Orbital lymphangioma
Introduction
Introduction to orbital lymphangioma Lymphangioma (lymphangioma) is a mass of lymphatic vessels covered by endothelial cells, which are divided into capillary, spongy and cystic lymphangioma, the latter being more common. Occurs in children and young people, and a few cases exist at birth. The site of occurrence can be located in the skin, under the mucosa, and in deep organs, and can occur simultaneously in multiple locations. In the eye can invade the eyelids, eyelids and conjunctiva, more common in the orbital or intraorbital eyelids. basic knowledge The proportion of illness: 0.003% Susceptible population: occurring in children and young people Mode of infection: non-infectious Complications: optic atrophy, amblyopia
Cause
Causes of orbital lymphangioma
(1) Causes of the disease
Associated with abnormalities in the body.
(two) pathogenesis
This tumor may be a hamartoma that develops in the lymphatic vessels during embryonic development and is abnormally developed. Rootman classifies lymphangioma in vascular lesions that are not communicating with the systemic circulation, and considers lymphatic and combined venous lymphatic vascular malformations in the bloodstream. The kinetics are simple vascular hamartomas, from the embryonic venous system, which have different lymphatic vessels in part or in whole. The clinical features are based on the extent and location of the lesion. Histologically, these lesions are best understood as a vascular dysplasia. The tumor group, with similar basic components and relatively separate hemodynamics, features transparent, slurry-filled vascular ducts, collagen matrix mesh, with significant recurrence and stale hemorrhage, lymphocyte aggregation, abnormal blood vessels and irregularities The smooth muscle bundle, the lesion is composed of lymphatic vessels but with vein components, some of which develop into a new vascular plexus, which is repeated bleeding. The deeper the lesion, the more obvious the venous component, they are different in clinical, shallow lymph Tumors consist of most clear saclike structures, including chronic yellow or partially bloody cysts. Hemorrhagic cysts constitute.
Prevention
Orbital lymphangioma prevention
Pay attention to living habits, pay attention to the usual eating habits, and find early treatment.
Complication
Orbital lymphangioma complications Complications, optic atrophy, amblyopia
The wider the lesion, the more likely it is to have intracranial lesions. These intracranial venous malformations are developmental venous malformations.
Symptom
Symptoms of orbital lymphangioma common symptoms choroidal depression eyeballs can not be free to move the eyelid edema sag sag eyeball protrusion repeat optic nerve atrophy repeated bleeding
Most of the orbital lymphangioma occurs in infants and young children, and is often accompanied by eyelid and conjunctival invasion. Because of slow progress, long course of disease, and patients who are only seen after adulthood, the clinical manifestations of orbital lymphangioma are mainly eyeballs and eyelid enlargement. And conjunctival clear mass.
Lymphangioma often occurs on the upper or upper part of the iliac crest, the eyeball is prominent, and accompanied by downward displacement, the progress is slow, the degree of ocular protrusion is mostly mild or moderate, and there are more than 12 mm above the contralateral side, sometimes intratumoral hemorrhage, causing Sudden eyeball protrusion or sudden increase in eyeball protrusion, even out of the cleft palate, and then visible orbital or subconjunctival hemorrhage, eyeball protrusion is relieved, and some cases due to repeated bleeding, eyeballs appear intermittent, but not related to body position, this Points can be distinguished from varicose veins. Although the eyeballs caused by lymphangioma are obvious, the increase in internal pressure is not commensurate with it. The oppression of the eyeball can still be returned, and the soft tissue around the eyeball is raised forward, indicating the contents of the tumor, including the tumor. Before and around the displacement, lymphangioma can reach the first 1/3 of the sacral area and can be swollen and swollen. The surface is smooth, single or lobulated, the boundary is unclear, soft or elastic, and the light is visible through the light.
Intraorbital lymphangioma often spreads to or is accompanied by eyelid and conjunctival invasion, which is characterized by eyeball protrusion and hypertrophy of the eyelids, such as a diffuse mass of the dough, and cases of lesions invading the eyelids, eyelids, forehead and cheeks, The facial area is swollen and the lesion spreads to the conjunctiva. The blister-like mass of the iliac crest can be seen in the reversal of the eyelid. It is prominent in the cleft palate. The conjunctiva often sees a transparent papillary-like protrusion. Many of them can occupy the conjunctiva from the cornea to the dome. The eyelid hypertrophy Invasion of the upper levator caused ptosis, covering the cornea, extraocular muscle invasion, diplopia and eye movement disorders, the fundus is mostly normal, also visible optic disc edema or optic atrophy, accompanied by vision loss, vision loss due to pupillary obscuration Caused by amblyopia, or optic neuropathy.
Deep lymphangioma is characterized by sudden eyeball protrusion due to spontaneous bleeding, occasional occlusion of the eyeball, acute changes in upper respiratory tract infection, progressive bleeding leading to optic neuropathy, and combined lesions, including deep intercondylar space and eyelid or eye surface, It is characterized by repeated spontaneous bleeding, upper respiratory tract infection and potential internal optic neuropathy. The common disease changes are common in the supracondylar fissure and can be combined with intracranial venous malformation. The combined lesions not only involve deep and superficial eyelids. It can also involve other parts of the body such as the cheeks, neck, and face.
Examine
Examination of orbital lymphangioma
Pathological examination: Lymphangioma is a non-enveloped invasive tumor, the tumor is spongy, honeycomb or saclike, containing clear liquid, occasionally old blood cysts, in vascular lymphangioma (also known as In vascular tumors, there are both purple-red and mature veins, and lymphatic vessels containing clear liquid. Because of the thin lymphatic vessels, ruptures often occur during surgical separation, and shrinkage. Under light microscope, the diameter of the tube varies, and the shape is not First, it is formed by the expansion of a single layer of flattened endothelial cell-lined tubes. The tube lacks blood cells. According to the size of the tube, pathologists are often divided into capillary, spongy and cystic lymphangioma, but often in a specimen. Contains multiple components, mainly one, such as cystic lymphangioma, can have multiple large thin-walled cysts, spongy or capillary-like elsewhere, light red stain in the lumen, may be protein-containing lymph The matrix between the fluid and the lumen is an unequal amount of fibrous tissue, and sometimes the hemosiderin particles are visible, which are lysates of old bleeding, mature lymphocyte foci can be found in the interstitial, or follicles are scattered or formed, and Hair growth center There are small nourishing blood vessels in the interstitial, these small blood vessels are thin, or protrude into the lymphatic lumen, small blood vessels rupture and hemorrhage, enter the lymphatic vessels, clinical symptoms suddenly aggravate, hemorrhage in lymphangioma, surrounded by endothelial cells ( The lymphatic vessel wall is called a blood cyst or old bleeding, called a chocolate cyst. In general, the soft tissue is hemorrhage, surrounded by fibrous tissue composed of a pseudocyst, lacking endothelial or epithelial cell lining called hematoma. ), lymphangioma observation by electron microscopy, the wall of the tube is not accompanied by smooth muscle and vascular epithelial cells, and the endothelial cells are attached to the intermittent basement membrane, which is different from cavernous hemangioma.
1. Ultrasound examination: A super-existing shows that the lesion is obviously heterogeneous, and there is a low reflection or liquid level between multiple or single high reflections. Because the lesion has no obvious capsule, the shape of the lesion on the B-ultrasound is irregular. The boundary is unclear, the lesion can be limited or diffuse, often showing multi-cavity space-occupying lesions, with more septa, rarely single-cavity lesions, such as lesion invasion and sputum can show no extension from the eyelid to the iliac Regular lesions, uneven internal echo, lesions often surround the optic nerve.
2.CT: Most lesions are located around the eyeball, and are more common outside the muscle cone and in the muscle cone. Typical lymphangiomas have different cord-like shapes on the CT, elliptical or irregular high-density space-occupying lesions. Coronary CT showed that the lesions were mostly punctate high-density space-occupying lesions, infiltrating and surrounding the optic nerve and/or extraocular muscles, and could be filled with sputum, causing serious ocular protrusion. Due to the closeness of the density of blood loss and tumor, Plain CT is difficult to show bleeding or hematoma in the lesion, and the CT lesion is obviously enhanced. If the unenhanced lesion is likely to be bleeding.
3. MRI: Lymphangioma can cause repeated bleeding. It has some unique signals in MRI, so it is an ideal MRI examination object. It is very helpful for diagnosis. Lymphangiomas have different internal structures and cystic components. Signal, tumor can be monocystic or polycystic, homogenous or non-homogeneous irregular lesions, T1WI lesions and the extraocular muscles showed a slightly higher signal, compared with sputum fat showed a low signal, T2WI was significantly higher Signal, this type of MRI signal may be secondary to the lymphatic vessel and contains a clear liquid.
Acute hemorrhage in the lesion showed a low signal on the T1WI image compared with sputum fat. Because of its higher deoxyhemoglobin, it showed a low signal on T2WI. With the passage of bleeding, the tumor showed cis-magnetic and positive due to lysis of red blood cells. There is a high signal on both TlWI and T2WI in the increase of hemhemoglobin. The cystic part of the tumor may have a fluid cyst. The upper part of the cyst contains gangmoglobin which dissolves red blood cells, so it has higher signal than the precipitate of the cyst below; The hemoglobin component of hemoglobin, so the signal is lower, and with the further absorption of hemorrhage, methemoglobin becomes hemoside and ferritin. The high signal of T1WI and T2WI gradually becomes a low signal, and a few large lymphangiomas can be There are large arterial vessels with typical flow signals, and most of them are unevenly enhanced after enhancement.
After Gd-DTPA is enhanced, the degree of enhancement of lymphangioma is different. Generally, the wall or parenchymal part is enhanced, the cystic fluid is not enhanced, and the MRI tumor enhancement without fat suppression is not easy to distinguish from the high signal of sputum fat, so fat suppression is very important. Internal hemorrhage of tube tumors and lymphangiomas showed more changes in MRI signals.
Diagnosis
Diagnosis and diagnosis of orbital lymphangioma
In the adolescence, the eyeball protrudes from the lower part of the eye, and the upper part can be softened and the soft mass. The history of repeated subcutaneous or subconjunctival hemorrhage indicates the diagnosis of lymphangioma. The typical imaging appearance is the foamy high signal shape indicated by MRI. Especially, the signal during intratumoral bleeding is very helpful for diagnosis.
Because the wall of the lesion is thin, there is no incentive or self-induced blood after the sensation. The clinical manifestations of the eyeball are obviously aggravated or the tumor is obviously enlarged. The imaging examination reveals that the lumen is dilated, sometimes the fluid level is visible. Due to the sudden increase of the lesion, the striated muscle of the eyelid should be The identification of sarcoma, the most common malignant tumor in childhood, has a higher mortality rate, but ultrasound shows a low echo or low reflex, with clear boundaries.
