short case 9

8/8/2019 Short Case 9

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Short case 9/10


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47-year-old man

Hemodialysis

dyspnea


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HRCT in mediastinal window


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47year-old man with metastatic

pulmonary calcification due to chronic

renal failure. High-resolution CT image (1-

mm collimation, high-spatial-frequency

reconstruction algorithm) lung window

shows diffuse fluffy, poorly defined

nodules in the upper lobes.


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renal failure. High-resolution CT image in

mediastinal window shows parenchymal

calcifications.


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47year-old man with metastatic pulmonary

calcification due to chronic renal failure. High-

resolution CT image in mediastinal window at

the level of the carina shows multiple, ill-defined,calcified nodules (arrows). There are also

calcifications in the bronchial and tracheal walls.

Back


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lung window shows symmetric,

centrilobular, calcified nodules (short

arrow) and ground-glass opacity (long

arrow) in the lower lung zone.


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mediastinal window shows symmetric,

centrilobular, calcified nodules in the lower

lung zone.


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Computed Tomography (Open in original

size)



renal failure. Abdomen CT shows renal

atrophy (white arrow) and vascular

calcification (black arrow).


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Metastatic calcification refers to the deposition of calcium in normaltissue. The lung is one of the primary sites of metastatic calciumdeposition (1). Metastatic calcifications of lung parenchyma arerelated to chronically elevated serum calcium-phosphorus productas in chronic renal failure, primary hyperparathyroidism, Dhypervitaminosis, milk alkali syndrome or diffuse myelomatosis (2).

Calcium salts are predominantly deposited in the alveolar walls, andto a lesser extent in bronchial wall, pulmonary arteries, and veins(3). Calcium preferentially deposits in relatively alkaline tissues;therefore, it is not surprising that the lung apex is more commonlyinvolved than the lung base (2). The degree of respiratory distressoften does not correlate with the degree of macroscopic calcification.Patients with extensive calcification may be asymptomatic, while

others with subtle calcification or normal chest radiographs mayhave severe respiratory compromise


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Metastatic pulmonary calcification is a well known complication of end-stage renalfailure and its treatment (5). While common at autopsy in patients with renal failure,the process is often undiagnosed antemortem. Many patients with MPC areasymptomatic, but in some cases can cause fulminant respiratory failure and earlydeath. Symptoms include dyspnea and chronic, non-productive cough (4).Because chest radiograph is insensitive in depicting small amounts of calcification, itis frequently normal. MPC has been described as confluent or patchy airspace

opacities simulating pulmonary edema or pneumonia on chest radiographs. MPC canalso appear as a diffuse interstitial process or as discrete or confluent calcifiednodules (2, 6).HRCT, with its excellent sensitivity in the detection of small amounts of calcification,is being increasingly used to diagnose MPC. Several CT patterns have beendocumented to date. The first pattern is multiple diffuse calcified nodules that areeither distributed throughout the whole lung or show a predilection for the apices. Thesecond pattern is diffuse or patchy areas of ground-glass opacity or consolidation.Finally, MPC may appear as a confluent high attenuation parenchymal consolidation

in a predominantly lobar distribution, mimicking lobar pneumonia. Associated findingsinclude calcification in the bronchial walls, myocardium and within the vessels of thechest wall (2, 6). In our case showed multiple, symmetrical, centrilobular, calcifiednodules and patchy areas of ground-glass opacity throughout both lungs.

Additionally, this case also showed calcification in the bronchial and tracheal walls


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Multifocal pulmonary parenchymal calcification is associated withinfection (varicella zoster tuberculosis, histoplasmosis), MPC,silicosis, diffuse parenchymal amyloidosis, alveolar microlithiasis,haemosiderosis secondary to mitral stenosis and fat embolismassociated with adult respiratory distress syndrome. It also occurs intreated metastases and in metastatic malignancies such as

osteogenic sarcoma, chondrosarcoma, mucin-producingadenocarcinomas and thyroid malignancies (7). The calcifiednodules in diffuse parenchymal amyloidosis are locatedpredominantly in the subpleural areas of the mid and lower zonesand are associated with hilar lymphadenopathy, interlobular septalthickening, and consolidation and ground-glass opacities. Thecalcified pulmonary nodules in alveolar microlithiasis are smaller

(about 1 mm in diameter), typically occur in the lower zones and theparacardiac regions and may be associated with apical bullae andsubpleural cysts. Tuberculoma usually presents as a solitary well-defined calcified nodule..


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Miliary tuberculosis is characterized by

small, well-defined, discrete nodules, 1-2

mm in diameter, evenly distributedthroughout both lungs. Occasionally, some

may calcify. Hilar lymphadenopathy with a

peripheral egg-shell calcification iscommon in silicosis (8). renal failure is

potentially reversible, and may resolve

after parathyroidectomy, renal transplant,

or adequate dialysis. Resolution ofsymptoms has been seen after correction

of hypercalcemia well (4).


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The most likely cause for multifocalpulmonary parenchymal calcification in thepatients with chronic renal failure is MPC.

The predilection of calcification for theupper lung area and associated withcalcification in the bronchial walls,myocardium and within the vessels of the

chest wall may be supported the diagnosisofMPC.Pulmonary calcification associated with


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In conclusion, HRCT is a valuable imaging

technique in the diagnosis ofMPC. HRCT

may obviate the need for open lung

biopsy. Therefore, it is important for the

radiologist to recognize the HRCT patterns

of this disease process afflicting patients

with chronic renal failure


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Metastatic pulmonary calcification characterizedby diffuse calcium deposition in the lungs isknown to occur in patients with chronic renalfailure. We present a case of a 47-year-old man

with chronic renal failure presented withdyspnea, high-resolution computed tomographyof the chest revealed multiple, centrilobular,calcified nodules and patchy areas of ground-glass opacity throughout both lungs, consistent

with metastatic pulmonary calcification.Calcification was also seen in the bronchi andtrachea.


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Metastatic Pulmonary Calcification in a

Patient with Chronic Renal Failure


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ray (AP view) (A) femur was apparently

normal however, in retrospective it showed

a faint ill-defined sclerotic lesion (arrow),

latest x-ray (B) showed a cortical basedwell defined lytic lesion in the right

proximal femoral diaphysis with sclerotic

margins (arrow).


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Plain radiograph of the pelvis in a 6-year-

old child who presented with left hip pain.

The radiograph shows a well-defined area

of sclerosis surrounded by a ring ofradiolucency in the left femoral neck. Note

the absence of periosteal reaction that

suggests intramedullary or cancellousosteoid osteoma.


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Radiograph of the right great toe in a 24-

year-old woman shows a partly calcified

lesion in the medulla of the distal phalanx,

with no periosteal new bone formationsuggestive of osteoid osteoma


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Lateral view of the thoracic spine in a 56-year-oldwoman who presented with abdominal pain. Initialfindings of chest radiography, intravenous pyelography,barium enema, and cholecystogram were normal. An

isotope bone scan showed focal increased uptake in thevertebral body of the lower thoracic spine. Plainradiograph corresponding to the site of the abnormalityshows a radiolucent lesion in the posterior aspect of theT11 vertebral body with surrounding sclerosis that wasdiagnosed as giant osteoid osteoma and later wasconfirmed at histologic analysis


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Radiograph of the hip in an 8-year-old

child who presented with left hip pain and

restriction of movement. An intra-articular

osteoid osteoma in the medial aspect ofthe femoral neck has resulted in periosteal

new bone formation


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Anteroposterior radiograph of the

lumbar spine in a 52-year-old man who

had a biopsy-proven giant osteoid

osteoma (osteoblastoma) and whopresented with severe low back pain.

Radiograph shows a well-defined

sclerotic lesion in the left side of the L4vertebral body.


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Twin girls with growth retardation

age of nine months


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DP radiograph of the hand of one of the

twins, showing shortness of both the

metacarpals and phalanges, with lack of

tubulation.


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Coned hand radiograph of the second

twin, demonstrates cone-shaped

epiphyses of 3rd and 4th proximal

phalanges


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The forearm bones are shortened with

respect to the humerus, and the bones are

under-tubulated. In addition, the radius is

shorter than the ulna


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Lateral skull radiograph showing large

calvarium with frontal bossing.


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Lateral radiograph of whole spine,

showing mild thoracolumbar kyphosis.


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Lateral radiograph of vertebrae showing

oval shaped vertebral bodies, with beaking

of the anterior portions.


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X-ray findings (Fig. 3-8):

1. Hands: Phalanges and metacarpal bones were shortand thick. Epiphyses were cone-shaped. Carpal boneswere normal and appropriate to age.

2. Long tubular bones:Radius and ulna weredisproportionately shorter than humerus, and radius wasshorter than ulna. Lower extremities were normal.3. Spine: Vertebral body height were decreased, andcentral protrusion of body anterior portion (beaking) wasseen. Interpedicular distance was normal. Mild kyphosis

was present at lower thoracic region.4. Calvarium: Dolicocephaly with prominent frontal andoccipital bones was seen. There was frontal bossing.Facial bones were normal


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Skeletal dysplasias comprise a group of

hereditary disorders characterised with growth

and development disturbances of cartilage and

bones. They can be epiphyseal, metaphyseal ordiaphyseal. The disease results in abnormal,

and disproportionate shape and length of

affected bones. Though molecular tests are

available for some of its variants, radiologicaland clinical findings are very important for the

diagnosis [


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Acromesomelic dysplasia is a rare form of

skeletal dysplasia, with autosomal

recessive inheritance pattern. The disease

is characterised by both acro- andmesomelia. Other bone abnormalities

affecting calvarium or vertebrae may also

be present


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Characteristic radiological findings occur within the firstor second year of life: cone-shaped epiphyses of hands,shortness of radius comparing to ulna, short and broadphalanges, oval-shaped vertebrae with anterior beaking,and frontal bossing of calvarium are the radiologicalcharacteristics of the diagnosis. Hypoplasia of the baseof the iliac bone, irregular ossification of superioracetabulum and superior inclination of clavicles may alsoaccompany. Fusion of growth plate in hands, bendingdeformity of radius, dorsal subluxation of radial head,widening of central protrusion of vertebrae, posteriorwedging and increase in severity of kyphosis may occuror become more pronounced during the following years


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Lymphangitic carcinomatosis.

Posteroanterior chest radiograph shows

numerous bilateral linear opacities.


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Pleural effusion, interlobular septal

thickening, nodular thickening of the right

major fissure


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Diffuse reticulonodular opacities and a mass at the right middle lung


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p g gwere depicted at the chest radiograph (not shown). Furtherinvestigation with CT and HRCT of the lungs was performed.CT-HRCT revealed: (1) bilateral pleural effusion, (2) mediastinal

lymphadenopathy, (3) nodule with spiculated margins in the rightlower lobe, (4) smooth and nodular thickening of the interlobularsepta, (5) nodular thickening of the peribronchovascularinterstitium,in the right middle and lower lobe, (6) nodular thickeningof the right major fissure and (7) subpleural nodules.Imaging findings were compatible with pulmonary lymphangiticcarcinomatosis. The nodule in RLL was considered to be the

possible cause.Bronchoscopy confirmed the diagnosis of pulmonary lymphangiticcarcinomatosis (PLC). Transbronchial biopsy of the nodule in theRLL revealed primary lung carcinoma (non-small cell lungcarcinoma) which caused PLC


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Pulmonary lymphangitic carcinomatosis is aterm that refers to tumour growth in thelymphatic system of the lung. The most commonmalignancies to produce lymphangitic

carcinomatosis (LC) are breast carcinoma, lung,stomach, pancreas, prostate, cervix, or thyroidand metastatic adenocarcinoma from anunknown primary site. The most common clinical

manifestation is dyspnoea. It is typicallyinsidious in onset but progresses rapidly andwithin a few weeks can cause severe disability.


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The chest radiograph is normal in

approximately 50% of cases of proven LC.

Radiographic manifestations ofLC

include: i) reticular or reticulonodularopacities, ii) septal lines, iii) hilar and

mediastinal lymphadenopathy, iv) pleural

effusion.

HRCT is the imaging method of choice for diagnosing


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HRCT is the imaging method of choice for diagnosingLC. HRCT findings ofLC include

: 1) smooth or nodular peribronchovascular interstitialthickening,

2) smooth or nodular interlobular septal thickening,

3) smooth or nodular thickening of fissures,

4) smooth or nodular subpleural interstitial thickening,

5) thickening of the peribronchovascular interstitium(peribronchial cuffing),

6) preservation of normal lung architecture at the lobularlevel despite the presence of these findings,

7) lymph node enlargement and 8) pleural effusion


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Although the findings ofLC are characteristic, they canalso be seen in other conditions like pulmonary oedema,sarcoidosis, CWP (Coal Workers Pneumoconiosis) orsilicosis, lymphocytic interstitial pneumonia, amyloidosis.The following clues to differential diagnosis should be

considered: a) in sarcoidosis and CWP septal nodulesare commonly seen but septal thickening is usually lessextensive than that seen in patients with lymphangiticspread of tumour, b) in sarcoidosis and CWP distortionof lung architecture may be present, a finding that is notseen in patients with LC, c) pleural effusion is rare in

sarcoidosis or silicosis and finally d) in pulmonaryoedema interstitial thickeninig is smooth and there areno peribronchovascular and subpleural nodules


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Non small cell lung carcinoma with

pulmonary lymphangitic carcinomatosis.


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A newborn infant

congenital heart disorder

localized soft tissue swelling and

erythema,


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Axial CT image of the foot demonstrating

the large region of heterotopic ossification

at the dorsum of the foot anterior to the

talus. Mineralization is most notable at theperiphery of the mass


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Sagittal reformatted CT of the foot

demonstrating the large region of

heterotopic ossification at the dorsum of

the foot anterior to the talus. Mineralizationis most notable at the periphery of the

mass.


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Heterotopic ossification refers to the formation of lamellar bonewithin soft tissues, secondary to genetic, traumatic, or iatrogeniccauses (1,2). It can present as small, clinically-insignificant flecks ofbone or large deposits of ossification. Its initial radiographicappearance can be confused with metastatic calcification inhypercalcemic disorders or more worrisome entities such asparosteal osteosarcoma or juxtacortical chondroma. Thepathogenesis for heterotopic bone formation is not fully understood.Though there are inherited disorders such as fibrodysplasiaossificans progressiva and progressive osseous heteroplasia whichare associated with advanced and often disfiguring manifestations ofheterotopic ossification, these disorders are rare and tend to arisefrom spontaneous mutations (1,2,3)


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Sagittal reformatted CT of the foot

demonstrating the large region of

heterotopic ossification at the dorsum of

the foot anterior to the talus. Mineralizationis most notable at the periphery of the

mass.


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A 17-year-old boy presented with a two

year history of uncontrolled hypertension


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that 10%40% of extra-adrenalparagangliomas are malignant (3). Distant

metastasis is the only reliable criteria for

confirming malignancy. Local tissueinvasion or pathological evidence of

nuclear pleomorphism or mitotic activity

does not necessarily imply malignancy

There are six major anomalies of the

inferior vena cava which include


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duplication of the IVC, transposition of the IVC,

azygos continuation of the IVC,

circumaortic renal collar, retroaortic renal

and retrocaval ureter (6).


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The persistence of right and left supracardinal veins results indouble IVC.

Transposition of IVC results from regression of the rightsupracardinal vein with persistence of the left supracardinal vein.Azygos continuation of the IVC is due to failure to form the rightsubcardinalhepatic anastomosis, with resulting atrophy of the rightsubcardinal vein.

Consequently, blood is shunted from the suprasubcardinalanastomosis through the retrocrural azygos vein. A circumaortic leftrenal vein results from persistence of the dorsal limb of theembryonic left renal vein and of the dorsal arch of the renal collar. Aretroaortic renal vein develops from the persistence of the dorsalvenous anastomosis of the supracardinal and subcardinal veins with

regression of the ventral aspect of the two venous systems Aretrocaval ureter occurs when the right posterior cardinal vein

short case 9

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