Cases reported "septo-optic dysplasia"

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11/15. diffusion-tensor imaging in septo-optic dysplasia.

    One of the morphological correlates of septo-optic dysplasia is hypoplasia of the optic nerves. As of now, it remains unknown, in how far this disorder also affects the organization of the optic radiation. Using diffusion-tensor imaging (DTI), the non-invasive evaluation of large fiber tracts including the optic radiation has become possible. We have compared DTI-data from a patient suffering from septo-optic dysplasia with those of a group of eleven healthy control subjects. The anisotropy showed statistically significant reduction in the patient with septo-optic dysplasia within the visual fiber tracts and an unordered eigenvector map. A comparison of the anisotropy in the pyramidal tract showed no significant difference. Since the patient was congenitally blind, it remains unclear whether the findings are the results of the underlying disorder or occur in all congenitally blind patients. One might presume, that, in order for the optic radiation to fully develop, an afferent input to the lateral geniculate body is necessarry. ( info)

12/15. septo-optic dysplasia with digital anomalies--a recurrent pattern syndrome.

    A distinct form of septo-optic dysplasia (SOD) comprises limb malformations in addition to the characteristic CNS and ocular abnormalities. To date, there have been 4 reports, citing 5 affected patients with this combined phenotype. We now add a further case and present neuroradiological images of the CNS findings in this condition. The striking consistency of the limb malformations and their overlap with the Streeter's band phenotype is emphasized. ( info)

13/15. prenatal diagnosis of absence of the septum pellucidum associated with septo-optic dysplasia.

    septo-optic dysplasia (SOD; De Morsier syndrome) is a rare congenital disorder characterized by the absence of the septum pellucidum (SP), hypoplasia of the optic chiasma and nerves, and various types of hypothalamic-pituitary dysfunction. We report on two fetuses with absence of the SP diagnosed by ultrasound examination at 29 and 30 gestational weeks. In the first case the diagnosis of SOD was suspected in utero and confirmed postnatally; to the best of our knowledge this is the first report of the prenatal diagnosis of SOD. In the second case absence of the SP appeared to be isolated and no visual or endocrine impairment were detected after birth. ( info)

14/15. Infantile infection and diabetes insipidus in children with optic nerve hypoplasia.

    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 associated diabetes insipidus are reported. The diagnosis of SOD was suspected only after ophthalmological evaluation; further evaluation led to the diagnosis of panhypopituitarism. CONCLUSIONS: A high index of suspicion is required to diagnose SOD in children when the disorder presents with infantile infection and hypernatraemia. Early warning signs of neonatal jaundice and hypoglycaemia should prompt ophthalmological evaluation. ( info)

15/15. Monocular temporal hemianopia with septo-optic dysplasia.

    A 26-year-old woman displayed a monocular temporal hemianopic defect together with an ipsilateral afferent pupillary defect and bowtie optic nerve hypoplasia. MRI revealed a thin right optic nerve, an asymmetrically thinned chiasm, and an absent septum pellucidum. Monocular temporal visual field loss from organic lesions is quite rare but has been reported in conjunction with compressive lesions at the optic nerve-optic chiasm junction. This is the first report to demonstrate this visual field defect together with bowtie optic nerve hypoplasia. ( info)
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