spleenomegaly & hypersplenism etiology pathogenesis and surgical management

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 cms in breadth

Weighs 150 -200gms

Accessory spleens called splenunculi

7cm

14 cm

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• Pitting • Culling• Storage of

platelet• Phagocytosis • Iron reutilisation

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

properdin, interferon

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• Budd–Chiari syndrome• splenic vein obstruction Infections• hepatic schistosomiasis• hepatic echinococcosis

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 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,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

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

orogastric tube is used during the 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 techniques like endovascular stapling, hemoclips

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