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Van Wyk-Grumbach Syndrome: Atypical Presentation in Childhood Pituitary Macroadenoma with Primary Hypothyroidism

R. Raghavendran, Professor, Department of Neurosurgery, Institute of Neurosurgery, Rajiv Gandhi Government General Hospital & Madras Medical College, Chennai 600001, India. , MD Zaheeruddin Harsoori Mohammed Minhajuddin1 , Sivaraman Vigneshwar2 , Perumal Jayakumar3 , Priya Senthilkumar4 , Radhakrishnan Raghavendran5

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International Journal of Neurology and Neurosurgery 13(2):p 21-24, April-June 2021. | DOI: http://dx.doi.org/10.21088/ijnns.0975.0223.13221.3

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MD Zaheeruddin Harsoori Mohammed Minhajuddin, Sivaraman Vigneshwar, Perumal Jayakumar, et al./Van Wyk-Grumbach
Syndrome: Atypical Presentation in Childhood Pituitary Macroadenoma with Primary Hypothyroidism/Int J Neurol Neurosurg. 2021; 13(2):21–24.
 

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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.

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MD Zaheeruddin Harsoori Mohammed Minhajuddin, Sivaraman Vigneshwar, Perumal Jayakumar, et al./Van Wyk-Grumbach
Syndrome: Atypical Presentation in Childhood Pituitary Macroadenoma with Primary Hypothyroidism/Int J Neurol Neurosurg. 2021; 13(2):21–24.
 

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

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