Surgery In Splenic Disorders Dr. Durga Sai Swaroop Thota, Final yr post graduate, Department of General Surgery, Kamineni Institute of Medical Sciences. INTRODUCTION 1. When is surgery to spleen considered? 2. Goals of splenectomy 3. Indications of splenectomy 4. Surgical approach to patient with splenic disorders 5. Complications 6. Splenic preservation When is surgery to spleen considered? � When the spleen destroys red blood cells so rapidly � � � � that severe anemia develops When the spleen so depletes stores of white blood cells that infection is likely When the spleen so depletes stores of platelets that bleeding is likely When the spleen is so large that it causes pain or puts pressure on other organs When the spleen is so large that parts of it bleed or die Goals of splenectomy 1. Cure or palliation of particular disease causing splenomegaly � 2. 3. Palliation from hypersplenism causing refractory cytopenias that require frequent transfusion Relief from symptomatic splenomegaly � 4. Like early satiety, abdominal pain, and weight loss from mass effect Diagnosis of splenic pathology � � 5. Idiopathic (immune) thrombocytopenic purpura (ITP) and hemolytic anemias When Malignant tumours like angiosarcomas, malignant fibrous histiocytomas, and plasmacytomas is suspected diagnosis of lymphoma Control of splenic hemorrhage Indications of splenectomy BENEFITED (COMMON) MAY BE BENEFITED(UN COMMON) THROMBOCYTOPENIAS IMMUNE THROMBOCYTOPENIC PURPURA THROMBOTIC THROMBOCYTOPENIC PURPURA(TTP) ANEMIAS HEREDITORY SPHEROCYTOSIS AUTOIMMUNE HAEMOLYTIC ANEMIAS SICKEL CELL ANEMIAS THALASEMIAS HEREDITORY ELLIPTOCYTOSIS MYELOPROLIFERATIVE AND MYELODYSPLASTIC DISORDERS CML POLYCYTHEMIA VERA MYELOFIBROSIS ESSENTIAL THROMBOCYTOSIS MYELOPROLIFERATIVE DISORDERS LYMPHOPROLIFERATIVE DISORDERS HODGKINS, NON HODGKINS, CLL, HAIRY CELL LEUKAEMIA NEUTROPENIAS FELTYS SYNDROME NON HAEMATOLOGIC SPLENIC DISORDERS SPLENIC MALIGNACIES, RUPTURE,PARASITIC CYSTS VASCULAR PROBLEMS� SPLENIC VEIN THROBOSIS, and PSUEDOCYSTS SPLENIC ABSCESS SPLENIC CYSTS STORAGE DISORDES THROBOCYTOPENIAS Idiopathic thrombocytopenic purpura � Indications of splenectomy:� if a patient has two relapses on steroid therapy � refractory thrombocytopenia (thrombocytopenia recurs while the patient is receiving more than 15 mg of prednisone per day) � if the platelet count remains low � ITP has persisted for more than 6–9 months. � Up to two-thirds of patients will be cured by surgical intervention � 15% will be improved, but no benefit will be derived in the remainder THROMBOTIC THROMBOCYTOPENIC PURPURA(TTP) � Indications:� In patients who do not respond to medical therapy or � Those with chronically relapsing disease � A Retrospective review of 33 patients:- plasmapheresis dependant patients who underwent splenectomy had a postoperative relapse rate of 0.07 per patient-year � Patients who were not continuously dependent on plasmapheresis, splenectomy reduced the relapse rate from 0.74 to 1 per patient-year (Br J Heme 2005;130:768). ANEMIAS Hereditary spherocytosis � Splenectomy brings about a return of hematocrit to normal or near-normal values � Anemia is usually mild, deferral of operation until adolescence is preferable, to lessen the risk of overwhelming postsplenectomy sepsis � Indications:� Young child who develops frequent hemolytic crises. � Conjunction with an acute infection. � Manifested by a profound decrease in hematocrit necessitating transfusion. � Who require frequent blood transfusion. Acquired autoimmune haemolytic anaemia � Indications:� Chronic autoimmune hemolytic anemias secondary to antibodies of the IgG type � Corticosteroids are ineffective � Patient is developing complications from long-term steroid treatment � If corticosteroids are contraindicated � Major hemolytic crises, hemoglobinuria, and renal failure � Eighty per cent of patients respond to splenectomy. � Splenectomy is not beneficial in patients with IgM-mediated hemolysis (cold agglutinin disease) because the liver rather than spleen is the site of red cell sequestration and destruction Sickle cell disease � Splenectomy is of benefit in a few patients in whom excessive splenic sequestration of red cells aggravates the anaemia. � Indication:� Major episode of "acute splenic sequestration � A minor episode plus the presence of hypersplenism � Splenic sequestration � usually occurring before age 2 � presents as fever, cough, diarrhea, vomiting, drowsiness, and bone pain � If shock is present, the episode is considered major Thalassaemia (synonyms: Cooley Cooley’’s anaemia anaemia,, Mediterranean anaemia anaemia)) � Indications:� Who require frequent blood transfusion (when blood transfused reaches a value of 200 ml/kg body weight/year) � If hemolytic antibodies have developed as a result � Severe thrombocytopenia � Secondary hypersplenism � Symptomatic splenomegaly. � Operative morbidity from cerebral or mesenteric vascular accidents has been lessened by administration of aspirin and dipyridamole MYELOPROLIFERATIVE AND MYELODYSPLASTIC DISORDERS Chronic myelogenous leukemia (CML) � Splenectomy had no effect on survival or disease progression, but it did increase the rate of thrombosis and vascular accidents � Splenectomy is indicated only for palliation of symptomatic splenomegaly or hypersplenism that significantly limits therapy Polycythemia vera and essential thrombocytosis � Splenectomy can be required to treat symptomatic splenomegaly or pain from splenic infarcts � Splenectomy can result in severe thrombocytosis, causing thrombosis or hemorrhage, which requires perioperative antiplatelet, anticoagulation, and myelosuppressive treatment. Myelofibrosis and myeloid metaplasia � Indications for splenectomy include � symptomatic splenomegaly and � transfusion-dependent anemias � patients are at increased risk for postoperative hemorrhage and thrombotic complications after splenectomy Lymphoproliferative disorders Chronic lymphocytic leukemia (CLL) � Indications:� Symptomatic splenomegaly and � Severe hypersplenism � One retrospective study demonstrated that early splenectomy (the majority of which were for hypersplenism or cytopenias) in some patient subgroups was associated with improved survival (J Am Coll Surg 1997;185:237). Non-Hodgkin and Hodgkins Lymphomas � Indications:� For palliation of hypersplenism and � cytopenias or � For diagnosis in patients with suspected persistent or recurrent disease after systemic therapy � Splenectomy plays an important role in the diagnosis and staging of patients with isolated splenic lymphoma � Improved survival has been shown in patients undergoing splenectomy (Cancer 1993; 71:207). NEOPLASMS � Primary benign � Primary malignant � Metastatic Benign Neoplasms � Haemangioma � Littoral cell angioma � Lymphangioma � Other rare tumours � Inflammatory pseudotumor of the spleen � Hamartomas � Peliosis � Angiomyolipoma, lipoma, hemangiopericytoma, and fibroma. Haemangioma � Indications:� Multiple haemangiomas � Large and symptomatic � Which have potential to transform into angiosarcoma � Severe hypersplenism � Spontaneous rupture(25%) Littoral cell angioma � Endothelial cell neoplasm arising from the cells lining the sinus channels of the splenic red pulp � Benign neoplasm cured with splenectomy � Indications:� Association with malignant lymphoma and other visceral organ cancers � Recurrent disease � Evidence of malignant transformation into malignant littoral cell hemangioendothelioma Lymphangioma � Partial splenectomy � Indications:- Small in size � Focal symptomatic lesions � Total splenectomy:� � Indications:� � � � � Large in size Mass effect Complicated with bleeding, rupture, comsumptive coagulopathy and hypersplenism Diffuse invoLvement of spleen Generlised lyphagiomatosis with multi visceral involvement Primary Malignant Tumors � Rare � Angiosarcomas, malignant fibrous histiocytomas, and � � � � plasmacytomas Angiosarcoma is the most common nonlymphoid primary malignant neoplasm of the spleen primary treatment is splenectomy Metastasis is frequent and often involves the liver. Spontaneous rupture has been reported and is associated with a dismal outcome Metastatic Tumors � Lung, stomach, pancreas, breast, melanoma, and colon � Isolated splenic metastasis is unusual� associated with colorectal, ovarian, endometrial, and renal carcinoma � Indications:� No other sites of disease found on thorough evaluation Non-hematologic splenic disorders Splenic cysts Splenic cysts Non-Parasitic cysts True cysts(primary) Deroofing and spleenectomy parasitic cysts Pseudocysts (secondary) Hydatid cysts(majority) Aspiration and injection of hypertonic saline splenectomy Splenic abscess � Eitiology:� Infected splenic embolus or � in association with typhoid and paratyphoid fever, � osteomyelitis, � otitis media and puerperal sepsis � Pathophysiology:- abscess may rupture � left subphrenic abscess �peritonitis � Initial management:� treatment involves that of the underlying cause and � drainage of the splenic abscess by percutaneous means under radiological guidance � Treatment of choice � Splenectomy � Difficult in patients with extensive parasplenic inflammation and adhesions� operative splenectomy may be performed after preliminary aspiration of the abscess 30 Gaucher Gaucher’’s disease � Associated with Enormous splenic enlargement � Splenectomy is indicated only for severe symptoms related to the splenomegaly � Partial splenectomy may be considered as a temporizing procedure in young children � Partial splenectomy has been performed in an attempt to lessen the risk of postsplenectomy infection � After partial splenectomy, symptomatic splenic enlargement and worsening hypersplenism recur after 3 to 8 years and necessitate later total splenectomy. OTHER INDICATIONS FOR SPLENECTOMY � Occult Rupture of the Spleen � Congestive splenomegaly � Wandering spleen � splenunculi Occult Rupture of the Spleen � True rupture:� True "spontaneous rupture" of a normal-sized spleen is rare. � Mechanisms:� � � � repeated vomiting and retching� producing increased traction on the lienogastric ligament abnormal splenic mobility and torsion degenerative changes in intrasplenic arteries acute or chronic splenic congestion � Splenic rupture secondary to underlying splenic pathology � Much more commoner than true rupture � Eitiology:� � � � � � Infective� eg., infectious mononucleosis Neoplasms(primary and metastatic) Infiltrative conditions:- sarcoidosis, amylodosis, gauchers disease Haemolytic anemias Some external factors� eosion associated with gastric ulcer and pancreatitis vascular, thrombotic and haemostatic disorders Congestive Splenomegaly � Indications:� Left-sided portal hypertension � Hypersplenism resulting from splenic vein thrombosis � Persistent severe thrombocytopenia after a portalsystemic shunt � Splenectomy alone for the management of thrombocytopenia associated with hepatic cirrhosis and bleeding esophageal varices is inappropriate because platelet counts usually rise to safe levels after one of the portal-systemic shunting procedures Wandering spleen � Due to increased splenic mobility on an elongated splenic � � � � � pedicle Results from congenital weakness or atresia of the lienorenal ligament as a result of failure of fusion of the dorsal mesogastrium, from which the spleen develops embryologically Present as an asymptomatic abdominal mass or, more commonly, as an abdominal mass with associated intermittent abdominal pain A more serious situation is presentation as an acute abdomen resulting from persistent splenic torsion and infarction Nonemergency situation, splenopexy deserves more serious consideration in the young child, who is more susceptible to postsplenectomy sepsis. Indiations:� Acute splenic torsion � Infarction Splenunculi � Supernumerary or accessory spleens or splenules � Small nodules of spleen that are detached from the rest of the organ � They are benign and asymptomatic present � Near the splenic hilum (most common) � Gastrosplenic ligament � Splenorenal ligament � Pancreatic tail � Greater omentum � Small bowel mesentery � Stomach or bowel wall � Scrotum � If accessory splenic tissue is found, reexploration should be considered, although long-term response to removal of an accessory spleen is uncommon in cases of ITP (N Engl J Med 2002;346:995). Surgical approach to patient with splenic disorders � Preoperative considerations in splenectomy � Open splenectomy � Laproscopic spleenectomy � Staging laprotomy Preoperative Considerations in Splenectomy � Vaccinations � Polyvalent pneumococcal vaccine (Pneumovax) � Meningococcal vaccine � Haemophilus influenzae type B � Considerations for Transfusion � Patients with hematologic disease � Patients with severe thrombocytopenia � Preoperative imaging � Other consideration � Counseling of patients for conversion from laproscopic to open splenectomy or a hand assisted approach Open splenectomy Step 1:-selection of incision Step 2:-division of short gastric vessels Step3:-division of splenic attachments Step 4:- control of splenic hilum Step 1:-Selection of incision Step 2:-Division of short gastric vessels Step3:-Division of splenic attachments Step 4:- Control of splenic hilum Laproscopic splenectomy Step 1 • Patient positioning and theatre set-up Step 2 • Port placement Step 3 • Exposure •The vascular attachments are divided next using a 30- or 45-mm Step 4 endoscopic linear cutting stapler Step 5 • The spleen is next placed in a large specimen retrieval bag Step 6 • Morcellation of the spleen Patient positioning and theatre setup Port placement Exposure The vascular attachments are divided next using a 30- or 45-mm endoscopic linear cutting stapler The spleen is next placed in a large specimen retrieval bag Morcellation of the spleen Hand-assisted laparoscopic splenectomy (HALS) Contraindications to Laparoscopic Splenectomy � Absolute contraindications:� Massive splenomegaly (>30 cm length) � Portal hypertension � Splenic trauma, unstable patient � Relative contraindication:� Moderate splenomegaly (>20–25 cm) � Severe uncorrectable cytopenia � Splenic vein thrombosis � Splenic trauma, stable patient � Bulky hilar adenopathy � Morbid obesity OPERATIVE STAGING OF PATIENTS WITH LYMPHOMA � Hodgkin's disease � Indications:� � to define more accurately the extent of disease to tailor subsequent radiotherapy to involved areas � Diagnosis is made usually by excision of an enlarged cervical, axillary, or inguinal lymph node � Once a histologic diagnosis has been made, a diagnostic evaluation is carried out to determine the clinical stage of the disease � Based on clinical stage, which patients should be considered for possible operative staging � Procedure:� Long vertical abdominal incision, � Splenectomy, � wedge biopsy of the liver, � Systematic biopsy of iliac and para-aortic lymph nodes, and � wedge biopsy of left iliac crest bone and marrow Complications of splenectomy complications intraoperative Early post operative Late post operative complications Intra-operative Complications pancreatic injury vascular stomach bowel colon Diaphragmatic injury Intra-operative Complications � pancreatic injury:� 0% to 6% � A closed suction drain should be placed adjacent to the pancreas, and a drain amylase obtained prior to removal after the patient is eating a regular diet � Vascular injury:� � � � Haemorrhage(MC) 2-3%� Open splenectomy 5%� laproscopic splenectomy Bleeding during laparoscopic splenectomy may necessitate conversion to a hand-assisted or open procedure � Bowel injury:� Because of the close proximity of the splenic flexure to the lower pole of the spleen, it is possible to injure the colon during mobilization � Stomach:� Gastric injuries can occur by direct trauma or can result from thermal injury during division of the short gastric vessels. Use of energy devices too close to the greater curvature of the stomach can result in a delayed gastric necrosis and perforation � Diaphragmatic injury:� during the mobilization of the superior pole especially with perisplenitis � careful dissection of the splenophrenic ligament can minimize its occurrence Early Postoperative Complications Pulmonary (10%) Subphrenic abscess(2-3%) Wound problems(4-5%) Thrombocytosis and thrombotic complications(8-10%) Ileus Early Postoperative Complications � Pulmonary complications:� 10% � Atelectasis to pneumonia and pleural effusion � Subphrenic abscess:� 2% to 3% � uncommon after laparoscopic splenectomy (0.7%) � Treatment:-percutaneous drainage and the intravenous antibiotics � Wound problems:� 4% to 5% � hematomas, seromas, and wound infections � Thrombocytosis and thrombotic complications:� Due to occurrence of thrombocytosis, alterations in platelet function, and a low-flow stasis phenomenon in the ligated splenic vein. Late Postoperative Complications � Overwhelming postsplenectomy infection (OPSI):� Nonspecific flulike symptoms rapidly progressing to fulminant sepsis, consumptive coagulopathy, bacteremia, and ultimately death within 12 to 48 hours � Organisms:- Encapsulated bacteria especially � � � Streptococcus pneumoniae, H. influenzae type B, and Neisseria meningitidis � Treatment:- early supportive care and high-dose third- generation cephalosporins � Prevention:- immunization Splenosis � Presence of disseminated intra-abdominal splenic tissue, which usually occurs after splenic rupture or marcellation of spleen during laproscopic splenectomy � Prevention:� care should be taken during splenic morcellation to avoid bag rupture and spillage of splenic tissue. Splenic preservation � Partial splenectomy:� Indications:� � As a palliation for severe splenomegaly causing mass effect Young patients � Splenic replantation :� Placing small fragments of splenic tissue with in the omentum � This “omental omelet” of multiple splenic fragments measuring no more than 1 cm in size is constructed � The splenic omelets uniformly survive and take up isotope, but the histology of these surviving segments shows that the perifollicular lymphoid tissue is absent. � The patients with splenic replantation do not respond in the same way as patients with splenic salvage in the normal position with maintained splenic blood flow.
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