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Genetic and Rare Diseases Information Center (GARD)

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Choroid plexus papilloma

Other Names for this Disease
  • Papilloma of choroid plexus
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Your Question

I have a close friend whose neonate was born with severe hydrocephalus which was diagnosed at 32 weeks gestation. At 3 weeks of age the baby was diagnosed with a choroid plexus papilloma through MRI. The physicians plan to do a spinal MRI to see if it has spread. Have you found that children developing this condition in utero are more or less likely to have long term complications?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What factors affect the long-term outlook for individuals with choroid plexus papilloma?

The prognosis for choroid plexus papilloma (CPP) is determined mainly by how completely the lesion is removed during surgery. Complete removal of intraventricular CPPs almost always cures the condition and it has been estimated that the survival rate at 5 years for CPPs after removal is approximately 100%. Examples of rare complications have been described and generally involve neurologic deficits from the surgical procedure.[1]

Although there may not be residual effects of the CPP specifically, the extent and duration of the presence of hydrocephaly can affect cognitive and/or physical development in affected individuals.[2] Hydrocephalus may continue after surgery in some cases and is then often managed by CSF shunting. Complications that result in neurological or psychological problems may influence overall outcome.[1]
Last updated: 12/11/2012

How might hydrocephalus affect development?

Hydrocephalus is characterized by excessive accumulation of fluid (cerebrospinal fluid, or CSF) in the brain. Accumulation results in the abnormal widening (dilation) of spaces in the brain called ventricles. This can create harmful pressure on the tissues of the brain.[2] The brain can accommodate ventricular dilatation to a certain extent without significant neuronal damage, but as the process continues, irreversible brain damage can occur.[3]

The effects of hydrocephalus can be difficult to predict and partially depend on the severity, timeliness of the diagnosis and the success of treatment. Early diagnosis and treatment improves the chance of a good recovery, but the degree to which relief of CSF pressure after treatment can minimize or reverse damage is not well understood. Hydrocephalus causes risks to cognitive and physical development, but many affected individuals benefit from rehabilitation and educational intervention, leading lives with few limitations. If hydrocephalus is left untreated, it may be fatal.[2]

In general, there is a greater chance for an abnormal outcome with fetal hydrocephalus (present in utero) compared to neonatal hydrocephalus (beginning shortly after birth) because of the period it is left untreated in utero. Prenatal factors such as progression, degree of cortical mantle thinning to less than 1.5 cm, and in-utero duration of greater than four weeks are associated with a poorer prognosis.[3]
Last updated: 12/11/2012