Dados do Trabalho

Título

CLINICAL AND NEUROIMAGING FEATURES OF CONGENITAL CYSTIC MALFORMATIONS OF THE POSTERIOR FOSSA: AN ILLUSTRATED GUIDE

Introdução

Congenital abnormalities of the posterior fossa, whose diagnosis can be made in the pre- and postnatal periods, represent a spectrum of morphological malformations that occur during embryonic development. Its pathophysiology is related to disorders involving the main components of this brain region, such as the pons, the medulla oblongata, the fourth ventricle, and the cerebellum.

Objetivo

To describe the clinical and neuroimaging features of congenital cystic malformations of the posterior fossa through an illustrated guide.

Método

To compose the illustrated guide, self-authored drawings were produced. Moreover, using our personal archive, we added neuroimaging exams of children affected by the aforementioned conditions.

Resultados e Conclusões

Congenital cystic anomalies of the posterior fossa include Dandy-Walker malformation (DWM), Blake’s pouch cyst (BPC), mega cisterna magna (MCM), arachnoid cyst (AC), and cerebellar vermis hypoplasia (CVH). DWM presents with signs and symptoms of increased intracranial pressure. Typical neuroimaging features are a hypoplastic, elevated and rotated vermis, cystic dilatation of the fourth ventricle, and widening of the posterior fossa. BPC can manifest macrocephaly, hydrocephalus, ataxia, seizures, and dizziness. Its radiological findings include presence of a retro or infra cerebellar cyst due to dilation of the fourth ventricle. Most children with isolated MCM are asymptomatic. Radiologically, the cisterna magna and the posterior fossa are enlarged, but the vermis is intact. AC is usually asymptomatic, but it may also course with headache and increased intracranial pressure. On imaging, the cyst frequently presents ovoid with isointense content relative to cerebrospinal fluid. CVH leads to a milder clinical picture, for instance, a mild cognitive impairment. The radiological pattern is partial hypoplasia of the inferior vermis without other notable changes.
Thus, through an illustrated guide, we summarize the clinical and neuroimaging aspects of cystic congenital malformations of the posterior fossa in order to provide a didactic guide for neurologists and child neurologists.

Palavras Chave

Neuroanatomy; Neuroimaging; Congenital Abnormalities.

Declaração de conflito de interesses de TODOS os autores

The authors have no conflict of interests to declare.