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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 multi
2、ple clefts.,Embryoiogy, 12 to 15 cm in length, 4 to 8 cm in width and 3 to 4 cm in thickness, Average weight is about 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
3、) 9 superior mesenteric artery 13 superior mesenteric artery, The spleen receives its arterial supply from the splenic artery, which originates in the celiac axis. After its origin, the splenic artery courses along the superior edge of the pancreas, with multiple branches into the pancreatic parench
4、yma. The artery then g ives off several branches into the spleen, the first being the superior polar artery. There are other arterial vessels to the spleen from the left gastroepiploic artery and the short gastric artery., The splenic veins follow the arterial distribution closely, and the main sple
5、nic vein emerges from the spleen following a course to join the superior mesenteric vein, 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 : filtrat
6、ion immunological reservoir hematopoietic, 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, immunoglobuli
7、n and antibody synthesis , affects the capability 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 ot
8、her species.,Pathological Conditions of the Spleen, Splenic 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 f
9、requent indications for splenectomy. Pathologicalcauses of splenic rupture include infiltration of the spleen by reactive lymphoid cells or by neoplastic cells. Most cases attributed to spontaneous rupture of the spleen are actually due to an undiagnosed pathological process.,Conditions associated w
10、ith defective or absent splenic function are grouped together as 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 splene
11、ctomy ,these can be divided into those conditions in which the spleen 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).,Pathologic
12、al Conditions of the Spleen,TABLE 1 Disorders Associated With Hypersplenism 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. A
13、cquired disorders of erythrocytes 1.Autoimmune hemolytic anemia 2.Parasitie 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 d
14、iseases, parasitie diseases(e.g.,kala-azar),Langerhans cells histiocytosis, malignant histiocytosis B. Infiltrative disorders:leukemias,lymphomas,plasma cell dyscrasias, myeloid metastatic carcinnoma C. Vascular abnormalities D. Splenic cysts E. Hamartomas 3. Miscellaneous conditions A. Hyperthyroid
15、ism B. Hypogammaglobulinemia C. Progressive multifocal leukoencephalopathy Source: Reiman (1997).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 primar
16、y neoplasm of the spleen and can present with splenomegaly, Lesions can be solitary or multiple, and 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 sple
17、nectomy,Benign Lesions of the Spleen,Gross images: It weighed 425 grams and measured 15 x 9 x 7 cm. A splenectomy was performed.,Micro images:representative section of tumor, Lymphangioma, These lesions are less common than hemangiomas, and are usually subcapsular, appearing as soft, compressible, m
18、ulticystic lesions on the splenic surface., When large, they present with splenomegaly as an indication., There are case reports of patients presenting with hypersplenic syndromes, consumptive coagulopathy, and even portal hypertensionwith these lesions.,Benign Lesions of the Spleen, Peliosis, These
19、 rare lesions bear a superficial resemblance to vascular neoplasms of the spleen., They consist of blood-filled cysts distributed in patches or diffusely, and can result in splenomegaly., Intraperitioneal hemorrhage can result from the rupture of these lesions.,Benign Lesions of the Spleen, Hemangio
20、endothelioma, These rare lesions is thought to be intermediate between hemangioma and angiosarcoma., They usually contain cellular atypia, differentiating them from hemangiomas., These lesions may present with splenomegaly or rupture, and should suggest the possibility of a malignabt vascular neopla
21、sm., Hamartomas, These are focal developmental abnormalities within the normal spleen, rather than being true neoplasms., They consist 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
22、affect the spleen.,. Lymphoproliferative disorders a. Non-Hodgkins lymphoma b. Hodgkins disease c. Chronic lymphocytic leukemia d. Hairy cell leukemia e. plasmacytoma f. Waldenstrms macroglobulinemia . Myeloproliferative disorders a. Chronic myelogenous leukemia b. polycythemia vera c. Myelofibrosis
23、 (agnogenic myeloid metaplasia) d. Essential thrombocythemia .Vascular tumors a. Hemangiosarcoma b. Lymphangiosarcoma . Metastatic tumors: breast,lung,melanoma,etc .Other lesions a. Sarcoma: fibrosarcoma, leiomyocarcoma, Kaposis sarcoma Source: Adapted from Giles and Lim(1997). 7,Maligant Lesions of
24、 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 fatal, or may behave as one of the most indolent and well-tolerated malignancies afflicting man., The role of the surgeon in Non-Hodg
25、kins lymphoma is usually limited to the biopsy of a single peripheral lymph node to establish a tissue diagnosis., Splenectomy for Non-hodgkins lymphomas Symptomatic splenomegaly NHL confined to the spleen or with prominent splenic involvement, Non-Hodgkins lymphoma are systemic disease at the time
26、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 usually originates in a single nodal group and predictably proceeds in a stepwise progression from one contiguous node group to
27、 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., Historically, staging laparotomy with splenectomy were essential guides to treatment Improvement in imaging and chemotherapy h
28、ave 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 B-cell lineage and is characterized by an accumulation of incompetent lymphocytes., CLL is incurable, but it
29、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 lymphoproliferative disorder that affects middle-aged men, It present with pancytopenia and splenomegaly
30、 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 symptomatic splenomegaly., Myeloproliferative Disorders,This disease include chronic myelogenous leu
31、kemia (CML), myelofibrosis, polycythemia vera, and essential thrombocythemia., The role of splenectomy remains controversial in this disease .,Micro images: CML,myelofibrosis,polycythemia vera,essential thrombocythemia, Hemangiosarcoma, This is a rare primary tumor of the spleen in humans., Treatmen
32、t is surgical, and no effective adjuvant therapies have been identified.,Indications for Splenectomy, There are two surgical procedures performed in reference to the spleen: partial splenectomy splenectomy. A splenectomy can be conducted by conventional open technique (OS) or by laparoscopic means (
33、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, Sickle Cell Disease, Idiopathic Autoimmune Hemolytic Anemia, Idiopathic Thro
34、mbocytopenia 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 splenectomies are performed for trauma., Splenic injury is often suspected in the injured patients on the ba
35、sis of mechanism of injury and the presence of associated injuries such as left lower rib fractures. Some patients undergo CT scan of the abdomen and reveal injuries such as those seen in Figure 1.,Figure 1., Once splenic injury is identified, there are three options: nonoperative management, spleni
36、c 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 Hematoma Subcapsular, nonexpanding,10% surface Lacera
37、tion 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 subcapsular hematoma with active bleeding, int
38、raparenchymal 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(25%) Hematoma Completely shattered spleen V
39、ascular Hilar vascular injury with devascularized spleen Source: 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 patient, mechanism of injury, age, inte
40、rval from time of injury, associated injuries, and preexisting medical conditions., 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 nonoperative management
41、 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 determination, abs
42、ence 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., Patients are conside
43、red 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 (capsul
44、ar avulsions and superficial parenchymal fractures) are managed by topical hemostatic agents.The argon beam coagulator may be helpful in the management of these lesions. Mattress sutures over Teflon pledgets mays be useful to close deeper wounds., Grade III and IV splenic injury requires complete mo
45、bilization of spleen to expose the hilum. Division of the short gastric vessels is required. Partial splenectomy may be indicated.,Splenectomy for Hematological Disorders,TABLE 4 Indications for Elective Splenectomy. ITP(idiopathic thrombocytopenia purpura) Hereditary spherocytosis Autoimmune hemoly
46、tic anemia Staging for Hodgkins disease Lymphoma Thrombocytopenic thrombotic purpura AIDS-related thrombocytopenia Leukemia Splenic abscess Gauchers disease Myelofibrosis Splenic infarct, Hereditary Spherocytosis, Autosomal dominant fashion Most common hemolytic anemia for which splenectomy is advis
47、ed Defective erythrocyte membrane causes trapping and disintegration within the spleen Presents with anemia, reticulocytosis, jaundice, and splenomegaly Diagnosis made by peripheral blood smear Splenectomy is the only therapeutic modality (wait until age 4 to 6),Splenectomy for Hematological Disorde
48、rs, Thalassemia, Autosomal dominant 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, painful splenic infarction, and increased transfusion requirements Greater risk of post-splenectomy sepsis,Splenectomy for Hematological Dis