P133

S. Tahi*a (Dr), A. Heddama (Pr), D. Meskinea (Pr)

a EPH BOLOGHINE, Alger, ALGÉRIE

* s.tahi@outlook.com

Introduction

(KS) defined by the association of isolated hypogonadotropic hypogonadism(IHH)and hyposmia or anosmia.

Primary empty sella results from congenital incompetence of the sellar diaphragm and is not a classic finding regarding IHH.To our knowledge,a similar association has been reported only in 4 occasions

Case report

A 28-year old male presenting pubertal development fault and anosmia,the third in family of five children from a consanguineous marriage,had a strabismus history of the left eye operated at the age of 11,no hearing complaints and no familial history of hypogonadism or olfactory impairment.

he had BMI:30,Pubic hair was at stage 2 (P2).absence of axillary chest and facial hair Bilateral Gynecomastia S3,Bilateral cryptorchidism and micro penis.

Smell Identification Test:anosmia and the laboratory tests confirmed hypogona-dotropic hypogonadism .

hypothalamic-pituitary MRI;empty sella with hernia intra cerebro spinal fluid.olfactory bulbs MRI however,returned without abnormalities.

the pelvic region MRI:2 testicles with reduced size situated in the middle third of inguinal canal and a left varicocele grade III,small prostate visualized.

Review ophthalmologist:excavation and c/d was symmetrical in the 2 eyes which have a congenital origin

KS was the most likely diagnosis,the patient was put under testétorone injection and sent to urology surgeon,the subsequent evolution marked by significant clinical response after 6 months of androgen therapy.

Conclusion

A possible constitutional anomaly of the sellar diaphragm may be involved in the occurrence of the empty sella turcica observed in these patients.Even considering that primary empty sella usually occurs with normal pituitary function,its detection is important to allow an adequate evaluation and follow-up.

L’auteur n’a pas transmis de déclaration de conflit d’intérêt.