Initial Management at Presentation
- Removal of CSF to decrease ventricular volume and inflammation: When progressive ventricular dilation is verified, various therapies are used to remove CSF and thereby lessen ICP and CSF inflammatory byproducts. These include lumbar and ventricular puncture, EVD, ventricular catheters attached to subgaleal reservoirs, ventriculosubgaleal shunt, and endoscopic choroid plexus cauterization in combination with ETV.
- Acetazolamide and furosemide: Although these agents can reduce CSF production by up to 50%, they are accompanied by side effects such as electrolyte disorders and a need for prolonged use. The results of large trials showed that the combination of the two agents significantly increased the risk of impairment or disability at 1 year with no decrease in death or ventriculoperitoneal shunt requirement. They cannot be recommended as an option for the management of PHH (4, 63, 109).
- Ventriculoperitoneal shunt when dependency on CSF removal established: When the initial management fails to clear the CSF pathways, the established treatment option is ventriculoperitoneal shunting. Most authors consider the minimum acceptable weight for ventriculoperitoneal shunt insertion to be >2000 g. Other criteria for ventriculoperitoneal shunt placement are CSF protein levels ideally <100 mg/dl and no evidence of infection and abdominal impairment (e.g., necrotizing enterocolitis, which premature neonates develop infrequently). There has been considerable debate in the past on the type of valve and its opening pressure suitable for neonates, but no convincing evidence exists about the ventriculoperitoneal shunt type and specific valve mechanism that may be particularly suitable to infants with PHH (9).
- Subsequent follow-up: Follow-up is the same as for any shunt.
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