title: Rhombencephalosynapsis - isolated anomaly or complex malformation?
abstract: BACKGROUND
  Rhombencephalosynapsis (RES) is a rare malformation of the posterior cranial fossa, characterized by fusion of the cerebellar hemispheres, medial cerebellar peduncles and dentate nuclei. Over the period of 7 years 8 cases of this anomaly have been diagnosed in two pediatric centers in Warsaw including one on the prenatal magnetic resonance imaging (MRI).
  
  
  METHODS
  Material consists of involves one fetus examined at the gestational age of 27 and 33 weeks and 7 children (5 girls and 2 boys) aged 8 months - 16 years. All of them underwent brain MRI with the use of 1.5T scanners.
  
  
  RESULTS
  In 1 case RES was an isolated anomaly, in 1 case it was accompanied by hydrocephalus only, in the remaining 6 cases RES was an element of a complex malformation. The additional anomalies were as follows: callosal hypoplasia in 3 children, abnormalities of gyration in 2, brainstem hypoplasia in 2, isolated fourth ventricle in 1, abnormal white matter signal intensity in 4 (in 2 cases in supratentorial compartment, in 1 in the cerebellum and in 1 in the pons), abnormally dilated extraaxial fluid collections in 2, syringohydromyelia in 2. In 5 cases RES was total, in 3 - partial.
  
  
  CONCLUSIONS
  Rhombencephalosynapsis has a very characteristic appearance on magnetic resonance imaging which allows diagnosis of this malformation at any age, including prenatal period.
authors.affiliation: Department of Diagnostic Imaging, Institute of Mother and Child, Warsaw, Poland.
authors.forename: Monika
authors.initials: M
authors.lastname: Bekiesińska-Figatowska
authors.forename: Elżbieta
authors.initials: E
authors.lastname: Jurkiewicz
authors.forename: Sylwia
authors.initials: S
authors.lastname: Szkudlińska-Pawlak
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Malczyk
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Nowak