Morning glory syndrome associated with posterior pituitary ectopia and hypopituitarism

Abstract

Purpose: To report a patient with morning glory syndrome in combination with posterior pituitary ectopia and to emphasize the need for early recognition of this syndrome as an important step towards the diagnosis and treatment of the systemic anomalies that may be associated with it. Methods: We present a 7-year-old boy who showed short stature, nystagmus, inward deviation and low vision. Ophthalmological and general physical examinations, further endocrine evaluation and magnetic resonance imaging (MRI) of the brain and sella turcica were performed. Results: Both fundi showed symptoms of morning glory syndrome. The discs were pink and deeply excavated, and were surrounded by a ring of chorioretinal pigmentary disturbance. Magnetic resonance imaging revealed the absence of the infundibulum and posterior pituitary ectopia. Growth hormone studies confirmed the diagnosis of growth hormone deficiency. Therapy with recombinant human growth hormone was initiated. Conclusions: Although most cases of morning glory syndrome occur as isolated ocular abnormalities, it may occur in association with systemic anomalies, including posterior pituitary ectopia and hypopituitarism. A complete general physical examination and growth evaluation is important for early detection and treatment, resulting in benefit for these patients.