Morning Glory Syndrome Research Articles

Reversible Carotid Artery Narrowing in Morning Glory Disc Anomaly

A 14-year-old boy with morning glory disc anomaly (MGDA) and normal visual and neurologic function displayed marked carotid artery narrowing on magnetic resonance angiography (MRA). This narrowing disappeared on a follow-up MRA six months later. Optic coherence tomography and scanning laser polarimetry disclosed a normal retinal nerve fiber layer in the eye with MGDA. MGDA has been reported in association with irreversible carotid artery stenosis leading to moya moya disease. This case suggests that mild cases of MGDA may be associated with reversible carotid artery narrowing owing to vasospasm.

Morning Glory Syndrome: Clinical, Computerized Tomographic, and Ultrasonographic Findings

To evaluate the ophthalmic, radiologic, and ultrasonographic findings in morning glory syndrome. Retrospective review of patients' charts. Twenty-one patients were included. Visual acuity was 20/200 or better in 50% of the eyes. Three eyes developed a retinal detachment. When computerized tomography of the orbit demonstrated peripapillary scleral staphyloma and intraocular calcifications, this correlated with poor visual acuity. Computerized tomography of the brain revealed abnormalities in three asymptomatic patients. B-scan ultrasonography demonstrated retinal tissue overhanging the peripapillary scleral staphyloma. Morning glory syndrome has a spectrum of severity, with most patients retaining useful vision. Orbital and cerebral computerized tomography scan as well as B-scan ultrasonography may help with diagnosis and management.

Scientific contributions and citations

Scientific contributions and citations

Hypothalamic Hypopituitarism Presenting with Pituitary Malformation and Morning Glory Syndrome: A Case Report

We report a 3-yr-old male with short stature, speech delay, episodes of convulsion, and right morning glory syndrome. He had four episodes of febrile convulsion from the age of 1 yr 6 mo, and two episodes of afebrile convulsion with hypoglycemia. His height was 90.2 cm (-2.3 SD) and his growth rate was decreased from about 2 yr of age. Magnetic resonance imaging of the brain showed an atrophic anterior pituitary lobe, invisible stalk, and ectopic posterior lobe. Septo-optic dysplasia was deniable. We tested pituitary function. Cortisol response to CRH was normal (peak: 21.0 μg/dl). TSH response to TRH was prolonged and exaggerated (peak: 33.0 μU/ml at 120 min), and FT4 response was absent (peak: 0.74 ng/dl). After supplement of l-T4, GH response to arginine was decreased (peak: 5.1 ng/ml). We diagnosed hypothalamic hypopituitarism. We started treatment with l-T4 and growth hormone. Patients with congenital ocular anomaly including morning glory syndrome should be evaluated for brain and pituitary structure with MRI, and further endocrinological examinations should also be considered.

Congenital disorders of the optic nerve: excavations and hypoplasia

The principal congenital abnormalities of the optic disc that can significantly impair visual function are excavation of the optic disc and optic nerve hypoplasia. The excavated optic disc abnormalities comprise optic disc coloboma, morning glory syndrome, and peripapillary staphyloma. Optic nerve hypoplasia manifests as a small optic nerve, which may or may not be accompanied by a peripapillary ring (the double ring sign). In addition, the optic disc cupping, which occurs as a sequel to some cases of periventricular leucomalacia, can arguably be classified as a type of optic nerve hypoplasia. All of these conditions can be unilateral or bilateral and can impair visual function mildly or severely. It is essential that children with poor vision due to any of these conditions are managed by treating refractive errors, giving occlusion therapy in selected cases, and optimising the conditions at home and at school in an attempt to ensure that impaired vision does not impede development or education.

This Issue of Acta

There are many important scientific original papers in the current issue. In the first one Reinhard and coworkers studied endothelial cell loss after penetrating keratoplasty. Two hundred and thirty-three patients were divided into three classes depending on the degree of HLA matching between donor and recipient. It is known that proper HLA matching decreases the risk of immune reactions after keratoplasty. Despite this, and somewhat surprising, the authors found that better HLA matching was not associated with lower rates of endothelial cell loss than groups with more mismatches. This paper and histocompatibility and grafting in general are discussed in the Guest Editorial by Ehlers. There are two epidemiological papers in this issue of Acta. Bergman et al. performed a highly unusual prospective study of a representative population sample between 70 and 97 years of age in Gothenburg. The subjects were examined at age 70 in 1971, and then again at ages 82, 85, 95 and 97. The study sheds new light on visual function at higher ages. At 82 years 20% of subjects demonstrated decreased vision, defined as visual acuity ≤0.5. At 97 years of age 80% had reduced visual function. Reduced blood folate levels, smoking and high BMI correlated with reduced visual acuities at higher ages. The study clearly shows that ‘normal VA’ is age dependent, but also raises questions about what the requirements should be for defining a normal subject at very high ages. The other epidemiological study published in the current issue also deals with visual acuity. This Inter99 study has been conducted in Copenhagen. Very different age groups have been studied and in this, much younger, population, the most important cause of visual impairment was strabismic amblyopia. Myopia was twice as common in subjects with a university degree as in those without such a degree. Quality of life has very properly come into the focus of ophthalmic researchers. Chabert and coworkers studied the influence of uveal melanoma diagnosis and therapy on QoL. Their results are encouraging; compared to patients with other types of cancer patients with uveal melanoma had relatively satisfactory QoL levels, that were also not seriously reduced by radiotherapy. Fledelius and coworkers present two papers dealing with retinopathy of prematurity. The first one has a provoking subtitle: Has progress reached a plateau? This suggestion by the authors – that ‘the apparent progress in the fight against ROP over many years seems to have come to a halt’– agrees with the impression of many ROP specialists, and is a challenge for specialists in neonatology and ophthalmologists working with premature infants. The accompanying paper presents epidemiological county-based ROP data; one of the differences was a ROP frequency of 31% in 1998–2001 compared to only 10% in the preceding 4-year period. Despite the suggested worsening the authors propose a lowering of gestational age and birthweight limits for ROP screening. Fechtner and coworkers present results from two large 3-month randomised studies with altogether 544 patients comparing Xalatan and Cosopt. There was no difference in IOP-lowering efficacy between the two treatment alternatives. Ocakoglu et al. measured optic nerve head and peripapillary blood flow with the Heidelberg flowmeter in 22 patients with unilateral exfoliation syndrome and 30 age-matched controls. The exfoliation eyes had lowest blood flow, but flow was also somewhat lower in the nonexfoliative fellow eyes than in the normal controls. Exfoliations have been shown to be an independent risk factor for glaucoma damage, and reduced blood flow in exfoliation eyes has, of course, often been considered a possible explanation. In a study of vigabatrin-treated patients using a novel type of visual field testing Frisén reports perimetric deficits that were proportional to the total dose of vigabatrin. There are two papers about pterygium in this issue of Acta. In a retrospective study Frau and coworkers report very positive surgical results in 104 patients operated with corneo-conjunctival autografts. They saw no recurrences at all. In a study from Jordan Al-Bdour and coworkers studied risk factors for this condition. Sun exposure was the most important risk factor, and the authors recommend preventive measures. There are two experimental studies in the current issue. Wasselius et al. studied Cystatin C in the rat and mouse, and found a striking similarity of localization of Cystatin C and cathepsin B, indicating that in the eye the former substance may be a regulator of the latter. Lin and coworkers found a significant association of p21 codon 31 polymorphism and POAG in China. The Diagnosis/Therapy in Ophthalmology section shows an unusual case of an isolated metastasis to the eye from a gastric adenocarcinoma. In the current issue there are also three case reports, all worth reading, on morning glory syndrome (cf. the cover illustration!), on telangiectasis in optic disc maculopathy, and on ERG abnormalities after methotrexate therapy, plus – as always – a number of interesting letters.

Optic nerve atrophy in propionic acidemia

Objective Propionic acidemia is a rare metabolic disorder that is diagnosed in the early neonatal period. The disorder is characterized by life-threatening ketoacidosis, lethargy, failure to thrive, and developmental delay. Herein we report the ocular findings in a prospective series of six patients with propionic acidemia. Design Prospective case series. Participants Six children (three male and three female) between the ages of 2 and 10 years with propionic acidemia who were examined at Children's Hospital Los Angeles. Methods A complete ophthalmic examination was performed on each of the six children. The examination included visual acuity testing, ocular motility, anterior segment examination, and funduscopic evaluation. Emphasis was placed on the function of the optic nerve and on the appearance of the optic disc, looking for possible atrophic changes. Main outcome measures The clinical appearance of the optic disc and evidence of optic neuropathy. Results Optic nerve atrophy was present exclusively in all of the male patients in the series; none of the female patients demonstrated any detectable impairment of optic nerve function. The optic nerve atrophy was symmetric and age dependent and varied from moderate to severe. There were no other anterior or posterior segment abnormalities, other than one case of unilateral morning glory syndrome, diagnosed at birth. There was no correlation between metabolic control and the development and progression of optic nerve atrophy. Conclusions Males with propionic acidemia have moderate to severe bilateral optic atrophy.

Optic disc coloboma (the morning glory syndrome) and optic nerve coloboma associated with transsphenoidal meningoencephalocele

The association optic disc coloboma (the morning glory syndrome), optic nerve coloboma, and transsphenoidal meningoencephalocele is extremely rare. We describe an original case of optic disc coloboma in association with optic nerve coloboma, microphthalmos, optic nerve hypoplasia, and transsphenoidal meningoencephalocele. There were no midfacial anomalies (hypertelorism, broad nasal root, cleft lip, and cleft plate) and pituitary dysfunction. For patients who had optic disc-nerve coloboma without midfacial anomalies, neuroimaging should be prompted to look for transsphenoidal encephalocele/meningoencephalocele and other anomalies. We present demonstrative fundoscopic pictures, ultrasound and magnetic resonance imaging of this original case of a 7-year-old patient.

Morning glory disk anomaly, choroidal coloboma, and congenital constrictive malformations of the internal carotid arteries (moyamoya disease)

Purpose: To report a patient with congenital constrictive malformations of the internal carotid arteries and a morning glory disc anomaly (MGDA). Methods: Descriptive case report. Results: A 14-year-old girl underwent serial ophthalmologic examinations since the age of seven years because of blurred vision and floaters. A right optic disk anomaly was present that was most compatible with an MGDA accompanied by an inferior choroidal coloboma. Because of the association of such disc malformations with moyamoya disease, the patient underwent cerebral MRI and MRA that showed narrowing of both internal carotid arteries, a finding consistent with early-stage moyamoya disease. Conclusion: This case illustrates the importance of recognizing the association of MGDA with moyamoya disease, a treatable condition that may have devastating neurological consequences.

A case of bilateral morning glory disc anomaly and associated transsphenoidal encephalocele

Morning glory disc anomaly (MGDA) is a congenital ocular defect characterized by a funnel shaped excavation of the posterior fundus incorporating the optic nerve head. Not only has the condition been associated with both rhegmatogenous and non-rhegmatogenous retinal detachments, it has been associated with systemic abnormalities such as midline and paramidline facial anomalies, transsphenoidal encephalocele, and endocrine abnormalities. Coordinated care of these patients may be useful in ruling out associated systemic anomalies, especially in children where intervention can be beneficial in correcting growth pattern abnormalities related to endocrine dysfunction.

Morning glory syndrome: Mr imaging

We present two cases of the Morning Glory Syndrome (MGS), with the most detailed MR images to date of this rare congenital optic nerve dysplasia. Though the embryology of this syndrome remains controversial, we feel the MR appearance can be diagnostic of the non-familial syndrome and be reliably distinguished from the similar appearing optic disc coloboma, which may be genetically inherited. MR imaging also allows the most sensitive detection and characterization of any associated intracranial anomalies, thus enabling more accurate determination of prognosis for the patient and their family.

Neuro-ophthalmic Manifestations of Lyme Disease

Lyme disease is a multisystem disorder caused by infection with the Borrelia burgdorferi spirochete. The diagnosis of Lyme disease usually is based on several clinical criteria, with supportive data from laboratory testing. The presence of the bullseye skin lesion, erythema migrans, is the single pathognomonic criterion. In the 20 years since the initial description of Lyme disease in the United States, B. burgdorferi has been implicated as an etiologic agent in numerous ophthalmic and neuro-ophthalmic syndromes, involving most structures from the cornea to the cranial nerves. Neuro-ophthalmic and ocular manifestations of Lyme disease include meningitis with papilledema, cranial neuropathies, follicular conjunctivitis, nummular keratitis, and intraocular inflammation. Although an association with Lyme disease has been purported for numerous other syndromes, a definite causal relationship has not been proved in many cases. During a period of rapidly increasing awareness of Lyme disease, a high index of suspicion and poorly defined criteria for its presence have resulted in over-diagnosis of Lyme disease. In the authors' experience, the incorrect diagnosis of Lyme disease initially has been made in patients with allergic conjunctivitis, keratoconus, morning glory syndrome, craniopharyngioma, meningioma, CNS lymphoma, paraneoplastic syndrome, multiple sclerosis, sarcoid, syphilis, and functional illness. Nevertheless, this treatable infection must be an important consideration in the differential diagnosis of certain ocular or neurologic diseases.

Ultrasonography of the Optic Disc

Echographic examinations of 13 eyes with pathologic changes of the optic nerve head were performed in the Dokuz Eylül University Deparmet of Ophtalmology. Elevated lesions of the optic disc such as papilloedema, drusen, tilted disc, remnant of hyloid artery and melanocytoma were detected by special characteristics. Excavated lesions such as optic nerve coloboma, morning glory syndrome, glaucomatous cupping and staphyloma were also demonstrated by their distinct properties. Ultrasonography of the optic nerve head using both A and B mode is concluded to be a useful tool in dtecting and varifying various acquired and congenital pathologic changes of the optic disc.

A rare vascular disc abnormality associated with morning glory syndrome

Abstract Morning glory syndrome is a non-hereditary, usually unilateral malformation of the optic disc associated with various retinal abnormalities. We present a case of morning glory syndrome associated with a rare feature of different arteriolar circulation pattern of upper and lower halves of the optic disc. We believe that meticulous clinical and fluorescein angiographic evaluation is a must as a vast range of ocular vascular abnormalities and related complications may occur in cases with morning glory syndrome.

Communication between the subretinal space and the vitreous cavity in the morning glory syndrome

The aim was to describe a pathogenic mechanism for a rhegmatogenous retinal detachment in a 69-year-old man with the morning glory syndrome. During vitreous surgery for a retinal detachment, a membrane was removed that covered the optic disc anomaly and produced traction on the peripapillary retina. A retinal hole was found in tissue lying within the optic cup, and the hole was sealed using a autologous plasma-thrombin mixture. Silicone oil was used for retinal tamponade. A retinal hole in tissue lying within the optic cup provided a fluid pathway between the vitreous cavity and the subretinal space. Following vitrectomy surgery, bubbles of silicone oil passed through the retinal hole into the subretinal space of the macula. This case demonstrates that a retinal hole in tissue lying within the optic disc anomaly of the morning glory syndrome provides a communication for fluid between the subretinal space and the vitreous cavity, resulting in a rhegmatogenous retinal detachment. Vitreous replacement with silicone oil resulted in the migration of silicone bubbles into the subretinal space.

Basal encephaloceles with morning glory syndrome, and progressive hormonal and visual disturbances: case report and review of the literature

We report an 11-year-old girl with progressive hypopituitarism and visual loss of the right eye caused by trans-sphenoidal and sphenoethmoidal encephaloceles associated with morning glory syndrome. She was first seen at the age of 8 years, because of polydipsia and polyuria, and examination at that time revealed pituitary dwarfism and morning glory syndrome with visual disturbance of the right eye. Hormonal examinations revealed deficiency of growth hormone (GH) and anti-diuretic hormone (ADH). MR image showed trans-sphenoidal and sphenoethmoidal encephaloceles. At the age of 9 years, she was found at another institution to be blind in the right eye. Our examination of the patient at the age of 11 years revealed no change of the findings for the encephaloceles or optic system. Hormonal examination disclosed deficiencies of all hormones except for thyrotropin (TSH). This patient showed progressive hormonal and optic disturbances during the follow-up period. The natural course is still unclear, but our review of reported cases of trans-sphenoidal encephalocele with hormonal disturbance revealed that the most frequent findings were GH and ADH disturbance (over 60%), most patients (77.8%) showed progression of hormonal disturbance, and 40% of those with optic dysfunction showed progression. A patient with basal encephalocele with hormonal and/or optic disturbances requires careful long-term follow up.

Repair of Retinal Detachment Associated with Congenital Excavated Defects of the Optic Disc

Excavated defects of the optic disc have been associated with retinal detachment in the macula and beyond. Included among these defects are congenital pits of the optic disc, optic nerve colobomas, and the morning glory syndrome. We report eight consecutive patients with congenital, excavated defects of the optic disc and associated retinal detachment managed with pars plana vitrectomy, laser photocoagulation, and intravitreal gas injection.

Optic nerve coloboma (morning glory syndrome): CT findings.

This study demonstrates computed tomographic (CT) findings of morning glory syndrome. CT examination of the orbits was performed in three patients. Images of 2-mm-thick sections were acquired at 2-mm intervals without use of contrast material. Excellent demonstration of coloboma was achieved in each case. Because both magnetic resonance imaging and ultrasonography have limitations in imaging of coloboma, CT is the imaging method of choice in diagnosis of this disorder.

Cilioretinal Arteries in Conjunction with a Pit of the Optic Disc

Cilioretinal arteries were noted to accompany pits of the optic disc in 16 of 25 cases (64%). Fourteen of the 16 cases were found to have 2 cilioretinal arteries (86%). The cilioretinal arteries were directly related to the pit of the disc and emerged either from the bottom or the margin of the pit in a proportion of 86%. This finding reinforces the assumption that the optic disc pit belongs to the same spectrum of congenital anomalies which also includes optic disc coloboma, megalopapilla and morning glory syndrome. A fluorescein angiography study showed hyperfluorescence of the pit in 12/16 cases and in particular in all the cases in which both cilioretinal arteries emerged from the pit. Hypofluorescence was noted in 7/9 cases in which the pit was not associated with cilioretinal arteries. Our findings could support the view that hyperfluorescence of the pit, which is not a constant phenomenon, mostly depends on the presence of cilioretinal arteries emerging from the pit.

Papillenanomalien bei (peri)orbitalen Enzephalozelen - Ein Beitrag zur gemeinsamen Pathogenese dieser Fehlbildungen

Eleven patients (6 female, 5 male) with unilateral (6) or bilateral (5) (peri)orbital encephaloceles (nasofrontal in 4 cases, naso-orbital in 3, spheno-orbital in 4), were ophthalmologically examined. All had normal anterior segments, but ipsilateral or bilateral anomalies of the optic nerve head such as coloboma, pits, morning glory syndrome, dysplasia, tilted disk syndrome, hypoplasia, or a persistent hyaloid artery. In regard to the pathogenesis of (peri)orbital encephaloceles associated with optic disk malformations a common clefting defect in the neuroectodermal and neurocristal midline structures of the head is postulated, taking place in the fourth to sixth week of development.