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Spleenomegaly & hypersplenism etiology pathogenesis and surgical management
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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