Brief Answer:
Rare disease.

Detailed Answer:
Hello,
Thank you for choosing HealthcareMagic for asking your query.
I am sorry but I got your question a few minutes ago only.

I've gone through your mother's report.

She is having a condition called portal Portal hypertension is defined by a pathologic increase in the pressure of the portal venous system. Cirrhosis is the most common cause of portal hypertension, but it can also be present in the absence of cirrhosis as in the case of your mother, a condition referred to as "noncirrhotic portal hypertension".

Portal vein carries blood to the liver in the path it meets splenic vein which arises from the spleen. The blood flowing through them will finally be drained in the liver. If somehow this flow is blocked the blood will start accumulating in vein and spleen causing splenomegaly and portal hypertension
Hemoglobin is just 7.4, a blood transfusion is usually done at Hb levels less than 6 mg/dl.

Liver function tests and blood sugar are almost fine.

CT scan reports clearly are suggestive of portal hypertension without liver involvement.

Splanchnic venous thrombosis is one of the most important causes of noncirrhotic portal hypertension. I would suggest you go for an MRI abdomen to rule out the presence of clot or blockage in splenic vein. In the absence of thrombosis of splenic vein, it's a rare disease characterized by intrahepatic portal hypertension.

As the cause is usually idiopathic(unknown) treatment is very difficult usually aimed at controlling symptoms of the patient.

The etiology can be classified into five categories: 1) immunological disorders 2) chronic infections, 3) exposure to medications 4) genetic predisposition and 5) prothrombotic conditions.


INCH diagnosis is based on clinical criteria and the formal exclusion of any other causes of portal hypertension. criteria's are 1) presence of unequivocal signs of portal hypertension, 2) absence of cirrhosis, advanced fibrosis or other causes of chronic liver diseases, and 3) absence of thrombosis of the hepatic veins or of the portal vein at imaging.

Treatment of INCPH relies on the prevention of complications related to portal hypertension.


Sad Truth-
*There is a lack of a specific positive test that leads to an INCPH diagnosis. It is based on clinical criteria and the formal exclusion of other causes of PH; this represents a clinical challenge, even in
experienced liver units. Consequently, INCPH is frequently unrecognized, and in many instances, patients are misdiagnosed *

Liver function tests are usually within normal range; jaundice is rarely seen at diagnosis. Transient impairments in liver function may occur in the context of variceal bleeding or infection. Anemia, leukopenia, and thrombocytopenia are common due to hypersplenism as in your mother's case. However, a liver biopsy can be done.


Treatment
Management of portal hypertension:
Primary and secondary prevention of variceal bleeding by the use of non-selective beta-blockers and endoscopic variceal ligation. Transjugular intrahepatic portosystemic shunting is an effective alternative in patients who fail to respond to medical and endoscopic therapy.

Liver transplantation may be required in some patients.

Please ask if you are unable to understand any point. It's a complex topic and to explain everything in lay man's language was rather not feasible.

Follow ups are most welcome.

Regards.

---

Brief Answer:
Line of treatment by your doctor is good.

Detailed Answer:
Hi again,
As explained above treatment of choice for non cirrhotic portal hypertension is use of non-selective beta-blockers (Betacap 40mg).However if symptoms are not controlled well by medicines then shunt surgery is considered to be the treatment of choice in patients.Shunt surgery effectively prevents rebleeding from esophageal varices and also improves portal gastropathy, portal
biliopathy and is effective in subsiding symptoms in more then 75% of cases.Overall prognosis and results of shunt surgery are much better then medical management.The overall survival at 5 years around 80%
and operative mortality <1%.
As per your query spleenectomy would reduce the pressures in portal veins hence would relieve the symptoms.However options of shunt surgery can also be discussed with you gastroenterologist.

Regards.

---