小兒脾疾病臨床診療指南

1、小兒脾疾病臨床診療指南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 p

2、ostsplenectomy 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 post

3、operative 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

4、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

5、 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, ret

6、iculocytosis, 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 oth

7、er organs, especially the livermanagementthe 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

8、 hematologic and immunological disease in whom medical therapy has failedthe 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, thalasse

9、mia, 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 la

10、paroscopic 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 pat

11、ients. 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 delive

12、red 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) sh

13、ould 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

14、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

15、short gastric vessels is easily performed while the lateral attachments are st訂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 in

16、strument 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, flui

17、ds, 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 port.with irrigationportion of the spleen, or placing the spleen in the splenic bed, may help to retrieve the organ13. the enlarged

18、siightly to enable removal of the spleen.in cases where it is difficult to place filling the sac partially loosely around the middle pelvis and the sac in the umbi1ical inci si on can beonce inside the bag, the spleen can be divided into pieces by digital fracture with ring forceps, or by using an a

19、utomatic tissue morcellator.surgical therapy of splenic cystsnonparasitic splenic cysts that are symptomatic or are over 5 cm in diameter should be removed either by partial splenectomy or near-toteil cystectomy with decapsulation.5 these procedures can be performed laparoscopically. 4 three or 4 ca

20、nullas are used. for decapsulation, electrocautery scissors4 or ultrasonic shears may be used it remains tinclear whether fulgurization of the rcmaining cyst wall and omentopexy prevent recurrences.the optimal treatment of hydatic splenic cysts is still debatable. most authors recommend splenectomy

21、following medical treatment 8, but successful conservative management by percutanous puncture under sonographic guidance and instillation of alcoholic or hypobaric solutions is reported9 partial cystectomy has been recommended for selected cases using laparoscopic techniqucs 10associated proceduresc

22、hildren with hemolytic disorders may suffer from concomitant gallstone disease. laparoscopic cholecystectomy has been advocated as the procedure of choice for these patients and can often be performed at the time of the splenectomy.complications and adverse effectsthe risk of postsplenectomy infecti

23、ons depends on the etiology of the disease. the majority of infections occur within 2 years after splenoctomy and are fatal in almost 50% of cases. the incidence is low in patients with spherocytosis, itp and splenic trauma and higher in hodgkin? s disease (8%)-splenoportal thrombosis following sple

24、nectomy is a rare complication.12, 14 the true rate of thrombotic complications after splenectomy is not defined, but early diagnosis and prompt initiation of anticoagulant therapy is mandatory. port site splcnosis has been reported after laparoscopicsplenectomyl3 and has to be considered in the dif

25、ferential diagnosis of port site pain and a palpable nodule. leaks along the pancreatic tai 1 can also rarely occur after splencctomy.summarynonoperative therapy is the first-line management for almost all splenic diseases in patients with hematologic and immunological disease in whom medical therap

26、y has failed, and in patients with splenic cysts, the laparoscopic approach is feasible and represents an appropriate option.references1. tulman s, holcomb gw, karamanoukian iil, et al: pediatr laparoscopic splenectomy. j pediatr surg 28:689-692, 19932. bax nma, van der zee d: laparoscopic splenecto

27、my: is this the way to do it? pediatr endosurg & innov tech 5:281-286, 20013. rescorla fj, engum sa, west kw, tres scherer lr 3rd, rouse tm, grosfeld jl: laparoscopic splenectomy has become the goldstandard in children. am surg 68:297-301, 20024. van der zee dc, kramer wl, ure bm, mokhaberi b, b

28、ax nm: laparoscopic management of a large posttraumatic splenic cyst ina child. surg endosc 13:1241-2, 19995. morgenstern l: nonparasitic splenic cysts: pathogenesis, classification, and treatment. j am coll surg 194:306-14, 20026. stylianos s. compliance with evidence-based guidelines in children w

29、ith isolated spleen or liver injury: a prospective study. jpediatr surg. 2002 mar;37(3):453-6.7. reddy vs, phan hh, o' neill ja, neblett ww, pietsch jb, morgan wm, cywes r, holcomb gwiii: laparoscopic versus opensplenectomy in the pediatric population: a contemporary single-center experience. am

30、 surg 67:859-63, 20018. durgun v, kapan s, kapan m, karabicak i, aydogan f, goksoy e: primary splenic hydatidosis. dig surg 20:38-41, 20039 ormeci n, soykan i, palabiyikoglu m, i de iman r, erdem ii, bektas a,sari oglu m: a new therapeutic approach for treatment ofhydatid cysts of the spleen. dig dis sci 47:2037-44, 200210. bickel a, loberant n, singer-jordan j, goldfeld m, daud g, eitan a: the laparoscopic approach to abdominal hydatid cysts:a prospective nonselectivc study using the isolated hypobaric technique. archsurg 136