Clinicoradiological aspects of pontine tegmental cap dysplasia: Case report of a rare hindbrain malformation

Autor: Aanchal Bhayana, Sunil K Bajaj, Ritu N Misra, S Senthil Kumaran
Jazyk: angličtina
Rok vydání: 2018
Předmět:
Zdroj: Indian Journal of Radiology and Imaging, Vol 28, Iss 01, Pp 18-21 (2018)
Druh dokumentu: article
ISSN: 0971-3026
1998-3808
DOI: 10.4103/ijri.IJRI_25_17
Popis: Malformations involving the brainstem are very rare and present with a varied spectrum of clinical symptoms due to multiple cranial nerve palsies and pyramidal tract involvement. Of these, pontine tegmental cap dysplasia is a very unusual malformation, characterized by ventral pons hypoplasia and an ectopic dorsal band of tissue, projecting into the fourth ventricle, from dorsal pontine tegmentum. A 4-year-old male child, presenting with left facial nerve palsy, revealed hypoplastic ventral pons and an ectopic structure on magnetic resonance imaging (MRI). The ectopic structure was isointense to pons, arose from the left side of dorsal pontine tegmentum, at pontomedullary junction and protruded into the fourth ventricle, impinging upon the left seventh and eighth cranial nerves. Diffusion tensor imaging (DTI) depicted abnormal white matter tracts in ectopic tissue with absent transverse pontine fibres and abnormal middle and superior cerebellar peduncles. The typical MRI appearance, coupled with DTI, helped us reach an accurate diagnosis of pontine tegmental cap dysplasia, in a setting of neurological dysfunction.
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