INTRODUCTION Septo-optic dysplasia (De Morsier syndrome) is defined as the association between optic nerve hypoplasia, midline central nervous system malformations and pituitary dysfunction. CASE REPORT Third child born to nonconsanguineous parents, female, adequate pre-natal medical care, cesarean term delivery due to breech presentation, Apgar score 3 at the first minute and 8 at 5 minutes,...

BACKGROUND Fibrous dysplasia of bone frequently involves the anterior base of the cranium and results in encasement of the optic-nerve canals. It has been assumed that such encasement leads to constriction and eventual blindness. There is controversy about whether patients should be regularly monitored or whether they should undergo prophylactic decompression of the optic nerve. This question i...

Optic nerve hypoplasia with hypopituitarism and intact septum pellucidum is a variant of septo-optic dysplasia or deMorsier's syndrome.' Although neonatal jaundice has been seen with this syndrome, the association with severe prolonged cholestatic jaundice has not been emphasised. We describe three patients who presented with cholestatic jaundice and were found to have optic nerve hypoplasia an...

Septo-optic dysplasia is an uncommon diagnosis comprised in part of a unique composition of congenitalmalformations of the central nervous system. It is defined by three principle findings: optic nerve hypoplasia, absence of the septum pellucidum, and clinical pituitary dysfunction. The syndrome may be associated with various cerebral defects including ectopic posterior pituitary, dysgenesis of...

Optic nerve hypoplasia with hypopituitarism and intact septum pellucidum is a variant of septo-optic dysplasia or deMorsier's syndrome.' Although neonatal jaundice has been seen with this syndrome, the association with severe prolonged cholestatic jaundice has not been emphasised. We describe three patients who presented with cholestatic jaundice and were found to have optic nerve hypoplasia an...

Case presentation: Female, 3 months old, born preterm, weighing 2285 g, by vaginal delivery. With 25 days of life, she was hospitalized due to jaundice, with total bilirubin at admission 23 mg/dL, dehydration and low weight gain. The infant remained on phototherapy for one day, partial improvement remaining, however, dehydrated, presenting hypernatremia, serum sodium levels reaching 170 mmol/L....