Huge bilateral ovarian cysts in adulthood as the presenting feature of Van Wyk Grumbach syndrome due to chronic uncontrolled juvenile hypothyroidism
A 24-year-old woman presented to us with history of secondary amenorrhea, galactorrhea, abdominal pain, and distension of 6 months duration. Detailed inquiry revealed history of prolonged neonatal jaundice, delayed developmental milestones, short stature, and poor scholastic performance since childhood. She attained menarche at age of 7.5 years. She had irregular cycles with duration varying from 30 to 45 days with 4-6 days flow till 6 months back when she developed secondary amenorrhea. Examination was significant for short stature (height: 114.2 cm; <3rd percentile, standard deviation score (SDS)), weight 25.3 kg (<3rd percentile; SDS), subnormal intelligence, facial puffiness, pallor, pedal edema, dry coarse skin, and cold extremities. Thyroid was not palpable. She had spontaneous galoctorrhea from her left breast. Heart sounds were muffled. Abdominal examination revealed a painless palpable cystic mass sized 11 cm × 10 cm involving the whole abdomen []. She had delayed relaxation of deep tendon reflexes.\nInvestigations revealed microcytic hypochromic anemia, elevated serum TSH (>75 μIU/ml), elevated prolactin (>150 ng/ml) with low free thyroxine (T4) and luteinizing hormone (LH). FSH levels were normal. Serum for tumor markers revealed elevated CA-125 levels, normal beta human chorionic gonadotropin (HCG), and alpha fetoprotein []. Blood glucose, liver function tests, renal function tests, and electrolytes were normal. Abdominal ultrasonography and computed tomography showed a large uniloculor cystic SOL in right adnexa measuring 119 × 81 × 90 mm and a multicystic SOL in left adnexa measuring 55 × 45 × 49 mm suggestive of ovarian cysts []. The uterus was bulky with thickened heterogeneous cervix. Moderate pericardial effusion was noted on echocardiography. Levothyroxine was started at 50 μg per day which was gradually increased to 100 μg per day over 4 weeks. Last evaluated 12 weeks after the initial diagnosis, there was resolution of pericardial effusion, reduction in size of bilateral ovarian cysts along with normalization of CA-125 levels.