"Bridging the gap between hypothyroidism and precocious puberty: A case report of Von Wyk Grumbach Syndrome".

Abstract

Introduction: Von Wyk Grumbach syndrome (VWGS) is a rare clinical entity that presents with the triad of precocious puberty, polycystic ovaries, and severe hypothyroidism. VWGS is an uncommon cause of precocious puberty characterized by dissociation of gonadarche and adrenarche. Unlike other aetiologies of precocious puberty, VWGS is associated with absent growth acceleration and delayed bone maturation due to prolonged, untreated hypothyroidism. The diagnosis is often delayed or overlooked, as the overt or unusual pubertal manifestations may overshadow the subtle signs of hypothyroidism. Failure to identify hypothyroidism as cause of ovarian cyst and precocious puberty may lead to catastrophic ovarian resection and oophorectomy in prepubertal girls and adolescents.

Case presentation: We present a case of VWGS in a 07 years old girl presenting with painful abdomen with precocious puberty and menarche .Relevant review of the literature on this uncommon clinical entity is also presented.

Conclusion: This study underscores the importance of recognizing the atypical presentation of prolonged hypothyroidism with precocious puberty in young children. It emphasizes the need for clinicians to consider the rare entity of VWGS, which could help prevent delays in appropriate management.