EC Paediatrics

Chiari malformations (CM) are intricate brain deformities first described and characterized by Hans Chiari in 1891 [1]. They constitute a group of disorders involving abnormalities in the posterior fossa and posterior brain. There are seven types of CM: 0, I, 1.5, II, III, IV, and V [1]. Chiari malformation type III (CM III) is an exceedingly rare condition characterized by a low occipital or upper cervical encephalocele and downward displacement of the brainstem. Type III carries a grim prognosis, marked by a high mortality rate and severe neurological and developmental deficits in surviving patients [2]. We present the case of a newborn female with a lumbo-sacral mass, clubfoot, and dysmorphic facial features. An MRI revealed an open lumbosacral myelomeningocele associated with an occipital encephalocele and downward protrusion of the cerebellum, fitting within the framework of a Chiari type 3 malformation.

 Keywords: Chiari Malformation Type III; MRI; Encephalocele; Amygdaloid Protrusion

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