Guidelines for Surgical Treatment of Pediatric Spleen Diseases

Introduction

The 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 benefits are also described with laparoscopic therapy of splenic cysts.

Definition

The 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 include splenomegaly, splenic destruction of one or more cell 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.

Management

The 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 thrombocytopenia 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, Gaucher’s disease, and portal hypertension. The frequency of splenectomy for portal hypertension has considerably decreased in recent years.

Surgical Therapy

Numerous 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 splenectomy with respect to the degree of splenomegaly. Some authors find splenic artery embolization a useful procedure in obese patients.2

Open Splenectomy

Open 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 splenorenal ligament 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 patients 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 be covered with omentum or a synthetic mesh.

Laparoscopic Splenectomy

The number of the ports used depends on the surgeon’s preference and range from 3 to 5 1,2,3,7. Ligation of the short gastric vessels is easily performed while the lateral attachments are still intact. A retractor or other instrument may be used to push the spleen medially to enable cutting the lateral peritoneal attachments and the splenocolic 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 mesentery, and omentum should be inspected for accessory splenic tissue. The spleen is placed into a sac which is introduced via the umbilical or anterior axillary port.

In cases where it is difficult to place the spleen into the endoscopic bag, filling the sac partially with irrigation fluids, tying an umbilical tape loosely around the middle portion of the spleen, or placing the spleen in the pelvis and the sac in the splenic bed, may help to retrieve the organ13. The umbilical incision can be enlarged slightly to enable removal of the spleen. Once inside the bag, the spleen can be divided into pieces by digital fracture with ring forceps, or by using an automatic tissue morcellator.

Surgical Therapy of Splenic Cysts

Nonparasitic splenic cysts that are symptomatic or are over 5 cm in diameter should be removed either by partial splenectomy or near-total cystectomy with decapsulation.5 These procedures can be performed laparoscopically. 4 Three or 4 canullas are used. For decapsulation, electrocautery scissors4 or ultrasonic shears may be used. It remains unclear whether fulgurization of the remaining cyst wall and omentopexy prevent recurrences.

The optimal treatment of hydatic splenic cysts is still debatable. Most authors recommend splenectomy following medical treatment 8, but successful conservative management by percutanous puncture under sonographic guidance and instillation of alcoholic or hypobaric solutions is reported.9 Partial cystectomy has been recommended for selected cases using laparoscopic techniques.10

Associated Procedures

Children 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 Effects

The risk of postsplenectomy infections depends on the etiology of the disease. The majority of infections occur within 2 years after splenectomy 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 splenectomy 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 splenosis has been reported after laparoscopic splenectomy13 and has to be considered in the differential diagnosis of port site pain and a palpable nodule. Leaks along the pancreatic tail can also rarely occur after splenectomy.

Summary

Nonoperative therapy is the first-line management for almost all splenic diseases. In patients with hematologic and immunological disease in whom medical therapy has failed, and in patients with splenic cysts, the laparoscopic approach is feasible and represents an appropriate option.

References

International Pediatric Endosurgery Group (IPEG) 11300 West Olympic Boulevard, Suite 600 Los Angeles, CA 90064 E-mail: ipegweb@ipeg.org

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