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Med Surg 1 > MIDTERMS: Spina Bifida & Hydrocephalus > Flashcards

MIDTERMS: Spina Bifida & Hydrocephalus Flashcards

1
Q

What is the second most common childhood disability?

A

Spina bifida (neural tube defect).

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2
Q

When does the failure of neural tube closure occur in spina bifida?

A

On the 28th day of gestation.

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3
Q

What ultrasound markers are associated with spina bifida?

A

Lemon sign: Lemon-shaped cranial vault
Banana sign: Banana-shaped cerebellum

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3
Q

Which environmental factors are associated with spina bifida?

A

Low socioeconomic status
Maternal obesity
Heat exposure (e.g., maternal fever, hot environments)
Exposure to anticonvulsant drugs (Valproic acid, Carbamazepines)

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3
Q

What is the banana sign in spina bifida?

A

The cerebellum assumes a banana shape due to downward traction of the spinal cord in neural tube defects.

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3
Q

What distinguishes Spina Bifida Occulta from other types?

A

There is no herniation of neural elements, and it may present with skin markers like pigmented nevi, hirsute patches, or dimples.

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3
Q

Which CNS malformation is commonly associated with spina bifida?

A

Arnold-Chiari malformations.

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4
Q

What is the difference between meningocele and myelomeningocele?

A

Meningocele: Herniation of meninges filled with CSF, no neural tissue.
Myelomeningocele: Herniation includes dysplastic neural tissue, causing sensorimotor deficits and bowel/bladder dysfunction.

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4
Q

What is the best time to perform an amniocentesis for spina bifida diagnosis?

A

Between 16-18 weeks of gestation.

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4
Q

What is the significance of Alpha-fetoprotein (AFP) in spina bifida diagnosis?

A

Elevated AFP levels in amniotic fluid (13-15 weeks) indicate open neural tube defects due to CSF leakage.

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4
Q

What is the lemon sign, and how is it related to spina bifida?

A

The lemon sign refers to an abnormally shaped fetal head (frontal bones flattened), seen in 98% of open spina bifida cases.

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4
Q

What is Spina Bifida Aperta/Cystica?

A

An open defect where the contents of the spinal cord herniate through the vertebral opening. It includes meningocele (herniation of meninges) and myelomeningocele (herniation of neural tissue and meninges).

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4
Q

What factors contribute to spina bifida in prenatal diagnosis?

A

Genetic predisposition
Environmental influences (e.g., maternal diet, heat exposure)
Maternal obesity
Prenatal drug exposure

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5
Q

What are common presentations of post-neurulation defects?

A

Skin-covered lesions associated with malformations of the conus, filum terminale, or caudal spinal canal tumors.

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5
Q

What is Spina Bifida Occulta?

A

A condition where the vertebrae are affected, but the neural and meningeal elements are not herniated. It occurs with incomplete closure of posterior vertebral structures.

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5
Q

What are the neural tube defects resulting from failed neurulation during the 3rd and 4th weeks after conception?

A

Spina bifida
Craniorachischisis
Anencephaly

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5
Q

What are the warning signs of Spina Bifida Occulta?

A

Pigmented nevus
Angioma
Hirsute patch (faun’s tail)
Dimple or dermal sinus on overlying skin

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5
Q

What occurs during the post-neurulation phase (4th to 7th weeks)?

A

Canalization and retrogressive differentiation of the caudal portion of the spinal cord.

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5
Q

What is the cause of anencephaly?

A

Failure of closure at the cephalic end of the neural tube, resulting in an undeveloped cerebrum and no skull formation.

5
Q

What spinal regions are most commonly affected by myelomeningocele?

A

70-75% affect the lumbar and sacral areas.

5
Q

What are the characteristics of thoracic-level Spina Bifida?

A

Upper extremities are spared.
Weakness in intercostal, abdominal, and back muscles.
Kyphosis and kyphoscoliosis.
Legs may be flaccid but can show spasticity if the distal spinal cord is preserved.

5
Q

What motor and sensory deficits are associated with Spina Bifida?

A

Motor and sensory deficits depend on the level of the spinal cord anomaly.
Motor paralysis is usually of the Lower Motor Neuron (LMN) type.
Neurogenic bowel and bladder dysfunctions.
Joint abnormalities are also common.

6
Q

Which malformation is associated with myelomeningocele?

A

Arnold-Chiari type II malformation.

7
Q

What are the key clinical features of L1-L3 (upper lumbar) Spina Bifida?

A

Hip flexors and adductors are innervated.
Knee extensors are functional but not fully.
Hip flexion and adduction contractures often lead to early paralytic hip dislocation.
Pelvic obliquity and equinus deformity of the foot due to gravity.

8
Q

What deficits are seen with L4-L5 (lower lumbar) Spina Bifida?

A

Normal knee extensor strength.
Weak hip abductors and extensors.
Coxa valga and acetabular dysplasia are common.
Late hip dislocation may occur.
Calcaneus foot position, with no plantar flexion.

9
Q

What are the characteristics of sacral-level Spina Bifida lesions?

A

Stronger plantar flexion with active toe movements.
Intrinsic foot muscles remain weak, leading to Pes Cavus (high arch) and clawing of the toes.

10
Q

What are the associated sensory and physical deficits of Spina Bifida?

A

Partial or complete sensory deficits can lead to skin injuries and pressure sores.
Neurogenic bladder and bowel dysfunctions.
Obesity due to reduced physical activity and muscle paralysis.

10
Q

What are the urinary dysfunctions in patients with Spina Bifida?

A

Neurogenic bladder dysfunction occurs in 95% of patients.
Can present as a spastic or small-capacity bladder, leading to urinary incontinence.
Increased risk of UTI, pyelitis, and pyelonephritis.

10
Q

What is the purpose of a Reciprocating Gait Orthosis in Spina Bifida treatment?

A

A reciprocating gait orthosis (RGO) helps patients with lower extremity paralysis walk by facilitating reciprocal leg movements and improving mobility.

10
Q

What conditions in Spina Bifida patients do NOT require immediate surgery?

A

Complete paralysis of both legs (L1 lesions and above).
Significant hydrocephalus (HC) with an enlarged head at birth.
Severe spinal deformity (e.g., kyphosis or scoliosis).
Serious associated congenital malformations (e.g., congenital heart disease).
Evidence of cerebral birth injury.

10
Q

What is precocious puberty in Spina Bifida patients?

A

Early puberty due to premature activation of the hypothalamic-pituitary-gonadal axis.
Common in 10-20% of patients with meningocele.
Presents as breast and testicular enlargement before ages 8-9.

10
Q

What are the characteristics of Arnold-Chiari Type II Malformation in Spina Bifida patients?

A

Caudal displacement of the medulla, pons, cerebellar vermis, and fourth ventricle into the cervical canal.
Present in 80-90% of patients with meningomyelocele, particularly in lumbar lesions.

10
Q

What causes obesity in children with Spina Bifida?

A

Reduced physical activity due to muscle paralysis.
Decreased lean body mass.
Recommended to reduce caloric intake by 10-20%, with a diet low in fat and carbohydrates, and high in protein and fiber.

10
Q

What are the two types of Hydrocephalus commonly associated with Spina Bifida?

A

Communicating Hydrocephalus: Inability to reabsorb CSF, leading to enlargement of ventricles.
Non-communicating Hydrocephalus: Obstruction of CSF flow due to congenital malformations or mass lesions.

10
Q

What are common symptoms of Hydrocephalus in children with Spina Bifida?

A

Increased head circumference.
Tense anterior fontanelle.
Thin scalp with dilated veins.
Lid retraction and impaired upward gaze.

11
Q

When is the repair of a cystic lesion in Spina Bifida typically performed?

A

The repair of a cystic lesion is typically done on the first day of life. If hydrocephalus (HC) is present, a ventriculoperitoneal shunt (VPS) is inserted, and VPS revision is done every 1-2 years.

12
Q

What common orthopedic problems are seen in Spina Bifida patients?

A

Scoliosis: Seen in 80-90% of patients with thoracic lesions by age 14-15.
Hip dislocation and pelvic obliquity: Common due to paralytic hip subluxation or dislocation.

13
Q

What is hydrocephalus and how is it related to Spina Bifida?

A

Hydrocephalus is the accumulation of excess cerebrospinal fluid (CSF) in the brain, leading to increased intracranial pressure (ICP). It is often associated with Spina Bifida, particularly in patients with Arnold-Chiari Type II malformation.

14
Q

What are the types of hydrocephalus?
Failure to reabsorb CSF by arachnoid granulations, leading to enlarged third ventricles.

A

Communicating Hydrocephalus

15
Q

What are the types of hydrocephalus?
Due to cerebral atrophy with no obstruction in the CSF flow.

A

Hydrocephalus Ex Vacuo

16
Q

What are the types of hydrocephalus?
Obstruction within the ventricular system due to congenital malformations, tumors, or arteriovenous malformations (AVM).

A

Non-communicating Hydrocephalus:

17
Q

What are the causes of Communicating Hydrocephalus (CHCP) in Spina Bifida?

A

Inability to reabsorb CSF by arachnoid granulations (non-obstructive).
Meningeal scarring caused by bacterial meningitis or intraventricular hemorrhage (IVH)

18
Q

What are the causes of Non-communicating Hydrocephalus (NCHCP) in Spina Bifida?

A

Intraventricular obstruction, often due to congenital malformations, tumors, or AVMs that block CSF flow.

19
Q

What are the important precautions for patients with hydrocephalus during therapy?

A

Provide adequate support to the head and neck during transfers.
Avoid prolonged head-down positions.
Ensure therapy goals consider the severity of hydrocephalus and its impact on function

20
Q
A

Med Surg 1 (15 decks)

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