Spina bifida

A birth defect where the spine doesn't close fully, from mild hidden forms to open forms that need lifelong neurosurgical follow-up.

Pathway
Spine — surgical
Reviewed by
Hussein Imran Mousa, consultant neurosurgeon
Reading time
4 min read

At a glance

What it is

A spinal dysraphism caused by incomplete closure of the embryonic neural tube, typically in the first weeks of pregnancy. Three forms: spina bifida occulta (bone defect only; up to 10% of people have this incidentally, rarely symptomatic), meningocele (meninges herniate through the bony defect but cord is intact; rare, usually mild), and myelomeningocele / open spina bifida (cord and/or its coverings are exposed; always diagnosed at birth or prenatally). Open spina bifida is surgically closed within days of birth to prevent further cord damage — but damage already present at birth cannot be reversed. Adults with open forms require lifelong neurosurgical monitoring.

Symptoms

Severity and distribution depend on the level of the defect — higher lesions affect more functions. Open spina bifida: variable lower-limb weakness or paralysis, bowel and bladder dysfunction, hydrocephalus (excess CSF), Chiari II malformation (universal in open SB), tethered cord, syringomyelia, and scoliosis. Closed spina bifida occulta: usually none; a tethered cord occasionally develops when the defect is larger. Skin markers over the spine (fatty pad, dimple, hair tuft, hyperpigmentation) may indicate an underlying anomaly. Associated conditions can cause upper-limb weakness, neck pain, swallowing or respiratory difficulty, and worsening neurological function over time.

Approach

Neonatal surgery: closure of myelomeningocele within 48–72 hours of birth. Associated hydrocephalus treated with VP shunt or endoscopic third ventriculostomy. In adults, neurosurgical intervention is directed at complications: tethered cord release (when progressive symptoms develop), Chiari decompression (posterior fossa craniectomy for symptomatic Type II Chiari), shunt revision or replacement for malfunctioning CSF shunts, and syrinx management. Symptomatic pain, spasticity, neuropathy, and bladder dysfunction are managed with physiotherapy, neuromodulation, and pharmacological support.

What spina bifida is

Spina (Latin: spine) bifida (Latin: split). Spina bifida encompasses a spectrum of neural tube defects in which the posterior elements of the developing spine fail to fuse correctly. The neural tube — precursor to the brain and spinal cord — normally closes by the 28th day of embryonic development. Folic acid deficiency, genetic factors, and certain environmental exposures increase the risk of failed closure.

**Classification**

*Spina bifida occulta* — the mildest and most common form. The bony vertebral arch is incompletely fused, but the spinal cord and meninges are entirely enclosed. Affects up to 10% of people, the majority of whom never develop symptoms. Occasionally a larger defect is associated with tethered cord.

*Meningocele* — the meninges (spinal coverings) herniate through the bony defect, forming a cyst visible at the back. The spinal cord itself remains in place and is not directly involved. Neurological complications are uncommon but the cyst must be surgically repaired.

*Myelomeningocele (open spina bifida)* — the most severe form. Both the meninges and the spinal cord (or nerve roots) are exposed through the defect. The open lesion is visible at birth and is always repaired surgically within the first days of life to prevent further damage from desiccation and infection. Despite surgery, the neurological deficit present at birth persists.

**Associated conditions in open spina bifida**

*Hydrocephalus* affects about 80–90% of people with open spina bifida; nearly all require a shunt or endoscopic third ventriculostomy. Shunts can malfunction and may require revision throughout life.

*Chiari II malformation* is present in virtually all patients with open spina bifida. Brain tissue (cerebellum and brainstem) is displaced downward into the spinal canal. Many Chiari II malformations are asymptomatic, but progressive symptoms — apnea, repeated aspirations, swallowing difficulty, neck pain, upper-limb weakness — indicate the need for surgical decompression.

*Tethered spinal cord* — scar tissue or a thickened filum terminale can restrict the cord's movement, pulling on it during growth. It may be congenital or develop post-operatively. Progressive back pain (often aggravated by bending, cross-legged sitting, or carrying), leg weakness, bladder/bowel deterioration, or worsening scoliosis are indications for surgical release.

*Syringomyelia* — fluid-filled cavities in the spinal cord, often secondary to Chiari malformation or tethered cord. Managed by addressing the underlying cause; syrinx drainage is used when conservative options are insufficient.

**Adult neurosurgical care**

Adults with open spina bifida continue to benefit from neurosurgical follow-up. Shunt malfunction, progressive tethering, Chiari progression, and syrinx expansion can develop insidiously. Baseline MRI of the full neuraxis, periodic imaging, and review of neurological function are standard elements of long-term care. A specialist familiar with the lifelong complications of spina bifida is invaluable.

Treatment options at Alimran Medical Center

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The secretary schedules first appointments during clinic hours; please have prior imaging, operative notes and a current medication list available at the time of the call.

This page provides general information about spina bifida and is not a substitute for individual medical assessment. If your symptoms are severe or worsening, contact a clinician promptly.