bubble_chart Overview

This disease (retinal periphlebitis) is also known as juvenile recurrent retinal membrane vitreous hemorrhage. Henry Eales first elucidated the relationship between persistent vitreous hemorrhage and retinal membrane veins in 1882 and provided a detailed report, hence it is also called Eales disease. It is more common in males aged 20 to 30. Both eyes often develop symptoms within a year, and it is prone to recurrence.

bubble_chart Etiology

The etiology of this disease is diverse. Subcutaneous node infection is recognized by most scholars as a common cause. Regarding its pathogenesis, except for a few cases where subcutaneous node bacteria directly invade through hematogenous spread or local extension, the majority are caused by a type III hypersensitivity reaction induced by subcutaneous node bacterial toxins. In such cases, there are no active subcutaneous nodes systemically or in the eyes, or only old sexually transmitted disease lesions (such as calcified foci in the lungs or hilar regions). The subcutaneous node bacterial toxin skin test is strongly positive and can induce a focal reaction, suddenly exacerbating retinal vein inflammation and vitreous hemorrhage. Septic foci, such as chronic tonsillitis, dental caries, and skin abscesses, are also relatively common causes of this disease. Additionally, there have been reports of conditions like thromboangiitis obliterans, ascariasis, syphilis, sarcoidosis, and Behcet's syndrome causing this disease.

bubble_chart Pathological Changes

Pathologically, two distinct conditions can be observed. One is granulomatous, characterized by nodular infiltration of lymphocytes around the retinal vessel walls, interspersed with epithelioid cells and giant cells. The venous walls may exhibit bulging, and the venous endothelial cells may also proliferate, potentially obstructing the lumen and causing vascular stasis in severe cases. Fissures can be seen in the retinal tissue, covered by endothelial cells, possibly representing the onset of vascular anastomosis. The other, more common type manifests as nonspecific inflammatory changes. Both types of venous inflammation may exhibit lesions attached to the myopic retinal membrane and vitreous hemorrhage. In rare cases, adjacent small stirred pulses may also be affected. Fibrin clots near the lesions, along with retinal and vitreous hemorrhages, eventually organize into membranes, forming proliferative vitreoretinopathy.

Periphlebitis of the retina is mostly primary, with lesions confined to the retina. It can also result from the spread of uveal inflammation, known as secondary retinal vasculitis.

bubble_chart Clinical Manifestations

Visual impairment depends on the size of the affected blood vessels, the amount of bleeding, and the location. If the lesion is located in the peripheral retinal vessels with minimal bleeding, patients may experience no symptoms or only floaters. When the lesion involves larger veins with significant bleeding that breaks through the internal limiting membrane into the vitreous, or even with minor bleeding located in the macula, sudden vision loss occurs, which can severely decrease to counting fingers, hand motion, or even light perception.

The fundus can only be observed when retinal hemorrhage does not enter the vitreous or when there is minimal vitreous hemorrhage. Changes in retinal veins are commonly seen in small peripheral branches, featuring dilated and tortuous vessels with kinking, accompanied by white sheathing. Nearby, flame-shaped or patchy hemorrhages are present, interspersed with grayish-white exudates with blurred borders, partially obscuring the veins and making them appear interrupted or truncated. At the lesion site, the retina shows grade I edema and opacity, and sometimes adjacent small arteries may also be affected, exhibiting white sheathing or being covered by exudates. The vitreous appears dark red and turbid due to blood. Initial episodes of vitreous hemorrhage (1–2 times) resolve relatively quickly, with most absorption occurring within weeks, leading to vision improvement. If the macula remains unaffected, vision may return to normal or near-normal.

In the early stages, the disease typically affects only one or a few peripheral venous branches but may later involve larger veins. In some cases, larger veins are affected from the outset. When the optic disc or nearby veins are involved, the disc becomes edematous and opaque, with marked retinal hemorrhage and edema, and star-shaped exudates may appear in the macula.

During active inflammation, occasional choroidal inflammatory lesions may be observed, appearing as patchy, grayish-yellow or grayish-white areas with blurred borders, located behind the retinal vessels, either near or at a distance from the inflamed retinal veins.

During the quiescent phase, retinal hemorrhage and edema resolve, leaving white sheathing on the venous walls. If venous occlusion occurred during inflammation, the affected branch may appear segmentally or entirely as a white line, with nearby neovascularization or anastomoses, along with scar-like white patches and pigment spots.

The disease is prone to recurrence. After multiple episodes, vitreous hemorrhage becomes increasingly difficult to absorb, eventually leading to organization, manifesting as fibrous membranes of varying extent and morphology. These membranes may harbor neovascularization, and the fragile walls of these new vessels are prone to rupture, further increasing the likelihood of recurrent hemorrhage. Additionally, contraction of these fibrous membranes can easily lead to tractional retinal detachment.

bubble_chart Diagnosis

This disease mostly affects both eyes. However, the severity of the eye disease and the frequency of recurrence are not consistent. When one eye has significant vitreous hemorrhage preventing fundus examination, the other eye should be fully dilated and examined for the fundus, regardless of whether symptoms are present. If one or several small venous branches in the peripheral fundus show engorgement, tortuosity, nearby hemorrhages and/or exudative lesions, or whitish sheathing or opacity of the vessel wall, these findings can serve as direct clinical diagnostic evidence.

As for the disease cause, it should be identified as thoroughly as possible to ensure targeted treatment. A detailed comprehensive physical examination and necessary laboratory tests should be performed, such as chest fluoroscopy or radiography to check for subcutaneous nodes or sarcoidosis. Examinations of the skin, oral cavity, and ENT should be conducted to check for septic sexually transmitted disease foci or superficial ulcers, along with tests for anti-streptolysin O (ASO), syphilis rapid plasma reagin (RPR), blood count, and routine urine and stool tests.

Since this disease is mostly caused by subcutaneous node hypersensitivity reactions, a subcutaneous node tuberculin test (Mantoux test) might theoretically seem necessary, but in practice, it is largely unnecessary. A positive skin test does not equate to the eye disease being caused by subcutaneous nodes; only a positive focus reaction provides sufficient grounds for diagnosis. Moreover, a single focus reaction could lead to a sudden worsening of the condition and devastating damage to vision. Therefore, if the patient has a history of subcutaneous nodes or shows systemic inactive subcutaneous node lesions (such as calcified foci in the hilar or pulmonary regions), and no other suspicious disease causes are found through examinations, this can be inferred as the cause. Even if subcutaneous nodes cannot be confirmed, a course of diagnostic anti-subcutaneous node treatment may be attempted.

bubble_chart Treatment Measures

The treatment of this disease can generally be divided into two stages. In the first stage, immediately after sudden bleeding occurs, the patient should be advised to avoid strenuous activities and rest as much as possible. Provide ample explanations to alleviate the anxiety and fear caused by the sharp decline in vision. Administer oral or injectable medications such as adrenobazone, vitamin K, C, Rutine, and calcium (the use of adrenal corticosteroids remains controversial). Alternatively, cooling blood and stopping bleeding Chinese medicinals can be taken orally (Unprocessed Rehmannia Root, lalang grass rhizome, common bletilla pseudobulb, hairyvein agrimonia herb, carbonized Platycladus orientalis, carbonized lotus rhizome node, Forsythia, Sophora Flower, etc.). After 3–4 weeks of this treatment, if there is no recurrence of bleeding, the patient can proceed to the second stage. The focus of the second stage is disease cause treatment, with the goal of preventing recurrence. If the disease cause is highly suspected to be subcutaneous node or subcutaneous node, oral isoniazid (0.3g, once daily) can be administered. Isoniazid has relatively minor side effects, allowing for prolonged use. If necessary, vitamin B₆ and zinc supplements can be added (long-term use of isoniazid may lead to zinc deficiency). If the condition is suspected to be caused by a septic sexually transmitted disease focus, potential sources such as dental caries, tonsillitis, or paranasal sinusitis should be addressed. In terms of Chinese medicinals, based on Chinese medicine pattern identification, patients often exhibit "yin deficiency with excessive fire syndrome," so the treatment primarily involves "nourishing yin and subduing yang" herbs. The Anemarrhena, Phellodendron, and Rehmannia Decoction (Anemarrhena, Phelloendron Bark, Unprocessed Rehmannia Root, Moutan Bark, Cornus, Alisma, Chinese Yam, Poria) can be used. Additional herbs may be included depending on the disease cause: for subcutaneous node conditions, add Bamboo Leaf, Solomonseal Rhizome, stemona root, Muskroot-Like Semiaquilegia Root, common bletilla pseudobulb, etc.; for septic infections, add Lonicera, Forsythia, Viola, Dandelion, vietnamese sophora root, etc.

This disease is a chronic sexually transmitted disease prone to recurrent episodes. Therefore, the second stage of treatment must be adhered to for a year or longer. If fresh bleeding suddenly recurs, revert to the first-stage treatment.

Generally, the first or second occurrence of vitreous hemorrhage can resolve relatively quickly on its own. However, after repeated episodes, absorption becomes difficult. For old vitreous hemorrhage, iodine therapy (10% potassium iodide iontophoresis) can be attempted, starting two months after the bleeding episode. Pathological estimates suggest that by this time, the venous vessel wall damage has sufficiently healed and stabilized, reducing the risk of iodine-induced rebleeding.

In recent years, some have advocated the use of laser coagulation of affected blood vessels to prevent recurrence, with successful cases reported in the literature.

bubble_chart Prognosis

The visual prognosis of this disease depends on whether the macula is damaged and whether tractional retinal detachment occurs. Cases with significant or recurrent vitreous hemorrhage generally have a poorer prognosis. However, as long as the macula is not affected, vision can recover to or near its original level once the hemorrhage is absorbed and the diseased vein stabilizes. Conversely, if the macula is involved, even minimal hemorrhage or a first-time episode can cause irreversible visual damage. Large amounts of vitreous hemorrhage, especially recurrent episodes, often do not resolve completely and eventually organize, leading to proliferative vitreoretinopathy or connective tissue contraction, which may result in tractional retinal detachment and a poor prognosis.