Endocrine Abstracts

Introduction

The empty sella syndrome is a rare disorder characterized by a flattened pituitary gland leaving place to the cerebrospinal fluid to fill in the sella turcica.The diagnosis is radiological and it is often discovered during pituitary disorders. Generally patients suffer from hypopituitarism, and primary hypothyroidism is found to be a rare association.

Observation

Herein the case of a young woman who consulted at the age of 33 year-old for recent onset of diabetes mellitus. The patient had an important retardation regarding the pondero-statural development ( Weight: 18.5 Kg, Height: 102 cm ) with impuberism ( S0, P0 Tanner stage ). The evaluation of the hypophyseal function showed firstly; a primary hypothyroidism (TSH >100 µU/mL, TPO antibodies negative ) with a thyroid atrophia on the cervical ultrasound, secondly an hypogonadotropic hypogonadism ( FSH 28.4 mUI/mL, LH 2.11 mUI/mL, Estradiol 13.4 pg/mL, Prolactin 125 mUI/L ), thirdly a corticotropic deficiency confirmed by an insulin induced hypoglycemia test and a low level of ACTH ( 7.72ng/L), and lastly, a complete GH deficiency. In front of this hypopituitarism, an hypothalamic- hypohyseal MRI revealed an empty sella turcica with a normal pituitary stalk. The procedure to follow was to treat the patient with hormonal replacement therapy, and her state significantly improved with a gain of 11 cm in a period of five months (Height: 113 cm ).

Conclusion:

This clinical presentation of the empty sella syndrome is atypical and only few similar cases were found in the literature.Hence the importance of diagnosing it, in order to improve the knowledge of this syndrome and its different aspects.