This document provides information on hypersplenism and its surgical management. It begins with definitions of hypersplenism and classifications of primary and secondary hypersplenism. The indications for splenectomy include bleeding varices, hereditary spherocytosis, massive splenic trauma, and others. The surgical technique involves mobilizing the spleen through ligation of attachments and dissection of the hilum before removing the spleen and ligating vessels. Precise ligation of the splenic artery and vein is important to safely remove an enlarged spleen.

1. HYPERSPLENISM:
SURGICAL
MANAGEMENT
DR PRANAY PANIGRAHI

2. INTRODUCTION
 The spleen was regarded by Galen as “an organ of
mystery,”by Aristotle as unnecessary, and by Pliny
as an organ that might hinder the speed of runners
 The term ‘hypersplenism’ first appeared in the
thesis of Anatole Chauffard in 1907 and
subsequently in the study of Morawitz and
Denecked

3. EMBRYOLOGY
 Spleen is the largest reticuloendothelial organ in the
body
 From the primitive mesoderm of dorsal mesogastrium
 Evident in the fifth week of gestation in an 8 mm
embryo
 The most common variation of splenic embryology is
the accessory spleen . Present in up to 14-29 % of
the population

4. ANATOMY
 The spleen is located in the left upperquadrant.
Lies between the 9th -11th rib and weighs about
150(75-250g). Measures about13x 7 x 45cm in
dimention.
Attachement
 Laterally- lienorenal ligament
 Anteriorly-gastrosplenic ligament (contains short
gastric arteries)
 Superiorly-splenophrenic ligament
 Inferiorly-splenocolic ligament

6. RELATIONS
 Anterior-fundus of
the stomach
 Medially- tail of
the pancreas
 Inferiorly- splenic
flexure
 Superiorly-
diaphragm
 Posteriorly- upper
part of the kidney

9. SPLENIC ARTEY & VEIN
Blood supply; spleen artery, short gastric arteries
 The distributed type :is the most common (70%) and is
distinguished by a short trunk with many long branches.
 The less common magistral type of splenic artery
(30%) has a long main trunk dividing near the hilum into
short terminal branches.
 The splenic vein joins the superior mesenteric vein to
form the portal vein and accommodates the major
venous drainage of the spleen.

11. NORMAL PHYSIOLOGIC ROLES
Red pulp(90%)- Cords and sinuses Phagocytosis
White pulp- Periarticular lymphatic sheets
Immunoglobulins.

12.  Reservior for platelets,monocytes,FVIII etc.
 Haematopoiesis in fetus
 Repairs and destruction of RBC’s by culling & pitting.
 Immune function: IgM ,properidin,tuftsin are produced
by spleen.
 prevention of infection By capsulated org.(H.influ
etc)role in phagocytosis.

13. APPROACH TO THE PATIENT
 The most common symptoms produced by
diseases involving the spleen are pain and a
heavy sensation in the LUQ


14.  Inspection may reveal fullness in the LUQ.
 Palpation.
 Percussion- Nixon, Castell, Percussion of Traube's
semilunar space.
 Auscultation- reveal a venous hum or friction rub.

15. HYPERSPLENISM
DEFINITION-
Hypersplenism is a clinical syndrome characterized by:
SPLENOMEGALY, although this may be only moderate
PANCYTOPENIA or a reduction in the number of one or more
types of blood cells, neutropenia is less common than anemia
and thrombocytopenia
HYPERPLASIA of the precursor cells in the marrow or a so
called maturation arrest
Decreased RBCsurvival
Decreased platelet survival.

16.  In Hypersplenism, its normal function
accelerates, and begins automatically to
remove cells that may still be normal in
function.
 Sometimes, the spleen will temporarily
sequestrate 90% of the body platelets and
45% of the red cells.

17.  Hypersplenism can be classified into three categories by its
etiological causes as follows.( Yunfu et al., 2016)
Primary hypersplenism
Cause is not clear.
1. Primary splenic hyperplasia
2. Non-tropical idiopathic splenomegaly
3. Primary splenic granulocytopenia
4. Primary splenic pancytopenia
5. Splenic anemia or thrombocytopenia

18. Secondary hypersplenism
Cause is clear
A. Infections - viral hepatitis, brucellosis, subacute or chronic
diseases, infectious mononucleosis syndrome and malaria.
B. Alcohol use such as long-term or excessive drinking
C. Portal hypertension (PH) such as liver cirrhosis of various
causes including Post-hepatitic Cirrhosis, Alcoholic
Cirrhosis, Biliary Cirrhosis, Fatty Liver Cirrhosis, Post-
hepatitic Autoimmune Cirrhosis, Schistosomiasis-induced
Cirrhosis, & Drug-induced Cirrhosis, as well as
Hemosiderosis And Portal Vein Thrombosis.
D. Granulomatous inflammation - Systemic Lupus
Erythematosus, Rheumatoid Arthritis, Chronic Syphilis,
Chronic Tuberculosis, Felty's Syndrome, & Sarcoidosis.

19.  Malignancies - Splenic lymphosarcoma, leukemia, and
cancer metastasis.
 Chronic hemolytic diseases such as hereditary
spherocytosis, autoimmune hemolytic anemia, and
thalassemia.
 Lipidosis such as Gaucher's disease, and Niemann-Pick
disease.
 Myeloproliferative disorders- Polycythemia Vera, Chronic
Myeloid Leukemia, Myelofibrosis

20. OCCULT HYPERSPLENISM
 Sometimes due to benign bone marrow hyperplasia and
sufficient bone marrow compensation, peripheral
cytopenias may not occur.
 In this case, hypersplenism becomes occult with no
symptoms.
 However, once the bone marrow hematopoietic function is
suppressed by factors such as infection or drugs,
monolineage or multilineage peripheral cytopenia occurs,
accompanied by clinical symptoms, which is not classified
as occult hypersplenism.

21. MASSIVE SPLENOMEGALY (>20CM,>1000GM)
Causes
 Leishmaniasis
 Malaria
 Myeloproliferative disease
 Portal vein obstruction/portal hypertension
 Schistosomiasis
 NiemannPick disease
 Mucopolysaccharidosis
 Lymphomas
 Gaucher disease
 Hereditary spherocytosis

22. ON CT OR POST-RESECTION WEIGHT
SPLENIC LENGTH (CM) SPLENIC WEIGHT
(GM)
Normal spleen Up to 13 <300
Mild splenomegaly >13–15 300–500
Moderate splenomegaly 16–20 500–1000
Massive splenomegaly >20 >1000
gm with etiological dia
gnosis

23. HACKETT’S GRADING SYSTEM FOR PALPABLE
SPLENOMEGALY
MILD-palpable <3cms below LCM
MODERATE-4-7 below LCM
SEVERE- >7cms below LCM

24. INVESTIGATION
 Ultrasound -
The spleen is considered to be normal in size if its length is <13
cm or its thickness is ≤5 cm
 CT Scanning-
In general, the spleen can be considered enlarged if its
craniocaudal length is more than 10cm on conventional
CT scans.
Spleen that extends below the lower third pole of the kidney
is also indicative of splenomegaly

25.  LiverSpleen Colloid Scanning-
A splenic length of greater than 14 cm is considered enlarged on
liverspleen scan .
Erythrocytes are labeled with chromium51, mercury197 ,rubidium-
81
 Splenectomy and Splenic Biopsy

26. LABORATORY STUDIES
 Complete blood cell count (CBC) with differential.
 Liver function testing
 Hepatitis B and C testing
 Lactate dehydrogenase (LDH)
 Erythrocyte sediumentation rate (ESR)
 Evaluation of peripheral blood smear for RBC morphology & sig
ns of myeloproliferative disorders underlying
bone marrow disorders
 Prothrombin time (INR) and activated partial
thromboplastin time (aPTT)

27. PREOPERATIVE PLANNING


28.  Blood grouping and Cross matching
 Platelets should not be administered preoperatively in
patient with ITP
 In myeloproliferative disorders administer low-dose
heparin and aspirin on the day before surgery upto 5
days postoperatively.
 Orogastric tube is used during the operation
 Preoperative embolization(massive spleen)
Perioperative steroids are usually given if a patient has
had prolonged steroid treatment

29. INDICATION OF SPLENECTOMY
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 myeloid leukemia )

30.  Lymphoma
 Primary hypersplenism
 Myelofibrosis
 Sickle-cell disease
 Splenic abscess
 Staging for hodgkins lymphoma
 Thalassemia
 Thrombotic thrombocytopenic purpura
 Radical gasterctomy involving removal of spleen

31. SURGICAL TECHNIQUE
 Splenectomy related to blunt abdominal trauma, staging
of Hodgkin disease an upper midline incision to facilitate
dissection of the lower peri-aortic and iliac nodes.
 In hematologic disorder, a left oblique subcostal incision
beginning to the right of the midline and proceeding
obliquely outward and downward approximately two
finger breadths below the costal margin give excellent
exposure.

32.  In patients with ITP and a small spleen, the
oblique muscles do not have to be divided. With
significant splenomegaly, the oblique muscles are
divided laterally in the direction of their fibers.
 Preoperative angiographic embolization can be
considered to reduce bleeding in cases of
massive splenomegaly.

33.  Splenectomy starts with mobilization and dissection down
to an ultimate pedicle of splenic artery and vein.
 Transection of the ligamentous attachments, including the
splenophrenic ligament at the superior pole and the
splenocolic and splenorenal ligaments at the inferior pole.
 This may be accomplished by blunt dissection or scissors
dissection.
 These ligaments are avascular except when the patient
has portal hypertension.
 Mobilized by continual retroperitoneal dissection

34. TRANSECTION OF LIGAMENTOUS ATTACHMENTS

35.  After the ligamentous attachments are transected, two
to six gastric vessels that run from the spleen to the
greater curvature of the stomach should be ligated in
continuity and divided
 Often, this can best be performed before delivering the
spleen into the wound.

37. Spleen mobilized and elevated into the wound following division of
ligament attachments and posterior dissection

38.  After these maneuvers are completed, the spleen can be
delivered into the wound by blunt dissection of the posterior
attachments.
 Care should be taken not to divide the posterior
attachments too far medially to avoid entering the splenic
vein.
 One should also avoid axial rotation of the spleen because
this may lead to disruption of the splenic artery or vein.

39. Dissection is carried out at the hilus as close to the spleen
as possible to avoid injury to the pancreas.
Individual ligation of the splenic artery or arterial branches
and the splenic vein or venous branches is generally
preferable.

40. Splenic artery ligation is managed by double ligation and
suture ligature, whereas the splenic vein can be doubly
ligated and divided.
In the case of a markedly enlarged spleen, occasionally one
must place a vascular clamp on the splenic vein and close
the lumen with continuous vascular suture.

41. LIGATION OF THE SPLENIC ARTERY AND SPLENIC VEIN IN RELATION TO
THE HILUS

42. Three major areas to be inspected for bleeding:-
(a) the inferior surface of the diaphragm.
(b) the greater curvature of the stomach and the region of
the short gastric vessels.
(c) the region of the hilus.
(d)short gastric vessels that have been divided.

43. An integral part of splenectomy for
hematologic disease is a thorough
exploration to detect any accessory
spleens.

44. PREOPERATIVE SPLENIC ARTERY EMBOLIZATION
(SPIGOS ET AL, 1979, )
 Applied in the treatment of PH, hypersplenism, and
bleeding esophagogastric varices.
 Increases platelet and leukocyte counts.
 Reduces splenic size, improves pancytopenia, and
stimulates the immune system.
RISKS OF SPLENIC ARTERY EMBOLIZATION (SAE)
 Post-embolization syndrome: pain, fever, ileus, pleural
effusion
 Pancreatitis
 Splenic abscess or rupture
 Peritonitis

45. SAE can be used preoperative intervention to
reduce vascularity and size of massive spleen in
preparation for a laparoscopic approach.
Embolization is achieved using microcoils and/ or
Gelfoam.

46. LAPAROSCOPIC SPLENECTOMY
 Laparoscopic techniques have improved and most
patients today are considered for elective
laparoscopic splenectomy.
 The complicating factors are a large spleen (>500
g), suspected perisplenitis (most common in
patients with previous infectious diseases of the
spleen or portal hypertension) and previous gastric
surgery.

47.  ITP patients and staging laparotomy is suited ideally for
laparoscopic approaches as well.
 Position- right side down
 Ports-
1. midline and 4 cm below the spleen tip,
2. near the tip of the 11th rib along the posterior axillary line, and a
third is a
3. half way between the other two, along the anterior axillary line.
Occasionally, a fourth port may be required.
 scissors with cautery or preferably the harmonic Scalpel can be
used to take down the lateral peritoneal attachments and can be
used to ligate short gastric vessels.
 ligate and divide the short gastric vessels then ligate the splenic
artery and vein.

49.  Specimen delivery - morselization of the spleen in a bag or port
site can be enlarged to facilitate removal
 If the spleen is too large, a small Pfannenstiel incision and
removing the spleen through a suprapubic area may be more
cosmetically satisfactory.
 Laparoscopic splenectomy in children has increased with
frequency and can be done for hematologic disease, hereditary
spherocytosis.
 Accessory spleens can be localized and removed
laparoscopically by following the pattern of the most common
location.

50. HAND-ASSISTED SPLENECTOMY
Hand-assisted laparoscopic surgery (HALS ---
 As an alternative to the LS approach with same positioning
Spleen greater than 22 cm in craniocaudal length or 19 cm in
width may benefit.
Merit
 Marked reduction in average operative time.
 This technique allows for a tactile feedback and atraumatic
manipulation of the enlarged spleen.
Demerit
Require a small incision (7–8 cm) for hand insertion and
specimen extraction.

51. SINGLE-INCISION LAPAROSCOPIC SURGERY
(SILS)
 One small transabdominal incision
 Theoretical benefits of less pain and better cosmetics.
 incision -periumbilical and is used as the specimen
extraction site.
 Technical challenging for solid organs- since all
instruments are closely aligned together.
 Limited degrees of movement

52. ROBOTIC SPLENECTOMY
 Unique three-dimensional visualization of the surgical field.
 Facilitates movement with higher precision than standard
laparoscopy.
 Robotic splenectomy is very similar to standard laparoscopy,
although not as cost effective.
 No clear benefit of robotic versus laparoscopic splenectomy.

53. Splenoptosis (wandering spleen) refers to a rare
condition in which the spleen hangs by a long pedicle
from the mesentery and may present itself as an
asymptomatic mass or with symptoms of intermittent or
acute abdominal pain due to torsion.
Treatment involves splenectomy in cases of ischemia but
splenopexy should be considered in other cases

54. POST OPERATIVE MANAGEMENT
 Remove NG tube and the suction drain when
drainage is minimal (usually 24 - 48hours)
 Commence oral when bowel activity resumes.
 Long term oral penicillin 250mg daily.
 Pneumococcal vaccine 2weeks post op.
 Anti-malaria prophylaxis.

55. COMPLICATIONS
Early
Acute gastric dilatation
Fundal ischemia- hematemesis, perforation
Pancreatic fistula
Portal vein thrombosis
Reactionary hemorrhage from splenic vessel
The most common site of bleeding is the short gastric
vessels - 4% to 16% of patients
 Late
Infection; pneumococcal, viral, OPSI thrombocytosis

56. OVERWHELMING POSTSPLENECTOMY
INFECTION (OPSI)
Post Splenectomy leads to reduced IgM, tuftin, properdin
and other antibodies, phagocytosis of encapsulated bacteria
is defective.
 Post-splenectomised patient is more prone for
Pneumococcal septicaemia (commonest), N. meningitides,
H. infl uenzae, Babesia microti infections.
 Incidence is 4%.
 Common in first two years after splenectomy.

57. Clinical Features-
 Prodromal phase—fever, chills, sore throat.
 Hypotension, shock.
 DIC.
 Respiratory distress, coma, death.
 Mortality for fully developed OPSI—50-70%.
Prevention
 Pneumococcal vaccine should be given to all splenectomised
patients.
 Polyvalent pneumo-vac is given 2-3 weeks prior to surgery and
soon after recovery from surgery and it is repeated once in 5
years (Given to patients older than 2 years).
 Other vaccines - meningococcal vaccine (only to those who
travel with high-risk), H. influenzae ‘B’ vaccine (to all whatever
the age, once in 10 years).
 In malaria endemic areas, anti-malarial prophylaxis is given for
patients after splenectomy.

58. Treatment of OPSI
 Antibiotics like Cefoperazone, Ceftazidime, Amikacin
 Ventilatory support—ICU care.
 Blood transfusion.
 Immunoglobulin transfusion.
 Nutrition (TPN) and maintaining of urine output.

59. PREVENTION OF OPSI
 Life long prophylaxis using benzathine penicillin 12-24 lac
units—controversial in adults.
 Pneumococcal vaccine given 2-3 weeks prior to
splenectomy— 70% protection.
 H. influenzae-B type vaccine.
 Meningococcal vaccine is given only to high-risk groups,
as its effects are short term. So it is not routinely given

60. CONCLUSION
 Management and treatment should therefore be
administered taking into account the specific etiology and be
individualized for each patient.
 Available treatment options include non-surgical and
surgical methods.
 Surgical outcome following splenectomy is usually
satisfactory
 Continuous basic and clinical studies will advance our
understanding of the underlying mechanisms of the
development of hypersplenism, and provide better
management strategies for the treatment of patients with
hypersplenism.