Purely cystic form of choroid plexus papilloma with acute hydrocephalus in an infant: Case report

Infants with acute hydrocephalus often present with nonspecific neurological signs, and cystic choroid plexus papilloma (CPP) is a very rare cause of acute obstructive hydrocephalus. The authors present the case of a 1-year-old girl who became irritable, started vomiting, and became comatose within a day. Magnetic resonance (MR) imaging revealed a cystic lesion in the third ventricle as well as hydrocephalus. Although the aqueduct appeared to be patent, phase-contrast MR imaging showed no pulsatile flow of cerebrospinal fluid in the ventricles. An emergent endoscopic third ventriculostomy was performed. Endoscopic examination revealed a highly mobile cyst attached by a pedicle to the choroid plexus adjacent to the Monro foramen in the lateral ventricle. The cyst was totally excised during the endoscopic procedure and was subsequently diagnosed as a CPP on the basis of histopathological findings. Purely cystic CPP is a very rare pathological entity; however, when it does occur, it can cause obstructive hydrocephalus which, without rapid diagnosis and surgical intervention, could lead to sudden death.