Thyroxine replacement promptly corrects vaginal bleeding in precocious menarche due to Van Wyk Grumbach syndrome

Sapna Vinit Amin, Sunanda Bharatnur, Akhila Vasudeva, Shripad S. Hebbar

Abstract


Van Wyk Grumbach syndrome is a rare complication of a common endocrine disorder, i.e., hypothyroidism, but occurs when it is long standing during periods of pre-pubertal growth.  It can occur in both sexes, in boys it is associated with testicular enlargement and in girls, the usual presenting features are early onset menarche, enlarged bilateral multicystic ovaries and delayed skeletal growth. Diagnosing this condition is very important as unnecessary surgical intervention for ovarian enlargement is avoided. Prompt replacement therapy with thyroxine not only corrects menstrual abnormality, but also ovaries return to normal over period of time.  In this case report, we describe an unusual case of irregular vaginal bleeding, large multicystic ovaries and delayed bone age in a young girl due to autoimmune thyroiditis who promptly responded to levo-thyroxine.


Keywords


Van Wyk Grumbach syndrome, Precocious puberty, Delayed bone age, Multicystic ovaries

Full Text:

PDF

References


Lafranchi S. Disorders of thyroid gland. In: Kliegman MR, Stanton BMD, Geme JS, Schor NF, Behrman RE, eds. Nelson Text Book of Pediatrics. 19th ed. Philadelphia, PA: Saunders; 2011: 1894-1918.

Piziak VK, Hahn HB. Isolated menarche in juvenile hypothyroidism. Clin Pediatr (Phila). 1984;23(3):177-9.

Van Wyk JJ, Grumbach MM. Syndrome of precocious menstruation and galactorrhoea in juvenile hypothyroidism: an example of hormonal overlap in pituitary feedback. J Pediatr. 1960;57:416-35.

Ryan GL, Feng X, d’Alva CB, Zhang M, Van Voorhis BJ, Pinto EM, et al. Evaluating the roles of follicle-stimulating hormone receptor polymorphisms in gonadal hyperstimulation associated with severe juvenile primary hypothyroidism. J Clin Endocrinol Metab. 2007;92(6):2312-7.

Kroeze WK, Sheffler DJ, Roth BL. G-protein-coupled receptors at a glance. J Cell Sci. 2003;116:4867-9.

Indumathi CK, Bantwal G, Patil M. Primary hypothyroidism with precocious puberty and bilateral cystic ovaries. Indian J Pediatr. 2007;74(8):781-3.

Tanner JM, Whitehouse RH, Cameron N, Marshall WA, Healy MJR, Goldstein H. Assessment of skeletal maturity and prediction of adult height. In: Tanner JM, Whitehouse RH, Cameron N, Marshall WA, Healy MJR, Goldstein H, eds. A Book. 2nd ed. London: Academic Press; 1983.

Milner GR, Levick RK, Kay R. Assessment of bone age: a comparison of the Greulich and Pyle and the Tanner and Whitehouse methods. Clin Radiol. 1986;37:119-21.

Rastogi A, Bhadada SK, Bhansali A. An unusual presentation of a usual disorder: Van Wyk-Grumbach syndrome. Indian J Endocrinol Metab. 2011;15(Suppl 2):S141-3.

Anjana Hulse. Van Wyk-Grumbach syndrome and pituitary hyperplasia in a six-year-old girl. Apollo Med. 2014;11(1):40-2.

Khawaja NM, Taher BM, Barham ME, Naser AA, Hadidy AM, Ahmad AT, et al. Pituitary enlargement in patients with primary hypothyroidism. Endocr Pract. 2006;12(1):29-34.

Campaner AB, Scapinelli A, Machado RO, Dos Santos RE, Beznos GW, Aoki T. Primary hypothyroidism presenting as ovarian tumor and precocious puberty in a prepubertal girl. Gynecol Endocrinol. 2006;22(7):395-8.

Ozgen T, Güven A, Aydin M. Precocious puberty in a girl with Down syndrome due to primary hypothyroidism. Turk J Pediatr. 2009;51(4):381-3.

Browne LP, Boswell HB, Crotty EJ, O’Hara SM, Birkemeier KL, Guillerman RP. Van Wyk and Grumbach syndrome revisited: imaging and clinical findings in pre- and post-pubertal girls. Pediatr Radiol. 2008;38(5):538-42.

Baranowski E, Högler W. An unusual presentation of acquired hypothyroidism: the Van Wyk-Grumbach syndrome. Eur J Endocrinol. 2012;166(3):537-42.

Durbin KL, Diaz-Montes T, Loveless MB. Van wyk and Grumbach syndrome: an unusual case and review of the literature. J Pediatr Adolesc Gynecol. 2011;24(4):e93-6.

Tran S, Kim EE, Chin AC. Severe menorrhagia, unilateral ovarian mass, elevated inhibin levels, and severe hypothyroidism: an unusual presentation of Van Wyk and Grumbach syndrome. J Pediatr Surg. 2013;48(1):e51-4.