SPINA BIFIDA

Case Study
 INTRODUCTION

Spina bifida is a birth defect where the spine

doesn't close properly during early
development. Spina bifida is a congenital
condition characterized by incomplete closure
of the spinal column during early fetal
development.

Spina bifida is typically classified into three

main types: occulta, meningocele, and
myelomeningocele. Among its different forms,
meningocele and syringomyelia are complex
problems affecting the nervous system.
Introduction
This case is about a patient with meningocele from T4 to T6) and
syringomyelia from T4 to T9. The patient also has obstructive hydrocephalus
because of a Chiari II malformation (a problem where the lower part of the
brain is squeezed into the spinal canal). He was admitted in the neuro female
ward with a chief complaint of headache and increase in the head
circumference.

Our patient belongs to spina bifida cystica with meningocele. Spina bifida
cystica refers to a form of spina bifida where there is a visible sac or cyst
protruding from the back. In the case of meningocele, this sac contains the
meninges but not the spinal cord or nerve roots.

Final Diagnosis: Meningocele T4 - T6 with Syringomyelia T4 - T9, Obstructive

Hydrocephalus Secondary to Chiari II Malformation
 ETIOLOGY

PREDISPOSING FACTORS PRECIPITATING FACTORS

● Age ● Nutrition
● Heredity ● Anticonvulsants
● Genetic Susceptibility ● Diabetes
● Unknown ● Obesity
● Increase body
temperature
PATHOPHYSIOLOGY

PREDISPOSING PRECIPITATING
FACTORS FACTORS
● Age ● Nutrition
● Heredity 1st Month of ● Anticonvulsants
● Genetic ● Obesity
Susceptibility ● Diabetes
● Unknown Central Nervous
System Begins ● Increase body
temperature

Defect in the spinal

Defect in the
closure of the
neural tube
 Protruding sac
through the defect
Dx:
● Transillumination Meningocele
● CT scan

If treated: If not

Surgical repair of No direct flow of

VP shunt CSF to the spinal
cord

Good Obstruction of fluid

in the brain

CSF unable to
circulate
 s/sx
● Increased ICP Hydrocephalus Accumulation of
● Increase head CSF in the brain
Circumference
● Visual Problem
Fluid may possibly
● Seizures
forced

Displacement of
If treated: foramen
If not

VP Chiari II
Fluid still
accumulated
s/sx:
GOOD Shunt
● Headache
complication
Learning disabilities ● Muscle weakness
● Increased ICP
s/sx: ● Nausea
● Headache Mental retardation ● dizziness
● Nausea and vomiting
● fever
 If not treated

Compression of the
spinal

s/sx:
● Choking
● Arm stiffness
● Difficulty in
feeding and
swallowing
SIGNS AND SYMPTOMS

• Weakness of four limbs, loss of feeling, or trouble moving

body parts below the level of the myelomeningocele or
uncoordinated muscle movements
• Problems with bladder (pee) and bowel (poop) control
• Too much spinal fluid in the brain (hydrocephalus)
• Problem with how the brain is formed (Chiari malformation)
• Learning problems or delayed developmental milestones
• Vision problems
• Seizures
RISKS

● Neurological Impairment
● Surgical Risks
● Infection
● Musculoskeletal Deformities
● Bladder and Bowel Dysfunction
● Pressure Ulcers
● Respiratory Complications
● Chronic Pain
MANAGEMENT
Medical

● Neurological Assessment - Monitor for changes in symptoms, motor

function, sensory deficits and signs of disease progression.
● Pain management - Manage chronic pain associated associate with
syringomyelia or musculoskeletal problems by pharmacological
interventions, physical therapy and psychological interventions.
● Respiratory Monitoring - Assessment for respiratory dysfunction and
management of complications such as sleep apnea, hypoventilation or
respiratory failure.
MANAGEMENT
Surgical

Endoscopic Endonasal Approach (EEA). This innovative, minimally invasive

technique uses the nose and nasal cavities as natural corridors to access
hard-to-reach or previously inoperable tumors or lesions. Benefits of EEA
include:
● no incisions to heal
● no disfigurement
● faster recovery time
EEA has proven safe and effective in children. More than 100 children have
been treated by UPMC skull base surgeons using EEA — more than at any
other neurosurgery center in the world.
 MANAGEMENT
Surgical
MRI- Using this test, a physician can determine if there is a syrinx in your spine
or another abnormality, such as a tumor. In some cases, multiple MRI images may
be taken in rapid succession (called dynamic MRI) to show the flow of fluid
around the spinal cord and within the syrinx. You might receive an injection of a
dye or contrast agent to enhance the MRI images.

Ventriculoperitoneal (VP) shunt- drains excess cerebrospinal fluid (CSF) when

there is an obstruction in the normal outflow or there is a decreased absorption
of the fluid.
Electroencephalogram (EEG)- Records the electrical activity of the brain through
a series of electrodes on the scalp.
- Used to diagnose and evaluate seizures disorders identify tumors, brain
abscesses or infections and to confirm of brain death
 MANAGEMENT
Nursing
- Provide detailed information to the parents or caregivers about the
condition, the planned surgical intervention, potential complications, and the
importance of follow-up care.
- Monitor vital signs, neurological status, and other relevant parameters during
the surgery.
- Emphasize strict aseptic technique to prevent infection, as the meninges are
exposed during the procedure
- Perform frequent neurological assessments to detect any changes in motor
or sensory function. Report any abnormalities promptly.
- Provide education on long-term care, potential complications, and the
importance of regular follow-up appointments with healthcare providers.
 MANAGEMENT
Nursing
- Provide detailed information to the parents or caregivers about the
condition, the planned surgical intervention, potential complications, and the
importance of follow-up care.
- Monitor vital signs, neurological status, and other relevant parameters during
the surgery.
- Emphasize strict aseptic technique to prevent infection, as the meninges are
exposed during the procedure
- Perform frequent neurological assessments to detect any changes in motor
or sensory function. Report any abnormalities promptly.
- Provide education on long-term care, potential complications, and the
importance of regular follow-up appointments with healthcare providers.
 MANAGEMENT
Pharmacological
These medications are used in conjunction with some form of bladder emptying
technique to prevent upper urinary tract complications and to facilitate social
continence. Among the drugs used are the following:

● Anticholinergics (oxybutynin chloride, hyoscyamine sulfate)

In a 2005 study, researchers determined that anticholinergics delivered in doses
of 0.2 mg/kg/day proved to be effective and quite safe for the treatment of
high-risk urinary problems during infancy for children less than a year old.
Additionally, it can help to improve the preservation of kidney function in children
with severe dysplasia by reflux
 MANAGEMENT
Pharmacological
These medications are used in conjunction with some form of bladder emptying
technique to prevent upper urinary tract complications and to facilitate social
continence. Among the drugs used are the following:

● Anticholinergics (oxybutynin chloride, hyoscyamine sulfate)

In a 2005 study, researchers determined that anticholinergics delivered in doses
of 0.2 mg/kg/day proved to be effective and quite safe for the treatment of
high-risk urinary problems during infancy for children less than a year old.
Additionally, it can help to improve the preservation of kidney function in children
with severe dysplasia by reflux
 MANAGEMENT
Pharmacological
In essence, this class of drug is used to protect kidney functions and upper
urinary tract function as well.
Examples of anticholinergics and their doses for children
➔ Oxybutynin (Uropan© 5 mg susp, tab) 0.2-0.4 mg/kg
➔ Tolterodine (Detrusitol©, Toldin©, Toldex©, 1-2 mg tab, 4 mg long acting tab)
2-4 mg/day 2 doses (0.5-2 mg/day)

● Tricyclic antidepressants (imipramine hydrochloride; may act through

anticholinergic effects)

Tricyclic antidepressants are often used as an alternative to anticholinergics for

SB bladder dysfunction, as such, they are referred to as an off-label form of
medical treatment. Tricyclic antidepressants typically work by increasing
norepinephrine and serotonin levels. However, in relation to SB, they are used for
their anticholinergic and direct muscle relaxant effects on the urinary bladder
 MANAGEMENT
Pharmacological
● Acetazolamide- an effective alternative to shunting by halting progression
of hydrocephalus until such time as sutures can become fibrosed and
spontaneous arrest can occur.
● Furosemide- reduce the production of cerebrospinal fluid, have been
suggested as non‐invasive therapies to reduce hydrocephalus and the need for
ventriculo‐peritoneal (V‐P) shunting.