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Myelomeningocele
Updated: Jul 2, 2009
Introduction
Background
Myelomeningocele (also called meningomyelocele) is a complex congenital spinal anomaly that causes varying degrees of spinal cord malformation, or myelodysplasia. It is commonly referred to as spina bifida and is classified as a defect of the neural tube (ie, the embryonic structure that develops into the spinal cord and brain). Patients with myelomeningocele present with a spectrum of impairments, but the primary functional deficits are lower limb paralysis and sensory loss, bladder and bowel dysfunction, and cognitive dysfunction.1 Medical, surgical, and rehabilitation issues arise in the patient with myelomeningocele from birth through adulthood.2 (See image below and Image 1.)
The lumbar region of a newborn baby with myelomeningocele. The skin is intact, and the placode-containing remnants of nervous tissue can be observed in the center of the lesion, which is filled with cerebrospinal fluid.
Pathophysiology
Neural tube defects are the result of a teratogenic process that causes failed closure and abnormal differentiation of the embryonic neural tube during the first 4 weeks of gestation. The most common neural tube defects are anencephaly and myelomeningocele. Anencephaly results from failed closure of the rostral end of the neural tube, resulting in incomplete formation of the brain and skull. Myelomeningocele results from failed closure of the caudal end of the neural tube, resulting in an open lesion or sac that contains dysplastic spinal cord, nerve roots, meninges, vertebral bodies, and skin. (See image below and Image 2.) The anatomic level of the myelomeningocele sac roughly correlates with the patient's neurologic, motor, and sensory deficits.3
Myelomeningocele in a newborn.
Myelomeningocele is associated with abnormal development of the cranial neural tube, which results in several characteristic CNS anomalies. The Chiari type II malformation is characterized by cerebellar hypoplasia and varying degrees of caudal displacement of the lower brainstem into the upper cervical canal through the foramen magnum. This deformity impedes the flow and absorption of cerebrospinal fluid (CSF) and causes hydrocephalus, which occurs in more than 90% of infants with myelomeningocele. Cerebral cortex dysplasia, including heterotopias, polymicrogyria, abnormal lamination, fused thalami, and corpus callosum abnormalities, also occurs frequently. Mesodermal structures surrounding the neural tube, such as the vertebra and ribs, also may be malformed. (See image below and Image 3.)
T1-weighted, coronal magnetic resonance imaging (MRI) scans of the brain show a Chiari II malformation. Note the stretching of the brainstem, aqueduct, and fourth ventricle.
Myelomeningocele often occurs with multiple system congenital anomalies. Commonly associated anomalies are facial clefts, heart malformations, and genitourinary tract anomalies. Urinary tract anomalies, such as solitary kidney or malformed ureters, may contribute to increased morbidity in the presence of neurogenic bladder dysfunction.
Frequency
United States
Neural tube defects are the second most common type of birth defect after congenital heart defects, and myelomeningocele is the most common form of neural tube defect. In the United States, approximately 1500 infants are born with myelomeningocele each year. Birth incidence of the disease was reported to be 4.4-4.6 cases per 10,000 live births from 1983-1990. Rates varied by region, with the highest rates occurring in Appalachia. The rate of myelomeningocele and other neural tube defects has declined over the last 3 decades. This is attributed to the widespread availability of prenatal diagnostic services and to improved nutrition among pregnant women.
International
The rates of myelomeningocele vary widely among countries and by geographic regions within countries. Neural tube defects occur at frequencies (per 10,000 births) ranging from 0.9 in Canada and 0.7 in central France, to 7.7 in the United Arab Emirates and 11.7 in South America. Low socioeconomic status is associated with higher risk in many populations. In the last 50 years, epidemics of myelomeningocele have occurred in Boston, Mass; Rochester, NY; Dublin, Ireland; The People's Republic of China; and Jamaica.
Mortality/Morbidity
In the United States, the leading identified cause of infant death is birth defects, and myelomeningocele is one of the most common birth defects. In general, survival and degree of neurologic impairment depend on the level of the spinal segment involved, the severity of the lesion, and the extent of associated abnormalities.
The mortality rate for infants with myelomeningocele is increased over the general population risk in the first year of the life. Mortality rates reported for untreated infants range from 90-100% based on several studies dating from the turn of the century through recent years. Most untreated infants die within the first year of life. Death in the first 2 years of life for those untreated usually results from hydrocephalus or intracranial infection. The likelihood that a 2-month-old infant untreated for myelomeningocele lives 7 years is only 28%.
Survival rates for infants born with myelomeningocele have improved dramatically with the introduction of antibiotics and developments in the neurosurgical treatment of hydrocephalus. Early death in treated and untreated patients is associated with advanced hydrocephalus and multiple system congenital anomalies. In the United States, antibiotics, sac closure, and ventriculoperitoneal shunt placement are the standard of care and are implemented in the perinatal period in 93-95% of patients. Supportive care only may be recommended in cases associated with an irreparable sac, active gross CNS infection or bleeding, and/or other gross congenital organ anomalies causing life-threatening problems.
Paraplegia from the myelodysplasia typically causes some impairment of motor and sensory function below the level of the lesion, along with neurogenic bowel and bladder and sexual dysfunction. Musculoskeletal complications may be caused by progressive bony and joint deformities, pathologic fractures, and muscle deterioration.
Many other neurologic problems may manifest acutely or chronically at birth or later, resulting in global cognitive and/or specific motor impairments.1 These neurologic complications are related to a number of distinct cerebral and intraspinal pathologies. Cognitive dysfunction is most strongly correlated with the presence of hydrocephalus, along with hydrocephalus-related illness parameters (ie, the necessity of shunting, number of shunt revisions, shunt infections, and additional structural abnormalities of the CNS).4 Cognitive function has also been related to the level of the lesion. Upper-level lesions have been associated with a higher frequency of mental retardation and lower scores on measures of intelligence, academic skills, and adaptive behavior.
Renal compromise occurs because of problems related to neurogenic bladder. Despite advances in the management of neurogenic bladder, renal failure is still the leading cause of death in patients with myelomeningocele after the first year of life.
Race
Epidemiologic studies have shown that the prevalence of myelomeningocele varies across time, by region, and by race and ethnicity. In the United States, data from state and national surveillance systems from 1983-1990 showed rates highest for Hispanics and whites and lowest for Asians. The rates are 0.15% in the white population and 0.04% in the black population; a higher proportion of whites than blacks have thoracic-level malformations.
Sex
The birth prevalence rate of myelomeningocele was slightly higher in females than in males (1.2:1), based on data from state and national surveillance systems from 1983-1990. A higher proportion of females than males exhibit thoracic-level malformations.
Age
Myelomeningocele is a common congential anomaly and therefore is present at birth.
Clinical
History
Myelomeningocele is diagnosed at birth or in utero. At birth, a midline defect in the posterior elements of the vertebrae is noted with protrusion of the meninges and neural elements through an external dural sac.
Physical
- The most obvious finding on physical examination is some degree of motor and sensory loss.1
- Neurologic impairment is classified by traditional neurosegmental levels based on the clinically determined strength of specific muscle groups. The functional motor level does not always correspond to the anatomic level of the lesion. In addition, it is important to realize that the motor paresis may be asymmetrical, that it may not correspond to the sensory level, and that it may be combination of upper and lower motor neuron lesions. Serial measurements and accurate documentation of the functional level of the lesion allow for early detection of progressive neurologic deterioration related to a variety of associated CNS problems.
- In addition to determining the functional neurosegmental level, it is important to distinguish the type of paralysis, either spastic or flaccid. Most patients with myelomeningocele have a flaccid paraparesis below the spinal cord lesion. An estimated 10-25% of patients have been reported to have a spastic paraparesis. This presentation is presumed to be related to an intact but isolated segment of cord distal to the lesion. Spastic paraparesis has been associated with a poorer prognosis for walking and higher rates of orthopedic procedures.
- Myelomeningocele patients frequently are described as belonging to certain groups, based on the neurosegmental level of the lesion. This approach is useful for general functional prognosis and anticipation of specific musculoskeletal complications.
- In the thoracic group, innervation of the upper limb and neck musculature and variable function of trunk musculature are present with no volitional lower limb movements. Patients with thoracic malformations tend to have more involvement of the CNS and associated cognitive deficits.
- In the high-lumbar group, variable hip flexor and hip adductor strength is characteristic, and absence of hip extensors, hip abductors, and all knee and ankle movements are noted.
- In the low-lumbar group, hip flexor, adductor, medial hamstring, and quadriceps strength is present; strength of the lateral hamstrings, hip abductors, and ankle dorsiflexors is variable; and strength of the ankle plantar flexors is absent.
- In the sacral-level group, strength of all hip and knee groups is present, and ankle plantar flexor strength is variable.
- Involvement of the upper extremities is also common. Spasticity in the upper extremities occurs in approximately 20% of patients with myelomeningocele. It has been related to the number of shunts required to control hydrocephalus and has been shown to adversely affect independence in activities of daily living. In patients with hydrocephalus, lack of upper extremity coordination is also seen. This lack of coordination also may be related to Chiari II malformation, motor-learning deficits, and/or delayed development of hand dominance. Affected children have problems with fine motor tasks, particularly when timed. New-onset weakness or spasticity in the upper extremities may be a hallmark of progressive neurologic dysfunction.
- Spinal and lower extremity deformities and joint contractures are prevalent in children with myelomeningocele. Multiple factors may be involved, including intrauterine positioning, other congenital malformations, muscle imbalances, progressive neurologic dysfunction, poor postural habits, and reduced or absent joint motion.
- Spinal deformities may be congenital or acquired. Vertebrae and rib anomalies are associated with congential or early development of severe kyphotic and scoliotic deformities. Acquired scoliosis is neuromuscular in origin and is related to muscle imbalances.5 Increased lumbar lordosis and kyphosis of the entire spine or localized to the lumbar region are also observed. All of the spinal deformities occur more frequently in groups with higher spinal lesions.
- The lower extremity deformities that occur are related to the functional level of the lesion. Thoracic and high-lumbar groups tend to have increased prevalences of lumbar lordosis, hip abduction and external rotation contractures, knee flexion, and equinus contractures of the ankles. Unopposed hip flexion and adduction contractures in the high-lumbar group frequently result in dislocated hips. The mid- and low-lumbar groups often have hip and knee flexion contractures, increased lumbar lordosis, genu valgus and calcaneal valgus malalignment, and overpronated feet. Patients in the sacral group often exhibit mild hip and knee flexion contractures and increased lumbar lordosis with various ankle and foot positions.
- Children with myelomeningocele are often short in stature. This has been related to multiple factors, including (1) structural issues such as abnormalities of the spinal column and lower limb contractures; (2) functional spinal level, which influences the amount of neurotrophic input from the lower extremities on appendicular skeletal growth; and (3) alteration in the hypothalamic-pituitary axis, likely related to hydrocephalus.
- Ocular muscle palsies, swallowing and eating problems, and abnormal phonation are signs of cranial nerve dysfunction. These symptoms may be related to the Chiari II malformation, hydrocephalus, and/or brainstem dysplasia.
Causes
The etiology in most cases of myelomeningocele is multifactorial, involving genetic, racial, and environmental factors.
- Other offspring in a family with 1 affected child are at greater risk of neural tube defect than are children without affected siblings.
- The risk is 1 in 20-30 for subsequent pregnancies, and if 2 children are affected, the risk becomes 1 in 2. An increase in the risk of myelomeningocele has also been reported for second- and third-degree relatives of affected individuals.
- Most infants born with myelomeningocele are born to mothers with no previously affected children.
- A small number of cases are linked to specific etiologic factors.
- Up to 10% of fetuses with a neural tube defect detected in early gestation have an associated chromosome abnormality.
- Associated chromosome abnormalities include trisomies 13 and 18, triploidy, and single gene mutations.
- In women with pregestational diabetes, the risk of having a child with a CNS malformation, including myelomeningocele, is 2-10 fold higher than the risk in the general population. The mechanism underlying this teratogenic effect is not well defined but is related to the degree of maternal metabolic control. The risk of women who develop gestational diabetes is lower than the risk of women with pregestational diabetes, but it might not be as low as in the general population.
- Other risk factors for myelomeningocele include maternal obesity, hyperthermia (as the result of maternal fever or febrile illness or associated with the use of saunas, hot tubs, and tanning beds), and maternal diarrhea.
- Intrauterine drug exposures to valproate, carbamazepine, and drugs to induce ovulation are identified risk factors.
- The risk of having a child with myelomeningocele has also been associated with maternal exposures to fumonisins, electromagnetic fields, hazardous waste sites, disinfection by-products found in drinking water, and pesticides.
- Research in the 1980s showed correction of folic acid deficiency as an effective means of primary and recurrent prevention.6
- After 1991, 50% of cases of neural tube defects are related to a nutritional deficiency of folic acid and, thus, are preventable.
- In September 1992, the US Public Health Service (USPHS) recommended intake of folic acid at a dosage of 0.4 mg/d for all women anticipating pregnancy.
- In February 1996, the USPHS announced mandatory folic acid fortification of enriched cereal grain, a measure expected to increase the daily intake of folic acid in women of reproductive age by approximately 100 mcg/d.
- After fortification, an estimated 24% decline in myelomeningocele rates was reported to have occurred between 1996 and 2001, based on data from United States surveillance systems.
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Further Reading
Related eMedicine topics:
Chiari II Malformation
Management of Spina Bifida, Hydrocephalus and Shunts
Myelodysplasia and Neurogenic Bladder Dysfunction
Neural Tube Defects
Neural Tube Defects in the Neonatal Period
Spina Bifida
Spinal Dysraphism/Myelomeningocele
Clinical guidelines:
Management of neurogenic bladder in children. In: Guidelines on paediatric urology.
European Association of Urology - Medical Specialty Society
European Society for Paediatric Urology - Medical Specialty Society. 2008 Mar. 10 pages. NGC:006508
Neural tube defects.
American College of Obstetricians and Gynecologists - Medical Specialty Society. 2001 (revised 2003 Jul). 11 pages. NGC:003131
Clinical trials:
Management of Myelomeningocele Study (MOMS)
2 Week Open-Label Pharmacodynamic and Pharmacokinetic Study of Multiple Doses of a Darifenacin Liquid Oral Suspension in Children (6 - 15 Yrs) With Neurogenic Detrusor Overactivity
Keywords
myelomeningocele, spina bifida, bifida, hydrocephalus, malformation Chiari, meningocele, tethered cord, neural tube, Chiari syndrome, meningomyelocele, hydrocephaly, neural tube defect, neural tube defects, open spinal dysraphism, myelodysplasia, spina bifida cystica, spina bifida aperta, open spina bifida
