Balram Sharma, Hema Singh, Sanjay Saran, Sandeep Kumar Mathur
Thyroid Research and Practice 2017 14(2):81-85
The association in young females of long-standing primary hypothyroidism, isosexual precocious pseudopuberty, and multicystic enlarged ovaries was first described in 1960 by Van Wyk and Grumbach. In this case study, we report a girl with precocious puberty, poor linear growth, decreased vision, and a large pituitary pseudotumor due to long-standing hypothyroidism with regression of all components following thyroxine (T4) supplementation. This girl aged 12 years and 3 months presented in Endocrinology Department with complaints of early menarche starting at the age of 8 years with normal cycles along with early progressive breast development starting almost simultaneously. On examination, she had a reduced growth for age (<5th centile) with adequate breast development (Tanner Stage 3) but no pubic or axillary hair development. Physical and biochemical examination for blood indices revealed a microcytic hypochromic anemia. Most importantly, she had an elevated thyroid stimulating hormone >150 μIU/ml (0.35–5.5) and a free T4 (FT4) and free triiodothyronine below normal limits suggestive of primary hypothyroidism. Furthermore, serum prolactin levels were elevated along with an elevated serum follicle-stimulating hormone, luteinizing hormone, and estradiol. Multicystic ovaries and a bulky uterus on ultrasound were suggestive of precocious puberty. Magnetic resonance imaging scan of the sella turcica was suggestive of a pituitary macroadenoma. Posttreatment with gluten-free diet, iron supplements, and T4 replacement, her thyroid function, hemoglobin, and prolactin normalized along with a regression in the size of the ovary. Therefore, in patients of this age presenting with a pituitary macroadenoma, anemia, precocious puberty, and primary hypothyroidism, medical management was preferred over neurosurgical intervention so as to avoid permanent hypopituitarism and lifelong hormone replacement therapy.
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