Skip to main content

Advertisement

Log in

Hydranencephaly associated with cerebellar involvement and bilateral microphthalmia and colobomas

  • Case Report
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Hydranencephaly is an encephaloclastic central nervous system disorder characterised by severe destruction of the cerebral hemispheres with preservation of posterior fossa structures. We present MRI and neurosonography features of a unique case of hydranencephaly involving cerebellum (in the form of complete liquefaction of cerebellar hemispheres) and cerebral hemispheres with associated bilateral microphthalmia and ocular colobomas. This is an exceptional case as to the best to our knowledge. In humans, such a severe involvement of cerebellum has not been reported in cases of hydranencephaly. It is essential to distinguish hydranencephaly from gross hydrocephalus, as treatment and prognosis of the two are totally different. During differentiation, it is important to remember that severe cerebellar involvement can be seen in hydranencephaly.

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.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

References

  1. Barkovich AJ (2005) Pediatric neuroimaging, 4th edn. Lippincott Williams & Wilkins, Philadelphia

    Google Scholar 

  2. Poe LB, Coleman LL, Mahmud F (1989) Congenital central nervous system anomalies. Radiographics 9:801–826

    CAS  PubMed  Google Scholar 

  3. Filly RA (1994) Ultrasound evaluation of the fetal neural axis. In: Callen PW (ed) Ultrasonography in obstetrics and gynecology, 3rd edn. Saunders, Philadelphia, pp 199–218

    Google Scholar 

  4. Lemire RJ, Loeser JD, Leech RW et al (1975) Normal and abnormal development of the human nervous system. Harper & Row, Hagerstown, pp 251–255

    Google Scholar 

  5. Dublin AB, French BN (1980) Diagnostic image evaluation of hydranencephaly and pictorially similar entities with emphasis on computed tomography. Radiology 137:81–91

    CAS  PubMed  Google Scholar 

  6. Sharp NJ, Davis BJ, Guy JS et al (1999) Hydranencephaly and cerebellar hypoplasia in two kittens attributed to intrauterine parvovirus infection. J Comp Pathol 121:39–53

    Article  CAS  PubMed  Google Scholar 

  7. Sutton LN, Bruce DA, Schut L (1980) Hydranencephaly versus maximal hydrocephalus: an important clinical distinction. Neurosurgery 6:34–38

    CAS  PubMed  Google Scholar 

  8. Srikantha U, Morab JV, Sastry S et al (2009) Outcome of ventriculoperitoneal shunt placement in grade IV tubercular meningitis with hydrocephalus: a retrospective analysis in 95 patients. J Neurosurg Pediatr 4:176–183

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Kiran M. Sargar.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Taori, K.B., Sargar, K.M., Disawal, A. et al. Hydranencephaly associated with cerebellar involvement and bilateral microphthalmia and colobomas. Pediatr Radiol 41, 270–273 (2011). https://doi.org/10.1007/s00247-010-1749-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-010-1749-9

Keywords

Navigation