Pediatric Neurological Disorders
Nursing Notes: Spina Bifida & Hydrocephalus
Comprehensive study guide for nursing students on congenital neurological conditions, their pathophysiology, assessment, and nursing management.
Table of Contents
Spina Bifida
A congenital neural tube defect affecting the spinal cord
Introduction
Spina bifida is a congenital neural tube defect that occurs during the first month of embryonic development. The condition is characterized by incomplete closure of the vertebral column and neural tube, leading to varying degrees of spinal cord and nerve damage.
Key Facts
- Occurs in approximately 1 in 1,500-2,000 births worldwide
- More common in females than males
- Risk factors include maternal folate deficiency, family history, and certain medications
- Preventable in up to 70% of cases with adequate folic acid supplementation before and during early pregnancy
Neural Tube Formation and Defects
Pathophysiology
Spina bifida results from the failure of the neural tube to close completely during embryonic development, typically between days 21-28 after conception. This leads to malformation of the vertebrae and, depending on the type, exposure of the spinal cord and/or meninges.
NEURAL Mnemonic for Pathophysiology
- Neural tube closure failure (days 21-28)
- Embryonic development disruption
- Unfused vertebral arches
- Risk factors: folate deficiency, genetics, environment
- Abnormal spinal cord/nerve development
- Lumbar/sacral regions most commonly affected
The condition develops due to a combination of genetic and environmental factors. Key factors include:
- Folate Deficiency: Inadequate folic acid before and during early pregnancy
- Genetic Factors: Family history increases risk by 3-5%
- Medications: Anticonvulsants (valproic acid, carbamazepine)
- Maternal Diabetes: Increases risk 2-10 fold
- Hyperthermia: Maternal fever or hot tub use during early pregnancy
- Obesity: BMI > 30 increases risk by approximately 30%
Pathophysiological Cascade in Spina Bifida
Types & Classification
Spina bifida exists on a spectrum of severity and is classified into several types based on the extent of the defect and tissues involved:
| Type | Description | Presentation | Severity |
|---|---|---|---|
| Spina Bifida Occulta | Gap in one or more vertebrae without spinal cord involvement | Often asymptomatic; may have dimple, tuft of hair, or birthmark over affected area | Mild; often incidental finding |
| Meningocele | Meninges protrude through vertebral opening into a sac filled with CSF | Visible sac on back; spinal cord intact with minimal neurological symptoms | Moderate |
| Myelomeningocele | Meninges and spinal cord/nerves protrude through opening | Visible sac containing neural tissue; significant neurological deficits | Severe; most common form |
| Myeloschisis (Rachischisis) | Open neural tube with exposed neural tissue; no protective sac | Open lesion with visible neural plate; severe neurological deficits | Most severe form |
Anatomical Comparison of Spina Bifida Types
Clinical Manifestations
The signs and symptoms of spina bifida vary widely depending on the type and level of the defect. Clinical manifestations range from asymptomatic in mild cases to severe neurological impairments in more extensive defects.
DEFECTS Mnemonic for Clinical Manifestations
- Deficit in motor function (weakness, paralysis)
- Elimination problems (bowel/bladder dysfunction)
- Foot and leg deformities (clubfoot, hip dislocation)
- Exposed neural tissue/meninges (in open defects)
- Cutaneous markers (dimple, hair tuft, hemangioma)
- Tethered cord syndrome (progressive symptoms)
- Sensation abnormalities below lesion level
| System | Clinical Manifestations | Nursing Assessment Focus |
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| Neurological |
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| Genitourinary |
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| Gastrointestinal |
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| Musculoskeletal |
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| Integumentary |
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Diagnosis
Diagnosis of spina bifida can occur prenatally or postnatally through various screening and diagnostic procedures:
Prenatal Diagnosis
- Maternal Serum Alpha-Fetoprotein (MSAFP): Elevated levels at 16-18 weeks may indicate NTDs
- Ultrasound: Can detect about 90% of cases of open spina bifida; typically performed at 18-20 weeks
- Amniocentesis: Measures AFP and acetylcholinesterase in amniotic fluid
- Fetal MRI: For detailed evaluation of fetal anatomy when ultrasound results are inconclusive
Postnatal Diagnosis
- Physical Examination: Visual inspection may reveal sac or cutaneous markers
- Neurological Assessment: Evaluates motor and sensory function
- Imaging Studies:
- X-rays: Show vertebral defects
- MRI: Detailed images of spinal cord and associated anomalies
- CT scan: Evaluates bony defects
- Ultrasound: Assesses spinal cord and related structures
- Urodynamic Studies: Evaluate bladder function
Diagnostic Criteria
The definitive diagnosis of spina bifida is based on:
- Visual confirmation of the defect (open spina bifida)
- Radiographic evidence of vertebral anomalies
- Neurological examination findings consistent with the level of the defect
- Associated findings such as Chiari II malformation, hydrocephalus, or tethered cord
Treatment
Management of spina bifida requires a multidisciplinary approach and depends on the type and severity of the defect. Treatment goals include preserving neurological function, preventing complications, and maximizing quality of life.
Surgical Interventions
- Prenatal Surgery:
- Fetal surgery between 19-25 weeks gestation
- Reduces need for VP shunt
- Improves motor outcomes
- Reduces Chiari II malformation
- Postnatal Closure:
- Typically performed within 24-48 hours after birth
- Closes defect to prevent infection and further damage
- May involve neurosurgery and plastic surgery techniques
- Ventriculoperitoneal (VP) Shunt: For hydrocephalus management
- Tethered Cord Release: If symptomatic tethering occurs
- Orthopedic Procedures: For scoliosis, joint contractures, or foot deformities
Medical Management
- Neurological Care: Monitoring and management of neurological status
- Urological Management:
- Clean intermittent catheterization
- Anticholinergic medications
- Prophylactic antibiotics
- Urological surgery if needed
- Bowel Management:
- Dietary modifications
- Medications for constipation
- Bowel training programs
- Orthotic Devices: Braces, splints, adaptive equipment
- Rehabilitation: Physical, occupational, and speech therapy
Multidisciplinary Care for Spina Bifida
Nursing Management
Nursing care for patients with spina bifida is comprehensive and focused on holistic management of the condition and its complications. The nursing process follows the standard assessment, diagnosis, planning, implementation, and evaluation framework.
SPINA Mnemonic for Nursing Priorities
- Skin integrity preservation
- Prevention of infection and complications
- Integration of multidisciplinary care
- Neurological status monitoring
- Assistance with activities of daily living
1. Nursing Assessment
Physical Assessment
- Complete neurological assessment
- Assess motor and sensory function below defect level
- Head circumference measurement for hydrocephalus
- Defect site examination (if visible)
- Skin integrity assessment
- Musculoskeletal assessment for deformities
- Urinary and bowel function assessment
- Growth and development evaluation
Psychosocial Assessment
- Family coping and adaptation
- Parent-child bonding
- Support systems
- Knowledge about condition and care requirements
- Economic resources
- Home environment assessment
- Impact on siblings
- School/social integration
2. Nursing Diagnosis
- Risk for infection related to neural tube defect
- Impaired physical mobility related to neurological impairment
- Risk for impaired skin integrity related to immobility and sensory deficits
- Urinary elimination, impaired related to neurogenic bladder
- Bowel incontinence related to neurogenic bowel
- Risk for injury related to sensory/perceptual alterations
- Delayed growth and development related to physical disability
- Deficient knowledge (parental) related to home care management
- Compromised family coping related to having a child with chronic condition
- Risk for latex allergy response related to repeated exposure
3. Nursing Interventions
Preoperative Care (Newborn with Open Defect)
- Position prone or side-lying to prevent pressure on defect
- Maintain sterile, moist saline dressing over defect
- Monitor for signs of infection or CSF leakage
- Implement latex precautions from birth
- Monitor neurological status for changes
- Assess head circumference every 4 hours
- Support parent-infant bonding while protecting defect
Postoperative Care
- Monitor vital signs per protocol
- Maintain proper positioning (per surgical orders)
- Assess surgical site for healing, infection, drainage
- Monitor neurological status for changes
- Provide pain management
- Monitor for signs of increased intracranial pressure
- Initiate feeding as ordered; monitor tolerance
Ongoing Care
Neurological Management
- Regular assessment of neurological status
- Monitor for signs of shunt malfunction or infection
- Assess for signs of tethered cord
- Document developmental milestones
Skin Care
- Regular skin assessments, especially pressure points
- Implement pressure-relieving measures
- Teach weight shifting techniques
- Educate on the importance of routine skin checks
Urinary Management
- Teach clean intermittent catheterization (CIC)
- Monitor for UTI signs
- Ensure adequate hydration
- Implement timed voiding program if appropriate
Bowel Management
- Establish bowel program
- Educate on diet and fluid intake
- Teach manual stimulation techniques
- Administer medications as prescribed
Family Education and Support
- Teach all aspects of home care
- Provide information about the condition and expected outcomes
- Connect family with support resources
- Educate about early signs of complications
- Assist with school integration planning
- Provide guidance on promoting independence
- Teach latex precautions
4. Expected Outcomes & Evaluation
- Patient maintains skin integrity without breakdown
- Patient/family demonstrates proper wound care techniques
- Patient/family correctly performs clean intermittent catheterization
- Patient maintains urinary elimination without infection
- Patient establishes regular bowel elimination pattern
- Patient demonstrates age-appropriate developmental progress
- Patient utilizes adaptive equipment appropriately
- Family verbalizes understanding of condition and management
- Family identifies and accesses appropriate community resources
- Patient participates in age-appropriate activities with adaptations as needed
Ongoing evaluation should be conducted at regular intervals to assess progress toward these outcomes and adjust interventions as needed.
Key Nursing Responsibilities
- Coordination of Care: Serve as liaison between different specialists
- Education: Provide comprehensive teaching to patient and family
- Advocacy: Advocate for patient’s needs within healthcare system and community
- Prevention: Focus on preventing complications through vigilant assessment and early intervention
- Support: Provide emotional support and connect families with resources
- Empowerment: Promote independence and self-management appropriate to developmental stage
Hydrocephalus
Abnormal accumulation of cerebrospinal fluid (CSF) within the ventricles of the brain
Introduction
Hydrocephalus is a condition characterized by an abnormal accumulation of cerebrospinal fluid (CSF) within the ventricles of the brain, leading to increased intracranial pressure and potential brain damage. The term “hydrocephalus” comes from the Greek words “hydro” (water) and “cephalus” (head).
Key Facts
- Occurs in approximately 1-2 per 1,000 births
- Can be congenital or acquired
- Often associated with neural tube defects (70-90% of myelomeningocele cases)
- Can occur at any age, including adults and elderly
- Treatment typically involves surgical intervention
- Outcomes vary widely depending on cause, timing of intervention, and other factors
Normal CSF Circulation vs. Hydrocephalus
Pathophysiology
Hydrocephalus results from an imbalance between the production and absorption of cerebrospinal fluid (CSF), leading to accumulation within the ventricular system. In normal physiology, CSF is produced by the choroid plexus in the ventricles, circulates through the ventricular system, and is absorbed into the bloodstream through arachnoid granulations.
FLUID Mnemonic for Pathophysiology
- Flow obstruction of CSF pathways
- Lack of absorption at arachnoid villi
- Unbalanced production vs. absorption
- Increased intracranial pressure results
- Damage to brain tissue from pressure
Pathophysiological mechanisms involved in hydrocephalus include:
Overproduction of CSF
- Rare cause
- Usually due to choroid plexus papilloma
- Normal production: 0.35-0.40 mL/min
- Total volume: 125-150 mL in adults
Obstruction of CSF Flow
- Most common cause
- Can occur at foramina of Monro, aqueduct of Sylvius, or outlets of fourth ventricle
- Due to congenital malformations, tumors, hemorrhage, infection
Defective Absorption
- Impaired function of arachnoid villi
- Can result from subarachnoid hemorrhage, meningitis
- Scarring or fibrosis of subarachnoid space
Pathophysiological Cascade in Hydrocephalus
Types & Classification
Hydrocephalus can be classified based on several factors, including etiology, flow dynamics, time of onset, and intracranial pressure characteristics.
Classification of Hydrocephalus
| Classification | Types | Description | Common Causes |
|---|---|---|---|
| Based on CSF Flow | Non-communicating (Obstructive) |
Blockage within the ventricular system preventing CSF flow between ventricles |
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| Communicating | CSF flows freely between ventricles but is blocked after exiting the ventricular system or inadequately absorbed |
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| Based on Onset | Congenital | Present at birth due to genetic factors, developmental disorders, or intrauterine infections |
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| Acquired | Develops after birth due to injury, disease, or other factors |
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| Based on Pressure | High Pressure | Increased intracranial pressure with enlarged ventricles |
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| Normal Pressure (NPH) | Ventricular enlargement with intermittently elevated pressure; common in elderly |
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Special Types
- Ex-vacuo Hydrocephalus: Ventricular enlargement due to loss of brain tissue (not true hydrocephalus)
- Arrested Hydrocephalus: Compensated state where CSF production and absorption reach equilibrium
- Multiloculated Hydrocephalus: Multiple isolated CSF compartments requiring complex treatment
- Benign External Hydrocephalus: Enlargement of subarachnoid spaces with normal ventricles in infants
Clinical Manifestations
The signs and symptoms of hydrocephalus vary significantly based on age of onset, cause, and progression rate. Clinical presentations differ markedly between infants, children, and adults.
HYDRO Mnemonic for Infant Clinical Signs
- Head circumference increased (macrocephaly)
- Yielding or bulging fontanelles
- Dilated scalp veins
- Reflexes abnormal (especially eyes: “setting sun” sign)
- Observable irritability and high-pitched cry
Infants
- Rapid head growth (crossing percentiles)
- Bulging fontanelle
- Separated sutures
- Prominent scalp veins
- “Setting sun” sign (downward gaze with visible sclera above iris)
- High-pitched cry
- Irritability
- Poor feeding
- Vomiting
- Lethargy
- Seizures
Children
- Headache (worse in morning or with position change)
- Nausea and vomiting
- Blurred or double vision
- Unsteady gait
- Poor coordination
- Irritability
- Drowsiness
- Personality changes
- Declining school performance
- Papilledema
- Increased head circumference (in younger children)
Adults
- Headache
- Nausea and vomiting
- Lethargy
- Visual disturbances
- Balance problems
- Cognitive impairment
- Urinary incontinence (especially in NPH)
- Gait disturbances (magnetic gait in NPH)
- Memory problems
- Altered level of consciousness
Normal Pressure Hydrocephalus (NPH) Triad
NPH in adults presents with a classic triad of symptoms:
- Gait disturbance: Wide-based, slow, shuffling (“magnetic gait”)
- Urinary incontinence: Initially urgency, progressing to incontinence
- Cognitive decline: Memory impairment, psychomotor slowing, apathy
Remember with mnemonic “WET”: Walking, Encephalopathy, Toileting
Clinical Signs of Hydrocephalus in Infants
Diagnosis
Diagnosing hydrocephalus involves a combination of clinical assessment, physical examination, and imaging studies to confirm ventricular enlargement and identify the underlying cause.
Clinical Assessment
- History:
- Growth and developmental milestones
- Onset and progression of symptoms
- Birth history
- Family history
- Associated conditions
- Physical Examination:
- Head circumference measurement (plot on growth chart)
- Fontanelle palpation
- Cranial suture assessment
- Neurological examination
- Developmental assessment
- Ophthalmologic examination
Diagnostic Imaging
- Ultrasound:
- Primary tool for infants with open fontanelles
- Can be used serially to monitor progression
- Non-invasive and radiation-free
- CT Scan:
- Quickly identifies ventricular enlargement
- Can detect obstructions, hemorrhage, tumors
- Often first-line in emergency situations
- MRI:
- Provides detailed images of brain structure
- Best for identifying cause of obstruction
- Can assess CSF flow with special sequences
- Preferred for surgical planning
| Specialized Tests | Description | Clinical Application |
|---|---|---|
| Lumbar Puncture | Measurement of CSF pressure and analysis of CSF composition |
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| CSF Flow Studies | Special MRI sequences to visualize CSF flow patterns |
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| Intracranial Pressure Monitoring | Continuous measurement of ICP via implanted device |
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| Cisternography | Radioactive tracer injected into CSF to track flow |
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| Large-Volume Lumbar Tap | Removal of 30-50 mL of CSF followed by symptom assessment |
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Diagnostic Considerations
- Differential Diagnosis: Benign macrocephaly, subdural effusion, brain atrophy, other causes of increased ICP
- Radiographic Features: Ventricular enlargement out of proportion to sulcal enlargement
- Comorbid Assessment: Screen for associated anomalies, especially with congenital hydrocephalus
- Serial Monitoring: Regular head circumference measurements in infants, imaging studies at intervals
Treatment
Treatment of hydrocephalus aims to reduce intracranial pressure, relieve symptoms, and address the underlying cause when possible. Surgical intervention is the mainstay of therapy, though medical management may be appropriate in specific situations.
Ventriculoperitoneal (VP) Shunt System
Surgical Management
- CSF Shunting Procedures:
- Ventriculoperitoneal (VP) Shunt: Most common; drains CSF from ventricle to peritoneal cavity
- Ventriculoatrial (VA) Shunt: Drains to right atrium of heart
- Ventriculopleural Shunt: Drains to pleural cavity
- Lumboperitoneal Shunt: Used for communicating hydrocephalus
- Endoscopic Third Ventriculostomy (ETV):
- Creation of alternative CSF pathway
- Bypasses obstruction by creating hole in floor of third ventricle
- Avoids shunt dependence and complications
- Best for obstructive hydrocephalus
- Choroid Plexus Cauterization:
- Reduces CSF production
- Sometimes combined with ETV
- Used in selected pediatric cases
Medical Management
- Acetazolamide & Furosemide:
- Reduce CSF production
- Temporary measure only
- Limited efficacy
- May be used in premature infants with posthemorrhagic hydrocephalus
- Serial Lumbar Punctures:
- Temporary relief of pressure
- Limited use in posthemorrhagic hydrocephalus
- Bridge to definitive treatment
- Treatment of Underlying Cause:
- Antibiotics for infection
- Tumor treatment
- Management of hemorrhage
- Ventricular Access Device:
- Reservoir placed for repeated access
- Used in premature infants
- Temporary measure before definitive treatment
Shunt Complications
Mechanical Complications
- Obstruction (most common)
- Disconnection
- Catheter migration
- Fracture of tubing
- Valve malfunction
- Over/under drainage
Infectious Complications
- Shunt infection (5-15% risk)
- Ventriculitis
- Meningitis
- Abdominal infection/peritonitis
Other Complications
- Subdural hematoma (overdrainage)
- Slit ventricle syndrome
- Seizures
Signs of Shunt Malfunction
Remember with mnemonic SHUNT:
- Symptoms of increased ICP (headache, vomiting)
- High fever (infection)
- Unusual behavior or mental status changes
- Neurological deterioration
- Tension along shunt tract or at incision sites
Nursing Management
Nursing care for patients with hydrocephalus requires comprehensive assessment, monitoring, and interventions throughout all phases of treatment. The nursing process should address both the physiological and psychosocial aspects of care.
DRAIN Mnemonic for Nursing Priorities
- Detect early signs of increased ICP or complications
- Regular neurological assessments
- Avoid infection through meticulous technique
- Instruct patient/family on management and follow-up
- Note developmental progress and provide support