Massive Splenomegaly

Splenomegaly is enlargement of the spleen beyond normal dimensions.

There is no single universal definition of massive splenomegaly, but practical descriptions include:

• Craniocaudal splenic length greater than approximately 20 cm on imaging
• Spleen extending into the pelvis
• Spleen crossing significantly toward the right side of the abdomen
• Splenic weight above approximately 1 kg at surgery or pathology

Massive splenomegaly should trigger a focused search for a limited group of important diseases.

Major causes include:

• Chronic myeloid leukaemia
• Primary myelofibrosis
• Lymphoma
• Hairy cell leukaemia
• Thalassaemia and chronic haemolytic states
• Portal hypertension
• Chronic malaria
• Visceral leishmaniasis
• Gaucher disease
• Other myeloproliferative neoplasms

The problem can be both systemic and mechanical.

A very large spleen can produce:

• Severe hypersplenism
• Early satiety and malnutrition
• Splenic infarction
• Abdominal pain
• Increased risk of traumatic rupture

Patients can describe:

• Left upper quadrant dragging pain
• Early satiety
• Abdominal distension
• Reduced oral intake
• Weight loss
• Fatigue
• Symptoms from anaemia or thrombocytopenia

On examination the spleen:

• Moves inferomedially with inspiration
• Has a palpable notch in many patients
• Cannot usually be ballotable like an enlarged kidney

Look for the cause:

• Lymphadenopathy
• Hepatomegaly
• Portal hypertension
• Ascites
• Pallor
• Bruising
• Fever
• Bone pain
• Constitutional symptoms

A sudden onset of severe left upper abdominal pain in a massively enlarged spleen should prompt assessment for:

• Infarction
• Subcapsular haemorrhage
• Rupture

Laboratory evaluation

Obtain:

• Full blood count
• Peripheral film
• Reticulocyte count
• Liver function
• Coagulation profile
• Haemolysis studies where appropriate

Further testing depends on phenotype.

Examples include:

• BCR::ABL testing when CML suspected
• JAK2, CALR or MPL assessment for myeloproliferative disease
• Bone marrow examination when haematological disease suspected
• Malaria testing where epidemiologically relevant
• Visceral leishmaniasis testing where appropriate

Imaging

Ultrasound: confirms enlargement and portal system anatomy.

Contrast CT abdomen: useful when defining:

• Exact splenic size
• Infarction
• Focal lesions
• Lymphadenopathy
• Liver disease
• Portal or splenic vein thrombosis
• Surgical anatomy

Before elective splenectomy for a massive spleen, evaluate portal and splenic venous anatomy carefully.

Disease directed treatment

Treat the cause whenever possible.

Examples:

• Tyrosine kinase inhibitor for CML
• Disease specific treatment for myelofibrosis or lymphoma
• Treatment of chronic infection
• Management of portal hypertension

A dramatically enlarged spleen can shrink substantially after effective systemic therapy, eliminating or simplifying the need for surgery.

Indications for splenectomy

Consider surgery for:

• Severe refractory pain
• Marked early satiety and nutritional compromise
• Severe symptomatic hypersplenism
• Transfusion dependence clearly improved by removal
• Recurrent infarction
• Rupture
• Selected diagnostic uncertainty
• Failure of disease directed therapy to control major splenic symptoms

In some haematological disorders splenectomy is palliative rather than curative.

Preoperative preparation

For elective splenectomy:

• Correct major anaemia where feasible
• Crossmatch blood
• Correct clinically important coagulopathy
• Assess platelet count and function
• Evaluate portal venous system
• Administer asplenia vaccines at least 14 days before surgery where possible.

Operative approach

Laparoscopic splenectomy is standard for many elective spleens, but very massive spleens substantially increase:

• Operative difficulty
• Blood loss
• Conversion risk
• Specimen extraction problems

Options include:

• Laparoscopic splenectomy
• Hand assisted laparoscopic splenectomy
• Open splenectomy

The approach should reflect spleen size, disease, portal hypertension and surgeon expertise rather than insisting on laparoscopy for every patient.

Operative principles

Gain early vascular control where possible.

The splenic artery can be controlled early to:

• Reduce splenic inflow
• Decrease congestion
• Reduce spleen volume modestly
• Reduce operative blood loss

Divide short gastric vessels carefully.

Mobilise the spleen without capsular tearing.

Protect the pancreatic tail.

Search for accessory spleens, particularly when surgery is being performed for a haematological disorder where residual splenic tissue could contribute to recurrence.

Common sites include:

• Splenic hilum
• Gastrosplenic ligament
• Greater omentum
• Pancreatic tail region

Thrombosis prevention

Very large spleen size, portal hypertension and enlarged splenic vein increase the risk of postoperative portal or splenic vein thrombosis.

Use standard postoperative VTE prophylaxis unless contraindicated.

Maintain a low threshold for Doppler ultrasound or contrast CT with:

• New abdominal pain
• Fever
• Unexpected thrombocytosis
• Abnormal liver function
• Persistent postoperative ileus
• Gastrointestinal symptoms suggestive of mesenteric extension