Pediatric Radiology

, Volume 24, Issue 8, pp 543–546 | Cite as

Vermian agenesis without posterior fossa cyst

  • C. Adamsbaum
  • V. Moreau
  • C. Bulteau
  • J. Burstyn
  • F. Lair Milan
  • G. Kalifa
Selected papers from the 31st Congress of the European Society of Pediatric Radiology, Brussels, Belgium, June 1–3, 1994


We report 11 cases of vermian partial agenesis without posterior fossa cyst or hemispheric abnormalities. Characteristic MR signs were: absence of the posterior lobe, hypoplasia of the anterior lobe, a narrow sagittal cleft separating the hemispheres (“buttocks sign”) and fourth ventricle deformity. The main clinical signs were complex oculomotor dysfunction and developmental delay. None of the patients had respiratory symptoms. Consideration is given to the relationship between Joubert syndrome and this entity as well as to embryological data.


Public Health Clinical Sign Respiratory Symptom Developmental Delay Posterior Fossa 
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Copyright information

© Springer-Verlag 1994

Authors and Affiliations

  • C. Adamsbaum
    • 1
  • V. Moreau
    • 1
  • C. Bulteau
    • 2
  • J. Burstyn
    • 3
  • F. Lair Milan
    • 1
  • G. Kalifa
    • 1
  1. 1.Department of Pediatric RadiologySaint Vincent de Paul HospitalParis Cedex 14France
  2. 2.Department of Pédiatric NeurologySaint Vincent de Paul HospitalParis Cedex 14France
  3. 3.Department of Pediatric OphthalmologySaint Vincent de Paul HospitalParis Cedex 14France

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