41 DAVID SUTTON

DAVID SUTTON PICTURES

DR. Muhammad Bin Zulfiqar PGR-FCPS III SIMS/SHL

• Fig. 41.1 Thalassaemia.(A) Lobulated soft-tissue masses due to extramedullary haematopoiesis are present adjacent to the thoracic spine. (B) In another patient, paravertebral extramedullary haematopoietic tissue is shown in the lower sections of a CT examination of the thorax.

• Fig. 41.2 Thalassaemia (boy aged 7). Gross marrow hyperplasia has expanded and thinned overlying cortical bone. Medullary trabeculae have been destroyed and the residual ones are coarsened. Inset-early changes of the same type in a finger of a child aged 4.

• Fig. 41.3 Thalassaemia (boy aged 15). A chest film shows gross expansion of bone structures due to marrow hyperplasia. Note particularly involvement of the ribs and scapulae.

• Fig. 41.4 Thalassaemia. Considerable bone expansion, cortical thinning and simplification of trabecular pattern are demonstrated in the forearm of a boy of 15.

Fig. 41.5 Thalassaemia. Considerable marrow expansion has produced a flask shape of the distal femur. The coarsened trabecular pattern and cortical thinning are obvious

• Fig. 41.6 Thalassaemia. Thickening of the outer table of the skull in the frontal area with perpendicular striation-'hairbrush sign'.

• Fig. 41.7 Sickle cell disease. Infarction in the proximal femoral metaphysis has produced a large defect with avascular necrosis of the femoral head. These features are similar to those of Perthe's disease.

• Fig. 41.8 Sickle cell disease. Endosteal bone deposition has resulted in diffuse sclerosis beneath the articular surface (the 'snow-cap' sign) due to medullary infarction. Note the lack of distinction between cortical and medullary bone in the upper humeral shaft, again due to endosteal deposition of bone.

• Fig. 41.9 Sickle cell disease. A bone scan performed 16 hours after the onset of severe pain in a boy with known sickle cell disease. Acute infarction of L2 has resulted in a relative photon deficiency in this area. Previous infarctions, in varying phases of evolution, are shown as areas of increased activity (see particularly L1 and midthoracic vertebrae).

• Fig. 41.10 Sickle cell disease. Soft-tissue swelling surrounds an expanded proximal phalanx. Medullary expansion is present with simplification of trabecular pattern and penetration of the cortex. The distinction between these changes and osteomyelitis is extremely difficult.

• Fig. 41.11 Sickle cell disease, infarction in childhood. (A) At presentation, periosteal new bone formation surrounds the diaphysis of the fourth finger metacarpal. (B) Ten months later resolution has occurred and growth has proceeded normally. The distinction between infarction and infection may be very difficult. In this case no specific treatment was given.

• Fig. 41.12 Sickle cell disease. Flat depressions within the vertebral bodies with sloping sides typify metaphyseal infarct ('the vertebral step sign' or H-shaped vertebra). Frank destruction of the vertebral body with narrowing of the contiguous disc spaces is due to associated salmonella osteomyelitis.

• Fig. 41.13 Sickle cell disease with salmonella osteomyelitis. (Nigerian boy aged 4). Extreme destructive changes in the long bones have been caused by infection superimposed upon infarction. Numerous sequestra are present.

( Courtesy of Mr. Joeffery Walker)

• Fig. 41.14 Acute leukemia. Extensive metaphyseal radiolucencies are present with adjacent periosteal new bone formation.

• Fig. 41.15 Lymphatic leukaemia. Metaphyseal radiolucencies are present around the knee. Endosteal sclerosis is present adjacent to these lesions, obscuring the corticomedullary junction. Minor periosteal new bone formation is present in the upper tibia and fibula.

• Fig. 41.16 Lymphatic leukaemia. (A) Erosions of the medial side of the proximal metaphyses of both humeri were present in this 8 year old. The disease was in an aleukaemic phase, not an uncommon finding even when skeletal changes are present. (B) The same child complained of back pain. Multiple vertebral collapse is shown with the preservation of disc-space height. Overall bone density is reduced with a simplified trabecular pattern.

• Fig. 41.17 Chronic lymphatic leukaemia-adult type. Diffuse medullary infiltration is shown in the humerus and scapula with cortical erosion.

• Fig. 41.18 Myeloid metaplasia Widespread but patchy areas of sclerosis are shown throughout the pelvis and lumbar spine.

• Fig. 41.19 Coronal intermediate weighted (A) and T2 –weighted (B) MR images of the knee in a patient with myelofibrosis showing replacement of the normal high-signal-intensity fatty marrow by fibrosis. This, and the abnormal bone deposition around the trabeculae, results in the diffuse low signal intensity from the medullary cavity.

• Fig. 41.20 Myeloid metaplasia (woman aged 63). All the bones are diffusely dense with lack of distinction between cortical and medullary bone. The spleen is grossly enlarged (arrows).

• Fig. 41.21 Hodgkin's disease. An expanding, destructive lesion involves the body of the sternum, with anterior and posterior soft-tissue masses. Bizarre changes in this bone should always arouse suspicion of a lymphoma.

• Fig. 41.22 Hodgkin's disease. (A) The common pattern of endosteal sclerosis and patchy bone destruction is shown in the vertebral body of T9 in an adult man. Similar changes are also present at TI 1 . These features are virtually diagnostic. (B) In another patient, an intravenous enhanced CT section of the abdomen demonstrates a densely sclerotic lumbar vertebral body associated with a large paravertebral soft-tissue mass. Sagittal (C) and coronal (D) T,- weighted images show low signal intensity within the upper lumbar vertebral body due to diffuse sclerosis and the extent of the soft-tissue mass. This extends into the central spinal canal and circumferentially around the thecal sac.

• Fig. 41.22 Hodgkin's disease. (A) The common pattern of endosteal sclerosis and patchy bone destruction is shown in the vertebral body of T9 in an adult man. Similar changes are also present at TI 1 . These features are virtually diagnostic. (B) In another patient, an intravenous enhanced CT section of the abdomen demonstrates a densely sclerotic lumbar vertebral body associated with a large paravertebral soft-tissue mass. Sagittal (C) and coronal (D) T,- weighted images show low signal intensity within the upper lumbar vertebral body due to diffuse sclerosis and the extent of the soft-tissue mass. This extends into the central spinal canal and circumferentially around the thecal sac.

• Fig. 41.23 Hodgkin Disease. A) Diffuse sclerosis is present in the bodies of L2 and L3 in a young woman at presentation with the disease. (B) Two years later, following treatment, the appearances have reverted to normal. (Lymphographic contrast medium is present in para-aortic nodes.)

• Fig. 41.24 Hodgkin's lymphoma. T,-weighted (A) and STIR (B) sagittal images demonstrate extensive abnormality of the marrow of the lumbar spine. In addition, a huge mass of lymph nodes is demonstrated anteriorly, wrapped around the abdominal aorta and displacing the superior mesenteric artery. An axial image (C) demonstrates not only body and left ala involvement of the sacrum, but also subcutaneous and spinal canal extension of the tumour. The thecal sac is displaced to the right.

• Fig. 41.25 Hodgkin's disease. A typical anterior scalloping of L4 is due to pressure erosion from enlarged lymph nodes. The cortex is preserved, as are the disc spaces.

• Fig. 41.26 Non-Hodgkin's lymphoma. A conventional radiograph (A) is virtually normal save for the slight suggestion of patchy ill-defined bone destruction. Subsequent T,-weighted coronal (B) and axial (C) MR images demonstrate not only extensive marrow replacement but also a substantial enveloping soft-tissue mass. The degree and extent of tumour involvement of bone was virtually impossible to appreciate from the radiographic examinations.

• Fig. 41.26 Non-Hodgkin's lymphoma. A conventional radiograph (A) is virtually normal save for the slight suggestion of patchy ill-defined bone destruction. Subsequent T,-weighted coronal (B) and axial (C) MR images demonstrate not only extensive marrow replacement but also a substantial enveloping soft-tissue mass. The degree and extent of tumour involvement of bone was virtually impossible to appreciate from the radiographic examinations.

• Fig. 41.27 Non-Hodgkin's lymphoma. A purely destructive lesion is present in the distal femur of a woman patient. The margins are ill defined with cortical destruction. Periosteal new bone formation is present adjacent to this destruction. These appearances resemble metastasis and osteosarcoma.

• Fig. 41.28 Non-Hodgkin's lymphoma. Advanced changes are shown in the femoral shaft, with dramatic resolution 11 months later following local radiotherapy.

• Fig. 41.29 Non-Hodgkin's lymphoma. Extensive patchy destruction of the cranium was present in this adult patient with generalised disease.

• Fig. 41.30 (A) CT of the pelvis in non-Hodgkin's lymphoma showing diffuse sclerosis within the right innominate bone, with ill-defined periosteal bone formation on the inner surface. A soft-tissue mass is evident. (B) The extent of these changes is easier to appreciate on the axial STIR image, where additional lesions within the left innominate bone are also evident.

• Fig. 41.31 (A,B) Non- Hodgkin's lymphoma. Multifocal disease was found at presentation in an elderly patient with low back pain. In addition to a pathological fracture of a lumbar vertebral body, ill-defined endosteal defects are present in the femoral shaft (arrows).

• Fig. 41.32 Burkitt's tumour. A large destructive lesion in the mandible of this African child is typical of this form of lymphoma.

• Fig. 41.33 Mastocytosis (man aged 34). A localised area of endosteal sclerosis is present in the body of Lt. In addition, ill defined thinning of trabeculae is demonstrated in L2 and patchier changes in the upper surface of L3.

• Fig. 41.34 Mastocytosis. A coarse pattern of generalised sclerosis is shown.

• Fig. 41.35 Plasmacytoma of sacrum. (A) An adult man exhibits a well defined radiolucent defect involving the left sacral ala. (B) A CT scan demonstrated the extensive destructive nature of the tumour, seen clearly to cross the midline. Note the marked cortical thinning with absence of sclerosis or periosteal new bone formation.

• Fig. 41.36 Plasmacytoma of pelvis. This very extensive lesion was unaccompanied by any systemic abnormality. Bone expansion is associated with coarse trabeculation, producing a soap-bubble appearance. It is much more common for the widespread form

• Fig. 41.37 Plasmacytoma presenting with paraparesis. (A) Conventional radiograph of the thoracic spine showing vertebra plana (arrows). (B) CT demonstrating the degree of bony destruction, associated paravertebral mass and marked posterior extension into the spinal canal resulting in severe cord compression.

• Fig. 41.38 Plasmacytoma. (A) Conventional cervical spine radiograph showing osteolytic destruction of C6 with pathological collapse of the vertebral body. (B) Sagittal T 1 -weighted MR image showing the intermediate signal- intensity lesion of C6 resulting in focal extradural compression of the spinal cord.

• Fig. 41.40 Myelomatosis. Diffuse marrow involvement has resulted in an overall reduction in bone density similar to that seen in osteoporosis. However, the rather patchy nature of radiolucencies should raise the possibility of myeloma.

• destructive Fig. 41.41 Typical localised lesions of myeloma are demonstrated in the upper femur of an adult woman. The sharply defined rounded defects with endosteal erosions of the cortex are characteristic.

• Fig. 41.42 Histiocytosis. A purely osteolytic lesion is present in the mandible, with well-defined, slightly scalloped margins. The lamina dura has been destroyed. The teeth seem to 'float in air'.

• Fig. 41.43 Histiocytosis. (A) Lateral skull radiograph showing a well-defined osteolytic lesion with a narrow zone of transition and no sclerosis in the posterior parietal region. (B) Osteolytic lesion of the innominate bone with ill-defined peripheral sclerosis. (C) CT of the innominate lesion showing destruction of the anterior cortex of the iliac wing and the ill-defined surrounding sclerosis of this healing lesion.

• Fig. 41.44 Histiocytosis. Extensive involvement of a bone, here the clavicle, is often associated with layered periosteal new bone causing bony expansion. Ill-defined areas of resorption may be visualised in the lesion. This was the only abnormality found in a young girl over several years' follow-up.

• Fig. 41.45 Histiocytosis. Vertebral lesions in the thoracic spine are shown on a lateral tomogram. The bodies of T7 and 8 have collapsed with a slight increase in bone density. Note the relative preservation of the disc spaces.

• Fig. 41.46 Histiocytosis. A healing diaphyseal lesion exhibits periosteal new bone formation and minimal sclerosis around the margins of the radiolucency.

• Fig. 41.47 Histiocytosis. Extensive skull involvement in a child with the Hand-Schuller-Christian type of lesion. The areas of destruction in the flat bones of the skull have a map-like configuration.

• Fig. 41.48 Histiocytosis: Hand-Schuller-Christian type. Very extensive radiolucencies are present both in the metaphysis and diaphysis of this child's femur. A healed pathological fracture is present. Histiocytosis should always be considered in the differential diagnosis of bizarre bone lesions.

• Fig. 41.49 Histiocytosis. Adult pulmonary involvement (man aged 20). (A) A localised view from a chest radiograph demonstrates a coarse interstitial pulmonary fibrosis. Note also a pathological fracture of the left fourth rib due to a bony deposit. (B) A CT scan demonstrates peripheral interstitial pulmonary fibrosis with thickened interlobular septa and irregular honeycombing.

• Fig. 41.50 Histiocytosis: Letterer-Siwe type. Massive destructive lesions are present throughout the skeleton, but affect particularly the metadiaphyseal areas of the long bones. A similar appearance could be produced by metastases from a neuroblastoma or the advanced stages of leukaemia.

• Fig. 41.51 Gaucher's disease (woman aged 20). Abnormal modelling of the distal ends of the femora has resulted in a typical flask-shaped appearance. An osteolytic lesion with a coarse trabecular pattern is present in the right femur.

• Fig. 41.52 Gaucher's disease. Infarctions in vertebral bodies have produced the 'bone within a bone' appearance throughout the lumbar spine in this child.

• Fig. 41.53 Gaucher's disease-acute bone infarction. (A) A radiograph of a 1 3-year-old girl, with known Gaucher's disease, presenting with acute hip pain of 12 hours' duration. Slight endosteal sclerosis is shown in the inferior pubic ramus and an area of ill-defined radiolucency in the intertrochanteric region. (B) The delayed phase of a bone scan reveals the femoral head and neck to be markedly photon deficient consistent with acute infarction. Abnormally increased activity is present also at site of previous disease.

• Fig. 41.54 Gaucher's disease. This adolescent has considerable deformity of the femoral head and acetabulum secondary to episodes of infarction. Evidence of degenerative arthritis is already present.

• Fig. 41.55 Haemophilia. The former epiphyses are disproportionately presenting a 'squared' appearance. Hyaline cartilage thickness at the ankle joint is reduced.

• Fig. 41.56 (A,B) Haemophilia. Typical appearances in an adult patient subject to recurrent haemarthroses since childhood. As well as the enlarged, squared appearance of the former epiphyses, hyaline cartilage width is reduced, and osteophytes are present due to secondary osteoarthritis. Areas of radiolucency within medullary bone probably represent old intraosseous haemorrhages.

Fig. 41.57 (A,B) Haemophilia. Repeated intra-articular haemorrhages have caused overgrowth of the epiphyses, particularly the head of the radius. The joint capsule is distended and the synovium is amorphously dense due to the deposition of haemosiderin from recurrent haemarthroses. A subarticular cyst is present in the olecranon fossa, and degenerative changes, in the form of hyaline cartilage thinning and osteophyte formation, are present.

• Fig. 41.58 Haemophilia. Sagittal T,-weighted image showing loss of hyaline cartilage over the medial compartment and very low signal intensity synovium due to haemosiderin deposition.

• Fig. 41.59 Haemophiliac pseudotumour. A huge destructive lesion in the tibia, with relatively well-defined margins, is associated with some periosteal reaction. Although an initial impression may be of a malignant tumour, changes of haemophilic arthropathy can be seen in the knee and ankle.