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RT Journal Article
SR Electronic
T1 Role of MR Imaging in Prenatal Diagnosis of Pregnancies at Risk for Joubert Syndrome and Related Cerebellar Disorders
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 424
OP 429
DO 10.3174/ajnr.A1867
VO 31
IS 3
A1 Saleem, S.N.
A1 Zaki, M.S.
YR 2010
UL http://www.ajnr.org/content/31/3/424.abstract
AB BACKGROUND AND PURPOSE: JSRD are rare autosomal recessive brain malformations. We hypothesized that MR imaging can assess fetuses at risk for JSRD and might influence their diagnoses. MATERIALS AND METHODS: We prospectively performed cranial MR imaging for 12 fetuses (mean GA, 23 weeks; SD, 3.7) at 25% recurrence risk for JSRD. We correlated prenatal MR imaging findings with postnatal MR imaging and clinical outcome. Retrospectively, we compared posterior fossa measurements of the cases with those of 24 age-matched fetuses with proved normal brain MR imaging. Institutional review board approval and consents were obtained. Statistical methods included a t test and ANCOVA tests. RESULTS: Fetal MR imaging correctly diagnosed 3 cases at 22, 28, and 29 weeks of gestation as JSRD, and 9 cases as normal. In JSRD-affected fetuses, prenatal MR imaging detected narrow pontomesencephalic junction (isthmus) with deepening of the interpeduncular fossa and thick horizontally placed superior cerebellar peduncles (MTS), deformed anteriorly convex floor of the fourth ventricle, and midline cerebellar cleft in place of the hypoplastic vermis. Measurements on axial fetal MR imaging at pontomesencephalic junction, ratio of AP diameters of interpeduncular fossa to midbrain/isthmus, and ratio of the AP to transverse diameters of the fourth ventricle were significantly higher in JSRD-affected fetuses than in nonaffected cases and the control group. CONCLUSIONS: MR imaging can diagnose JSRD in at-risk pregnancies by detecting posterior fossa signs. Measurements at the pontomesencephalic junction may enhance fetal MR imaging accuracy in diagnosing JSRD. ANCOVAanalysis of covarianceAPanteroposteriorCMcisterna magnaCOACH syndromecerebellar vermis hypoplasia/aplasia, oligophrenia, ataxia, coloboma, and hepatic fibrosis syndromeCORScerebello-oculo-renal syndromeDWMDandy-Walker malformationERGelectroretinogramsGAgestational ageIPinterpeduncular fossaJSJoubert syndromeJSRDJoubert syndrome and related cerebellar disordersMRIMR imagingMTSmolar tooth signOFD-VIorofacial-digital type VI syndromeUSultrasonographyVEPvisual-evoked potentials