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Sphenopharyngeal Encephalocele Presenting With Partial Hypopituitarism and Diabetes Insipidus

Encephalocele is a cystic protrusion of intracranial structures through a skull base defect. There are several classifications of encephaloceles according to their contents and location. Encephaloceles can be classified as anterior (frontal, sincipital, and basal), posterior, parietal, and nonmidline encephalocele. Basal, the most infrequent (1.5%), are divided into 5 types. The transsphenoidal type may be intrasphenoidal or transsphenoidal, protruding through the floor of the sphenoid sinus into the nasal cavity or nasopharynx. Antenatal or postnatal clinical presentation and diagnosis depend on the size and contents of the defect, associated cranial or vascular anomalies, related symptoms, and endocrinologic disturbances. We report the unique case of a 31-year-old woman with sphenopharyngeal encephalocele, associated with partial hypopituitarism, diabetes insipidus, and hypoplastic right internal carotid artery. There were no craniofacial, optic, or somatic anomalies, and no mental deficit. We review the optimal diagnostic procedures, treatment, prognosis, and follow-up of this very rare entity.

sphenopharyngeal encephalocele ; partial hypopituitarism ; diabetes insipidus

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