Hypothalamic hamartomas are benign lesions in the hypothalamus that can cause precocious puberty. They do this by secreting GnRH inappropriately at a young age. The type of precocious puberty and other symptoms depend on the location and size of the hamartoma. Sessile hamartomas near the mammillary bodies commonly cause gelastic seizures. Larger hamartomas spanning from the mammillary body to the infundibulum can cause both precocious puberty and gelastic seizures. Treatment involves long-acting GnRH analog injections or nasal sprays. Surgery may be considered for cases refractory to medication, when tissue diagnosis is needed, or due

1. Hypothalamic hamartoma

2. What is precocious puberty ???
• Secondary sexual characters Before 8 years in females and 9 yrs in
males

3. What is hypothalamic hamartoma
• Hypothalamic hamartomas are benign developmental lesions
comprising of dysplastic neurons, interspersed with glial tissue.
• The only and most common endocrine abnormality detected in these
lesions is the GnRH hypersecretion at an inappropriate age.( rarely
GH)

8. • BASED ON THE IMAGING FINDINGS:
• Isolated CPP, fromtuber cinereum They are usually pedunculated
parahypothalamic hamartomas
• The sessile intrahypothalamic close to the mammillary bodies with
gelastic seizures and DRE
• Large HH with broad-based attachment to the third ventricular floor
can present with features of both DRE and CPP.
•

9. Schematic illustration depicting the diencephalon in midsagittal section. (a) Sessile hypothalamic hamartomas (HH) arising from hypothalamic floor attached to mamillary body are more prone
to develop DRE. (b) Pedunculated HH arising from the 3rd ventricular floor close to the infandibulum usually present with CPP. (c) Large HH with broad attachment spanning from mamillary
body till the infandibulum present with a combination of DRE and CPP.

10. CPP
• long-acting GnRH analogs like leuprolide (7.5 / 11.25 mg) and
triptorelin (7.5 / 11.25 mg), 1 or 3 monthly depot im INJ
• GnRH nasal sprays (Busarelin/Nefarelin)

11. SURGERY IN HH
• Surgery may be offered to the following subset of patients:
• (i) refractory to medical (GnRHa) therapy,
• (ii) in the presence of diagnostic dilemma where tissue diagnosis is
required, and
• (iii) those who are unable to afford medical therapy due to financial
reasons, especially in the resource constrained nations