Chronic Budd Chiari syndrome

Findings and interpretation:

• The liver is enlarged, and demonstrates heterogeneous enhancement and a patchy hepatogram with areas of hypoperfusion.
• There is splenomegaly and splenic varices, as well as multiple collateral veins in the abdominal wall.
• The caudate lobe is disproportionately enlarged compared to the rest of the liver.
• There are multiple enhancing nodules spread throughout the liver.
• There are multiple intrahepatic collateral vessels, some of which drain directly into the IVC.
• There is no enhancement of the hepatic veins.
• The intrahepatic IVC shows a focal stenosis which may indicate presence of an IVC web.

Pertinent negative findings:

• No IVC/ portal vein thrombosis.

Incidental:

• There are cholecystectomy clips and fluid in the gallbladder fossa indicating recent cholecystectomy.

The findings represent sequelae of chronic hepatic vein occlusion and thrombosis with compensatory hypertrophy of the caudate lobe, regenerative nodule formation and the development of porto-systemic collaterals / shunts and splenomegaly as a result of portal venous hypertension.

Principal diagnosis:

Chronic Budd Chiari syndrome, likely caused by IVC web.

Differential diagnosis:

None in this case.

Management:

Alert referring physician of findings if not known case.

Recommend discussion at hepatobiliary MDT to discuss treatment options, which may include angioplasty and stenting of the IVC by interventional radiology or liver transplantation.

Multi-system scleroderma / systemic sclerosis

Findings and interpretation:

Radiographs:

• There is resorption of the tufts / distal phalanges of most of the fingers bilaterally indicating acro-osteolysis.
• There is generalised joint space narrowing involving the PIP and DIP joints in most fingers bilaterally.

CT:

• There are prominent reticular opacities and septal thickening with associated mild ground glass opacification in a peripheral, subpleural distribution seen in both lungs; these show a cranio-caudal gradient with greater disease involvement in the lower lobes and at the bases.
• There is mild peripheral bilateral traction bronchiectasis.
• There is cyst formation in both lung bases indicating early honeycombing.
• There is bilateral mild fissural and pleural thickening.
• The oesophagus is dilated and shows an air-fluid level.

Pertinent negative findings:

• There is no significant erosive arthropathy or soft tissue calcification.

The findings indicate a multi-system disease manifesting as a fibrotic interstitial lung disease with usual interstitial pneumonia pattern in the lungs, involvement of the gastro-intestinal system manifesting as oesophageal fibrosis and achalasia, and involvement of the musculoskeletal system with bony erosion and arthropathy.  

Principal diagnosis:

Multi-system scleroderma

Differential diagnosis:

Differential diagnosis of UIP:

• IPF
• Amiodarone lung fibrosis
• Asbestosis

Differential diagnosis of acro-osteolysis:

• Hyperparathyroidism
• Raynaud’s disease
• Trauma/frostbite
• Psoriatic arthropathy

The constellation of findings makes an alternative diagnosis extremely unlikely.

Management:

Recommend discussion at pulmonary/connective tissue disease MDT regarding further management.

Acute pancreatitis complicated by bleeding pseudo-aneurysm

Findings and interpretation:

• There is a large volume of hyperdense material in the retroperitoneum, lesser sac and surrounding the body and tail of the pancreas; this extends down the left paracolic gutter into the pelvis. There is a large collection in the pelvis demonstrating a haematocrit level and representing a haematoma.
• There is free simple fluid around the liver, in the right paracolic gutter and extending into the pelvis.
• There is ill defined soft tissue and fat stranding surrounding the pancreas.
• There is a wedge shaped hypodensity in the right kidney representing a renal infarct. There is a filling defect in the right renal artery representing a thrombus.
• On the arterial phase sequence there is a sac originating from the splenic artery which fills with contrast and empties on the portal venous phase, representing a pseudo-aneurysm.

Pertinent negative findings:

• There is no definite contrast extravasation.
• No splenic vein thrombosis.

Incidental findings:

• There is a calcified lesion in the right lobe of the liver posteriorly, representing a granuloma.

The findings represent acute pancreatitis which was complicated by formation of a pseudo-aneurysm in the splenic artery. This has bled, causing a large volume of intra-abdominal bleeding. Thrombosis of the right renal artery is likely caused by a hypercoagulable state, resulting in a right renal infarct.  

Principal diagnosis:

Acute pancreatitis complicated by bleeding from a splenic pseudo-aneurysm.

Differential diagnosis:

• This may have been a true splenic aneurysm that has ruptured.

Management:

Urgently contact referring physician and inform of findings.

Recommend interventional radiology referral with a view to splenic artery embolisation.

Pigmented villonodular synovitis

Findings and interpretation:

Radiographs:

• Mild degenerative change in both knee joints.
• There is a small erosion with sclerotic borders at the intercondylar eminence of the right knee.

MRI:

• There are multiple low T1 and PD signal intensity nodules scattered throughout the left knee joint; these nodules aggregate in the posterior aspect of the knee joint to form lobulated masses. Irregular low signal intensity synovial thickening is also seen in the posterior aspect of the knee joint.
• These show low signal intensity on both T1 and proton density weighted sequences.
• There are lobulated masses extending posteriorly into the popliteal fossa, showing heterogeneous signal intensity with loculated and septated fluid collections of high PD and intermediate T1 signal intensity. The walls and septations show low PD and T1 signal intensity. These surround the heads of the gastrocnemius muscle, as well as the semi-membranosus and semi-tendinosus tendons.
• On the gradient echo sequence, the synovium and synovial nodules and popliteal fossa masses show blooming artefact, indicating haemosiderin deposition.

Pertinent negative findings:

• There is no evidence of erosive arthropathy.
• The cruciate ligaments and menisci are intact.

The findings represent nodular masses arising from the synovium, as well as synovial thickening and proliferation with marked haemosiderin deposition indicating repeated haemarthrosis.

Principal Diagnosis:

Pigmented villonodular synovitis of the left knee. The right knee is also likely involved, yet the demonstrated radiographic features are not specific.  

Differential diagnosis:

• Haemophilic arthropathy (this typically shows a greater degree of erosion, as well as epiphyseal overgrowth). Also, the age group at which this condition presents is typically much younger.

Management:

• Alert referring physician of diagnosis. Recommend orthopaedic referral.
• Biopsy may be performed for histological diagnosis.
• This is usually treated by synovectomy.

Langerhans cell histiocytosis / Letterer Siwe disease

Findings and interpretation:

CT head and neck:

• There are multiple, scattered, well defined lytic bony lesions involving the skull base and calvarium, as well as the facial bones and mandible. Some of the lesions, such as that involving the left lateral orbital wall and greater wing of sphenoid, contain enhancing soft tissue masses.
• There is enhancement of the pituitary stalk.
• There is extensive involvement of the soft tissues of the neck with peripherally enhancing soft tissue lesions, which extend into the retropharyngeal space. This most likely represents extensive lymphadenopathy.

CT thorax and abdomen:

• There is diffuse involvement of both lungs with innumerable nodules of varying sizes and cysts.
• Multiple lytic lesions are again seen involving the scapulae and ribs.
• Grossly enlarged, ill defined lymph nodes are noted in both axillae.
• The thymus is enlarged with scattered punctate calcifications.
• There are lytic, expansile bony lesions in the iliac bones and femorae.
• There are bilateral, enlarged inguinal lymph nodes.

The findings indicate a multi-system disease involving the musculoskeletal, pulmonary, lymphatic and CNS systems, characterised by soft tissue proliferation and granuloma formation.

Principal diagnosis:

Multi-system Langerhans cell histiocytosis (Letterer-Siwe disease)

Differential diagnosis:

• Lymphoma: does not explain pulmonary findings. Imaging features make this diagnosis unlikely.
• Neuroblastoma: does not explain pulmonary findings. Imaging features make this diagnosis unlikely.

Management:

Recommend ultrasound guided biopsy of one of the superficial lymph nodes for histological diagnosis.

Recommend discussion at MDT regarding further management; this usually entails chemotherapy / steroid administration.

Acute ischaemic stroke caused by MCA thrombosis on a background of Takayasu’s arteritis

Findings and interpretation:

Non-contrast CT brain:

• There is hyperdensity of the M1 and M2 segments of the right MCA.

CT angiography:

• There is circumferential thickening of the aortic wall involving the arch and extending to the level of the mid-descending thoracic aorta. Arterial wall thickening is also seen at the origins of the left subclavian and common carotid arteries, and involves the entire right brachiocephalic artery; there is a significant focal stenosis of the proximal right subclavian artery.
• There is alternating occlusion and long segment, smooth luminal stenoses of both common carotid arteries and the left subclavian artery. Long segment stenosis extends into the left internal carotid artery.
• There is a filling defect at the terminal M1/proximal M2 segment of the right MCA causing a focal occlusion of this arterial segment. The M2 branches distal to the filling defect opacify via collaterals.

Pertinent negative findings:

• The remaining intracranial vessels are normal.
• Normal appearance of the main pulmonary arteries.

Incidental:

• Minimal posterior sub-pleural reticulation in the right lung.

The findings represent inflammatory arteritis of multiple large vessels. This has resulted in thrombo-embolism of the right MCA causing an MCA territory infarct.

Principal Diagnosis:

Acute ischaemic stroke caused by MCA thrombosis on a background of Takayasu’s arteritis

Differential diagnosis:

• Giant cell arteritis: typically affects older patients

Management:

Urgently inform referring physician of diagnosis with a view to initiate IV thrombolysis.

Refer to interventional neuro-radiology thrombectomy service if available; however, this might not be feasible due to arterial stenoses.