Introduction

Banti’s syndrome is a rare disorder characterised by splenomegaly, ascites, and portal hypertension due to either intrahepatic or pre-hepatic lesions, occurring in the absence of coexisting liver cirrhosis. Though it is a rare disease occurring all over the world, it is more common in developing countries. Banti’s syndrome is also referred to in the USA as hepatoportal sclerosis, it is described in Asia as non-cirrhotic portal fibrosis, idiopathic portal hypertension in Japan, and in Europe, it is still called Banti’s syndrome.¹

Causes of banti’s syndrome

Banti’s syndrome primarily affects the spleen, resulting in various clinical manifestations. Key characteristics of the condition include:

• Splenomegaly
• Varices visible on endoscopy 
• Cytopenia of one or more blood cell lines 
• High portal hypertension with patent portosystemic shunts 
• Absence of liver cirrhosis 
• Patency of hepatic veins
• Normal liver function tests 

In advanced stages, Banti’s syndrome is often complicated by the rupture of varices, leading to life-threatening haemorrhage.

Historical perspective

For a long time, great importance was attributed to the anaemia which almost constantly accompanies Banti’s syndrome. Micheli’s observation in 1903 highlighted that this type of anaemia is usually associated with any considerable splenic enlargement, whatever the cause. This led to a reduced focus on anaemia as a defining feature of the syndrome.²

Modern understanding 

Recent studies have drawn attention to significant splenic and portal congestion. This is considered a defining feature of Banti’s splenomegalies, distinguishing it from splenomegaly caused by other conditions. Evidence for this congestion includes: 

• A pronounced decrease in splenic volume following administration of epinephrine or after haemorrhage 
• Frequent occurrence of copious haemorrhages from the gastrointestinal tract 
• Occurrence of transitory or permanent ascites
• Hypertension in the splenic veins, often observed during surgical intervention

Pathological findings

Microscopic examination has revealed similarities in lesions, including:

• Connective and elastic hyperplasia of the splenic capsule and trabeculae
• Fibrosis and hyperplasia of the splenic pulp’s reticular framework

In most cases, the liver is unaffected, although varying degrees of Laennec’s cirrhosis have been reported.

Portal congestion and associated features

Evidence of portal congestion is frequently observed, such as:

• Dilation of sinuses and intrasplenic haemorrhages in the spleen
• Residual siderotic nodules
• Dilation of splenic veins, portal veins, and mesenteric veins
• Enlargement of collateral portal-systemic circulation pathways

Potential causes of splenic and portal congestion

Several factors have been identified as possible contributors to splenic and portal congestion, including:

• Thrombosis of the splenic and portal veins
• Cavernomatous transformation (clusters of abnormal blood vessels)
• Stenosis caused by external compression
• Advanced stages of hepatic cirrhosis³

Symptoms

One of the major symptoms of Banti’s syndrome is gastrointestinal (GI) bleeding. Other symptoms include:

• Fatigue and weakness
• Anaemia, often worsening due to hematemesis (vomiting blood) and melena (black, tarry stools) caused by GI bleeding
• Splenomegaly (enlarged spleen)
• Pancytopenia, which increases the risk of severe infections and bruising

Additional findings may include:

• Coagulation abnormalities
• Toxic metal exposure, such as arsenic, is a potential contributing factor⁴

Diagnosis

Banti syndrome is confirmed by a set of specialised tests, among which advanced imaging techniques are predominant, such as CT scan, MRI scan, and splenic venography, which provide an in-depth review of the clinical profile.

Blood profile along with laboratory investigations shows red blood cell anaemia, leukopenia (low white blood cell count) and thrombocytopenia (low platelet count). The bone marrow is initially normal, but it is later hyperplastic.

Some additional diagnostic procedures include endoscopy to detect oesophageal varices, liver function tests to assess liver health and rule out cirrhosis and tests for hepatitis B along with other potential underlying causes. Endoscopic sclerotherapy and endoscopic variceal band ligation can be performed in the emergency setting.⁵

Treatment

The management of the disease primarily focuses on controlling gastrointestinal bleeding due to portal hypertension and addressing hypersplenism.

Variceal ligation and endoscopic sclerotherapy have proved to be equally efficacious in patients with acute gastrointestinal bleeding in 95% of the patients. Recurrence rates are approximately 20%, with 3% of cases involving recurrent bleeding. In some instances, emergency shunt surgeries may be required.

Beta-blockers are used as prophylactic drugs in patients with portal hypertension. Additionally, ethanol, an orphan drug approved by the US FDA in 1988, is used for the treatment of oesophageal varices.

Surgical management is indicated in patients with symptomatic hypersplenism, severe anaemia requiring repeated blood transfusions, or repeated episodes of splenic infarction.⁶

Prognosis

The major challenges in managing patients with Banti’s syndrome are gastrointestinal haemorrhage and hypersplenism. With effective treatment of these complications, patients can achieve a good quality of life.

Both endoscopic sclerotherapy and variceal ligation have demonstrated equal effectiveness in the management of acute bleeding in about 95% of patients. Only a few cases require emergency shunt surgery. Variceal recurrence has been reported in about 20% of patients, but only 3% experience recurrent bleeding.

Beta-blockers are known to be useful in primary prophylaxis even in non-cirrhotic patients with portal hypertension. Surgical management is also warranted for patients with symptomatic hypersplenism, spontaneous bleeding, severe anaemia requiring transfusion, or repeated splenic infarcts.

The prognosis for patients with Banti’s syndrome is excellent. Even in patients with acute variceal bleeds, the mortality is significantly lower than seen in cirrhotic patients. Following successful eradication of gastro-oesophageal varices, reported 5-year survival rates are as high as 100%.⁷

Summary

Banti’s syndrome also referred to as hepatoportal sclerosis, non-cirrhotic portal fibrosis, or idiopathic portal hypertension, is a rare disease characterised by splenomegaly, portal hypertension, and ascites in the absence of liver cirrhosis. This disease is found worldwide but with increased prevalence in developing countries. Major clinical manifestations include splenomegaly, portal hypertension, esophageal varices, cytopenia, normal liver function tests, and the absence of cirrhosis.

Although the exact reason behind the emergence of Banti’s syndrome is not specified, this condition is associated with splenic and portal congestion, which can lead to a number of additional complications. The clinical manifestations are weakness, fatigue, anaemia, and bleeding from the GI tract. Diagnosis is carried out by clinical examination of the patient, blood analysis, CT scan, MRI scan, splenic venography, and endoscopy.

Treatment involves the management of gastrointestinal bleeding and hypersplenism by endoscopic variceal ligation or sclerotherapy, beta-blockers in the setting of portal hypertension, and surgical intervention in severe cases. Generally, the prognosis for Banti’s syndrome seems favourable, with excellent long-term survival rates when promptly treated. Even in cases of acute variceal bleeding, mortality rates stand much lower when compared to cirrhotic patients.

While Banti’s syndrome can lead to serious complications such as GI bleeding, it can be managed effectively in a way that a reasonable quality of life is preserved for such patients. Advances in research and therapeutic approaches have significantly improved outcomes for individuals with this rare disorder.