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Full-Endoscopic Surgery for Hypothalamic Hamartoma Resection
Published on: April 12, 2024
[Central precocious puberty due to hypothalamic hamartoma in Cameroon]
H D Mbassi Awa1, M T Abena Obama, A Pondy
1Service de Pédiatrie, CHU, Yaoundé, Cameroun. mbassiahd@yahoo.fr
Insights
Hypothalamic hamartoma can cause central precocious puberty. Treatment with gonadotropin-releasing hormone analogs can slow puberty, but accessibility is a challenge in developing nations.
Area of Science:
- Pediatric Endocrinology
- Pediatric Neurology
Background:
- Hypothalamic hamartoma is a rare cause of central precocious puberty (CPP).
- CPP presents with early onset of secondary sexual characteristics.
Observation:
- An 18-month-old girl presented with vaginal bleeding at 8 months, indicative of CPP.
- Clinical examination revealed Tanner stage 3 puberty; psychomotor development was normal, and no epilepsy was observed.
- Hormonal evaluation and brain MRI confirmed a hypothalamic hamartoma.
Findings:
- Magnetic resonance imaging (MRI) is crucial for diagnosing hypothalamic hamartoma.
- Long-acting gonadotropin-releasing hormone (GnRH) analog administration improved clinical symptoms.
- Neurosurgery is not indicated for patients without epileptic seizures.
Implications:
- Hypothalamic hamartoma should be considered in cases of CPP.
- Accessibility and cost of MRI and GnRH agonists pose challenges in low-income countries.
- GnRH agonists are effective in managing CPP, improving adult height, but affordability remains a barrier globally.
Abstract:
Hypothalamic hamartoma is an uncommon cause of central precocious puberty and sometimes of gelastic epilepsy and delayed development. The purpose of this report is to describe a case of central precocious puberty in an 18-month-old girl who was referred to our department for further investigation of vaginal bleeding that had appeared during the 8th month of life. The patient's puberty was compatible with Tanner stage 3, her psychomotor development was normal, and epilepsy was not observed. Diagnosis was based on abnormal hormonal workup findings and discovery of a hypothalamic hamartoma on brain magnetic resonance imaging. Neurosurgery was not performed. Administration of long-acting gonadotropin releasing hormone analog to slow puberty led to clinical improvement. Hypothalamic hamartoma must always be suspected in case of central precocious puberty. Although magnetic resonance imaging is the most reliable modality, it is not always available nor affordable in low-income countries. Neurosurgery is not indicated in patients without epileptic seizure. Gonadotropin releasing hormone agonists are usually indicated to slow down puberty and improve the adult height. However, the cost is prohibitive in developing countries.
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