The Spleen

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The Spleen –A surgical Perspective

By; Col. Abrar Hussain Zaidi

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Introduction •The spleen is a meeting place of medicine and surgery. •It interests surgeons because of the need for Splenectomy and associated problems

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Anatomy It lies in the left Hypochondrium covered with peritoneum Abuts ribs 9-12, the stomach, the left kidney, the splenic flexure of the colon, and the tail of the pancreas.  A normal spleen weighs 150 g and is approximately 11 cm in craniocaudal length.

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Spleens that are prominent below the costal margin typically weigh 750-1000 g. Spleens weighing 400-500 g indicate splenomegaly spleens weighing more than 1000 g indicate massive splenomegaly. Splenomegaly -- moderate if the largest dimension is 11-20 cm and severe if the largest dimension is greater than 20 cm. In many instances, the spleen enlarges as it performs its normal functions.

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Anatomy

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Anatomy

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Anatomy

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Anatomy

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Anatomy

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Anatomy

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Physiology The spleen is a functionally diverse organ with active roles in; Immunosurveillance Hematopoiesis.

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Functions of the spleen The 4 most are: Clearance of microorganisms and particulate antigens from the blood stream Synthesis of immunoglobulin G (IgG), properdin (ie, an essential component of the alternate pathway of complement activation), and tuftsin (an immunostimulatory tetrapeptide); Removal of abnormal red blood cells (RBCs) Embryonic hematopoiesis in certain diseases.

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Splenomegaly - Causes on the basis of the pathogenic mechanism Increased function Abnormal blood flow Infiltration

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Splenomegaly - Causes Increased function Removal of defective RBCs spherocytosis thalassemia hemoglobinopathies nutritional anemias sickle cell anemia Immune hyperplasia Response to infection (viral,bacterial,fungal,parasitic) mononucleosis, AIDS, viral hepatitis subacute bacterial endocarditis, bacterial septicemia splenic abscess, typhoid fever brucellosis,leptospirosis, tuberculosis histoplasmosis malaria, leishmaniasis, trypanosomiasis ehrlichiosis

Splenomegaly - Causes Increased function Disordered immunoregulation rheumatoid arthritis SLE Serum sickness Autoimmune hemolytic anemia Immune thrombocytopenia sarcoidosis drug reactions Extramedullary hematopoiesis Myelofibrosis Marrow infiltration by tumors, leukemias marrow damage by radiation, toxins

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Splenomegaly - Causes Abnormal blood flow Organ Failure cirrhosis congestive heart failure Vascular Hepatic vein obstruction Portal vein obstruction Budd-Chiari syndrome Splenic vein obstruction Infections Hepatic schistosomiasis hepatic echinococcosis

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Splenomegaly - Causes Infiltration Metabolic diseases Gauchers disease Niemann-Pick disease Alpha-mannosidosis Hurler syndrome and other Mucopolysaccharidoses Amyloidosis Tangier disease Benign and malignant infiltrations Leukemias(acute,chronic,lymphoid and myeloid) lymphomas(Hodgkins and non-hodgkins myeloproliferative disorders metastatic tumors(commonly melanoma) histiocytosis X Hemangioma,lymphangioma splenic cysts hamartomas eosinophilic granuloma

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The causes of massive splenomegaly (>1000 g) Thalassemia visceral leishmaniasis (Kala Azar) schistosomiasis Chronic myelogenous leukemia Chronic lymphocytic leukemia lymphomas hairy cell leukemia myelofibrosis polycythemia vera Gauchers disease Niemann Pick disease sarcoidosis Autoimmune hemolytic anemia Malaria

Splenomegaly - Causes on the basis of the Etiological factor Inflammatory splenomegaly: Acute enlargement in infections or inflammations an increase in the defense activities of the organ. The demand for increased antigen clearance from the blood Increased numbers of reticuloendothelial cells in the spleen and stimulate accelerated antibody production with resultant lymphoid hyperplasia.

Hyperplastic splenomegaly: Splenomegaly - Due to work hypertrophy Results from the removal of abnormal blood cells from the circulation (either cells with intrinsic defects or cells coated with antibody) OR, in some cases, as the result of extramedullary hematopoiesis (ie, myeloproliferative disease).

Congestive splenomegaly: Develops as a result of increased venous pressure. cirrhosis with portal hypertension, splenic vein occlusion (thrombosis),  congestive heart failure (CHF)  Infiltrative splenomegaly: Splenomegaly is the result of engorgement of macrophages with indigestible materials (eg, sarcoidosis, Gaucher disease, amyloidosis, metastatic malignancy).

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Spherocytosis

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Spherocytosis

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Hemolytic anemia Anemia is due to hemolysis, the abnormal breakdown of red blood cells (RBCs) Intravascular hemolysis Extravascular hemolysis Causes, ranging from relatively harmless to lifethreatening. The general classification >Inherited >Acquired. Treatment depends on the cause and nature of the breakdown.

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Hemolytic anemia In a healthy person, a red blood cell survives 90 to 120 days (on average) in the circulation, so about 1% of human red blood cells break down each day. The spleen (part of the reticulo-endothelial system) is the main organ which removes old and damaged RBCs from the circulation. In healthy individuals, the break down and removal of RBCs from the circulation is matched by the production of new RBCs in the bone marrow.

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Hemolytic anemia When the rate of RBC breakdown is increased, and exceed the rate RBCs Production- anemia Bilirubin accumulate causing jaundice, Tests Peripheral blood smear microscopy: fragments of the red blood cells ("schistocytes") can be present some red blood cells may appear smaller and rounder than usual (spherocytes) Reticulocytes are present in elevated numbers. The level of LDH in the blood is elevated Haptoglobin levels are decreased If the direct Coombs test is positive, hemolysis is caused by an immune process.

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Tests Increased bilirubin Haemosiderin in the urine indicates chronic intravascular haemolysis. Increased Urobilinogen in the urine. Clinical findings > pallor in mucous membrane and skin >jaundice urobilinogen in urine >Splenomegaly >Pigmented gallstones may be found.

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Treatment Splenectomy Indicated : If the Splenomegaly underlies hypersplenism After splenectomy,patients have an increased risk for infectious diseases. After splenectomy, patients should be vaccinated against Haemophilus influenzae and Streptococcus pneumoniae. They should receive annual influenza vaccinations. Long-term prophylactic antibiotics should be given

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Hypersplenism. Hypersplenism is not a specific disease but a syndrome or cluster of symptoms characterized by: >enlargement of the spleen (splenomegaly), >defects in the blood cells, >an abnormally high turnover of blood cells. >Anaemia >Coagulation defects It is almost always associated with such specific disorders as cirrhosis of the liver or certain cancers.

The decision to perform a splenectomy

-depends on the severity and prognosis of the disease that is causing the hypersplenism.

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Splenectomy- indications Splenectomy always required There are two diseases for which a splenectomy is the only treatment— Primary cancers of the spleen Hereditary Spherocytosis (HS) In HS, the absence of a specific protein in the red blood cell membrane leads to the formation of relatively fragile cells that are easily damaged when they pass through the spleen. The cell destruction does not occur elsewhere in the body and ends when the spleen is removed. HS can appear at any age, even in newborns, splenectomy not done under the age of five

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Splenectomy- indications Splenectomy usually required splenectomy is usually recommendedinclude: Immune (idiopathic) thrombocytopenic purpura (ITP). platelets are destroyed by antibodies in the body's immune system. A splenectomy is the definitive treatment for this disease and is effective in about 70% of cases of chronic ITP. Trauma. ruptured by blunt as well as penetrating injuries to the chest or abdomen. Abscesses. relatively uncommon high mortality rate. Rupture of the splenic artery. This artery sometimes ruptures as a complication of pregnancy. Hereditary elliptocytosis. rare disorder. It is similar to HS red blood cells with defective membranes

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Splenectomy- indications Splenectomy sometimes required Hodgkin's disease, a serious form of cancer that causes the lymph nodes to enlarge. A splenectomy is often performed in order to find out how far the disease has progressed. Autoimmune hemolytic disorders. These disorders may appear in patients of any age but are most common in adults over 50. The red blood cells are destroyed by antibodies produced by the patient's own body (autoantibodies). Myelofibrosis. Myelofibrosis is a disorder in which bone marrow is replaced by fibrous tissue. It produces severe and painful splenomegaly. A splenectomy does not cure myelofibrosis but may be performed to relieve pain caused by the swelling of the spleen. Thalassemia. Thalassemia is a hereditary form of anemia that is most common in people of Mediterranean origin. A splenectomy is sometimes performed if the patient's spleen has become painfully enlarged.

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SplenectomyPreparation Blood transfusion Platelet transfusion Prophylaxis Vaccination Antibiotics Patient counseling

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Splenectomy Procedure Approaches Open surgical – Emergency [ trauma,rupture] / Elective laparoscopic Open surgical A- After the abdomen is entered, the spleen is located, and the artery leading to it is tied off (B). The ligament connecting the stomach and spleen is cut (C), as is the ligament connecting the spleen and colon (D). This frees the spleen for removal (E). (Illustration by GGS Inc.) that may be associated with different disorders.

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Splenectomy.Examination through the gastrosplenic omentum

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Laparoscopic Splenectomy. – Displaying the splenic artery

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Post operative care Local wound Systemic sepsis Haemodynamic

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Morbidity and mortality rates The outcome of the procedure varies with the underlying disease or the extent of other injuries. Rates of complete recovery from the surgery excellent, in the absence of severe injuries or medical problems. Splenectomy for HS patients is usually delayed in children until the age of five to prevent unnecessary infections; reported outcomes are very good. In ITP 80%–90% of children achieve spontaneous and complete remission in two to eight weeks. A small percentage develop chronic or persistent ITP, but 61% show complete remission by 15 years. No deaths in patients older than 15 have been attributed to ITP.

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Summary The spleen is a meeting place of medicine and surgery. Not all causes of splenomegaly will require splenectomy as part of their treatment. Elective splenectomy is required: >to treat haematological disease with medically uncontrolled hypersplenism, >occasionally to stage and diagnose lymphoma, >to treat splenic cysts, turnouts and abscesses, > for the relief of the discomfort associated with splenomegaly. Most of splenectomies in our set up are done for trauma , part of radical tumour surgery idiopathic thrombocytopenic purpura (ITP) ,Thelsemia

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Resources

Tufail M, Majid N J, Dar H M, Javed A. Splenectomy — a six years experience Proceeding Shaikh Zayed Postgrad Med Inst Jun 2003;17(1):39-45. Shaikh Zayed Hospital, Lahore The complete medical records of 100 patients undergoing splenectomy over a 6 years period (June 1992 -December 1998) for various conditions at Surgical Unit II, Shaikh Zayed Hospital, Lahore, were reviewed. Of these 100 patients, there were 57 males and 43 females. The median age was 35 years (range 8 - 70 years). Spleen was severely enlarged (>20cm) in 45 cases, of moderate size (11 - 20cm) in 6 cases and of normal size in 49 cases. Average weight of the spleen reported after histopathology was 450gm (range 150gm - 2.6 Kg). The overall complication and mortality rates were 21% and 7% respectively. Of the 7 patients who died, 6 patients (85.71%) were cirrhotics. Among these, highest mortality was seen in the patients belonging to Child`s grade C (57.1%) and those patients who underwent splenectomy in the emergency setting as an additional procedure along with gastro-oesophageal devascularization for upper gastro-intestinal variceal bleeding (71.4%). The syndrome of Overwhelming Post Splenectomy Infection (OP SI) was not seen in any of our patients. Removal of a massively enlarged spleen with pronounced hypersplenism, especially in portal hypertension, can be taxing for even an experienced surgeon. The operation should be performed by a senior and experienced member of the surgical team. However, the large weight of the spleen does not constitute a contraindication to splenectomy, but indications must he carefully selected, and the operative and peri-operative management, must be appropriate.

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Re sour ces BOOKS Hiatt, J. R., Phillips, E. H., and L. Morgenstern, eds. Surgical Diseases of the Spleen. New York: Springer Verlag, 1997. Wilkins, B. S., and D. H. Wright. Illustrated Pathology of the Spleen. Cambridge, UK: Cambridge University Press, 2000. Schwartz Principles of surgery 7th edition 1999 Baily and love –short practice of surgery PERIODICALS Al-Salem A. H., and Z. Nasserulla. "Splenectomy for Children with Thalassemia." Internal Surgery 87 (October-December 2002): 269-273. Duperier, T., J. Felsherm, and F. Brody. "Laparoscopic Splenectomy for Evans Syndrome." Surgical Laparoscopy, Endoscopy & Percutaneous Techniques 13 (February 2003): 45-47. Schwartz, J., M. D. Leber, S. Gillis, et al. "Long-Term Follow-Up After Splenectomy Performed for Immune Thrombocytopenic Purpura (ITP)." American Journal of Hematology 72 (February 2003): 94-98. Svarch, E., I. Nordet, J. Valdes, et al. "Partial Splenectomy in Children with Sickle Cell Disease." Haematologica 88 (February 2003): 281-287.

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