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RHOMBOENCEPHALOSYNAPSIS MR-SEMIOTICS AND DIFFERENTIAL DIAGNOSIS
Author(s) -
M. V. Polyanskaya,
А. А. Демушкина,
Yu. A. Biryukova,
А. А. Алиханов
Publication year - 2018
Publication title -
lučevaâ diagnostika i terapiâ
Language(s) - English
Resource type - Journals
eISSN - 2079-5351
pISSN - 2079-5343
DOI - 10.22328/2079-5343-2018-9-2-28-33
Subject(s) - differential diagnosis , hypoplasia , medicine , aplasia , cerebellar hypoplasia (non human) , posterior cranial fossa , posterior fossa , neuroimaging , radiology , cerebellar vermis , cerebellum , pathology , anatomy , psychiatry
Rombencephalosynapsys (RES) is a rare variant of anomalies of the posterior cranial fossa structures characterized by dysplastic fusion of cerebellar hemispheres and absence or hypoplasia of cerebellar vermis. Purpose: to demonstrate the possibilities of modern neuroimaging in the RES diagnosis and to identify the main markers of the differential diagnosis in posterior fossa structures (PFS) anomalies. In the department of Radiology of Russian Children’s Clinical Hospital, we observed 3 patients with RES (1 girl and 2 boys). Patients age varied from 3 months to 9 years. The studies were performed on high-field MR system GE Discovery 750 W 3 T. Results: оn MRI in patients with RES we identified the spectrum of dysplastic anomalies of the vermis, from complete absence to partial aplasia with preservation of its anterior part. Also, there were 2 case with cerebellar dysplastic features, resemling RES. The transcerebellar sulci were estimated as the a clue diagnostic marker of RES. Conclusion: High-field MRI is the preferred diagnostic tool in the definition and differentiation of the developmental anomalies of the PFC in the children, especially in patients with RES.

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