Abstract

GARCIA SAEZ, Julieta et al. Van Wyk-Grumbach syndrome. Rev Cubana Endocrinol [online]. 2019, vol.30, n.2  Epub May 26, 2020. ISSN 1561-2953.

Van Wyk-Grumbach syndrome is characterized by long-lasting primary hypothyroidism associated with precocious puberty. A case of a 7-year-old female mestizo patient is reported. She came to consultation for vaginal bleeding, accompanied by hyperpigmentation of the areolas without galactorrhea, globular abdomen, myxedema, hirsutism, short stature, asthenia and school delay. Blood chemistry showed anemia, hypercholesterolemia and hypertransaminasemia. Imaging studies showed pericardial effusion, marked delay in bone age, increased dimensions of Turkish chair and large ovarian cyst with apparent surgical criteria. Hormonal studies confirmed the suspicion of primary hypothyroidism associated with hyperprolactinemia. Substitute treatment with levothyroxine sodium reversed the signs and symptoms of precocious puberty, which prevented ovarian cyst surgery; the recovery in the school and social environment was certain. Recognizing this entity avoids absolutely contraindicated treatments, such as the removal of cysts or the surgical treatment of pituitary hyperplasia.

Keywords : early puberty; primary hypothyroidism; Van Wyk-Grumbach syndrome.

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