Acta Neurol Belg DOI 10.1007/s13760-014-0398-4
NEURO-IMAGES
Diffuse choroid plexus hyperplasia (CPH) associated with multiple malformations Yong Hong • Qiang Xu • Y. S. J. Maimaiti Junming Ye • Gong Chen
•
Received: 10 October 2014 / Accepted: 26 November 2014 Ó Belgian Neurological Society 2014
Keywords
Choroid plexus hyperplasia Malformation
A 32-year-old right-handed male presented with interruptible dizziness for 1 year, and aggravated clinical symptoms for 2 weeks, with headache, vomit, blurred vision and hyposmia. Magnetic resonance anography and magnetic resonance spectroscopy showed normal intracranial vessels and non-tumorous lesion. Magnetic resonance imaging (MRI) revealed diffuse choroid plexus hyperplasia (CPH) in bilateral ventricles and the third ventricle (Fig. 1, white arrowhead), which were markedly enhanced (Fig. 2, white arrowhead) in enhanced MRI image. Diffuse CPH lead to hydrocephalus due to cerebrospinal fluid (CSF) overproduction, and multiple malformations with multiple atrophies and schizencephaly
(Fig. 2, pink arrowhead), empty sella (Fig. 2, red arrowhead). In posterior fossa, the malformation resembled Dandy–Walker complex with the enlarging fourth ventricle, large cyst and hypoplastic cerebellar vermis (Fig. 2, green arrowhead). The neuroradiological malformation was more severe than that in last year, which is associated with clinical progressive intracranial hypertension. This patient will be treated by endoscopic coagulation of the choroid plexuses and then CSF ventriculoperitoneal shunt. CPH with hydrocephalus and (or) multiple atrophy is not rare [1–3], but it is rather rare that it is also associated with multiple malformations (schizencephaly, empty sella, the Dandy–Walker complex). Without the history of trauma, infection, hemorrhage or surgery in this adult
Y. Hong Q. Xu Department of Radiology, Huashan Hospital, Fudan University, Shanghai 200040, China e-mail: [email protected] Y. S. J. Maimaiti G. Chen (&) Department of Neurosurgery, Huashan Hospital, Fudan University, No.12, Wulumuqi Mid Road, Shanghai 200040, China e-mail: [email protected]; [email protected] J. Ye Department of Anesthesia, First Affiliated Hospital, Gannan Medical University, Ganzhou 200040, Jiangxi, China
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Acta Neurol Belg Fig. 1 FLAIR images of MRI revealed diffusely increased size of choroid plexuses along the entire choroid fissure in bilateral ventricles (A1) and the third ventricle (A2, white arrowhead)
Fig. 2 B1 Markedly enhanced choroid plexuses (white arrowhead); B2 multiple malformations: multiple atrophy, schizencephaly (pink arrowhead), empty sella (red arrowhead), and enlarging fourth ventricle, large cyst and hypoplastic cerebellar vermis (green arrowhead) similar to Dandy–Walker complex (color figure online)
patient, the pathogeny may be correlated to duplication of chromosome 9 material and deletion of chromosome 1p26.33 [4, 5]. Conflict of interest We confirm the work described has not been published in whole or in part elsewhere,and it is not under consideration for publication elsewhere. This work does not receive any foundation and other requiring open access. All authors have no relevant financial interests and conflict of interest to disclose.
References 1. Cataltepe O, Liptzin D, Jolley L, Smith TW (2010) Diffuse villous hyperplasia of the choroid plexus and its surgical management. J Neurosurg Pediatr 5(5):518–522
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2. Nimjee SM, Powers CJ, McLendon RE, Grant GA, Fuchs HE (2010) Single-stage bilateral choroid plexectomy for choroid plexus papilloma in a patient presenting with high cerebrospinal fluid output. J Neurosurg Pediatr 5(4):342–345 3. Anei R, Hayashi Y, Hiroshima S, Mitsui N, Orimoto R, Uemori G, Saito M, Sato M, Wada H, Hododuka A, Kamada K (2011) Hydrocephalus due to diffuse villous hyperplasia of the choroid plexus. Neurol Med Chir (Tokyo) 51(6):437–441 4. Norman MG, Harrison KJ, Poskitt KJ, Kalousek DK (1995) Duplication of 9P and hyperplasia of the choroid plexus: a pathologic, radiologic, and molecular cytogenetics study. Pediatr Pathol Lab Med 15(1):109–120 5. Puvabanditsin S, Garrow E, Patel N, D’Elia A, Zaafran A, Phattraprayoon N, Davis SE (2008) Choroid plexus hyperplasia and monosomy 1p36: report of new findings. J Child Neurol 23(8):922–925
NEURO-IMAGES
Diffuse choroid plexus hyperplasia (CPH) associated with multiple malformations Yong Hong • Qiang Xu • Y. S. J. Maimaiti Junming Ye • Gong Chen
•
Received: 10 October 2014 / Accepted: 26 November 2014 Ó Belgian Neurological Society 2014
Keywords
Choroid plexus hyperplasia Malformation
A 32-year-old right-handed male presented with interruptible dizziness for 1 year, and aggravated clinical symptoms for 2 weeks, with headache, vomit, blurred vision and hyposmia. Magnetic resonance anography and magnetic resonance spectroscopy showed normal intracranial vessels and non-tumorous lesion. Magnetic resonance imaging (MRI) revealed diffuse choroid plexus hyperplasia (CPH) in bilateral ventricles and the third ventricle (Fig. 1, white arrowhead), which were markedly enhanced (Fig. 2, white arrowhead) in enhanced MRI image. Diffuse CPH lead to hydrocephalus due to cerebrospinal fluid (CSF) overproduction, and multiple malformations with multiple atrophies and schizencephaly
(Fig. 2, pink arrowhead), empty sella (Fig. 2, red arrowhead). In posterior fossa, the malformation resembled Dandy–Walker complex with the enlarging fourth ventricle, large cyst and hypoplastic cerebellar vermis (Fig. 2, green arrowhead). The neuroradiological malformation was more severe than that in last year, which is associated with clinical progressive intracranial hypertension. This patient will be treated by endoscopic coagulation of the choroid plexuses and then CSF ventriculoperitoneal shunt. CPH with hydrocephalus and (or) multiple atrophy is not rare [1–3], but it is rather rare that it is also associated with multiple malformations (schizencephaly, empty sella, the Dandy–Walker complex). Without the history of trauma, infection, hemorrhage or surgery in this adult
Y. Hong Q. Xu Department of Radiology, Huashan Hospital, Fudan University, Shanghai 200040, China e-mail: [email protected] Y. S. J. Maimaiti G. Chen (&) Department of Neurosurgery, Huashan Hospital, Fudan University, No.12, Wulumuqi Mid Road, Shanghai 200040, China e-mail: [email protected]; [email protected] J. Ye Department of Anesthesia, First Affiliated Hospital, Gannan Medical University, Ganzhou 200040, Jiangxi, China
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Acta Neurol Belg Fig. 1 FLAIR images of MRI revealed diffusely increased size of choroid plexuses along the entire choroid fissure in bilateral ventricles (A1) and the third ventricle (A2, white arrowhead)
Fig. 2 B1 Markedly enhanced choroid plexuses (white arrowhead); B2 multiple malformations: multiple atrophy, schizencephaly (pink arrowhead), empty sella (red arrowhead), and enlarging fourth ventricle, large cyst and hypoplastic cerebellar vermis (green arrowhead) similar to Dandy–Walker complex (color figure online)
patient, the pathogeny may be correlated to duplication of chromosome 9 material and deletion of chromosome 1p26.33 [4, 5]. Conflict of interest We confirm the work described has not been published in whole or in part elsewhere,and it is not under consideration for publication elsewhere. This work does not receive any foundation and other requiring open access. All authors have no relevant financial interests and conflict of interest to disclose.
References 1. Cataltepe O, Liptzin D, Jolley L, Smith TW (2010) Diffuse villous hyperplasia of the choroid plexus and its surgical management. J Neurosurg Pediatr 5(5):518–522
123
2. Nimjee SM, Powers CJ, McLendon RE, Grant GA, Fuchs HE (2010) Single-stage bilateral choroid plexectomy for choroid plexus papilloma in a patient presenting with high cerebrospinal fluid output. J Neurosurg Pediatr 5(4):342–345 3. Anei R, Hayashi Y, Hiroshima S, Mitsui N, Orimoto R, Uemori G, Saito M, Sato M, Wada H, Hododuka A, Kamada K (2011) Hydrocephalus due to diffuse villous hyperplasia of the choroid plexus. Neurol Med Chir (Tokyo) 51(6):437–441 4. Norman MG, Harrison KJ, Poskitt KJ, Kalousek DK (1995) Duplication of 9P and hyperplasia of the choroid plexus: a pathologic, radiologic, and molecular cytogenetics study. Pediatr Pathol Lab Med 15(1):109–120 5. Puvabanditsin S, Garrow E, Patel N, D’Elia A, Zaafran A, Phattraprayoon N, Davis SE (2008) Choroid plexus hyperplasia and monosomy 1p36: report of new findings. J Child Neurol 23(8):922–925
