Hydrocephalus due to cerebrospinal fluid overproduction by bilateral choroid plexus papillomas.
METHODS
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.


CONCLUSIONS
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.
Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, 980-8574 Aoba-ku, Sendai, Japan. fujimur@nsg.med.tohoku.ac.jp
Miki
M
Fujimura
Takehide
T
Onuma
Motonobu
M
Kameyama
Osamu
O
Motohashi
Hiroyuki
H
Kon
Katsuya
K
Yamamoto
Kiyoshi
K
Ishii
Teiji
T
Tominaga
D002555:Cerebrospinal Fluid / Q000378:metabolism*; D006801:Humans; D006849:Hydrocephalus / Q000134:cerebrospinal fluid* / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D021621:Imaging, Three-Dimensional; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009460:Neurologic Examination; D020288:Papilloma, Choroid Plexus / Q000134:cerebrospinal fluid* / Q000150:complications* / Q000473:pathology / Q000601:surgery; D013194:Staining and Labeling / Q000379:methods