Letter to the editor

Indian Pediatrics 2001; 38: 432-433  

Isolated Menarche and Multicystic Ovaries in a 7½ Year Girl with Hypothyroidism

Juvenile hypothyroidism is a common endocrine disorder which manifests as short stature and delayed puberty. However, there are anecdotal reports of precocious puberty, isolated menarche, multicystic ovaries, galactorrhea and macro-orchidism in these patients. We report an unusual case of hypo-thyroidism in girl with a lingual thyroid gland who presented with premature menarche without other features of precocity and multi-cystic ovaries.

A 9½-year-old girl presented with irregular menstrual bleeding since the age of 7½ years and short stature (height <5th percentile). In addition she had other overt features of hypothyroidism. Her hypothyroid Belliwicz score was 48. Thyroid was not palpable. Sexual development was: Pubic hair P1 and Breast B2. Her intelligence quotient (IQ) was 100 (average) by Vineland Social Maturity Scale. Investigations revealed: mild macro-cytic anemia, hypercholestrolemia, normal chest X-ray, T3 - 0.36 ng/dL (N: 0.86 -1.8), T4 1.7 µg/dL (N: 4.5-12.0), TSH - 51.0 mIU/ml (N: 0.2 - 5.0); prolactin - 877 mU/L (N: 78-455), thyroid scan – poorly functioning lingual thyroid gland, LHRH stimulation test – prepubertal response, bone-age – 6.9 years by Tanner-White House (TW) scoring system and ultrasound pelvis-multiple variable sized follicles in both ovaries. Based upon the clinical profile and investigations the patient was diagnosed as juvenile hypothyroidism (lingual thyroid gland), with incomplete GnRH independent precocious puberty. On four months follow-up, while on L-thyroxine, patient has gained 3 cm height and she did not have any more vaginal bleeding and there is a significant reduction in size of ovaries.

The entity of hypothyroidism with precocious puberty was first described by Kendle in 1905. The syndrome of advanced sexual development with hypothyroidism is apparently rare. Clinical features in girls include galactorrhea, increased pigmentation and enlargement of pituitary fossa, cysts in ovaries and menstrual disturbances in the form of irregular vaginal bleeding without advanced bone age and adrenarche(1-3). Boys present with macro-orchidism. The usual cause is Hashimoto’s thyroiditis. Therapy with thyroxine results in prompt alleviation of symptoms. The mechanism of precocity in hypothyroidism is GnRH independent. In 1960, Van Wyk and Grumback(1) suggested hormonal overlap in negative feed back regulation with increased secretion of gonadotrophins. Recently, Anasti and coworkers(4) have suggested that TSH directly interacts with human FSH receptors resulting in sexual precocity in hypothyroid patients.

To conclude rarely juvenile hypo-thyroidism can present as sexual precocity in form of isolated menarche with delayed bone age, retarded growth and multicystic ovaries. Treatment with thyroxine results in ameliora-tion of menstrual bleeding and ovarian cyst. Thus in any patient of the sexual precocity with retarded bone age, one should look for hypothyroidism.

Dinesh Dhanwal,
Nikhil Tandon,
Department of Endocrinology and Metabolism,
All India Institute of Medical Sciences,
New Delhi 110 029, India.

1. Van Wyk J, Greumbach MM. Syndrome of precocious menstruation and galactorrhea in juvenile hypothyroidism: An example of hormonal overlap pituitary feedback. J Pediatr 1960; 57: 416.

2. Rakover Y, Weiner E, Shalev E, Luboshitsky R. Vaginal bleeding: presenting symptoms of acquired primary hypothyroidism in a seven year old girl. J Pediatr Encocrinol 1993; 6: 197-200.

3. Chen CH, Tiu CM, Chon YH, Chen WY, Hwang B, Niu DM. Congenital hypothyroidism with multiple ovarian cysts. Eur J Pediatr 1999; 158: 851-852.

4. Anasti JN, Flack MR, Froehlich J, Nelson LM, Nirula BC. A potential novel mechanism for precocious puberty in juvenile hypothy-roidism. J Clin Endocrinol Metab 1995; 80: 276-279.