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

The deceptive coloboma

Background: Scleral fistulas are known to develop following blunt trauma in patients with retinochoroidal coloboma. These cases can be managed by surgical options such as silicone buckles, or with glue and scleral patch graft. Some cases have been shown to close spontaneously. We report the first?ever case managed by vitrectomy, endophotocoagulation, and gas tamponade. Purpose: We present a rare and interesting case of an atypical choroidal coloboma with traumatic scleral fistula due to blunt trauma manifesting with hypotony?related disc edema, maculopathy, and chorioretinal folds, which was managed surgically with vitrectomy, endophotocoagulation, and gas tamponade with a good anatomical and visual outcome. Synopsis: The video contains the case description and surgical management of a traumatic scleral fistula in a patient with atypical superotemporal choroidal coloboma. The patient developed hypotonic maculopathy and disc edema after 3 months following a blunt trauma sustained in a road traffic accident. A scleral fistula was suspected at the temporal edge of the coloboma but could not be accurately localized. In addition, due to the edge effect of the coloboma, the external repair was difficult. Hence, vitrectomy with internal tamponade was attempted. Highlights: The video highlights a different surgical approach to managing a traumatic scleral fistula at the edge of a retinochoroidal coloboma. There was a risk of leakage of intravitreal fluid into the orbit through the fistula; however, the gas bubble gave a better tamponade due to higher surface tension. It sealed the fistula presumably by creating a trap?door?like effect. The endophotocoagulation helped create adhesion between the tissues at the edge of the coloboma effectively sealing it. This was followed by a rapid recovery of the hypotony?related problems with good vision. Traumatic scleral fistula, at a difficult place such as the edge of a coloboma, can be successfully closed from an internal approach with vitrectomy, endolaser, and gas tamponade.

Spectrum of congenital and inherited ocular disorders seen in a genetic clinic: Experience of a developing ocular genetic service

Purpose: Hereditary causes are an important etiological category of childhood blindness. This study reports the real?world experience of a developing ocular genetic service. Methods: The study was carried out from Jan 2020 to Dec 2021 jointly by the Pediatric Genetic Clinic and the Department of Ophthalmology of a tertiary care hospital in North?West India. Children presenting to the genetic clinic with congenital or late?onset ocular disorder(s) and any individual (irrespective of age) suffering from an ophthalmic disorder and referred by an ophthalmologist for genetic counseling for himself/herself and/or his/her family member(s) were included. Genetic testing (exome sequencing/panel?based sequencing/chromosomal microarray) was outsourced to third?party laboratories with the cost of the test being borne by the patient. Results: Exactly 8.6% of the registered patients in the genetic clinic had ocular disorders. Maximum number of patients belonged to the category of anterior segment dysgenesis, followed by microphthalmia anophthalmia coloboma spectrum, lens disorders, and inherited retinal disorders in decreasing numbers. The ratio of syndromic ocular to isolated ocular disorders seen was 1.8:1. Genetic testing was accepted by 55.5% of families. The genetic testing was clinically useful for ~35% of the tested cohort, with the opportunity for prenatal diagnosis being the most useful application of genetic testing. Conclusion: Syndromic ocular disorders are seen at a higher frequency compared to isolated ocular disorders in a genetic clinic. Opportunity for prenatal diagnosis is the most useful application of genetic testing in ocular disorders.

Clinical characteristics and treatment progress of microphthalmos-anophthalmos-coloboma / 国际眼科杂志(Guoji Yanke Zazhi)

Microphthalmos-anophthalmos-coloboma(MAC)is structurally and clinically correlated congenital ocular malformation. Although MAC is relatively rare, they still become an important cause of blindness in children. At present, the etiology of MAC is not clear; a variety of genetic or environmental factors may cause MAC. Because of its special anatomical structure, MAC has complex clinical manifestations and multiple complications. The complications including cataract, angle-closure glaucoma, uveal effusion, retinal detachment, orbital cyst, which are often accompanied with increased risks for surgery and poor prognosis. By summarizing the clinical features, the common types of ocular complications, and the progress of the treatment, this article will enhance clinicians' cognition of MAC and guide clinicians to formulate personalized and safe treatment plans for patients.

Coloboma associated retinal detachment: Anatomical and functional results in the era of microincision vitrectomy surgery with an evaluation of risk factors for a recurrence

Purpose: To analyze the anatomical and visual outcomes of microincision vitrectomy surgery (MIVS) with silicone oil tamponade in eyes having coloboma?related retinal detachment (RD) and evaluate the risk factors for recurrence of RD. Methods: This was a retrospective, multicentric analysis of eyes having coloboma RD undergoing MIVS with silicone oil tamponade between March 2010 and July 2018. Results: We evaluated 148 eyes of 144 patients. The mean age of presentation was 17.4 ± 9.8 years (range: 2–65 years) and the mean follow?up duration was 13.1 ± 13.8 months (range: 3–84 months). The single operation success rate was 88.5% (131 eyes), with an overall successful outcome achieved in 90.5% (134 eyes). Recurrence of RD occurred in 17 eyes (11.5%) over a mean duration of 2.59 ± 3 months. The risk of recurrence was found to be higher in eyes where relaxing retinectomy (RR) was performed (odds ratio [OR]: 3.22; P = 0.05). A statistically significant improvement in vision was noted in the majority of cases from logMAR 1.85 ± 0.34 preoperatively to logMAR 1.33 ± 0.6 post?surgery (P = 0.002). Conclusion: MIVS with silicone oil tamponade provided an anatomical success rate of 90.5% in eyes with coloboma RD with a significant improvement in visual acuity. Eyes in which RR was performed were susceptible to higher rates of re?detachment.

Iridochorioretinal coloboma associated with buried optic nerve drusen: a case report / Coloboma irido-coriorretiniano associado a drusas enterradas do nervo óptico: Relato de caso

ABSTRACT Improper closure of the embryonic fissure results in ocular coloboma. Optic nerve head drusen are hyaline deposits located anterior to the lamina cribosa that grow and calcify over time. It is rarely associated with ocular coloboma, with only two cases reported. We present a patient with an irido-chorioretinal coloboma, poorly defined optic nerve limits in the right eye, and increased papillary vascular ramification and peripapillary atrophy in the left eye, without any visible drusen. Fundus autofluorescence, high-resolution optical coherence tomography, and B-scan ultrasonography confirmed the diagnosis of bilateral buried optic nerve head drusen. The association between irido-chorioretinal colobomas and optic nerve drusen in the absence of a systemic disease is exceptional. Our case demonstrates that multimodal imaging is important to correctly diagnose buried optic nerve head drusen.

RESUMO O coloboma ocular é o resultado de um fechamento impróprio da fissura embrionária. As drusas da cabeça do nervo óptico são depósitos hialinos localizados anteriormente à lâmina cribosa que crescem e se calcificam com o tempo. A associação de ambos é rara, com apenas dois casos descritos na literatura. Apresentamos um paciente com coloboma irido-coriorretiniano e limites do nervo óptico mal definidos em seu olho direito, e aumento da ramificação vascular papilar e atrofia peripapilar em seu olho esquerdo, sem drusas visíveis. Autofluorescência de fundo, tomografia de coerência óptica de alta resolução e ultrassonografia B-scan foram realizadas confirmando o diagnóstico de drusas enterradas bilaterais da cabeça do nervo óptico. A associação entre colobomas irido-coriorretinianos com drusas do nervo óptico na ausência de doença sistêmica é excepcional. Nosso caso demonstra a importância da imagem multimodal para o diagnóstico correto de drusas enterradas de cabeça do nervo óptico.

A novel mutation in PAX6 gene causing congenital iris coloboma with congenital cataract in a pedigree / 中华实验眼科杂志

Objective:To identify the pathogenic gene and inheritance pattern in a pedigree of congenital iris coloboma with congenital cataract.Methods:The method of pedigree investigation was adopted.A pedigree of congenital iris coloboma with congenital cataract was collected by Yunnan Disabled Rehabilitation Center and the 2nd Afliated Hospital of Kunming Medical University in February 2020.Ophthalmic examinations were carried out on the female proband, her parents, her children and her husband, and the clinical diagnosis was made.Genomic DNA was extracted from peripheral blood samples collected from the family members.The suspected pathogenic gene in the proband and her husband was screened by whole exome sequencing and was identified by bioinformatics analysis.The amino acid conservation was analyzed by UGENE software.The impact of the mutation on protein translation was predicted using MutationTaster software.The pathogenicity of the mutation was assessed according to the American College of Medical Genetics (ACMG) Standards and Guidelines.Pathogenic gene and mutations were verified by Sanger sequencing.This study adhered to the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of the 2nd Afliated Hospital of Kunming Medical University (No.PJ-2020-61).Written informed consent was obtained from each subject or custodian.Results:The proband showed large iris defects in both eyes with only a small amount of observable iris tissue in the periphery, lens cortical opacity and posterior capsule opacification, accompanied by nystagmus.A novel heterozygous frameshift variation c. 415dupA (p.R139fs) was located in exon 8 of PAX6 gene, and the variation was conservative across multiple species.The variation was in the highly conserved region of PAX6 gene and caused the dysfunction of PAX6 protein.The variation was graded as PVS1+ PM2+ PP1, a pathogenic variation, based on ACMG guidelines.The pedigree was consistent with co-segregation, indicating that the novel variation was pathogenic.The proband and her children were diagnosed, but her parents were phenotypically normal, in accordance with autosomal dominant inheritance. Conclusions:The novel frameshift variation c.415dupA (p.R139fs) on the exon 8 of PAX6 gene is responsible for congenital iris coloboma with congenital cataract in the pedigree.This is the first report of this novel variation in PAX6 gene.

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.

The Canabrava Ring, an open ring-shaped pupil expander for cataract surgery associated with iris colobomata: a case report / Aplicação de anel expansor de pupila pequena em formato de anel aberto para cirurgia de catarata em coloboma de íris: relato de caso

ABSTRACT In this report, we describe a new pupil expander device that was used to obtain adequate pupil dilation and centering in a patient with an iris coloboma. Specifically, we describe the case of a patient with an iris coloboma; a Malyugin ring was inserted to facilitate dilation during phacoemulsifi­cation surgery. One of the scrolls did not engage which resulted in an uneven distribution of forces and an eccentric pupil. A Canabrava Ring was then implanted that promoted effective pupillary dilation and remained stable and effective throughout the surgical procedure.

RESUMO Neste relato, descrevemos um novo dispositivo expansor pupilar que foi usado obter adequada dilatação e centralização da pupila em um paciente com coloboma de íris. Especificamente, descrevemos um caso de cirurgia de facoemulsificação em um paciente com coloboma de íris associado à pupila pequena e que, previamente, tentou-se sem sucesso o uso do expansor Malyugin Ring, que provocou uma dilatação pupilar descentrada. Entretanto, com o uso do expansor de íris Canabrava Ring, a pupila permaneceu dilatada e centrada durante toda a cirurgia, permitindo a realização de um procedimento seguro.

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.

Coloboma del iris, presentación de caso / Iris coloboma, case presentation / Iris coloboma, apresentação do caso

RESUMEN Introducción: el coloboma es un defecto congénito del sector inferior del iris o bien, una escotadura en el margen pupilar que otorga a la pupila un aspecto de cerradura. Presentación del caso: se presenta el caso de un paciente masculino de 53 años de edad con diagnóstico clínico de coloboma del iris en el cuadrante inferotemporal izquierdo. Discusión: el coloboma del iris, es hasta la actualidad una condición rara con una incidencia de 2.4-8.0/10,000 nacidos vivos. La mayoría de las causa de este tipo de coloboma es desconocida, y ocasionalmente puede producirse debido cirugía del ojo, traumatismo del ojo, afecciones hereditarias, algunos se deben a un defecto genético específico y un pequeño número de pacientes con coloboma tiene otros problemas hereditarios del desarrollo. Conclusiones: el coloboma del iris es una entidad poco frecuente, de diagnóstico casual, que suele ser asintomática; es importante al hacer el diagnóstico, realizar un examen físico bien detallado en el paciente para descartar síndromes asociados a esta malformación congénita.

ABSTRACT Introduction: the coloboma is a birth defect of the lower sector of the iris or a neckline in the pupil margin that gives the pupil a locking appearance. Case presentation: the case of a 53-year-old male patient with clinical diagnosis of iris coloboma in the left infernotemporal quadrant is presented. Discussion: the coloboma of the iris, is to present a rare condition with an incidence of 2.4-8.0/10,000 live births. Most of the causes of this type of coloboma are unknown, and can occasionally occur due to eye surgery, eye trauma, hereditary conditions, some are due to a specific genetic defect and a small number of patients with coloboma have other hereditary developmental problems. Conclusions: iris coloboma is a rare, casually diagnosed entity that is usually asymptomatic; it is important when making the diagnosis, performing a thorough physical exam on the patient to rule out syndromes associated with this congenital malformation.

RESUMO Introdução: o coloboma é um defeito de nascimento do setor inferior da íris ou um decote na margem da pupila que dá ao aluno uma aparência de bloqueio. Apresentação do caso: É apresentado o caso de um paciente do sexo masculino de 53 anos com diagnóstico clínico de iris coloboma no quadrante infernotemporal esquerdo. Discussão: o coloboma da íris, é apresentar uma condição rara com incidência de 2,4-8.0/10.000 nascidos vivos. A maioria das causas desse tipo de coloboma são desconhecidas, e podem o correr ocasionalmente devido a cirurgia ocular, trauma ocular, condições hereditárias, algumas são devido a um defeito genético específico e um pequeno número de pacientes com coloboma tem outros problemas hereditários de desenvolvimento. Conclusões: iris coloboma é uma entidade rara, casualmente diagnosticada que geralmente é assintomática; é importante ao fazer o diagnóstico, realizar um exame físico minucioso no paciente para descartar síndromes associadas a essa malformação congênita.

Spontaneous scleral rupture associated with retinochoroidal coloboma / Ruptura espontânea de esclera associada a coloboma coriorretiniano

Abstract We reported a case of ocular hypotony due to spontaneous scleral rupture in retinochoroidal coloboma and the treatment which was performed. This is a prospective case report in which a 21-year-old woman complained of abrupt vision loss in her left eye. Ocular hypotony due to spontaneous scleral rupture in retinochoroidal coloboma was identified through tests. An ultrassonography confirmed the scleral lesion but magnetic resonance imaging and fluorescein angiography have also proven to be useful. Due to the posterior location of the perforation, we opted for a sub-tenon injection of autologous blood and intraocular gas tamponade, which were successful, resulting in improved visual acuity and intraocular pressure. In this case, an alternative to invasive surgical procedure in the treatment of spontaneous scleral perforation and retinochoroidal coloboma was presented.

Resumo Relatamos um caso de hipotensão ocular secundária a ruptura escleral espontânea em coloboma coriorretiniano e o tratamento realizado. Trata-se de estudo de caso prospectivo de uma mulher de 21 anos de idade que se apresentou queixando perda súbita de visão no olho esquerdo. Ao exame, foi evidenciado hipotensão ocular em virtude de ruptura espontânea de esclera em coloboma coriorretiniano. Ultrassonografia confirmou a lesão da esclera embora ressonância magnética e angiofluoresceinografia também tenham sido úteis. Em virtude da localização posterior da perfuração, optamos por injeção sub-tenoniana de sangue autólogo e tamponamento intraocular com gás, que demonstraram ser úteis, resultando em melhora da acuidade visual e da pressão intraocular. Neste caso, apresentamos uma alternativa a procediemnto cirúrgico invasivo para tratamento de perfuração espontânea de esclera em coloboma coriorretiniano.

Pars-plana vitrectomy with phacofragmentation for hyperdense cataracts in eyes with severe microcornea and chorio-retinal coloboma: A novel approach

Purpose: To report the outcomes of pars-plana approach for the management of brunescent cataract in eyes with severe microcornea and associated chorio-retinal coloboma. Methods: This was a retrospective, single center, interventional case series performed in a tertiary eyecare center in central Medical records of consecutive cases of microcornea with coloboma who underwent pars-plana vitrectomy with phacofragmentation (PF) between January 2015 and December 2017 were reviewed. Results: The study group comprised of 30 eyes of 30 patients, of which 18 (60%) were males and 12 (40%) were females. The mean age of the patients was 41.9 years (range of 17� years). The mean corneal diameter was 6.7 mm with a range of 4�mm and all the eyes had dense cataract with nuclear sclerosis of grade 4 or more. The mean preoperative visual acuity was 1.97 (+/-0.067) Log MAR and the mean postoperative vision at 1 month was 1.6 (+/-0.39) Log MAR. Postoperatively, 21 patients (70%) gained ambulatory vision. The visual gain in all the patients was maintained over a mean follow-up period of 15.5 months. Conclusion: Pars-plana vitrectomy with PF can be considered in eyes with severe microcornea and brunescent cataracts, where cataract surgery through the limbal (anterior) approach is not only difficult but at times impossible due to anatomical restraints.

Exame ultrassonográfico no diagnóstico de coloboma retinocoroidiano bilateral / Ultrasonographic examination in the diagnosis of bilateral retinochoroid coloboma

Resumo Relatamos aqui o caso de E.R.S.S., feminino, 43 anos, diagnosticada com coloboma de retina e coroide bilateral, afim de enfatizar a importância dos exames pré-operatórios, até mesmo de uma boa tomada de projeção luminosa, a qual está sendo muitas vezes relegada a um patamar desprezível dentro da prática oftalmológica. Salientamos também que o exame ultrassonográfico prévio à indicação cirúrgica é de suma importância, no entanto, esse deve ser realizado por profissional experiente e sua correta interpretação deve ser exaustivamente procurada para que erros interpretativos não se transformem em conduta clínico cirúrgica inadequada e consequentes danos, muitas vezes, irreparáveis. Considerando-se todos os aspectos e complicações do coloboma já citados nesse relato, a conduta diante de um diagnóstico dessa malformação deve ser: pesquisar associação com outras doenças oculares e/ou sistêmicas (CHARGE: coloboma, cardiopatia congênita, atresia de coana com múltiplas anomalias), realizar tratamento e acompanhamento em caso de complicações (Ex. descolamento de retina, ambliopia e estrabismo) e prevenção é feita através de aconselhamento genético.

Abstract We report here the case of E.R.S.S. female, 43 years old, diagnosed with bilateral coloboma of choroid and retina, in order to emphasize the importance of preoperative exams, even a good shot of light projection, which is often being relegated to a negligible level in ophthalmological practice.We emphasize also that the ultrasound examination prior to the surgical indication is of paramount importance, however,this should be performed by experienced professional and the correct interpretation must be thoroughly searched for interpretative errors not become inadequate surgical clinical conduct and consequential irreparable damage. Considering all aspects and complications already mentioned in this report, coloboma to conduct before a diagnosis of this malformation should be: search for association with other eye diseases and/or systemic (CHARGE: coloboma, congenital heart defect, atresia of posterior nasal apertures with multiple anomalies), performing and monitoring treatment in case of complications (E.g. retinal detachment, amblyopia and strabismus) and prevention is made through genetic counseling.

Lensectomía refractiva en coloboma de iris / Refractive lensectomy for iris coloboma

RESUMEN El coloboma de iris es un defecto congénito, que se describe como un orificio, fisura o hendidura en dicha estructura.​ Esta condición tiene la posibilidad de ser hereditaria o aparecer sin historia familiar previa. Se presenta un caso de un paciente de 51 años de edad con coloboma bilateral de iris asimétrico e hipermetropía, quien acudió a la consulta médica con el deseo de independizarse de su corrección óptica habitual. Se propuso cirugía facorrefractiva de ambos ojos con pupiloplastia del ojo derecho, en el que se obtuvieron resultados visuales satisfactorios después de la intervención quirúrgica(AU)

ABSTRACT The iris coloboma is a congenital defect, present since birth, which is described as a hole, fissure or cleft in the mentioned structure. This condition has the possibility of being inherited or can appear without previous family history. The case is presented of a 51-year-old patient with bilateral asymmetric iris coloboma and farsightedness. The patient went to the doctor's office with the desire to become independent of his usual optical correction. Facorrefractive surgery of both eyes with pupilloplasty of the right eye was proposed, in which satisfactory visual results were obtained after the intervention(AU)

Combination of capsular tension ring and capsular tension segment for the management of lens coloboma >4 clock hours

Lens colobomas extending more than 4 clock hours and causing visual impairment require lens extraction along with capsular support devices with scleral fixation for adequate centration of the capsular bag and for prevention of capsular fornix aspiration with inadvertent extension of zonular dialysis intraoperatively. In this case series, we describe a technique for the management of isolated lens colobomas involving 4–5 clock hours by clear lens extraction and intraocular lens implantation using a combination of a capsular tension ring with a capsular tension segment (CTS) for the centration and stability of the capsular bag. Hoffman's corneoscleral pocket and half-bow sliding knot technique were used for scleral fixation of the CTS.