BACKGROUND During the past decades, surgical intervention has been the primary treatment modality for frontoorbital fibrous dysplasia involving optic nerve. However, controversy has surrounded the role of optic nerve decompression in a number of ways. Herein, we describe 3 patients with frontoorbital fibrous dysplasia involving optic nerve, who underwent a "well digging" subcraniotomy strategy ...

The clinical and neuroradiologic features in 22 patients with septooptic dysplasia are reviewed. The most consistent abnormality seen in 15 patients was absence of the septum pellucidum and flattening of the roofs and inferior pointing of the floors of the anterior horns of the lateral ventricles. Hypoplasia of the anterior optic pathways was seen in 10 patients and a primitive optic ventricle ...

The SOD phenotype is highly heterogeneous and diagnosis is made in the presence of at least two of these three features. [5] Only about a third of patients have all three features but patients with any component of the syndrome should be screened for the other features too. The cause of SOD is unknown but viral infections, gestational diabetes, environmental teratogens, vascular or degenerative...

Septo-optic dysplasia (SOD), also referred to as de Morsier syndrome, is a rare congenital condition, characterized by two of the classic triad features: midline brain abnormalities, optic nerve hypoplasia (ONH) and pituitary endocrine dysfunction. We report 5 children with SOD, originally referred to be evaluated due to short stature, who also presented bilateral optic nerve hypoplasia, nystag...

The SOD phenotype is highly heterogeneous and diagnosis is made in the presence of at least two of these three features. [5] Only about a third of patients have all three features but patients with any component of the syndrome should be screened for the other features too. The cause of SOD is unknown but viral infections, gestational diabetes, environmental teratogens, vascular or degenerative...

Background: During the past decades, surgical intervention is the primary treatment modality for fronto-orbital fibrous dysplasia involving optic nerve. However, controversy has surrounded the role of intra-canal decompression in a number of ways. Objective: Our philosophy of saving optic nerve is that treatment paradigms should be tailored to the individual. Herein, we describe three patients ...

BACKGROUND Bilateral optic nerve hypoplasia (BONH) is often associated with other central nervous system midline abnormalities (septo-optic dysplasia). Hormonal dysfunction, caused by anterior (cortisol) and posterior (ADH) pituitary involvement, can be sudden, severe, and life threatening. METHODS Case series. Three cases of septo-optic dysplasia (SOD) presenting as infantile infection with ...