[Precocious puberty and hypothalamic hamartoma: treatment with triptorelin during eight years]

Abstract

Background: Hypothalamic hamartoma, a rare cause of true sexual precocity, may develop slowly so that its diagnosis may be late.

Case report: A 7 1/2-month-old girl was admitted because she suffered from development of physical pubertal changes. Laboratory tests were consistent with premature activation of the hypothalamic-pituitary axis. Initial neuroradiological investigation failed to find any cause. The patient was given medroxyprogesterone that resulted in regression of secondary sexual characteristics but growth rates and skeletal maturity remained accelerated. For that reason, the patient was given triptorexine, LH-RH analogue, 18 months later that resulted in a deceleration of puberty growth rate and skeletal maturity. MRI performed at the age of 9 years showed a small hypothalamic hamartoma and the LH-RH analogue was stopped at the age of 10 years.

Conclusion: MRI is now the best technique for investigating the hypothalamo-hypophyseal area.