Primary Partial Empty Sella presenting with Prepubertal Hypogonadotropic Hypogonadism
A Case Report
DOI:
https://doi.org/10.15605/jafes.035.02.11Keywords:
PES, hypogonadotropic, hypogonadism, micropenisAbstract
Primary partial empty sella occurs when less than 50% of an enlarged or deformed sella turcica is filled with cerebrospinal fluid in the setting of unidentified etiologic pathological conditions. Prepubertal hypogonadotropic hypogonadism presenting as its main manifestation is rare since its peak incidence commonly occurs late at 30 to 40 years of age and has a sexual predilection for female. We described a case of 20-year-old male who presented with micropenis and absent secondary sex characteristics. Work up showed cranial MRI finding of partial empty sella, low testosterone, LH, FSH, Estradiol and Beta HCG levels. Sex hormone replacement may not improve fertility for this case but may help produce and maintain virilization and prevent future complications of hypogonadotropic hypogonadism.
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References
Agarwal JK, Sahay RK, Bhadada SK, Reddy VS, Agarwal NK. Empty sella syndrome. J Indian Acad Clin Med. 2001;2(3):198-202.
Chiloiro S, Giampietro A, Bianchi A, et al. Diagnosis of endocrine disease: Primary empty sella: A comprehensive review. Eur J Endocrinol. 2017;177(6):R275-85. https://www.ncbi.nlm.nih.gov/pubmed/28780516. https://doi.org/10.1530/EJE-17-0505.
Hitapoğlu N, Kurtoğlu S. Micropenis: Etiology, diagnosis and treatment approaches. J Clin Res Pediatr Endocrinol. 2013;5(4):217-23. https://www.ncbi.nlm.nih.gov/pubmed/2437909. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3890219.
https://doi.org/10.4274/Jcrpe.1135.
De Marinis L, Bonadonna S, Bianchi A, Maira G, Giustina A. Primaryempty sella. J Clin Endocrinol Metab. 2005;90(9):5471–7. https://www.ncbi.nlm.nih.gov/pubmed/15972577.
https://doi.org/10.1210/jc.2005-0288.
Maira G, Anile C, Mangiola A. Primary empty sella syndrome in a series of 142 patients. J Neurosurg. 2005;103(5):831–6. https://www.ncbi.nlm.nih.gov/pubmed/16304986. https://doi.org/10.3171/jns.2005.103.5.0831.
Rani PR, Maheshwari R, Reddy TSK, Prasad NR, Reddy A. Study of prevalence of endocrine abnormalities in primary empty sella. Indian J Endocrinol Metabol. 2013;17(Suppl 1):S125-6. https://www.ncbi.nlm.nih.gov/pubmed/24251130. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3830276. https://doi.org/10.4103/2230-8210.119527.
Ghatnatti V, Sarma D, Saikia U. Empty sella syndrome - beyond being an incidental finding. Indian J Endocrinol Metab. 2012;16(Suppl 2):S321-3. https://www.ncbi.nlm.nih.gov/pubmed/23565413. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3603061. https://doi.org/10.4103/2230-8210.104075.
Stelmachowska-Banaś M, Czajka-Oraniec I, Zgliczyński, W. Clinical and hormonal assessment of patients with empty sella on MRI. Postępy Nauk Medycznych. 2014;23(12):814-8. http://www.pnmedycznych.pl/wpcontent/uploads/2015/01/pnm_2014_814-818.pdf
Thomas Jr HM, Lufkin EG, Ellis 3rd GJ, Hartman CR, Hofeldt FD, Herman RH. Hypogonadotropism and "empty sella": Improvement in 2 cases with clomiphene citrate. Fertil Steril. 1973;24(4):252-9. https://www.ncbi.nlm.nih.gov/pubmed/4694501.
https://doi.org/10.1016/s0015-0282(16)39609-1.
Petak SM, Nankin HR, Spark RF, et al. American Association of Clinical Endocrinologists Medical Guidelines for clinical practice for the evaluation and treatment of hypogonadism in adult male patients--2002 update. Endocr Pract. 2002;8(6):440-56. https://www.ncbi.nlm.nih.gov/pubmed/15260010.
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