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Background: Anterior staphyloma is defined as a localized defect in the anterior eye wall with protrusion of the uveal tissue due to alterations in scleral thickness and structure. The causes could be congenital, trauma, or following infection. Surgical options include staphylectomy with graft (corneal/ corneoscleral), biosynthetic graft, enucleation or evisceration in a painful blind eye). Purpose: To demonstrate staphylectomy with careful preservation of ocular structures followed by optical keratoplasty in a single setting. Synopsis: A 39?year?old Asian Indian lady presented with complaints of blurring of vision and whitish opacity in both eyes since childhood, associated with progressive enlargement and protrusion of the black part of the left eye with severe pain at the time of presentation. Her clinical examination revealed leucomatous corneal opacity in both the eyes and anterior staphyloma and keratinization in the left eye. Visual acuity was counting fingers close to face in the right eye and hand movements close to face in the left eye with projection of rays being accurate in both eyes. Ultrasound B scan showed posterior staphyloma in the right eye with sub?Tenon’s fluid and increased retinochoroidal thickness. The left eye posterior segment with B scan was normal. The available surgical options were evisceration with secondary orbital implant and ocular prosthesis or staphylectomy with corneal transplantation. To provide the best option for the patient in her eye with better visual potential, staphylectomy with corneal transplantation was done. Highlights: Painful blind eye is usually dealt with evisceration. This video demonstrates the technique of staphylectomy with corneal graft and restoration of structural integrity. Evisceration is a destructive procedure that involves removal of the intraocular contents. The patient will have to deal with the hassles of prosthesis maintenance and stigma of the artificial eye
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ABSTRACT A 12-year-old boy with Donnai-Barrow syndrome diagnosed intra-uterus presented esotropia, high myopia, nystagmus, and optic disk staphyloma in an ophthalmologic examination. The patient had associated Fanconi syndrome and sensorineural hearing loss as well as facial manifestations as hypertelorism, downward slanting of palpebral fissures and low ear implantation. Magnetic resonance imaging revealed agenesis of the corpus callosum. To our knowledge, this is the first reported case associated with esotropia, nystagmus, and optic disk staphyloma.
RESUMO Paciente do sexo masculino, 12 anos, com diagnóstico intrauterino de síndrome de Donnai-Barrow, apresentava ao exame oftalmológico esotropia, alta miopia, nistagmo e estafiloma de disco óptico. Associado ao quadro, apresentava síndrome de Falconi e perda auditiva neurossensorial, além de alterações faciais, como hipertelorismo, inclinação inferior das fissuras palpebrais e implantação baixa das orelhas. Ressonância magnética revelou agenesia de corpo caloso. Ao nosso conhecimento, este é o primeiro caso relatado associando esotropia, nistagmo e estafiloma de disco óptico.
Subject(s)
Humans , Male , Child , Abnormalities, Multiple , Optic Nerve Diseases/physiopathology , Esotropia/physiopathology , Nystagmus, Pathologic/physiopathology , Myopia/physiopathology , Renal Tubular Transport, Inborn Errors , Syndrome , Acidosis, Renal Tubular , Retinal Detachment , Cryptorchidism , Fanconi Syndrome/physiopathology , Agenesis of Corpus Callosum/physiopathology , Hernias, Diaphragmatic, Congenital , Hearing Loss, Sensorineural , Hypertelorism/physiopathologyABSTRACT
Objective:To observe the morphological characteristics of high myopia (HM) paravalvular abnormalities (PVA), and the correlation between different manifestations of PVA and myopic traction maculopathy (MTM) was analyzed.Methods:A cross-sectional clinical study. A total of 42 middle-aged and elderly patients with HM and PVA diagnosed by ophthalmology examination in Department of Ophthalmology, The Second Hospital of Hebei Medical University from June to December 2021 were included in the study. There were 24 eyes in 16 males and 48 eyes in 26 females. Age was (56.71±8.10) years old. Diopter was (-13.05±3.10) D. Axial length (AL) was (28.22±1.04) mm. According to the characteristics of ultra-wide-angle optical coherence tomography images, PVA morphology was divided into paravascular microfolds (PM), paravascular cysts (PC) and paravascular lamellar holes (PLH). MTM was divided into T0-T5 grades, of which MTM≥T3 was defined as severe MTM. The state of vitreoretinal junction was observed and the state of posterior vitreous detachment (PVD) was recorded, which divided into complete PVD and partial PVD. Partial PVD was divided into macular fovea adhesions and paravascular adhesions according to the vitreoretinal adhesions. Posterior scleral staphyloma (PS) was divided into 6 types by ultra-wide-angle fundus photography. Logistic regression model was used to analyze the factors related to MTM.Results:In 72 eyes, PM, PC and PLH were 72 (100.0%, 72/72), 62 (86.1%, 62/72) and 29 (40.3%, 29/72) eyes, respectively. Among them, there were 10 (13.9%, 10/72) eyes with PM alone, 33 (45.8%, 33/72) eyes with PM and PC, and 29 (40.3%, 29/72) eyes with PM, PC and PLH, respectively. There were 42 eyes with partial PVD (58.3%, 42/72), among which the macular fovea and paravascular adhesion were 22 (52.4%, 22/42) and 24 (57.1%, 24/42) eyes, respectively. PS was present in 50 eyes (69.4%, 50/72), among which 27 (54.0%, 27/50), 21 (42.0%, 21/50), 1 (2.0%, 1/50), and 1 (2.0%, 1/50) eyes were types Ⅰ to Ⅳ, respectively. Multivariate logistic regression analysis showed that AL[odds ratio ( OR)=16.139, 95% confidence interval ( CI) 4.062-64.120, P<0.001], PS ( OR=4.212, 95% CI 1.234-14.378, P=0.022), paravascular vitreoretinal adhesion (OR=3.478, 95% CI 11.124, P=0.036) were risk factors for PM, PC and PLH. MTM was present in 58 eyes (80.6%, 58/72), among which T1 was the most common type in 19 eyes (26.4%, 19/72). Univariate logistic regression analysis showed that the occurrence of MTM was significantly correlated to PS ( OR=4.190, 95% CI 1.240-14.157, P=0.021), coexistence of PM, PC and PLH ( OR=11.323, 95% CI 1.389-92.311, P=0.023), and PS were significantly correlated. There was no correlation with PVD ( OR=1.889, 95% CI 0.580-6.150, P=0.291) or PS ( OR=2.778, 1.786; 95% CI 0.700-11.023; 0.445-7.167; P=0.146, 0.413). There was significant difference in the incidence of severe MTM between PM alone, PM combined with PC and coexistence of PM, PC and PLH ( χ2=20.943, P<0.001). Conclusions:PM is the most common and earliest manifestation of PVA in middle-aged and elderly HM patients. AL, PS and paravascular vitreoretinal adhesion are risk factors for PM, PC and PLH. The coexistence of three PVA forms may be a marker of severe MTM.
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Posterior staphyloma (PS) refers to the local abnormal bulge of the posterior wall of the eyeball, which is one of the common pathological changes in high myopia.The appearance of PS is often accompanied by changes in fundus microstructure such as sclera, choroid and Bruch membrane.The abnormality of fundus microstructure leads to the decrease of its resistance to the expansion of ocular axis, or causes the remodeling of tissue structure in the corresponding area, resulting in the change of microvascular structure, and then giving rise to abnormal blood circulation in the posterior pole.The microcirculatory changes may be followed by changes in fundus microstructure, seriously affecting the visual acuity of the affected eyes.There is still no clear conclusion on the pathogenesis of PS in the world, and there are no good preventive measures or treatment methods.This article reviewed and summarized the interaction between PS, fundus structure and microcirculation in high myopia, so as to provide a theoretical basis for exploring the pathogenesis, prevention and treatment of PS and improving the visual function of high myopia.
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Purpose: To review surgical options, techniques, and outcomes of anterior staphyloma repair done following trauma and surgery. Methods: This was a retrospective case study of patients who underwent staphyloma repair with scleral or tibial periosteal patch grafts following trauma and surgery with a minimum follow-up of 3 months postoperatively. Preoperative risk factors, choice of graft materials, surgical details, and outcomes in terms of graft uptake and tectonic integrity were analyzed. Results: Seventeen eyes of 17 patients underwent successful staphyloma repair (scleral 15, tibial periosteal two). Mean follow-up was 47.1 months (3–159 months). Postoperative intraocular pressure rise noted in four eyes was controlled medically or surgically. Three patients underwent successful repeat patch grafting (graft melt one and recurrent ectasia two). Tectonic integrity of the eyeball was restored and maintained in all patients at the final follow-up. Conclusion: Comprehensive evaluation of the risk factors, control of ocular comorbid conditions, and early and meticulous surgery can optimize results.
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Background: Anterior staphyloma is defined as a localized defect in the anterior eye wall with protrusion of the uveal tissue due to alterations in scleral thickness and structure. The causes could be congenital, trauma, or following infection. Surgical options include staphylectomy with graft (corneal/ corneoscleral), biosynthetic graft, enucleation or evisceration in a painful blind eye). Purpose: To demonstrate staphylectomy with careful preservation of ocular structures followed by optical keratoplasty in a single setting. Synopsis: A 39?year?old Asian Indian lady presented with complaints of blurring of vision and whitish opacity in both eyes since childhood, associated with progressive enlargement and protrusion of the black part of the left eye with severe pain at the time of presentation. Her clinical examination revealed leucomatous corneal opacity in both the eyes and anterior staphyloma and keratinization in the left eye. Visual acuity was counting fingers close to face in the right eye and hand movements close to face in the left eye with projection of rays being accurate in both eyes. Ultrasound B scan showed posterior staphyloma in the right eye with sub?Tenon’s fluid and increased retinochoroidal thickness. The left eye posterior segment with B scan was normal. The available surgical options were evisceration with secondary orbital implant and ocular prosthesis or staphylectomy with corneal transplantation. To provide the best option for the patient in her eye with better visual potential, staphylectomy with corneal transplantation was done. Highlights: Painful blind eye is usually dealt with evisceration. This video demonstrates the technique of staphylectomy with corneal graft and restoration of structural integrity. Evisceration is a destructive procedure that involves removal of the intraocular contents. The patient will have to deal with the hassles of prosthesis maintenance and stigma of the artificial eye.
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Purpose: To report the anatomic and visual outcomes following macular buckling in patients affected by pathological myopia?associated foveoschisis (FS) and macular detachment with or without macular hole (MH). Methods: A retrospective interventional consecutive case series wherein 25 highly myopic eyes (mean axial length 28.46 mm; range, 25–33.8 mm) of 24 patients (16 females and 8 males; mean age 54.1 years; range, 35–74 years) presenting with macular detachment associated with a posterior staphyloma (PS), who underwent macular buckling, were evaluated. Patients with absence or reduction in subretinal fluid by more than 90% during the final follow?up along with inversion of contour of staphyloma were considered to have a successful anatomical outcome and those with improvement or maintenance in visual acuity were considered to have a successful functional outcome. The mean duration of follow?up was 11.2 months. Results: At the time of initial presentation, the mean age of the 24 patients was 54.1 ± 10.28 years. Macular detachment along with FS was present in all cases, whereas full?thickness macular hole?related retinal detachment was present in nine cases. Swept?source optical coherence tomography parameters showed reduction of FS with foveal reattachment in all eyes except one at last visit. Mean axial length decreased from 28.5 mm preoperatively (range 26–33.8 mm) to 26.2 mm (range 24–29.3 mm). The mean best?corrected visual acuity changed from 1.16 log MAR to 1.096 Log MAR (P = 0.165). Visual acuity improved in 10 eyes (40%), remained stable in 11 eyes (44%) and decreased in 4 eyes (16%). Conclusion: Macular buckling is a good surgical technique with encouraging anatomic and visual outcomes in patients with myopic macular detachment associated with PS. Highly selective cases of myopic traction maculopathy can have a viable option of macular buckle surgery in stabilizing the retinal tractional changes, and thereby, vision loss.
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@#High myopia complicated with fundus lesions is one of irreversible blinding eye diseases. Posterior seleral staphyloma(PSS)is one of the most basic pathologies in a series of complications of high myopia. This article reviews the pathogenesis, examination methods, classification and treatment of PSS in high myopia by sorting out domestic and foreign literature, providing a better understanding of the prevention and control of PSS.
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The main fundus changes of pathologic myopia (PM) are posterior staphyloma (PS) and myopic maculopathy (MM), which includes myopic atrophy maculopathy (MAM), myopic tractional maculopathy (MTM), myopic neovascular maculopathy (MNM) and so on. The clinical manifestations of PM-related fundus lesions are complex, and the classification of PM has been a research hotspot in recent years. The proposal of each classification shows an increasing understanding of PM, and each classification has its advantages but also imperfections. For MM, it is recommended to refine the MTM classification based on the ATN classification and adjust it according to the internal correlation between MAM and MNM. The rapid development of modern imaging technology will promote the continuous update of the classification, and its further improvement will also help to understand the development process of PM, which has important clinical value in preventing its occurrence and progression.
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The main fundus changes of pathologic myopia (PM) are posterior staphyloma (PS) and myopic maculopathy (MM), which includes myopic atrophy maculopathy (MAM), myopic tractional maculopathy (MTM), myopic neovascular maculopathy (MNM) and so on. The clinical manifestations of PM-related fundus lesions are complex, and the classification of PM has been a research hotspot in recent years. The proposal of each classification shows an increasing understanding of PM, and each classification has its advantages but also imperfections. For MM, it is recommended to refine the MTM classification based on the ATN classification and adjust it according to the internal correlation between MAM and MNM. The rapid development of modern imaging technology will promote the continuous update of the classification, and its further improvement will also help to understand the development process of PM, which has important clinical value in preventing its occurrence and progression.
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@#<p style="text-align: justify;">Aicardi Syndrome (AS) is a rare X-linked congenital disorder traditionally characterized by a triad of dysgenesis of corpus callosum, seizures, and chorioretinal abnormalities. Patients often have severe psychomotor delay and shortened life expectancy. However, Aicardi syndrome is a clinically heterogeneous disorder. We present a case of a 14-year-old with the traditional triad of history of infantile spasm, complete agenesis of the corpus callosum, and chorioretinal abnormality but with peripapillary staphyloma and with no psychomotor delays. Based on the review of literature, this is the first reported case of AS in the Philippines, the first reported case of AS with peripapillary staphyloma, and is one of the 3 reported cases of AS with normal psychomotor development. There remains no factor that can prognosticate cognitive function in AS at present including genetic testing.</p>
Subject(s)
Aicardi Syndrome , Spasms, InfantileABSTRACT
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.
Subject(s)
Optic Nerve , Congenital Abnormalities , Case Reports , Clinical Diagnosis , Anisometropia , Coloboma , Optic Nerve HypoplasiaABSTRACT
To report case of bilaterally symmetrical choroidal coloboma within posterior staphyloma with MHRD. This is a case report of a 50year old female presented with diminished vision in both eyes. On examination, she had Bilateral High Myopia with recent onset MHRD associated with symmetrically bilateral Choroidal Coloboma within posterior staphyloma and was operated for MHRD in left eye. Presence of choroidal coloboma within posterior staphyloma is rare and with coexisting macular hole makes pathophysiology of RD challenging to understand.
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PURPOSE: To develop software to measure the shortest radius in curvature of Bruch's membrane from optical coherence tomography (OCT), and then to apply it to various types of eyes. METHODS: Macular OCT images consisting of 12 images of 9 mm radial scans were studied. The horizontal to vertical pixel ratios were changed to 1:1, and Bruch's membrane was marked automatically on each image. Software to measure the radius of Bruch's membrane curvature was developed. The shortest radius on each image was defined as r (mm) and the average r of 12 images was defined as R (mm). The reciprocal of R was multiplied by the constant, 337.5, which was defined as the posterior staphyloma (PS) index. RESULTS: The OCT images of five eyes were analyzed by the software, which could calculate the curvature of Bruch's membrane automatically. The PS indices were 12.7, 23.7, and 66.4 in eyes without refractive error (Case I), in the high myopic group without posterior staphyloma (Case II), and in the high myopic group with posterior staphyloma (Case V), respectively. The PS index gradually increased according to aging in a 37-year-old patient (Case IV) with mild staphyloma from 34.6 to 39.5 over 4 years. CONCLUSIONS: PS index is a novel parameter to reflect the level of posterior staphyloma, but further studies are needed to apply it to clinical patients.
Subject(s)
Adult , Humans , Aging , Bruch Membrane , Myopia , Radius , Refractive Errors , Tomography, Optical CoherenceABSTRACT
PURPOSE: To evaluate the efficacy of anti-vascular endothelial growth factor (VEGF) treatment of eyes with foveal serous retinal detachment (SRD) associated with inferior staphyloma and to investigate choroidal thickness changes following anti-VEGF therapy. METHODS: In this observational case series, eyes with inferior staphyloma accompanied by foveal SRD were treated with a single intravitreal anti-VEGF injection, followed by further injections as needed. Changes in height and width of subretinal fluid (SRF) and visual acuity after treatment were assessed. Choroidal thickness was measured at the subfovea, 1.5 mm superior and inferior to the fovea using enhanced depth imaging optical coherence tomography at baseline and 1 month after initial anti-VEGF therapy. RESULTS: Six eyes from six patients were included. One month after the initial injection, the mean SRF height and width had decreased significantly from 112.5 ± 40.1 to 44.5 ± 48.7 µm (p = 0.046) and from 1,401.8 ± 627.3 to 690.7 ± 634.7 µm (p = 0.028), respectively. Mean choroidal thickness at the superior point decreased from 218.7 ± 59.3 to 200.5 ± 61.0 µm (p = 0.046). SRF resolved completely in three of the six eyes (50%) with a mean of 6.8 ± 5.9 injections (range, 1 to 15). All eyes experienced at least one recurrence of exudation, at a mean interval of 4.8 months. Mean visual acuity improvement was 0.17 logarithm of the minimum angle of resolution units at a mean of 28.7 months follow-up. CONCLUSIONS: Anti-VEGF therapy resulted in an SRF decrease and modest visual improvement in eyes with foveal SRD associated with inferior staphyloma. Reduction in superior choroidal thickness appeared to contribute to the clinical improvements that were observed.
Subject(s)
Humans , Choroid , Endothelial Growth Factors , Follow-Up Studies , Recurrence , Retinal Detachment , Retinaldehyde , Subretinal Fluid , Tomography, Optical Coherence , Visual AcuityABSTRACT
Since its introduction by Charles L. Schepens, macular buckle (MB) surgery has evolved over the past 60 years. Optical coherence tomography (OCT) has given a paradigm shift to the understanding of myopic macula, thereby helping in objective evaluation of the various manifestation of traction maculopathy. Staphyloma evaluation by ultrasound, wide-field fundus photography, and MRI scans along with OCT has led to the resurgence of MB surgery in the treatment of myopic traction maculopathy (MTM). Various surgical techniques with different buckle materials are being performed with encouraging anatomical and functional success rates. This article reviews the literature to explain the current concept of MB surgery based on its evolution, different kinds of buckle materials, rationale for planning MB surgery, and different surgical techniques for the management of MTM.