Watching for Warning Signs and Complications

While most children with myelomeningocele live active and healthy lives, certain complications require prompt medical attention. Because these issues often involve the brain and spinal cord, the most important tool you have is your knowledge of your child’s “baseline”—how they act, move, and use the bathroom when they are well ^[1][2]. Trusting your intuition when you see a change is often the first step in getting the care your child needs.

Emergency Action: If your child exhibits severe symptoms—such as apnea (breathing pauses), severe choking, blue coloring (cyanosis), severe lethargy, unresponsiveness, seizures, or rapidly worsening vomiting and headache—call 911 or go to the nearest emergency department immediately. Do not delay care by waiting for an outpatient neurosurgery appointment. Contact your specialty team in parallel once emergency care is underway.

Shunt Malfunction: The Scan Isn’t Everything

If your child has a Ventriculoperitoneal (VP) Shunt to manage hydrocephalus, you must be alert for signs that it is no longer draining fluid correctly. A common and dangerous misconception is that a “normal” or “unchanged” brain scan (CT or MRI) means the shunt is working. In reality, a child can have a life-threatening shunt malfunction with little to no change in the size of their brain’s fluid spaces on imaging ^[3].

Symptomatic worsening should always be taken seriously, regardless of what the scan shows ^[1][4].

• Warning Signs in Infants: A bulging or tense fontanelle, unusual irritability, repeated vomiting, or a rapidly increasing head circumference ^[5][6].
• Warning Signs in Children: Persistent headaches, new or worsening vomiting, extreme sleepiness (lethargy), or changes in vision or personality ^[5][7].
• Infection Signs: Fever, redness along the shunt track (the tubing under the skin), or drainage from the surgical sites ^[8].

Chiari II Malformation: Urgent Red Flags

Almost all children with myelomeningocele have a Chiari II Malformation visible on imaging. While many have no symptoms, it can occasionally compress the brainstem, which controls vital functions ^[9][10]. Go to the emergency department for:

• Stridor: A high-pitched, noisy sound when the child breathes in ^[6][10].
• Apnea or Bradycardia: Pauses in breathing (especially during sleep) or an unusually slow heart rate ^[10][11].
• Dysphagia: Significant difficulty swallowing, frequent choking, or “wet-sounding” vocalizations after eating ^[10].

Tethered Cord Syndrome: Watching for Change

As a child grows, the spinal cord can sometimes become stuck or “tethered” to the surrounding tissue. This stretches the cord and causes gradual nerve damage ^[12]. Interval clinical deterioration (a change from a previous state) is the most important clue ^[2]. Signs requiring specialty evaluation include:

• Movement Changes: New or progressive weakness in the legs, a change in how the child walks (gait), or new foot deformities ^[2][13].
• Bladder/Bowel Decline: A child who was previously continent begins having new accidents, or urodynamic tests show higher pressures ^[2][12].
• Orthopedic Changes: Sudden or rapid worsening of scoliosis ^[12].

Recognizing Urinary Tract Infections (UTIs)

Diagnosing a UTI in a child with a neurogenic bladder requires careful balance. Many children with spina bifida have asymptomatic bacteriuria—meaning bacteria live in their bladder without causing an infection ^[14][15]. Treating these bacteria when the child isn’t sick leads to antibiotic resistance ^[14]. A standard urinalysis is often “positive” even when they are healthy.

However, if a child is febrile (has a high fever) or appears systemically ill (toxic), do not delay empiric antibiotics.

• The Best Practice: When feasible, doctors should obtain a properly collected formal urine culture before giving antibiotics.
• When to Treat: The culture results, combined with clinical symptoms like fever, new incontinence, malaise, or flank pain, guide whether the child needs UTI treatment ^[16][17].

Creating an Emergency Plan

Ask your multidisciplinary team for a written Emergency Protocol Card that you can hand to ER doctors unfamiliar with spina bifida ^[18]. It should include your child’s baseline neurologic status, shunt type (and programmable setting), a bold latex allergy warning, and direct contact numbers for their neurosurgeon and urologist.