Radiographics finding in sickle cell anemia

Radiographics finding in sickle cell anemia
Dr.h.karampour

Background Sickle cell anemia is :
an autosomal recessive genetic condition characterized by a defective form of hemoglobin (hemoglobin S), which promotes the aggregation and distortion of red blood cells. Anemia results from the rapid removal of the abnormal red blood cells by the reticuloendothelial system Another consequence of the presence of abnormal circulating cells is the obstruction of microcirculation, producing ischemia and infarction. Infarcts are common in several organs and they are responsible for most clinical and radiological manifestations. Dr.h.karampour

Sickle cell anemia can affect several organs and systems
Sickle cell anemia can affect several organs and systems. Acute vaso-occlusive crisis are the most common and earliest clinical manifestation, usually appearing in the paediatric age. Although bones, brain, lungs and spleen are usually affected, vaso-occlusive crisis may occur in almost any organ. Dr.h.karampour

Findings Thoracic manifestations Musculoskeletal manifestations
Abdominal manifestations Central nervous system manifestations Dr.h.karampour

Thoracic manifestations
The lungs are frequently involved, with infarcts, emboli and increased propensity to pneumonia. Chest radiographs show a consolidation that help to confirm the clinical diagnosis . Dr.h.karampour

Dr.h.karampour

Pneumonia may lead to acute chest syndrome, which pathogenesis is probably related to infectious agents or fat emboli from an infarcting bone. Dr.h.karampour

Pleural effusion is very common either associated to pneumonia or acute chest syndrome . Dr.h.karampour

Right lateral decubitus chest radiography showing pleural effusion Dr.h.karampour

Effects of intramedullary hyperplasia. Posteroanterior chest radiograph shows a thickened trabecular pattern and loss of corticomedullary differentiation in the ribs, changes caused by hematopoietic bone marrow. Stage II avascular necrosis in the left humeral head and striation of the intramedullary cavity in the proximal left humerus also are visible. Dr.h.karampour

Chest computed tomographic (CT) scan acquired the following day helps conﬁrm right lower lobe pneumonia and right pleural effusion, as well as revealing a small area of airspace disease in the posterior left lower lobe. Dr.h.karampour

Photograph of the coronally sectioned lungs show dark peripheral area in both lungs,findings consistent with infarct ,and central ,red areas of recent pulmonary hemorrage and vascular congestion Dr.h.karampour

Chronic lung disease in an asymptomatic 14-year-old boy with SCA. Chest radiograph shows a ﬁne reticular pattern throughout both lungs and cardiomegaly (a result of the anemia). Partially visualized is a ﬂattened, irregular, right humeral head (arrow), an appearance indicative of osteonecrosis. Dr.h.karampour

Musculoskeletal manifestations
Effects of Intramedullary Marrow Hyperplasia Extramedullary Hematopoiesis Thrombosis and Infarction of Bone Epiphyseal Infarction Growth Effects Osteomyelitis and Septic Arthritis Muscle and SoftTissue Involvement Dr.h.karampour

Bone imaging modalities CT MRI Bone radionuclide scan Ultrasound Dr.h.karampour

CT 1. Can detect lesions not seen on routine radiographs or when plain film evaluation is difficult due to nature of lesion or anatomic site 2. best technique in assessment of matrix mineralization, cortical detail, and detection of the cystic and fatty Lesions 3. Can identify subtle bony abnormalities (occult fractures) and their relationship to adjacent organs 4. May fail to demonstrate early vascular and marrow abnormalities Dr.h.karampour

MRI 1. Method of choice for imaging the soft tissues of MSK system 2. Sensitive to changes in bone marrow (i.e. subtle marrow edema, marrow infarcts) 3. Can detect and stage primary bone tumors and occult bony metastases 4. Difficulty visualizing fine bone detail or small calcifications Dr.h.karampour

Bone radionuclide scan Sensitive, but nonspecific for measuring bone formation and perfusion 2. Detects lesions not otherwise seen on radiograph 3. Effective for imaging bone marrow and identifying areas of red marrow replacement 4. Effective screen for detecting multiple lesions 5. Lacks sufficient spatial resolution to detail extent and anatomic involvement of disease Dr.h.karampour

Ultrasound High frequency transducers allow markedly improved image resolution 2. Can detect joint effusions, subperiosteal fluid, soft tissue masses, congenital hip dysplasias, etc. 3. Ineffective in visualizing bone detail Dr.h.karampour

Effects of Intramedullary Marrow Hyperplasia Increased red cell destruction and consequent anemia are the main stimuli for the persistence and subsequent expansion of red (hematopoietic) marrow. Red marrow is present throughout the fetal skeleton. After birth, the red marrow undergoes a gradual conversion into yellow or fatty marrow. The process starts in the extremities of the appendicular skeleton and gradually advances into the more central skeletal structure. In a healthy adult, red marrow is present only in the axial skeleton—the spine, sternum, pelvis, ribs, and proximal long bones. As the epiphyses develop and ossify, only yellow marrow is produced in them. Dr.h.karampour

The demand for increased production of red cells in sickle cell anemia stops the conversion to yellow marrow in the peripheral skeleton and leads to the persistence of appendicular red marrow throughout life. In infants with sickle cell disease, red marrow extends to all of the bones. With increasing age, it recedes from the small bones of the extremities (hands and feet) but persists in the ankles, wrists, and shafts of the long bones . Dr.h.karampour

Dr.h.karampour

Persistent red marrow in a 29-yearold woman. Sagittal T1-weighted MR image of the spine shows low signal intensity indicative of cellular (red) marrow. Vertebral endplate concavity at multiple levels is due to bone softening. Dr.h.karampour

2. The constant stimulation of red cell production leads to the widening of medullary spaces and thinning of cortical bone, which may result in pathologic fractures. Coarsening of the normal trabecular pattern is seen in both the long and the ﬂat bones . Dr.h.karampour

Effects of intramedullary hyperplasia. Posteroanterior chest radiograph shows a thickened trabecular pattern and loss of corticomedullary differentiation in the ribs, changes caused by hematopoietic bone marrow. Stage II avascular necrosis in the left humeral head and striation of the intramedullary cavity in the proximal left humerus also are visible. Dr.h.karampour

3. Osteopenia also results from this process and may be visible on radiographs. Bone marrow expansion is manifested in the skull as a widening of the diploic space with thinning of the inner and outer tables . Dr.h.karampour

Bone marrow expansion within the skull vault. Posteroanterior radiograph obtained in a 12-year-old boy with heterozygous sickle cell disease (Hb SC) demonstrates widening of the medullary cavity with thinning of the inner and outer tables (arrows). Dr.h.karampour

4. Osteoporosis of the skull vault may produce a granular appearance on radiographs. Vertical “hair-onend” striations that project from the outer aspect of the vault also are seen. Such striations are due to the prominence of trabeculae and to new bone formation . Dr.h.karampour

Bone marrow expansion within the skull vault. Lateral radiograph shows vertical hair-on-end striations in the occipital region. The medullary cavity is not widened. Dr.h.karampour

5. in the spine, cortical thinning and softening of bone produce a smooth biconcave deformity of the vertebral bodies: Adjacent intervertebral disks compress the endplates, giving the vertebrae the characteristic “ﬁsh-mouth” appearance .Vertebral collapse with resultant kyphosis also is seen . Dr.h.karampour

Fishmouth vertebral deformities. Lateral radiograph of the lumbar spine in a young man shows the effects of bone softening and resultant compression of vertebrae by adjacent intervertebral disks (arrows). Dr.h.karampour

Extramedullary Hematopoiesis The most common site is the liver, but the spleen also may be affected, and soft-tissue hematopoietic masses may developin the thorax ,adrenal glands, and skin. Computed tomography (CT) and magnetic resonance (MR) imaging both are used to demonstrate the locations of such masses. Like normal intramedullary hematopoietic tissue, the soft-tissue masses have intermediate signal intensity on both T1-weighted and T2-weighted MR images. Radionuclide imaging with technetium 99m ( 99m Tc)-labeled sulfur colloid also is useful for identifying the hematopoietic nature of focal masses Dr.h.karampour

Extramedullary hematopoiesis. T2-weighted coronal (a) and sagittal (b) MR images of the thoracolumbar spine in a 47-year-old woman show a right-sided paravertebral soft-tissue mass (arrow). The mass had intermediate signal intensity on both T1- and T2-weighted images, similar to the signal intensity of normal intramedullary hematopoietic tissue. Vertebral endplate depression due to central infarction also is depicted. Dr.h.karampour

Thrombosis and Infarction of Bone Infarcts typically occur in the medullary cavities and epiphyses and often are the source of painful bone crises, although they also may be clinically silent and discovered incidentally at radiography. Dr.h.karampour

In infants and young children, infarction often occurs in the diaphyses of small tubular bones in the hands and feet. Infarction at these sites is termed sickle cell dactylitis or “hand-foot” syn-drome , and results from the presence and persistence of red marrow in these regions. Severe pain at such infarcted sites is thought to be precipitated by cold-induced vasoconstriction. Sickle cell dactylitis is common between the ages of 6 months and 2 years but is rare after the age of 6 years because of the regression of red marrow in these areas with increasing age . Radiographs depict patchy areas of lucency with periosteal reaction. In more severe cases, bone destruction and resultant deformity may be seen . Dr.h.karampour

Hands and feet Dr.h.karampour

Dr.h.karampour

Dactylitis in the hands of an infant. Radiograph shows periosteal new bone formation along the diaphyses of the ﬁrst three metacarpals of the right hand (arrows) and early destructive lesions in the base of the second metacarpal of both hands (arrowheads). Dr.h.karampour

Dactylitis in the feet of a 1-year-old child. Radiograph shows periosteal new bone formation along the shafts of the metatarsals in the right foot (arrows) and marked destructive changes that may lead to permanent deformity of the fourth metatarsal in the left foot. Dr.h.karampour

Both in children and in adults, the long bones are commonly affected. Acute infarcts cause osteolysis. Later, intramedullary lucency and sclerosis become evident with a patchy distribution. If cortical bone is also infarcted, subperiosteal new bone may form either through incorporation into existing cortical bone (thickening of the cortex) or through layered deposits along the inner surface of the cortex (causing a laminated, “bone-withinbone” appearance) . Dr.h.karampour

(a) Initial lateral radiograph shows periosteal new bone formation along the tibial diaphyses (arrows), particularly in the upper third of the right tibia, with normal bone texture. (b) Lateral radiograph obtained 6 weeks later shows distortion of the bone texture, with incorporation of the region of periosteal reaction into cortical bone and resultant cortical thickening in both tibiae, as well as linear serpiginous areas of sclerosis in the left tibial shaft. These are typical radiographic features of chronic infarction Dr.h.karampour

The appearances of infarction at scintigraphy with 99m Tc methylene diphosphonate (MDP) vary with time and may be difﬁcult to interpret if the duration of symptoms is not known at the time of scanning. In the ﬁrst few days after infarction, decreased or normal radiotracer uptake is seen . Dr.h.karampour

Other bones, such as the pelvis, ribs, and spine, may become markedly sclerotic be cause of medullary infarction as dystrophic medullary calciﬁcation occurs and new bone is laid down on infarcted bone . Dr.h.karampour

In the spine, MR imaging has good sensitivity for depicting ischemic changes of the vertebral endplates and for demonstrating edema in acute infarction Dr.h.karampour

Infarction of the ribs in patients with sickle cell disease Dr.h.karampour

Epiphyseal Infarction frequently seen in the femoral and humeral heads, and more often bilateral than avascular necrosis in other diseases. Lesions quantiﬁed on MR images also are larger . Frequently, initial radiographs appear normal, and the earliest signs of avascular necrosis are seen on MR images (in particular, T2-weighted inversion recovery images), which show regions of high signal intensity indicative of bone marrow edema. Often, a serpiginous double line that consists of a hyperintense inner border and hypointense periphery can be seen at T2-weighted imaging. The “double line” sign results from the highsignal-intensity inﬂammatory response of bone with granulation tissue, inside the low-signalintensity reactive bone interface . As osteonecrosis progresses, changes become evident at radiography. Early radiographic signs include lucency and sclerosis within the epiphysis; subsequently, crescent-shaped subchondral lucencies develop; and eventually, depression of the articular surface, collapse, and fragmentation occur. Dr.h.karampour

(a) Anteroposterior radiograph obtained in a 44-year-old man shows stage IV avascular necrosis in the left hip and a normal right hip. (b, c) Coronal T2weighted short inversion time inversion recovery MR images show stage I avascular necrosis in the right hip as well as ﬂattening of the left femoral head (c),a feature that helped conﬁrm the diagnosis of stage IV avascular necrosis. Dr.h.karampour

Growth Effects The effects of sickle cell anemia on growth are thought to result from bone infarction. Epiphyseal shortening arises from vascular compromise, which causes damage to the growth plate, slowing or halting cartilage growth and leading to shortened bone . Dr.h.karampour

1.Marked carpal deformities in the left wrist in a young woman. Radiograph shows the fusion of several intercarpal joints, a condition that affected the patient’s grip. 2. Growth disturbance in the distal radius in a 12year-old girl. Anteroposterior radiograph of the left wrist shows epiphyseal shortening and a cup deformity of the adjacent metaphysis Dr.h.karampour

Premature fusion of growth plates often occurs centrally because of the ingrowth of metaphyseal vessels; however, in tibiotalar slant deformity, premature fusion occurs laterally because of local ischemia (1). As a result, slanting of the articular surfaces of the distal tibia and talus is seen . Dr.h.karampour

Epiphyseal necrosis Dr.h.karampour

In the spine, endplate depressions of the vertebral bodies are another manifestation of growth disturbance caused by ischemia and infarction. The H-shaped vertebral deformity is thought to be a result of central growth plate infarction. It can be distinguished from marrow hyperplasia by the characteristic sharp steplike appearance of the vertebral endplate Dr.h.karampour

lateral frontal In the spine, infarction of the central growth plate of the vertebral bodies cause endplate depressions. As a consequence, multiple vertebras became H-shaped Dr.h.karampour

H-shaped vertebrae Dr.h.karampour

lytic and sclerotic areas with a patchy distribution in the tibial shaft Dr.h.karampour

If cortical bone is also infarcted, subperiosteal new bone may form, producing periosteal reaction the periosteum is separated from the tibial cortex Dr.h.karampour

separation of the periosteum from the tibial cortex Dr.h.karampour

thickening of the cortex, causing a laminated, bone-within-bone“ appearance
Dr.h.karampour

Osteomyelities Osteomyelitis and septic arthritis are frequent and serious complications of sickle cell disease. It is most common in the diaphysis of femur, tibia and humerus. Vertebras may also be involved. Radiographic findings features are also nonspecific and initially are often normal. Signs of periosteal inflammation, osteopenia and sclerosis may take 8 to 10 days to become evident and are seen both in infarction and infection. Ultrasonography may be useful on characterization of soft-tissue changes and fluid collections. Although a small volume of pure-fluid may be expectable adjacent to infarcted bone, a larger, non-pure fluid collection favors the diagnosis of osteomyelitis . Dr.h.karampour

Musculoskeletal ultrasound performed with a high-frequency probe showing a fluid-collection adjacent to the tibial shaft. The dimensions of the collection (6mm thickness) and the non-pure fluid content are sugestive of osteomyelitis. Dr.h.karampour

Chronic osteomyelitis. Anteroposterior (a) and lateral (b) radiographs of the left tibia and ﬁbula in an infant show an involucrum that surrounds the shaft of the ﬁbula. Dr.h.karampour

Osteomyelitis in a 19-year-old woman with left distal tibial and ankle pain. Anterior views of the lower legs, obtained with 99m Tc MDP scintigraphy in the dynamic phase (a), blood pool phase (b), and late static phase (c), show increased tracer uptake in the region of the left ankle (arrowhead), a ﬁnding suggestive of infection. In c, a slight uptake asymmetry between the upper tibiae also is visible. This appearance represents infarction of the left tibia (arrow) with remodeling. Dr.h.karampour

Osteomyelitis of the left femur in a 24year-old patient with sickle cell disease. Axial gadolinium-enhanced T1-weighted fat-suppressed MR image shows heterogeneous marrow enhancement, a rounded low-signal-intensity ﬂuid collection adjacent to the shaft, and enhancement of the soft tissues around the shaft and of the adjacent musculature. The areas of enhancement are likely to be infected. Dr.h.karampour

Chronic osteomyelitis in a 19-year-old woman with known homozygous sickle cell disease. Coronal T2-weighted inversion-recovery MR image obtained at a follow-up examination shows cortical thickening and medullary high signal intensity in the left femur as well as multiple soft-tissue ﬂuid collections (arrows) that were later found to be abscesses. Dr.h.karampour

Acute bone infarction. Axial T2-weighted MR images of the femur in a 13-year-old boy with leg pain. (a) Initial image shows regions of both high and low signal intensity within the medullary cavity and high signal intensity in the vastus intermedius muscle, features that may represent either infarction or infection. Infection was not suspected clinically. (b) Follow-up image obtained after 4 weeks of standard management shows resolution of the area of low signal intensity within the medullary cavity and the area of high signal intensity in the adjacent muscle, a ﬁnding indicative of infarction. Dr.h.karampour

Septic arthritis in a 19-year-old woman with left-sided hip pain and fever. (a, b) Axial T2-weighted inversion recovery MR images show a left hip effusion (arrow in a) that extends into a multilocular ﬂuid collection adjacent to the anterior margin of the hip (b). Diffuse edema also is visible within the superﬁcial soft tissues lateral to both hips. Dr.h.karampour

Muscle and SoftTissue Involvement . (a) Longitudinal high-frequency (12-MHz) US image of the left ankle shows a hypoechoic collection (arrow) with some internal echoes and layering, located approximately 1 cm deep to the Achilles tendon. Thick pus was aspirated from this area with US guidance. (b) Sagittal T1-weighted MR image shows a low-signal-intensity fluid collection within the pre-Achilles fat space (arrow). Areas of intermediate signal intensity due to the persistence of appendicular red marrow in the medullary cavities of the distal tibia and calcaneum (arrowhead) make the assessment of any changes due to bone involvement difficult; therefore, this sequence cannot be used to exclude osteomyelitis. (c) Sagittal T2-weighted inversion recovery MR image shows the high-signal-intensity fluid collection in the pre-Achilles space (arrow) and a small ankle joint effusion. Bone involvement may be excluded on the basis of this image. Dr.h.karampour

Muscle and SoftTissue Involvement
Dr.h.karampour

Central nervous system manifestations
Neurological manifestations of SCD are common and include symptomatic infarction, silent ischaemia and intracranial haemorrhage Vasculopathy Territorial Infarction Silent Cerebbral Iscemia Intra Cranial Hemorrage Dr.h.karampour

VASCULOPATHY This is the hallmark feature of the disease and the underlying cause for many of its radiological manifestations. Sickle cell vasculopathy can involve both large and small vessels, although typically the terminal internal carotid artery (ICA), proximal anterior cerebral artery (ACA) and middle cerebral artery (MCA) are affected leading to stenosis. Over time and with progressive occlusion of the main intracranial arteries, asocalled“Moya Moya” (Japanese: puff of smoke) appearance is seen, which is characterized by the formation of numerous tiny collaterals. Dr.h.karampour

Vasculopathy of the main intracranial arteries may result in compensatory dilatation of pial vessels, which can manifest itself on ﬂuid-attenuated inversion–recovery imaging as a characteristic “ivy sign”. Dr.h.karampour

Dr.h.karampour

TERRITORIAL INFARCTION Dr.h.karampour

SILENT CEREBRAL ISCHAEMIA
(a) T2 and fluid-attenuated inversion–recovery (FLAIR) images demonstrating increased signal involving the cortex and white matter of the right parietal lobe and in the watershed zones of both frontal lobes. Corresponding diffusion-weighted imaging (DWI) image and apparent diffusion coefficient (ADC) map showing restricted diffusion in the right parietal lobe abnormality indicating an acute infarct. The frontal lobe changes represent chronic watershed infarction. (b) T2 weighted images at resentation and 2 years later demonstrating progressive silent ischaemia, most marked in the left frontal lobe. Dr.h.karampour

SILENT CEREBRAL ISCHAEMIA Dr.h.karampour

Intracranial hemorrage Dr.h.karampour

Abdominal manifestations
Pigmented gallstones are the most common gastrointestinal manifestation of sickle cell anemia and are observed in around 50% of patients . Dr.h.karampour

The spleen is also frequently involved. It has a slow, tortuous microcirculation that favours sickling of red blood cells and, as consequence, splenic infarctions are very common. Over time, multiple infarctions progress to autosplenectomy. At imaging, a small, calcified spleen is seen. Dr.h.karampour

Dr.h.karampour

Another splenic complication is sequestration syndrome. Dr.h.karampour

The kidney is also susceptible for sickling of red blood cells and infarction, especially in the medulla, where vasa recta flow through a hypertonic interstitium. Capillary obliteration result in medullary and papillary necrosis. At imaging, up to 50% of patients have enlarged kidneys. At ultrasonography kidneys may have normal echogenicity, may be diffusely hyperechogenic or may have increased medullary echogenicity with normal cortical echogenicity Dr.h.karampour

Dr.h.karampour

Thanks for your attention
Dr.h.karampour

Radiographics finding in sickle cell anemia

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