Easy as ABC Ed Godfrey Tristan Barrett Siobhan

Easy as ABC Ed Godfrey Tristan Barrett Siobhan Whitley Addenbrooke’s Hospital

37 year-old male • • Acute right upper quadrant pain Raised bilirubin, ALP, ALT Normal WCC, CRP Normal ultrasound and CT examinations

FRFSE T 2 w axial

LAVA FS T 1 w, unenhanced

LAVA FS T 1 w, portal phase

Dual echo, in and opposed phase T 1 w

Easy as ABC • Primary diagnosis? • Aetiology? • How would you confirm your suspicions?

Easy as ABC • Primary diagnosis? – Acute Budd-Chiari syndrome • Aetiology? • How would you confirm your suspicions?

Normal central enhancement. Peripheral hypoenhancement.

Easy as ABC • Primary diagnosis? – Acute Budd-Chiari syndrome • Aetiology? – Paroxysmal nocturnal haemoglobinuria • How would you confirm your suspicions?

Note longer TE for the in phase image – iron deposition causes signal loss in the kidneys.

Easy as ABC • Primary diagnosis? – Acute Budd-Chiari syndrome • Aetiology? – Paroxysmal nocturnal haemoglobinuria • How would you confirm your suspicions? – Flow cytometry for glycosylphosphatidylinositol linked proteins.

Key learning points • Normal enhancement centrally, with peripheral hypoenhancement of the liver indicates Budd-Chiari syndrome. • Iron deposition causes signal loss on the inphase images due to a longer TE. • Iron deposition in the kidneys is highly suggestive of paroxysmal nocturnal haemoglobinuria.