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Sprengel Deformity
Joseph Junewick, MD FACR
over 5 years ago
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Case History

2 year old female with precocious puberty.

Case Detail

Anatomy: Brain-Spine
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 5 years ago
Updated: over 5 years ago
Tags: PEDS
Modality/Study Types: MR
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Case Images

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Reveal Findings

MR – Sagittal T1, T2 and postgadolinium T1 and axial T2-FLAIR images show a nonenhancing T1-isonintense and mildly T2-hyperintense lesion emaning from the inferior aspect of the hypothalamus; the pituitary stalk is deviated anteriorly but the pituitary gland is normal.

Reveal Discussion

Hypothalamic hamartoma is a developmental mass of the tuber cinereum of the hypothalamus. Patients are most often males in their first or second decade of life. These lesions are divided into two main types: parahypothalamic hamartomas and intrahypothalamic hamartomas. Parahypothalamic hamartomas are pedunculated masses that are attached to the floor of the hypothalamus by a narrow base and are more likely to be associated with precocious puberty. Intrahypothalamic hamartomas arise within the substance of the hypothalamus itself and are more often associated with gelastic seizures. At pathologic analysis, hypothalamic hamartomas contain nerve cells that resemble those of the normal hypothalamus, along with normal glial cells. Hypothalamic hamartomas are isointense to mildly hypointense on T1-weighted images and isointense to hyperintense on T2-weighted images, with no contrast enhancement or calcification. Stability further supports the diagnosis.

Reveal Diagnosis

Parahypothalamic Hypothalamic Hamartoma

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