Introduction:
One of the most common eye disorders is myopia. The clinical features of myopia vary on its clinicopathological entity. C bleeding is one of the most common findings among the many fundus lesions. Among several types of bleeding seen in such cases, vitreous hemorrhage is the most common. It is generally categorized by the presence of extravasation within the vitreous cavity. But in certain cases, massive bleeding can be seen overlying the optic disc extending into the surrounding papillary (under the retina) space. Such hemorrhage is intrapapillary hemorrhage with adjacent peripapillary subretinal hemorrhage (IHAPSH).
The clinical entity of papillary and peripapillary hemorrhages was first reported in 1975. This clinical condition is rare and often heals spontaneously. But recent studies have shown its strong correlation with choroidal neovascularization. It is important to understand the diagnostic and clinicopathological features of peripapillary and papillary hemorrhage.
Who Are Affected?
Intrapapillary and peripapillary hemorrhage is a rare clinical entity. Only 4.5 percent of people with pathological myopia show papillary and peripapillary hemorrhage. In 90 percent of cases, peripapillary hemorrhage and intrapapillary hemorrhage occur. Though it was first seen in western people, most of these cases are in the east Asian population. Mostly it is seen in young individuals, and women are more affected than men.
What Is the Cause?
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The exact mechanism of papillary and peripapillary hemorrhages is not known. But it is strongly associated with tilted discs in pathological myopia. In pathological myopia, peripapillary scleral expansion occurs, as a result of which temporal flattening of the optic disc is observed. The optic disc's oval appearance en-face (front face) is known as a tilted disc. The tilted disc's high superior and nasal margins pull out the retinal and choroidal tissue in and around the elevated edge. As a result, border tissues and optic nerve overhang in the superonasal and nasal region of the optic disc.
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This change in the position generates a vitreopapillary traction in the prelaminar blood vessels. The prelaminar blood vessels in this region are rich with arterial blood supply from the peripapillary choroidal arteries and posterior short ciliary arteries. The blood drains into the central retinal vein with minor contributions to the peripapillary choroidal veins.
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Increased vitreopapillary traction and other factors lead to sudden or spontaneous bleeding from the blood-rich prelaminar vessels. Other factors which may aid this event are crowded optic discs, the hemodynamic effects of the Valsalva maneuver, bleeding disorders, the use of atropine, and Terson's syndrome.
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The development of scalar creeps in pathological myopia is also associated with papillary and peripapillary hemorrhages. Increased intraocular pressure and extraocular muscle contractions lead to stretching and thinning of the sclera. The blood vessels which are already constricted and atrophied are stressed further. The stress causes a rupture in the blood vessels and leads to hemorrhage.
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Scleral creeps cause linear breakages in Bruch's membrane. The liner breakage is extended, and the elevated vascular endothelial growth factor (VEGF) levels in the aqueous humor are responsible for forming neovascular membranes. These newly formed blood vessels are responsible for subretinal hemorrhage.
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In the elderly, hyaline-like calcified nodules are seen within the optic nerve head. This small proteinaceous structure known as optic disc drusen may also cause elevated optic disc. This elevation leads to ischemic optic neuropathy and retinal vein occlusion. The occlusion results in the development of papillary subretinal hemorrhage.
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Another factor responsible for forming papillary and interpupillary hemorrhage is the detachment of the posterior vitreous membrane. Reduction in cortical adhesion results in this detachment. Unlike older individuals, posterior vitreous detachments seen in young patients are partial, extending only up to the nasal margin. Posterior vitreous detachment starts superiorly from the macular legion. It goes nasally to the head of the optic nerve to the inferonasal margin. Because of the detachment process, shearing force is generated in the superior hemidisc area. As a result, tearing of the superficial vessels occurs, and as the force is transmitted through the sub-retinal layers of the eye, causing subretinal bleeding.
What Are the Signs and Symptoms?
In most cases, papillary and interpupillary hemorrhages are not reported because of mild symptoms or spontaneous healing. But documented symptoms are shadow floating in the visual field, mild vision loss, a blind spot in the visual field, and difficulty reading and writing.
Eye examination reveals:
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The titled appearance of the optic disc with a blurred disc margin and the presence of a small patch of nasal and subretinal hemorrhage.
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The peripapillary glial ring can be observed in case of total posterior vitreous detachment.
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Subretinal bleeding is present at the nasal side of the peripapillary region. This region appeared deformed with a non-hemorrhagic detachment of the adjacent retina.
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The nasal region of the optic nerve appears to be obliquely inserted with an elevated and blurring appearance.
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Hemorrhagic spots can be seen at the margin of the optic disc, and crescent-shaped blood can be seen in the posterior pole.
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Microaneurysms and capillary telangiectasia can be observed in blood vessels.
What Are the Diagnostic Methods?
The diagnostic test for papillary and peripapillary hemorrhage is as follows.
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Fundus Fluorescein Angiography - In this method, fluorescein dye is injected through the vein, and an image of the fundus is taken with the help of a camera and barrier filters. The barrier filter helps to capture the light emitted from the excited fluorescein.
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Ocular Ultrasound - Ocular ultrasound is of two types.
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Ultrasound or amplitude scan - Helps determine the distance between two structures to measure ocular axial length.
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Ultrasound or brightness scan - Most commonly used method to determine the size and echotexture of a lesion.
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Optical Coherence Tomography Angiography (OCTA) - This is a non-invasive method for studying laser light deflection from red blood cells. As a result, underlying microvasculature can be studied along with the flow and perfusion rates.
What Is the Treatment?
Papillary and peripapillary hemorrhages usually do not need any treatment. Patients are often cured without treatment with good visual recovery. Diagnosis of the underlying cause of pathological myopia is a must to cure it.
Conclusion
Papillary and peripapillary hemorrhages are rare entities. The clinical features and pathogenesis of these hemorrhages are distinctive. The cause of such hemorrhages must be identified, although most cases are cured spontaneously. The identification will help us further understand its correlation with other ocular disorders.
