Van
Wyk-Grumbach syndrome (VWGS) is characterized by juvenile hypothyroidism, delayed
bone age, isosexual precocious puberty and massive ovarian cysts. We report
such a case in a 7-year-old girl who presented with a bilateral pelvic pain,
abdominal distension and signs of hypothyroidism. Physical examination showed a
breast development and lack of pubic and axillary hair. There was no
clitoromegaly. Pelvic ultrasonography and CT scan showed enlarged bilateral multiloculared
multicystics ovarian masses. The pituitary MRI was recommended before an
elevated prolactin, revealed a large sellar and suprasellar mass. The patient
was started on thyroid replacement using L-Thyroxine. Clinically, the patient’s
fatigue, pelvic pain, and abdominal distension are resolved. During the follow-up,
her thyroid functions and serum prolactin normalized within 8 months.
Ultrasonography revealed regression of ovarian cysts in 2 months measuring,
with normal sized ovaries in 14 months.
Van Wyk, J.J. and Grumbach, M.M. (1960) Syndrome of Precocious Menstruation and Galactorrhea in Juvenile Hypothyroidism. An Example of Hormonal Overlap in Pituitary Feedback. The Journal of Pediatrics, 57, 416-435. http://dx.doi.org/10.1016/S0022-3476(60)80250-8
Durbin, K.L. and Diaz-Montes, T. (2011) Van Wyk and Grumbach Syndrome: An Unusual Case and Review of the Literature. Journal of Pediatric and Adolescent Gynecology, 24, e93-e96. http://dx.doi.org/10.1016/j.jpag.2010.08.003
Hemady, Z.S., Siler-Khodr, T.M. and Najjar, S. (1978) Precocious Puberty in Juvenile Hypothyroidism. The Journal of Pediatrics, 92, 55-59. http://dx.doi.org/10.1016/S0022-3476(78)80070-5
Gordon, C.M., Austin, D.J., Radovick, S. and Lufer, M.R. (1997) Primary Hypothyroidism Presenting as Severe Vaginal Bleeding in a Prepubertal Girl. Journal of Pediatric and Adolescent Gynecology, 10, 35-38. http://dx.doi.org/10.1016/S1083-3188(97)70042-1
Chattopadhyay, A. and Kumar, V. (2003) Polycystic Ovaries, Precocious Puberty and Acquired Hypothyroidism: The Van Wyk and Grumbach Syndrome. Journal of Pediatric Surgery, 38, 1390-1392. http://dx.doi.org/10.1016/S0022-3468(03)00403-2
Passeri, E., Tufano, A. and Locatelli, M. (2011) Large Pituitary Hyperplasia in Severe Primary Hypothyroidism. The Journal of Clinical Endocrinology & Metabolism, 96, 22-23. http://dx.doi.org/10.1210/jc.2010-2011
Hulse, A. (2014) Van Wyk-Grumbach Syndrome and Pituitary Hyperplasia in a Six-Year-Old Girl. Apollo Medicine, 11, 40-42. http://dx.doi.org/10.1016/j.apme.2013.10.009
