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DOI: http://dx.doi.org/10.21088/ijnns.0975.0223.13221.3

Abstract

Van WykGrumbach syndrome is a constellation of hormonal abnormalities which includes hypothyroidism, precocious puberty and ovarian cysts in pre and pubertal girls. The usual presentation is ascites, pericardial and pleural effusion, enlarged pituitary, elevated prolactin, ovarian hormones. Here, we present a 6 year old female, who presented with precocious puberty associated with hypothyroidism and had features suggestive of Van Wyk– Grumbach syndrome. This is presented to highlight the rare presentations of Pediatric Sellar Pituitary enlargement with bleeding PV, walking difficulty, thelarche her hormonal profile showed elevated TSH, Prolactin, Inhibin B and LDH, USG abdomen showed multiple ovarian cysts with adrenal mass, MRI brain showed enlarged pituitary gland and bone scans revealed a delayed bone age. This rare entity should be kept in mind in cases of ovarian cysts, especially those with isosexual precocity, to prevent delay in medical management and unnecessary surgical misadventures. Keywords: Hypothyroidism; Pituitary; Vanwyk Grumbach.

Corresponding Author : R. Raghavendran, Professor, Department of Neurosurgery, Institute of Neurosurgery, Rajiv Gandhi Government General Hospital & Madras Medical College, Chennai 600001, India.