Concluded Case

Jaundice in NCPF

30/F, no comorbidities, k/c/o NCPF (biopsy proven) with PHTN on anticoagulation for acute PVT Chief Complaints Jaundice since 1 month without any prodromal or cholestatic symptoms Physical Examination Massive splenomegaly Investigations Bili= 5/3.5, OT/PT = 34/23, ALP = 413 CECT is as shown below showing dilated portal and splenic vein with a partial thrombus in portal vein Management What should be the management of this patient?

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Concluded answer

Cholestatic jaundice in a c/o NCPF is most likely due to portal cavernoma cholangiopathy as portal cavernoma can be seen in 40% cases of NCPF. As the patient was not in cholangitis and there were large veins suitable for shunting, the patient was referred to surgical gastroenterology for surgical shunt procedure as it is the definitive therapy in this patient.

All Answers

Cholestatic jaundice in a c/o NCPF is most likely due to portal cavernoma cholangiopathy as portal cavernoma can be seen in 40% cases of NCPF. As the patient was not in cholangitis and there were large veins suitable for shunting, the patient was referred to surgical gastroenterology for surgical shunt procedure as it is the definitive therapy in this patient.

Non cirrhotic portal fibrosis is a syndrome of obscure pathology charaterised by venopathy leading to portal hypertension,huge splenomegaly associated with bleeding from varices specially in young adult without any change of structure and function of liver. No permanent treatment is available Only prophylaxis and symptomatic treatment Surgery venous shunt mat be helpful In this case associated with thrombus anticoagulopathic treatment with thronboembolectomy .

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