Endocrine Abstracts

Introduction: Congenital trans-sphenoidal meningocele (TM) is a rare clinical entity. It can be traumatic, congenital or from tumour origin. It results from a defect in the sphenoid bone ontogenesis. In its classical form, the TM is revealed in adults, frequently in women in the fourth or fifth decade by rhinorrhea or cerebrospinal fluid leak through a bony defect in the sphenoid floor. Our aim is to report a case of large TM without rhinorrhea, and as a part of midline abnormalities.

Case report: An adolescent girl aged 19, was sent to our department for diabetes insipidus. Her medical history showed that she was treated since the age of four for growth retardation related to an isolated GH deficiency, but without any cerebral exploration. At 18 she complained from polyuria and polydipsia. Clinical and paraclinical examinations argued for diabetes insipidus with well proved GH deficiency. Other pituitary functions were preserved. Cerebral MRI described a large dehiscence of the sellar floor with passage of cerebrospinal fluid through the sphenoidal sinus and persistence of meningeal peripheral wall defining a trans-sphenoidal meningocele which appears to be a part of the midline abnormalities as it is associated to cleft lip and hypertelorism.

Unlike other cases reported in the literature, our patient does not have rhinorrhea which represents a high risk for meningitis which is a life threatening condition for patients with this congenital malformation.

Conclusion: Congenital transsphenoidal meningocele is a rare condition, but should be kept in mind as a part of midline abnormalities and as a cause of pituitary insufficiency, cerebrospinal fluid leak and meningitis.