A case of multiple ovarian cysts in a prepubertal girl with severe hypothyroidism due to autoimmune thyroiditis

Abstract

Some children with profound and long-standing hypothyroidism present with signs of paradoxical sexual maturation. In females, it is characterized by breast development, vaginal bleeding, lack of pubic hair, and delayed bone age. A case of multiple ovarian cysts in a prepubertal girl with severe hypothyroidism due to autoimmune thyroiditis is reported. A 7-year, 8-month-old girl presented with precocious puberty and vaginal bleeding. A pelvic ultrasound revealed markedly enlarged ovaries with numerous cysts which disappeared dramatically after thyroid replacement. When large multiple ovarian cysts with vaginal bleeding are found in a prepubertal girl who has a retarded bone age, the possibility of associated hypothyroidism should be considered. Health care providers should keep this in mind because this constellation of symptoms can be completely reversed with the initiation of thyroid replacement.

An unusual presentation of acquired hypothyroidism: the Van Wyk–Grumbach syndrome

“…Girls with this syndrome have breast development, follicular cysts and menstruation in the absence of pubic or axillary hair, which depends on adrenal androgens (2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22). Histopathological analysis of resected ovaries and ovarian cysts shows cystic follicles and little if any luteinisation.…”

“…Loss of negative feedback from thyroid hormones in primary hypothyroidism not only results in high TRH levels, hyperplasia of the TSHsecreting cells in the pituitary but also stimulates prolactin secretion (5,6,13,14,30,31,32). In addition, elevated oestrogen concentrations reduce prolactin inhibitory factor via a negative feedback mechanism, causing a further increase in prolactin (8,33).…”

“…Positive thyroid peroxidase antibodies confirmed autoimmune thyroiditis; TPO antibody titre was 378 kU/l (normal range 0-35 kU/l). Further blood tests highlighted a raised prolactin at 1310 mU/l, deficient vitamin D (16.2 nmol/l) and a suppressed parathyroid hormone (PTH) of 7 ng/l (13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29), in the presence of normal calcium and phosphate. She had a raised white blood cell count of 14.9 gG/l, raised ESR and severe, hyporegenerative, microcytic anaemia (Table 2).…”

“…Biochemically, low free thyroxine (T 4 ) is combined with raised levels of thyroid stimulating hormone (TSH), prolactin and oestradiol. Typically, luteinising hormone-releasing hormone (LHRH) stimulation shows an FSH-dominated prepubertal response with suppressed luteinising hormone (LH), confirming gonadotropin-releasing hormone (GnRH)-independent precocious pseudopuberty (2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22). Most cases in the literature are secondary to autoimmune thyroid disease but there are some case reports where the syndrome is secondary to unrecognised congenital hypothyroidism (5,10,12).…”

“…Most cases in the literature are secondary to autoimmune thyroid disease but there are some case reports where the syndrome is secondary to unrecognised congenital hypothyroidism (5,10,12). It is important to recognise this syndrome as symptoms regress with thyroid hormone replacement and patients spontaneously enter true puberty at an appropriate time (2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22).…”