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CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2018; 28(01): 18-21
DOI: 10.4103/ijri.IJRI_25_17
Neuroradiology & Head and Neck Imaging

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

Aanchal Bhayana
Department of Radiodiagnosis, Safdarjung Hospital and VM Medical College, All India Institute of Medical Sciences, New Delhi, India
,
Sunil K Bajaj
Department of Radiodiagnosis, Safdarjung Hospital and VM Medical College, All India Institute of Medical Sciences, New Delhi, India
,
Ritu N Misra
Department of Radiodiagnosis, Safdarjung Hospital and VM Medical College, All India Institute of Medical Sciences, New Delhi, India
,
S Senthil Kumaran
Department of Nuclear Medical Resonance, All India Institute of Medical Sciences, New Delhi, India
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Subject Editor: Financial support and sponsorship Nil.
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Abstract

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.

Keywords

Brainstem malformations - cranial nerve palsies - hindbrain malformations - pontine tegmental cap dysplasia


Publication History

Article published online:
26 July 2021

© 2018. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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  • References

  • 1 Jissendi-Tchofo P, Doherty D, McGillivray G, Hevner R, Shaw D, Ishak G. et al. Pontine Tegmental Cap Dysplasia: MR Imaging and Diffusion Tensor Imaging Features of Impaired Axonal Navigation. Am J Neuroradiol 2009; 30: 113-9
    CrossrefPubMedGoogle Scholar
  • 2 Barth PG, Majoie CB, Caan MW, Marian A, Weterman J, Kyllerman M. et al. Pontine tegmental cap dysplasia: Anovel brain malformation with a defect in axonal guidance. Brain 2007; 130: 2258-66
    CrossrefPubMedGoogle Scholar
  • 3 Stankiewicz P, Lupski JR. Genome architecture, rearrangements and genomic disorders. Trends Genet 2002; 18: 74-82
    CrossrefPubMedGoogle Scholar
  • 4 Maeoka Y, Yamamoto T, Ohtani K, Takeshita K. Pontine hypoplasia in a child with sensorineural deafness. Brain Dev 1997; 19: 436-9
    CrossrefPubMedGoogle Scholar
  • 5 Ouanounou S, Saigal G, Birchansky S. Möbius syndrome. AJNR Am J Neuroradiol 2005; 26: 430-2
    PubMedGoogle Scholar
  • 6 Harding B, Vossough A, Goldberg E, Santi M. Pontine tegmental cap dysplasia: Neuropathological confirmation of a rare clinical/radiological syndrome. Neuropathol Appl Neurobiol 2016; 42: 301-6
    CrossrefPubMedGoogle Scholar
  • 7 Singh D, Hsu CC, Kwan GN, Korah I. Pontine Tegmental Cap Dysplasia: MR Evaluation of Vestibulocochlear Neuropathy. J Neuroimaging 2015; 25: 1038-43
    CrossrefPubMedGoogle Scholar
  • 8 Nixon JN, Dempsey JC, Doherty D, Ishak GE. Temporal bone and cranial nerve findings in pontine tegmental cap dysplasia. Neuroradiol 2016; 58: 179-87
    CrossrefPubMedGoogle Scholar
  • 9 Doherty D, Millen KJ, Barkovich AJ. Midbrain and hindbrain malformation: Advances in clinical diagnosis, imaging, and genetics. Lancet Neurol 2013; 12: 381-93
    CrossrefPubMedGoogle Scholar