)

There is a widely accepted theory where series of interrelated, time-dependent defects occur during the embryonic development of the primitive ventricular system. What is this called?

→ Chiari II Malformation 1st before hydrocephalus

This is a neurological structure that is:

■ unable to accommodate the hindbrain and brainstem structures → abn positioning

■ 25% of neonates with spina bifida - head circumferences measuring below the fifth percentile

Small posterior fossa

This is a neurological structure that is unable to accommodate the hindbrain and brainstem structures → abn positioning

Small posterior fossa

This is a neurological structure where 25% of neonates with spina bifida - head circumferences measuring below the fifth percentile

Small posterior fossa

What are the cardinal features of Arnold-Chiari II Malformation?

Cardinal Features

■ Myelomeningocele

■ venting of the intracranial CSF

■ hypoplasia of the posterior fossa

■ herniation of the hindbrain

■ compressive damage to cranial nerve

What neurological impairment is this?

○ pathological fixation of the spinal cord

○ abnormal caudal location

○ produces mechanical stretch, distortion, and ischemia with daily activities, growth, and development

● Tethered Spinal Cord

What neurological impairment is this?

○ pathological fixation of the spinal cord

● Tethered Spinal Cord

What neurological impairment is this?

○ abnormal caudal location

● Tethered Spinal Cord

What neurological impairment is this?

○ produces mechanical stretch, distortion, and ischemia with daily activities, growth, and development

● Tethered Spinal Cord

What neurological impairment is this?

• dilatation of the central canal of the spinal cord

• If symptomatic

  • Presents c rapidly progressive scoliosis, a change in strength or coordination of the upper or lower extremities, and spasticity

● Hydromyelia

What neurological impairment is this?

• dilatation of the central canal of the spinal cord

● Hydromyelia

What neurological impairment is this?

• If symptomatic

  • Presents c rapidly progressive scoliosis, a change in strength or coordination of the upper or lower extremities, and spasticity

● Hydromyelia