Partial splenectomy in Gaucher's disease

In 11 children with hypersplenism due to Gaucher's disease, partial splenectomy was planned with the aim to prevent the development of postsplenectomy sepsis and also to slow the advance of the disease in the rest of the reticuloendothelial system by permitting continuing accumulation of the beta-glucocerebroside in the remaining splenic tissue. In seven children, partial splenectomy was performed successfully, the weight of the splenic tissue removed ranging from 400 to 3,680 g. The postoperative course was uneventful and the average duration of hospitalization was 12 days. In subsequent follow-up, isotope scanning demonstrated continuing growth of the splenic remnant and there were no episodes of postsplenectomy sepsis nor evidence of increased accumulation of beta-glucocerebroside in the liver or bones. These children showed a marked improvement in the growth curve and dramatic improvement in the hematologic picture. Of the four remaining children, in two, partial splenectomy was followed by complete removal of the remaining spleen due to necrosis, whereas in two, total splenectomy was performed since the huge spleens were extensively infarcted. Our experience suggests that partial splenectomy is the treatment of choice in the management of young patients with hypersplenism due to Gaucher's disease.     

Partial splenectomy in children: An alternative for splenectomy in the pathological staging of Hodgkin's disease

Background: The more accurate staging of Hodgkin's disease in children is achieved with a staging laparotomy and splenectomy. A disadvantage of the splenectomy is the high risk for an overwhelming postsplenectomy sepsis (OPSI). Therefore, the partial splenectomy was introduced as an alternative to splenectomy in the staging of Hodgkin's diseases in children. Methods: During the period 1982–1988, 12 children with Hodgkin's disease underwent a staging laparotomy with partial splenectomy. All patients were preoperatively vaccinated withPneumococcus vaccine. The first three patients received 44 Gy locoregional radiotherapy, whereas nine patients received 25 Gy locoregional radiotherapy and two courses of MOPP/ABVD (mitoxin, oncovin [vincristine], procarbazine, prednisone/adriamycin, bleomycin, vinblastine, decarbazine). Results: The morbidity was negligible. The pathological stage changed in three patients (25%). During a median follow-up of 6 years (range 4–10), no OPSI was diagnosed. One patient developed a secondary leukaemia. Conclusions: Staging laparotomy for Hodgkin's disease is being performed with less frequency because the majority of patients are treated with chemotherapy and low-dose radiation therapy. After splenectomy and chemotherapy regimens with alkylating agents, there is an increased risk for secondary acute leukemia. With partial splenectomy an adequate staging of the disease can be achieved, allowing a more tailored therapy so that systemic chemotherapy will not be used as frequently, resulting in a lower treatment morbidity without decreasing survival.The results of this study were presented at the 46th Annual Cancer Symposium of The Society of Surgical Oncology, Los Angeles, California, March 18–21, 1993.     

Partial splenectomy for littoral cell angioma

Littoral cell angioma is a rare primary splenic tumor that is difficult to differentiate preoperatively from other benign and malignant splenic lesions and may have immunogenic origins. Preoperative ultrasound and computed tomographic scan showed that our patient's lesion was solitary and localized. Total splenectomy is usually required because littoral cell angioma is generally large and/or diffuse. However, a partial splenectomy was able to be performed, making this the first reported partial splenectomy for littoral cell angioma.     

Partial splenectomy for symptomatic splenic hamartoma

Lower pole splenectomy with preservation of residual splenic function was successfully performed in a 3-year-old boy with a symptomatic splenic hamartoma. Following resection, several of the patient's constitutional symptoms resolved. This is the first reported case of a splenic hamartoma treated by partial splenectomy.     

Partial splenectomy for nonparasitic splenic cysts

Four cases of partial splenectomy for large, nonparasitic splenic cysts are described. In all four patients it was possible to successfully preserve most of the splenic parenchyma. With the increasing realization of the importance of splenic function, the feasibility of this operative approach to splenic cysts is proposed.     

脾部分切除术治疗非寄生虫性脾囊肿六例分析

脾囊肿临床少见,近年来随着B超体检的广泛应用,其发现率逐渐升高.脾囊肿的治疗方式多样.综合近年来文献,脾囊肿的手术治疗多倾向于微创、尽可能保留脾脏组织、保留脾脏功能.2006年5月至2008年5月北京朝阳医院行6例开腹脾脏部分切除术治疗脾脏非寄生虫性囊肿均收到较好的疗效.现回顾性分析报道如下.     

Partial splenectomy for a splenic cyst

Partial splenectomy for a large splenic cyst was performed in a seven-year-old girl referred for splenomegaly. The increased risk of sepsis in splenectomized patients induced the successful attempt at partial splenectomy. The surgical procedure described involved removal of the cyst while preserving as much of the spleen as possible. In view of the planocellular epithelial lining, the pathological diagnosis was of a rare congenital, intrasplenic cyst.     

脾部分切除术治疗闭合性脾破裂

目的 探讨外伤性脾破裂脾部分切除的治疗效果。方法 回顾性分析行部分性脾切除治疗的28例外伤性脾破裂患者的临床资料。结果 本组脾部分治疗失败进行脾切除1例，治疗成功率96．4％，无死亡病例。27例出院随访1－58个月，未发现并发症；其中15例随访22个月以上，血液学检查，免疫学功能测定等指标均在正常范围，^99mTC扫描和B型超声检查示保留脾显像良好。结论 脾部分切除对合适的脾破裂病例是安全，有效的治疗，且保留的脾具有正常的脾功能。     

脾部分切除术治疗非寄生虫性脾囊肿六例分析

脾囊肿临床少见,近年来随着B超体检的广泛应用,其发现率逐渐升高.脾囊肿的治疗方式多样.综合近年来文献,脾囊肿的手术治疗多倾向于微创、尽可能保留脾脏组织、保留脾脏功能.2006年5月至2008年5月北京朝阳医院行6例开腹脾脏部分切除术治疗脾脏非寄生虫性囊肿均收到较好的疗效.现回顾性分析报道如下.     

Partial splenectomy prevents splenic sequestration crises in sickle cell disease

Purpose

Acute splenic sequestrations (SSs) are potentially fatal complications in sickle cell disease (SCD). Total splenectomies in young patients may predispose them to a higher risk of overwhelming infections, whereas partial splenectomy may maintain immunocompetence. We present our series of partial splenectomies in patients with multiple SS episodes.

Methods

We retrospectively reviewed the records of 6 patients who underwent open partial splenectomies for SS. Data on their clinical courses were collected and analyzed.

Results

None of the 6 patients had SS postprocedure, down from 2.1 ± 1.0 (P = .003) sequestrations per year and 3.5 ± 1.4 (P = .002) total sequestrations per patient. The transfusion requirements were significantly reduced postoperatively (10.2 ± 5.6 vs 2.0 ± 3.1 per year; P = .002). There was no increase in the infection-related hospital admissions during the period of follow-up (1.5 ± 1.8 vs 0.8 ± 0.8 per year after partial splenectomy; P = .363). The upper pole was preserved in all cases with blood supply off the main splenic artery.

Conclusions

Partial splenectomy decreases the risk of SS in SCD and reduces the need for blood transfusions. Infection rates did not increase after the procedure during the follow-up period. Partial splenectomy should be considered for patients who experience multiple acute SS crises or have long-term transfusion requirements.     

脾部分切除治疗脾良性肿瘤(附16例报告)

目的:探讨脾部分切除术治疗脾良性肿瘤的疗效.方法:对16例脾良性肿瘤施行脾部分切除手术.其中6例采用脾部分切除加大网膜包裹残面术,10例采用脾部分切除加止血凝胶覆盖.对术后并发症进行统计.结果:16例中1例术后出现左膈下脓肿,1例脾部分坏死,余无并发症发生.平均住院时间12d.结论:脾部分切除术治疗脾良性肿瘤术后并发症少,住院时间短,是安全有效的手术.     

Partial splenic embolization. An effective alternative to splenectomy for hypersplenism

Splenectomy for massive splenomegaly and hypersplenism carries a significant morbidity and mortality. We have used partial splenic embolization (PSE) as an effective alternative to splenectomy. Ten PSE procedures were performed on nine patients without mortality and with minimal morbidity. The age of the patients ranged from 8 months to 32 years (mean 14 years). The causes of splenomegaly and hypersplenism included cystic fibrosis with cirrhosis (2), tyrosinemia and cirrhosis (1); thalassemia (1), hemophilia with Human Immune Deficiency Virus infection (2), chronic hepatitis with portal hypertension (1), malignant histiocytosis (1), and Wiskott-Aldrich Syndrome (1). All procedures were performed under local anesthesia with sedation. A percutaneous femoral artery approach to the splenic artery was used to deliver Ivalon sponge particles (280-800 microns) into the spleen. Splenic infarction was assessed by postembolization angiograms. All of the patients except one demonstrated improvement of hematologic parameters. In one patient, however, cytopenia improved only after a second embolization. In the total series, there was an early mean rise of 8,600/mm3 in the leukocyte count (range 2,900-14,900) and 212,000/mm3 in the platelet count (range 30,000-718,000). Follow-up ranged from 4 months to 7 years. Improvement of the blood picture has been persistent in seven of the eight patients who showed initial improvement. Transient procedural complications included fever (5), pleural effusion (2), pneumonia (1), and splenic abscess (1). One patient had paralytic ileus lasting for 10 days and one patient developed a streptococcal peritonitis 3 weeks after embolization. No patient developed pancreatitis or vascular compromise of other abdominal viscera.(ABSTRACT TRUNCATED AT 250 WORDS)     

Perioperative complications of splenectomy for hematologic disease.

A 15-year study of perioperative complications was carried out in 142 adults who underwent splenectomy for hematologic disease at the University of Alberta Hospital in order to obtain recent statistics on morbidity and mortality. The patients were grouped into four diagnostic categories: idiopathic thrombocytopenic purpura (71 patients), lymphoproliferative disorders (34 patients), myeloproliferative disorders (12 patients) and miscellaneous disorders (25 patients). Splenectomy was carried out for therapeutic reasons in 93% of patients and to establish a diagnosis in 7%. The overall complication rate was 22% (31 of 142) and the death rate was 6% (7 of 142). Infection accounted for 42% of the complications. Steroid or antibiotic therapy preoperatively did not significantly affect the infection rate. Drains, if removed within the first week, also did not affect the postoperative infection rate. Spleen size and the interaction between diagnosis and the presence of thrombocytopenia were predictors of the need for intraoperative transfusion.     

Partial splenectomy for hereditary spherocytosis: a multi-institutional review

Background/Purpose

Partial splenectomy has emerged as a surgical option for selected children with hereditary spherocytosis, with the goal of reducing anemia while preserving splenic function. This multi-institutional study is the largest series to date examining outcomes data for partial splenectomy in patients with hereditary spherocytosis.

Methods

Data were collected retrospectively from 5 North American pediatric hospitals. Sixty-two children underwent partial splenectomy for hereditary spherocytosis between 1990 and 2008.

Results

At 1 year following partial splenectomy, mean hemoglobin significantly increased by 3.0 ± 1.4 g/dL (n = 52), reticulocyte count decreased by 6.6% ± 6.6% (n = 41), and bilirubin level decreased by 1.3 ± 0.9 mg/dL (n = 25). Patients with poor or transient hematologic response were found to have significantly more splenic regeneration postoperatively compared with patients with a durable clinical response (maximal spleen dimension, 9.0 ± 3.4 vs 6.3 ± 2.2 cm). Clinically significant recurrence of anemia or abdominal pain led to completion splenectomy in 4.84% of patients. No patients developed postsplenectomy sepsis.

Conclusions

Our multi-institutional review indicates that partial splenectomy for hereditary spherocytosis leads to sustained and clinically significant improvement in hematologic profiles and clinical symptoms in most patients. Our data support partial splenectomy as an alternative for selected children with hereditary spherocytosis.     

Partial splenectomy for benign cystic lesions of the spleen

Cystic lesions of the spleen are uncommon, about 600 cases being reported in the world literature. This report concerns three pediatric patients treated by partial splenectomy for benign cyst. In the world literature most of the cystic lesions of the spleen are treated by splenectomy and more recently by partial splenectomy. The infected lesions are treated by splenectomy or by incision and drainage only. Partial splenectomy has evident advantage over splenectomy. Although in the world literature we have not found a single case of infected splenic benign cyst treated by partial splenectomy, we believe that this procedure also has an advantage over drainage as the infected and necrotic part of the cyst is removed, thereby reducing morbidity and further possible complications.     

Laparoscopic splenectomy for lymphoproliferative disease

Background: Elective laparoscopic splenectomy (LS) achieves excellent results for benign hematologic diseases. The role of LS for hematologic malignancies is harder to define owing to associated splenomegaly and patient disease that may alter outcome. Methods: Retrospective review of single institution experience 1996 through 2002. To limit variability of disease processes, only patients with immune thrombocytopenic purpura (ITP) and lymphoproliferative disease (LPD) were studied. Results: A total of 211 LS have been performed, including 73 for LPD and 86 for ITP. Patients with LPD were significantly older, 61 vs 46 years p<0.001; male, 45 (62%) vs 33 (38%), p<0.001; and larger splenic weight, 680 vs 162 g, p<0.001. Fifty-nine patients (81%) with LPD were operated with standard LS with a conversion rate of 15%. Hand-assisted LS was performed in 14 patients (19%), and three were converted to open. Compared to ITP, patients with LPD had longer operative time, 148 vs 126 min, p<0001, and higher blood loss, 200 vs 100 cc, p = 0.004. There was one mortality (0.6%), and morbidity occurred in six patients (8%) with LPD and seven (8%) with ITP. The median length of stay was 3 days for LPD and 2 days for ITP, p = 0.03. Forty-six patients were principally operated for a diagnosis, and 27 (60%) were found to have lymphoma. Conclusions: LS can be performed safely in patients with LPD, and when used judiciously with hand-assisted techniques can be performed with low conversion and morbidity rates. Splenectomy plays an important role in establishing the diagnosis of lymphoma in LPD.     

Partial splenectomy and overwhelming infection in rats

Susceptibility to overwhelming sepsis in rats was measured by intravenous Streptococcus pneumoniae challenge 5 weeks after removal of 25, 50, or 75% of the spleen, with sham splenectomy and total splenectomy groups included for comparison. The LD₅₀ (given in organisms per animal) for total splenectomy was 1.02 × 10³, for 75% splenectomy, 1.79 × 10⁴, for 50% splenectomy, 4.69 × 10⁴, for 25% splenectomy, 4.90 × 10⁵, and for sham splenectomy, 8.04 × 10⁶. All differences were significant at P < 0.05 except for that between 50 and 75% splenectomy. Thus sham, 25, 50, and 75% splenectomy were all associated with a higher LD₅₀ than total splenectomy, and the LD₅₀ increased in proportion to the size of the splenic remnant. No threshold for protection against overwhelming infection was noted, but a rapid fall in LD₅₀ from 75% to total splenectomy may be indicative of a critical splenic mass within that range.     

Partial splenectomy for children with congenital hemolytic anemia and massive splenomegaly

Partial splenectomy is an alternative to total splenectomy for the treatment of congenital hemolytic anemias (CHAs) in children, although the feasibility of this technique in the setting of massive splenomegaly is unknown. This study was designed to evaluate the safety and efficacy of partial splenectomy in children with CHAs and massive splenomegaly. This retrospective study examined 29 children with CHAs who underwent partial splenectomy. Children were divided into 2 groups based on splenic size: 8 children had splenic volumes greater than 500 mL, whereas 21 children had splenic volumes less than 500 mL. Outcome variables included perioperative complications, transfusion requirements, hematocrits, reticulocyte counts, bilirubin levels, splenic sequestration, and splenic regrowth. All 29 children underwent successful partial splenectomy with 0.02 to 10 years of follow-up. After partial splenectomy, children overall had decreased transfusion requirements, increased hematocrits, decreased bilirubin levels, decreased reticulocyte counts, and elimination of splenic sequestration. Children with massive splenomegaly had similar outcomes compared with children without massive splenomegaly. Long-term complications included 3 mild infections, 4 cases of gallstones requiring cholecystectomy, and 1 child who required completion splenectomy. Partial splenectomy is a safe, effective, and technically feasible option for children with various CHAs, even in the setting of massive splenomegaly.