Bridging the Gap between Hypothyroidism and Precocious Puberty: A Case Report of Van Wyk Grumbach Syndrome.

INTRODUCTION

Van 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 the dissociation of gonadarche and adrenarche. Unlike other etiologies 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 the cause of ovarian cysts and precocious puberty may lead to catastrophic ovary resection and oophorectomy in prepubertal girls and adolescents.

CASE PRESENTATION: We present a case of VWGS in a 7-year-old girl presenting with a painful abdomen with precocious puberty and menarche. A 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. This emphasizes the need for clinicians to consider the rare entity of VWGS, which could help prevent delays in appropriate management.