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Choroid Plexus Hyperplasia : Report of Two Cases with Unique Radiologic Findings

  • Joo Whan Kim (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Waka Hisamura (Jikei University School of Medicine) ;
  • Seung-Ki Kim (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Ji Hoon Phi (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine)
  • 투고 : 2023.06.19
  • 심사 : 2023.10.16
  • 발행 : 2024.05.01

초록

Choroid plexus hyperplasia (CPH), also known as diffuse villous hyperplasia of choroid plexus, is a rare condition characterized by excessive production of cerebrospinal fluid (CSF), resulting in hydrocephalus. Diagnosing CPH can be challenging due to the absence of clear imaging criteria for choroid plexus hypertrophy and the inability to assess CSF production non-invasively. As a result, many CPH patients are initially treated with a ventriculoperitoneal (VP) shunt, but subsequently require additional surgical intervention due to intractable ascites. In our study, we encountered two CPH patients who presented with significantly enlarged subarachnoid spaces, reduced parenchymal volume, and prominent choroid plexus. Initially, we treated these patients with a VP shunt, but eventually opted for endoscopic choroid plexus cauterization (CPC) to address the intractable ascites. Following the treatment with endoscopic CPC, we observed a gradual reduction in subarachnoid spaces and an increase in parenchymal volume. In cases where bilateral prominent choroid plexus, markedly enlarged subarachnoid spaces, and cortical atrophy are present, CPH should be suspected. In these cases, considering initial treatment with combined endoscopic CPC and shunt may help minimize the need for multiple surgical interventions.

키워드

과제정보

This study was supported by a grant from SNUH Kun-hee Lee Child Cancer & Rare Disease Project, Republic of Korea (No. 22A-003-0100; to Phi JH).

참고문헌

  1. Anei R, Hayashi Y, Hiroshima S, Mitsui N, Orimoto R, Uemori G, et al. : Hydrocephalus due to diffuse villous hyperplasia of the choroid plexus. Neurol Med Chir (Tokyo) 51 : 437-441, 2011 https://doi.org/10.2176/nmc.51.437
  2. Aziz AA, Coleman L, Morokoff A, Maixner W : Diffuse choroid plexus hyperplasia: an under-diagnosed cause of hydrocephalus in children? Pediatr Radiol 35 : 815-818, 2005 https://doi.org/10.1007/s00247-005-1456-0
  3. Bering EA Jr, Sato O : Hydrocephalus: changes in formation and absorption of cerebrospinal fluid within the cerebral ventricles. J Neurosurg 20 : 1050-1063, 1963 https://doi.org/10.3171/jns.1963.20.12.1050
  4. Bernstock JD, Tafel I, Segar DJ, Dowd R, Kappel A, Chen JA, et al. : Complex management of hydrocephalus secondary to choroid plexus hyperplasia. World Neurosurg 141 : 101-109, 2020 https://doi.org/10.1016/j.wneu.2020.05.211
  5. Boxill M, Becher N, Sunde L, Thelle T : Choroid plexus hyperplasia and chromosome 9p gains. Am J Med Genet A 176 : 1416-1422, 2018 https://doi.org/10.1002/ajmg.a.38697
  6. Cataltepe O, Liptzin D, Jolley L, Smith TW : Diffuse villous hyperplasia of the choroid plexus and its surgical management. J Neurosurg Pediatr 5 : 518-522, 2010 https://doi.org/10.3171/2009.12.PEDS0960
  7. Davis LE : A physio-pathologic study of the choroid plexus with the report of a case of villous hypertrophy. J Med Res 44 : 521-534.11, 1924
  8. Hallaert GG, Vanhauwaert DJ, Logghe K, Van den Broecke C, Baert E, Van Roost D, et al. : Endoscopic coagulation of choroid plexus hyperplasia. J Neurosurg Pediatr 9 : 169-177, 2012 https://doi.org/10.3171/2011.11.PEDS11154
  9. Hussain ZB, Hussain AB, Mitchell P : Extra-axial cerebrospinal fluid spaces in children with benign external hydrocephalus: a case-control study. Neuroradiol J 30 : 410-417, 2017 https://doi.org/10.1177/1971400917719298
  10. Iplikcioglu AC, Bek S, Gokduman CA, Bikmaz K, Cosar M : Diffuse villous hyperplasia of choroid plexus. Acta Neurochir (Wien) 148 : 691-694; discussion 694, 2006 https://doi.org/10.1007/s00701-006-0753-1
  11. Li D, Shokuhfar T, Pantalone J, Rothstein B, Alden TD, Shaibani A, et al. : Choroidal artery embolization in the management of cerebrospinal fluid overproduction: case report and review of the literature. J Neurosurg Pediatr 23 : 737-748, 2019
  12. Paez-Nova M, Andaur K, Campos G, Garcia-Ballestas E, Moscote-Salazar LR, Koller O, et al. : Bilateral hyperplasia of choroid plexus with severe CSF production: a case report and review of the glymphatic system. Childs Nerv Syst 37 : 3521-3529, 2021 https://doi.org/10.1007/s00381-021-05325-2
  13. Philips MF, Shanno G, Duhaime AC : Treatment of villous hypertrophy of the choroid plexus by endoscopic contact coagulation. Pediatr Neurosurg 28 : 252-256, 1998 https://doi.org/10.1159/000028660
  14. Tamburrini G, Caldarelli M, Di Rocco F, Massimi L, D'Angelo L, Fasano T, et al. : The role of endoscopic choroid plexus coagulation in the surgical management of bilateral choroid plexuses hyperplasia. Childs Nerv Syst 22 : 605-608, 2006 https://doi.org/10.1007/s00381-006-0070-2
  15. Thomale UW : Integrated understanding of hydrocephalus - a practical approach for a complex disease. Childs Nerv Syst 37 : 3313-3324, 2021 https://doi.org/10.1007/s00381-021-05243-3
  16. Welch K, Strand R, Bresnan M, Cavazzuti V : Congenital hydrocephalus due to villous hypertrophy of the telencephalic choroid plexuses. Case report. J Neurosurg 59 : 172-175, 1983 https://doi.org/10.3171/jns.1983.59.1.0172
  17. Zahl SM, Egge A, Helseth E, Wester K : Clinical, radiological, and demographic details of benign external hydrocephalus: a population-based study. Pediatr Neurol 96 : 53-57, 2019 https://doi.org/10.1016/j.pediatrneurol.2019.01.015