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Systemic causes of CSF rhinorrhea
Published in Jyotirmay S. Hegde, Hemanth Vamanshankar, CSF Rhinorrhea, 2020
Hemanth Vamanshankar, Jyotirmay S Hegde
A rare congenital abnormality first described by Kindler in 1970.1 It is due to the failure of closure of embryonic choroid fissure. Presentation is usually unilateral, with loss/ impaired vision, hypertelorism, strabismus and leukocoria. Fundoscopic examination reveals a funnel-shaped optic nerve head with a central white point of glial tissue surrounded by an elevated annulus of chorioretinal pigment disturbance, thus giving the appearance of a morning glory flower2,3 (Figures 8.1 and 8.2). Other abnormalities associated with morning glory syndrome are cleft palate, hypopituitarism, mental retardation, and agenesis of the corpus callosum. This syndrome is seen in 67.7% of basal encephaloceles (another rare entity seen in 1:35000 live births).4 Spontaneous CSF rhinorrhea is the usual presentation in cases of basal encephaloceles.
Retinal Tears and Detachments
Published in Amy-lee Shirodkar, Gwyn Samuel Williams, Bushra Thajudeen, Practical Emergency Ophthalmology Handbook, 2019
Non rhegmatogenous retinal detachments: This can be due to a range of diseases described below: Inflammatory disease: Such as Vogt–Koyanagi–Harada (VKH) syndrome, posterior scleritis and other chronic inflammatory processes. This traditionally causes multiple serous detachments.Neoplasia: Choroidal melanoma, metastasis, choroidal haemangioma, multiple myeloma, capillary retinal haemangioma can all present with exudative retinal detachments.Congenital abnormalities: Optic pits, morning-glory syndrome and choroidal coloboma may also present with detachment of the neuro-sensory retina.Vascular: Coats’ disease and malignant hypertension both have exudative retinal detachments.Ocular hypotension: Glaucoma drainage surgery may result in choroidal detachments which can mimic retinal detachments. If the choroidal detachments are not touching they can often resolve once the intraocular pressure has been re-established.
FEVR phenotype associated with septo-optic dysplasia
Published in Ophthalmic Genetics, 2019
David L. Zhang, Michael P. Blair, Janice L. Zeid, Syeda S.T. Basith, Michael J. Shapiro
This hypothesis is supported by a variety of optic nerve hypoplasia cases associated with FEVR. Morning glory syndrome (MGS) is another condition where abnormalities in vascular development are associated with optic nerve anomalies. She et al. analyzed 86 eyes of 74 patients with MGS, noting a high prevalence of peripheral retinal nonperfusion in these cases (19). Rojanaporn et al. suggested that maldevelopment of blood vessels at the optic disc during development also occurs in the retinal periphery, resulting in nonperfusion (20). The high prevalence of this comorbidity may point to a new locus where FEVR is not the primary outcome. However, none of the cases described above have been accompanied by genetic testing results, and thus have not directly eliminated a coincidental FEVR mutation with an optic nerve mutation.
OCT Imaging in Infants
Published in Seminars in Ophthalmology, 2022
Sushma Jayanna, Subhadra Jalali, Tapas R Padhi, Komal Agarwal, Jay Chhablani
Morning glory syndrome is a unilateral, congenital anomaly of an optic disc with deep excavation of disc, abnormal radiation vessels across the margins and central tuft of glial tissue. Most common complication being rhegmatogenous retinal detachments because of slit-like breaks along the margin. OCT shows neurosensory detachment involving the posterior pole. Other causes of loss of visual acuity apart from retinal detachment like foveal hypoplasia or aplasia can also be diagnosed with help of OCT which shows reduced or absent foveal contour. Associated CNS abnormalities include Aicardi syndrome, neurofibromatosis type 2 and PHASE syndrome.91
Endoscopic trans-nasal repair of basal encephalocele associated with morning glory syndrome
Published in British Journal of Neurosurgery, 2022
Ali Alshamrani, Fehid Habalrih, Ikhlass Altweijri, Saad Alsaleh, Abdulrazag Ajlan
Morning glory syndrome (MGS) is a rare congenital abnormality which was first described by Reis and by Handman as an enlarged, funnel-shaped optic disc and an elevated pigmented peripapillary tissue caused by the failure of the embryonic choroid fissure to close properly and may affect any part of the eye at the optic nerve. It was named by Kindler due to the retinal fundoscopic appearance similar to the tropical flower of the same name and it is seen in 67.7% of basal encephalocele cases.4,5