Pediatric Clinics of North America - CIPERJ

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508 TRACY & RICE limited splenic volume can preserve the splenic phagocytic response to Streptococcus pneumoniae, several groups (including the authors) have adopted a goal of removing 80% to 90% of the enlarged spleen for most conditions [16,37–39]. The residual 10% to 20% volume is equivalent to approximately one third of the normal splenic volume, estimated as sufficient for immune preservation. Preoperative considerations include assessing splenic volume by ultrasonography and ensuring that patients are vaccinated adequately, in case during surgery the need to convert to total splenectomy is determined. Because some children have a splenic anatomy that is not amenable to partial splenectomy, all children undergoing partial splenectomy should be prepared preoperatively for potential total splenectomy. Immunizations against encapsulated bacteria, including polyvalent pneumococcal vaccination (Pneumovax, Prevnar) and meningococcal vaccination (Meningovax, Menactra) should be administered, and H influenzae type b vaccination if prior administration cannot be confirmed. Partial splenectomy can be performed using an open laparotomy or by a laparoscopic approach, and the choice of approach is dependent a surgeon’s experience and preference. In cases of massive splenomegaly (O500 mL volume), the authors prefer the open approach, although further refinement of surgical techniques may allow for good clinical outcomes by laparoscopy. With either approach, the spleen first is exposed carefully. The splenic vasculature is examined and vessels ligated to ensure adequate perfusion of the splenic remnant. The authors generally prefer to preserve the upper pole of the spleen given the risk for torsion associated with nonfixation of the lower pole, although other groups prefer preservation of the lower pole with associated splenopexy, similar to treatment for a wandering spleen [40]. Before proceeding with splenic transection, adequate flow is confirmed to the upper pole via one or two short gastric vessels. If vessels to the upper pole are absent or if severe intraoperative bleeding is encountered, the authors convert to a total splenectomy. In the authors’ experience, intraoperative conversion to total splenectomy is required in less than 5% of cases. After assessing the vessels, the spleen is devascularized partially to maintain flow from the short gastric arcades to the upper pole, and the ischemic portion of the spleen is allowed to demarcate visually between the pink vascularized tissue and the dark devascularized portion. Before splenic transection, the devascularized spleen then can be compressed manually for two reasons: first, to autotransfuse a substantial volume of blood back into the patient, and second, to enable an easier surgical transaction of this 3-D mass. The splenic parenchyma then is divided using a surgical stapler (for small or normal spleens) or with electrocautery, TissueLink device, or LigaSure (for cases of massive splenomegaly). After parenchyma transection, bleeding from the splenic bed can be controlled with an argon beam coagulator, suture ligation of vessels, and topical hemostatic agents (Fig. 1).
PARTIAL SPLENECTOMY FOR HEREDITARY SPHEROCYTOSIS 509 Fig. 1. Intraoperative photograph of spleen after selective devascularization of lower pole and main body. Upper pole remains perfused from short gastric vessels, allowing for resection of 85% of the splenic parenchyma. The authors’ goal of performing partial splenectomy, retaining approximately 30% of normal splenic volume, can require 90% to 95% resection in patients who have massive splenomegaly. Their previous published experience has shown that intraoperative surgeons’ estimates of retained splenic volume correlate closely with sonographic measurements of postoperative retained splenic parenchyma [12]. After the procedure, ultrasonography should be performed periodically to assure blood flow to and from the splenic remnant and to estimate the residual splenic volume. The authors routinely perform an ultrasonography measurement in the early postoperative period (ie, before discharge) and then at 1 month and 2 months and thereafter at 6- to 12-month intervals. Along with regular sonographic assessment of the splenic remnant, the authors prescribe postoperative antibiotic prophylaxis with oral penicillin (or a suitable alternative) for at least 1 year and continue long-term prophylaxis at the discretion of the hematologist. With the laparoscopic or open approach, the authors also advise activity restrictions for 6 weeks after surgery to minimize the risk for postoperative bleeding. Evaluation of splenic function after partial splenectomy Demonstration that splenic function is preserved after partial splenectomy is critically important in assessing its role in the management of children who have clinically severe HS. The gold standard for evaluating splenic immunologic function is the liver-spleen radionuclide scan, which detects the ability of the splenic macrophages to phagocytose technetium-99m sulfur colloid. Uptake of the radionuclide within the spleen remnant is an indicator of functioning splenic tissue, although concerns about radioactive exposure limits its frequent use in children. The quantitation of circulating erythrocytes with vesicles, which normally are removed by splenic filtration,
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Pediatr Clin N Am 55 (2008) xiii-xi
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Pediatr Clin N Am 55 (2008) 287-304
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DECISION ANALYSIS IN PEDIATRIC HEMA
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Nietert et al [30] Treatment of sic
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VENOUS THROMBOEMBOLISM IN CHILDREN
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PEDIATRIC ARTERIAL ISCHEMIC STROKE
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CARE OF PATIENTS WITH VASCULAR ANOM
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358 RODRIGUEZ & HOOTS Fig. 1. X-lin
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Table 1 Clotting factor concentrate
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Table 1 (continued ) Factor VIII (o
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364 RODRIGUEZ & HOOTS was administe
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366 RODRIGUEZ & HOOTS Antifibrinoly
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368 RODRIGUEZ & HOOTS infections an
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370 RODRIGUEZ & HOOTS Fig. 3. Schem
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372 RODRIGUEZ & HOOTS protein is no
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374 RODRIGUEZ & HOOTS [2] Berntorp
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376 RODRIGUEZ & HOOTS [42] Carcao M
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378 ROBERTSON et al von Willebrand
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380 ROBERTSON et al Fig. 2. A propo
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382 ROBERTSON et al a heterogenous
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384 ROBERTSON et al of considering
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386 ROBERTSON et al Type 2M Type 2M
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388 ROBERTSON et al Desmopressin is
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390 ROBERTSON et al References [1]
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392 ROBERTSON et al [46] Nesbitt IM
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394 BLANCHETTE & BOLTON-MAGGS A key
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396 BLANCHETTE & BOLTON-MAGGS recep
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398 BLANCHETTE & BOLTON-MAGGS and p
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400 BLANCHETTE & BOLTON-MAGGS Fig.
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402 BLANCHETTE & BOLTON-MAGGS in di
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404 BLANCHETTE & BOLTON-MAGGS the s
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406 BLANCHETTE & BOLTON-MAGGS findi
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408 BLANCHETTE & BOLTON-MAGGS as a
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410 BLANCHETTE & BOLTON-MAGGS splen
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412 BLANCHETTE & BOLTON-MAGGS with
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414 BLANCHETTE & BOLTON-MAGGS react
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416 BLANCHETTE & BOLTON-MAGGS [16]
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422 FASANO & LUBAN of storage and t
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424 FASANO & LUBAN Leukocyte reduct
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426 FASANO & LUBAN RBCs is decrease
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Table 1 Blood component characteris
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430 FASANO & LUBAN Alloimmunization
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432 FASANO & LUBAN of individual un
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434 FASANO & LUBAN patients have an
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436 FASANO & LUBAN adults; therefor
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438 FASANO & LUBAN [21]. Meta-analy
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440 FASANO & LUBAN Pretransfusion m
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442 FASANO & LUBAN [55,56]. This is
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444 FASANO & LUBAN [20] Roseff SD,
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THALASSEMIA UPDATE 449 hypochromia
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THALASSEMIA UPDATE 451 Fig. 1. Chan
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THALASSEMIA UPDATE 453 delivery of
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THALASSEMIA UPDATE 455 thromboses i
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