53
SPLEENOMEGALY & HYPERSPLENISM ETIOLOGY PATHOGENESIS AND SURGICAL MANAGEMENT By Dr Aravind

Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Embed Size (px)

DESCRIPTION

Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Citation preview

Page 1: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

SPLEENOMEGALY & HYPERSPLENISM

ETIOLOGY PATHOGENESIS AND SURGICAL MANAGEMENT

By

Dr Aravind

Page 2: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 3: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Anatomy of Spleen

Develops from mesenchymal cells in the dorsal mesogastrium during the fifth week of gestation.

Page 4: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Located between 9th and 11th ribs on left side

It is about 14cms in length and 7 cms in breadth

Weighs 150 -200gms

Accessory spleens called splenunculi

7cm

14 cm

Page 5: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Ligaments

• Gastrosplenic ligament

• Lienorenal ligament• Lineophrenic

ligament• Splenocolic ligament

Page 6: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Blood supply

Artery• Splenic artery• Short gastric

arteries Veins• Splenic vein

Page 7: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Red pulp(90%)• Cords and sinuses• Phagocytosis • Open circulation White pulp• Periarticular

lymphatic sheets• Immunoglobulins

Page 8: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Functions of spleen

Cellular• Pitting • Culling• Storage of

platelet• Phagocytosis • Iron reutilisation

Immunological• Synthesis of Ig M• Lymphocytes• Tuftsin, opsonin,

properdin, interferon

Page 9: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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.

Page 10: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 11: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 12: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Causes

Based on pathological mechanism divided

Increased function Abnormal blood flow Infiltration

Page 13: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Increased function

Removal of defective RBCs spherocytosis thalassemia hemoglobinopathies nutritional anemias early sickle cell anemia

Page 14: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 15: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Extramedullary hematopoiesis myelofibrosis marrow infiltration by tumors, leukemias marrow damage by radiation, toxins

Page 16: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Abnormal blood flow

Organ Failure • cirrhosis Vascular• hepatic vein obstruction• portal vein obstruction• Budd–Chiari syndrome• splenic vein obstruction Infections• hepatic schistosomiasis• hepatic echinococcosis

Page 17: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Infiltration

Metabolic diseases • Gauchers disease• Niemann–Pick disease• alpha-mannosidosis• Hurler syndrome and other

mucopolysaccharidoses• amyloidosis• Tangier disease

Page 18: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 19: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Causes of massive spleenomegaly

visceral leishmaniasis (kala-azar) chronic myelogenous leukemia myelofibrosis malaria primary lymphoma of spleen

Page 20: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 21: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Investigations• Fragility test - increased• Increased serum bilurubin• Increase dreticulocyte count• Increased feca lurobilonogen• Pheripheral smear spherocytes • Ultrasound of abdomen

Page 22: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Treatment Splenectomy is the sole treatment Associated gall stones -

Cholecystectomy Splenectomy should be delayed in

children till they reach 7 years

Page 23: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 24: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Clinical features• Usually after 50 years • female to male 2;1 • acute onset• Anemia,• Jaundice• Splenomegaly in 50% in patients • gall stones in 25%

Page 25: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 26: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Thalassamias

Thalassemia major (mediterranean anemia,Cooly’s 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

Page 27: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 28: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 29: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 30: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 31: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Clinical fetures• Purpuric patches over skin and mucus

membrane• Epistaxis• Heamaturia Hemarthrosis• GIT bleeding• Intracranial bleed• Hess test

Page 32: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Investigation• Bleeding time increased• Clotting and prothrombin time normal• Platelet count decreased• Bone marrow increased megakaryocytes• Anemia and neutropenia not present• Spleenomegaly

Page 33: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 34: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Indications for Splenectomy in ITP• Relapse • Girls reaching menarche• Refractory to treatment• Pregnancy with bleeding problems

Page 35: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Thrombotic thrombocytopenic purpura (TTP)

Arterioles and capillaries of micro circulation involved

Anemia Thrombocytopenia Altered mental functions neurological

deficits Plasmaphoresis spleenectomy

Page 36: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Feltys syndrome

Rheumatiod arthritis Mild lekopenia Spleenomegaly

Page 37: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Spleenectomy Indications

Absolute

Bleeding varices due to splenic vein thrombosis

Hereditary spherocytosis Massive splenic trauma Primary splenic malignancy

Page 38: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 39: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

orogastric tube is used during the operation

Page 40: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Types of surgery

Open Laparoscopic Thoracoabdominal approach

(Abandoned)

Page 41: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Open Spleenectomy

Anesthesia – General Position - Supine Incision – Left sub costal Midline in case of trauma

Page 42: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 43: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

short gastric vessels are divided between ligatures

Splenic vessels are isolated

Page 44: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Tail of pancreas is displaced medially to avoid injury

Splenic hilum is held enboc between three clamps and divided

Page 45: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Search for accesory spleens should be done in elective cases

Page 46: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Complications for open spleenectomy

Bleeding Left lower lobe atelectasis Subphrenic abcess Thrombosis of the splenic vein Injury to the tail of the pancreas

Page 47: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Laproscopic Spleenectomy

Most of cases laproscopic speenectomy can be done

Patient Rt decubitus position

Page 48: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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 techniques like endovascular stapling, hemoclips

Page 49: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 50: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Complications

Injury to Diaphragm which is rare in open spleenectomy

Page 51: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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

Page 52: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Post Splenectomy care

Immunisation

Page 53: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

Antibiotic prophylaxis Controversial To prevent OPSI Pencillins given for two years after

spleenectomy in children