Very rare cause of precocious puberty. Association of primary hypothyroidism (usually acquired: thyroiditis) with precocious pseudopuberty, multicystic ovaries and pituitary adenoma with or without hyperprolactinemia, and short stature. Very high TSH levels stimulate FSH receptors. In addition to the classic signs of hypothyroidism, there are:

-        in girls: progressive breast enlargement (sometimes galactorrhea), multicystic ovaries, vaginal bleeding; absence of pubic and axillary hair. The clincal  presentation may require surgery: ovarian tumour, torsion of an ovary, abdominal pain.

-        in boys: enlarged testicles, no signs of virilization.

sella turcica: sometimes enlarged

Bone age: delayed.

Biology: collapsed T4 levels, very high TSH levels, sometimes falsely elevated CA125 antigen.

All abnormalities regress when thyroid therapy is administered.

Anesthetic implications:

check thyroid hormone levels, thyroid hormone replacement therapy

References :

-        Razi SM, Gupta AK, Gupta DC, Gutch M, Gupta KK, Usman SI.
Van Wyk-Grumbach syndrome with Kocher-Debré-Sémélaigne syndrome: case report of a rare association.
Eur Thyroid J 2017;6:47–51

-        Egodawaththe NS, Seneviratne SN, Gunasekara S, Amarasekara SM, Weerasekara K.
Van Wyk-Grumbach syndrome and oligosyndactyly in a 6-year-old girl: a case report.
Journal of Medical Case Reports 2020 ; 14:166

-        Gupta J, Lin-Su K.
Van Wyk-Grumbach syndrome in a female pediatric patient with trisomy 21: a case report.
Int J Pediatr Endocrinology  2020:2, doi.org/10.1186/s13633-020-0072-y

-        Boddu SK, Ayyavoo A, Nagarajappa VH, Kalenahalli KV, Muruda S, Palany R.
Van Wyk Grumbach syndrome and ovarian hyperstimulation in juvenile primary hypothyroidism: lessons from a 30-Case cohort.
J of the Endocrine Society 2023 ; 7 : 1–8, doi.org/10.1210/jendso/bvad042        

Updated: May 2024