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Diamond Black Fan Syndrome and Its Association with Portal Cavernoma - Abstract

Kashif Chauhan, Naveed Alizai

 

ABSTRACT

Aim

To establish an association between patients diagnosed with Diamond Black Fan syndrome and subsequently developing Portal Cavernoma.

Method

We are treating two patients diagnosed with Diamond-Black Fan Syndrome. Both have multipl oesophago-gastroduodenoscopies (for esophageal varies), ultrasound scan of abdomen, blood transfusions, liver biopsies and subsequently developed portal cavernoma. First patient is 6 years old, had been diagnosed wit Diamond-Black Fan anaemia at 3years of age and portal cavernoma at 4 years of age and the second one is 23 years old, Diamond-Black Fan anaemia was diagnosed at 10 years of age, Portal Cavernoma at 11 years of age.

Results

Both patients have repeated blood transfusion leading to iron deposition in the liver with non-cirrhotic liver changes causing portal hypertension with esophageal varices and developed portal cavernoma. They have repeated hospital admission requiring intensive treatment and are managed conservatively. Collateral circulations around porta hepatic are seen on ultrasound and MRI. Medical management is successful so far and no surgical intervention is needed.

Conclusion

It is a rare condition and its association with portal cavernoma has never been reported. Patients who has diagnosed with Diamond-Black Fan anaemia should be monitored closely with repeated ultrasound scan of liver for early detection of portal cavernoma and if there is any suspicious an MRI should be done for the best management and treatment of this condition. Surgical intervention is a major procedure. Proposed intervention will be porto-systemic shunt or biliary intestinal bypass

Keywords: Diamond Black Fan syndrome, leukaemia, portal cavernoma

 

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