Basal encephaloceles with morning glory syndrome, and progressive hormonal and visual disturbances: case report and review of the literature

We report an 11-year-old girl with progressive hypopituitarism and visual loss of the right eye caused by trans-sphenoidal and sphenoethmoidal encephaloceles associated with morning glory syndrome. She was first seen at the age of 8 years, because of polydipsia and polyuria, and examination at that time revealed pituitary dwarfism and morning glory syndrome with visual disturbance of the right eye. Hormonal examinations revealed deficiency of growth hormone (GH) and anti-diuretic hormone (ADH). MR image showed trans-sphenoidal and sphenoethmoidal encephaloceles. At the age of 9 years, she was found at another institution to be blind in the right eye. Our examination of the patient at the age of 11 years revealed no change of the findings for the encephaloceles or optic system. Hormonal examination disclosed deficiencies of all hormones except for thyrotropin (TSH). This patient showed progressive hormonal and optic disturbances during the follow-up period. The natural course is still unclear, but our review of reported cases of trans-sphenoidal encephalocele with hormonal disturbance revealed that the most frequent findings were GH and ADH disturbance (over 60%), most patients (77.8%) showed progression of hormonal disturbance, and 40% of those with optic dysfunction showed progression. A patient with basal encephalocele with hormonal and/or optic disturbances requires careful long-term follow up.