Spleenomegaly & hypersplenism etiology pathogenesis and surgical management

1. SPLEENOMEGALY &
HYPERSPLENISM
ETIOLOGY PATHOGENESIS AND
SURGICAL MANAGEMENT
By
Dr Aravind

2.  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

3. Anatomy of Spleen
 Develops from
mesenchymal cells
in the dorsal
mesogastrium
during the fifth
week of gestation.

4.  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

5. Ligaments
• Gastrosplenic ligament
• Lienorenal ligament
• Lineophrenic ligament
• Splenocolic ligament

6. Blood supply
 Artery
• Splenic artery
• Short gastric
arteries
 Veins
• Splenic vein

7.  Red pulp(90%)
• Cords and sinuses
• Phagocytosis
• Open circulation
 White pulp
• Periarticular
lymphatic sheets
• Immunoglobulins

8. Functions of spleen
 Cellular
• Pitting
• Culling
• Storage of platelet
• Phagocytosis
• Iron reutilisation
 Immunological
• Synthesis of Ig M
• Lymphocytes
• Tuftsin, opsonin,
properdin, interferon

9. 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.

10. 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

11. 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

12. Causes
 Based on pathological mechanism divided
 Increased function
 Abnormal blood flow
 Infiltration

13. Increased function
 Removal of defective RBCs
 spherocytosis
 thalassemia
 hemoglobinopathies
 nutritional anemias
 early sickle cell anemia

14.  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

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

16. Abnormal blood flow
 Organ Failure
• cirrhosis
 Vascular
• hepatic vein obstruction
• portal vein obstruction
• Budd–Chiari syndrome
• splenic vein obstruction
 Infections
• hepatic schistosomiasis
• hepatic echinococcosis

17. Infiltration
 Metabolic diseases
• Gauchers disease
• Niemann–Pick disease
• alpha-mannosidosis
• Hurler syndrome and other
mucopolysaccharidoses
• amyloidosis
• Tangier disease

18.  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

19. Causes of massive spleenomegaly
 visceral leishmaniasis (kala-azar)
 chronic myelogenous leukemia
 myelofibrosis
 malaria
 primary lymphoma of spleen

20. 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

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

22. Treatment
 Splenectomy is the sole treatment
 Associated gall stones - Cholecystectomy
 Splenectomy should be delayed in
children till they reach 7 years

23. 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

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

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

26. 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

27.  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

28. 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

29.  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

30. 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

31.  Clinical fetures
• Purpuric patches over skin and mucus
membrane
• Epistaxis
• Heamaturia Hemarthrosis
• GIT bleeding
• Intracranial bleed
• Hess test

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

33.  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

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

35. Thrombotic thrombocytopenic
purpura (TTP)
 Arterioles and capillaries of micro circulation
involved
 Anemia
 Thrombocytopenia
 Altered mental functions neurological deficits
 Plasmaphoresis
 spleenectomy

36. Feltys syndrome
 Rheumatiod arthritis
 Mild lekopenia
 Spleenomegaly

37. Spleenectomy Indications
Absolute
 Bleeding varices due to splenic vein
thrombosis
 Hereditary spherocytosis
 Massive splenic trauma
 Primary splenic malignancy

38.  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

39. 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

40. Types of surgery
 Open
 Laparoscopic
 Thoracoabdominal approach (Abandoned)

41. Open Spleenectomy
 Anesthesia – General
 Position - Supine
 Incision – Left sub costal
Midline in case of trauma

42.  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

43.  short gastric vessels
are divided between
ligatures
 Splenic vessels are
isolated

44.  Tail of pancreas is
displaced medially
to avoid injury
 Splenic hilum is held
enboc between
three clamps and
divided

45.  Search for accesory
spleens should be
done in elective
cases

46. Complications for open
spleenectomy
 Bleeding
 Left lower lobe atelectasis
 Subphrenic abcess
 Thrombosis of the splenic vein
 Injury to the tail of the pancreas

47. Laproscopic Spleenectomy
 Most of cases
laproscopic
speenectomy can
be done
 Patient Rt decubitus
position

48.  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

49.  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

50. Complications
 Injury to Diaphragm which is rare in open
spleenectomy

51. 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

52. Post Splenectomy care
 Immunisation

53.  Antibiotic prophylaxis
 Controversial
 To prevent OPSI
 Pencillins given for two years after
spleenectomy in children