Septo-optic dysplasia (SOD) is a developmental abnormality characterised by optic nerve hypoplasia, brain malformations and hypothalamic-pituitary deficiencies. Morphological, ophthalmologic and endocrine findings were studied in 11 children with SOD - 5 males and 6 females. Brain malformations were found in 10 children and included aplasia of the septum pellucidum (4), corpus callosum (3) and fornix (1), pituitary abnormalities (9), cortical malformations (4) and some additional findings in single patients. Developmental abnormalities of the optic nerve were seen in 9 children - unilateral (6) and bilateral (2) optic nerve hypoplasia and dysplastic changes (1). Blindness was present in 2 children bilaterally and in 6 children unilaterally. Nine children suffered from strabism and 4 from nystagmus. Endocrine symptoms were present in 9 patients. Most prevalent was growth hormone deficiency (8), followed by central hypothyroidism (7), hypocorticalism (6), hypogonadism (2) and diabetes insipidus (1). Early diagnosis and interdisciplinary approach are essential in adequate care for children with SOD.

        Key words: septo-optic dysplasia, De Morsier syndrome, optic nerve hypoplasia, growth hormone deficiency