Morning glory syndrome and basal encephalocele.

摘要

BACKGROUND: Morning glory syndrome (MGS) is a congenital optic disc dysplasia often associated with craniofacial anomalies, especially basal encephalocele. Clinical presentations are varied and often occult. CASE REPORT: We describe a case of bilateral MGS associated with basal encephalocele that was detected by chance when treatment was sought for respiratory distress. CONCLUSIONS: MGS and basal encephalocele should always be suspected in cases of midline deficiencies, particularly when ophthalmic signs of strabismus or poor vision are present. CT and MRI should be performed to delineate the extent of the lesion and a complete hormone screening should be carried out to exclude pituitary deficiency. The pathogenesis of MGS and basal encephalocele are unknown; however, it is thought to occur during the 5th week of embryonic development.