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CAR: Anomalies - CNS
Spina bifida

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Introduction Antenatal Postnatal West Midlands Data


Spina bifida is a failure in the closure of the vertebrae at the base of the neural tube resulting in the herniation or exposure of the spinal cord and/or meninges. The incidence of spina bifida is similar to that of anencephalus. As with other neural tube defects there are reported associations with maternal insulin dependent diabetes, hyperthermia, obesity at conception, autosomal trisomies, and low serum folate concentrations.

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In the West Midlands programmes for the prenatal diagnosis of neural tube defects are based on maternal serum AFP screening performed at 16 to 20 weeks gestation and ultrasound screening, which is widely performed at 16 to 22 weeks for this purpose. AFP serum screening is thought to be 80% sensitive for open spina bifida. Ultrasound is based on examination of the fetal head, particularly the shape of the skull and the appearance of the cerebellum. Spina bifida is most often diagnosed using the cranial signs of an abnormal shape of the fetal skull (lemon shape) and flattening and loss of the median sulcus in the cerebellum (banana shape). Hydrocephalus is often associated with spina bifida and the spine itself can be examined to demonstrate failure of closure of the vertebral arches, lack of skin cover and herniation.

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The prognosis for cases of spina bifida is not necessarily fatal and depends on the location, size, and extent of the defect, and the presence of hydrocephalus. Cases with high extensive lesions with obvious hydrocephalus at birth and other congenital defects are universally poor. In surviving infants, spina bifida can result in severe disability affecting the sensory and motor function of the lower limbs, bowel, and bladder. Predicting the outcome of open spina bifida cases is problematic, with some cases having a good outcome, while others have severe disability. The options are either early surgical closure, with or without shunting of hydrocephalus or waiting for a time to allow the lesion to heal by granulation.

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To be added.

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© Perinatal Institute 2011