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Anemia of Prematurity - Portal Neonatal

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Head ultrasound can be performed during the neonatal period to evaluate the extent <strong>of</strong> ventricular<br />

enlargement. Initially, the ventricles may be normal or only slightly enlarged. However, after the NTD<br />

is closed surgically, the ventricles <strong>of</strong>ten enlarge. Incidence <strong>of</strong> hydrocephalus associated with<br />

myelomeningocele ranges from 80-95%. In 2 studies performed in the 1980s and 1990s,<br />

approximately 85-90% <strong>of</strong> all patients with NTD required a VP shunt for progressive hydrocephalus.<br />

The highest incidence in shunt dependence occurs in thoracic lesions; the lowest incidence occurs in<br />

sacral lesions. The risk <strong>of</strong> shunt revision in this population may be no different from that <strong>of</strong> other<br />

children with shunts. Approximately 40-50% <strong>of</strong> all children with NTDs require shunt revision in the first<br />

year and approximately 10% every year after that.<br />

An MRI may reveal defects in cellular migration in the cerebral cortices. These include gray matter<br />

heterotopia, schizencephaly, gyral abnormalities, agenesis and thinning <strong>of</strong> the corpus callosum,<br />

abnormal thalami, and abnormal white matter findings.<br />

Meaningful surgical treatment <strong>of</strong> myelomeningocele was not undertaken until the invention <strong>of</strong> the<br />

shunt valve by Holter in the 1950s. Prior to that, closure <strong>of</strong> a myelomeningocele was possible, but the<br />

ensuing uncontrolled hydrocephalus decreased the chance <strong>of</strong> survival. In the 1980s, the US<br />

Department <strong>of</strong> Health and Human Services issued the Baby Doe directive, stating that medical and<br />

surgical treatment could not be withheld simply because a neonate is handicapped. Although the<br />

directive was struck down, the decision to operate on NTD in neonates was already an accepted<br />

practice in the United States. Furthermore, outcome studies by McClone, Shurtleff, and others<br />

presented a more positive outcome than had previously been thought for these children.<br />

Timing <strong>of</strong> myelomeningocele repair<br />

In the 1960s, the birth <strong>of</strong> a patient with myelomeningocele was a neurosurgical emergency, and<br />

immediate closure <strong>of</strong> the defect was required. Studies have subsequently shown that closure within<br />

48 hours was both safe and effective. A study by Charney et al comparing delayed closure (3-7 d) to<br />

immediate closure (

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