Abstract
Case report
A 10-month-old boy, with congenital deafness and blindness associated with chromosomal deletion [46XY, del(13)(q32)], presented with intractable ascites 9 months after ventriculo-peritoneal shunting for congenital hydrocephalus. Revision of the ventriculo-atrial shunt resulted in shunt failure 1 month later. External ventricular drainage revealed cerebrospinal fluid (CSF) overproduction (2,000 ml/day). Magnetic resonance imaging showed marked lobular enlargement of the bilateral choroid plexuses extending from the trigone to the body and inferior horn of the lateral ventricle. Multi-staged resection was performed via bilateral temporo-occipital transcortical approaches, and CSF production significantly decreased to 100 ml/day postoperatively. Histological assessment of the villous surface suggested villous hyperplasia of the choroid plexus and thorough evaluation including the proximal portion of the lobular lesion near the attachment revealed choroid plexus papilloma. He was discharged after ventriculo-peritoneal shunting without additional neurological deficits except for hyperreflexia of the left extremities.
Conclusion
CSF overproduction caused by bilateral choroid plexus papillomas can result in hydrocephalus. Radical resection of the bilateral ventricular lesions should be considered for this entity. Thorough evaluation of the surgical specimen is recommended because histological examination of only the lobular surface of the choroid plexus lesion may fail to identify choroid plexus neoplasm.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Baar HS, Galindo J (1964) Bilateral papilloma of the choroid plexus. J Maine Med Assoc 6:95–99
Britz GW, Kim DK, Loeser JD (1996) Hydrocephalus secondary to diffuse villous hyperplasia of the choroid plexus. Case report and review of the literature. J Neurosurg 85:689–691
Bucholz RD, Pittman T (1991) Endoscopic coagulation of the choroid plexus using the Nd:YAG laser: initial experience and proposal for management. Neurosurgery 28:421–426
Eisemberg HM, McComb JG, Lorenzo AV (1974) Cerebrospinal fluid overproduction and hydrocephalus associated with choroid plexus papilloma. J Neurosurg 40:381–385
Gudeman SK, Sullivan HG, Rosner MJ, Becker DP (1979) Surgical removal of bilateral papillomas of the choroid plexus of the lateral ventricles with resolution of hydrocephalus. Case report. J Neurosurg 50:677–681
Hirano H, Hirahara K, Asakura T et al (1994) Hydrocephalus due to diffuse villous hypertrophy of the choroid plexus in the lateral ventricles: case report. J Neurosurg 80:321–323
Husag L, Costabile G, Probst C (1984) Persistent hydrocephalus following removal of choroid plexus papilloma of the lateral ventricle. Neurochirurgia (Stuttg) 27:82–85
Milhorat TH, Hammock MK, Davis DA et al (1976) Choroid plexus papilloma. I. Proof of cerebrospinal fluid overproduction. Childs Brain 2:273–289
Philips MF, Shanno G, Duhaime AC (1998) Treatment of villous hypertrophy of the choroid plexus by endoscopic contact coagulation. Pediatr Neurosurg 28:252–256
Rickert CH, Wiestler OD, Paulus W (2002) Chromosomal imbalances in choroid plexus tumors. Am J Pathol 160:1105–1113
Welch K, Strand R, Breanan M et al (1983) Congenital hydrocephalus due to villous hypertrophy of the telencephalic choroid plexuses. Case report. J Neurosurg 59:172–175
Acknowledgements
The authors are grateful to Drs. Reizo Shirane and Hidefumi Jokura (Department of Neurosurgery, Tohoku University Graduate School of Medicine) for valuable suggestions about the treatment of this patient.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Fujimura, M., Onuma, T., Kameyama, M. et al. Hydrocephalus due to cerebrospinal fluid overproduction by bilateral choroid plexus papillomas. Childs Nerv Syst 20, 485–488 (2004). https://doi.org/10.1007/s00381-003-0889-8
Received:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-003-0889-8