Van Wyk Grumbach syndrome - a rare association of a common prepubertal condition

Mukta Waghmare; Hemanshi Shah; Vikrant Kumbhar; Shalika Jayaswal

doi:10.18203/2320-6012.ijrms20162339

Authors

Mukta Waghmare Department of Paediatric Surgery, T.N.M.C and B.Y.L. Nair Hospital, Mumbai, Maharashtra
Hemanshi Shah Department of Paediatric Surgery, T.N.M.C and B.Y.L. Nair Hospital, Mumbai, Maharashtra
Vikrant Kumbhar Department of Paediatric Surgery, T.N.M.C and B.Y.L. Nair Hospital, Mumbai, Maharashtra
Shalika Jayaswal Department of Paediatric Surgery, T.N.M.C and B.Y.L. Nair Hospital, Mumbai, Maharashtra

DOI:

https://doi.org/10.18203/2320-6012.ijrms20162339

Keywords:

Van Wyk Grumbach, Hypothyroidism, Pre-pubertal girls, Ovarian cysts

Abstract

Van Wyk Grumbach syndrome is a sequel of long standing untreated primary hypothyroidism. It occurs in both sexes. In boys, it is associated with testicular enlargement. Girls present with features of early onset menarche, enlarged bilateral multicystic ovaries and delayed skeletal growth. Prompt replacement therapy with Thyroxine corrects the menstrual abnormality and the ovaries also return to normal over a period of time. We describe an unusual case of irregular vaginal bleeding, giant bilateral ovarian cysts and delayed bone age in a young girl due to severe hypothyroidism who promptly responded to levo-thyroxine.

Metrics

Metrics Loading ...

References

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

Isher HK. Large multicystic ovaries in a young girl with severe hypothyroidism: an unusual association. J Obstet Gynaecol Can. 2012;34(3):110-1.

Ashraf F, Nandi ER, Akhter P, Yasmin N. Van Wyk Grumbach Syndrome: a rare case report. J Curr Adv Med Res. 2015;2(1):24-7.

Rastogi A, Bhadada SK, Bhansali A. An unusual presentation of a usual disorder: van wyk-grumbach syndrome. Indian J Endocr Metab. 2011;15:S141-3.

Amin SV, Bharatnur S, Vasudeva A, Hebbar SS. Thyroxine replacement promptly corrects vaginal bleeding in precocious menarche due to Van Wyk Grumbach syndrome. Int J Contemp Pediatr. 2015;2:151-4.

Duarte DR, Chang CV, Minicucci MF, Zornoff LA, Inoue RM, Matsui M, Okoshi MP, Azevedo PS, Matsubara LS, Paiva SA. Myxedema ascites with elevated serum CA 125 concentration. Am J Med Sci. 2006;331:103-4.

Motamed F, Eftekhari K, Kiani MA, Rabbani A. Ovarian cyst enlargement in a 14 year old female with persistent ascities. Severe hypothyroidism and elevated serum CA-125 level Iran Red Crescent Med J. 2012;14(6):379-81.

Fatema A, Eva R, Akhter P. Van wyk grumbach syndrome-a rare case report. J Curr Adv Med Res. 2015;2(1):2427.

Bhattacharya M, Mitra AK. Regressive of precocious puberty in child with hypothyroidism after thyroxine therapy. Indian Pediatr. 1992;29:96-8.