小儿脾疾病临床诊疗指南.doc

小儿脾疾病临床诊疗指南Guidelines for Surgical Treatment of Pediatrie Spleen DiseasesIntroductionThe most frequent conditions requiring spleen surgery are hematological and immunological disorders, and trauma> Splenectomy represents the most common splenic procedure, but has potential disadvantages such as postsplenectomy sepsis in 1 in 100 patients. The first report describing laparoscopic splenectomy in children was published in 1993 by Tulman and Holcomb. 1 In general, the benefits of the laparoscopic technique are decreased postoperative pain, a shorter duration of postoperative ileus, a lower postoperative morbidity, and a shorter hospitalization・ 1,2, 3 These bonefits are also described with laparoscopic therapy of splenic cysts. DofinitionThe normal adult spleen is about 12 cm long and 7 cm wide and weighs 100-200 g. In children, spleens four times larger than normal for age are considered massive. Splenomegaly may be caused by disorders of immunoregulation or splenic blood flow, diseases with abnormal erythrocytes, and infiltrative or infectious diseases.The term hypersplenism (primary or secondary) applies to any clinical situation in which the spleen removes excessive quantities of erythrocytes, granulocytes, or platelets from circulation. Criteria for the diagnosis of hypersplenism inelude splenomegaly, splenic destruction of one or more cel 1 lines, normal or hyperplastic cellularity of the bone marrow with normal representation of the cell line deficient and, variably, reticulocytosis, circulating immature platelet forms, increased band forms of neutrophils.Splenic cysts are uncommon in children. The diagnosis may be based on gross findings and the presence or abscence of an epithelial lining.5 Hydatid disease of the spleen is often associated with involvement of other organs, especially the liver・ManagementThe importance of preserving the spleen in order to maintain the host， s immunologic response has been widely recognized. Therefore, nonoperative therapy is the first-line management for almost all splenic conditions. Splenectomy is reserved for patients with hematologic and immunological disease in whom medical therapy has failed・The most common indications for splenectomy in children are hereditary spherocytosis (HS) and idiopathic thrombocytopeniei purpura (ITP)2・ Other indications for splenectomy are traumatic hemmorhage, sickle cell disease, thalassemia, hemoglobin H, Coomb s anemia, cancer staging in Hodgkin" s disease, leukemia, Gauchers disease, and portal hypertension. The frequency of splencctomy for portai hypertension has considerably decreased in recent years.SURGICAL THERAPYNumerous authors have reported on the benefits of laparoscopic versus open splenectomy 1,2,7 and the laparoscopic technique is becoming the preferred approach. There is no consensus on contraindications for laparoscopic splcnectomy with respect to the degree ofsplenomegaly. Some authors find splonic artery embolization a useful procedure in obese patients. 2OPEN SPLENECTOMYOpen splenectomy is performed in most cases through a high left subcostal or midline incision. In cases of traumatic rupture, the spleen is grasped medially and the splenotenal 1igament is divided. After dissection of diaphragmatic peritoneal attachments, the spleen is delivered in the direction of the incision. The short gastric vessels are ligated and divided. The hilum is exposed after dividing attachments to the colon. The tail of the pancreas should be gently separated from the spleen. The splenic vessels are individually ligated and divided. Accessory spleen(s) should be removed, if present.In pationts with traumatic injury, splenectomy should be avoided if possible. Suturing of the splenic laceration or partial splenectomy is preferred. In cases of partial splenectomy, ligation of the lower splenic segmental artery and vein may be indicated・ The cut edge of the spleen is sutured with interrupted absorbable sutures, which may be placed over pledgets. The cut surface may bo covered with omontum or a synthotic mesh.LAPAROSCOPIC SPLENECTOMYThe number of the ports used depends on the surgeon" s preference and range from 3 to 5 1,2,3,1. Ligation of the short gastric vessels is easily performed while the lateral attachments are st订1 intact. A retractor or other instrument may be used to push the spleen medially to enable cutting the lateral peritoneal attachments and the splcnocolic ligaments with cauterizing scissors or the harmonic scalpel. An instrument is passed posterior to the spleen to retract the hilum anteriorly, and the connective tissue is dissected until the splenic vessels are freed adequately for application of clips, suture, or endovascular staples・ The pancreas, the mesentory, and omentumthe spleen into the endoscopic bag, fluids, tying an umbilical tapeshould be inspected for accessory splenic tissue. The spleen is placed into a sac which is introduced via the umbi1ical or anterior axillary。