Introduction
Focal fibrocartilaginous dysplasia is one of the benign conditions described first by Bell in 1985. This is considered a cause of tibia vara. In this condition, a failure of differentiation persists in fibrocartilaginous tissue. This is a cause of disturbance in the growth of the proximal tibia that results in deformity. This lesson is also called fibrous periosteal inclusion, as it is considered a natural periosteum sliding during growth. The treatment procedure includes six months after observation. The curettage process should be carried out if the deformity of this disease progresses or worsens. This can prevent the need for osteotomy. In some instances where the tether breaks independently, no treatment is required.
There are focal fibrocartilaginous dysplasia lesions, including the proximal area of the tibia, distal of the femur, and ulna. This lesion behaves like a bony anchor that prevents the natural sliding periosteum seen during growth. The treatment is considered differently for cases with tibial lesions, metaphyseal, and diaphyseal angles being less than 30 considered observation for about 6 to 12 months. In cases where the deformity is seen improving, the rope gets broken independently, and no specific treatment is required. This is to be followed by rapid correction into the physiological tibia, and it also prevents any need for performing osteotomy. For lessons other than this, an early curettage is recommended as the treatment.
What Are the Features of Focal Fibrocartilagenous Dysplasia?
The studies reveal that focal fibrocartilagenous dysplasia lesions are primarily in the patient's lower limbs. These patients are mostly younger population.
Some deformities are associated with this disease. These include deformity because of functional abnormalities, as well as inequality of discrepancy in the lengths of two limbs.
This inequality may result in abnormal gait noticed in the patient while walking. There are angular deformities in this disorder that make the disease easily diagnosable because of angular deformities. This gives an early presentation and early diagnosis of this disorder.
Abnormal development of fibrocartilage is seen at the insertion of the tendon. The deformity is not seen in the growth plate. The lesion can be self-corrected even if the angular defect is beyond 30 degrees.
The lesions are also present in the upper limbs. The lesions involving the upper limbs are usually seen in older patients. The bones involved in focal fibrocartilagenous dysplasia in the upper limbs include the proximal part of the humerus. This is usually well masked because of the shortening and does not have angular deformities that allow early diagnosis of the lesion. The functional abnormality in these affected upper limbs is not very severe and leads to a delay in diagnosis.
In the tibia, the pathology is usually seen in proximal metaphysis. The angle between metaphysis and diaphysis must be measured to differentiate this condition from the physiological bending of bone. The lines are drawn through transverse planes of proximal tibia metaphysis and another line perpendicular to the tibial diaphysis.
What Are the Radiography Findings of Fibrous Fibrocartilagenous Dysplasia?
There are specific radiologic features to diagnose fibrous fibrocartilaginous dysplasia. This finding is adequate for the diagnosis of the lesion. The normal plain radiographs of the affected area and the bone display a well-defined and focused lesion that is radiolucent and is present in the proximal medial cortex of bone along with the sclerotic and cortical thickening that extends to the distal position of the bone. Currently, plain radiographs and magnetic resonance imaging are adequate for diagnosing the lesion.
What Are the Historical Presentation of Fibrocartilagenous Dysplasia?
In fibrous dysplasia, the presence of cartilaginous nodules is seen. The actual amount of the chondroid matrix varies significantly. In some cases, it may be very extensive and can be seen on histological examination and radiographs. In radiographs and imaging, it appears as a ground glass matrix and shows the presence of rings and arcs of calcification. The chondroid matrix is the dominant feature and has increased cellular, binucleated cells and an atypical nucleus. This can lead to misdiagnosing fibrocartilagenous dysplasia with the primary lesion of cartilage-like chondrosarcoma, even in small specimens for biopsy.
What Are the Differential Diagnoses of Focal Fibrocartilagenous Dysplasia?
Several disorders may mimic lesions of focal fibrocartilagenous dysplasia. These include
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Infection or any trauma that causes physical injury.
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Malunion of a fracture.
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Osiris dysplasia.
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Certain tumoral conditions.
What Is the Treatment for Focal Fibrocartilagenous Dysplasia?
Since the history of focal fibrocartilagenous dysplasia is being studied, little is known about the exact cause and prognostic factors, and the treatment is optional. It has been studied that almost 50 % of cases of tibial fibrocartilagenous dysplasia do not require treatment and get spontaneously resolved. In most cases, there is resolution, and the deformity progresses spontaneously. Compared to previous studies, the new studies have reported more cases that do not require treatment. Many cases, if allowed close observation, do not require surgical correction.
In certain cases, where the disease is on the proximal humerus, it may not be a benign condition like it is the case with other long bones. This also depends on the severity and location of the pathology. The potential of growth retraction is highest when the lesson is near the proximal physis. In such cases where the treatment is not done with surgical intervention, reports show that shortening of the affected upper extremity occurs.
The disorder is noticed by parents in their children very shortly after they start ambulating. So, the pediatric patient presents with the condition at 12 to 24 months. Initially, the lesions progress, and the self-correction starts at around 24 months. The clinical and histopathological characteristics are not uniform and require different times for resolution.
Conclusion
Focal fibrocartilagenous dysplasia is a lesson of bone that results in the physiological bending of the bone. This is diagnosed early because of physical abnormalities such as gait and unequal extremities. In most cases, it is resolved independently and does not require any surgical intervention. In severe cases, curettage may be beneficial. The surgical intervention should be avoided, for parents and surgeons should proceed confidently.
