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A Quick & Dirty Approach to Skeletal Dysplasias

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Whether you think processed the or partially, if you offer your solid and frequent processes then stories will Show non-state seas that are mostly for them. Signal intensity on T1- and T2-WI and the degree of contrast enhancement depend on the amount and degree of bony trabeculae, cellularity, collagen, and cystic and haemorrhagic changes. The higher the number of bony trabeculae, the lower the T2 signal, and vice versa—the fewer bony trabeculae, the higher T2 signal. FD lesions may also contain small cystic areas, which make the T2 signal brighter.

All lesions showed some degree of enhancement on post-contrast T1-WI. Active lesions show avid enhancement, while inactive lesions show milder enhancement [ 8 ]. The enhancement pattern may be patchy central, rim, homogeneous or a combination. This technique is helpful for the evaluation of complex cases of FD, such as in patients with compression of neurological structures in the brain and spinal canal.

The technique may be useful in assessing malignant change and demonstrating the extension of tumour into the surrounding soft tissues. Diffusion-weighted imaging DWI may be helpful in differentiating benign from malignant osseous lesions, especially in the skull [ 9 ]. ADC values in skull lesions correlate with cell density and can potentially narrow the differential diagnoses for indeterminate lesions Fig.

MRI in fibrous dysplasia FD. The left rib lesion red arrow demonstrates restricted diffusion, which requires further evaluation to rule out malignant transformation. Technetium 99m-methyldiphosphonate 99m-Tc-MDP bone scan is used to detect metabolically active lesions and assess the extent of disease, especially in young patients [ 10 ]. Radiographs are used selectively to monitor the progression of the lesions initially identified on bone scans.

Fibrous dysplasia for radiologists: beyond ground glass bone matrix

After an initial diagnostic bone scans, a follow-up bone scan is not recommended. Nuclear medicine imaging in fibrous dysplasia FD. Multimodality imaging in polyostotic fibrous dysplasia FD.

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With time, mesenchymal cells that carry GNAS mutations undergo apoptosis, leading to a decreased number of FD cells and, thus, changing the classic radiographical appearance of ground glass to a more dense and sclerotic pattern Fig. Craniofacial lesions in older individuals typically become less homogeneous on CT, developing discrete radiolucent, cystic-appearing areas Fig. Rapid expansion of lesions is concerning for possible malignant transformation or ABC development.

Age-related changes in fibrous dysplasia FD. CT of the head on the same patient at age of 6 a , 7 b and 14 years c. Bisphosphonates, which are used in the treatment of FD-related osseous pain, change the appearance of the bone and are responsible for the development of parallel sclerotic metaphyseal bands. These bands develop in any growing child treated with bisphosphonates, and are not specific to FD [ 12 ].

Histologically, these bands are composed of horizontally arranged trabeculae containing calcified cartilage Fig. Bisphosphonate-induced lines. Administration of bisphosphonates results in the development of the parallel sclerotic metaphysial bands, which can be seen on radiographs green arrows and T1-weighted MRI blue arrows. The course and spectrum of FD complications is primarily determined by the disease location and burden.

Many FD patients suffer quality of life impairment, which is increased in patients with greater disease burden and more pronounced in patients with polyostotic FD and MAS, secondary to their greater risk of developing complications such as deformities or fractures. Pain and deformities, as well as benign and malignant bony matrix transformations, may occur in FD of any location. Depending on the lesion location, the disease may have different presentations and complications. Pain occurs in about two out of three patients with FD, is more prevalent and severe in adults than in children, and can significantly impact mobility and quality of life [ 13 ].

Interestingly, there is no association between the extent or location of disease and the likelihood of having pain; patients with mild FD may have debilitating pain, and patients with extensive disease may be pain-free. Patients with new onset of pain should undergo clinical and radiological evaluation for underlying metabolic, functional and orthopaedic complications or possible malignant transformation.

Fractures are common and are most prevalent between the ages of 6 and 10 years, declining thereafter Fig. Several factors may predispose patients to fractures. One of them is hyperthyroidism that can cause clinically significant bone mineral loss through the direct stimulation of bone resorbtion. Moreover, abnormal FD cells secrete the protein fibroblast growth factor 23 FGF , which is responsible for hypophosphatemic rickets and, therefore, increased osteomalacia and fracture rates.

Fracture rates increase with higher disease burden and FGF levels. Fractures in fibrous dysplasia FD. Benign secondary changes include aneurysmal bone cyst-like changes and myxoid changes Figs. When an ABC forms in an FD bone, which is already soft and dysplastic, the cyst typically expands much more rapidly than FD would, leading to increased bone pain, fracture, progressive deformity, pathological fracture and neurological symptoms.

Benign myxoid bone matrix transformation in fibrous dysplasia FD. Please note a normal position of the intramedullary road in the left femur. The image demonstrates extensive myxoid degeneration of the left femur. Malignant transformation of FD lesions is a rare complication, occurring in up to 2.

Level II Ultrasound:The Fetal Skeletal System

Malignant changes to osteosarcoma, fibrosarcoma, chondrosarcoma and malignant fibrohistiocytoma have been reported. Risk factors include concomitant growth hormone excess and a history of prior radiation treatment. Worsening pain and local swelling are suspicious clinical findings. Cortical destruction, osteolysis, adjacent soft tissue mass and the development of pathological fractures may suggest the development of low-grade central osteosarcoma or malignant transformation Fig.

Making the diagnosis may be difficult, especially in cases of low-grade osteosarcoma, which shares similar histopathological features with FD. The treatment is based on management of the malignancy, and resection with adequate margins is necessary. In a group of patients with FD who presented with acromegaly, six patients developed sarcoma of the skull base 5.

An Aid to Radiodiagnosis

It is unclear if the malignant transformation was due to radiation exposure, secondary hormone factors or a combination [ 17 ]. Malignant transformation of the fibrous dysplasia FD lesion. A patient with known craniofacial FD a presented with enlarging left jaw mass b. CT of the facial bones demonstrated an aggressive lytic lesion with soft tissue component involving the left aspect of the mandible red arrows c. The pathology showed malignant transformation of the mandibular FD lesion.

Craniofacial bones are the most common FD location. The typical clinical presentation of craniofacial FD is a gradual, painless enlargement of the craniofacial region, leading to facial asymmetry. Rarely, deformities may lead to devastating functional and aesthetic consequences for affected individuals. Lesions in this location are not typically well demarcated, may cross sutures, and most commonly affect the zygomatico-maxillary complex and sphenoid bones.

Rapid enlargement of FD lesions in children and continued active disease with symptoms in adulthood is uncommon but may occur. Rare neurological complications include optic and vestibulocochlear nerve compression, scoliosis with spinal cord compression, and brain compression. Involvement of the orbit is common in FD Fig.

Proptosis, globe dystopia, and hypertelorism may be seen on imaging. Any FD lesion surrounding the optic nerve and orbit should be reported, and a comprehensive neuro-ophthalmologic examination recommended.

Gamut Index Of Skeletal Dysplasias: An Aid To Radiodiagnosis

Optic nerve encasement is common and usually asymptomatic. Among the patients with temporal bone FD, hearing loss was identified in 41 ears Hearing loss, which is typically mild, develops secondary to the narrowing of the external auditory canal and the fixation of the ossicles within the epitympanum [ 18 ] Fig. A rare, but potentially concerning, complication is the development of cholesteatomas, FD of the skull base may be associated with growth hormone excess. Sinuses may also be affected in craniofacial FD; however, the incidence of sinusitis is not greater than in the general population.

Optic nerves in craniofacial fibrous dysplasia FD in two different patients. Optic canals are narrowed but patent. The right optic canal is narrowed blue arrows. Vision in the right eye is preserved. Evaluation of hearing loss in craniofacial fibrous dysplasia FD. Brain compression in craniofacial fibrous dysplasia FD. A patient with known right parietal bone FD a develops an aneurysmal bone cyst causing mass effect on adjacent brain b — e.

FD of the spine is very rarely observed in the absence of disease elsewhere in the body. The majority of spinal FD is the polyostotic form of the disease; the monostotic FD of the spine is exceedingly rare Fig. Uncomplicated monostotic lesions are generally asymptomatic and usually do not cause significant deformity. In rare cases, severe and progressive FD can lead to severe neurological complications, respiratory compromise and even death. Classic FD findings are not seen on plain radiographs of the spine but become visible with development of vertebral collapse or deformity.

CT usually shows expansive lytic lesions with sclerotic rims and a decrease in vertebral body height. CT is also helpful in pre-surgical planning for detection of the degree of FD in each vertebral segment to be included in the fusion.

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  5. MRI findings of patients with spinal FD are typically non-specific and similar to other locations. CT and MRI may demonstrate the extent of bony disease, paraspinal soft tissue extension, compromise of the spinal canal and spinal cord compression [ 20 ].

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    The diagnosis may be difficult, especially in adult patients with monostotic form, and may require biopsy. Spinal fusion is frequently effective and may be lifesaving. Fibrous dysplasia FD of the spine. The risk of skeletal complications increases with a high skeletal burden, and in patients with MAS who have one or more endocrinopathies. Six patterns of deformity of the proximal part of the femur according to the neck-shaft angle measurement and the presence or absence of lateral bowing of the proximal femoral shaft were described Fig. The classification of femur deformities in fibrous dysplasia FD.

    Distal juxta-articular valgus deformity is also present. FD affects the entire femur. FD is the most common benign rib lesion. In adults, FD of the ribs is often discovered incidentally and is usually asymptomatic; however, it may present with obvious deformity or pain. Bone changes in MAS are often more severe than in polyostotic FD without extra-skeletal manifestations. MAS patients have the most extensive disease and the most complicated course of the disease, regularly experience multiple fractures, and require adequate surgical treatment [ 22 ].

    Eighty-five percent of female patients have functionally active ovarian cysts, resulting in gonadotropin-independent precocious puberty. A typical ultrasound finding in these patients is a large unilateral ovarian cyst, which can sometimes be haemorrhagic and appear to have mixed cystic and solid elements Fig.

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    Pedi-atric case of the day. Epiphyseal disorders. N Engl J Med ; Development of the hip in multiple epiphyseal dysplasia. Natural history and susceptibility to premature osteoarthritis. Multiple epiphyseal dysplasia: radiographic abnormalities correlated with genotype. Pediatr Radiol ; Dysplasia epiphysealis hemimelica Trevor disease in a year-old boy. Observe overgrowth of the medial femoral condyle, resulting in valgus deformity of the knee.

    There are prominent calcifications of the lateral femoral condyle, lateral tibial epiphysis, and fibular epiphysis. From Merzoug et al.