Abstract
Congenital hydrocephalus includes both fetal hydrocephalus and postnatal hydrocephalus due to various prenatal pathological conditions. With the recent advancement of the neuro-imaging techniques applied for prenatal diagnosis, neurosurgeons have come to notice patients with congenital hydrocephalus more frequently than previously. In the present report, we described standard treatment for and prognostic factors of this disease. Cerebrospinal fluid (CSF) shunt is the standard procedure in children with a body weight more than 2,000 g. In the case of a baby with insufficient body weight, CSF drainage via a miniature Ommaya reservoir can be applied tentatively before installation of CSF shunt. Endoscopic third ventriculostomy is not recommended especially for neonates with congenital hydrocephalus. Fetal examination by neuro-imagings is crucial to arriving at a proper diagnosis, because postnatal prognosis differs a great deal among various types of associated central nervous system anomalies. Congenital hydrocephalus uncomplicated by significant anomalies of the central nervous system has a relatively favorable prognosis. Postnatal prognosis is poor in hydroanencephaly, semilober or alober holoprocencephaly, encepahlocele with extrusion of the cerebral ventricle and severe dysgenesis of the cerebellar vermis in Dandy-Walker syndrome. There are other prognostic factors involved in the hydrocephalic condition itself. For example, a cerebral mantle thinner than 2 cm, or early prenatal diagnosis of hydrocephalus are poor prognostic factors. Knowledge of these data can help neurosurgeons perform standard treatment of this disease in consideration of evidence-based medicine (EBM). At the time of diagnosis, however, parents become very confused and depressed and, if they are informed of even standard treatment and of the most likely postnatal prognosis solely according to EBM, they tend to seek termination of pregnancy or to refuse postnatal treatment for the child. To avoid such a situation so disadvantageous for the patient, counseling should be introduced as one of the clinical applications of narrative-based medicine (NBM). When the hydrocephalus is diagnosed before the 22nd gestational week, counseling can help the parent make a deeply considered decision on termination of the pregnancy. When it is diagnosed at the 22nd gestational week or after (the legal termination is prohibited in this period), counseling can help the parents to accept and raise their child with congenital hydrocephalus. We know that clinical data based on the clinical evidence (EBM) are essential for treatment of this disease. However, with the introduction of the practical application of NBM, we can provide more satisfactory and better medical treatment for children with congenital hydrocephalus and more support for their family.
