Skip to main content
Log in

“Growing cerebellum” requiring operative decompression following perinatal ventriculoperitoneal shunting

  • Case-Based Review
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Introduction

While cases of acquired Chiari I malformation following ventriculoperitoneal shunting for posthemorrhagic hydrocephalus have been reported, true disproportionate cerebellar growth is rare, with no previous cases requiring posterior fossa decompression reported.

Clinical presentation

We present a premature neonate who underwent ventriculoperitoneal shunt placement for suspected posthemorrhagic hydrocephalus. He subsequently developed a symptomatic Chiari I malformation with volumetric measurements demonstrating disproportionate growth of the cerebellum. He did not demonstrate thickening of the supratentorial or posterior fossa cranium. The patient underwent an extradural posterior fossa decompression, with resolution of symptoms.

Outcome and conclusions

We review the extant literature regarding the development of Chiari malformation type I as a manifestation of craniocerebral disproportion (CCD) following shunt placement for posthemorrhagic hydrocephalus of prematurity. Most previous reports reflect a mechanism that includes underdevelopment of the intracranial posterior fossa (or supratentorial) volume. The case presented in this report, as well as one additional case, indicates that there may exist a variant mechanism, characterized by rapid growth of the cerebellum itself, in the absence of one of the rare syndromes associated with primary macrocerebellum. While this case was effectively managed with extradural posterior fossa decompression, previous reports indicate that supratentorial cranial expansion procedures are preferable in some cases. As such, pediatric neurosurgeons should be able to distinguish the patterns of craniocerebral disproportion when considering treatment options for these patients. Further investigation regarding these uncommon patients may better describe the underlying mechanisms.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Kazan S, Güra A, Uçar T et al (2005) Hydrocephalus after intraventricular hemorrhage in preterm and low–birth weight infants: analysis of associated risk factors for ventriculoperitoneal shunting. Surg Neurol 64:S77–S81. https://doi.org/10.1016/j.surneu.2005.07.035

    Article  PubMed  Google Scholar 

  2. Loop JW, Foltz EL (1972) Craniostenosis and diploic lamination following operation for hydrocephalus. Acta Radiol Diagn 13:8–13

    Article  CAS  Google Scholar 

  3. Sandler AL, Goodrich JT, Daniels LB et al (2013) Craniocerebral disproportion: a topical review and proposal toward a new definition, diagnosis, and treatment protocol. Childs Nerv Syst 29:1997–2010. https://doi.org/10.1007/s00381-013-2257-7

    Article  PubMed  Google Scholar 

  4. Ryoo HG, Kim S-K, Cheon J-E et al (2014) Slit ventricle syndrome and early-onset secondary craniosynostosis in an infant. American Journal of Case Reports 15:246–253. https://doi.org/10.12659/AJCR.890590

    Article  PubMed  PubMed Central  Google Scholar 

  5. Osuagwu FC, Lazareff JA, Rahman S, Bash S (2006) Chiari I anatomy after ventriculoperitoneal shunting: posterior fossa volumetric evaluation with MRI. Childs Nerv Syst 22:1451–1456. https://doi.org/10.1007/s00381-006-0118-3

    Article  PubMed  Google Scholar 

  6. Caldarelli M, Novegno F, Di Rocco C (2009) A late complication of CSF shunting: acquired Chiari I malformation. Childs Nerv Syst 25:443–452. https://doi.org/10.1007/s00381-008-0760-z

    Article  PubMed  Google Scholar 

  7. D’Arco F, Ugga L, Caranci F et al (2016) Isolated macrocerebellum: description of six cases and literature review. Quant Imaging Med Surg 6:496–503. https://doi.org/10.21037/qims.2016.06.10

    Article  PubMed  PubMed Central  Google Scholar 

  8. Benvenisti H, Bassan H, Shiran S et al (2015) “Growing” cerebellum in an infant after shunt insertion. Pediatr Neurol 52:222–225. https://doi.org/10.1016/j.pediatrneurol.2014.10.003

    Article  PubMed  Google Scholar 

  9. Hoffman HJ, Tucker WS (1976) Cephalocranial disproportion: a complication of the treatment of hydrocephalus in children. Child’s Brain 2:167–176

    CAS  PubMed  Google Scholar 

  10. Poretti A, Mall V, Smitka M et al (2012) Macrocerebellum: significance and pathogenic considerations. Cerebellum 11:1026–1036. https://doi.org/10.1007/s12311-012-0379-1

    Article  PubMed  Google Scholar 

  11. Torreman M, Smit L, van der Valk P, et al (1993) A case of macrocephaly, hydrocephalus, megacerebellum, white matter abnormalities and Rosenthal fibres. Dev Med Child Neurol 35:732–736

  12. van der Knaap MS, Salomons GS, Li R, et al (2005) Unusual variants of alexander’s disease: atypical alexander’s disease. Ann Neurol 57:327–338. https://doi.org/10.1002/ana.20381

  13. Gripp KW, Hopkins E, Doyle D, Dobyns WB (2010) High incidence of progressive postnatal cerebellar enlargement in Costello syndrome: brain overgrowth associated with HRAS mutations as the likely cause of structural brain and spinal cord abnormalities. Am J Med Genet A 152A:1161–1168. https://doi.org/10.1002/ajmg.a.33391

    Article  PubMed  PubMed Central  Google Scholar 

  14. Schaefer GB, Bodensteiner JB, Buehler BA et al (1997) The neuroimaging findings in Sotos syndrome. Am J Med Genet 68:462–465. https://doi.org/10.1002/(SICI)1096-8628(19970211)68:4<462::AID-AJMG18>3.0.CO;2-Q

    Article  CAS  PubMed  Google Scholar 

  15. Di Rocco C, Velardi F (2003) Acquired Chiari type I malformation managed by supratentorial cranial enlargement. Childs Nerv Syst 19:800–807. https://doi.org/10.1007/s00381-003-0837-7

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Todd C. Hankinson.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Ethics approval

This paper does not require the authorization of the Ethical Committee of our Hospital.

Informed consent

Individual patient consent was not required as the study was retrospective one and was undertaken with local institutional approval.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hengartner, A.C., Putty, M., Young, M. et al. “Growing cerebellum” requiring operative decompression following perinatal ventriculoperitoneal shunting. Childs Nerv Syst 37, 13–19 (2021). https://doi.org/10.1007/s00381-020-04864-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-020-04864-4

Keywords

Navigation