Abstract
Van Wyk-Grumbach syndrome (VWGS) is characterized by juvenile hypothyroidism, delayed bone age, isosexual precocious puberty and massive ovarian cysts. We report such a case in a 7-year-old girl who presented with a bilateral pelvic pain, abdominal distension and signs of hypothyroidism. Physical examination showed a breast development and lack of pubic and axillary hair. There was no clitoromegaly. Pelvic ultrasonography and CT scan showed enlarged bilateral multiloculared multicystics ovarian masses. The pituitary MRI was recommended before an elevated prolactin, revealed a large sellar and suprasellar mass. The patient was started on thyroid replacement using L-Thyroxine. Clinically, the patient’s fatigue, pelvic pain, and abdominal distension are resolved. During the follow-up, her thyroid functions and serum prolactin normalized within 8 months. Ultrasonography revealed regression of ovarian cysts in 2 months measuring, with normal sized ovaries in 14 months.
Highlights
Van Wyk-Grumbach syndrome (VWGS) is characterized by juvenile hypothyroidism, delayed bone age, isosexual precocious puberty and massive ovarian cysts, with reversal to aprepubertal state following thyroid hormone replacement therapy [1]
Van Wyk and Grumbach first reported multicystic ovaries with precocious puberty in patients with hypothyroidism [3]. The characteristic of this syndrome is that the primary hypothyroidism has classically been associated with delayed growth and delayed puberty in children, but in this syndrome it can be associated with precocious puberty
All symptoms subside with thyroxine replacement, the endocrine abnormalities resolve, and even the ovarian cysts decrease in size or altogether disappear, as in the present case during follow-up [10] [11]
Summary
Van Wyk-Grumbach syndrome (VWGS) is characterized by juvenile hypothyroidism, delayed bone age, isosexual precocious puberty and massive ovarian cysts, with reversal to aprepubertal state following thyroid hormone replacement therapy [1]. This syndrome has been described since 1905, but the term Van Wyk and Grumbach syndrome was coined in 1960 [2]. Undiagnosed, this syndrome can lead to unnecessary surgery of the ovaries, while all the symptoms may get sorted with thyroxine hormone replacement. We report such a case in a 7-year-old girl
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