title: Congenital basis of posterior fossa anomalies.
abstract: The classification of posterior fossa congenital anomalies has been a controversial topic. Advances in genetics and imaging have allowed a better understanding of the embryologic development of these abnormalities. A new classification schema correlates the embryologic, morphologic, and genetic bases of these anomalies in order to better distinguish and describe them. Although they provide a better understanding of the clinical aspects and genetics of these disorders, it is crucial for the radiologist to be able to diagnose the congenital posterior fossa anomalies based on their morphology, since neuroimaging is usually the initial step when these disorders are suspected. We divide the most common posterior fossa congenital anomalies into two groups: 1) hindbrain malformations, including diseases with cerebellar or vermian agenesis, aplasia or hypoplasia and cystic posterior fossa anomalies; and 2) cranial vault malformations. In addition, we will review the embryologic development of the posterior fossa and, from the perspective of embryonic development, will describe the imaging appearance of congenital posterior fossa anomalies. Knowledge of the developmental bases of these malformations facilitates detection of the morphological changes identified on imaging, allowing accurate differentiation and diagnosis of congenital posterior fossa anomalies.
authors.affiliation: Department of Radiology, and Pediatric Radiology, The University of Texas Medical Branch, Galveston, TX, USA.
authors.forename: Claudia
authors.initials: C
authors.lastname: Cotes
authors.affiliation: Department of Diagnostic and Interventional Imaging, University of Texas Health Science Center at Houston, Houston, TX, USA.
authors.forename: Eliana
authors.initials: E
authors.lastname: Bonfante
authors.affiliation: University of Pennsylvania; Philadelphia, PA, USA.
authors.forename: Jillian
authors.initials: J
authors.lastname: Lazor
authors.affiliation: Texas Children's Hospital, Houston, TX, USA.
authors.forename: Siddharth
authors.initials: S
authors.lastname: Jadhav
authors.affiliation: Texas Children's Hospital, Houston, TX, USA.
authors.forename: Maria
authors.initials: M
authors.lastname: Caldas
authors.affiliation: Department of Radiology, and Pediatric Radiology, The University of Texas Medical Branch, Galveston, TX, USA.
authors.forename: Leonard
authors.initials: L
authors.lastname: Swischuk
authors.affiliation: Department of Diagnostic and Interventional Imaging, University of Texas Health Science Center at Houston, Houston, TX, USA riascosr@hotmail.com.
authors.forename: Roy
authors.initials: R
authors.lastname: Riascos
mesh_terms: D000015:Abnormalities, Multiple; D016080:Arachnoid Cysts / Q000151:congenital* / Q000196:embryology; D001139:Arnold-Chiari Malformation / Q000196:embryology; D002526:Cerebellar Diseases / Q000151:congenital* / Q000196:embryology; D002531:Cerebellum / Q000002:abnormalities; D003388:Cranial Fossa, Posterior / Q000002:abnormalities* / Q000196:embryology; D003616:Dandy-Walker Syndrome / Q000196:embryology; D005124:Eye Abnormalities / Q000196:embryology; D006223:Hamartoma Syndrome, Multiple / Q000151:congenital* / Q000196:embryology; D006801:Humans; D052177:Kidney Diseases, Cystic / Q000196:embryology; D008636:Mesencephalon / Q000002:abnormalities* / Q000196:embryology; D012160:Retina / Q000002:abnormalities / Q000196:embryology; D012249:Rhombencephalon / Q000002:abnormalities* / Q000196:embryology; D058494:Walker-Warburg Syndrome / Q000196:embryology
keywords: Posterior fossa anomalies; congenital posterior fossa abnormalities; development posterior fossa; embryology posterior fossa; imaging posterior fossa