Beware of Myelomeningocele (Spina Bifida) in infants

Beware of Myelomeningocele (Spina Bifida) in infants

Myelomeningocele (Spina Bifida)

A myelomeningocele is a flat or raised neural plaque partly devoid of skin in the midline over the spine due to abnormal development of the spinal cord and associated deficiency of the dorsal laminae and spines of the vertebrae. It is usually found in the lumbar region. The absence of the various coverings that normally protect the cord allows meningitis to occur easily. If there are no active movements in the legs and the anus is patulous, the infant will probably be incontinent of urine and faeces for life and never be able to walk unaided. Thoracic lesions and kyphosis are signs of poor prognosis.
Infants with a good prognosis need urgent treatment, so all affected infants should either be seen by a consultant paediatrician without delay or sent to a special centre, where selection for surgery can be made. About 30% of the infants have surgery as a result of this policy. During the first operation the lesion on the back is covered by skin.
Most of these infants develop progressive hydrocephalus later and those considered suitable for surgery require insertion of a catheter with a valve from a cerebral ventricle to the peritoneal cavity to reduce the cerebrospinal fluid pressure.
Hydrocephalus can be detected by ultrasound examination of the brain. Serial measurements show whether ventricular size is increasing rapidly. In addition, progressive hydrocephalus is confirmed by measuring the circumference of the head at its largest circumference (occipitofrontal) every three days with a disposable paper tape measure, plotting these values on a growth chart, and showing that the head is growing faster than normal.
Raised concentrations of a-fetoprotein are found in the amniotic fluid when the fetus has an open myelomeningocele or anencephaly. In anencephaly there is absence of the cranial vault and most of the brain.
A routine anomaly scan at 18–24 weeks of gestation will detect most neural tube defects and the parents may decide that the pregnancy should be terminated in view of the poor prognosis. Anencephaly is a lethal condition but some infants survive for a few hours or days after birth.


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References: Bernard Valman. 2002. ABC of the First Year. London: BMJ Books. Image source: http://www.flickr.com/photos/laaradj_saadaoui/3461465263/

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