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1、,Spleen Disease,The splenic primordium becomes evident during the fifth week of gestation as an outgrowth of the dorsal mesogastrium,which migrates to the left upper quadrant.The gross appearance of the spleen is the result of its development from multiple anlage,resulting in an organ with multiple
2、clefts.,Embryoiogy,12 to 15 cm inlength,4 to 8 cm inwidth and 3 to 4 cmin thickness,Average weight isabout 150 to 250 g,lies in the shelter of the 9th to 11th rib at the left side of the abdominal cavity,Anatomy,A pancreas B spleen C duodenum 5 Hepatic portal vein 8 celiac artery(trunk)9 superior me
3、senteric artery 13 superior mesenteric artery,The spleen receives its arterial supply from thesplenic artery,which originates in the celiac axis.After its origin,the splenic artery coursesalong the superior edge of the pancreas,with multiple branches into the pancreaticparenchyma.The artery then g i
4、ves off several branches into the spleen,the first being the superior polar artery.There are other arterial vessels to thespleen from the left gastroepiploic artery and the short gastricartery.,The splenic veins follow the arterial distribution closely,and the main splenic vein emerges fromthe splee
5、n following a course to join the superior mesentericvein,forming the portal vein.,Functions of the Spleen,Circulation through the spleen is about 150 to 200 ml/min,or about 5%of the cardiac output.The spleen has traditionally been ascribed four functions:filtration immunological reservoir hematopoie
6、tic,Filtration abnormal or senescent red blood cells,particulate antigens such as microorganisms or antigen-antibody complexes.,Immunological trapping of antigens,homing of lymphocytes,antibodyand lymphokine production,microphage activation,immunoglobulin and antibody synthesis,affects the capabilit
7、y of cellular populations in other lymphoid organs,Reservoir,Hematopoietic,Spleen harbors about one-third of the total platelet mass and a large number of granulocytes.,The hematopoietic functions are minimal in humans and much more prominent in other species.,Pathological Conditions of the Spleen,S
8、plenic Rupture,Benign Lesions of the Spleen,Maligant Lesions of Spleen,Splenic Rupture,The spleen can rupture from three underlying causes:trauma,spontaneous rupture,and pathological rupture.,Traumatic rupture of the spleen remains the most frequent indications for splenectomy.Pathologicalcauses of
9、splenic rupture includeinfiltration of the spleen by reactive lymphoidcells or by neoplastic cells.Most cases attributed to spontaneous ruptureof the spleen are actually due to an undiagnosed pathological process.,Conditions associated with defective or absent splenic function are grouped together a
10、s being conditions of hyposplenism.Those conditions are characterized by the presence of Howell-Jolly bodies in the peripheral circulation.Conditions associated with hypersplenism remain the most frequent indication for elective splenectomy,these can be divided into those conditions in which the spl
11、een is normal but increased destruction of abnormal blood elements causes hypersplenism,and those in which there is a primary disorder of the spleen that results in increased destruction of abnormal blood cells.(Table 1).,Pathological Conditions of the Spleen,TABLE 1 Disorders Associated With Hypers
12、plenism 1.Disorders associated with sequestration of abnormal blood cells in an intrinsically normal spleen.A.Congenital disorders of erythrocytes 1.Hereditary spherocytosis 2.Hereditary elliptocytosis 3.Hemoglobinopathies B.Acquired disorders of erythrocytes 1.Autoimmune hemolytic anemia 2.Parasiti
13、e disease(e.g.,malaria,babesiosis)C.Autoimmune thrombocytopenia D.Autoimmune neutropenia 2.Disorders of spleen resulting in sequestration of normal blood cells A.Disorders of cordal macrophages:Bantis syndrome,storage diseases,parasitie diseases(e.g.,kala-azar),Langerhans cells histiocytosis,maligna
14、nt histiocytosis B.Infiltrative disorders:leukemias,lymphomas,plasma cell dyscrasias,myeloid metastatic carcinnoma C.Vascular abnormalities D.Splenic cysts E.Hamartomas3.Miscellaneous conditions A.Hyperthyroidism B.Hypogammaglobulinemia C.Progressive multifocal leukoencephalopathySource:Reiman(1997)
15、.3,Benign Lesions of the Spleen,Hemangioma Lymphangioma Peliosis Hemangioendothelioma Hamartomas Other Benign Lesions Nonparasitic Cysts Parasitic Cysts,Hemangioma,This is the most common benign primary neoplasm of the spleen and can present with splenomegaly,Lesions can be solitary or multiple,and
16、are usually blue-red,well-circumscribed nodules.,Microscopically,they usually appear as endothelium-lined spaces,and are known as cavernous hemangiomas.,Treatment of these lesions is usually splenectomy,Benign Lesions of the Spleen,Gross images:It weighed 425 grams and measured 15 x 9 x 7 cm.A splen
17、ectomy was performed.,Micro images:representative section of tumor,Lymphangioma,These lesions are less common than hemangiomas,and are usually subcapsular,appearing as soft,compressible,multicystic lesions on thesplenic surface.,When large,they present with splenomegaly as an indication.,There are c
18、ase reports of patients presenting with hypersplenic syndromes,consumptive coagulopathy,and even portal hypertensionwith these lesions.,Benign Lesions of the Spleen,Peliosis,These rare lesions bear a superficial resemblance to vascular neoplasms of the spleen.,They consist of blood-filled cysts dist
19、ributed in patches or diffusely,and can result in splenomegaly.,Intraperitioneal hemorrhage can result from the rupture of these lesions.,Benign Lesions of the Spleen,Hemangioendothelioma,These rare lesions is thought to be intermediate between hemangioma and angiosarcoma.,They usually contain cellu
20、lar atypia,differentiatingthem from hemangiomas.,These lesions may present with splenomegaly or rupture,and should suggest the possibility of a malignabt vascular neoplasm.,Hamartomas,These are focal developmental abnormalities within the normal spleen,rather than being true neoplasms.,They consist
21、of normal cellular elements in disarray,and are usually found incidentally,Benign Lesions of the Spleen,Maligant Lesions of Spleen,There are many malignancies that affect the spleen.,.Lymphoproliferative disorders a.Non-Hodgkins lymphoma b.Hodgkins disease c.Chronic lymphocytic leukemia d.Hairy cell
22、 leukemia e.plasmacytoma f.Waldenstrms macroglobulinemia.Myeloproliferative disorders a.Chronic myelogenous leukemia b.polycythemia vera c.Myelofibrosis(agnogenic myeloid metaplasia)d.Essential thrombocythemia.Vascular tumors a.Hemangiosarcoma b.Lymphangiosarcoma.Metastatic tumors:breast,lung,melano
23、ma,etc.Other lesions a.Sarcoma:fibrosarcoma,leiomyocarcoma,Kaposis sarcomaSource:Adapted from Giles and Lim(1997).7,Maligant Lesions of Spleen,Non-Hodgkins Lymphoma,Non-Hodgkins lymphoma is a diverse group of disease with a wide range of biological behaviors.,They may be very aggressive and rapidly
24、fatal,or may behave as one of the most indolent and well-tolerated malignancies afflicting man.,The role of the surgeon in Non-Hodgkins lymphoma is usually limited to the biopsy of a single peripheral lymph node to establish a tissue diagnosis.,Splenectomy for Non-hodgkins lymphomas Symptomatic sple
25、nomegaly NHL confined to the spleen or with prominent splenic involvement,Non-Hodgkins lymphoma are systemic disease at the time of diagnosis,and require the use of systemic therapy(e.g.,chemotherapy)rather than regional therapy(e.g.,radiation)for treatment.,Hodgkins disease,Hodgkins Lymphoma usuall
26、y originatesin a single nodal group and predictably proceeds in a stepwise progression fromone contiguous node group to the next.,Below the diaphragm,the spleen becomes involved by Hodgkins Lymphoma before proceeding along the periaortic lymph nodes to the iliac and inguinal nodal basins.,Historical
27、ly,staging laparotomy with splenectomy were essential guides to treatment Improvement in imaging and chemotherapy have minimized the role of the surgeon,Chronic lymphocytic Leukemia(CLL),This is the most common of the chronic leukemias,usually found in patients over 60 years of age.,It is usually of
28、 B-cell lineage and is characterized by an accumulation of incompetent lymphocytes.,CLL is incurable,but it is managed with a variety of chemotherapeutic agents and sometimes splenectomy.,Splenectomy is indicated in those patients who progress despite chemotherapy.,Hairy Cell Leukemia,This is a rare
29、 lymphoproliferative disorder that affects middle-aged men,It present with pancytopenia and splenomegaly and is characterized by the identification of“hairy cell”in the peripheral circulation.,Splenomegaly is thus reserved for patients who fail to respond to systemic chemotherapy or who have massive
30、 symptomatic splenomegaly.,Myeloproliferative Disorders,This disease include chronic myelogenous leukemia(CML),myelofibrosis,polycythemia vera,and essential thrombocythemia.,The role of splenectomy remainscontroversial in this disease.,Micro images:CML,myelofibrosis,polycythemia vera,essential throm
31、bocythemia,Hemangiosarcoma,This is a rare primary tumor of the spleen in humans.,Treatment issurgical,and no effective adjuvant therapies have beenidentified.,Indications for Splenectomy,There are two surgical procedures performed in reference to the spleen:partial splenectomy splenectomy.A splenect
32、omy can be conducted by conventional open technique(OS)or by laparoscopic means(LS).,Indications for Splenectomy,Splenectomy for Trauma,Management of Splenic Injuries In Children,Management of Splenic Injuries In Adults,Splenectomy for Hematological Disorders,Hereditary Spherocytosis,Thalassemia,Sic
33、kle Cell Disease,Idiopathic Autoimmune Hemolytic Anemia,Idiopathic Thrombocytopenia Purpuma(ITP),Thrombotic Thrombocytopenia Purpuma(TTP),Splenectomy for Trauma,The spleen is the organ most commonly injured in blunt abdominal trauma,and thus the majority of splenectomiesare performed for trauma.,Spl
34、enic injury is often suspected in the injured patients on the basis of mechanism of injury and thepresence of associated injuries such as left lower rib fractures.Some patients undergo CT scan of the abdomen and reveal injuriessuch as those seen in Figure 1.,Figure 1.,Once splenic injury is identifi
35、ed,there are three options:nonoperative management,splenic salvage(repair of the injury or partial splenectomy),or splenectomy.In these critically injured patients with multiple intraabdominal injuries,splenectomy is the only procedure to be considered.,TABLE 3 Grading of Traumatic Splenic Injuries
36、Hematoma Subcapsular,nonexpanding,10%surface Laceration Capsular tear,nonexpanding,1cm parenchymal depth Hematoma Subcapsular,nonexpanding,10%-50%surface area Laceration Capsular tear,active bleeding,1-3cm,not involving a trabecular vessel Hematoma Subcapsular,50%surface area or expanding,ruptured s
37、ubcapsular hematoma with active bleeding,intraparenchymal hematoma,2cm or expanding Laceration 3cm parenchymal depth or involving trabecular vessels Hematoma Ruptured intraparenchymal hematoma with active bleeding Laceration Laceration involving segmental or hilar vessel with major devascularization
38、(25%)Hematoma Completely shattered spleen Vascular Hilar vascular injury with devascularized spleenSource:Adapted from Lipshy et al.(1996).20,Splenic injuries are graded by severity(Table 3).,The splenic management of a patient with a splenic injury is guided by the overall stability of the injured
39、patient,mechanism of injury,age,interval from timeof injury,associated injuries,and preexisting medicalconditions.,In a recent series of adults with splenic injuries,18%were treated nonoperatively,22%underwent splenorrhaphy,and 60%underwent splenectomy.,Management of Splenic Injuries In Children,The
40、 nonoperative management of splenic injury has been applied primarily in children.,Guidelines for the nonoperative management of splenic injuries in children include documentation of splenic injury by imaging studies,admission to the ICU with close observation,hemodynamic stability,serial hematocrit
41、 determination,absence of other intraabdominal injuries,transfusion requirements less than 50%of the blood volume,and absence of neurottrauma,Management of Splenic Injuries In Adults,The nonoperative management of splenic injuries in adults is less established as a standard than in children.,Patient
42、s are considered candidates for nonoperative management by meeting criteria such as:hemodynamic stability,minimum transfusion requirement,absence of associated intraabdominal injury,stable defect on repeat imaging.an ability to perform reliable serial abdominal examinations.,Grade I to II injuries(c
43、apsular avulsions and superficial parenchymalfractures)are managed by topical hemostatic agents.The argon beamcoagulator may be helpful in the management of these lesions.Mattresssutures over Teflon pledgets mays beuseful to close deeper wounds.,Grade III and IV splenic injury requires complete mobi
44、lization of spleen to expose the hilum.Divisionof the short gastric vessels is required.Partial splenectomymay be indicated.,Splenectomy for Hematological Disorders,TABLE 4 Indications for Elective Splenectomy.ITP(idiopathic thrombocytopenia purpura)Hereditary spherocytosisAutoimmune hemolytic anemi
45、aStaging for Hodgkins diseaseLymphomaThrombocytopenic thrombotic purpuraAIDS-related thrombocytopeniaLeukemiaSplenic abscessGauchers diseaseMyelofibrosisSplenic infarct,Hereditary Spherocytosis,Autosomal dominant fashion Most common hemolytic anemia for whichsplenectomy is advised Defective erythroc
46、yte membrane causestrapping and disintegration within thespleen Presents with anemia,reticulocytosis,jaundice,and splenomegaly Diagnosis made by peripheral blood smear Splenectomy is the only therapeuticmodality(wait until age 4 to 6),Splenectomy for Hematological Disorders,Thalassemia,Autosomal dom
47、inant transmission Defect in the synthesis rate of hemoglobin Thalassemia major(homozygous)presents with pallor,retarded body growth,enlarged head,and intractable ulcers Diagnosis made by nucleated RBCs(target cells)in smear Splenectomy reserved for patients with markedly symptomatic splenomegaly,pa
48、inful splenic infarction,and increased transfusion requirements Greater risk of post-splenectomy sepsis,Splenectomy for Hematological Disorders,Sickle Cell Disease,Due to homozygous inheritance of HbS Single amino-acid substitution of valine for glutamic acid Spleen commonly enlarged during the firs
49、t decade of life but then undergoes progressive atrophy due to repeated attacks of vaso-occlusion and infarction In general,splenectomy should be avoided in patients with SCD Already immunocompromized,Splenectomy for Hematological Disorders,Idiopathic Autoimmune Hemolytic Anemia,This disease is char
50、acterized by hemolysis of normal erythrocytes after exposure to circulating antibodies.The spleen serves as a source of antibody in this process.The initial therapy is steroid administration,Reserving splenectomy for patients in whom steroids are ineffective or contraindicated.,Splenectomy for Hemat
