Neurosurgical Consultants: Spine Disorders: Congenital & Developmental Disorders Overview

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Martin L. Lazar, MD, FACS, FAANS
James E. Bland, MD, FAANS

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SPINA BIFIDA & MYELOMENINGOCOELE

Spina Bifida is one of the most serious neural tube defects that is also compatible with prolonged life. Its severity varies from the occult type (with no clinical findings) to a completely open spine (rachischisis) with severe neurological disability and death. In Spina Bifida Cystica, the protruding sac can contain meninges (meningocele), Spinal Cord (myelocele), or both (myelomeningocele). Spina Bifida is most common in the lower thoracic, lumbar, or sacral region and usually extends for 3 to 6 vertebral segments. The sac in a myelomeningocele usually consists of meninges with a central neural plaque. If not well covered with skin, the sac can easily rupture, increasing the risk of meningitis.

When the Spinal Cord or lumbosacral nerve roots are involved in the Spina Bifida, as is usual, varying degrees of paralysis occur below the affected level. Since this paralysis occurs in the fetus, orthopedic problems are often present at birth (i.e. clubfoot, arthrogryposis, dislocated hip and other extremity issues.) The paralysis frequently affects the urinary bladder and rectal functions, and the resulting genitourinary disorder can eventually lead to severely damaged kidneys. Kyphosis can be associated with Spina Bifida and can hinder surgical closure preventing the infant from lying on their back. Hydrocephalus is commonly associated with Spina Bifida and may be related to Aqueduct of Sylvius stenosis or an Arnold-Chiari Malformation. Other congenital anomalies may be present as well.

DIAGNOSIS

Prenatal Testing

Prenatally, open Spina Bifida can be diagnosed by finding elevated a-fetoprotein levels in maternal serum and in amniotic fluid. Ultrasound can show bony spinal defects and soft tissue masses.

Neuroimaging

In cases of Myelomeningocoele, X-rays of the Spine, Skull, hips and lower extremities (if they are malformed) are usually done soon after birth. Further testing depends on the associated defects and may include intracranial studies (CT or ultrasound).

Spina Bifida may never cause any symptoms. In some cases, symptoms become apparent in adolescence or later (including adulthood). In these instances, CT and/or MRI Scans are helpful. Occasionally Myelography and post-myelographic CT scans are used to more definitively identify the critical anatomical details of the anomaly (See Figure 1).

Figure 1: Post-myelographic CT Scan of Spina Bifida
This Lumbar Vertebra's Spinous Process is "BIFID" (meaning "split in two") indicated by the Double-headed Arrow. The Lamina (on either side) are abnormal (Horizontal Arrows) as well. In this case the Spinal Cord is split into 2 separate segments (Vertical Arrows).
This represents a case that was not identified until later life when as an adult the patient developed urinary incontinence.

Urinary tract evaluation is essential and includes urinalysis, urine culture, Blood chemistry analysis (BUN and creatinine), IVP (intravenous pyelogram) and ultrasound.

TREATMENT

Treatment requires a united effort by specialists from several disciplines. Initially important are neurosurgical, urologic, orthopedic, pediatric, and social service evaluations. Thorough evaluation of the infant and counseling of the family should generally precede intervention. It is important to assess the type, level, and extent of the lesion; the infant's general health status and associated deficits; the family's strengths, desires, and resources; and the community resources, including ongoing care. Once evaluation is complete, a decision can be made on how aggressive treatment should be.

Surgery

If the defect is leaking CSF, antibiotics and urgent neurosurgical evaluation and repair will reduce the risk of meningeal or ventricular infection. Hydrocephalus may require a shunt procedure.

Figure 2: Operative Photo. Myelomeningocoele.

This is a large Myelomeningocoele in a 1 day-old infant. The defect extends over the majority of the lumbar spine. The Neural Plaque (Curved Arrow indicating the exposed dark area) represents Spinal Cord tissue that is markedly abnormal. The Meningeal portion of the defect is seen on either side of the Neural Plaque (Vertical Arrows). There is some abnormal skin (Horizontal Arrow) associated with defect at the periphery of the Myelomeningocoele at the lower end and asymmetrically more on the Left than the right.

Surgical closure can be challenging. The associated Hydrocephalus must be treated lest the repair of the Myelomeningocoele disintegrate due to the CSF pressure on it.

Figure 3: Operative Photo. Myelomeningocoele in a Newborn

This Myelomeningocoele involves 3 Lumbar segments. The Neural Plaque (Horizontal Arrow) can be seen through the intact, thin meningeal envelope.

Figure 4: Operative Photo. Myelomeningocoele Repair (same patient as Figure 3)
A multilayer repair is standard for these development defects. The first layer (Arrow indicates the sutures) has meticulously reconstructed as it covers the Neural Plaque. The surgeons (Drs. Lazar & Bland) are preparing to close the 2^nd layer.

Figure 5: Operative Photo. Myelomeningocoele Repair (Same Patient as Figure 3 & 4)
There are several reliable techniques that are used to close most of the skin defects. Occasionally the defects are large enough to require special plastic surgery skin flap techniques.

Kidney function must be monitored carefully and urinary tract infection (UTI) should be treated promptly. Obstructive kidney disease at either the bladder outlet or ureteral level must be treated vigorously, especially when infection occurs.

Orthopedic care should begin early. If a clubfoot is present, it should initially be managed with a cast. The hip joints should be checked closely for dislocation. Other continuing orthopedic concerns are scoliosis, pathologic fractures, development of pressure sores, and muscle weakness and spasm, which may cause further deformities.

PROGNOSIS

Prognosis is determined by the number and severity of abnormalities and is poorest for patients with total paralysis below the lesion, kyphosis, hydrocephalus, early hydronephrosis, and associated congenital defects. With proper care, however, many children do well. Loss of renal function and shunt complications are the usual causes of death in older patients with Spina Bifida.

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This page last edited on 2/22