Síndrome de Morning Glory / Morning Glory Syndrome

Introducción: la anomalía congénita del disco óptico conocida como "morning glory" (MG) es un desarrollo anómalo del nervio óptico, caracterizado por una cavidad amplia en forma de embudo del disco en el punto donde salen las fibras del nervio de la retina. Su presentación más frecuente es unilateral. La mayoría de los casos son aislados y no se asocian con anomalías sistémicas. Objetivo: se presenta el caso de un niño de 1 año con MG y los hallazgos imagenológicos. Caso clínico: presentamos el caso de un paciente masculino de 1 año con sospecha de patología visual; en el estudio se realizó resonancia magnética cerebral (RM) en la que se identificó excavación en forma de embudo de la papila del globo ocular izquierdo, alteración en la morfología del quiasma óptico con desplazamiento inferior asimétrico y engrosamiento del infundíbulo y del tallo hipofisiario. Discusión: el diagnóstico de MG es clínico, las imágenes aportan información adicional sobre anomalías oculares, craneofaciales y vasculares asociadas. En este caso además de la lesión ocular había anomalías de estructuras de línea media como el infundíbulo y el tallo hipofisiario, además de engrosamiento quiasmático. Conclusión: la valoración por imágenes en fundamental, no solo para describir la alteración del nervio óptico, si no para identificar y describir anomalías asociadas para realizar manejo integral del paciente.

Introduction: the congenital optic disc anomaly known as morning glory disc anomaly (MGDA) is an alteration of normal optic nerve development, characterized by an enlarged funnel-shaped cavity of the optic disc, the point in the eye where the optic nerve fibers leave the retina. The anomaly is typically unilateral. Most are isolated cases with no associated systemic anomalies. Objective: a case is presented in a one-year-old boy with MGDA and the corresponding imaging findings. Case reports: we present the case of a one-year-old male patient with suspected visual impairment. Cerebral magnetic resonance imaging (MRI) revealed a papillary funnel-shaped excavation in the left eye, alteration in the morphology and inferior asymmetric displacement of the optic chiasm and thickening of the pituitary infundibular stalk. Discussion: MGDA is diagnosed clinically, however, imaging provides additional information regarding the associated ocular, craniofacial and vascular abnormalities. In this case, in addition to the ocular lesion there were abnormalities of midline structures, such as, the pituitary infundibular stalk, as well as, optic chiasm thickening. Conclusion: imaging is fundamental, not only to describe the anatomic alteration of the optic nerve, but also to identify related abnormalities in order to provide patients a comprehensive management

A Case of Renal Coloboma Syndrome Caused by Spontaneous Mutation of PAX2 / 罕见病研究

This article reports a case of a child with full-length (paired box family, PAX) PAX2 mutation leading to renal coloboma syndrome. The patient is an 11-year-old boy presented with persistent foamy urine and unexplained renal failure. The boy has suffered from vision decline ever since infancy. Genetic testing confirms the mutation of the PAX2 splice site (c.862-1G > A). Sanger sequencing shows no mutation at this site in his parents and demonstrates a spontaneous mutation. His clinical manifestations also confirms diagnosis of renal coloboma syndrome. The PAX2 mutation was responsible for the boy's progression to end-stage renal disease and extrarenal manifestations.

Estafiloma peripapilar, presentación clínica y diagnósticos diferenciales en anomalías congénitas de nervio óptico / Peripapillary staphyloma, clinical presentation, and differential diagnoses in congenital optic nerve anomalies

Un paciente de 3 meses con sospecha de estrabismo presenta al examen una gran anisometropía con -9.50 esfera en su ojo izquierdo y al fondo de ojo destaca una papila alterada similar a la papila de Morning-Glory. Las anomalías congénitas de nervio óptico (ACNO) son un grupo de condiciones que se presentan clínicamente como baja agudeza visual, estrabismo y/o nistagmus. El diagnóstico dife-rencial es clínico y complejo por la sobre posición morfológica entre estas. Los principales diagnósticos diferenciales son la papila deMorning-Glory, el coloboma y la hipoplasia de nervio óptico. Se recomienda manejo multidisciplinario por sus asociaciones sistémicas.

A 3-month-old patient with a suspicion of strabismus on examination has a great anisometropia with -9.50 sphere in his left eye and at the fundus examination an altered papilla similar to the Morning-Glory papilla. Congenital optic nerve abnormalities (ANOC) are a group of conditions that present clinically as low visual acuity, strabismus, and nystagmus. The differential diagnosis is clinical and complex due to the morphological position between them. The main differential diagnoses are the Morning-Glory papilla, the coloboma, and optic nerve hypoplasia. Multidisciplinary management is recommended for its systemic associations.

Morning glory disc anomaly and facial hemangiomas in a girl with moyamoya syndrome

Moyamoya disease (MMD) is a chronic progressive, occlusive cerebrovascular disease in the circle of Willis and the feeding arteries. Morning glory disc anomaly (MGDA) is characterized by an abnormal excavated optic disc with radial emergence of blood vessels from the rim of the disc. We describe a case of moyamoya syndrome (MMS), a rare entity among Indian ethnicity, associated with MGDA and regressed facial capillary hemangiomas, which are relatively less reported presentations of MMD. This report emphasizes on the role of neuroimaging in MGDA, so as to facilitate early detection and management of life-threatening intracranial pathologies such as MMS.

Estudio farmacobotánico de Ipomoea purpurea (L) Roth (Convolvulaceae) / Pharmacobotanical study of Ipomoea purpurea (L.) Roth (Convolvulaceae)

Ipomoea purpurea (L.) Roth (Convolvulaceae) es una especie que se desarrolla en las zonas tropicales de ambos hemisferios. Por el atractivo de sus flores pertenece al grupo conocido como "Common morning glory", especies empleadas por diversos grupos nativos en rituales mágico - religiosos y popularmente como alucinógeno, analgésico, antiinflamatorio y purgante, entre otros. Dada la gran similitud de I. purpurea con otras especies del mismo género (características ecológica, morfológicas y fitoquímicas), se plantean actualmente discusiones de identificación incluso a nivel taxonómico. El objetivo de este estudio es determinar las principales características diagnósticas para la identificación de I. purpurea y registrar sus atributos morfoanatómicos generales. Se trabajó con material fresco proveniente de INTA Castelar y ejemplares de herbario. Se analizaron las hojas, tallos, raíces, flores y semillas. Se realizaron disociados leves, fuertes, raspados, reducción a polvo, cortes transversales, improntas de epidermis y peeling. Se realizaron reacciones histoquímicas para almidón, lípidos y antocianos. El material se observó mediante microscopía de luz clara, contraste de fase (DIC) y luz polarizada y se realizó microscopía cuantitativa. Entre los caracteres principales pueden mencionarse: en las hojas, epidermis con estomas paracíticos y anomocíticos, tricomas tectores unicelulares de tres tamaños diferentes, tricomas glandulares pluricelulares, drusas. En los tallos se observaron los mismos tricomas que en las hojas y tubos laticíferos. En la raíz, laticíferos, almidón y drusas. En la flor, tricomas tectores similares a los de las hojas pero con base ensanchada y tricomas glandulares, drusas, antocianinas y granos de polen equinados-pantoporados. En las semillas, esclereidas, lípidos, granos de almidón de diferente tamaño y de formas esférica y ovoidal, ambos con hendidura presente, drusas y cavidades, aparentemente, de tipo esquizógeno. Los tres tamaños diferentes de tricomas tectores, los tricomas glandulares y las cavidades presentes en la semilla constituyen la primera observación para esta especie. Este estudio proporciona datos relevantes para I. purpurea y provee elementos de diagnóstico para ser empleados en la diferenciación respecto de otras especies taxonómicamente afines. (AU)

Anomalía de Morning Glory: reporte de caso y revisión de la literatura / Morning Glory Anomaly: Case report and literature review

Objetivo: Describir el caso clínico de una paciente con anomalía de Morning Glory (MGA) y documentar acerca de esta entidad lo encontrado en la literatura. Diseño del estudio: Reporte de caso clínico. Métodos: Descripción de un caso clínico de una paciente con MGA en uno de sus ojos quien consultó al Servicio de Oftalmología del Hospital Santa Clara. Resultados: Se presenta el caso de una paciente añosa con MGA sin compromiso sistémico. Esta entidad descrita como parte de las anomalías del disco óptico, de predominio unilateral, en mujeres, se asocia generalmente con agudeza visual baja y anomalías oculares como aniridia, membrana pupilar persistente y catarata,entre otras, así como anomalías cerebrales principalmente en los raros casos bilaterales. A pesar de la ausencia de un tratamiento específico para la anomalía, la intervención correcta en la edad pediátrica de este grupo de pacientes, puede hacer una diferencia en su futuro, con un seguimiento periódico y manejo interdisciplinario apropiado.

Objective: To describe the clinical case of a patient with Morning Glory Anomaly (MGA). Study design: Case report. Methods: Description of a clinical case of a patient with MGA in one of her eyes who consulted the Ophthalmology Service of the Santa Clara Hospital. Results: The case of an elderly patient with MGA without systemic compromise is presented. Th is entity is described as part of the unilateral optic disk abnormalities, more frequent in women and is associated with low visual acuity and ocular abnormalities such as aniridia, persistent pupillary membrane and cataract, as well as cerebral abnormalities in the rare cases of bilateral presentations. Despite the absence of specifi c treatment for the anomaly, correct intervention in the pediatric age of this group of patients can make a difference in their future, with periodic monitoring and appropriate interdisciplinary management.

Superação de dormência em sementes de três espécies de Ipomoea / Overcoming dormancy seed in three Ipomoea species

O conhecimento do método de superação de dormência mais adequado para cada espécie auxilia na identificação do tipo de dormência, bem como na definição de estratégias de manejo que permitam o controle mais eficaz. As espécies do gênero Ipomoea encontram-se amplamente distribuídas nos estados brasileiros, as mais comumente encontradas em lavouras de soja na região Sudoeste do Paraná são I. purpurea, I. indivisa e I. grandifolia. Assim, neste trabalho, o objetivo foi selecionar métodos eficazes de superação da dormência nas espécies Ipomoea indivisa, I. grandifolia e I. purpurea e determinar como eles influenciam na cinética da germinação das sementes dessas espécies. Os tratamentos utilizados foram água quente, escarificação mecânica, escarificação mecânica + resfriamento, escarificação química e testemunha. Na avaliação do efeito dos tratamentos, determinou-se a porcentagem de germinação, tempo médio, índice de velocidade e frequência relativa de germinação. A escarificação com ácido sulfúrico incrementou a germinação das sementes de I. indivisa e I. purpurea e o índice de velocidade de germinação àquelas de I. purpurea. O tratamento com água quente foi o mais eficiente em promover a germinação das sementes de I. grandifolia, e em reduzir o tempo médio de germinação das sementes de I. grandifolia e I. purpurea, de forma que, nos tratamentos de superação de dormência, há sincronização do processo de germinação.

The knowledge of the method of overcoming dormancy for each species helps in identification of dormancy type and to outline the most appropriate management strategies for effective control measures. The species of the genus Ipomoea are widely distributed in the Brazilian states, the most commonly found in soybean crops in the Southwest region of Paraná are I. purpurea, I. indivisa and I. grandifolia. In this research the aim was select methods of overcoming seed dormancy for species Ipomoea indivisa, I. grandifolia and I. purpurea, and determine how these methods influence the kinetic germination of these species. The treatments consisted of hot water, chiseling, chiseling plus cooling, chemical scarification and control. In evaluating the effect of the treatments were determined the germination percentage, mean germination time, germination speed index and relative frequency of germination. Scarification with sulfuric acid increased the germination of I. indivisa and I. purpurea seeds and the germination speed index of I. purpurea seeds. The hot water treatment was more effective in promoting germination of I. grandifolia seeds, and reduce the average time of germination of I. grandifolia and I. purpurea seeds, so that in treatments for overcoming dormancy there are synchronization of the germination process.

A Case of Intravitreal Bevacizumab Injection for the Treatment of Choroidal Neovascularization in Morning Glory Syndrome

PURPOSE: We report a case of intravitreal bevacizumab injection for the treatment of choroidal neovascularization in morning glory syndrome. CASE SUMMARY: A 51-year-old male visited our hospital for a 1.5-year visual disturbance in his right eye. The patient's best-corrected visual acuity was 0.1 in the right eye. After fundus examination, we found characteristic findings of morning glory syndrome with submacular hemorrhage and serous retinal detachment in the right eye. Optical coherence tomography, fluorescein angiography and indocyanine green angiography were performed for evaluation. Retinoschisis, subretinal fluid, and choroidal neovascularization were detected, and thus bevacizumab was injected in the right eye. After intravitreal bevacizumab injection, retinoschisis was improved, and subretinal fluid was decreased. However, retinal pigment epithelial detachment was newly detected, and serous retinal detachment persisted. After 2 months, a second bevacizumab injection was performed. After these intravitreal bevacizumab injections at 1 and 2 months, visual acuity was 0.4 and 0.6, respectively. Visual acuity improved to 1.0 after 3 months. Visual acuity was maintained for at least 6 months with no relapse of choroidal neovascularization. CONCLUSIONS: The choroidal neovascularization in morning glory syndrome was effectively treated with intravitreal bevacizumab injections.

Correlación clínico-radiológica de las anomalías congénitas excavadas del disco óptico / Clinical and radiological correlation of excavated congenital anomalies of the optic disk

Introducción: el diagnóstico de las anomalías congénitas excavadas del nervio óptico se ha beneficiado con la introducción y desarrollo de las técnicas imagenológicas; particularmente en los casos atípicos o con alteraciones oculares asociadas, donde el examen clínico no puede ser concluyente. Caso clínico: se presenta un paciente con una anomalía congénita del nervio óptico y se discuten las características clínico-imagenológicas que sustentan su diagnóstico diferencial. Se recomienda la evaluación conjunta, por parte de oftalmólogos y radiólogos, de pacientes con estas anomalías; sobre todo en aquellos donde las presentaciones fundoscópicas no son específicas. Conclusiones: el diagnóstico nosológico correcto garantiza una adecuada orientación sobre alteraciones sistémicas relacionadas y patrones de herencia, si los hay

Introducción: the diagnosis of excavated congenital anomalies of the optic nerve has benefitted from the introduction and development of radiological techniques, mainly in atypical cases or those with others related ocular disorders, where the clinical exam is not conclusive. Clincal case: Here is a patient with a congenital optic nerve anomaly. The clinical and radiological characteristics that explain the differential diagnosis of this case were discussed. Emphasis was made on the fact that patients with these anomalies and no specific fundus features must be evaluated by both ophthalmologists and radiologists. Conclusions: the right nosological diagnosis assures the appropriate orientation towards others related systemic associations and inheritance patterns, if any

Vitrectomy of rhegmatogenous retinal detachment in morning glory syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

We report a case of retinal detachment in morning glory syndrome(MGS). We think that identification of the retinal break, removal of the traction force by vitrectomy, the use of long-acting gas as endotamponade, all contributed to the successful treatment of the disease. The pathogenic mechanisms of the disease and the function of B-scan to the diagnosis are included.

A Case of Bilateral Morning Glory Syndrome

PURPOSE: To report a bilateral case of morning glory syndrome. CASE SUMMARY: On May 30, 2002, a six-year-old patient visited our clinic with impaired visual acuity of her left eye and was diagnosed as having a cataract on her left eye superimposed on a bilateral morning glory anomaly. According to cycloplegic refraction, the patient's corrected visions was 0.8 in the right eye and 0.1 in the left. On June 20, 2002, the patient received ultrasonographic phacoemulsification, intraocular lens implantation, synechiolysis and partial vitectomy in her left eye. Approximately 5.2 years after the surgery, according to manifest refraction, the patient's corrected visions were 0.8 in the right eye and 0.1 in the left, no prominent postoperative changes were observed on slit lamp microscopy and fundus examinations. The thickness of the central macula of her right eye had decreased according to optical coherence tomography; the physiologic scotoma size of the patient's right eye had increased with narrowed peripheral visual field of her left eye. CONCLUSIONS: Although monocular morning glory anomaly has previously been reported to occur, in the present study case, the anomaly occurred bilaterally. In morning glory patients, strabismus examination and additional evaluation of a patient's general state should be performed along with a regular fundus examination.

Absence of mutations in Pax6 gene in three cases of Morning Glory syndrome associated with isolated growth hormone deficiency / A ausência de mutações no gene PAX6 em três casos de síndrome de Morning Glory associada à deficiência isolada de hormônio do crescimento

Morning glory syndrome (MGS) is a congenital optic disc dysplasia often associated with craniofacial anomalies, especially basal encephalocele and hypopituitarism. Clinical signs are varied and often occult. The PAX6 gene is involved in ocular morphogenesis and is expressed in numerous ocular tissues during development especially in the developing central nervous system. The aim of the present study is to evaluate PAX6 in MGS associated with isolated growth hormone deficiency. Three pre-pubertal males (A, B and C) with MGS and short stature due to growth hormone deficiency, treated with recombinant human growth hormone with limited response, were reported. Two of them had basal encephalocele. Coding and non-coding sequences corresponding of PAX6 different transcripts were analyzed by direct sequencing. Nucleotide variations causing putative aminoacid change were not observed. Patient A presented the new IVS2+9G>A transition, whereas patients A and C were heterozygous for known single nucleotide polymorphisms (SNP) within the intron 4. In addition, two SNP heterozygoses were observed for patient C in both intron 9 and 13. Sequencing also revealed several nucleotide variations in patient B. Two heterozygoses for known polymorphisms were identified along with a novel C>A nucleotide change in intron 4. This patient also presented a low number on the TG repeat in intron 9 and a new IVS11+33A>T transversion. Gene regulation and transcription of PAX6 are complex processes; there are two major protein isoforms, PAX6(-5a) and PAX6(+5a), and nine transcripts described. Furthermore, extra transcription regulatory elements have been postulated within PAX6 introns. Considering that neither population distributions on PAX6 polymorphism nor their linkeages with diseases have been reported, a functional effect due to alterations described here cannot be discarded.

A síndrome de Morning Glory (SMG) é uma displasia óptica congênita associada a anomalias craniofaciais, principalmente encefalocele basal e hipopituitarismo. Os sinais clínicos são variados e muitas vezes ocultos. O gene PAX6 está envolvido na morfogênese ocular e se expressa em vários tecidos oculares durante o desenvolvimento do sistema nervoso central. O objetivo deste estudo foi avaliar o gene PAX6 na SMG associada com deficiência isolada de hormônio de crescimento. Foram relatados três pacientes pré-púberes (A, B e C) com SMG e baixa estatura por deficiência de hormônio de crescimento tratados com hormônio de crescimento recombinante humano. As seqüências codificadoras e não-codificadoras correspondentes ao PAX6 em diferentes transcritos foram analisadas por seqüenciamento direto. Variações nucleotídeas com trocas pontuais de aminoácidos não foram encontradas. O paciente A apresentou uma transição nova IVS2+9G>A, enquanto os pacientes A e C foram heterozigotos para um polimorfismo (SNP) no íntron 4. Ainda, dois SNPs em heterozigose nos íntrons 9 e 13 foram observados no paciente C. O seqüenciamento também mostrou várias variações nucleotídeas no paciente B. Dois SNPs conhecidos com a alteração nucleotídea nova C>A no íntron 4 foram observados em heterozigose. Este paciente também apresentou um baixo número de repetições TG no íntron 9 e uma nova transversão IVS11+33A>T. A regulação e a transcrição do gene PAX6 são um processo complexo; existem 2 isoformas principais da proteína, PAX6(-5a) e PAX6(+5a) e 9 transcritos descritos. Considerando que nem a distribuição de SNPs no PAX6 e nem as suas ligações com as doenças foram relatadas, um defeito funcional devido às alterações descritas não pode ser descartado.

The Clinical Characteristics and Imaging Findings of Morning Glory Syndrome / 华中科技大学学报（医学）（英德文版）

To investigate the features of CT, ultrasonography and fundus fluorescein angiography(FFA) of morning glory syndrome, the data on CT, A/B-scan ultrasonography and fundus fluorescein angiography (FFA) were retrospectively analyzed in 8 cases of morning glory syndrome (MGS).Among those cases, 6 were examined with CT, 4 with FFA and 8 with A/B-scan ultrasonography.Results showed that the characteristics of CT, A/B-scan ultrasonography and FFA in MGS included:(1) The attachment spot of optic nerve became thin and vitreous body protruded to the posterior wall of eyeball with a spherical shape on CT image; (2) in the early period of FFA, hypofluorescence appeared on the optic, the abnormal arteriae and veins around the optic papilla were displayed clearly and in the late period, optic disc was stained with fluorescein; (3) on B-scan ultrasonogram, the vitreous cavity extended to the posterior pole and optic papilla, and projected to the basal part of muscle cones and thus the posterior part of vitreous cavity looked like an upside-down bottleneck. Sometimes the echogenic band of retinal detachment could also be seen. On A-scan ultrasonogram, both vitreous cavity and bottleneck showed no ultrasonic echoes and presented a base line without any evident wave crest. It is concluded that CT, A/B-scan ultrasonography and FFA could show the imageological features of MGS from different aspects, which helps clinicians to differentiate it from other diseases such as optic disc coloboma. CT and A/B-scan ultrasonography, in particular, are considered to be reliable imageological methods for the accurate diagnosis of MGS and are superior to the traditional techniques.

Basal encephalocele associated with morning glory syndrome: case report

The basal encephaloceles refer to rare entities and they correspond to herniation of brain tissue through defects of skull along the cribiform plate or the sphenoid bone. A rare morning glory syndrome, with characteristic retinal defect has been reported in association with basal encephaloceles. Hypophysis hormonal deficiencies may occur. We accounted for a pituitary dwarfism with delayed diagnosed transsphenoidal encephalocele associated with morning glory syndrome, showing the alterations found in retinography, computed tomography and magnetic resonance imaging.

As encefaloceles basais são entidades raras e correspondem a herniações do tecido cerebral através de um defeito do crânio, ao longo da lâmina crivosa etmoidal ou do osso esfenoidal. A rara síndrome morning glory, com alterações de fundo de olho características pode apresentar-se associada à encefalocele basal. Deficiências hormonais hipofisárias podem ocorrer. Relatamos caso de nanismo hipofisário com encefalocele transesfenoidal de diagnóstico tardio associada à síndrome de morning glory, mostrando as alterações na retinografia, tomografia computadorizada e ressonância magnética.

CT diagnosis of morning glory syndrome (report of 7 cases) / 中华放射学杂志

Objective To study the CT findings and diagnostic role in morning glory syndrome (MGS). Methods CT study of 7 patients with MGS diagnosed by clinical information was reviewed retrospectively. Results CT findings included: (1) the crater like excavation of the disc in 3 patients; (2) the funnel shaped widening of the retrobulbar optic nerve, a pear shaped deformity of the globe in 2 patients; (3) two patients with cystic expansion of the optic nerve, which was of water density and was continuous with the vitreous humor. Conclusion The CT findings of MGS were particularly striking. CT is the imaging method of choice in the diagnosis of this disorder.

Image findings of morning glory syndrome / 中华超声影像学杂志

Objective To evaluate the significance of ultrasonography in diagnosis and differentiation of morning glory syndrome. Methods Ultrasonography,CT and fundus fluorescein angiography(FFA) features were retrospectively analyzed in 6 cases of morning glory syndrome.Results Ultrasonography, CT and FFA characters of morning glory syndrome were as follow: on the B-type ultrasonogram, the dark area of vitreous cavity extended to the posterior pole and optic papilla, projecting to the basal part of muscle cones, thus the posterior part of vitreous cavity looked like a converted bottleneck. Sometimes the light ribbon of retinal detachment can also be seen. On the A-type ultrasonogram, both vitreous cavity and bottleneck showed no ultrasonic echo and acted as an equable baseline without any evident wave crest. The attachment spot of optic nerve of eye turned thin and vitreous body protruded to the posterior wall of eyeball with spherical shape on CT imaging. In the earlier period of FFA, hypofluorescence appeared on the optic disc; then ,the abnormal arteriae and vein around the optic papilla were diplayed clearly; In the later period, optic discs were dyed with fluorescein. Conclusions Ultrasonography, CT and FFA showed imageological features of morning glory syndrome from different aspects, which was helpful for differentiating similar diseases such as optic disc coloboma. Especially, ultrasonography was considered as a more safe and reliable imageological method than other imageological methods in precisely diagnosing and differentiating morning glory syndrome and superior to the traditional fundus check which gives no integrity.

A Case of Epidermal Nevus Syndrome Associated with Morning Glory Syndrome and Intestinal Pseudoobstruction / 대한피부과학회지

Epidermal nevus syndrome is defined as the association of epidermal nevi with abnormalities of the other organ systems, and is regarded as one of the neurocutaneous syndromes. However, till now, epiderrnal nevus syndromi has not been reported with either Morning glory syndrome or intestinal pseudoobstruction. We experienced a case of a 7-year old rnale who had had multiple epidermal nevi on the scalp and forehead and solitary larg cafeau lait spot on his chest since birth. He had histories of failure to thrive and mental retardation, but no seizures. He also had histories of frequent diarrhea and abdominal distension. Physical examination showed abnormalities of the skin, teeth, and Morning glory syndrome of both oplic discs. Colon study, small bowel series and biopsy showed intestinal pseudoobstruction. Skeletal survey revealed a difference between the left upper extremity and the right one.

Treatment of Morning Glory Syndrome with Retinal Detachment

Morning glory syndrome is a rare congenital anomaly in optic disc, which is characterized by defect in optic disc due to optic nerve dysgenesis, abnormal retinal vessels, and chorioretinal pigment disturbance around optic disc. Retinal detachment in morning glory syndrome occurs in 26% to 38% of the cases, which is thought to be non-rhegmatogenous in nature and usually develops in posterior pole of the retina. Surgical treatment is indicated in cases with bullous and total retinal detachment in morning glory syndrome. The authors successfully reattached the detached retina in a 3 year old boy with morning glory syndrome using trans pars plana vitrectomy, scleral buckling, retinotomy, silicone oid injection, and endolaser photocoagulation. The authors followed up for 15 weeks after operation, and the vision of left eye was counting finger and the retina remained to be reattached.

Treatment of Morning Glory Syndrome with Retinal Detachment

Morning glory syndrome is a rare congenital anomaly in optic disc, which is characterized by defect in optic disc due to optic nerve dysgenesis, abnormal retinal vessels, and chorioretinal pigment disturbance around optic disc. Retinal detachment in morning glory syndrome occurs in 26% to 38% of the cases, which is thought to be non-rhegmatogenous in nature and usually develops in posterior pole of the retina. Surgical treatment is indicated in cases with bullous and total retinal detachment in morning glory syndrome. The authors successfully reattached the detached retina in a 3 year old boy with morning glory syndrome using trans pars plana vitrectomy, scleral buckling, retinotomy, silicone oid injection, and endolaser photocoagulation. The authors followed up for 15 weeks after operation, and the vision of left eye was counting finger and the retina remained to be reattached.

Morning Glory Syndrome

Recently we have experienced two cases of the morning glory syndrome which is an uncommon congenital anomaly of optic nerve head. Visual evoked potentials and fluorescein angiographic findings are described. The visual evoked potentials elicited with bright flashes and patterns revealed delayed latencies and subnormal amplitudes. The amplitude of the traces probably depends on the severity of the involvement of the optic nerve. Also, the possible macular abnormalities and the strabismic or anisometropic amblyopia developing in morning glory syndrome may contribute to the abnormal finding of visual evoked potentials.