Peripapillary Staphyloma Research Articles

Break in the intercalary membrane in a peripapillary staphyloma.

Break in the intercalary membrane in a peripapillary staphyloma.

Rare presentation of peripapillary staphyloma with normal visual acuity

Rare presentation of peripapillary staphyloma with normal visual acuity

Optic nerve disorders and reduced visual acuity in children. Differential diagnosis

Aims/Purpose: Low visual acuity (VA) in children requires meticulous investigation in search of possible organic causes. Once an alteration in the optic nerve (ON) has been detected, differential diagnosis is essential due to the different prognosis and management of each entity.Methods: We report the case of a 10‐year‐old black girl who went to the ophthalmologist for decreased VA in the left eye (LE) with no other history, signs or symptoms. On examination she had VA in the right eye (RE) 1 (Snellen decimal scale) and 0.1 LE. No torticollis or stereopsis, exotropia and convergence deficit in the LE. Normal fundus of eye (FE) RE; LE papilla larger and pale, centred staphylomatous excavation with central whitish area, vessels emanating from the periphery of the ON and alteration of the surrounding pigmentation, peripheral retina hypopigmented. Optical coherence tomography (OCT) of macula was normal; of the ON was very altered. Ultrasound showed protrusion of the LE “bottleneck” disc.Results: In view of the suspicion of Morning Glory Syndrome (MGS), magnetic resonance imaging (MRI) and angio‐MRI were requested to rule out alterations in the central nervous system (CNS), especially vascular malformations. After explaining the findings and poor visual prognosis we opted for occlusions in the RE, improving VA to 0.2 OI. We consulted paediatrics, genetic testing and close follow‐up, paying special attention to the retina due to the association with choroidal neovascularisation (CNV) and retinal detachment (RD).Conclusions: The characteristics of MGS can be confused with other pathologies, especially ON coloboma. Peripapillary staphyloma, ON dysplasia or aplasia, advanced glaucoma, Aicardi's disease and Papillorenal disease must be ruled out. Once diagnosed, possible systemic associations must be studied due to their potential severity and follow‐up is essential, as a high percentage will present ophthalmological complications.

Peripapillary intrachoroidal cavitation at the crossroads of peripapillary myopic changes.

To analyze the prevalence of peripapillary intra-choroidal cavitation (PICC) in eyes with gamma peripapillary atrophy (γPPA), in eyes with peripapillary staphyloma (PPS) and in those combining γPPA and PPS and to analyze border tissue discontinuity in PICC. This prospective cross-sectional non interventional study included highly myopic eyes. Non-highly myopic eyes were used as control. Radial and linear scans centered on the optic nerve head were performed using spectral-domain optical coherence tomography. Variables were analyzed along the twelve hourly optical coherence tomography sections in both eyes of each subject. A total of 667 eyes of 334 subjects were included: 229 (34.3%) highly myopic eyes and 438 (65.7%) non highly myopic eyes. The mean age of the highly myopic group was 48.99±17.81y. PICC was found in a total of 40 eyes and in 13.2% (29/220) of highly myopic eyes. PICC was found in 10.4% (40/386) of eyes with γPPA, in 20.5% (40/195) of eyes with PPS and in 22.7% (40/176) of those combining γPPA and PPS. All the eyes with PICC showed the co-existence of γPPA and PPS whereas none of the eyes presenting only one of these entities exhibited PICC. A border tissue discontinuity in the γPPA area was found in all eyes with PICC. We confirm the presence of a border tissue discontinuity in the γPPA area of all eyes with PICC. These findings suggest the involvement of mechanical factors in the pathogenesis of PICC which may contribute to PICC-related visual field defects.

Impact of peripapillary staphylomas on the vascular and structural characteristics in myopic eyes: a propensity score matching analysis

PurposeTo apply propensity score matching to evaluate the impact of peripapillary staphylomas (PPS) on vascular and structural characteristics in the myopic eyes.MethodsThis was a prospective, cross-sectional study. Forty-one control eyes and 41 eyes with PPS were analyzed. The eyes were selected using propensity score matching analysis based on the age and axial length. All subjects underwent ophthalmologic examinations for assessing vessel and structure parameters using swept-source optical coherence tomography (SS-OCT), OCT angiography, color fundus photography, and ocular biometry.ResultsAs compared with control eyes, the eyes with PPS had shallower anterior chamber depth (3.61 ± 0.24 mm vs 3.77 ± 0.24 mm, P = 0.004), higher intraocular pressure (IOP) (16.59 ± 2.88 mmHg vs 14.53 ± 2.45 mmHg, P = 0.002), and higher myopic spherical equivalent (− 11.52 ± 3.22D vs − 9.88 ± 2.20D, P = 0.009). while corneal curvature and lens thickness between the two groups were not statistically different. Compared with control eyes, increased macular deep vessel density, reduced macular choriocapillaris and radial peripapillary capillary, and thinning retinal layer, ganglion cell complex, choroidal layer as well as the superior and inferior peripapillary retinal nerve fiber layer were observed in eyes with PPS, apart from larger disc area, parapapillary atrophy area, and degree of disc rotation. Logistic regression analysis revealed that the IOP (P = 0.046), disc rotation (P = 0.003), and average peripapillary choroidal thickness (P = 0.009) were associated with the presence of PPS.ConclusionClose association of PPS with exacerbation of myopia and anatomical alterations was observed which not only affected the eye posterior segment but also the anterior segments. We further identified significant reductions in the radial peripapillary capillary and macular choroidal perfusion with the increase in macular deep retinal flow blood of myopic eyes with PPS. Higher IOP, thinner peripapillary choroidal thickness, and rotated optic disc were risk factors for the presence of PPS.

Understanding Posterior Staphyloma in Pathologic Myopia: Current Overview, New Input, and Perspectives.

Posterior staphyloma (PS) is considered the hallmark of pathologic myopia and is defined as an outpouching of a circumscribed portion of the eyeball with a radius of curvature smaller than that of the adjacent zone. Although more common in eyes with high myopia, it can affect those without it. The presence of PS is associated with a structurally and functionally worse course of high myopia that can lead to visual disability. Unfortunately, the pathogenesis of PS is unclear so far. Thus, due to the increasing prevalence of myopia which has been further exacerbated by the advent of COVID-19 lockdown, researchers are eager to elucidate the pathogenesis of pathologic myopia and that of its complications, especially PS, which will allow the development of preventive strategies. The aim of this work was to review the morphological characteristics of PS with emphasis on similarities with peripapillary staphyloma and to discuss the pathogenesis of PS considering recent suggestions about that of peripapillary staphyloma.

Abnormalities of the contralateral eye in unilateral congenital anophthalmic or blind microphthalmic patients

To describe the prevalence, distribution, and features of anterior and posterior segment abnormalities in the contralateral eye in cases unilateral of anophthalmia and blind microphthalmia. The medical records of patients with unilateral congenital anophthalmia and blind microphthalmia referred to Beijing Tongren Hospital between January 2017 and December 2021 were reviewed retrospectively to investigate the prevalence of abnormalities of the fellow eye. A total of 168 patients were included. Of these, 28 (16.7%) had fellow eye abnormalities, 4 (2.4%) with anterior segment involvement in the contralateral eye. All 28 had fundus abnormalities. The most common posterior segment finding was coloboma (7.7%), followed by optic nerve dysplasia (3.0%), familial exudative vitreoretinopathy (FEVR) or FEVR-like fundus (1.8%), morning glory disk anomaly (1.8%), and retinal nerve fiber layer defect (1.2%). High myopia fundus changes (0.6%), retinal folds (0.6%), maculopathy (0.6%), peripapillary staphyloma (0.6%), and Bergmeister optic disk (0.6%) were also noted. Patients with unilateral congenital anophthalmia or blind microphthalmia have a high probability of contralateral eye disease. The most common abnormality is coloboma.

Analysis of Peripapillary Intrachoroidal Cavitation and Myopic Peripapillary Distortions in Polar Regions by Optical Coherence Tomography.

PurposeTo compare the peripapillary polar characteristics in eyes combining peripapillary staphyloma and gamma peripapillary atrophy according to whether peripapillary intrachoroidal cavitation (PICC) was present or absent (combination-group).Patients and methodsThis prospective non-interventional cross-sectional study included 667 eyes of 334 subjects. From the polar peripapillary regions to the opening of Bruch’s membrane, the following elements and their topographic relationships were analyzed using optical coherence tomography sections: configuration of the posterior curvature of the choroid, visibility of the subarachnoid space (SAS), and suprachoroidal detachment (SCD). Chi-squared and Fisher exact tests were used for statistical analysis.ResultsThe protrusion of the posterior choroidal wall, with anterior elevation on either side, observed in both groups progressed and transformed into a wedge-shaped deformity on the side of gamma peripapillary atrophy. This wedge configuration was significantly more frequent in PICC-group than in combination-group (p = 0.004 and p < 0.001) for the upper and lower poles, respectively. SAS was more frequently observed in PICC-group than in combination-group (p = 0.002 and p < 0.001) for the upper and lower poles, respectively. SCD was detected exclusively in PICC-group (p < 0.001, both poles). The wedge-shaped configuration and the SCD were aligned antero-posteriorly with the SAS.ConclusionWe confirmed that PICC is an SCD. We observed its constant alignment with the SAS. We suggest that the tensile forces of the optic nerve sheaths during adduction promote the collapse of the scleral flange onto the SAS, leading to PICC. Further studies are warranted to confirm this hypothesis.

Optic Disc Anomalies with Excavation in the Disc (Optic Disc Pit, Morning Glory Disc Anomaly, Optic Disc Coloboma, Peripapillary Staphyloma)

Optic disc pits, optic disc coloboma, morning glory disc anomaly, and peripapillary staphyloma are the leading optic disc anomalies with excavation in the disc. These anomalies can be seen together with some syndromes and in this case, can be evaluated as part of the spectrum of the disease. However, when looking at each anomaly in terms of its pathogenesis and embryological origin, a different etiopathogenesis comes to the fore. In addition, ocular/systemic relations with anomalies may differ, and in this case, it is interpreted that each anomaly should be considered as a different disease. In this article, optical disc anomalies with excavation in the disc are discussed and current clinical findings, diagnosis, and treatment are revealed.

Occurrence of myopic maculopathy in eyes with staphylomas of various localization

To study the occurrence frequency of various forms of myopic maculopathy in scleral staphylomas of different localization in patients with pathologic myopia. The study included 105 patients with high myopia (105 eyes, axial length ≥26 mm). The main group consisted of 50 patients with myopic scleral staphylomas (50 eyes). The comparison group - 55 patients (55 eyes) without scleral staphylomas. In the main group, myopic maculopathy was detected in 82% of eyes, it was absent in 96% of eyes in the comparison group. In the main group there were: staphyloma of the posterior pole of the eye with involvement of the optic nerve head (ONH) - 44% of eyes; macular staphyloma - 28% of eyes; peripapillary staphyloma - 12% of eyes; staphyloma below the ONH - 16% of eyes. Atrophic, neovascular and traction maculopathy was revealed in 22 eyes with staphylomas of the posterior pole of the eye. Dome-shaped changes in the macula were revealed in 17 patients (19 eyes) of the main group (15 eyes with staphylomas of the posterior pole of the eye, 4 eyes with macular staphylomas). The highest average value of axial length (30.94±0.81 mm) was noted in eyes with macular staphylomas, and the lowest (28.58±1.62 mm) - with staphylomas near the ONH. The presence of myopic maculopathy is associated with the presence of staphylomas with the odds ratio of 120.7 (95%, confidence interval 24.7-589.3). Staphyloma of the posterior pole of the eye and macular staphyloma were associated with the most severe forms of myopic maculopathy and low visual acuity.

Aicardi Syndrome with Peripapillary Staphyloma: A Case Report and Review of Literature

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.

Huge peripapillary staphyloma with craniofacial clefts: A case report.

We reported the occurrence of a congenital unilateral huge peripapillary staphyloma in association with craniofacial clefts for the first time. A 1-year-old boy presented with a large defect on his left eyelid, a wide oblique columella nasi and an atypical wedge-shaped extension of the unilateral anterior hairline. Magnetic resonance imaging (MRI) examinations revealed there were cracks on his nasal septum, palate, and superior alveolar midline. Moreover, we surprisingly uncovered a gourd-shaped eyeball with the compressed optic nerve on the right side, while the right eye seemed normal from appearance. Under anaesthesia, fundus examination of the right eye showed a 15 mm-deep excavation surrounding the optic disc with defective choroid and dysplastic optic papilla. We reconstructed the left eyelid of the patient to protect his cornea and would make other solutions according to the results of follow-up. Peripapillary staphyloma and craniofacial clefts are two dissimilar rare congenital anomalies. In this patient, we firstly observed the co-existence of the two defects, which may provide the experience to the diagnosis and treatment of peripapillary staphyloma and craniofacial clefts. This case also gives us the pathogenic inspiration for further studies of peripapillary staphyloma and craniofacial clefts.

Retinal Detachment associated with Optic Nerve Anomalies: Clinical Features, Pathogenesis, Management

Congenital anomalies of the optic nerve head are a group of structural malformations of the optic nerve head and surrounding tissues, which may cause visual impairment. Retinal detachment commonly occurs in association with congenital anomalies of the optic disc, including morning glory disc anomaly, optic disc pit, optic disc coloboma, peripapillary staphyloma, and Aicardi syndrome. Notably, visual impairment and retinal detachment may not be the only problem in these patients, some of these entities will be related to neurologic and systemic features, which sometimes may be life-threatening. This review presents an overview of the clinical features of these optic disk anomalies and current therapeutic approaches for the treatment of retinal detachment associated with them.

Editors' note: Teaching NeuroImages: Morning glory disc anomaly

In the article “Teaching NeuroImages: Morning glory disc anomaly,” Dr. Poillon et al. presented a case of a 7-month-old girl with strabismus who was diagnosed with a morning glory disc anomaly (MGDA) in the right eye and found to have a glial tuft at the optic nerve insertion. In response, Drs. Karimi and Sanjari argue that the image depicted appears to be a peripapillary staphyloma and not MGDA. They note the importance of making this distinction because vision can be preserved aside from an enlarged blind spot in the setting of a peripapillary staphyloma. Replying to these comments, Drs. Lecler and Poillon acknowledge the importance of differentiating between the 2 conditions but highlight 2 important features that supported their diagnosis of MGDA—the radial aspect of the retinal vessels on fundoscopy and the presence of abnormal tissue at the optic nerve insertion on ultrasound and MRI—neither of which would be expected with a peripapillary staphyloma. They note that their diagnosis was confirmed by the French National Center of Reference for MGDA and report that a retinal detachment occurred in the first year of follow-up, further supporting the diagnosis. In the article “Teaching NeuroImages: Morning glory disc anomaly,” Dr. Poillon et al. presented a case of a 7-month-old girl with strabismus who was diagnosed with a morning glory disc anomaly (MGDA) in the right eye and found to have a glial tuft at the optic nerve insertion. In response, Drs. Karimi and Sanjari argue that the image depicted appears to be a peripapillary staphyloma and not MGDA. They note the importance of making this distinction because vision can be preserved aside from an enlarged blind spot in the setting of a peripapillary staphyloma. Replying to these comments, Drs. Lecler and Poillon acknowledge the importance of differentiating between the 2 conditions but highlight 2 important features that supported their diagnosis of MGDA—the radial aspect of the retinal vessels on fundoscopy and the presence of abnormal tissue at the optic nerve insertion on ultrasound and MRI—neither of which would be expected with a peripapillary staphyloma. They note that their diagnosis was confirmed by the French National Center of Reference for MGDA and report that a retinal detachment occurred in the first year of follow-up, further supporting the diagnosis.

Diabetic Papillopathy on Peripapillary Staphyloma in a 50-year-old Korean Patient

Diabetic Papillopathy on Peripapillary Staphyloma in a 50-year-old Korean Patient

Optic disk drusen associated with congenital peripapillary staphyloma

We described a rare association between peripapillary staphyloma and optic disk drusen in a woman with 47 years old and normal vision. We described a rare association between peripapillary staphyloma and optic disk drusen in a woman with 47 years old and normal vision.

Bilateral optic disc pathologies as an accompanying feature of comitant strabismus in children.

To describe and report accompanying bilateral optic disc pathologies in children with comitant strabismus. Twenty-eight patients (16 female, 12 male) aged between 1 and 12years who had comitant strabismus and bilateral optic disc pathologies were included in the study. Visual acuity, refractive errors, amount of deviation and types of optic disc pathologies were all recorded. Each patient underwent complete neurological and ophthalmological examination. Of the 28 patients, 14 (50.0%) had esotropia and 14 (50.0%) had exotropia. The mean age was 4.18±3.03years. The mean deviation angle was 35.30±13.26 prism diopters (PD) (20-70 PD). Optic atrophy as being the most common pathology was found in nine (32.1%) patients. Six (21.4%) patients had temporal disc pallor, six (21.4%) patients had opticnerve hypoplasia, and seven (25%) patients had other optic disc anomalies (tilted disc, megalodisc, disc coloboma, peripapillary staphyloma). Optic disc pathologies were found to be isolated in 12 patients. Nine of 13 patients with congenital optic disc pathologies had esotropia, whereas 10 of 15 patients with optic atrophy or optic disc pallor had exotropia. Comitant strabismus in children can be associated with congenital or acquired optic disc pathologies. It is worthy of note that esotropia was more common in patients with congenital optic disc pathologies, whereas exotropia was more frequent in patients with optic atrophy or optic disc pallor. The findings ofthe present study show that complete ophthalmological examination including fundus evaluation should be carried out in all patients with strabismus even though the cause of ocular misalignment is obvious.

Characteristics of Peripapillary Staphylomas Associated With High Myopia Determined by Swept-Source Optical Coherence Tomography

To analyze the morphologic features of peripapillary staphylomas associated with high myopia by swept-source optical coherence tomography (OCT). Validity analysis. Highly myopic patients (refractive error >8.0 diopters or axial length >26.5mm) with a peripapillary staphyloma who had undergone swept-source OCT were studied. The non-highly myopic fellow eyes in patients with unilateral high myopia were also studied. The presence of a peripapillary staphyloma was determined by stereoscopic ophthalmoscopic examinations. The OCT features of the peripapillary staphylomas and other peripapillary lesions were evaluated. Twenty-five eyes of 16 patients were studied. Twenty-two eyes were highly myopic and the remaining 3 eyes of the patients with unilateral high myopia were non-highly myopic. A peripapillary staphyloma was seen in the swept-source OCT images as a gently sloping excavation around the optic disc in all 25 eyes. The posterior sclera in the area of a peripapillary staphyloma was observed to bow posteriorly, with the local curvature steeper than the curvature of the adjacent scleral regions. The sclera at the edge of a peripapillary staphyloma appeared to be elevated inward from the surrounding sclera, and the choroid at the staphyloma edge appeared to be compressed and thinned. A peripapillary intrachoroidal cavitation was a frequent complication and was found in 13 eyes with a peripapillary staphyloma (52.5%). Swept-source OCT can detect the slight changes of the sclera and choroid in the area of a peripapillary staphyloma, and we recommend its use indetermining the presence of a peripapillary staphyloma.

Morning Glory Syndrome and the Role of 3D OCT in Its Management

Congenital optic disc anomalies that can significantly impair visual function include excavation of the optic disc and optic nerve hypoplasia. Optic disc excavations include Optic disc coloboma, Morning Glory Syndrome (MGS), Peripapillary staphyloma and Optic disc pit. We describe A case of a 70 year old lady with MGS, and the role of 3D Optical coherence tomography (OCT) in the management of MGS.

Evaluation of congenital excavated optic disc anomalies with spectral-domain and swept-source optical coherence tomography.

To investigate the anatomic characteristics of congenital excavated optic disc anomalies by using fundus photography, spectral-domain optical coherence tomography (SD-OCT), and swept-source optical coherence tomography (SS-OCT). Fourteen eyes from 13 patients with congenital excavated optic disc anomalies underwent a complete ophthalmologic examination that included best-corrected visual acuity evaluation, fundus photography, and SD-OCT. SS-OCT was performed in cases of peripapillary staphyloma cases in which the excavation depth could not be detected with SD-OCT. On the basis of the funduscopic and OCT findings, patients were classified as morning glory syndrome, optic disc coloboma, or peripapillary staphyloma. Seven eyes with morning glory syndrome were characterized by the presence of the preretinal tractional membrane in front of the excavated optic disc and could be divided into two groups: three eyes without retinal excavation, and four eyes with retinal excavation. Four eyes with optic disc coloboma showed inferiorly decentered scleral excavations with shallow optic disc excavation detectable by SD-OCT. Three eyes with peripapillary staphyloma showed deep excavation, the depth of which could not be detected by SD-OCT. SS-OCT and enhanced depth imaging SD-OCT images focused on the bottom revealed membranous structure at the bottom of the excavation in two cases with peripapillary staphyloma. SD-OCT and SS-OCT are helpful for differential diagnosis of excavated optic disc anomalies. Morning glory syndrome, optic disc coloboma, and peripapillary staphyloma were respectively characterized by the presence of a preretinal tractional membrane, inferiorly decentered excavation, and an excavation deeper than that observed in morning glory syndrome and optic disc coloboma.