ABSTRACT
The most likely cited mechanism for congenital third nerve palsy is perinatal injury. However associated brain stem signs and neurologic anomalies can occure. We present three cases of congenital third nerve palsy in septo-optic dysplasia (de Morsier syndrome). All children had bilateral optic nerve hypoplasia, mid brain malformations and hypothalmohypophseal dysfunction. One child died during febril illness despite intensive medical care. Conclusion: In children with congenital third nerve palsy we have to pay attention to optic nerve hypoplasia for early diagnosis (brain ultasound, MRI, endocrinologic workup) and treatment of de Morsier syndrome.
