Sclerosing cholangitis

Findings and interpretation:

CT:

• There is irregularity of the intrahepatic bile ducts, with short segments of dilated peripheral ducts at different sites, abruptly transitioning to non-dilated ducts. The extrahepatic bile ducts are dilated.
• Dilated proximal small bowel loops in the left side of the abdomen and into the pelvis, transition point in the pelvis with abrupt transition into collapsed loops.
• Moderate amount of free fluid in the pelvis.
• The gallbladder shows thickened, irregular walls.
• On the delayed phase there is enhancement around the intrahepatic bile ducts. The extrahepatic bile ducts appear thickened and show enhancement.

ERCP:

• There is segmental dilatation of the intra and extrahepatic bile ducts, with multi-focal strictures and intervening segments of non-dilated or mildly dilated bile ducts.
• There is mural irregularity of the bile ducts.
• A double pigtail stent has been placed with one end in the duodenum and the other end in the common hepatic duct (adequate position).

Pertinent negative findings:

• No calculi in the biliary system.
• No mass lesions causing biliary obstruction.
• No evidence of large bowel inflammation (inflammatory bowel disease).
• No obstructing bowel lesion / hernia is detected.

The findings represent chronic inflammation of the bile ducts. The gallbladder changes may represent chronic cholecystitis due to longstanding cystic duct obstruction, or acute inflammation. Mechanical small bowel obstruction is present, caused by obstruction in the pelvis; this may be due to adhesions / inflammation in the pelvis.  

Principal diagnosis:

Auto-immune / primary sclerosing cholangitis and small bowel obstruction.

Differential diagnosis:

Ischemic cholangiopathy

Management:

Refer for hepato-biliary MDM discussion with regards to possibility of liver transplantation.   

Angio-invasive aspergillosis

Findings and interpretation:

• There are clustered pulmonary nodules in the lungs bilaterally, more numerous and larger on the right; these follow a random pattern of distribution and lower lung zone predominance.
• Some of the larger nodules show a surrounding ground-glass halo, which reflects haemorrhage around the nodule.
• There are bilateral mediastinal and hilar surgical clips, indicating prior bilateral pulmonary transplantation.
• There are a few pre-tracheal lymph nodes which do not exceed 1cm in the short axis; these show a non-specific appearance and are likely reactive in nature.
• The pancreas is completely replaced by fatty tissue, indicating complete pancreatic lipomatosis.

Pertinent negative findings:

• There are no grossly enlarged mediastinal lymph nodes.
• No discrete areas of ground glass opacification / mosaic pattern.

The findings indicate opportunistic infection in the setting of lung transplantation. Pancreatic lipomatosis indicates that the patient suffers from cystic fibrosis.

Principal diagnosis:

Angio-invasive aspergillosis complicating lung transplantation in a patient with cystic fibrosis.

Differential diagnosis:

• Other opportunistic infection such as CMV / mucormycosis.
• Post transplant lympho-proliferative disorder.
• Wegener’s granulomatosis (this is highly unlikely in the context of lung transplantation).

Management:

Urgently alert referring physician of findings to start treatment.

Refer to pulmonary MDM to discuss further investigations to confirm diagnosis, such as bronchio-alveolar lavage or CT guided biopsy for histological diagnosis.

T-cell lymphoma complicating coeliac disease

Findings and interpretation:

• There is marked circumferential, nodular thickening of the wall of a short segment of ileum. The involved segment of bowel shows aneurysmal dilatation with oral contrast media within.
• There are multiple enlarged lymph nodes in the ileal mesentery adjacent to the abnormal ileal segment. These exceed 1cm in short axis diameter and show loss of the normal reniform shape / fatty hilum. Smaller lymph nodes are detected more cranially at the root of the mesentery.
• There is a reduced number of jejunal folds, with an increased number of ileal folds, indicated reversal of the jejuno-ileal fold pattern.

Pertinent negative findings:

• There is no bowel obstruction / small bowel dilatation.
• There is no evidence of metastatic spread to the liver / lungs / distant lymph node groups.

Incidental:

• Subcapsular hypodense segment IVA lesion most likely reflects focal fatty sparing.

The findings represent a malignant neoplasm arising from the ileum. Reversal of the small bowel fold pattern is characteristic of coeliac disease. The enlarged mesenteric lymph nodes most likely indicates spread of malignancy from the small bowel rather than a manifestation of coeliac disease.  

Principal diagnosis:

T-cell lymphoma of the small bowel on a background of coeliac disease.

Differential diagnosis:

• Gastro-intestinal stromal tumour (this is far less likely, as it is not associated with coeliac disease).

Management:

Alert referring physician of findings.

Recommend discussion at oncology / gastrointestinal MDT with regards to image guided biopsy / further management by chemotherapy.

Cerebral arteriovenous malformation

Findings and interpretation:

CT brain:

• There are superficial tubular structures in the right temporal lobe, which are hyperdense compared to normal brain parenchyma.

MRI brain:

• There is a large cluster of extra-axial serpiginous structures overlying the right temporal lobe, replacing part of the brain parenchyma in that region.
• These structures show flow void on the T2 images, and vascular flow on the time of flight sequence.
• Arterial phase post-contrast sequence shows communication with the intracranial arteries emanating from the circle of Willis on the right side.
• Enlarged superficial cortical veins drain into the superior sagittal sinus and the right transverse sinus.   

Pertinent negative findings:

• No evidence of acute / previous haemorrhage.

The findings represent a cluster of abnormal vessels with arterial and venous components, draining into superficial cortical veins.

Principal diagnosis:

Cerebral arterio-venous malformation.

Differential diagnosis:

None

Management:

Refer to interventional neuro-radiologist for conventional angiography, and possible endovascular embolisation.

Lymphangioleiomyomatosis

Findings and interpretation:

Chest radiograph:

• The lung volumes are increased bilaterally.
• There are diffuse reticular opacities in both lungs.

CT thorax:

• There are diffuse small sized, thin walled cysts in a generalized distribution in the lungs bilaterally.
• The cysts are spherical and of similar shape and size.
• There is a minor degree of lung scarring the right upper lobe.

Pertinent negative findings:

• No nodules / pleural effusions / septal thickening in the lungs. No pneumothorax.
• The visualized upper portions of the kidneys are normal with no fatty masses detected.

The findings represent an interstitial, cyst forming disease of the lung.

Principal diagnosis:

Lymphangioleiomyomatosis

Differential diagnosis:

• Tuberous sclerosis complex (unlikely as the upper poles of the kidney do not show angiomyolipomas)
• Langerhans cell histiocytosis (unlikely as this entity typically shows irregular cysts of varying sizes, upper lobe predominance and pulmonary nodules)

Management:

Alert physician of findings and recommend respiratory physician referral.

Obtain complete imaging study of the kidneys to rule out tuberous sclerosis complex (CT abdomen/pelvis or ultrasound).

Recommend discussion at pulmonary MDT with regards to further management / confirming diagnosis by tissue biopsy.

Myelofibrosis complicated by portal vein thrombosis

Findings and interpretation:

• There is no contrast opacification of the portal vein or its main branches; multiple small peri-portal vessels are present.
• There is marked splenomegaly with retroperitoneal varices.
• There is a moderate amount of free fluid around the liver and spleen, in the pelvis and in the mesentery.
• There is increased bone density of all visualised bones, indicating diffuse osteosclerosis.

Pertinent negative findings:

• No enlarged lymph nodes.
• No liver disease.

Incidental:

• Chronic cholecystitis with cholelithiasis.

The findings represent portal venous thrombosis with cavernous transformation, which is causing ascites. Marked splenomegaly and diffuse osteosclerosis are characteristic of myelofibrosis. This condition causes a hypercoagulable state, predisposing to portal vein thrombosis.  

Principal diagnosis:

Myelofibrosis causing portal vein thrombosis.

Differential diagnosis:

• Sclerotic metastases
• Lymphoma causing splenomegaly

Management:

Alert referring physician of findings.

Refer for haematology MDM to discuss further management / treatment options.