Macrocrania Hydrocephalus |
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Incidence | Illustration |
Congenitally or peri-/postnatally acquired; the former causes are decreasing due to early prenatal diagnosis (1 isolated congenital hydrocephalus in 1000 newborns. With myelomeningocele, congenital hydrocephalus occurs in up to 90 percent as a concomitant malformation), the latter (acquired) causes are increasing on account of rising prematurity (perinatal intracranial hemorrhage). | show details |
Clinical significance | |
Without treatment: | |
Etiology | Illustrations |
Disturbance in cerebrospinal fluid (= CSF) circulation or absorption. | show details |
Pathology, anatomical types | Illustrations |
There are numerous pathologies leading to hydrocephalus, which have a substantial influence on its prognosis. | show details |
Pathophysiology | |
Disturbance in cerebrospinal fluid circulation causes CSF accumulation, leading to an increase in the intracranial pressure (= ICP), the dilatation of the CSF spaces (mainly of the ventricles), to a reduction of the cerebral blood flow, and to irreversible parenchymal damage. | |
Clinical presentation (history, findings, clinical skills) | Illustrations |
The clinical presentation depends on the age of the child and on the rate of development of the disturbance in CSF circulation. Measurement of head circumference: Mean of three values obtained in the largest frontooccipital plane. | show details |
Natural history | Illustration |
a) Death (nearly 50 % within 10 years) or | show details |
Differential diagnosis | Illustrations |
1. Treated hydrocephalus with shunt insufficiency. | show details |
Work-up examinations | Illustrations |
In case of an open fontanel: ultrasound as screening examination. In every case: CT or MRI. Examinations to discover the cause of the hydrocephalus. | show details |
Therapy | Illustrations |
Ventriculoperitoneal shunt. In selected cases: endoscopic ventriculostomy. | show details |
Prognosis | Illustrations |
The prognosis depends on the cause of the hydrocephalus and the promptness of treatment. With treatment, normal global intelligence is observed in 2/3 of the cases, in isolated congenital hydrocephalus in 50 %. In 90 % there is a life-long shunt dependency with the possibility of shunt failures. In case of endoscopic ventriculostomy, similar follow-up times as in shunted patients are not yet available. | show details |