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.     

A laparoscopic approach to partial splenectomy for children with hereditary spherocytosis

Background Partial splenectomy is sometimes used for children with hereditary spherocytosis (HS) to reduce hemolysis while retaining some splenic immune function. Previous reports have described a partial splenic resection through a laparotomy incision. Whereas laparoscopic total splenectomy for HS is well-established, laparoscopic partial splenectomy (LPS) has not been described. The authors have developed a novel LPS technique that combines the benefits of partial splenectomy with those of a laparoscopic approach. Methods A chart review was conducted for three children with HS who underwent LPS, with approximately one-fourth of the spleen left on the basis of the short gastric arterial supply. Results The mean preoperative spleen size was 17.6 cm. The mean preoperative hemoglobin count was 100 g/l, and the postoperative hemoglobin count was 133 g/l. All three patients reported reduced malaise and increased energy levels. There was no recurrent anemia at the 1- to 2-year follow-up evaluation. Conclusion The LPS procedure is a safe and effective approach to HS that resolves anemia, potentially retains some splenic immunity, and confers the benefits of a minimal access technique.     

Laparoscopic Partial Splenectomy: Indications and results of a multicenter retrospective study

Introduction Partial splenectomy (PS) in children is a surgical option in haematological diseases and focal splenic tumours. The aim of this study was to describe the feasibility and the results of laparoscopic partial splenectomy in children in these two indications by a multicentric retrospective study. Methods The authors reviewed the files of all children who underwent laparoscopic PS between March 2002 and September 2006 in two paediatric surgical centers. The data of 11 children were collected and included clinical presentation, age, gender, radiographic examinations, surgical procedure, need for blood transfusion and early complications. Results From March 2002 to September 2006, laparoscopic PS had been performed on 11 children (6 boys, 5 girls) aged 23 months to 11 years (mean 7, 9). Four children had splenic focal tumours and seven had haematological diseases: six hereditary spherocytosis (HS) and one hemoglobinosis E. During the surgical procedure for haematological diseases 75–80% of the splenic tissue was removed. When PS was performed for focal splenic tumours, the splenic remnant was around 70%. No preoperative complications occurred (no bleeding, no diaphragmatic injury). Neither preoperative nor conversion was necessary. One postoperative complication occurred (left pleural effusion) but required no further treatment. The mean hospital stay was 7.7 days (range from 3 days to 10 days). No infectious postoperative complications occurred; the mean follow up was 21.1 months (range 3–52 months). Conclusion Laparoscopic partial splenectomy is feasible and safe in children with hypersplenism or focal splenic tumours. Partial splenectomy is a good way to prevent postsplenectomy infections by preservation of the immune role of spleen in children with haematological diseases. This technique performed for focal splenic tumours allows the surgeon to choose the size of the splenic remnant.     

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.     

Laparoscopic splenectomy in pediatric patients with hematologic diseases.

OBJECTIVE: The aim of this study was to evaluate our experience with laparoscopic splenectomy in pediatric patients with hematologic diseases. METHODS: A retrospective chart review was performed to analyze the following: indication for splenectomy, pre- and peri-operative management, surgical technique, complications, duration of hospitalization, and outcome. RESULTS: Eleven patients underwent laparoscopic splenectomy for the following indications: recurrent thrombocytopenia (<10,000) in seven with chronic immune thrombocytopenic purpura; anemia in two with hereditary spherocytosis; and hypersplenism in one and recurrent splenic sequestration in another with homozygous hemoglobin S. The six girls and five boys had a median age of 7 years. The median operative time was 180 minutes, and the median hospitalization was 72 hours. Among the patients with immune thrombocytopenic purpura (median platelet count, 153,000), those patients (n=3) with platelet counts of <100,000 did not suffer any bleeding complications. The patient with hypersplenism and homozygous hemoglobin S required a small incision in the left lower quadrant to facilitate removal of a 558-gram spleen. This patient also underwent cholecystectomy for cholelithiasis. The operative time was 295 minutes, and he was hospitalized for 5 days because of atelectasis. CONCLUSIONS: Laparoscopic splenectomy is a safe and effective procedure in children with hematological disorders.     

Partial splenectomy before a hematopoietic stem cell transplantation in children

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.     

The role of prophylactic cholecystectomy during splenectomy in children with hereditary spherocytosis.

BACKGROUND/PURPOSE: Hereditary spherocytosis is an autosomal dominant disorder associated with an intrinsic defect in the red blood cell membrane often necessitating splenectomy to prevent sequestration of spherocytes. When cholelithiasis is present, these patients undergo cholecystectomy at the same surgical setting as splenectomy. After splenectomy alone, it is uncertain whether the amount of hemolysis is adequately decreased to prevent subsequent gallstone formation. This study set out to evaluate the frequency in which symptomatic cholelithiasis subsequently develops in children treated by splenectomy alone. METHODS: All patients less than 18 years old with hereditary spherocytosis who underwent splenectomy without cholecystectomy at our institution during the past 27 years were included in this study. A retrospective chart review and telephonic patient follow-up was performed. Gallstones were excluded in these patients either by preoperative ultrasound scan, or by intraoperative palpation of the gallbladder. The main study outcomes of this group included documented cases of cholelithiasis, subsequent need for cholecystectomy secondary to cholelithiasis, and questionnaire to determine the incidence of "subclinical" cholelithiasis (not reported to a physician). RESULTS: Twenty-three subjects were identified who met the inclusion criteria. Complete follow-up data were obtained for 17 of these patients (74%). The mean age at splenectomy was 6.6 +/- 0.69 years, and the mean follow-up was 15.65 +/- 2.03 years (median, 18 years). None of the patients in this series subsequently have undergone cholecystectomy, nor have any had either clinical or subclinical evidence of cholelithiasis since splenectomy. CONCLUSION: Prophylactic cholecystectomy at the time of splenectomy is not indicated in patients with hereditary spherocytosis who do not have gallstones.     

Open heart operation in a patient with hereditary spherocytosis: a case report.

A 9-year-old girl who had ostium secundum atrial septal defect (ASD) and hereditary spherocytosis (HS) is described. The patient had a history of splenectomy for HS and underwent repair of the ASD under cardiopulmonary bypass (CPB), however, no significant or persistent hemolysis was observed during and after CPB. Only 10 patients with HS who underwent cardiac operations using CPB have been reported. The case is presented due to its rarity.     

Near-total splenectomy: a new technique for the management of hereditary spherocytosis

OBJECTIVE: The authors used a new surgical technique of near-total splenectomy (NTS) and report their experience. SUMMARY BACKGROUND DATA: Total splenectomy is indicated for the management of patients with hereditary spherocytosis but may be complicated by severe infections and thromboembolic events. Studies have shown that partial or subtotal parenchymal resections can lead to excessive regeneration of the residual parenchyma. The resulting onset of hemolysis requires total splenectomy in a significant portion of patients. Our hypothesis was that a more radical approach to open resection permanently decreases recurrent hemolysis while potentially ensuring immune function. METHODS: This longitudinal cohort study included 42 patients with moderate to severe hereditary spherocytosis who underwent NTS according to an open procedure developed by the authors. The end criterion was to conserve a remnant spleen of 10 cm in size. RESULTS: Patient age ranged between 2 and 42 years. Mean resected spleen weight was 580 g; mean remnant volume was 10 cm (range, 8-11 cm). A surgical complication (loss of spleen) occurred in 1 patient. Six-month to 6-year follow-up data was available on 22 patients; 21 of 22 showed preserved phagocytosis and normal blood circulation of the remnant; 1 of 22 experienced secondary remnant necrosis. On average, the remnant spleen grew back to four and a half times its postoperative size. No patients required transfusions, developed gallstones, or symptomatic hemolysis. CONCLUSIONS: This new technique of NTS is safe, effective, and can minimize the late sequelae of secondary splenectomy.     

腹腔镜下脾切除脾血管的解剖基础及临床应用

目的探讨腹腔镜下脾血管解剖的特点及临床应用价值。方法分析完全腹腔镜脾切除术(TLS)47例资料。其中肝硬化门静脉高压症21例,地中海贫血19例,原发性血小板减少性紫癜2例,球形红细胞增多症2例,脾血管瘤1例,脾囊肿1例,原发性脾功能亢进1例。根据胰尾段内结扎脾动脉后,脾脏呈现颜色改变,总结脾血管腔镜下解剖分型。结果脾血管为Ⅰ型(分支型)者占72.3%(34/47);Ⅱ型(主干型)占19.1%(9/47);Ⅲ型(迷走型)占4.3%(2/47);腔镜下解剖不清2例(4.3%)。46例成功完成TLS,手术成功率97.9%(46/47),14例同时行腹腔镜下贲门周围血管离断术,3例地中海贫血因胆囊结石同时行腹腔镜下胆囊切除术;1例因凝血功能障碍术中广泛创面渗血而中转开腹行创面止血。43例经脾动脉结扎,45例经脾门血管分级离断技术得以完成手术。TLS手术时间50～240min,(110±35)min。出血量20～1500ml,(160±87)ml。结论腹腔镜下脾血管绝大部分以分支型为主,但可以在胰尾上缘结扎脾动脉阻断脾脏的大部分血供,脾门血管可以进行分级离断。脾动脉结扎、脾门血管分级离断是控制术中出血、防止胰腺损伤的有...     

Laparoscopic splenic procedures in children: experience in 231 children

OBJECTIVES: The purpose of this report is to evaluate the efficacy of and complications observed after laparoscopic splenic procedures in children. METHODS: Review of a prospective database at a single institution (1995-2006) identified 231 children (129 boys; 102 girls; average age 7.69 years) undergoing laparoscopic splenic procedures. RESULTS: Two hundred twenty-three children underwent laparoscopic splenectomy (211 total; 12 partial) by the lateral approach. Indication for splenectomy was hereditary spherocytosis (111), immune thrombocytopenic purpura (36), sickle cell disease (SCD) (51), and other (25). Four (2%) required conversion to an open procedure. Eight additional laparoscopic splenic procedures were performed: splenic cystectomy for epithelial (4) or traumatic (2) cyst, and splenopexy for wandering spleen (2). Average length of stay was 1.5 days. Complications (11% overall, 22% in SCD patients) included ileus (5), bleeding (4), acute chest syndrome (5), pneumonia (2), portal vein thrombosis (1), priapism (1), hemolytic uremic syndrome (1), diaphragm perforation (2), colonic injury (1), missed accessory spleen (1), trocar site hernia (1), subsequent total splenectomy after an initial partial (1), and recurrent cyst (1). Subsequent operations were open in 3 (colon repair, hernia, and missed accessory spleen) and laparoscopic in 2 (completion splenectomy, and cyst excision). There were no deaths, wound infections, or instances of pancreatitis. CONCLUSIONS: Laparoscopic splenic procedures are safe and effective in children and are associated with low morbidity, higher complication rate in SCD, low conversion rate, zero mortality, and short length of stay. Laparoscopic splenectomy has become the procedure of choice for most children requiring a splenic procedure.     

Laparoscopic subtotal splenectomy in hereditary spherocytosis

BACKGROUND: Clinical manifestations of hereditary spherocytosis can be controlled by splenectomy. The use of this procedure has been restricted due to concerns regarding exposure of patients to a lifelong risk of overwhelming infections. Subtotal splenectomy, which removes 85-90% of the enlarged spleen, is a logical alternative. In the first cases performed by laparoscopy we have chosen to preserve the upper pole. However, this technique showed some disadvantages, especially concerning the correct intraoperative evaluation of the splenic remnant volume. Therefore, we developed a new variant of the procedure by preserving the lower pole of the spleen. METHODS: Based on the authors' experience in laparoscopy (176 laparoscopic splenectomies), 10 laparoscopic subtotal splenectomies were performed in patients with hereditary microspherocytosis, preserving either the upper or the lower splenic pole. RESULTS: Patient age ranged between 5 and 35 years. The mean volume of the remnant spleen was 41.4 cm3. There were no complications, and no transfusions were needed. Follow-up for 1-30 months was available. CONCLUSIONS: Subtotal splenectomy appears to control hemolysis while maintaining splenic function. The laparoscopic approach is safe and effective and should be considered the procedure of choice in hereditary microspherocytosis. Laparoscopic subtotal splenectomy presents an advantage over open subtotal splenectomy, resulting in decreased blood loss, shorter hospital stay, no conversions, fewer operative and postoperative complications, and excellent remission rates. On the basis of our experience, the preservation of the lower pole of the spleen seems to be a first-line option for the optimal evaluation of the residual splenic mass.     

Mitral and aortic valve replacement with tricuspid annuloplasty in a patient suffering from hereditary spherocytosis

A 51-year-old woman was admitted with increasing shortness of breath. Preoperative evaluation disclosed MSr + ASr + TR. She had been undergone a splenectomy at 35-year-old for hereditary spherocytosis (HS). The patient underwent successful mitral valve replacement with a No. 25 Bj?rk-Shiley (BS) valve, aortic valve replacement with a No. 21 BS valve, and tricuspid valve annuloplasty by means of membrane oxygenator (COBE). Haptoglobin and poloxamer 188 were used for prevention of hemolysis during extracorporeal circulation. No significant hemolysis occurred intra-or postoperatively. A very rare case of open heart surgery associated with HS was reported.     

Splenic infarction due to concomitant hereditary spherocytosis and sickle cell trait

Concomitant hereditary spherocytosis and sickle cell trait, although extremely rare, could potentially lead to splenic sequestration or infarction. We report here the first case of splenic infarction in a child with hereditary spherocytosis and sickle cell trait while flying on a commercial aircraft. The presence of hypoxia, hemoconcentrated erythrocytes, and sickle hemoglobin created the perfect environment for clinical sequelae.     

Laparoscopic subtotal splenectomy in the treatment of hereditary spherocytosis

Clinical manifestations of hereditary spherocytosis, the most common red blood cell membrane disorder, can be controlled by splenectomy. However, concerns regarding exposure of patients to a life long risk for overwhelming infections have restricted its use, especially ?n children. Subtotal splenectomy, as long as 80% to 90% of the enlarged spleen is removed, is a logical alternative. Subtotal splenectomy was effective ?n decreasing the hemolytic rate, while maintaining the phagocytic and immune function of the spleen. This surgical procedure should be considered ?n transfusion-dependent infants and children whit hereditary spherocytosis and ?n older patients whit erythrocyte membrane defects. Based on our experience ?n laparoscopy (120 laparoscopic splenectomies) and open subtotal splenectomy (5 cases) we performed 2 laparoscopic subtotal splenectomies in patients with hereditary microspherocytosis with good short term results. We have had no problem with blood loss and no transfusions were needed. The procedure can be performed safely and easily with all the traditional advantages of a minimally invasive approach. In order to evaluate the long term clinical benefit a minimal follow-up of 5 years is needed.     

Synchronous splenectomy during cholecystectomy for hereditary spherocytosis: Is it really necessary?

Background/Purpose

Expert guidelines recommend performing synchronous splenectomy in patients with mild hereditary spherocytosis (HS) and symptoms of gallstone disease. This recommendation has not been widely explored in the literature. The aim of this study is to determine if our data support expert opinion and if different practice patterns should exist.

Methods

This is an IRB-approved retrospective study. All HS patients under 18 years of age who underwent cholecystectomy for symptomatic gallstones at a single institution between 1981 and 2009 were identified. Patients who underwent cholecystectomy without concurrent splenectomy were reviewed retrospectively for future need for splenectomy and evidence of recurrent gallstone disease.

Results

Of the 32 patients identified, 27 underwent synchronous splenectomy. The remaining 5 patients underwent cholecystectomy without splenectomy and had a mean age of 9.4 years. One of the 5 patients eventually required splenectomy for left upper quadrant pain. None of the remaining 4 required hospitalization for symptoms related to hemolysis or hepatobiliary disease. Median follow-up is 15.6 years.

Conclusion

The need for splenectomy in patients with mild HS and symptomatic cholelithiasis should be assessed on a case by case basis. Our recommendation is to not perform synchronous splenectomy in conjunction with cholecystectomy for these patients if no indication for splenectomy exists.     

Partial splenectomy for Gaucher''s disease.

Gaucher's disease is an autosomal recessive disorder caused by deficiency of beta glucocerebrosidase, resulting in an accumulation of glucocerebroside in the reticuloendothelial system. These patients have massive splenomegaly and bone pain, but may have normal life expectancy. Traditionally, splenectomy has been used to treat hypersplenism, but may be associated with a high incidence of postsplenectomy sepsis and accelerated hepatic and bone lipid deposition. Two children are reported who had partial splenectomy for symptoms of Gaucher's disease. Both patients had laboratory evidence of hypersplenism. A 90% splenectomy was performed, and the residual splenic fragment was wrapped in Vicryl mesh. Both patients are currently asymptomatic with normal hematologic parameters. Postoperative radionuclide scans demonstrate increase in the size of the residual splenic fragment. Partial splenectomy may benefit patients with Gaucher's disease, but long-term follow-up care is necessary.     

Is splenectomy for massive splenomegaly safe in children?

PURPOSE: To study and analyze the causes, etiology, morbidity, mortality and therapeutic value of splenectomy performed for massive splenomegaly in children. METHODS: The medical records of 115 children less than 18 years old who had splenectomy for various hematological disorders were reviewed. Twenty of them had splenectomy for massive splenomegaly (spleen weight > or =1,000 g). The records of these were reviewed for age at operation, gender, hematological diagnosis, indication for splenectomy, operative procedures, postoperative complications, and outcome. RESULTS: Twenty children had splenectomy for massive splenomegaly. There were 16 males and 4 females. Their ages ranged from 4 to 15 years (mean 11.2). Twelve had sickle cell disease, 5 had sickle-beta-thalassemia, 1 had beta-thalassemia major, 1 had thalassemia intermediate, and 1 had chronic myeloid leukemia. The indications for splenectomy were hypersplenism in 11, recurrent splenic sequestration crisis in 8, and splenic abscess in 1. The transfusion requirements in the patient with beta-thalassemia major decreased markedly postoperatively from 18 transfusions/year to only 4 transfusions/year; and for those with hypersplenism, there was a marked improvement in their blood parameters following splenectomy. The patient with thalassemia intermediate required no more blood transfusions. There was no mortality. The immediate postoperative morbidity was 10% for those with massive splenomegaly compared with 6.3% for those with splenomegaly <1,000 g. CONCLUSIONS: With good perioperative management, splenectomy in children with massive splenomegaly is both safe and effective.     

Laparoscopic partial vs total splenectomy in children with hereditary spherocytosis

Background

Open partial splenectomy provides reversal of anemia and relief of symptomatic splenomegaly while theoretically retaining splenic immune function for hereditary spherocytosis. We recently developed a laparoscopic approach for partial splenectomy. The purpose of the present study is to compare the outcomes in a group of patients undergoing laparoscopic partial splenectomy (LPS) with those in a group of children undergoing laparoscopic total splenectomy (LTS) over the same period.

Methods

Systematic chart review was conducted of all children with hereditary spherocytosis who had LTS or LPS from 2000 to 2006 at the Hospital for Sick Children, Toronto, Ontario, Canada. T tests were used for continuous data, and χ² for proportional data; P value of less than .05 was considered significant.

Results

There were 9 patients (14 males) in each group. Groups were similar in sex, age, concomitant cholecystectomy, and preoperative hospitalizations, transfusions, and spleen size. Estimated blood loss was greater in the LPS group (188 + 53 vs 67 + 17 mL; P = .02), but transfusion requirements were similar (1/9 vs 0/9). Complication rate was similar between groups. The LPS group had higher morphine use (4.1 + 0.6 vs 2.4 + 0.2 days; P = .03), greater time to oral intake (4.4 + 0.7 vs 2.0 + 0.2 days; P = .01), and longer hospital stay (6.3 + 1.0 vs 2.7 + 0.3 days; P = .005) than the LTS group. Nuclear scan 6 to 8 weeks postoperatively demonstrated residual perfused splenic tissue in all LPS patients. No completion splenectomy was necessary after a mean follow-up of 25 months.

Conclusion

These data suggest that LPS is as effective as LTS for control of symptoms. However, LPS is associated with more pain, longer time to oral intake, and longer hospital stay. These disadvantages may be balanced by retained splenic immune function, but further studies are required to assess long-term splenic function in these patients.