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Diencephalic syndrome: clinical features and imaging findings
T Y Poussaint1, P D Barnes, K Nichols
1Department of Radiology, Children's Hospital, Boston, MA 02115, USA.
AJNR. American Journal of Neuroradiology
|September 20, 1997
Summary
Diencephalic syndrome in children, often caused by hypothalamic/chiasmatic astrocytomas, presents with failure to thrive. Imaging is crucial for diagnosing these aggressive, large tumors in young children.
Area of Science:
- Pediatric Oncology
- Neuroradiology
- Pediatric Neurology
Background:
- Diencephalic syndrome is a rare condition in children.
- Hypothalamic/chiasmatic astrocytomas are a significant cause.
- Early diagnosis is often delayed due to non-specific symptoms.
Purpose of the Study:
- To highlight the critical role of imaging in diagnosing diencephalic syndrome.
- To emphasize the characteristics of astrocytomas associated with this syndrome.
Main Methods:
- Retrospective analysis of nine pediatric patients with diencephalic syndrome.
- Review of clinical records, imaging, and follow-up data.
- Histopathological confirmation of low-grade astrocytoma in biopsy samples.
Main Results:
- All patients presented with failure to thrive; tumors involved the hypothalamus and chiasm.
- Large tumor size (median 3.5 cm) and homogeneous enhancement were common findings.
- Hydrocephalus, metastases, and aggressive tumor behavior were observed, with high recurrence rates.
Conclusions:
- Diencephalic syndrome due to hypothalamic/chiasmatic astrocytoma requires prompt imaging for diagnosis.
- These tumors are larger, occur in younger children, and are more aggressive than other astrocytomas in this region.
- Imaging is vital for identifying tumor extent, complications like hydrocephalus, and potential metastases.