ESPE Abstracts (2019) 92 P3-330

ESPE2019 Poster Category 3 Late Breaking Abstracts (69 abstracts)

Van-Wyk Grumbach Syndrome Associated with Trisomy 21: A Case Report

Nihad Selim & Nadhira Bouchair


Algeria


Introduction: Van-Wyk Grumbach syndrome (VWGS) described in 1960 associate Primary hypothyroidism to early puberty, polycystic ovaries and pituitary adenoma with or without hyperprolactinemia. It is a very rare cause of precocious puberty, which the etiopathogenis is not yet very clear.

Observation: We report the case of an 8 year old girl known for trisomy 21, she presented a Primary hypothyroidism treated initially with Levothyroxine at low dose and developed 6 months later an early puberty with vaginal bleeding and polycystic ovaries suggesting the diagnosis of VWGS. Her MRI showed a pituitary macroadenoma that resolved completely after only 90 days of adequate dose of levothyroxine with clinical and emotional improvement.

Conclusion: The Van-Wyk Grumbach syndrome is very rare, but remains an important entity to know because of its good prognosis under medical treatment that avoids the use of unnecessary surgeries (polycystic ovaries) and risky (pituitary macroadenoma) .

Volume 92

58th Annual ESPE

Vienna, Austria
19 Sep 2019 - 21 Sep 2019

European Society for Paediatric Endocrinology 

Browse other volumes

Article tools

My recent searches

No recent searches.