splenomegaly

SPLENOMEGALYBY

PROF GOUDA ELLABBAN

SUEZ CANAL UNIV. EGYPT

Background Anatomy

Largest single mass of lymphoid tissue.Oval in shape and has an notch in the anterior border.

Lies beneath the the left half of the diaphragm close to the 9th,10th,11th ribs.

Normally, its lower pole extends forward only as far as the midaxillary line. it is surrounded by peritoneum.Blood supply:

Arterial: large splenic artery (branch of the celiac artery). The splenic artery divided into about 6 branches which enter the spleen hilumVenous: the splenic vien which join the superior mesenteric vein to form portal vein.

BackgroundFunction

The spleen is a sophisticated filter that monitors and manages blood cells and immune functionsDuring fetal development the spleen produces red and white blood cellsBy the fifth month of gestation the spleen no longer has hematopoietic function but retains the capacity throughout lifeRed cells that pass through the spleen undergo a “cleaning” or repairAbnormal and old cells are destroyed

DefinitionsSplenomegaly is an enlargement of the spleen beyond its normal size.

Hypersplenism is a clinical syndrome that is characterized by splenic enlargement, any combination of anemia, leucopenia or thrombocytopenia, compensatory bone marrow hyperplasia and improvement after splenectomy.

Causes of splenic enlargement

Infective

Blood disease

Metabolic

Circulatory

Collagen disease

neoplastic


InfectiveBacterial

TyphoidTyphusTBBrucellosisSepticemiaSplenic abscess


InfectiveViral

Glandular feverEBVHIV-related thrombocytopenia

SpirochetaetalSyphilisLeptospirosis

ParasiticMalariaSchistosomiasisKala-azarHydatid custtrypanosomiasis


Blood diseaseMyeloid and lymphocytic leukaemiaLymphomaPernicious anemiaPolycythemia veraThrombocytopenic purpuraHemolytic anemias (e.g. sickle cell, thalassaemia)Myelofibrosis


CirculatoryPortal hypertension (cirrhosis, portal vein thrombosis)Congestive heart failureHepatic vein obstructionSegmental portal hypertension (pancreatic carcinoma, splenic vein thrombosis)infarction

MetabolicRicketsAmyloidProphyriaGaucher’s disease


Collagen diseaseStill diseaseFelty syndrome

NeoplasticAngiomaPrimary fibrosarcomaHodgkin’s lymphomaOther lymphomasMyelofibrosis

Non parasitic cystsCongenitalAcquired

Clinical presentationmild abdominal pain that is vague in nature Increased abdominal girth Febrile illness (infectious)Pallor, dyspnea, bruising, and/or petechiae (hemolytic process)History of liver disease (congestive)

Signs of cirrhosis (eg, asterixis, jaundice, telangiectasias, gynecomastia, caput medusa, ascites)

Weight loss, constitutional symptoms (neoplastic)Pancreatitis (splenic vein thrombosis)Alcoholism, hepatitis (cirrhosis) Heart murmur (endocarditis, congestive failure)

Physical signs

The spleen enlarges inferiorly and medially (phrenico-colic ligament)It is firm, smooth and usually spleen shaped.It often has a definite notch on its upper edge.You cannot get above it.It moves with respiration.It is dull to percussion.

Investigations

Lab studies

Imaging studiesHistologic

Findigs

Lab studies

Perform a CBC count with platelet count.

The term hypersplenism describes some of the sequelae often observed with splenomegaly. Criteria for a diagnosis of hypersplenism include the following:

Anemia, leukopenia, thrombocytopenia, or combinations thereof, plus cellular bone marrow, splenomegaly, and improvement after splenectomyThrombocytopenia: Approximately 30% of the total platelet mass exists as an exchangeable pool in the spleen.

Lab studies

Anemia: The etiology of the anemia observed in splenomegaly is the result of sequestration and hemodilution.

Leukopenia: Increased destruction or sequestration of leukocytes causes the leukopenia observed in splenomegaly. Leukopenia is closely related to neutropenia.

Imaging studies

Computed tomographyA CT scan remains the most useful preoperative investigation to measure splenic volume; to exclude lymph nodes at the splenic hilum; and to detect accessory spleens, splenic abscess, and perisplenitis.Findings that indicate radiologic distinction between benign and malignant lesions are inconsistent and cannot be relied upon to establish or refute a diagnosis of malignancy.CT scan is the study of choice for identification of inflammatory changes.CT scan is sensitive for detecting mass lesions, infarcts, and cysts.

Imaging studies

SplenoportographyThis is used to evaluate portal vein patency and the distribution of collaterals before shunt operations for cirrhosis.Findings can help identify the cause of idiopathic splenomegaly, especially in children.

Angiography: Findings are used to differentiate splenic cysts from other splenic tumors.

Imaging studiesSpleen scans

Label erythrocytes with chromium Cr 51, mercury Hg 197, rubidium Rb 81, or technetium Tc 99m, and alter the cells by treatment with heat, antibody, chemicals, or metal ions so that the spleen sequesters them after infusion.A spleen scan is a good noninvasive technique for evaluating spleen size; a close correlation exists between spleen length on scan images and spleen weight after splenectomy.A spleen scan is useful for detecting space-occupying lesions in the splenic substance, evaluating loss of spleen functions, assessing for the absence of a spleen, or determining the presence of an accessory spleen.

Ultrasound: This is a noninvasive, highly sensitive, and specific technique for the evaluation of spleen size.

Treatment

Medical Treatment

Surgical Treatment

Medical TreatmentSuccessful medical treatment of the primary disorder can lead to regression of the hypersplenism without the need for surgery. Chemotherapy is used for hematologic malignancy.Antibiotics are used for infection, with the exception of that associated with splenic abscess. This requires surgical intervention.Immunosuppression is used for autoimmune or inflammatory disorders, treatment of cirrhosis, and CHF.All patients scheduled for elective splenectomy (either diagnostic or therapeutic) should receive a pneumococcal vaccine. Also consider administering prophylaxis against Haemophilus influenzae and Neisseria meningitidis.

Common indications for splenectomy:

Trauma:-Accidental-Operative

Oncological:-Diagnostic -Therapeutic

Haematological:-ITP-Hypersplenisim

Portal hypertension:-Variceal surgery.

Peroperative preparation:

- In the presence of bleeding tendency..

Transfusion of blood

Fresh-frozen plasma

Cryoprecipitate

Platelets

May be required

- Antibiotics prophylaxis.

Surgical TreatmentSplenectomy is indicated to help control or stage basic disease. These diseases can include hereditary spherocytosis, autoimmune thrombocytopenia or hemolysis, or Hodgkin disease.Splenectomy is also indicated for the treatment of chronic, severe hypersplenism. This can occur in conditions such as hairy cell leukemia, Felty syndrome, agnogenic myeloid metaplasia, thalassemia major, Gaucher disease, hemodialysis splenomegaly, or splenic vein thrombosis.Splenectomy allows treatment of hematologic disease.

Surgical TreatmentThe use of open versus laparoscopic splenectomy (LS) is now a controversy that has yet to be decided.

The greatest danger in either approach is hemorrhage. The value of preoperative splenic artery control or embolization remains unclear, but this issue is applicable to either approach. The technical feasibility of LS has been established. A teaching institution offers the optimal environment for LS. LS has replaced the open approach for many elective splenectomies. LS is associated with reduced pain, a more rapid resumption of regular diet, shortened hospitalization, and an earlier return to normal activities. The complication rate for LS remains low, with extremely rare wound or bleeding complications.

Problems inherent to laparoscopic treatment of splenomegaly include structural friability of the spleen, increased difficulties in the manipulation of an enlarged spleen, and exposure and access to the splenic hilum.

Surgical TreatmentOccasionally, severe thrombocytosis occurs following splenectomy, and this requires therapy to reduce the platelet count and prevent thrombotic complications.

This is most common in patients with massive splenomegaly from myeloproliferative disorders. Monitoring the platelet count following splenectomy in patients at risk of this complication permits early detection and prompt initiation of therapy.

An onset of fever several days following splenectomy can be due to a recrudescence of malaria.

This should be considered as a cause of fever in patients who have lived in areas commonly associated with malaria and in persons who abuse intravenous drugs who share needles. With Plasmodium malariae infection, this may occur decades after the initial infection. Malaria from Plasmodium vivax (3-7 y) and Plasmodium falciparum (~1 y) remain active for shorter intervals after the initial infection.

Complications of splenectomy;

Immediate complications:- Hemorrhage: Slipped ligature from the splenic artery.- Haematemesis: Gastric mucosa damage- Left basal atelectasis and pleural effusion.Surrounding structures damage:- Stomach - Tail of the Pancreas: Pancreatitis, pancreatic fistula.

Platelets may rise, and it this exceeds one million per milliter, aspirin is required to prevent thrombosis.

Post- splenectomy septicemia - Streptoeoccus pneumonia.- N. Neisseria meningitidis - E. coli- H. InfluenzaVaccinationsOpportunist post-splenectomy infection

Overwhelming postsplenectomy infection

(OPSI), also known as postsplenectomy sepsis syndrome, begins as a nonspecific flulike prodrome that is followed by a rapid evolution to full-blown bacteremic septic shock accompanied by hypotension, anuria, and clinical evidence of disseminated intravascular coagulation, thus making this syndrome a true medical emergency. The subsequent clinical course often mirrors that of the Waterhouse-Friderichsen syndrome, with bilateral adrenal hemorrhages noted at autopsy

Preventative strategies

Preventative strategies for OPSI fall into 3 major categories: education, immunoprophylaxis, and chemoprophylaxis. Asplenic patients should be encouraged to wear a Medi-Alert bracelet and carry a wallet card explaining their lack of a spleen. Patients should also be aware of the need to notify their physician in the event of an acute febrile illness, especially if associated with rigors or systemic symptoms.


An appropriate factor in preventing OPSI entails vaccination. This has best been defined for S pneumoniae. Unfortunately, the most virulent pneumococcal serotypes tend to be the least immunogenic, and evidence indicates that the efficacy of the vaccine is poorest in younger patients, who would be at higher risk. However, under ideal conditions in a healthy immunocompetent host, the vaccine offers a 70% protection rate. Pneumococcal vaccine should be performed at least 2 weeks before an elective splenectomy. If the timeframe is not practical, the patient should be immunized as soon as possible after recovery and before discharge from the hospital.


Most authorities recommend antibiotic prophylaxis for asplenic children, especially for the first 2 years after splenectomy. Some investigators advocate continuing chemoprophylaxis in children for at least 5 years or until aged 21 years. However, the value of this approach in older children or adults has never been adequately evaluated in a clinical trial.

Thank you

splenomegaly

Health & Medicine

splenic vien

large splenic artery

white blood cells

spleen hilum venous

blood supply

superior mesenteric

congestive failure

old cells