intraorbital cavernous hemangioma
Introduction
Introduction to cavernous hemangioma in the orbit Cavernous hemangioma (cavernoushemangioma) is named for the cavernous vascular sinus cavity in the tumor. It is the most common tumor in the adult, which accounts for 10% to 23% of the tumor in the sputum. Women are more common than men. The disease is a congenital hamartoma. The tumor consists of sinusoidal and fibrous spaces of varying sizes. It has a complete capsule. Due to the slow growth of the tumor, it is often found after puberty due to eyeball protrusion. basic knowledge The proportion of illness: the incidence rate is about 0.005%-0.007% Susceptible people: adults are most common Mode of infection: non-infectious Complications: eyeballs, eye movement disorders
Cause
Causes of cavernous hemangioma in the orbit
(1) Causes of the disease
It is not clear. In the past, some scholars believed that this tumor was transformed from capillary hemangioma. The intraluminal pressure was increased and it was highly dilated, forming vascular sinus. However, clinical manifestations and pathological tissues did not seem to support this statement. Cavernous hemangioma There are smooth muscle cells in the blood vessel wall. According to the degree of vascular development, it is more mature than capillaries. It is a tumor formed by various cellular components. Like capillary hemangioma, it is also a hamartoma.
(two) pathogenesis
Not very clear.
Prevention
Prevention of cavernous hemangioma in the orbit
Mainly pay attention to life and develop good habits.
Complication
Complications of cavernous hemangioma in the orbit Complications, eyeballs, eye movement disorders
Eyeballs and eye movement disorders are more common.
Symptom
Symptoms of cavernous hemangioma in the orbital eye Common symptoms Visually impaired eyeballs can not be free of keratitis Retinal edema congestion eyeball displacement eyeball protrusion optic atrophy
Eyeball protrusion is a common clinical sign of intraorbital tumor, but this sign does not appear at the beginning, because it compresses the surrounding fat, so that it absorbs and the eye position is compensated. When the tumor is larger than 10mm, the visible eyeball appears, and the sponge is visible. Hemangioma often causes slow, progressive eyeballs, early lack of symptoms and other signs, the patient himself is not aware, the eyeballs are prominent at the time of treatment, and most of them are one-sided, the difference of bilateral eyeballs exceeds 2mm until the eyeball When the lesion is mostly located behind the ball, the direction of the eyeball is mostly axial forward. The tumor is connected with the systemic circulation by small blood vessels, and there is a capsule, so the eyeball protrusion is not affected by the receptor position. This point is related to capillary hemangioma and vein. Different hemangioma, the latter two tumors increase in size and eyeball protrusion, the tumor is a small tumor located in front of the equator of the eyeball or at the tip of the iliac crest, often does not cause eyeball protrusion, the former appears eyeball shift, the latter early vision Decreased and primary optic atrophy.
Visual acuity is also common. Cavernous hemangioma can cause vision loss, accounting for 65.8% of all cases. The tumor is located at the posterior pole after oppression, and the axis of the eye is shortened, causing hyperopia and astigmatism; choroid, retinal folds and edema, degeneration, Primary tumor in the tip of the sacral region, optic nerve loss in the early optic nerve compression, sometimes misdiagnosed as posterior optic neuritis or primary optic atrophy, because the eyeball is not prominent, the visual loss is completely lost, the patient is also present, located in the middle of the sputum The tumor, the initial visual acuity remains normal, unless the volume is large, oppression of the optic nerve or eyeball, and late vision is found.
Eyelid and conjunctival changes in the posterior part of the sacral cavernous hemangioma are normal, or with exposed keratitis, congestion and edema, the tumor in the anterior often causes eyelid bulge, the skin or conjunctiva sees purple blue, the conjunctiva itself change.
The late stage of ocular dyskinesia may occur. The cavernous hemangioma shows chronic expansion and does not infiltrate the extraocular muscle. It does not affect the function of the ocular motor nerve and extraocular muscle in the early stage. In the advanced stage, due to the mechanical obstruction of the tumor, the eyeball is restricted in the direction of the tumor. This sign is found in approximately 40% of cases.
Fundus examination has a certain significance. The tumor originated from the tip of the eye, causing optic atrophy in the early stage. The anterior tumor in the muscle cone compresses the optic nerve, and optic disc edema occurs. About 32% of the tumors are exposed to the eyeball. The fundus can be found in the fundus. Such as posterior pole bulge, choroidal folds, retinal edema, radial texture or macular degeneration, these signs are caused by direct compression or affecting local blood circulation.
Located in the anterior or surrounding space of the tumor, the finger is swept between the eyeball and the wall of the iliac crest, which can affect the tumor, about 34%, medium hardness, slightly elastic or sac sexy, smooth surface, clear boundary, can be pushed, There is a sense of floating, deep tumors can not be paralyzed, with the finger backwards pressing the eyeball has elastic resistance, only the tumor at the tip of the sputum can not cause increased pressure, and can not affect the tumor, only the imaging examination can find the lesion.
Examine
Examination of cavernous hemangioma in the orbit
Pathological examination: cavernous hemangioma is mostly round, purple-red, with a complete capsule. The capsule is formed by the continuous connective tissue of the vascular sinus. It is part of the tumor itself and cannot be separated from the tumor. The small nutrient arteries communicate with the whole body blood vessels, and the veins are also very thin. The tumors are cut open. The cross section is a blood sinus filled with blood. The blood is discharged, the tumor volume is obviously reduced, and the sponge-like pit is seen under the light microscope. The tumor is composed of a large expanded cavernous sinus sinus. The sinus wall is lined with flat and thin endothelial cells. The interstitial is an unequal amount of fibrous tissue, often with glassy changes. Some areas lack interstitial, adjacent to the sinus cavity. The endothelial cells are close to each other, and some interstitial mucus liquefaction or fat cell accumulation, even smooth muscle bundles appear in the interstitial, occasionally the interstitial contains lymphocytes, plasma cells and macrophages and other chronic inflammatory cells, under electron microscope It can be seen that there are basement membranes and 2 to 3 well-differentiated smooth muscle cells in addition to endothelial cells.
1. X-ray examination: early as normal, long-term sputum internal pressure caused by the general expansion of sputum volume and increased density, accounting for about 35% of cavernous hemangioma, but lack of specific changes.
2. Ultrasound exploration: Ultrasound has a high diagnostic rate for cavernous hemangioma. An experienced doctor may promptly indicate the histological diagnosis of the tumor and determine the size, location and relationship with important surrounding structures. Due to the high frequency of the ophthalmic special ultrasound transducer and the poor penetrating power, small tumors 1cm after the eyeball are often difficult to determine.
(1) Ultrasound exploration of type A: the boundary of the tumor is clear, the peak of the internal echo is higher, and the sensitivity of the tissue can reach 60% to 95%. It is the most reflexible in the tumor of the sac, and the peak of the tumor is connected with the baseline. The angle is less than 45°.
(2) B-mode ultrasound exploration: cavernous hemangioma has a unique sonogram, the lesion is round or elliptical, with tumor halo, clear boundary, smooth, inner echo is strong and strong, and the distribution is uniform, moderate sound attenuation By pressing the eyeball with the probe, it can be seen that the tumor axis is shortened, that is, the compression deformation, and the internal echo of the tumor is more and evenly distributed, which is a specific ultrasound sign.
(3) Doppler ultrasound: in most cases, there is a lack of color blood flow in the tumor, which is due to slow blood flow and does not reach the display threshold; pulse Doppler shows a flat blood flow spectrum, ie venous blood flow spectrum Because the sinusoids are venous, the blood flow velocity is consistent.
3. CT scan: CT can accurately indicate the presence of tumor, determine the spatial location, the number of tumors and the secondary changes caused by the tumor. The CT image of cavernous hemangioma has the following characteristics:
More than 1 is located in the muscle cone, the outer side of the optic nerve, the optic nerve is pushed and displaced, and the coronal plane is more convenient for judging the position of the optic nerve;
2 The tumor is round, elliptical or pear-shaped. The boundary is clear, smooth, and the internal density is homogeneous. The density is slightly higher than that of the adjacent soft tissue. The CT value is generally greater than +55HU, and calcium deposit may be found in the tumor.
3 After the contrast agent was injected, the enhancement was obvious, the enhancement value was greater than 25Hu, and the consistency was enhanced;
In 450% of cases, the sacral cavity is enlarged. In most cases of cavernous hemangioma, a triangular transparent zone is retained at the tip of the sac, but the black triangle is often absent from the tip or backward spread. The density and enhancement values depend on the cavernous vessel. In addition to the structure of the tumor, it is also affected by many factors, such as the uneven distribution of blood vessels and fibrous tissues in the tumor, or the inclusion of capillary components, the density may be inconsistent and enhanced, and contrast enhancement is also affected by the injection and scanning time interval. If one side scans while injecting, because the tumor supplying artery is slender, the contrast agent is not filled with the lesion area, or the interval is longer, the contrast agent in the tumor has been discharged, and the enhancement value is small, the contrast agent is full of tumor field, and the vascular sinus cavity is more Large, enhanced CT values can be greater than 100HU, such high enhancement values are rare in other sputum tumors, and CT can reveal the number of tumors, showing the exact location and alignment of the tumor, which is very important for the complete removal of the tumor, because The exploration of small tumors may be missed. CT is based on density to judge the lesions. CT plain films are difficult to treat cavernous hemangioma and schwannomas. , extrathecal meningioma, leiomyoma and benign stromal tumors were identified.
4. MRI: More clearly showing the location, extent, boundary and surrounding structure of the tumor, it is crucial to show the relationship between the tumor and the optic nerve. In this respect, MRI is superior to CT, using SE pulse sequence, and the tumor is moderate in T1WI. Intensity signal, signal intensity is lower than fat, similar to extraocular muscle, higher than vitreous, high signal in T2WI tumor, obvious increase in signal after injection of Gd-DTPA, observation signal intensity should consider TR and TE length, these two parameters Significantly affects signal intensity, MRI shows that the structure of the tumor is not as good as B-mode ultrasound.
5. Red blood cell scintigraphy: The red blood cells were labeled with 99mTc, and scintigraphy was performed on E-CT, and the tumor was concentrated with radionuclide.
Diagnosis
Diagnosis and diagnosis of cavernous hemangioma in the orbit
diagnosis
Orbital cavernous hemangioma is mainly seen in adults, progressive eyeballs, no redness and swelling in appearance, B-ultrasound shows round, elliptical space-occupying lesions, medium echoes are evenly distributed, mildly compressible, CT shows tumors in adulthood It is round or oval, most of which are cavernous hemangioma, and a few are schwannomas.
Differential diagnosis
Cavernous hemangioma has the common clinical features of benign tumors. Several common tumors to be identified include schwannomas, meningiomas, benign pleomorphic adenomas of the lacrimal gland, and angioendothelioma.
1. schwannomas: mostly located outside the muscle cone, sometimes tenderness, B-ultrasound echo less, strong sound, color Doppler ultrasound visible rich color blood flow in the tumor, pulsed Doppler examination visible arterial spectrum CT scan CT value is lower, the degree of enhancement after injection of intensive agent generally does not exceed 20HU; MRI occurs in the tip of the iliac crest often finds dumbbell-shaped cranial palsy to communicate with the tumor, and the cavernous hemangioma generally does not exceed the border of the sacral wall.
2. Meningioma: Most of the vision loss, optic disc edema, secondary atrophy and optic nerve ciliary vessels, B-ultrasound to detect less echo within the tumor and attenuated, incompressible; CT shows optic nerve tubular, fusiform thickening or conical mass MRI can be found in tumors from the optic nerve sheath, which can spread in the tube or in the brain.
3. Lacrimal gland pleomorphic adenoma: occurred in the lacrimal gland, B-ultrasound internal echo, tumor compression of the eye to make it significantly deformed; CT found lacrimal gland bone resorption, cavernous hemangioma hardly occurred in the lacrimal gland.
4. Isolated neurofibromas: The clinical manifestations are similar to those of cavernous hemangioma, but the B-mode ultrasound map has less echo and is not compressible; CT shows that the edges are not smooth.
5. Intraorbital vascular epithelioma is rare: Ultrasound exploration echo is less than cavernous hemangioma, and can not be compressed, color Doppler ultrasound shows abundant red and blue blood flow.
