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Indications and complications of splenectomy for children with sickle cell disease.

BACKGROUND: Sickle cell anemia (SCA), which is characterized by high hemoglobin (Hb) F level and persistent splenomegaly into the older age group (up to 18 years of age) or even adults, is one of the commonest hemoglobinopathies in the Eastern Province of Saudi Arabia. This makes them liable to develop splenic complications requiring splenectomy. This is a review of our experience in the management of 134 children with SCA who had splenectomy as part of their management at our hospital, with emphasis given to the indications and complications of splenectomy. PATIENTS AND METHODS: The medical records of all children who had splenectomy at our hospital were retrospectively reviewed for the following: age at splenectomy, sex, Hb electrophoresis, indication for splenectomy, preoperative investigations, type of surgery, spleen weight, histology, perioperative management, and postoperative complications. RESULTS: From 1990 to 2004, 170 children with various hematologic disorders had splenectomy at our hospital. Of these, 134 had SCA (118 had sickle cell disease and 16 had sickle-beta-thalassemia). Recurrent acute splenic sequestration crisis (ASSC) was the commonest indication for splenectomy in 103 (76.9%) patients, followed by hypersplenism in 18 (13.4%). Seven (5.2%) of our patients had splenectomy for splenic abscess (SA) and 2 had splenectomy for massive splenic infarction; 103 (61 boys, 42 girls) patients with a mean age of 7.6 years (range, 1.8-13 years) had splenectomy for ASSC. Their mean Hb F level was 20.5% (range, 9.2%-39.6%). Thirty-two of them had major attacks. Their Hb levels at the time of admission ranged from 1.4 to 4.1 g/dL (mean, 2.5 g/dL). The remaining 71 had minor recurrent attacks. Eighteen had splenectomy for hypersplenism and all had a significant increase in their blood parameters after splenectomy. Seven had splenectomy for SA. In 5 patients, Salmonella was the causative organism; in 1, it was Enterobacter sakazaki, whereas in 1, no organisms were identified. Two of our patients had splenectomy for massive splenic infarction because of persistent left upper quadrant abdominal pain, and 1 had splenectomy for splenomegaly with a nonfunctioning spleen. Twenty-eight (21%) of our patients had splenectomy and cholecystectomy. In 4 patients, this was because of symptomatic gallstones, whereas in the other 24, it was because of asymptomatic gallstones discovered on ultrasound. There was no mortality, but 8 (6%) developed postoperative complications. CONCLUSIONS: With good perioperative management, splenectomy in children with SCA is not only safe, but also beneficial in treating SA, reducing the patients' transfusion requirements, eliminating the risks of ASSC, and eliminating the discomfort and mechanical pressure of the enlarged spleen. Abdominal ultrasound should be done routinely preoperatively for all children with SCA undergoing splenectomy, and if gallstones are discovered, they should undergo concomitant cholecystectomy. This is even so for asymptomatic gallstones. The addition of cholecystectomy to splenectomy does not increase the morbidity, but eliminates the subsequent complications of gallstones and simplifies their future management in case of abdominal crisis as the possibility of cholecystitis is eliminated.

Adolescent↗

[Splenectomy in haematologic diseases. Clinical indications and surgical technique].

After the introduction of corticosteroids fifty years ago the indications for splenectomy in benign haematological diseases became more controversial, also due to the morbidity and mortality associated at that time with open splenectomy. The advent of minimally invasive techniques has provided safe procedures for removal of the spleen in cases of benign as well as malignant haematological disease. Laparoscopic splenectomy has been performed for spleens of normal size or larger size or weight. In this study the indications in haematological diseases and the results after splenectomy are analysed. From June 1998 to December 2004 107 patients with benign or malignant haematological disease were referred to our unit for splenectomy. Splenomegaly was defined as a spleen diameter > 15 cm and weight > 400 g and was present in 53% of cases in this series. Open splenectomy was carried out in 30 cases and laparoscopic splenectomy in 77. Operating time, blood loss, conversion rate, need for transfusion, complications, length of hospital stay and operative morbidity were analysed for both open and laparoscopic procedures. In the laparoscopic splenectomy group the outcomes after removal of spleens < 15 cm and > 15 cm were compared. Clinical results after splenectomy in haemolytic anaemia and idiopathic thrombocytopenic purpura are reported. In the open splenectomy group the spleens were larger and heavier, hence the operating time was greater than in the laparoscopic splenectomy group. The mean age of patients in the open group was 65 years as against 43 years in the laparoscopic group. Morbidity was 23% in the open group and 10% in the laparoscopic group. Mortality was nil in both groups. The overall conversion rate in the laparoscopic group was 2.6% owing to extensive adhesions and bleeding in 2 large spleens measuring > 27 cm and weighing > 2 kg (conversion rate for larger spleens: 6.2%). Spleens > 15 cm were associated with greater blood loss (p < 0.01), longer operating times and a longer hospital stay. No cases of overwhelming post-splenectomy infection were registered in either group. The healing rate for idiopathic thrombocytopenic purpura after splenectomy was 87%, while for haemolytic anaemia it was 100%. In this study splenomegaly was associated with malignant haematological disease occurring in patients aged 65 on average in whom an open splenectomy was generally carried out. Benign diseases occurred in patients aged 43 on average in whom laparoscopic splenectomy was the preferred procedure. Conversion rate, morbidity and length of hospital stay were comparable with those of other series. Laparoscopic splenectomy can be considered the gold standard procedure for benign disease in young patients and also as a safe procedure in selected cases of malignant haematological disease.

Adolescent↗

Effect of splenectomy on slowing human immunodeficiency virus disease progression.

BACKGROUND: Lymphoreticular tissue is the most important site for human immunodeficiency virus (HIV) replication in HIV-infected individuals. OBJECTIVE: To compare the long-term effect of splenectomy on survival and time to development of acquired immunodeficiency syndrome in subjects who had undergone splenectomy with subjects who had not undergone splenectomy. DESIGN: A cohort study with a follow-up of up to 13.4 years. SETTING: Subjects were recruited from a hospital outpatient clinic population and a multicenter study of patients with hemophilia. PARTICIPANTS: Forty-five HIV-infected individuals were observed prospectively for up to 13.4 years (17 had undergone splenectomy and 28 had not undergone splenectomy). Five subjects underwent splenectomy before acquiring HIV infection and 12 underwent splenectomy during the asymptomatic phase of HIV infection. The group who did not undergo splenectomy consisted of HIV-infected individuals who were asymptomatic at study enrollment. MAIN OUTCOME MEASURES: A Cox proportional hazards model was used to test the effects of splenectomy on survival and time to development of acquired immunodeficiency syndrome when adjusting for potential confounders (age, initial CD4+ cell count, and treatment with antiretroviral drugs). Splenectomy was treated as a time-dependent covariate to account for the variation in its timing. RESULTS: During the average follow-up of 8.6 years, 9 (53%) of the 17 subjects who underwent splenectomy and 23 (82%) of the 28 subjects who did not undergo splenectomy died; acquired immunodeficiency syndrome developed in 6 (35%) of the subjects who underwent splenectomy and 23 (82%) of the subjects who did not undergo splenectomy. Splenectomy was associated with a significant reduction of risk of developing acquired immunodeficiency syndrome (adjusted relative risk [RR] <0.4, P<.05), whereas the effect on risk of mortality approached, although it did not reach, significance (adjusted RR approximately 0.5, P approximately .10). CONCLUSION: The absence of a spleen during the asymptomatic phase of HIV infection seems to have a beneficial effect on HIV disease progression.

Acquired Immunodeficiency Syndrome↗

The potential for treatment of idiopathic thrombocytopenic purpura with anti-D to prevent splenectomy: a predictive cost analysis.

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder affecting both children and adults that can be manifested by severe bleeding episodes. Adult ITP patients have a low rate of spontaneous remission, and symptomatic patients commonly undergo splenectomy; however, maintenance therapy may increase the rate of remission, allowing splenectomy to be avoided. Anti-D is a recently licensed treatment for ITP that has the potential to delay, and possibly avoid, the need for splenectomy. We used preliminary data from an ongoing clinical trial to evaluate the costs involved in using anti-D therapy for 1 year with the intent of avoiding the need for splenectomy. We accounted for different possible outcomes at the completion of the clinical trial. An economic model with a theoretical cohort of 100 patients was developed using the model of an ongoing clinical trial. The average wholesale price was used to determine the cost of an infusion of anti-D based on an average dose ($1,213 per infusion). The cost of splenectomy was determined by a literature review ($16,000). Costs were calculated for all known patient outcomes; where outcomes were unknown and likely to vary, all possible outcomes were accounted for (splenectomy or no splenectomy). In our theoretical cohort, 31 of 100 patients were taken off anti-D and received splenectomy, 32 of 100 were stable after receiving anti-D and would not need splenectomy, and 37 of 100 had Indeterminate outcomes after receiving anti-D. When compared with the cost of the hypothetical scenario of initially giving all 100 patients splenectomy ($1.6 million), a minimum of 47 patients would have to avoid splenectomy to result in a cost savings for our cohort of 100 patients. The group of 47 patients avoiding splenectomy would be composed of the 32 patients comprising the stable group and at least 15 of the 37 patients comprising the group with indeterminate outcomes. If all 37 of the patients in the group with indeterminate outcomes avoid splenectomy, $363,000 and 69 spleens would be saved. Our data suggest that in the phase III trial of maintenance anti-D therapy versus immediate splenectomy, anti-D therapy will be a cost-effective option if 47% or more of patients avoid splenectomy.

Adult↗

Results of laparoscopic and open splenectomy for nontraumatic diseases.

The medical records of patients who had undergone splenectomy for nontraumatic diseases of the spleen between 1997 and 2000 were reviewed. The aim of the study was to evaluate the short-term outcomes of open and laparoscopic splenectomies and to determine whether some well-known benefits of laparoscopic surgery could be observed in patients who underwent laparoscopic splenectomy for nontraumatic splenic diseases. The data of 44 patients were available for analysis and included 20 patients (45.5%) who underwent laparoscopic splenectomy and 24 patients (54.5%) who underwent open splenectomy. Various parameters were reported for open and laparoscopic procedures separately, including associated surgical procedures, spleen weight, postoperative mortality and morbidity rates, perioperative blood transfusions, use and length of abdominal drainage, accessory spleen removal, operative times, length of hospital recovery, and hematologic parameters on admission to and discharge from the hospital. Laparoscopic splenectomy was successfully completed in all 20 considered patients with no conversion to open splenectomy. The supine position and four trocars were adopted in all patients. Accessory spleens were found in four (9.0%) patients: two (4.5%) during open splenectomy and two (4.5%) during laparoscopic splenectomy. The postoperative mortality rate was 2.7% (a case of myocardial infarction). The morbidity rate was 9% (four patients), but no postoperative complications occurred after laparoscopic splenectomy. A significant statistical difference was shown by the increase in platelet counts after open versus laparoscopic splenectomy. The open and laparoscopic mean operative times (73.70 +/- 13.42 minutes and 78.42 +/- 14.63 minutes, respectively) were comparable. These times were comparable also considering patients who underwent only splenectomy. Mean recovery time was shorter after laparoscopic splenectomy (3.95 +/- 0.60 days) than after open splenectomy (7.0 +/- 1.68 days). After open procedures, however, the mean recovery time was shorter in uncomplicated cases (6.68 +/- 1.49 days) than in the open group as a whole. Authors conclude that many well-known advantages of the laparoscopic approach. especially those related to its low invasiveness, can be observed in patients requesting splenectomy for nontraumatic diseases of the spleen, without lowering the efficacy of this operation. They suggest that such advantages can be entirely displayed when selection criteria of the patients are applied.

Adult↗

Abdominal wound tumor recurrence after open and laparoscopic-assisted splenectomy in a murine model.

PURPOSE: The cause of abdominal wall tumor recurrences after laparoscopic surgery for cancer remains unknown. A recent study from our laboratory using a murine splenic tumor model suggests that poor surgical technique (i.e., crushing of the tumor) and not the CO2 pneumoperitoneum is responsible for port wound tumors. However, in that experiment no actual laparoscopic procedure or manipulation was performed. The purpose of the current study was to determine the rate of abdominal wound tumors after laparoscopic-assisted splenectomy performed via a CO2 pneumoperitoneum vs. open splenectomy using the mouse splenic tumor model. METHODS: To establish splenic tumors, female BALB/c mice (N=72) were given subcapsular splenic injections of a 0.1-ml suspension containing 10(5) C-26 colon adenocarcinoma cells via a left flank incision at the initial procedure. Eight days later, animals were randomized into one of two groups: 1) laparoscopic-assisted splenectomy, or 2) open splenectomy. Laparoscopic-assisted splenectomy animals had three laparoscopic ports placed and then underwent laparoscopic mobilization of the spleen under a CO2 pneumoperitoneum followed by extracorporeal splenectomy via a subcostal incision. Group 2 animals underwent open splenectomy via a subcostal incision after three port incisions were made in the same locations as for laparoscopic-assisted splenectomy mice. The incision was closed after 20 minutes in both groups. Ten days later, the mice were killed and inspected for abdominal wall tumor implants. The experiment was performed via two separate trials. RESULTS: When results of the two trials were combined, there was no significant difference in the incidence of animals in each group with at least 1 port tumor (open, 21 percent; laparoscopic-assisted splenectomy, 33 percent; P=0.14). However, the overall incidence of port site tumors (number of ports with tumors/total number of ports for each group) was significantly higher in the laparoscopic-assisted splenectomy group than in the open group (20 vs. 7 percent; P=0.01). The subcostal incisional tumor recurrence rate was also higher in the laparoscopic-assisted splenectomy group (50 vs. 21 percent; P=0.02). as was the perioperative mortality rate (21 vs. 7 percent; P=0.08). Results of the two individual trials were also considered separately. The incidence of port wound tumors decreased significantly from the first to the second laparoscopic-assisted splenectomy trial (36 vs. 9 percent; P=0.003), although the incidence of tumors at the subcostal incision and the mortality rate for the two laparoscopic-assisted splenectomy group trials were not significantly different. The open group tumor incidences did not change from trial to trial. CONCLUSIONS: Overall, significantly more port and incisional tumors were noted in the laparoscopic-assisted group. Although not statistically significant, mortality rate of the laparoscopic-assisted group was higher than the open group. The reasons for these findings are unclear. Laparoscopic mobilization was quite difficult and required excessive splenic manipulation, which may have liberated tumor cells from the primary tumor and facilitated port tumor formation. With increased experience, less manipulation was required to complete mobilization. Of note, the incidence of port tumors in the laparoscopic-assisted splenectomy group decreased significantly from the first to the second trials; therefore, it is possible that surgical technique is a factor in port tumor formation. However, the persistently high tumor incidence at the subcostal incision site argues against the hypothesis that the second trial's laparoscopic mobilizations were less traumatic. The CO2 pneumoperitoneum may also be a factor. Further studies are warranted to clarify these issues.

Abdominal Neoplasms↗

Partial splenectomy before a hematopoietic stem cell transplantation in children.

UNLABELLED: Hematopoietic stem cell (HSC) engraftment is delayed in children with hypersplenism, and splenectomy may improve HSC engraftment. However, the use of total splenectomy in children is limited because of concerns for postsplenectomy sepsis. In this study, the authors sought to assess the role of partial splenectomy for children with hypersplenism undergoing HSC transplantation. METHODS: Five children with a variety of conditions and associated hypersplenism underwent partial splenectomy before an HSC transplantation at the authors' institution between 2000 and 2003. Primary outcome measures were rates of neutrophil and platelet engraftment. Secondary outcome measures included perioperative complications, splenic regrowth, graft-versus-host disease, and infection rate. All outcomes were compared with recipients of an HSC transplant from both age-matched nonsplenectomized children (n = 497) and hypersplenic children who underwent total splenectomy (n = 10). Outcomes were compared using Wilcoxon's rank sum test. RESULTS: The rate of both neutrophil and platelet engraftment was faster in children who underwent either partial or total splenectomy as compared with nonsplenectomized children (mean rates of neutrophil engraftment were 26, 19, and 19 days for the nonsplenectomy, total splenectomy, and partial splenectomy groups, respectively; mean rates of platelet engraftment were 97, 37, and 45 days for the nonsplenectomy, total splenectomy, and partial splenectomy groups, respectively). Graft-versus-host disease rates were similar between the 3 groups. The mean percentage of splenic regrowth after partial splenectomy was 39%. There were no perioperative complications. CONCLUSIONS: Partial splenectomy may be safely performed before HSC transplantation and, similar to total splenectomy, may improve the rate of HSC engraftment. Although this series has a limited number of patients, the use of partial splenectomy appears to be safe and may allow for splenic salvage to minimize the risk of postsplenectomy sepsis.

Child↗