Precocious puberty and large multicystic ovaries in young girls with primary hypothyroidism.
OBJECTIVE
To describe 2 cases of primary hypothyroidism, precocious puberty, large multicystic ovaries, possible diagnostic dilemma, unilateral oophorectomies, and subsequent response to levothyroxine replacement therapy.


METHODS
We present the clinical, biochemical, radiologic, and histopathologic findings in 2 patients with rare cases of Van Wyk-Grumbach syndrome and megaovaries, who underwent unilateral oophorectomy.


RESULTS
Two patients, an 8-year-old girl and a 3-year-old girl (cases 1 and 2, respectively), were referred to our center. Both patients presented with precocious puberty and vaginal bleeding and had undergone unilateral oophorectomy before referral. In the first patient (case 1), the surgical intervention was a consequence of torsion of the left megaovary, necessitating emergency oophorectomy. Oophorectomy in the second patient (case 2) was a result of initial diagnostic confusion, inasmuch as a sexcord stromal tumor was suspected. A detailed history, physical examination, and laboratory results pointed toward primary hypothyroidism due to Hashimoto's thyroiditis and thyroid dysgenesis, respectively. Serial ultrasound studies of the abdomen and pelvis revealed large multicystic ovaries, with progressive enlargement (including regrowth from an apparent ovarian "postsurgical remnant"). Both patients responded dramatically after initiation of levothyroxine replacement therapy, with no further vaginal bleeding and reversal of megaovary to normal size (in case 1).


CONCLUSIONS
In a highly selected minority of children with untreated primary hypothyroidism, there is development of precocious puberty and progressively enlarging multicystic ovaries. The precise endocrine, neuroanatomic, and neurophysiologic bases for this phenomenon are unclear. Nevertheless, the entire clinicopathologic picture, including giant ovaries, dramatically reverts to normal status with the restoration of a euthyroid state by means of simple levothyroxine replacement therapy.
Sanjeevaiah
Aravind Raj
AR
Samatvam Endocrinology Diabetes Center, Bangalore, India.
Sanjay
Subbarayappa
S
Deepak
Tejesweni
T
Sharada
Ardanareshwaran
A
Srikanta
Sri S
SS
D000293:Adolescent; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007037:Hypothyroidism / Q000150:complications* / Q000188:drug therapy; D010052:Ovariectomy; D011085:Polycystic Ovary Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D011629:Puberty, Precocious / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D013974:Thyroxine / Q000627:therapeutic use; D016896:Treatment Outcome
D013974:Thyroxine