spontaneous intrabulbar hemorrhage
Introduction
Introduction It is a common malignant tumor in the uvea, usually seen in 40 to 60 years old. It has nothing to do with sex or left and right eyes. It can occur in any part of the choroid, but it is common in the posterior pole of the eye. Sometimes spontaneous intraocular hemorrhage can occur, but The overall incidence probability is two parts per million. There are two ways to grow clinically: Limitations: Localized growth between the sclera and the choroidal glass membrane, flat oval. Due to the limitation of the sclera and the glass membrane, the growth is slow. If the glass membrane is broken, it will rapidly expand in the subretinal space to form a mushroom-like tumor with a large base and a thin neck. Diffuse: characterized by extensive diffuse infiltration, tumor cells infiltrated by blood vessels and lymphatic sheaths, and spread along the choroid plane, so the course of disease is longer than the limitations, and the development is slow. Except for irregular pigmentation in the fundus, there is no significant increase in the remaining.
Cause
Cause
(1) Causes of the disease:
It is still unclear and may be related to race, family and endocrine factors. Of the 3706 cases of uveal melanoma that were followed up for 17 years, 16 cases (0.4%) were pregnant women, aged around 30 years old, all of which were found in the second half of pregnancy. The relationship between the onset and pregnancy and endocrine is uncertain. Genetic factors: Singh conducted a family survey of 4,500 patients with uveal melanoma and found that there were 56 families with 56 patients with this disease and 0.6% with a family history. Other factors: sun exposure, certain viral infections, exposure to certain carcinogenic chemicals may be related to the onset of the disease.
(2) Pathogenesis:
Most tumors originate in the choroidal macrovascular layer. Regarding the origin of tumor cells, it is generally believed that there are two possibilities, one from the ciliary nerve sheath cells, namely Schwann cells; the other from the stromal melanoblast, which is commonly referred to as the pigment. Carry a small cell (chromatophore). The incidence of the former is high, accounting for about 4/5 of all uveal malignant melanoma, while the latter is only 1/5. Under the action of tumorigenic factors, malignant transformation of melanocytes in the choroid leads to the formation of tumor-like nodules.
Examine
an examination
Related inspection
Ophthalmic examination of the eye and sacral area CT examination
1. Clinical symptoms:
Choroidal melanoma, located in the periphery of the fundus, often has no symptoms at an early stage. For example, in the posterior pole, patients often complain of vision loss, visual field defects, visual distortion, blurred eyes, color vision changes, and increased long-distance diopter. Severe visual acuity can occur when the tumor enlarges and secondary retinal detachment occurs.
2. Clinical examination:
Fundus changes: localized early can see a local uplift in the fundus, a gray-white ~ blue gray ~ brown yellow ~ dark brown flat mass can be seen on the retina. Once the tumor breaks through the vitreous membrane and enters the retina, retinal detachment will occur. It is initially solid and detached. It is hemispherical, mushroom-like, with clear surrounding conditions, wrinkles around the retina, and late retinal detachment. In the early stage of diffuse, there is no obvious increase in the fundus. Since the glass membrane is mostly intact and the retina is rarely affected, there are sometimes only a few old retinal choroidal lesions in the fundus, which are easily overlooked.
The degree of retinal detachment is not necessarily parallel to the size and developmental stage of the tumor, and in principle there are no holes.
Intraocular pressure: normal or low at the beginning. As the tumor increases, the lens and iris are pushed forward by the tumor, blocking the anterior chamber angle, causing aqueous circulation disorder, elevated intraocular pressure, and secondary glaucoma.
Inflammation: Uveitis and optic neuritis can occur due to stimulation of tumor tissue toxins.
Blood vessels: In enlarged tumor heads, sometimes enlarged blood vessels in the tumor tissue can be seen faintly. Spontaneous intra-ball hemorrhage can sometimes occur.
Extraocular transfer: due to the enlargement of the tumor, it can erode the weakness of the sclera, such as blood vessels along the sclera, nerve conduits, etc., which are transferred outside the ball to the eyelids to cause eyeball protrusion, thereby invading adjacent tissues.
Whole body metastasis: mostly blood transfer, common in liver, subcutaneous tissue, central nervous system, lung, stomach, bone marrow and so on.
The whole course of disease can be divided into four stages: intraocular phase, secondary glaucoma, extraocular spread and systemic metastasis, but the four phases of evolution are not necessarily gradual. If there are cases without glaucoma, there is an extraocular spread or systemic metastasis. The clinical examinations are different from the course of the disease and the performance is different.
(1) Intraocular phase: The growth of the tumor has two forms of nodular and diffuse expansion, so the fundus also has different findings.
1 Nodular growth: The tumor begins in the choroidal and middle vascular layers. It is restricted by the sclera and the inner part by the Bruch membrane. It can only slowly spread to the periphery along the choroid plane. The elevation is not high. It is round or round yellow-yellow or even gray-black plaque, and the retina covering it has no obvious change. Thereafter, the choroid at the tumor is continuously thickened, and the degree of bulging is continuously increased, and the retina is lifted from the back. The pigment epithelial layer is partially atrophied and partially hyperplasia, which makes the retina on the surface of the tumor appear uneven and pigmented. Once the Bruch membrane and pigment epithelial layer are broken, the tumor loses its original restriction and grows rapidly under the retinal neuroepithelial layer, forming a mushroom-shaped mass with a large head, narrow neck and wide bottom. The retina then bulges, and a serous detachment occurs at the neck slope of the tumor due to fluid accumulation. The subretinal fluid can be deposited downward by gravity, and a separate detachment of the retina occurs at a distance from the tumor.
In a small number of early cases, although the tumor is small, retinal detachment has occurred at the contralateral serrated edge. Fuchs calls it the ora serrate detachment, which is considered to be diagnostically meaningful, and the mechanism by which the serrated detachment occurs is unclear.
Due to the rapid growth of tumors and the massive supply of tumor tissue due to blood supply disorders, it can induce severe intraocular inflammation or elevated intraocular pressure, and some of them contain vitreous hemorrhage. At this time, the fundus is invisible.
A small number of tumors originate from a segment of the ciliary nerve that is still in the scleral aqueduct, or the path between the origin of the tumor and the vortex vein on the sclera is very close. At this time, the tumor can spread to the eye quickly, without causing the above-mentioned fundus changes.
2 diffuse growth: this type is rare. Shields reported 3,500 cases of choroidal melanoma, 111 of which accounted for 3% of diffuse growth. Tumors develop along the choroid plane, grow slowly, and have a long course. The outer layer begins to gradually occupy the full layer of the choroid, which is a diffuse flat mass with a thickness of generally 3 to 5 mm and no more than 7 mm. The Bruch membrane is mostly intact, the retina is rarely involved, and retinal detachment is only found in individual cases. Good visual acuity is maintained when the tumor is not damaged and the macula is not damaged.
This type of tumor is prone to extraocular metastasis, which may be due to the early entry of the tumor into the suprachoroidal space, which has a wide range, and the chance of destroying the sclera or spreading along the scleral nerve and vascular pores is more. The prognosis was poor, with tumor metastasis rates of 16% and 24% at 3 and 5 years. The 5-year mortality rate after enucleation was 73%.
(2) Glaucoma period: In the early stage, the intraocular pressure is not only not high, but sometimes decreased. When the tumor continues to occupy a certain space within the ball, the increase in intraocular pressure is easy to understand. However, in some cases, the tumor volume is not large, and the intraocular pressure is significantly increased. The reason may be related to the tumor located near the vein (especially the vortex vein), which leads to venous reflux disorder, and may also be caused by tumor tissue necrosis. Or tumor cells spread in the iris corneal corner. Shields studied 2111 patients with uveal melanoma, 2% of choroidal melanoma secondary glaucoma, neovascular glaucoma is the most common, followed by lens opal septum forward.
(3) Extraocular spread: The blood vessels and nerves that diffuse through the sclera, that is, vortex veins and other channels that penetrate the sclera and provide a channel for tumor spread, are called scleral vessels. Vortex veins are the most important pathway for extraocular diffusion of choroidal melanoma. The tumor directly invades and penetrates the sclera and spreads out of the ball. If it is worn after the equator, it will grow into the sputum, which is characterized by eyeball protrusion and conjunctival edema. It can invade the sinus and sinus in a short period of time and invade the skull. It is extremely rare to spread back along the vascular nerve and catheter through the optic nerve sieve. If worn in the anterior segment of the eyeball, it is often worn at the corresponding part of the ciliary body. Extraocular diffusion of choroidal melanoma is not uncommon, and the incidence rate reported in the literature is 10% to 23%.
(4) systemic metastasis: mainly by blood transfer, liver metastasis is the earliest and most common, heart, lung followed; central nervous system is rare.
Throughout the course of the disease, death can occur as a result of systemic metastasis of the tumor. The higher the systemic metastasis rate, the higher the rate. According to statistics, 33% of patients with early stage tumor metastasis, 44% of glaucoma, and 91% of extraocular spread. If there is a recurrence after surgery, the case fatality rate is 100%.
With the continuous improvement of clinical technology, the correct diagnosis rate of choroidal melanoma is clearly improved. All clinical data should be combined, comprehensive analysis and careful judgment. In addition to detailed medical history and clinical symptoms, detailed clinical examinations, especially ophthalmoscopy can be used as a basis for diagnosis, and the following must be noted.
1. Some cases in the early stage of the tumor may have visual distortion and discoloration. The number of farsightedness in individual cases continues to increase, suggesting that the posterior choroid has a space-occupying lesion that moves the retina forward.
2. Visual field examination: The visual field defect of malignant melanoma is larger than the actual area of the tumor. The blue visual field defect is larger than the red visual field defect.
3. The corneal sensation in the vicinity of the choroidal melanoma can be reduced in the anterior segment of the eye. The adjacent sclera and iris vessels can expand. The iris can be combined with iris sputum, iris neovascularization, and pupillary pigment valgus. When the tumor is necrotic, it may be combined with iridocyclitis, anterior chamber empyema, anterior chamber pigmentation, and anterior chamber hemorrhage.
4. The diagnostic value of scleral transillumination of scleral transillumination is not reliable. For example, hemorrhage under the retinal pigment epithelial layer can also cover the light, and the tumor with a small volume or a small cystic pigment can also see through the light.
5. FFA: Comprehensive analysis of the early stage of angiography, arteriovenous phase and late stage, attention to the differentiation of choroidal hemangioma and choroidal metastasis.
6. Ultrasound exploration can detect the solid image of the tumor. It is more valuable when the refractive interstitial opacity ophthalmoscope cannot be examined, or if there is severe retinal detachment and the tumor is covered by it.
7. CT scan and magnetic resonance imaging (MRI) CT scan shows a thickening of the eye ring, protruding into the ball or outside the ball. In the enhanced examination, the tumor blood vessels are rich, the blood-retinal barrier is destroyed, and the vortex vein is involved and strengthens. However, similar to ultrasound exploration, regardless of CT scan or magnetic resonance, one is not qualitative, and the other is limited for small tumors.
8. Intraocular pressure and tumor location, size and various complications, intraocular pressure can be normal, reduced or increased. The anterior choroidal melanoma is compressed by the lens and iris, which can close the corner of the anterior chamber to produce secondary glaucoma. Tumor necrosis, macrophage phagocytic cells, pigment particles or necrotic debris, etc., are released into the anterior chamber resulting in an increase in intraocular pressure. It can also cause an increase in intraocular pressure caused by neovascular glaucoma or anterior chamber hemorrhage due to iris neovascularization.
9. Total physical examination: Because choroidal malignant melanoma is most likely to transfer to the liver via blood circulation, liver ultrasound exploration and liver scintigraphy can check for tumor metastasis. Similarly, chest X-ray or CT scans are also necessary.
Diagnosis
Differential diagnosis
Scleral congestion: refers to the expansion and congestion of the blood vessels of the conjunctiva and sclera in some cases, showing whitening and redness.
The most striking symptom of inflamed eyes is redness. For example, acute and chronic conjunctivitis, various keratitis, iridocyclitis, scleritis, glaucoma, and chemical burns of the eyes are the causes of eye congestion.
Scleral hemorrhage: refers to the conjunctival small blood vessel rupture and hemorrhage is concentrated under the conjunctiva, which is called subconjunctival hemorrhage. The subconjunctival hemorrhage has different shapes and sizes, often in the form of lamellae or lumps, and also affects the global conjunctiva into large pieces. A small amount is bright red, and the amount is purple, which occurs mostly in the cleft palate area. Over time, bleeding tends to move toward the limbus, and there is also a concentration of gravity below the conjunctiva. The bleeding is first bright red or dark red, and later turns pale yellow, and finally disappears without leaving traces.
Redness of the eyes: it is a manifestation of congestion. Eyeball congestion is divided into two types: shallow and deep. The former is bright red, called "conjunctival hyperemia"; the latter is dark red, called "ciliary congestion". Both have a "mixed congestion". The different congestion in these cases is very meaningful in the examination and diagnosis of doctors, and it is also an important basis for judging the severity of eye diseases.
The conjunctiva becomes lighter or paler: the conjunctiva becomes lighter or paler. When the anemia is different, the color of the conjunctiva becomes lighter or paler.
The conjunctiva is a soft, smooth, transparent film that is applied to the inner surface of the eyelid and the surface of the eye. In normal living organisms, the conjunctiva is rosy and its deep capillaries can be observed through it.
