10.4 Neural Tube Defects: Myelomeningocele, Encephalocele & Transport of the Newborn With an Open Spinal Defect
Key Takeaways
An open myelomeningocele is covered with a sterile, non-adherent dressing moistened with warm saline and then plastic wrap, and the infant is kept prone or side-lying so nothing presses on the sac.
Infants with myelomeningocele are managed with latex precautions from birth because of their high risk of latex sensitization.
The Chiari II malformation found in most infants with myelomeningocele can cause stridor from vocal cord paralysis, apnea, and swallowing dysfunction, so the airway and breathing pattern are watched closely.
Hydrocephalus is common; serial head circumference, fontanelle tension, and signs of increased intracranial pressure are monitored and handed off.
Surgical closure is usually performed within about 48–72 hours to reduce infection risk, so the transport goal is to protect the sac and neural tissue, prevent infection and hypothermia, and deliver the infant to a neurosurgical center promptly.
Neural Tube Defects: Myelomeningocele & Encephalocele
Neural tube defects (NTDs) result from failed closure of the neural tube in the first month of gestation, often before a pregnancy is recognized. Folic acid supplementation before conception and in early pregnancy substantially reduces risk. That is why the U.S. Public Health Service recommends 0.4 mg daily for all people who could become pregnant, with higher doses after a previously affected pregnancy.
Types of Neural Tube Defects
| Defect | Description | Transport relevance |
|---|---|---|
| Myelomeningocele (open spina bifida) | Spinal cord and meninges protrude through a vertebral defect, usually lumbosacral; the sac may be intact or leaking CSF | Most common transport NTD; sac protection, infection prevention, neurosurgical closure |
| Meningocele | Meninges and CSF herniate without neural tissue | Better neurological outcome; still protect the sac |
| Encephalocele | Brain tissue and meninges protrude through a skull defect, often occipital | Protect the sac, support the airway, and position to avoid pressure |
| Anencephaly | Absence of the cranial vault and most of the brain; not survivable | Comfort-focused care with the family; transport decisions guided by goals of care (Section 1.3) |
| Spina bifida occulta | Skin-covered vertebral defect, sometimes with a dimple, hair tuft, or lipoma | Not an emergency |
Stabilizing and Packaging an Open Myelomeningocele
- Position prone or side-lying. Nothing should press on the sac. Support the hips and legs in a neutral position (hip dislocation and clubfoot are common associated findings).
- Cover the defect with a sterile, non-adherent dressing moistened with warm sterile saline, then cover with plastic wrap or a sterile bowel bag to limit evaporation and heat loss. Avoid dry gauze, which sticks to the placode, and avoid antiseptics that could injure neural tissue.
- Keep stool away from the sac. A plastic drape taped below the defect toward the buttocks acts as a barrier.
- Prevent hypothermia. The exposed defect and moist dressing increase heat loss. Use a pre-warmed incubator and servo control (Section 3.4).
- Watch for CSF leak. A leaking sac increases the risk of meningitis and ventriculitis. Many centers start antibiotics when the sac is open or leaking. Follow medical control.
- Latex precautions from birth. Infants with spina bifida have a high risk of latex allergy. Use latex-free gloves, catheters, and equipment.
- Avoid rectal temperatures and rectal manipulation near a lumbosacral defect when possible, and record lower-extremity movement and anal tone as a baseline neurological examination.
Associated Problems to Anticipate
- Chiari II malformation: Most infants with myelomeningocele have downward displacement of the hindbrain. Brainstem dysfunction can cause stridor from vocal cord paralysis, central apnea, bradycardia, and swallowing dysfunction with aspiration. Monitor breathing continuously, and have a plan for airway support.
- Hydrocephalus: Common, and may be present at birth or develop after closure. Measure head circumference, assess the fontanelle and sutures, and watch for signs of increased intracranial pressure (bulging fontanelle, sunsetting eyes, apnea, bradycardia, vomiting). Many infants later need a ventriculoperitoneal shunt (shunt malfunction is covered in Section 13.2).
- Neurogenic bladder and bowel: Urinary retention with risk of renal injury. Monitor urine output. Clean intermittent catheterization may be needed.
- Orthopedic findings: Hip dislocation, clubfoot, and contractures. Position gently.
Positioning Challenges in Transport
A prone infant is harder to assess and intubate. Before departure:
- Secure the airway plan. If intubation is needed, the infant can be placed supine briefly on a doughnut-shaped support or rolled towels that keep pressure off the sac.
- Make sure monitoring leads, pulse oximetry, and IV access work in the prone position.
- Pad pressure points and keep the head in a neutral position.
Surgery, Timing, and the Family
- Closure timing: Postnatal closure is usually performed within about 48–72 hours of birth to reduce infection risk. Transport should not be delayed for non-urgent tests.
- Prenatal (fetal) repair: Some infants have had in-utero closure after the Management of Myelomeningocele Study (MOMS) showed reduced need for shunting and improved motor function. These infants are often born preterm and need preterm care.
- Family communication: Parents may have known the diagnosis prenatally or be learning it for the first time. Explain the plan clearly and involve them before departure (Section 2.4).
Encephalocele Specifics
Occipital encephaloceles can be large. Support the sac in a padded ring, keep the infant side-lying, and protect the airway. Large lesions may make positioning for intubation very difficult. These infants may also have hydrocephalus and associated syndromes.
Pre-Departure Checklist for an Open Neural Tube Defect
| Item | What to confirm |
|---|---|
| Sac | Intact or leaking; size and location; dressing moist, non-adherent, and covered with plastic; no pressure from positioning aids |
| Neurological baseline | Spontaneous leg movement, response to touch, anal wink, head circumference, fontanelle tension |
| Airway and breathing | Stridor, apnea, or weak cry (Chiari II); airway plan for the prone infant |
| Thermal status | Pre-warmed incubator, skin probe away from the defect, temperature trend |
| Fluids and glucose | NPO; IV dextrose-containing maintenance fluid; glucose check (Section 9.3) |
| Infection | Antibiotics per medical control if the sac is open or leaking; time of first dose |
| Urine | Voiding or bladder distension; catheterize with latex-free supplies if retention is present |
| Latex | Latex-free equipment in the transport bag and a latex alert on the handoff |
| Family | Parents informed, questions answered, contact details for the neurosurgical center |
This checklist doubles as the handoff structure at the receiving unit, where the neurosurgical team will compare its examination with the transport baseline.
Antibiotics, Fluids, and Nutrition
- Many centers give broad-spectrum antibiotics (for example, ampicillin plus gentamicin) when the defect is open or leaking CSF, because meningitis and ventriculitis are the main early threats. Follow medical control and document the time of the first dose.
- Keep the infant NPO before surgery with IV dextrose-containing fluids. Watch fluid and heat losses from the moist dressing.
- Do not delay transport for imaging. Ultrasound of the head and kidneys and MRI are done at the receiving center.
A newborn has an open lumbosacral myelomeningocele with a thin, intact sac. How should the transport team dress and position the infant?
Supine with dry sterile gauze taped firmly over the sac
Supine with povidone-iodine-soaked gauze to prevent infection
Prone or side-lying, with a sterile non-adherent dressing moistened with warm saline covered by plastic wrap
Prone with the sac left uncovered to allow inspection
Which precaution should be started from birth for an infant with myelomeningocele?
Contact isolation for MRSA
Avoidance of all intravenous fluids
Airborne isolation
Latex-free equipment and gloves
During transport, an infant with myelomeningocele develops inspiratory stridor and brief episodes of central apnea with bradycardia. Which associated condition most likely explains these findings?
Neurogenic bladder with urinary retention
Chiari II malformation causing brainstem dysfunction
Hip dislocation
Latex allergy
Sections you finish are checked off in the contents.