Orbital inflammatory pseudotumor
Introduction
Introduction to orbital inflammatory pseudotumor Idiopathic orbital inflammatory pseudotumor is a common clinical inflammatory lesion of the eyelid. Because of the variable symptoms, it is often easy to cause misdiagnosis. Especially, it is easy to misdiagnose other orbital tumors or eyelid diseases as inflammatory pseudotumor. The histopathological classification of orbital inflammatory pseudotumor is as follows: lymphocyte proliferative type (mainly lymphocyte proliferation, visible lymphoid follicles and other structures), fibrous tissue proliferative type (mainly fibrous tissue proliferation, few cellular components) and Mixed type (between two types). The pathological features of inflammatory pseudotumor depend on different stages of the tissue, different parts and lesions in the sputum obtained by the operation. Basic cell types include lymphocyte plasma cells, fibroblast macrophages, giant cells, epithelioid cells, reticulocyte vascular endothelial cells, and uncommon polymorphonuclear cells, eosinophils, and the like. Clinically, it is divided into lacrimal gland type, myositis type, periorbital diffuse type and mass type according to the location of lesion invasion and imaging findings. The clinical manifestations of lesions at each position are different. basic knowledge The proportion of illness: 5% to 7.6% (about 5% to 7.6% of eyelid disease) Susceptible people: no special people Mode of infection: non-infectious Complications: conjunctival hyperemia
Cause
Causes of orbital inflammatory pseudotumor
(1) Causes of the disease
May be related to abnormal immune response mechanisms.
(two) pathogenesis
The pathogenesis is unknown, and the relevant immunopathological mechanisms are not well understood. Some people think that there are local autoantigens that can attract autoantibodies or immunocompetent cells in the blood, but this antigen has not been isolated yet. Many pathological features indicate that inflammatory pseudotumor is an Arthus reaction, that is, antigens or antibodies in the blood encounter corresponding amounts of antibodies or antigens in the orbital soft tissue, causing inflammation in different tissues of the iliac crest, and limited local lymphatic tissue around the blood vessels. Cell and eosinophil infiltration indicates that some substances of extravasation can adsorb these cells. Inflammatory pseudotumor is effective for corticosteroids and radiotherapy, which is further confirmed as the result of immune response, but acute or subacute phase of orbital inflammatory pseudotumor It is rare to confirm the diagnosis by biopsy, which brings difficulties to immunological research. The orbital tissue is not directly exposed to common allergens in vitro, there is no lymphatic and lymphoid tissue in the sputum, and the occurrence of non-specific inflammatory pseudotumor in the sputum and blood The abnormal component in the body may be related to immune complexes, and the animal model of the orbital idiopathic pseudotumor is still Perfect.
Eosinophilia in non-specific inflammatory pseudotumor tissues of the eyelids, and some eosinophils release toxic granular proteins into the connective tissue of the stroma, which may damage the intraocular tissues and extraocular muscles, and non-specific inflammation. Markers such as erythrocyte sedimentation rate acceleration and fever are not manifested in patients with orbital inflammatory pseudotumor, indicating that the disease is not a systemic immune disease; on the other hand, many proteins in the eyelids may be immunogenic, but only rarely The protein acts as an original target antigen causing an autoimmune response, in some cases.
The inflammation caused may be limited to some tissues of the eyelids, leading to extraocular myositis, peripheral neuritis, peripheral scleritis and lacrimal gland inflammation; in other cases, diffuse inflammation leads to the involvement of most ocular tissues, and clinical manifestations may overlap each other. The study revealed that 52.5% of patients with non-specific eyelid inflammation had extracorporeal sarcolemma 55kDa, 64kDa protein-reactive antibody, and 95kDa and 45kDa protein antibodies were detected in the serum of 50% of patients with specific eyelid inflammation. 55kDa in normal individual serum. The protein antibody detection rate was 16%, the 64kDa protein antibody detection rate was 20%, and the 95kDa and 45kDa protein antibody detection rates were 24% and 20%, respectively. In most non-specific eyelid inflammation patients, the above protein antigen antibodies It is higher than normal people, especially the 55kDa protein antibody is more obvious, so the extraocular sarcolemmal protein antigen and its corresponding reactive antibody may play a role in the pathogenesis of the disease.
Prevention
Orbital inflammatory pseudotumor prevention
Pay attention to the usual life, pay more attention to the diet, and find early treatment for the patient.
Complication
Orbital inflammatory pseudotumor complications Conjunctival congestion
Eyeball shift, conjunctival hyperemia, diplopia, eyeballs can not move freely, eyelid pain.
Symptom
Symptoms of orbital inflammatory pseudotumor Common symptoms Eyeball can not be free to move edema Eyelid pain Optic atrophy Conjunctiva Congestion Eyeball Highlight Double vision
Clinical manifestations can be acute or chronic, and a tissue in the sputum can be involved, or multiple tissues can be involved at the same time, and the condition is easy to repeat.
The main symptoms and signs of orbital inflammatory pseudotumor are related to inflammatory edema and cellular infiltration of intraorbital tissue:
1. Eyeball protrusion and displacement Most patients have this sign. Tissue edema, mass formation, and extraocular muscle enlargement can increase the internal volume of the sputum. Due to tissue push, the eyeball is accompanied by eye movement. See lacrimal gland type or myositis type.
2. The basic histopathological changes of edema and hyperemia inflammatory pseudotumor are tissue edema and inflammatory cell infiltration. Due to the increase of internal pressure, blood circulation disorder aggravates tissue edema. Therefore, in addition to conjunctival congestion and edema, it is often accompanied by eyelid swelling. Especially in the anterior part of the iliac crest, edema and congestion are more obvious, severe conjunctival congestion and edema can be prominent beyond the cleft palate, prolonged prolapse, resulting in rough conjunctiva, erosion, necrosis, lacrimal gland edema mainly located On the lateral side of the ankle, the upper temporal margin is S-shaped and the cleft palate is deformed.
3. Eye movement disorder and double-view granuloma type and myositis type common eye movement disorder, accounting for about 1/2. Fiber-hardening type obviously affects eye movement, and often is multi-directional movement disorder, even eyeball fixation, eyesight is still good. At the time of diplopia, the lacrimal gland type inflammatory pseudotumor less affected the extraocular muscles, and only showed mild upper eye movement limitation.
4. In the sacral area, the lesion is located in the anterior part of the iliac crest. It often touches the mass at the time of percussion. It can be touched above the iliac crest and below the iliac crest. The boundary is clear and can be pushed. It is round or nodular, and can be one or more. Medium hardness or hard, some lumps need to force the mass to move forward when the eyeball is pressed.
5. Periorbital pain about 1 / 3 patients have spontaneous pain, which occurs with intra-orbital tissue edema, mass formation, increased intra-orbital pressure and inflammation affecting the periosteum, sclerosing inflammatory pseudotumor with pain more common.
6. Vision loss When inflammation involves optic nerve or apical inflammatory mass formation oppression of the optic nerve, can reduce vision, fiber sclerosis type damage to visual impairment, even black Mongolian, early changes in the fundus, visible optic disc edema; late manifested as optic nerve Shrinking.
Examine
Examination of orbital inflammatory pseudotumor
1. Immunological examination Polymerase chain reaction can detect the rearrangement of clonal immune protein heavy chain gene, monoclonal is a malignant tumor, inflammatory pseudotumor is polyclonal, no other special laboratory tests.
2. Pathological examination of the pathological features of orbital inflammatory pseudotumor depends on the different tissues and lesions in the sputum obtained by the operation at different stages. The basic cell types are lymphocytes, plasma cells, fibroblasts, macrophages, giant cells. Epithelioid cells, reticular cells, vascular endothelial cells and uncommon polymorphonuclear leukocytes, eosinophils, which are significantly different from lymphoma formation of single lymphocytes, and these cell types are also different at different stages of the lesion.
In the early stage, it is characterized by tissue edema, lymphocytes, plasma cells, eosinophils and polymorphonuclear leukocytes infiltration; children with more eosinophils in the diseased tissue.
When the lesion progresses, the fibrous connective tissue increases, lymphocytes and plasma cells are dispersed in the connective tissue, the extraocular muscles become thick due to fibroplasia, and the lacrimal gland acinus and connective tissue around the duct increase; as the disease progresses, the extraocular muscle fibers The lacrimal gland secretion function disappears, the ducts proliferate, the lacrimal gland structure is destroyed, the lymphoid follicles are formed in the chronic phase, and the germinal center is also accompanied by eosinophils.
A small number of inflammatory pseudotumors, especially younger patients, may have vasculitis, lymphocytes, polymorphonuclear leukocytes and eosinophils deposited in the vessel wall, causing local damage; lymphocytes, occasionally eosinophils appear in the blood vessels Around, a vascular cuff sign is created.
Interfacial or visible sputum fat necrosis, the released lipids are engulfed by macrophages, and there are multinucleated giant cells, lymphocytes, tissue cells, fibroplasia and fatty granuloma formation around the necrotic foci.
3. X-ray examination of inflammatory pseudotumor often shows normal or increased eyelid density, diagnosis is difficult, and this has rarely been used for diagnosis, showing that bone changes are better.
4. Ultrasound exploration The ultrasound display is different due to the location and pathological morphology of the lesion.
(1) Lymphocyte infiltration type: Due to the infiltration of more cells in the tissue, a reflective interface is formed between the cells and the interstitial, and the ratio of cells to interstitial is different in different parts, so the reflected echoes are different, and the type A ultrasound is low. Waveform or lack of waveform, the back boundary is high-point wave, B-mode ultrasound shows that the lesions in the sacral lesions vary in size, the shape is irregular, the boundary is clear, the internal echo is less or moderate, the sound attenuation is moderate, the posterior border can be displayed, and the lesion involves the eyeball When the membrane and the ball wall, the fascial sac edema, T-type sign can be seen, this sign is common in the lesion range is large, occupying all the eyelids, ultrasound can also see the anechoic zone into the ball, like the ball within the lesion, at this time A comprehensive analysis should be combined with other imaging findings to establish a diagnosis.
(2) Fibrous inflammatory pseudotumor: histological morphology sees more collagen fibers, less cell infiltration, less acoustic reflex interface, A-type ultrasound shows less echo in the lesion, sound attenuation is obvious, and the reflected wave in the lesion is gradually reduced. B-mode ultrasound shows that the shape of the lesion is irregular, the boundary is clear or unclear, there is a little internal echo in the front of the lesion, the inner echo is lacking, the sound attenuation is obvious, the posterior border is not displayed, and the pressure has no morphological change.
(3) Inflammatory pseudotumor lacrimal gland type: the lesion is mainly located in the lacrimal gland, which may involve the unilateral lacrimal gland, and may also involve the bilateral lacrimal glands. The type A ultrasound shows that the enlarged lacrimal gland has a medium and dense reflection wave, and the posterior boundary shows clearly. B-mode ultrasound showed that the lacrimal gland was swollen, the shape was elliptical, the boundary was clear, the internal echo was less or the blocky strong echo was seen, and the distribution was uneven.
(4) Myositis type: the lesion mainly affects the extraocular muscles, which may involve one or more muscles. It may also have a mass lesion in the iliac crest during the extraocular muscle lesions. The extraocular muscles are swollen and appear as fusiform or spherical. There is little or no echo in the muscles.
(5) inflammatory pseudotumor optic neuritis type: lesions involving the optic nerve and surrounding tissues, some with optic nerve surrounding mass, ultrasound showed optic nerve thickening, internal echo increased, and echo distribution is uneven, may have optic disc edema, the ball Prominent inside.
5. Color Doppler ultrasound showed that in some cases, the blood supply was rich in lesions, and there were more colored blood flow signals, which were diffuse or tubular, some blood flow was the arterial spectrum, and some lesions had few blood flow signals.
6. CT scan for inflammatory pseudotumor, CT is superior to ultrasound, CT findings are consistent with the diversity of clinical and pathological morphology, CT shows that there are irregularly shaped masses in the sputum fat, which can present multiple lumps, and the boundaries are not neat. The high-density mass, uneven internal density, accompanied by peri-sclera inflammation and optic neuritis, shows thickening of the eye wall, blurred borders, thickening of the optic nerve, high-density block shadow and the eye wall is "casting" embedded Together, more common in sclerosing inflammatory pseudotumor, the mass can occupy the eyelids, the extraocular muscles and optic nerve are covered by the mass, and even the eyeball is deformed by compression. The various types of inflammatory pseudotumor can exist alone or in combination. The image features are diverse. In addition, CT of the inflammatory tumor often shows swelling and hypertrophy of the eyelid, prominent eyeball, and enlarged sacral cavity.
Lacrimal gland type inflammatory pseudotumor may involve one side of the lacrimal gland, and may also involve the bilateral lacrimal glands. The lacrimal gland is consistently enlarged, mostly rounded, located at the front of the outer edge of the iliac crest. The extension is flat, high in density and uneven in reinforcement.
Myositis-type inflammatory pseudotumor involves an extraocular muscle or multiple extraocular muscles, which can be seen in one eyelid or bilateral eyelids. Extraocular muscle swelling can affect tendons and stagnation points. The oblique section of the superior rectus and the inferior rectus muscle during horizontal scanning is easily mistaken for the tumor, and the coronary scan image can help confirm the diagnosis.
7. MRI imaging is an inflammatory tumor mainly composed of lymphocytic infiltration. The lesion shows a middle signal on T1WI, T2WI is higher than or equal to the signal intensity of fat, and the fiber sclerotic inflammatory tumor. Because of the collagen fiber component, the lesion is Both T1WI and T2WI showed low signal, and the myositis-like extraocular muscle T1WI was medium signal intensity, and T2WI was medium or high signal intensity.
Diagnosis
Diagnosis and diagnosis of orbital inflammatory pseudotumor
diagnosis
Orbital inflammatory pseudotumor is characterized by inflammatory diseases and neoplastic diseases, so the clinical manifestations are diverse and need to be confirmed under the support of imaging examination. A few cases are diagnosed only with the support of histopathology.
Acute inflammatory pseudotumor due to acute onset, conjunctival hyperemia, eyeball protrusion, double vision and other easy to diagnose, but chronic or subacute cases, simple eye signs and symptoms, diagnosis is quite difficult, should be used as an auxiliary examination to help diagnose, in difficult cases Among them, CT scan plays a decisive role in diagnosis, and different tissues according to CT display can be divided into five categories.
If CT diagnosis is still not possible, the diagnosis should be performed. For such difficult cases, the tissue should be removed and the pathological diagnosis should be performed. For patients with deep lesions and large surgical damage, eyelid puncture should be performed under the guidance of CT examination. Aligning the site where the lesion is located, aspirating or wearing a part of the tissue for cytology, the positive rate is high, but some (about 10%) cases are negative, especially chronic inflammatory pseudotumor, most of the lesions are fibrotic. The examination rate is low, so it is not suitable for eyelid aspiration biopsy.
Except for routine histopathological examination, the specimens can also be examined by electron microscopy, immunohistochemistry L26, UCHL1, , and polymerase chain reaction (PCR). Electron microscopy can identify the inflammatory pseudotumor. Cells, such as lymphocyte nucleus, less cytoplasmic organelles, irregular villi on the surface of B lymphocytes, smooth surface of T lymphocytes, such as lymphocytes with only T or B single lymphocytes; inflammatory pseudotumor or reactive hyperplasia Both T and B lymphocytes should be present at the same time. There is a rich rough endoplasmic reticulum in the plasma cytoplasm. Macrophages swallow the cell debris, lysosomes and curly cell membranes. The immunoblasts are large and the cytoplasm is short. The rough endoplasmic reticulum, which is mitochondria and polysomes, is recognized.
Immunohistochemical staining L26 positive for B lymphocytes, UCHLl positive for T lymphocytes; light chain , expression, single light chain expression of or , one of which is positive for malignant tumors, double light chain for expression of , All were positive for inflammatory pseudotumor or reactive hyperplasia.
Differential diagnosis
It is mainly differentiated from lacrimal gland epithelial tumors. The latter CT shows that the tumors are expansively growing, often with ostium formation. Malignant people may have bone destruction. In the ultrasound, the latter is a multi-echo-occupying lesion, but in the elderly bilateral lacrimal glands. Lymphoma should be excluded when swollen.
From the perspective of imaging, it should be differentiated from invasive lesions of the eyelids, such as malignant tumors, vascular malformations and other eyelid inflammations, but the clinical recurrence and hormonal therapy are effective in differential diagnosis.
The most easily confused with this disease is the extraocular muscle hypertrophy caused by thyroid-related eye disease. The latter is mostly bilateral, the most common is the inferior rectus muscle, and most of the muscle is abdominal hypertrophy, and the muscle attachment point is normal.
CT scan and optic nerve sheath meningiomas are differentiated. The latter often has tubular thickening and sputum tip enlargement. The latter hormone therapy is ineffective. If clinical identification is difficult, biopsy can be considered for diagnosis. Clinical manifestations and imaging findings show extraocular Muscle hypertrophy supports inflammation, although lesions surround the optic nerve like optic nerve tumors.
