Neonatal hydrocephalus can arise from a multitude of disturbances, among them congenital aqueductal stenosis, myelomeningocele or posthemorrhagic complications in preterm infants. Diagnostic work-up comprises transfontanellar ultrasonography, T2 weighted MRI and clinical assessment for rare inherited syndromes. Classification of hydrocephalus and treatment guidelines is based on detailed consensus statements. The recent evidence favors catheter-based cerebrospinal fluid diversion in children below 6 months, but emerging techniques such as neuroendoscopic lavage carry the potential to lower shunt insertion rates. More long-term study results will be needed to allow for individualized, multidisciplinary decision making. This article gives an overview regarding contemporary pathophysiological concepts, the latest consensus statements and most recent technical developments.
KeywordsCerebrospinal Fluid, Hydrocephalus, Neonate, Neuroendoscopy, VP-Shunt
The Cerebrospinal Fluid SystemThe cerebrospinal fluid (CSF) system provides mechanical protection of the brain against mild trauma, is involved in nutrient supply and waste removal, and plays a role in the circadian rhythm [1]. In humans it comprises 4 cavities in the brain, the ventricles, which normally take up about 20% of the CSF. In addition, How to cite this paper: Krause, M., Panteliadis, C.P., Hagel, C., Hirsch, F.W., Thome
HydrocephalusHydrocephalus is a disbalance between CSF production and resorption.
Clinical PresentationMost children with hydrocephalus suffer from a chronic disease with a rather slow progress of symptoms. Only 10% -15% of newborns with hydrocephalus show signs of elevated intracranial pressure [11].In preterm infants, hydrocephalic growth of the neurocranium is preceded by an ultrasonographically detectable enlargement of the ventricles. At this time, the endochondral bone formation of the skull fragments still allows for a certain flexibility and the small children often suffer from a multitude of additional regulatory deficits owing to their immaturity. Therefore the signs of increased intracranial pressure such as bradycardia or respiratory insufficiency are less obvious than in older children. In newborns or infants under the age of 2, the crossing of percentiles of the head circumference compared to body height and weight is the most important sign of isolated hydrocephalus [11].Typical concurring symptoms are a tight, bulging anterior fontanel, dehiscence of skull sutures, increased visibility of scalp veins and a huge frontal bone (frontal bossing). Retraction of the upper eye-lids, together with Parinaud's upgaze palsy can lead to the rare, but very impressive sunset phenomenon, with both irises plunging half below the lower eye-lids.A number of hereditary syndromes, which mostly follow a recessive inheritance, are associated with hydrocephalus (Table 2). Thus, pediatric patients suffering from hydrocephalus of unknown etiology should be examined thoroughly for stigmata indicating an inherited disease.In older children acu...