Introduction

The first report of a choroidal rupture goes back to 1854, when von Graefe described it for the first time. It involves a tear of the retinal pigment epithelium (RPE), Bruch’s membrane, and choroidal tissue as a consequence of trauma to the eye. Choroidal rupture caused by blunt trauma to the eye may occur at the site of the impact in the case of direct ruptures, or as a consequence of a countercoup, just opposite to it, in indirect tears.

According to published series, up to 80% of choroidal ruptures are classified as indirect, and the vast majority of these are caused by blunt trauma. Most of them take place temporal to the optic disc and usually affect the macular area and the fovea, showing a crescent shape. On the other hand, open-globe injuries appear to be the cause of choroidal ruptures in up to 28% of the cases. In these cases lesions are more typically located anteriorly, parallel to the ora serrata .

There are several theories about the mechanism that may cause an indirect choroidal break. The most accepted suggests that antero-posterior compressions and deformations cause an equatorial expansion of the globe. This force is conducted to the optic nerve and radiated from the peripapillary uveo-scleral tether. Bruch’s membrane tissue has properties that make it the most susceptible target to be damaged in indirect traumas. While retinal tissue is more elastic than Bruch’s membrane, the sclera shows more resistance to tension thanks to its stiffness. This is why they are both usually spared.

Diseases affecting the consistency of Bruch’s membrane are more susceptible to choroidal ruptures after trauma, like angioid streaks, as will be discussed later. Older theories relating the tears to vascular necrosis as a consequence of the trauma are not as supported as the previous one.

Ocular Manifestations and Clinical Course

The most common clinical presentation is a subretinal hemorrhage that may hide the actual tear in the early phases ( Fig. 19.1.1 ) and, in rare cases, there may be multiple breaks. After blood is resorbed, the choroidal rupture becomes visible taking a crescent shape, concentric to the optic nerve head ( Fig. 19.1.2 ). Although it has not been found to be a predictive factor by some authors, initial visual acuity (VA) ranges from 20/20 to light perception, depending on the affected area.

Fundus photograph showing choroidal rupture with subretinal hemorrhage.

Fundus photograph shows peripapillary choroidal rupture (A) and corresponding SD-OCT (B).

Visual prognosis is marked by location, presence of intraocular hemorrhage, retinal involvement (retinal tears and detachment, retinal edema), and development of choroidal neovascularization (CNV).

Secretan et al. published a review of 79 cases reaching several conclusions: They stated that ruptures located less than 200 µm from the foveal avascular zone (FAZ) and between 200 and 1500 µm from the FAZ were significantly more likely to develop CNV than those located farther than 1500 µm from the FAZ. They also found breaks larger than 4000 µm to predispose for the development of CNV, wherever they were located; 82% of their patients developed CNV during the first year of follow-up.

Treatment

Although traumatic CNV usually regress spontaneously, several treatments have been proposed through the years, from photodynamic therapy (PDT), going through surgical extraction and laser photocoagulation until the age of anti-vascular endothelial growth factor (VEGF) drugs ( Fig. 19.1.3 ).

Fundus photograph (A) shows choroidal rupture with suspicious CNV secondary to choroidal rupture. Fundus fluorescein angiography shows early hyperfluorescence (B) with late leakage (D). SD-OCT (E) suggests presence of CNV with subretinal fluid. After anti-VEGF therapy OCT shows scarred CNV with resolution of subretinal fluid.

Both bevacizumab and ranibizumab have shown good results for the treatment of CNV related to choroidal ruptures. Mean number of injections goes from 2.5 injections in 52 months up to monthly injections depending on the treatment regimen established. Rosina et al. state that patients free from CNV reactivation for about a year show a smaller recurrence rate. Furthermore, each recurrence increases the risk for further reactivation; therefore, frequent controls during follow-up are mandatory. Some authors report promising results, stabilizing or improving VA in 62.9% of the eyes treated with ranibizumab.

Introduction

Angioid streaks is the term used to describe visible irregular lines that spread radially form the optic nerve, with a variable width which funduscopically seem red-brown. It is their similarity to retinal vessels that is responsible for their name, and were first described in 1889 by Doyne during the exploration of traumatized eyes.

Angioid streaks are the ophthalmoscopic manifestations of calcific degeneration and irregular breaks in Bruch’s membrane together with a consequent atrophy of the RPE above them. Calcium deposits make Bruch’s membrane more brittle and less resistant to trauma (external, muscle traction, pressure on the eye), being responsible for the choroidal ruptures involved in these patients. Latest theories demonstrate an absence of a systemic antimineralization factor that leads to the calcification Bruch’s membrane and other connective tissues that are rich in elastic fibers. Although they are not necessarily present in every affected patient, systemic associations of angioid streaks include pseudoxanthoma elasticum, Ehlers–Danlos and Marfan syndromes, Paget’s disease, acromegaly and several blood dyscrasias like thalassemia, spherocytosis, and sickle-cell anemia. Other less common conditions associated to this pathology are listed in Table 19.2.1 .

Patients showing this disease are usually asymptomatic until complications arise. The most frequent of them is the development of traumatic CNV, and prognosis depends on the macular involvement.

Ocular Manifestations and Clinical Course

As described earlier, angioid streaks are funduscopically diagnosed and identified as irregular subretinal breaks or dehiscences that radiate from the optic nerve whose width is reported to vary from 50 to 500 μm. They may remain stable or increase their size, but they do not regress.

Similar to choroidal ruptures, patients stay asymptomatic as long as the macular area is not involved. Patients suffering from angioid streaks are very susceptible to choroidal breaks after the mildest of traumas, and 72–86% of cases present with CNV. These patients usually evolve to legal blindness as more than 70% of the cases are bilateral.

The early phase is characterized by metamorphopsia and/or blurred vision (ranging from 20/20 to light perception, depending on the location of the break) after mild trauma. The most common finding at this stage is a subretinal hemorrhage involving the macular area, which will later reveal CNV after a break in Bruch’s membrane at the affected spot. Fundus autofluorescence, fluorescein angiography (FFA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT) may help identify areas of angioid streaks or their complications ( Fig. 19.2.1 ).

Fundus photograph (A) and autofluorescence (B) images of an eye with angioid streaks and scarred choroidal neovascular membrane which is confirmed on SS-OCT scans (horizontal (C) and vertical (D)).

Treatment

There is no effective treatment to prevent the development of angioid streaks. Spaide et al. suggested the possibility that pyrophosphates could be used to treat pseudoxanthoma elasticum looking to halt abnormal mineralization of tissue including that seen in Bruch’s membrane, and so prevent the development of breaks and angioid streaks. Current therapeutic approaches are focused on the management of complications.

PDT, surgical extraction, laser photocoagulation, macular translocation, and transpupillary thermotherapy (TTT) were used with variable results and no significant long-term benefits. On the other hand, anti-VEGF intravitreal injections have surged as a new option during the last years as several studies have shown their potential benefits for the treatment of angioid streak-related CNV and seem to be able to stop the progression of CNV with significant improvement in visual outcomes. Tilleul et al. state that this kind of CNV seems to be more similar to myopia-related CNV than age-related macular degeneration (AMD), needing fewer injections than wet AMD. This group also highlights the importance of monitoring patients suffering from complications in one eye so as to be able to identify and treat the fellow eye in case of involvement as soon as possible and so prevent foveal scarring. Larger series with longer follow-up periods will be necessary to corroborate these reports of sustained visual improvement.

Introduction

Staphyloma was first described by Antonio Scarpa in 1801 when he noticed pronounced outward bulges in the posterior portion of two eyes from a cadaver. In 1977, Curtin created a classification of staphyloma into 10 different types according to its location and size. Ohno-Matsui renamed the staphyloma into six types according to their location and distribution, which include wide macular, narrow macular, peripapillary, nasal, inferior, and other staphylomas.

Since Scarpa’s first description of staphyloma in 1801, he didn’t make the link to myopia. It is until 1856, when Arlt established a connection between a posterior staphyloma and myopic refraction. Therefore, the presence of a posterior staphyloma and high myopia were thought to be synonymous. However, staphyloma can exit in eyes without long axial length or high refractive error, such as tilted disc syndrome or age-related staphyloma.

Pathologic Myopia

Posterior staphyloma in pathologic myopia may present different types of myopic maculopathy, such as chorioretinal atrophy, Fuchs spots, patchy chorioretinal atrophy, and myopic traction maculopathy ( Fig. 19.3.1 ). Spectral-domain OCT (SD-OCT) is a very powerful tool to identify pathologic features in the retina and choroid, which may not be easy to observe using indirect ophthalmoscopy. In general, SD-OCT show decreased choroidal thickness with or without intraretinal lesion, such as macula retinoschisis ( Fig. 19.3.1A ), decreased refractivity of ellipsoid zone ( Fig. 19.3.1B ), patchy chorioretinal atrophy ( Fig. 19.3.1C ), CNV ( Fig. 19.3.1D ), macula hole, and retinal detachment. Wang et al. has reported that choroidal thickness is a better indicator of the severity of myopic maculopathy than axial length or refractive error. Further study from the same group showed that choroidal thickness has the strongest association with lacquer crack formation vs axial length and refractive error.

Fundus photography and SD-OCT of posterior staphyloma in pathologic myopia. SD-OCT shows decreased choroidal thickness and macula retinoschisis ( Fig. 19.3.1A , right); decreased refractivity of ellipsoid zone ( Fig. 19.3.1B ), patchy chorioretinal atrophy ( Fig. 19.3.1C ), and CNV ( Fig. 19.3.1D ).

Peripapillary Staphyloma

Peripapillary staphyloma has been classified as type III staphyloma by Curtin. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone ( Fig. 19.3.2A and B ).

Fundus photography and SD-OCT of peripapillary staphyloma. SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone (A and B).

Tilted Disc Syndrome

Fuchs first stressed the difference between the inferior location of the congenital crescent (Fuchs’ coloboma) and the temporal location of the acquired myopic type. In tilted disc syndrome, the retinal vessels emerge from the optic nerve in a nasal direction before turning toward to the temporal side of the fundus. Spaide has classified tilted disc syndrome as type V of inferior staphyloma. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone ( Fig. 19.3.3A and B ).

Fundus photography and SD-OCT of tilted disc syndrome. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone (A and B, left); A and B, right are fellows, eyes without titled disc syndrome.

Age-Related Staphyloma

Recently, Wang et al. reported 16 eyes from 10 elderly patients (mean age 70 years old) with posterior staphyloma resembling pathologic myopic maculopathy, but without an axial length longer than 26.5 mm ( Fig. 19.3.4 ). They found this disease is characterized by decreasing choroidal thickness, decreased ellipsoid zone reflectivity seen in SD-OCT scans, and lateral protrusions seen in three-dimensional (3D) magnetic resonance imaging (MRI) scans. However, it did not fit the criteria for pathologic myopia of excessive axial length or refractive error values. They assumed that this type of staphyloma might cause visual impairment in elderly patients and might be associated with the aging process, which is different from the axial elongation staphyloma in highly myopic eyes.

Fundus photography and SD-OCT of age-related staphyloma. SD-OCT shows decreased choroidal thickness and decreased refractivity of ellipsoid zone (A and B, left), macula retinoschisis (C, left), macular retinoschisis and foveal detachment (D), and CNV (E).

Introduction

Staphyloma was first described by Antonio Scarpa in 1801 when he noticed pronounced outward bulges in the posterior portion of two eyes from a cadaver. In 1977, Curtin created a classification of staphyloma into 10 different types according to its location and size. Ohno-Matsui renamed the staphyloma into six types according to their location and distribution, which include wide macular, narrow macular, peripapillary, nasal, inferior, and other staphylomas.

Since Scarpa’s first description of staphyloma in 1801, he didn’t make the link to myopia. It is until 1856, when Arlt established a connection between a posterior staphyloma and myopic refraction. Therefore, the presence of a posterior staphyloma and high myopia were thought to be synonymous. However, staphyloma can exit in eyes without long axial length or high refractive error, such as tilted disc syndrome or age-related staphyloma.

Pathologic Myopia

Posterior staphyloma in pathologic myopia may present different types of myopic maculopathy, such as chorioretinal atrophy, Fuchs spots, patchy chorioretinal atrophy, and myopic traction maculopathy ( Fig. 19.3.1 ). Spectral-domain OCT (SD-OCT) is a very powerful tool to identify pathologic features in the retina and choroid, which may not be easy to observe using indirect ophthalmoscopy. In general, SD-OCT show decreased choroidal thickness with or without intraretinal lesion, such as macula retinoschisis ( Fig. 19.3.1A ), decreased refractivity of ellipsoid zone ( Fig. 19.3.1B ), patchy chorioretinal atrophy ( Fig. 19.3.1C ), CNV ( Fig. 19.3.1D ), macula hole, and retinal detachment. Wang et al. has reported that choroidal thickness is a better indicator of the severity of myopic maculopathy than axial length or refractive error. Further study from the same group showed that choroidal thickness has the strongest association with lacquer crack formation vs axial length and refractive error.

Fundus photography and SD-OCT of posterior staphyloma in pathologic myopia. SD-OCT shows decreased choroidal thickness and macula retinoschisis ( Fig. 19.3.1A , right); decreased refractivity of ellipsoid zone ( Fig. 19.3.1B ), patchy chorioretinal atrophy ( Fig. 19.3.1C ), and CNV ( Fig. 19.3.1D ).

Peripapillary Staphyloma

Peripapillary staphyloma has been classified as type III staphyloma by Curtin. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone ( Fig. 19.3.2A and B ).

Fundus photography and SD-OCT of peripapillary staphyloma. SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone (A and B).

Tilted Disc Syndrome

Fuchs first stressed the difference between the inferior location of the congenital crescent (Fuchs’ coloboma) and the temporal location of the acquired myopic type. In tilted disc syndrome, the retinal vessels emerge from the optic nerve in a nasal direction before turning toward to the temporal side of the fundus. Spaide has classified tilted disc syndrome as type V of inferior staphyloma. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone ( Fig. 19.3.3A and B ).

Fundus photography and SD-OCT of tilted disc syndrome. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone (A and B, left); A and B, right are fellows, eyes without titled disc syndrome.

Age-Related Staphyloma

Recently, Wang et al. reported 16 eyes from 10 elderly patients (mean age 70 years old) with posterior staphyloma resembling pathologic myopic maculopathy, but without an axial length longer than 26.5 mm ( Fig. 19.3.4 ). They found this disease is characterized by decreasing choroidal thickness, decreased ellipsoid zone reflectivity seen in SD-OCT scans, and lateral protrusions seen in three-dimensional (3D) magnetic resonance imaging (MRI) scans. However, it did not fit the criteria for pathologic myopia of excessive axial length or refractive error values. They assumed that this type of staphyloma might cause visual impairment in elderly patients and might be associated with the aging process, which is different from the axial elongation staphyloma in highly myopic eyes.

Fundus photography and SD-OCT of age-related staphyloma. SD-OCT shows decreased choroidal thickness and decreased refractivity of ellipsoid zone (A and B, left), macula retinoschisis (C, left), macular retinoschisis and foveal detachment (D), and CNV (E).