蕭其航¹、陳順郎^1,2、高育琳^1,2、陳文榮¹、謝佐宜¹、王紹全^1,2

¹中山醫學大學附設醫院泌尿科; ²中山醫學大學醫學院醫學系

Chi-Hang Hsiao¹, Sung-Lang Chen^1,2, Yu-Lin Kao^1,2, Wen-JungChen¹, Tzuo-Yi Hsieh¹, Shao-Chuan Wang^1,2

¹Department of Urology, Chung Shan University Hospital, Taichung, Taiwan

²Institute of Medicine, Chung Shan Medical University, Taichung, Taiwan

 

 

Introduction: Hypogonadotropic hypogonadism in adolescent is a rare and complicated condition which may be due to congenital deficiency like Kallmann syndrome or secondary (acquired) causes, including brain or pituitary apoplexy, head trauma, long term use of opioids or steroids, and certain systemic diseases. Herein we present a case of partial empty sella syndrome (ESS) with initial presentation of absent puberty.

Case History: A 15-year-old junior high school student was told to have cryptorchidism during health examination at school. He is a member of table tennis team with a reasonable developmental milestone of 170 cm in height and 68 kg in weight. On physical examination, short penis length with non-palpable testes within scrotum; no pubic hair or armpit hair; no Adam’s apple with sharp voice tone were noted. Hormone profile revealed testosterone under castration level (<0.20 ng/ml) and low level of follicle-stimulating hormone (FSH) (0.107mIU/ml) and luteinizing hormone (LH) (<0.10 mIU/ml), but normal plasma level of iron, thyroid-stimulating hormone (TSH) and prolactin (PRL). Brain magnetic resonance imaging (MRI) demonstrated a flattening of pituitary gland at the bottom of cerebrospinal fluid contained large sized sella turcica, suggestive of partial empty sella syndrome. Scrotal MRI showed bilateral testes atrophy within the scrotum. Owing to no known history of surgery/radiation/infection and no hyposmia/anosmia, hypogonadotropic hypogonadism due to primary partial empty sella syndrome was confirmed.

Conclusion: Empty sella syndrome is an anatomical condition characterized by appearance of the pituitary gland shrinks or gets flattened by cerebrospinal fluid, which usually related to infection, tumor, radiation, or surgery. A high incidence of pituitary dysfunction was documented in patients with empty sella syndrome. These consisted of panhypopituitarism, hyperprolactinemia, isolated adrenocorticotropic hormone (ACTH) deficiency, diabetes insipidus (DI) and even secondary hypogonadism. According to our case and articles review, we put emphasis on the need of thorough hypothalamus-pituitay hormonal evaluation and pituitary MRI survey.