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Indian Pediatr 2018;55: 77 |
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Primary Hypothyroidism Presenting as
Bilateral Ovarian Torsion
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* Sandeep Agarwala and Alisha
Gupta
Department of Pediatric Surgery, AIIMS, New Delhi,
India.
Email: [email protected]
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Van Wyk – Grumbach Syndrome, first described in 1960,
is characterized by isosexual precocious puberty, delayed bone age and
bilateral multicystic ovaries in the background of primary
hypothyroidism (secondary to autoimmune thyroiditis) [1,2]. The inherent
pathophysiology involves the action of raised Thyroid-stimulating
hormone (TSH) on Follicle-stimulating hormone (FSH) receptors, leading
to the development of pseudoprecocity. These hormones share a common
a- subunit
which acts through similar transmembrane receptors [3]. Such cases may
present with bilateral ovarian masses and end up with bilateral
oophorectomy if the primary physician is unaware of underlying
pathophysiology.
An 11-year-old girl presented to us with acute
abdominal pain of 7 days duration, associated with vomiting and
abdominal distension. The patient had achieved menarche 6 months ago.
There were no neurological complaints. The patient had short stature,
obesity, and features of peritonism and shock. Contrast-enhanced
computed tomography scan showed two multiloculated adnexal masses. Serum
a-fetoprotein
and b-human
chorionic gonadotropin levels were normal.
On surgical exploration, massive hemorrhagic ascites
was revealed. Both ovaries were enlarged (15 cm on right and 11 cm on
left) with multiloculated cysts. They had undergone torsion and were
grossly gangrenous. Bilateral salpingo-oophorectomy was done.
Histopathologic evaluation of the specimens revealed bilateral
follicular cysts with surrounding edema with large areas of hemorrhage.
Three months post-operatively, the child complained
of abnormal weight gain along with muscle weakness. Thyroid profile
showed raised TSH level (>100 uIU/mL) with low free T3 and T4, normal
FSH and LH and raised estradiol. On imaging, there was a delayed bone
age. Patient was treated with thyroid hormone replacement and within 1
month, she improved symptomatically with a reduction in weight and
improved muscle weakness. At present, she is 18-year-old with normal
breast and feminine hair development, on oral estrogen and thyroid
hormone replacement therapies, and is clinically and biochemically
euthyroid.
Any child of isosexual precocious puberty presenting
with bilateral ovarian masses should undergo bone age estimation, as
delayed bone age in this context clinches the diagnosis of Van
Wyk-Grumbach syndrome, which can further be confirmed by hormonal
analysis. Initiation of thyroid hormone replacement results in
improvement in endocrine abnormalities, final height achieved as well as
resolution of ovarian masses, thus preserving ovarian reserve in the
child [4]. Only in case of ovarian torsion and ischemia, surgery is
warranted. In all other less acute cases, pharmacotherapy may be
sufficient and avoids unnecessary surgery [5].
References
1. Van Wyk JJ, Grumbach MM. Syndrome of precocious
menstruation and galactorrhea in juvenile hypothyroidism: An example of
hormonal overlap in pituitary feedback. J Pediatr. 1960;57:416-y N35.
2. Gordon CM, Austin DJ, Radovick S, Laufer MR.
Primary hypothyroidism presenting as severe vaginal bleeding in a
prepubertal girl. J Pediatr Adolesc Gynecol. 1997;10:35-8.
3. Kroeze WK, Sheffler DJ, Roth BL. G-protein-coupled
receptors at a glance. J Cell Sci. 2003;116:4867-9.
4. Bhattacharya M, Mitra AK. Regression of precocious
puberty in a child with hypothyroidism after thyroxine therapy. Indian
Pediatr. 1992;29:96-8.
5. Van Voorhis BJ, Neff TW, Syrop CH, Chapler FK.
Primary hypothyroidism associated with multicystic ovaries and ovarian
torsion in an adult. Obstet Gynecol. 1994;83:885-7.
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