Abstract
Background
In 1960 Van Wyk and Grumbach described a syndrome of juvenile hypothyroidism, precocious puberty and ovarian enlargement. These findings undergo complete regression with thyroid hormone replacement therapy. This diagnosis can be made on the basis of imaging findings and thyroid function analysis, avoiding surgery.
Objective
To relate the distinctive clinical and imaging features and putative pathophysiological mechanism of a series of patients with Van Wyk and Grumbach syndrome (VWGS).
Materials and methods
Patients with VWGS diagnosed at two large children’s hospitals over a 6-year period beginning in 1999 were retrospectively reviewed. A literature review was also conducted.
Results
Five female patients were diagnosed with cystic ovarian enlargement and hypothyroidism at ages ranging from 9 to 17 years. Isosexual precocious puberty was found in prepubescent patients. Associated findings included delayed bone age, ascites, and pleural and pericardial effusions. Ovarian cyst involution occurred following treatment of the hypothyroidism.
Conclusion
The association of primary hypothyroidism with cystic ovarian enlargement and precocious puberty is important to recognize. In the absence of suspected ovarian torsion, surgery is unnecessary, as cyst regression occurs after appropriate thyroid hormone replacement. Noncompliance with hormone replacement therapy should be considered when cystic ovarian enlargement is noted in patients with a history of hypothyroidism.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Van Wyk JJ, Grumbach MM (1960) Syndrome of precocious menstruation and galactorrhea in juvenile hypothyroidism: an example of hormonal overlap in pituitary feedback. J Pediatr 57:416
Wood LC, Olichney M, Locke H et al (1965) Syndrome of juvenile hypothyroidism associated with advanced sexual development: report of two new cases and comment on the management of an associated ovarian mass. J Clin Endocrinol Metab 25:1289–1295
Lindsay AN, Voorhess ML, MacGillivray MH (1980) Multicystic ovaries detected by sonography. Am J Dis Child 134:588–592
Gilsanz V, Stanley P (1980) Pediatric. Case 3. Juvenile hypothyroidism with enlargement of the ovaries. AJR 134:1294–1295
Gregory JL, Wilson DM, Parker B (1992) The overlap syndrome: an unusual presentation. Am J Dis Child 146:421–422
Van Voorhis BJ, Neff TW, Syrop CH et al (1994) Primary hypothyroidism associated with multicystic ovaries and ovarian torsion in an adult. Obstet Gynecol 83:885–887
Riddlesberger MM Jr, Kuhn JP, Munschauer RW (1981) The association of juvenile hypothyroidism and cystic ovaries. Radiology 139:77–80
Chattopadhyay A, Kumar V, Marulaiah M (2003) Polycystic ovaries, precocious puberty and acquired hypothyroidism: the Van Wyk and Grumbach syndrome. J Pediatr Surg 38:1390–1392
Takeuchi K, Deguchi M, Takeshima Y et al (2004) A case of multiple ovarian cysts in a prepubertal girl with severe hypothyroidism due to autoimmune thyroiditis. Int J Gynecol Cancer 14:543–545
Hansen KA, Tho SP, Hanly M et al (1997) Massive ovarian enlargement in primary hypothyroidism. Fertil Steril 67:169–171
Sharma Y, Bajpai A, Mittal S et al (2006) Ovarian cysts in young girls with hypothyroidism: follow-up and effect of treatment. J Pediatr Endocrinol Metab 19:895–900
Campaner AB, Scapinelli A, Machado RO et al (2006) Primary hypothyroidism presenting as ovarian tumor and precocious puberty in a prepubertal girl. Gynecol Endocrinol 22:395–398
Niedziela M, Korman E (2001) Severe hypothyroidism due to autoimmune atrophic thyroiditis – predicted target height and a plausible mechanism for sexual precocity. J Pediatr Endocrinol Metab 14:901–907
Indumathi CK, Bantwal G, Patil M (2007) Primary hypothyroidism with precocious puberty and bilateral cystic ovaries. Indian J Pediatr 74:781–783
Gordon CM, Austin DJ, Radovick S et al (1997) Primary hypothyroidism presenting as severe vaginal bleeding in a prepubertal girl. J Pediatr Adolesc Gynecol 10:35–38
Chen CH, Tiu CM, Chou YH et al (1999) Congenital hypothyroidism with multiple ovarian cysts. Eur J Pediatr 158:851–852
Panico A, Lupoli GA, Fonderico F et al (2007) Multiple ovarian cysts in a young girl with severe hypothyroidism. Thyroid 17:1289–1293
Singh BM, Ammini AC, Kriplani A (2005) Ovarian cyst in juvenile hypothyroidism. Arch Gynecol Obstet 271:262–263
Anasti JN, Flack MR, Froehlich J et al (1995) A potential novel mechanism for precocious puberty in juvenile hypothyroidism. J Clin Endocrinol Metab 80:276–279
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Browne, L.P., Boswell, H.B., Crotty, E.J. et al. Van Wyk and Grumbach syndrome revisited: imaging and clinical findings in pre- and postpubertal girls. Pediatr Radiol 38, 538–542 (2008). https://doi.org/10.1007/s00247-008-0777-1
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-008-0777-1