PRIMARY PARTIAL EMPTY SELLA PRESENTING WITH PREPUBERTAL HYPOGONADOTROPIC HYPOGONADISM

A CASE REPORT

Abstract

Introduction This case report discusses the approach to diagnosis of a 20-year-old male presenting with micropenis with absent secondary sexual characteristics. Case The patient has eunuchoid habitus, gynecomastia, and genital and pubic hair development of Tanner Stage 1. He has a flaccid and stretched penile lengths of 2.5 and 3 centimeters respectively, palpable small, firm left testis and undescended right testis. Neurologic examination was unremarkable except for anosmia. Cranial MRI with contrast is suggestive of partially empty sella. Chromosome analysis revealed a kayotype with no numerical and structural aberrations and an XY sex chromosome. He has delayed bone age using Greulich-Pyle method and hormonal tests showed low testosterone, LH, FSH, estradiol and beta-HCG. Conclusion The incidence of primary partial empty sella varies depending on means of diagnosis ranging from 5.5-35% in the general population. It may manifest with various endocrine deficiencies. Prepubertal hypogonadotropic hypogonadism is its main manifestation, presenting as micropenis and lack of secondary sexual characteristics. It is rare with peak incidence at late 30 to 40 years of age and predilection for females with female to male ratio of approximately 5:1