Myelomeningocele in Children: Comprehensive Care and Surgical Interventions

Myelomeningocele is one of the most complex congenital conditions affecting children, requiring a multidisciplinary approach from prenatal diagnosis through lifelong care. As the most severe form of spina bifida, this neural tube defect occurs when the spinal cord and meninges protrude through an opening in the vertebrae, leaving delicate neural tissue exposed. Understanding its management is essential for paediatric nurses who play a crucial role in improving outcomes for these children and supporting their families.

What is myelomeningocele? 🔗

Myelomeningocele is a severe neural tube defect caused by incomplete closure of the spinal cord during early fetal development, typically within the first month of pregnancy. Unlike milder forms of spina bifida, myelomeningocele involves a fluid-filled sac protruding from the baby’s back that contains part of the spinal cord, nerves, and cerebrospinal fluid.

The condition affects approximately 1 in every 2,875 live births in the United States, with Hispanic women having the highest rates of having an affected child. The severity depends largely on where on the spine the defect occurs-the higher up the spinal cord the defect, the more serious the complications may be.

Causes and risk factors 🔗

The etiology of myelomeningocele is multifactorial, involving environmental, maternal, and genetic factors. Environmental influences include exposure to radiation, pollution, pesticides, and certain medications. Maternal factors encompass inadequate nutrition, insufficient folic acid supplementation, consumption of caffeine or alcohol, smoking, and conditions such as diabetes and obesity.

Valproic acid, commonly used to treat epilepsy, migraines, and bipolar disorders, has been linked to increased risk. However, most cases of myelomeningocele are sporadic, occurring without a clear family history.

The role of folic acid in prevention 🔗

Research has demonstrated that folic acid supplementation has a 72% protective effect against neural tube defects. A daily intake of at least 400 micrograms is recommended for all women of childbearing age. Since the introduction of mandatory folic acid fortification in grain products in many countries, scientists have observed a 28% reduction in neural tube defect prevalence.

Neurological dysfunction and associated complications 🔗

Children with myelomeningocele experience significant neurological impairment below the level of the spinal defect. This typically includes paralysis and loss of sensation in the lower limbs, along with bladder and bowel dysfunction due to affected nerve pathways.

Hydrocephalus 🔗

Eight out of ten children with myelomeningocele develop hydrocephalus, an abnormal buildup of cerebrospinal fluid in the brain’s ventricles. This occurs because the normal flow of CSF becomes obstructed, causing increased pressure within the skull. Most affected children require placement of a ventriculoperitoneal shunt-a flexible tube that drains excess fluid from the brain to the abdomen.

Chiari II malformation 🔗

The Chiari II brain malformation affects approximately 90% of children with open spina bifida. In this condition, parts of the cerebellum and brainstem herniate through the opening at the base of the skull into the spinal canal. Symptoms include difficulty swallowing, breathing problems, sleep apnea, and respiratory distress, which may require urgent neurosurgical intervention.

Additional complications 🔗

Children may also develop syringomyelia, where a fluid-filled cyst forms within the spinal cord, causing pain and mobility problems. Tethered spinal cord syndrome, where the cord becomes abnormally attached to surrounding tissues, can lead to progressive neurological deterioration. Orthopaedic issues such as clubfoot, hip dislocation, and scoliosis are common and require ongoing management.

Surgical interventions 🔗

Surgery is essential for closing the spinal defect and preventing further damage or infection. Two main approaches exist: prenatal (before birth) and postnatal (after birth) repair.

Prenatal surgical repair 🔗

Fetal myelomeningocele repair is typically performed between 19 and 26 weeks of gestation. During this procedure, surgeons open the uterus and close the opening in the baby’s back while still in the womb, protecting the spinal cord from further damage by amniotic fluid.

The Management of Myelomeningocele Study (MOMS) demonstrated that prenatal surgery significantly decreases the need for shunt placement and improves motor outcomes, with function improved at least two levels beyond what would be expected based on the anatomical level of the defect. However, prenatal surgery carries a risk of preterm delivery, with nearly 13% of participants delivering before 30 weeks.

Postnatal surgical repair 🔗

When prenatal surgery is not possible or chosen, closure of the myelomeningocele is performed immediately after birth if cerebrospinal fluid leakage is present, or within the first 24-48 hours otherwise. The procedure involves placing the exposed neural tissue back into the spinal canal and achieving watertight closure of the dura, fascia, and skin layers.

Nursing care: preoperative management 🔗

Highly skilled nursing care is essential from the moment of diagnosis. Before surgery, the primary goals are protecting the exposed neural tissue, preventing infection, and maintaining the infant’s stability.

Positioning and protection 🔗

The neonate must be positioned to avoid pressure on the lesion. Careful handling is essential to reduce damage to the exposed spinal cord. The sac should be covered with sterile saline-soaked gauze to keep the tissue moist and protected. Nurses should observe the sac for any signs of leakage or rupture.

Thermoregulation 🔗

The neonate with myelomeningocele is at increased risk of hypothermia due to the immaturity of their thermoregulatory system, inability to generate heat from muscle movement in the lower limbs, and evaporative heat loss from the exposed lesion. Using beanies and booties while keeping the lesion area appropriately covered helps maintain body temperature.

Latex-free environment 🔗

Most patients with spina bifida are sensitive to latex, which can trigger life-threatening anaphylaxis. All equipment, gloves, and supplies used must be strictly latex-free from the very first contact with the infant.

Neurological assessment 🔗

Nurses should perform careful baseline neurological assessments, including observing movement and response to stimuli in the lower extremities. Head circumference must be measured regularly to monitor for developing hydrocephalus, and fontanelles should be examined for signs of increased intracranial pressure.

Nursing care: postoperative management 🔗

After surgical repair, nursing care focuses on wound healing, preventing complications, and monitoring for deterioration.

Positioning and wound care 🔗

Postoperatively, the patient should be managed in a prone position with the head lowered and pelvis elevated. This positioning reduces pressure on the surgical site and helps prevent cerebrospinal fluid accumulation. Meticulous wound care with regular assessment for signs of infection, dehiscence, or CSF leakage is essential.

Monitoring for complications 🔗

Nurses must watch for signs of infection at the surgical site, including redness, swelling, or discharge. Postoperative complications can include wound infection, shunt malfunction, postoperative ileus, and symptoms of Chiari malformation. Signs of hydrocephalus such as increasing head circumference, bulging fontanelles, irritability, or vomiting require immediate reporting.

Nutrition and hydration 🔗

Adequate nutrition is critical for wound healing and growth. Initially, parenteral nutrition may be required, gradually transitioning to enteral feeds as tolerated. Fluid balance must be carefully monitored and documented.

Long-term support and rehabilitation 🔗

Children with myelomeningocele require lifelong, coordinated care from multiple specialists to maximise their potential and quality of life.

Mobility support 🔗

Treatment options include orthopaedic surgery, physical therapy, occupational therapy, and mobility aids such as braces, crutches, or wheelchairs. The goal is to maintain as much independence as possible while preventing secondary complications like pressure injuries and contractures.

Bladder and bowel management 🔗

Because the spinal cord and nerves controlling bladder and bowel function are typically affected, most children require ongoing management strategies. Medications, catheters, suppositories, and sometimes surgery are necessary to manage these issues. Clean intermittent catheterisation is often taught to families and eventually to the children themselves. Kidney problems due to poor urinary drainage are the most common cause of death in this population, making careful urological follow-up essential.

Family education and support 🔗

Parents and caregivers need comprehensive education about their child’s condition, including recognising signs of shunt malfunction, skin care to prevent pressure ulcers, catheterisation techniques, and strategies for promoting development. Support groups and social services can provide emotional support and practical assistance for families navigating this complex journey.

Prognosis and quality of life 🔗

With advances in surgical techniques and comprehensive care, the vast majority of children with myelomeningocele can expect to live fully functioning lives well into adulthood. Early intervention, coordinated multidisciplinary care, and family involvement are key factors in achieving optimal outcomes.

What do you think? How can paediatric nurses better support families of children with myelomeningocele through the transition from hospital to home care? What strategies have you found effective in helping children with this condition achieve greater independence?

References

1. https://www.ncbi.nlm.nih.gov/books/NBK546696/
2. https://www.cdc.gov/spina-bifida/data/
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7. https://www.biorxiv.org/content/10.1101/2025.01.06.631442v1.full
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15. https://www.seattlechildrens.org/conditions/myelomeningocele/