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    Spleenomegaly & Hypersplenism Etiology Pathogenesis and Surgical Management

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    Muhammad Saad
    This document discusses splenomegaly and hypersplenism, including their etiology, pathogenesis, and surgical management. It covers anatomy of the spleen, causes of splenomegaly such as hereditary spherocytosis and thalassemias, and indications for splenectomy in conditions like immune thrombocytopenic purpura. It also describes the open technique for splenectomy, including patient preparation, incision types, and steps like mobilizing and isolating the spleen and dividing attachments.

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    Spleenomegaly & Hypersplenism Etiology Pathogenesis and Surgical Management

    Uploaded by

    Muhammad Saad
    82 views53 pages
    This document discusses splenomegaly and hypersplenism, including their etiology, pathogenesis, and surgical management. It covers anatomy of the spleen, causes of splenomegaly such as hereditary spherocytosis and thalassemias, and indications for splenectomy in conditions like immune thrombocytopenic purpura. It also describes the open technique for splenectomy, including patient preparation, incision types, and steps like mobilizing and isolating the spleen and dividing attachments.

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    This document discusses splenomegaly and hypersplenism, including their etiology, pathogenesis, and surgical management. It covers anatomy of the spleen, causes of splenomegaly such as hereditary spherocytosis and thalassemias, and indications for splenectomy in conditions like immune thrombocytopenic purpura. It also describes the open technique for splenectomy, including patient preparation, incision types, and steps like mobilizing and isolating the spleen and dividing attachments.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    82 views53 pages

    Spleenomegaly & Hypersplenism Etiology Pathogenesis and Surgical Management

    Uploaded by

    Muhammad Saad
    This document discusses splenomegaly and hypersplenism, including their etiology, pathogenesis, and surgical management. It covers anatomy of the spleen, causes of splenomegaly such as hereditary spherocytosis and thalassemias, and indications for splenectomy in conditions like immune thrombocytopenic purpura. It also describes the open technique for splenectomy, including patient preparation, incision types, and steps like mobilizing and isolating the spleen and dividing attachments.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 53
    At a glance
    Powered by AI
    The key takeaways are that the spleen plays an important role in immunity and filtering blood. It can become enlarged (splenomegaly) due to various causes and may require surgical removal (splenectomy).

    The functions of the spleen are cellular (filtering blood and storing platelets) and immunological (producing antibodies and lymphocytes).

    The causes of splenomegaly can be increased function (filtering defective blood cells), immune hyperplasia (response to infection), or infiltration (by tumors or leukemia).

    SPLEENOMEGALY &

    HYPERSPLENISM
    ETIOLOGY PATHOGENESIS AND
    SURGICAL MANAGEMENT
    By
    Dr Aravind
    Spleen is the largest lymphoid organ of
    the body
    It plays important role in Red blood cells
    sequestration and immunity
    Store house of platelets
    Produces RBC and WBC in fetus during
    gestation period and some times in
    adults
    Anatomy of Spleen
    Develops from
    mesenchymal cells
    in the dorsal
    mesogastrium
    during the fifth
    week of gestation.
    Located between
    9th and 11th ribs on
    left side
    It is about 14cms
    in length and 7 7cm

    cms in breadth
    Weighs 150
    -200gms 14 cm

    Accessory spleens
    called splenunculi
    Ligaments
    Gastrosplenic
    ligament
    Lienorenal ligament

    Lineophrenic

    ligament
    Splenocolic ligament
    Blood supply
    Artery
    Splenic artery
    Short gastric
    arteries
    Veins
    Splenic vein
    Red pulp(90%)
    Cords and sinuses
    Phagocytosis
    Open circulation
    White pulp
    Periarticular
    lymphatic sheets
    Immunoglobulins
    Functions of spleen
    Cellular Immunological
    Pitting Synthesis of Ig M
    Culling Lymphocytes
    Storage of Tuftsin, opsonin,
    platelet properdin,
    Phagocytosis interferon
    Iron reutilisation
    Hyperspleenism
    Hypersplenism is a condition in which the
    spleen becomes increasingly active and
    then rapidly removes the blood cells
    Splenomegaly,
    Pancytopenia or a reduction in the number
    of one or more types of blood cells
    Maturation arrest
    decreased red blood cells survival
    decreased platelet survival.
    Spleenomegaly
    Normally spleen not palpable
    Size 2 to 3 times the size spleen is
    palpable
    Weight 400-500 gms
    Spleen size is not a reliable indicator of
    spleen function
    Clinical features
    Mass in left hypochondrium
    Notch felt
    Moves with respiration
    Dull on percussion
    Directed toward Rt iliac fossa
    Hook sign
    Can not insinuate fingers under Lt costal
    margin
    Causes
    Based on pathological mechanism
    divided
    Increased function
    Abnormal blood flow
    Infiltration
    Increased function
    Removal of defective RBCs
    spherocytosis
    thalassemia
    hemoglobinopathies
    nutritional anemias
    early sickle cell anemia
    Immune hyperplasia
    Response to infection (viral, bacterial, fungal, parasitic)
    mononucleosis, AIDS, viral hepatitis
    subacute bacteria endocarditis, bacterial septicemia
    splenic abscess, typhoid fever
    brucellosis, leptospirosis, tuberculosis
    histoplasmosis
    malaria, leishmaniasis, trypanosomiasis
    ehrlichiosis
    Disordered immunoregulation
    rheumatoid arthritis
    Systemic lupus erythematosus
    serum sickness
    autoimmune hemolytic anemia
    sarcoidosis
    drug reactions
    Extramedullary hematopoiesis
    myelofibrosis

    marrow infiltration by tumors, leukemias

    marrow damage by radiation, toxins


    Abnormal blood flow
    Organ Failure
    cirrhosis
    Vascular
    hepatic vein obstruction
    portal vein obstruction
    BuddChiari syndrome
    splenic vein obstruction
    Infections
    hepatic schistosomiasis
    hepatic echinococcosis
    Infiltration
    Metabolic diseases
    Gauchers disease
    NiemannPick 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 disease
    metastatic tumors (commonly melanoma)
    histiocytosis X
    hemangioma, lymphangioma
    splenic cysts
    hamartomas
    eosinophilic granuloma
    littoral cell angioma
    Causes of massive spleenomegaly

    visceral leishmaniasis (kala-azar)


    chronic myelogenous leukemia
    myelofibrosis
    malaria
    primary lymphoma of spleen
    Hereditary spherocytosis

    Autosomal dominant inheritance


    Most common congenital hemolytic anemia

    Red cell membrane lacks the necessary protein assembly.

    (spectrin &ankyrin)
    decrease cellular plasticity with membrane loss

    RBCs small,dense, deformed

    hemolysis(in the spleen )

    Clinical features
    hemolytic anemia,

    splenomegaly allmost always

    jaundice .

    Periodic exacerbation (follow viral infections )

    Pigmented gall stones,CBD stones, Cholangitis


    Investigations
    Fragility test - increased
    Increased serum bilurubin
    Increase dreticulocyte count
    Increased feca lurobilonogen
    Pheripheral smear spherocytes
    Ultrasound of abdomen
    Treatment
    Splenectomy is the sole treatment

    Associated gall stones -

    Cholecystectomy
    Splenectomy should be delayed in

    children till they reach 7 years


    Auto Immune Hemolytic anaemia

    Production of IgG and IgM autoantibodies specific


    for cell membrane proteins on erythrocytes
    Classified to
    Common warm antibodies (40-50% of cases )

    Due to Ig G antibodies

    Associated with CLL

    Less Common antibodies

    Due to Ig M antibodies

    The hemolysis occur intravascularly & not in

    within the spleen


    Clinical features
    Usually after 50 years

    female to male 2;1

    acute onset

    Anemia,

    Jaundice

    Splenomegaly in 50% in patients

    gall stones in 25%


    Treatment
    Corticosteroids produce remission in
    75%
    Splenectomy is indicated in warm
    antibodies anemia who fail to
    respond to 4-6 weeks of high dose
    corticosteroids
    Thalassamias
    Thalassemia major (mediterranean
    anemia,Coolys Anemia )
    Dominant autosomal inheritance
    Deficit in synthesis of peptide chain .(alpha, beta,
    gamma)leading to decrese in Hb-A
    Manifest at first year of life
    Failure to thrive
    Severe chronic anemia
    Large head, splenomegaly
    Leg ulcers
    Succeptiblity to infection
    Pigment gall stones 25% of patients
    Investigations
    Electrophoresis low Hb-A
    Persistance of Hb-F (fetal)
    Treatment
    Iron chelation
    Blood Transfusion
    Splenectomy may reduce the need for
    transfusion

    Thassemia minor - most patients are


    asymptomatic ,may have mild anemia
    Sickle Cell anemia
    Replacement of normal hemoglobin A
    (Hb-A) by sickle hemoglobin Hb-S
    Crescent shaped RBC more prone for
    trapping in spleen
    Spleenic micro infracts are common
    Initially splenomegaly and latter auto
    spleenectomy
    Clinical features
    Anemia
    Pain abdomen
    Leg ulcers
    Cerebral pulmonary and mesenteric infracts
    Diagnosis by electrophoresis
    Treatment
    Sodium cyanate
    Partial exchange transfusion
    Antibiotics
    Spleenectomy has limeted role
    Idiopathic Thrombocytopenic purpura
    (ITP)

    Results from destruction of platelets by


    circulating IgG antiplatelets factors
    originating from spleen
    Common in females
    Acute common in children
    Spontaneous remission
    Platelets below 50000/cc cause bleeding
    Regular follow up
    Clinical fetures
    Purpuric patches over skin and mucus
    membrane
    Epistaxis
    Heamaturia Hemarthrosis
    GIT bleeding
    Intracranial bleed
    Hess test
    Investigation
    Bleeding time increased
    Clotting and prothrombin time normal
    Platelet count decreased
    Bone marrow increased megakaryocytes
    Anemia and neutropenia not present
    Spleenomegaly
    Treatment
    Methly prednislone IV for 3-5 days
    Oral prednisolone 6-12 weeks
    IV immuniglobin 0.4- 1 gm/kg for 5 days
    Vincristin 2 mg/week 6 weeks
    Danazol 200mg tid
    Anti RhD antibodies
    Azathiprine
    Splenectomy
    FFP, platelets and whole blood transfusions
    Indications for Splenectomy in ITP
    Relapse
    Girls reaching menarche
    Refractory to treatment
    Pregnancy with bleeding problems
    Thrombotic thrombocytopenic purpura
    (TTP)

    Arterioles and capillaries of micro


    circulation involved
    Anemia
    Thrombocytopenia
    Altered mental functions neurological
    deficits
    Plasmaphoresis
    spleenectomy
    Feltys syndrome
    Rheumatiod arthritis
    Mild lekopenia
    Spleenomegaly
    Spleenectomy Indications
    Absolute

    Bleeding varices due to splenic vein


    thrombosis
    Hereditary spherocytosis
    Massive splenic trauma
    Primary splenic malignancy
    Relative
    Autoimmune hemolytic anemia
    Hypersplenism due to portal HTN
    Idiopathic thrombocytopenic purpura (ITP)
    Leukemia (chronic myloid leukemia )
    Lymphoma
    Primary hypersplenism
    Myelofibrosis
    Sickle-cell disease
    Splenic abscess
    Staging for hodgkins lymphoma
    Thalassemia
    Thrombotic thrombocytopenic purpura
    Radical gasterctomy involving removal of spleen
    Preoperative preparation
    Blood grouping and typing
    Cross matched blood
    Platelets should not be administered
    preoperatively in patient with idiopathic
    thrombocytopenic purpura
    In myeloproliferative disorders administer low-
    dose heparin, 5000 units twice daily, and aspirin
    on the day before surgery and to continue this
    regimen for 5 days postoperatively
    Vaccines against Streptococcus pneumonia,
    Haemophilus influenzae type B, and Neisseria
    meningitides are administered 14 days before
    operation
    Types of surgery
    Open
    Laparoscopic
    Thoracoabdominal approach
    (Abandoned)
    Open Spleenectomy
    Anesthesia General
    Position - Supine
    Incision Left sub costal
    Midline in case of trauma
    Mobilization of the
    spleen to the
    midline by division
    of the lateral and
    superior pole
    attachments
    The splenocolic and
    splenorenal
    ligaments at the
    lower pole are
    divided
    short gastric
    vessels are
    divided between
    ligatures
    Splenic vessels
    are isolated
    Tail of pancreas is
    displaced
    medially to avoid
    injury
    Splenic hilum is
    held enboc
    between three
    clamps and
    divided
    Search for
    accesory spleens
    should be done in
    elective cases
    Complications for open spleenectomy

    Bleeding
    Left lower lobe atelectasis
    Subphrenic abcess
    Thrombosis of the splenic vein
    Injury to the tail of the pancreas
    Laproscopic Spleenectomy
    Most of cases
    laproscopic
    speenectomy can
    be done
    Patient Rt
    decubitus position
    First abdomen searched for accessory
    spleens
    All Ligaments examined first
    gastrosplenic ligament is opened to see
    the tail of pancreas
    Splenocolic ligament is divided spleen
    retracted
    Short gastric vessels divided tail of
    pancres and vascular bundle are
    visualised
    Splenic vessels divided by various
    Spleen is held by
    lineophrenic
    ligament only
    A nylon bag is used
    as retrieval bag
    Brought near
    epigastric or
    supraumblical port
    and its open spleen
    morcellated
    removed piece meal
    Complications
    Injury to Diaphragm which is rare in
    open spleenectomy
    Common complications of both methods

    Postsplenectomy sepsis
    (increasesd incidence of pneumonia ,
    septicemia,meningitis )

    Overwhelming post splenctomy


    sepsis(0,8 % in adult,high in children)

    Thrombocytosis

    Splenosis
    Post Splenectomy care
    Immunisation
    Antibiotic prophylaxis
    Controversial
    To prevent OPSI
    Pencillins given for two years after
    spleenectomy in children

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