Peds RN Myelomeningocele

Myelomeningocele

Myelomeningocele: A Guide for Nursing Students

Introduction

Myelomeningocele, the most severe form of spina bifida, is a neural tube defect occurring when the spine and spinal cord do not form properly during fetal development. This condition results in a sac-like protrusion containing meninges, cerebrospinal fluid (CSF), and spinal nerves through an opening in the vertebrae. Nursing students need to understand the complexities of myelomeningocele, including its causes, clinical presentation, treatment options, and the role of nursing care in managing the condition.

Pathophysiology of Myelomeningocele

Myelomeningocele develops within the first 28 days of pregnancy when the neural tube, which eventually forms the brain and spinal cord, fails to close completely. As a result, part of the spinal cord and its coverings (the meninges) are exposed on the back. This condition commonly affects the lower spine but can occur at any level. The exposed nerves are often damaged, leading to paralysis or mobility issues below the defect, loss of sensation, and bladder and bowel dysfunction (Mayo Clinic, 2020).

Risk Factors and Prevention

The exact cause of myelomeningocele is unknown, but risk factors include insufficient folic acid intake during pregnancy, family history of neural tube defects, and exposure to certain medications or toxins. Folic acid supplementation before conception and during early pregnancy significantly reduces the risk of neural tube defects (Greene et al., 2016).

Clinical Presentation

Newborns with myelomeningocele present with a visible sac-like protrusion on the back, often covered with a thin layer of skin or, in severe cases, completely exposed tissue. Symptoms vary based on the location and extent of the spinal defect but commonly include:

Partial or complete paralysis and loss of sensation below the affected area.

Bladder and bowel dysfunction, leading to incontinence.

Hydrocephalus, which develops in 70-90% of cases and causes an accumulation of CSF in the brain (Sandler, 2010).

Diagnosis and Initial Management

Myelomeningocele is often diagnosed prenatally through ultrasound and elevated maternal serum alpha-fetoprotein (AFP) levels. After birth, immediate management includes protecting the exposed area to prevent infection and monitoring for complications. Surgical repair is usually performed within 24-48 hours after birth to close the defect, reduce infection risk, and prevent further nerve damage.

Nursing Care and Interventions

Nursing care for infants with myelomeningocele is complex and involves managing both immediate post-operative needs and ongoing care. Key nursing interventions include:

Pre-Operative Care: Protecting the exposed sac from injury and infection by covering it with a sterile, moist dressing and placing the infant in a prone position to prevent pressure on the defect. Nurses should monitor for CSF leakage, signs of infection, and changes in neurological function.

Post-Operative Care: After surgery, nursing care focuses on pain management, monitoring the surgical site for signs of infection, and continuing the prone position to protect the surgical area. Nurses also monitor for symptoms of increased intracranial pressure, as hydrocephalus may require a shunt.

Bladder and Bowel Management: Many infants with myelomeningocele require catheterization for urinary management and bowel regimens to manage incontinence. Nurses provide education to families on catheterization techniques, bowel management, and the importance of regular follow-up.

Hydrocephalus Monitoring and Management: If a shunt is placed to manage hydrocephalus, nurses should monitor for shunt malfunctions, signs of infection, and changes in neurological status. Shunt complications are common, and parents must be educated on recognizing symptoms that require immediate medical attention.

Skin Integrity and Mobility Support: Patients with reduced sensation are at risk for pressure ulcers, so nurses should ensure proper positioning, provide padding, and educate families on skin care to prevent breakdown. Physical therapy is often part of the care plan to maximize mobility and prevent contractures.

Patient and Family Education

Nurses play a key role in educating families on long-term care needs, including understanding the importance of ongoing therapy, managing potential complications, and accessing resources. Support groups and community resources can help families navigate the physical, emotional, and social challenges associated with myelomeningocele.

Long-Term Outcomes and Support

Children with myelomeningocele often face lifelong challenges requiring multidisciplinary care, including orthopedic support, physical therapy, and urologic management. With appropriate care, many individuals achieve a good quality of life, but the level of independence varies. Ongoing support from a healthcare team is essential for adapting to physical limitations and ensuring optimal health outcomes.

Conclusion

Myelomeningocele is a complex condition that requires comprehensive nursing care, family education, and long-term support. Nursing students who understand the pathophysiology, treatment options, and nursing interventions for myelomeningocele can provide effective and compassionate care to improve the quality of life for these patients and their families.

References

Greene, N. D., Leung, K. Y., Gay, V., Burren, K., Mills, K., & Chitty, L. S. (2016). Prevention and prenatal diagnosis of neural tube defects: Advances and challenges. Fetal Diagnosis and Therapy, 39(2), 95-102.

Mayo Clinic. (2020). Spina bifida. Retrieved from https://www.mayoclinic.org/diseases-conditions/spina-bifida/symptoms-causes/syc-20377860

Sandler, A. D. (2010). Children with spina bifida: Key clinical issues. Pediatric Clinics of North America, 57(4), 879-892.