spleenomegaly & hypersplenism etiology pathogenesis and surgical management
DESCRIPTION
Spleenomegaly & hypersplenism etiology pathogenesis and surgical managementTRANSCRIPT
![Page 1: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/1.jpg)
SPLEENOMEGALY & HYPERSPLENISM
ETIOLOGY PATHOGENESIS AND SURGICAL MANAGEMENT
By
Dr Aravind
![Page 2: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/2.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/3.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/4.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/5.jpg)
Ligaments
• Gastrosplenic ligament
• Lienorenal ligament• Lineophrenic
ligament• Splenocolic ligament
![Page 6: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/6.jpg)
Blood supply
Artery• Splenic artery• Short gastric
arteries Veins• Splenic vein
![Page 7: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/7.jpg)
Red pulp(90%)• Cords and sinuses• Phagocytosis • Open circulation White pulp• Periarticular
lymphatic sheets• Immunoglobulins
![Page 8: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/8.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/9.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/10.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/11.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/12.jpg)
Causes
Based on pathological mechanism divided
Increased function Abnormal blood flow Infiltration
![Page 13: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/13.jpg)
Increased function
Removal of defective RBCs spherocytosis thalassemia hemoglobinopathies nutritional anemias early sickle cell anemia
![Page 14: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/14.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/15.jpg)
Extramedullary hematopoiesis myelofibrosis marrow infiltration by tumors, leukemias marrow damage by radiation, toxins
![Page 16: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/16.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/17.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/18.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/19.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/20.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/21.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/22.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/23.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/24.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/25.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/26.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/27.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/28.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/29.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/30.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/31.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/32.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/33.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/34.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/35.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/36.jpg)
Feltys syndrome
Rheumatiod arthritis Mild lekopenia Spleenomegaly
![Page 37: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/37.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/38.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/39.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/40.jpg)
Types of surgery
Open Laparoscopic Thoracoabdominal approach
(Abandoned)
![Page 41: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/41.jpg)
Open Spleenectomy
Anesthesia – General Position - Supine Incision – Left sub costal Midline in case of trauma
![Page 42: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/42.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/43.jpg)
short gastric vessels are divided between ligatures
Splenic vessels are isolated
![Page 44: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/44.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/45.jpg)
Search for accesory spleens should be done in elective cases
![Page 46: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/46.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/47.jpg)
Laproscopic Spleenectomy
Most of cases laproscopic speenectomy can be done
Patient Rt decubitus position
![Page 48: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/48.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/49.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/50.jpg)
Complications
Injury to Diaphragm which is rare in open spleenectomy
![Page 51: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/51.jpg)
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](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/52.jpg)
Post Splenectomy care
Immunisation
![Page 53: Spleenomegaly & hypersplenism etiology pathogenesis and surgical management](https://reader033.vdocument.in/reader033/viewer/2022061512/554b44a6b4c905b5378b4ee3/html5/thumbnails/53.jpg)
Antibiotic prophylaxis Controversial To prevent OPSI Pencillins given for two years after
spleenectomy in children