ArticleAJNR. American journal of neuroradiology2009
Pontine tegmental cap dysplasia: MR imaging and diffusion tensor imaging features of impaired axonal navigation.
Article in AJNR. American journal of neuroradiology, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers, 1 of them a synthesis that pooled it.
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Who cites it
35 citing papers in PubMed, 1 synthesis or guideline pooled it, 86 citations in OpenAlex.
- Update on neuroimaging phenotypes of mid-hindbrain malformations.Neuroradiology · 2015Pooled it
- Partitioned Internal Auditory Canal: Association With Cochlear Aperture Anomalies.The Laryngoscope · 2026Article
- Pontine Tegmental Cap Dysplasia Presenting With Global Developmental Delay and Vestibulocochlear Nerve Aplasia: A Case Report.Clinical case reports · 2026Article
- Dominant and recessive ATOH1 variants cause distinct neurodevelopmental disorders with hearing loss.American journal of human genetics · 2026Article
- Case Report: A case of Joubert syndrome in twin pregnancy: MRI manifestations and literature review.Frontiers in pediatrics · 2026Article
- 선천성 뇌 질환의 영상 소견: 임상 화보.Journal of the Korean Society of Radiology · 2026Review
- Fetal imaging of posterior fossa malformations.Pediatric radiology · 2025Review
- Pontine tegmental cap dysplasia: the role of diffusion tensor imaging.BMJ case reports · 2023Article
- Dandy-Walker Phenotype with Brainstem Involvement: 2 Distinct Subgroups with Different Prognosis.AJNR. American journal of neuroradiology · 2023Article
- Central sleep apnea in patients with pontine tegmental cap dysplasia treated with supplemental oxygen: a case report.Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine · 2023Article
- Medullary Tegmental Cap Dysplasia: Fetal and Postnatal Presentations of a Unique Brainstem Malformation.AJNR. American journal of neuroradiology · 2023Review
- A rare cause of arterial hypertension in a child with developmental delay: Answers.Pediatric nephrology (Berlin, Germany) · 2022Article
- Diagnostic Approach to Cerebellar Hypoplasia.Cerebellum (London, England) · 2021Review
- Magnetic resonance imaging of the brainstem in children, part 1: imaging techniques, embryology, anatomy and review of congenital conditions.Pediatric radiology · 2021Review
- Tractography of the Cerebellar Peduncles in Second- and Third-Trimester Fetuses.AJNR. American journal of neuroradiology · 2021Article
- Visual Neuropsychology in Development: Anatomo-Functional Brain Mechanisms of Action/Perception Binding in Health and Disease.Frontiers in human neuroscience · 2021Review
- Delineation of the nerve fiber bundles of the infant brain associated with aging using phase difference-enhanced imaging: a preliminary study.Japanese journal of radiology · 2020Article
- Pontine tegmental cap dysplasia with a duplicated internal auditory canal.Radiology case reports · 2019Article
- Tegmento-vermian dysplasia: radiologic features of a novel described anatomic variation.Surgical and radiologic anatomy : SRA · 2018Article
- Temporal bone and cranial nerve findings in pontine tegmental cap dysplasia.Neuroradiology · 2016Article
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Authors and funding
8 authors at 5 institutions in 3 countries.
Funding
Abstract
background and purposeMalformations of the brain stem are uncommon. We present MR imaging and diffusion tensor imaging (DTI) features of 6 patients with pontine tegmental cap dysplasia, characterized by ventral pontine hypoplasia and a dorsal "bump," and speculate on potential mechanisms by which it forms. MATERIALS AND
methodsBirth and developmental records of 6 patients were reviewed. We reviewed MR imaging studies of all patients and DTIs of patient 3. Potential developmental causes were evaluated.
resultsAll patients were born uneventfully after normal pregnancies except patient 6 (in utero growth retardation). They presented with multiple cranial neuropathies and evidence of cerebellar dysfunction. Variable hypotonia and motor dysfunction were present. Imaging revealed ventral pontine hypoplasia and mild cerebellar vermian hypoplasia, in addition to an unusual rounded to beaklike "bump" on the dorsal surface of the pons, extending into the fourth ventricle. Color fractional anisotropy maps showed the bump to consist of a bundle of axons directed horizontally (left-right). The bump appeared, on morphologic images, to be continuous with the middle cerebellar peduncles (MCPs), which were slightly diminished in size compared with those in healthy infants. Analysis of the DTI was, however, inconclusive regarding the connections of these axons. The decussation of the MCPs, transverse pontine fibers, and longitudinal brain stem axonal pathways was also abnormal.
conclusionsOur data suggest that the dorsal transverse axonal band in these disorders results from abnormal axonal pathfinding, abnormal neuronal migration, or a combination of the 2 processes.
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