Hypersplenism ;its surgical management

Hypersplenism incidence, anatomy, presentation, invistigation, management, surgical management

1. HYPERSPLENISM: SURGICAL MANAGEMENT By D r. DEV RAJ PATEL IMS BHU VARANASI

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. Anatomy Spleen is located in the Left Upper Quadrant(LUQ). Lies between the 9th -11th rib weighs about 150gm( 75-250gm ). Measures about 13 x 7 x 3 cms .

4. Attachement :- Laterally- Lienorenal Ligament Anteriorly - Gastrosplenic Ligament (Contains Short Gastric Arteries and Left Gastro Epiploic artery) Superiorly- Splenophrenic Ligament Inferiorly- Splenocolic Ligament

5. Relations Anterior- fundus of the stomach Medially- tail of the pancreas Inferiorly- splenic flexure Superiorly- diaphragm Posteriorly - upper part of the kidney

6. Arterial supply - Splenic artery, Short Gastric arteries Distributed type- the most common ( 70% ) and is distinguished by a short trunk with many long branches. Magistral type of splenic artery ( 30% ) has a long main trunk dividing near the hilum into short terminal branches. Splenic vein joins the Superior Mesenteric Vein to form the Portal Vein and accommodates the major venous drainage of the Spleen.

7. EMBRYOLOGY Spleen is the largest reticulo -endothelial organ in the body. Developed from the primitive mesoderm of dorsal mesogastrium by fifth week of gestation. The most common variation of Splenic embryology is the accessory spleen (10-29 % of the population.)

8. HISTOLOGY AND FUNCTION OF SPLEEN Red pulp(90%)- Cords and sinuses - Phagocytosis White pulp- Periarticular lymphatic sheets - Immunoglobulins .

9. Reservior for platelets,monocytes,Factor VIII etc. Haematopoiesis in fetus Repairs and destruction of RBC’s by pitting & culling. Immune function : produces IgM , properidin,tuftsin Prevention of infection - By capsulated organism ( H.influenza etc) , role in phagocytosis .

10. Hypersplenism C linical syndrome characterized by: Splenomegaly Pancytopenia or a reduction in the number of one or more types of blood cells Improvement of cytopenias Post- Splenectomy Hyperplasia of the precursor cells in the marrow or so called maturation arrest Decreased RBC/platelet survival

11. In Hypersplenism , Splenic 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.

12. Classification of Hypersplenism Hypersplenism can be classified into three categories by its etiology as follows.( Yunfu et al., 2016) Primary hypersplenism Cause is not clear. Primary splenic hyperplasia Non-tropical idiopathic splenomegaly Primary splenic granulocytopenia Primary splenic pancytopenia Splenic Anemia or thrombocytopenia

13. Secondary hypersplenism Cause is clear Infections - viral hepatitis, brucellosis, subacute or chronic diseases, infectious mononucleosis syndrome and malaria. Alcohol Portal hypertension (PH) - 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. Granulomatous inflammation - Systemic Lupus Erythematosus , Rheumatoid Arthritis, Chronic Syphilis, Chronic Tuberculosis, Felty's Syndrome, & Sarcoidosis .

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

15. 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. Bone marrow hematopoietic function is suppressed by factors such as infection or drugs, peripheral cytopenia occurs, accompanied by clinical symptoms, which is not classified as occult hypersplenism .

16.   DEGREES OF SPLENOMEGALY BASED ON CRANIO-CAUDAL LENGTH 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  diagnosis

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

18. massive  splenomegaly ( >20cm, >1000gm ) Causes Leishmaniasis Malaria Myeloproliferative  disease Portal vein obstruction/portal hypertension Schistosomiasis Niemann­Pick  disease Mucopolysaccharidosis Lymphomas Gaucher  disease Hereditary  spherocytosis

19. clinical features Abdominal pain/tenderness. Early satiety due to splenic encroachment. Symptoms of anemia due to accompanying cytopenia . Febrile illness (infectious). Pallor, dyspnea , bruising, and/or petechiae (hemolytic process).

20. History of liver disease (congestive). Weight loss, constitutional symptoms ( neoplastic ). Pancreatitis ( splenic vein thrombosis). Alcoholism, hepatitis (cirrhosis). Examination Inspection may reveal fullness in the LUQ. Palpation. Percussion - Nixon, Castell , Percussion of Traube's semilunar space. Auscultation- may reveal a venous hum or friction rub

21. Investigation Ultrasound   - The spleen is  considered  to  be  normal in size if its length is <13 cm or its  thickness  is ≤5 cm Plain film   The spleen is normal in size if it is  not seen on the abdominal plain  film.  It is considered enlarged   ( if >6cm wide or >13.6 cm long) .

22. CT Scanning- In general, the  spleen can be  considered  enlarged if its craniocaudal  length  is more than 10cm . Spleen that extends below  the lower  third pole of the  kidney is also indicative of  splenomegaly

23. Liver­Spleen  Colloid Scanning- A   splenic  length of  greater than 14 cm is  considered  enlarged on  liver­spleen   scan . Erythrocytes  are labeled with  chromium­51,  mercury­197  , rubidium­81   Bone marrow examination is useful in diagnosis of histiocytoses , lysosomal storage disorders, and some infections(e.g., disseminated histoplasmosis ). .

24. MRI/ Doppler USG- portal/ splenic vein thrombosis - cavernomas MRI scan- liver hemangiomas hemochromatosis erlenmeyer flask sign( Gaucher ) PET scan - Dx & staging of lymphomas - determine metabolic cells in spleen Splenectomy  and  Splenic  Biopsy

25. Laboratory Studies Complete blood cell count (CBC) with  differential. Liver  function testing Hepatitis  B and C testing Lactate   dehydrogenase  (LDH) Erythrocyte   sediumentation  rate (ESR) Peripheral  blood smear for RBC morphology  &  signs of  myeloproliferative  disorders  underlying bone  marrow  disorders. Prothrombin   time  (INR) and activated partial thromboplastin  time ( aPTT )

26. TREATMENT Medical treatment Partial splenic embolisation Splenectomy 1.partial splenectomy 2.Total splenectomy :- -Open splenectomy -Laparoscopic splenectomy -Robotic splenectomy

27. Medical treatment Whole blood transfusion For anemia and leucopenia Platelet transfusion Correction of coagulopathy Pre-operative nutritional optimisation Treatment of underlying cause- portal hypertension, liver transplant for liver cirrhosis, anti- malarials for tropical splenomegaly

28. Transcatheter embolisation method It includes embolisation of certain branches of splenic artery leading to infarction of part of spleen. PVA, gelatin sponge are used for embolisation Hypersplenism is relieved if >50% of splenic area is embolised Partial splenic embolisation

29. Role in reducing portal pressure in hypersplenism due to portal hypertension Contraindications - pyemia , splenic abscess, coagulopathy Complications- post- embolisation syndrome (fever, abdominal pain, vomiting), lung atelectasis, pleural effusion, splenic abscess, deranged liver or renal function, portal vein thrombosis

30. splenectomy Preoperative Planning Vaccination against pneumococcus , meningococcosis , and H. influenzae should be administered to reduce the risk of OPSI

31. Blood grouping and Cross matching Platelets should not be administered preoperatively in patient with ITP In myeloproliferative disorders - 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 had prolonged steroid treatment

32. 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 )

33. Lymphoma Primary hypersplenism Myelofibrosis Sickle-cell disease Splenic abscess Staging for hodgkins lymphoma Thalassemia Thrombotic thrombocytopenic purpura Radical gasterctomy involving removal of spleen Splenectomy not indicated Hereditary hemolytic anemia of modrerate degree Acute leukemia Agranulocytosis

34. Surgical technique Open Splenectomy - in blunt abdominal trauma, staging of Hodgkin disease an upper midline incision given. - In hematological disorder, a left oblique subcostal incision approximately two finger breadths below the costal margin given. -Preoperative angiographic embolization can be considered to reduce bleeding in cases of massive splenomegaly

35. 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.

36. After the ligamentous attachments are transected, two to six gastric vessels should be ligated in continuity and divided

37. 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. Dissection is carried out at the hilus as close to the spleen as possible to avoid injury to the Pancreas.

38. Splenic artery ligation is managed by double ligation and suture ligature, where as the splenic vein can be doubly ligated and divided. Ligation of the splenic artery and splenic vein in relation to the hilus

39. Three major areas to be inspected for bleeding:- (a) the inferior surface of the diaphragm. (b) the greater curvature of the stomach and region of the short gastric vessels. (c) the region of the hilus . An integral part of splenectomy for haematological disease is a thorough exploration to detect any accessory spleens.

40. Preoperative Splenic Artery Embolization ( Spigos   et al, 1979, )   Embolization is achieved using microcoils and/ or Gelfoam . To reduce vascularity and size of massive spleen in preparation for a laparoscopic approach. Applied in the treatment of PH and bleeding esophagogastric varices .

41. Merit Faster Increase in platelet and leukocyte counts. Reduces splenic size, improves pancytopenia , and stimulates the immune system RISKS Post- embolization syndrome: pain, fever, ileus , pleural effusion Pancreatitis Splenic abscess or rupture Peritonitis

42. Laparoscopic Splenectomy Laparoscopic techniques is mostly preferred for elective splenectomy . The complicating factors are a large spleen (>500 g), suspected perisplenitis ( infections or portal hypertension) and previous gastric surgery. ITP patients and staging laparotomy is suited ideally for laparoscopic approaches as well.

43. Position- right side down Ports- At midline and 4 cm below the spleen tip, Near the tip of the 11th rib along the posterior axillary line 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.

44. Ligation and division of the short gastric vessels then splenic artery and vein secured 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 Pfannenstien incision and removing the spleen through a suprapubic area may be more cosmetically satisfactory.

45. 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.

46. Single-incision laparoscopic surgery (SILS) One small transabdominal incision Incision - periumbilical and is used as the specimen extraction site. Theoretical benefits of less pain and better cosmetic. Technical challenging for solid organs- since all instruments are closely aligned together. Limited degrees of movement

47. 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.

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

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

50. Overwhelming PostSplenectomy Infection ( Opsi ) Incidence - 4%. Due 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. influenzae, Babesia microti infections. Common in first two years after splenectomy but life long risk present.

51. Clinical Features- Prodromal phase—fever, chills, sore throat. DIC ,Hypotension, shock. Respiratory distress, coma, death. Mortality for fully developed OPSI—50-70%. .

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

53. Prevention Pneumococcal vaccine should be given to all splenectomised patients. Polyvalent pneumo-vac is given 2-3 weeks prior to surgery and repeated once in 5 years (>2 yr of age). 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

54. Management and treatment should therefore be administered taking into account the specific etiology and be individualized for each patient. 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 . CONCLUSION

55. Thank you