orbital fibrous dysplasia
Introduction
Brief introduction of orbital fibrosis abnormal proliferation Fibrousdysplasia is a self-limiting congenital benign fibrous fibrous disease with unknown etiology and slow progression. It is not uncommon in this disease. It accounts for about 25% of bone neoplasms and originates in the orbit. Less. There are three types of clinical, namely, single bone type, multiple bone without endocrine dysfunction type and multiple bones with endocrine dysfunction type. Most of the invaders are single bone type, and the above jaw and frontal bone are more common. Patients with multiple bones with endocrine dysfunction often exhibit secondary sexual precocity. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: osteosarcoma fibrosarcoma
Cause
Causes of ocular dysplasia
Cause:
At present, the exact cause is not clear, and may be related to local blood circulation disorder caused by trauma, infection, endocrine dysfunction and other factors.
Osteofibrosis is a fibrous bony lesion that is considered to be a structural abnormality that may be caused by idiopathic retention of mature bone during the osteogenic phase, involving only one or more bones.
Prevention
Prevention of abnormality of orbital fibrosis
The prognosis of vision varies with various cases, and the maxillary, frontal or ethmoid bones often do not cause blindness. However, when the sphenoid bone is involved, the optic nerve is compressed and extreme vision loss can occur. If the optic nerve is found early, blindness can often be prevented. For most patients, the prognosis of life is still good. This benign lesion can cause a soft tissue malignancy, either after spontaneous or radiotherapy. Therefore, careful follow-up of patients is necessary.
Complication
Ocular dysplasia dysplasia complications Complications Osteosarcoma fibrosarcoma
The most serious ocular complication is the oppression of the optic nerve involving the sphenoid bone, which can lead to chronic or acute vision loss (early detection can be treated by surgical decompression). In some cases, the sphenoid sinus mucinous cyst is secondary to blindness due to oppression of the optic nerve. The abnormality of bone fiber dysplasia becomes osteosarcoma, fibrosarcoma or other malignant lesions.
Symptom
Symptoms of orbital fiber abnormal hyperplasia common symptoms hearing loss facial deformity eyeball protruding ptosis vertebral field defect eyeball shift
There are three types of clinical, namely, single bone type, multi-bone without endocrine dysfunction type and multi-bone with endocrine dysfunction type. Most of the invading eyelids are single-bone type, and the upper jaw and frontal bone are more common. Endocrine dysfunction type often shows secondary sexual precocity.
The disease is a congenital bone disease, but it is asymptomatic and painless in childhood. When the lesion grows to a certain extent, the eyeball is prominent, and the facial deformity or visual acuity is reduced. It mainly occurs in young people under the age of 30, especially women. Symptoms may vary depending on the location involved. The symptoms are facial asymmetry, eye movement and eyeball protrusion, progressive aggravation, depending on the location of the disease, the symptoms are different, the cause of bone fibrosis in the orbital wall As the volume of the eyelids shrinks, headaches can occur, and the eyeballs can be prominently displaced. The posterior group of ethmoid sinus or sphenoid sinus can cause stenosis of the optic canal, causing vision loss, visual field defect, severe visual loss, and oppression of II, III. IV, V, VI have corresponding symptoms on the cranial nerve; lesions located in the tibia can affect the external auditory canal, and hearing impairment occurs; the frontal bone can produce ptosis and supraorbital nerve paresthesia; the forehead can be seen in the frontal diffuse bulge, without borders , painless, regardless of the symptoms caused by the site of the disease, the appearance of deformity due to a wide range of bone lesions is a common sign.
Examine
Examination of abnormal ocular fibrosis
Pathological examination: grossly observed lesions were grayish white, slightly softer than normal bone tissue, with a strong gritty-like sensation when cutting, no capsule, lesions starting from the medulla of bone, often leaving two layers of cortical shell, under light microscope Observed the size, shape and distribution of trabecular trabeculae, lesions with fibrous interstitial, spindle-like cells with consistent, benign appearance, most trabecular bone lack osteoblasts, this feature is different from ossification Fibroma.
1. X-ray examination can show the increase from bone ribbon to diffuse bone density, sclerosis, facial and skull base bone often involved, osteolytic changes are common in the skull, affected bone expansion combined with CT inhomogeneity Density and intact cortical bone are characteristic of bone fibrosis.
2. Ultrasound examination Because it is a bone proliferative lesion, such as sacral hyperplasia, ultrasound usually only shows a decrease in the fat pad or a bulge in the iliac crest.
3. CT scan CT is the main method for diagnosing this disease. The lesion mainly involves the temporal wall, frontal bone, ethmoid bone, maxilla, sphenoid bone and even the whole skull. CT shows that the bone hyperplasia is diffuse, no border, and the iliac wall is due to bone hyperplasia. And shrinking, showing a "small eyelid", such as involving the optic canal can be narrow.
4. MRI MRI is very important to understand the internal structural characteristics of the disease and to determine the extent of intraosseous and extra-bone. It determines the boundary between the tip of the eye and the optic canal more clearly than other images, the swelling of the bone and the lesion. Fibrous tissue exhibits low to moderate signal on T1WI and low to high signal on T2WI. The T2 signal of this disease is very important for diagnosis. Sometimes there are cystic cavities in the frontal bone lesion, which has a liquid level, due to the height of the fibrous matrix of the lesion itself. Vascularization can lead to the formation of aneurysmal bone cysts. Generally, bone hyperplasia itself does not enhance, but the fiber component can be enhanced with mild or moderate enhancement signals.
Pathological examination: grossly observed lesions were grayish white, slightly softer than normal bone tissue, with a strong gritty-like sensation when cutting, no capsule, lesions starting from the medulla of bone, often leaving two layers of cortical shell, under light microscope Observed the size, shape and distribution of trabecular trabeculae, lesions with fibrous interstitial, spindle-like cells with consistent, benign appearance, most trabecular bone lack osteoblasts, this feature is different from ossification Fibroma.
Diagnosis
Diagnosis and differentiation of orbital fibrosis abnormal proliferation
The important clinical auxiliary diagnosis is X-ray plain film combined with CT scan. The X-ray is diffuse bone mineral density, the boundary is unclear, CT scans a wide range of bone hyperplasia, the boundary is clear, and the bone window shows that the lesion density is lower than normal bone. The qualitative density, the unique signs of clinical skeletal deformities are helpful for diagnosis.
And bone fiber dysplasia should be identified is sphenoid ridge meningioma, especially bone fiber dysplasia involving only sphenoid bone, sphenoid ridge meningioma in addition to bone hyperplasia, in the orbital or intracranial adjacent Soft tissue shadows, especially MRI, are clear.
