Superior sagittal sinus thrombosis and Budd-Chiari syndrome due to paroxysmal nocturnal hemoglobinuria managed with transjugular intrahepatic portosystemic shunt: a case report.
BACKGROUND
Paroxysmal nocturnal hemoglobinuria (PNH), caused by somatic mutation of hematopoietic cells, is associated with complement-mediated hemolysis and a hypercoagulable state. Thrombotic complications in this disease are associated with reduced survival. We report a patient with PNH complicated by intracranial venous thrombosis and Budd-Chiari syndrome, who was managed with transjugular intrahepatic portosystemic shunt.


METHODS
A 26-year-old man presented with thrombosis of the superior sagittal and right sigmoid sinuses. Initial investigations did not reveal any underlying cause. Nine months later, he developed hepatic venous thrombosis. At this time, Ham test was positive. Flow cytometry confirmed the diagnosis of PNH. The patient was treated with transjugular intrahepatic portosystemic shunt; one episode of stent blockage one month later was managed successfully with balloon dilatation and restenting.


CONCLUSIONS
PNH should be considered in patients with unexplained venous thrombosis. Thrombosis in these patients needs to be managed with prolonged anticoagulation. For Budd-Chiari syndrome in patients with underlying PNH, transjugular intrahepatic portosystemic shunt may be a good option but caution is needed to prevent stent occlusion.
Sharma
Abhinav
A
Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, India.
Itha
Srivenu
S
Baijal
Sanjay S
SS
Gupta
Ritu
R
Sonkar
Atul
A
Aggarwal
Rakesh
R
D000328:Adult; D006502:Budd-Chiari Syndrome / Q000209:etiology*; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000601:surgery*; D006801:Humans; D008297:Male; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D020225:Sagittal Sinus Thrombosis / Q000209:etiology*