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Blakeʼs Pouch Cysts and Differential Diagnoses in Prenatal and Postnatal MRI

A Pictorial Review

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Abstract

Purpose

The clinical variability of Blakeʼs pouch cysts (BPC) may range from asymptomatic via ataxia to sequelae of decompensated hydrocephalus. On the other hand, Dandy-Walker malformation (DWM) and cerebellar vermis hypoplasia generally correlate with less favorable neurologic development. The aim was to illustrate the potential of prenatal and postnatal neuroimaging to distinguish a BPC or persistent BP from other posterior fossa malformations.

Methods

This pictorial review addresses the inconsistent nomenclature, clinical features, and magnetic resonance imaging (MRI) patterns of BPC and five differential diagnoses. The MRI findings of 11 patients, acquired at up to 3 T in 3 institutions, are demonstrated. Furthermore, the literature was searched for recent improvements in genetic and embryological background knowledge.

Results

Posterior fossa malformations often resemble each other and may even be imitated by sequelae of hemorrhagic, ischemic or infectious disruptions, i.e. congenital anomalies of morphology despite normal developmental potential. Hydrocephalus is a typical, albeit not always congenital finding in BPC. It is frequently associated with cerebellar disruptions and DWM; however, it is also a rare complication of posterior fossa arachnoid cysts. A moderately elevated vermis needs follow-up to confirm persistent BP versus vermian hypoplasia or DWM. The fetal cerebellar tail, previously assumed to be specific for DWM, may be imitated in cases of persistent BP.

Conclusion

The accurate diagnosis of isolated BPC is not always straightforward, which is especially critical in the context of fetomaternal medicine. A detailed description of posterior fossa malformations is to be preferred over unspecific terminology.

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Authors and Affiliations

  1. Institute of Radiology, Villach General Hospital, Villach, Austria

    Thomas Kau

  2. Division of Pediatric Radiology, Department of Radiology, Medical University of Graz, Graz, Austria

    Thomas Kau, Robert Marterer & Eszter Nagy

  3. Section of Neuroradiology, Department of Diagnostic Imaging and Intervention, University Children’s Hospital Zurich, Zurich, Switzerland

    Raimund Kottke

  4. Department of Pediatrics and Adolescent Medicine, Villach General Hospital, Villach, Austria

    Robert Birnbacher

  5. Division of Obstetrics and Maternal Fetal Medicine, Department of Gynecology and Obstetrics, Medical University of Graz, Graz, Austria

    Janos Gellen

  6. Department of Gynecology and Obstetrics, Villach General Hospital, Villach, Austria

    Janos Gellen

  7. Department of Pediatric Neurology, University Children’s Hospital Zurich, Zurich, Switzerland

    Eugen Boltshauser

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Correspondence to Thomas Kau.

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Conflict of interest

T. Kau, R. Marterer, R. Kottke, R. Birnbacher, J. Gellen, E. Nagy and E. Boltshauser declare that they have no competing interests.

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All investigations described in this manuscript were carried out with the approval of the responsible ethics committee and in accordance with national law and the Helsinki Declaration of 1975 (in its current revised form). Informed consent was obtained from patients if identifiable from images or other information within the manuscript. In the case of underage patients informed consent was obtained from the legal representatives.

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Author Contributions

T. Kau had the idea for this article, performed literature search, provided data, and drafted the article, E. Boltshauser performed literature search, provided data, and critically revised the article, R. Kottke provided data and critically revised the article, R. Marterer, R. Birnbacher, J. Gellen and E. Nagy commented on the data and critically revised the manuscript.

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Kau, T., Marterer, R., Kottke, R. et al. Blakeʼs Pouch Cysts and Differential Diagnoses in Prenatal and Postnatal MRI. Clin Neuroradiol 30, 435–445 (2020). https://doi.org/10.1007/s00062-019-00871-4

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