Partial splenectomy prevents splenic sequestration crises in sickle cell disease

doi:10.1016/j.jpedsurg.2009.06.007

Journal of Pediatric Surgery

Volume 44, Issue 11, November 2009, Pages 2088-2091

https://doi.org/10.1016/j.jpedsurg.2009.06.007 Get rights and content

Abstract

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.

Section snippets

Methods

After obtaining institutional review board approval, the records from January 2004 to December 2008 of all patients who underwent an open partial splenectomy for recurrent SS were retrospectively reviewed. Six patients met inclusion criteria. Five patients were treated at the University of Mississippi Medical Center (Jackson, Miss), with the sixth patient being treated at the University of Florida (Gainesville, Fla). Two board-certified pediatric surgeons performed the procedures. Patients were

Results

The mean age of patients undergoing partial splenectomy was 3 years (Table 1). Patient follow-up averaged 47 months with a range of 16 to 60 months. The average number of SS preoperatively was 2.1 ± 1.0 events per year per patient (range, 1-3) and 3.5 ± 1.4 total sequestrations per patient (range, 2-6). At splenectomy, an average of 80% of the spleen was removed (range, 75%-90%) starting at the lower pole. The main splenic artery and vein were left intact, whereas the individual vessels to the

Discussion

Sickle cell anemia is a hemoglobinopathy that occurs when both β-globin genes have the sickle cell mutation (Hb S), also referred to as homozygous SS[5]. Sickle cell disease encompasses patients not only with sickle cell anemia but also those that are compound heterozygotes where one β-globin gene mutation includes the sickle cell mutation, with the second β-globin mutation being one other than the sickle cell mutation, such as mutations associated with Hb C, Hb β-thalassemia, Hb D, and Hb O

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Cited by (13)

Splenic function is not maintained long-term after partial splenectomy in children with sickle cell disease
2020, Journal of Pediatric Surgery
Citation Excerpt :
In children with SCD, there was no significant change in hemoglobin at 42 months follow-up from a preoperative pretransfusion baseline mean of 11.8 ± 1.4 g/dl to a postoperative mean of 11.4 ± 1.9 g/dl, p = 0.5, or in reticulocyte counts from a preoperative mean of 3.9 ± 0.9% to a postoperative mean of 2.9 ± 1.1%, p = 0.09. Partial splenectomy has the theoretical advantage of reducing the threat of overwhelming septicemia by maintaining some immunocompetence (splenic function) compared to total splenectomy [3, 4, 6, 7]. Partial splenectomy continues to be performed in pediatric sickle cell disease patients because only short-term outcomes have been evaluated.
Partial splenectomy (PS) may allow preservation of splenic function in cases where splenectomy is indicated for hematologic diseases; however, the long-term outcomes are uncertain. We investigated the long-term outcomes of PS in patients with sickle cell disease (SCD).
A single-institution retrospective chart review was performed for children with SCD who underwent PS from 1997 to 2017. For comparison, we reviewed outcomes for patients who underwent PS for hereditary spherocytosis (HS). The primary endpoint was viability of the splenic remnant as inferred by the presence of remnant perfusion on ultrasound and/or liver spleen scan.
Nine patients with SCD and 26 patients with HS underwent PS at a median age of 11 (IQR, 9–14) and 7.5 (IQR, 6–13) years, respectively. All underwent laparoscopic PS with three (7.9%) conversions to open. Two SCD patients were lost to long-term follow-up. The remaining seven SCD patients had initial postoperative splenic remnant perfusion demonstrated by ultrasonography. By 42 months postoperatively, however, none had a functioning splenic remnant. The median time to loss of splenic remnant was 12.6 (IQR 9.2–28.5) months. In contrast, all HS patients demonstrated robust splenic remnant blood flow with a median follow-up of 46 (IQR 37–82) months.
No patient with SCD who underwent PS had viable splenic tissue for more than 42 months, likely due to continued autoinfarction typical of patients with this disease. Therefore, we believe that PS to preserve splenic function is not indicated in patients with SCD.
III.
Esplenectomia parcial en pacientes con drepanocitosis
2015, Anales de Pediatria
La esplenectomía total en la drepanocitosis se relaciona con riesgo de infecciones sobreagudas y con aumento de la incidencia de otros eventos, lo que no se ha comunicado en pacientes con esplenectomía parcial. En este estudio se caracterizó a los pacientes con drepanocitosis y esplenectomía parcial, y se comparó el comportamiento clínico y de laboratorio con los pacientes no esplenectomizados.
Se estudió a 54 pacientes con drepanocitosis sometidos a esplenectomía parcial durante la edad pediátrica, desde 1986 hasta el año 2011, en el Instituto de Hematología e Inmunología. Se compararon con 54 pacientes no esplenectomizados seleccionados por muestreo aleatorio con características similares.
La esplenectomía parcial se realizó a una edad media de 4,1 años, con una frecuencia mayor en la anemia drepanocítica (70,4%) y su causa más común fue la crisis de secuestro esplénico recurrente. Las complicaciones posoperatorias más frecuentes fueron: fiebre de origen desconocido (14,8%) y síndrome torácico agudo (11,1%). Después de la esplenectomía, aumentaron significativamente los leucocitos, neutrófilos y plaquetas; estos 2 últimos parámetros se mantuvieron elevados de manera significativa cuando se compararon con los pacientes no esplenectomizados. No hubo diferencias en la incidencia de los eventos clínicos, excepto el secuestro hepático, que fue más frecuente en los esplenectomizados.
La esplenectomía parcial constituyó un proceder seguro en los pacientes con drepanocitosis. No hubo diferencias en el cuadro clínico entre los niños esplenectomizados y los no esplenectomizados, salvo la mayor frecuencia de crisis de secuestro hepático en los primeros.
Total splenectomy in sickle cell disease is related to a high risk of fulminant sepsis and increased incidence of other events, which have not been reported in patients with partial splenectomy. In this study we examined the patients with sickle cell disease and partial splenectomy and compared the clinical and laboratory results with non-splenectomized patients.
We studied 54 patients with sickle cell disease who underwent partial splenectomy in childhood from 1986 until 2011 at the Institute of Hematology and Immunology. They were compared with 54 non-splenectomized patients selected by random sampling with similar characteristics.
Partial splenectomy was performed at a mean age of 4.1 years, with a higher frequency in homozygous hemoglobin S (70.4%), and the most common cause was recurrent splenic sequestration crisis. The most common postoperative complications were fever of unknown origin (14.8%) and acute chest syndrome (11.1%). After splenectomy there was a significant increase in leukocytes, neutrophils, and platelets, the latter two parameters remained significantly elevated when compared with non-splenectomized patients. There was no difference in the incidence of clinical events, except hepatic sequestration, which was more common in splenectomized patients.
Partial splenectomy was a safe procedure in patients with sickle cell disease. There were no differences in the clinical picture in children splenectomized and non-splenectomized except the greater frequency of hepatic sequestration crisis in the first group.
Comparative effectiveness of different types of splenectomy for children with congenital hemolytic anemias
2012, Journal of Pediatrics
Citation Excerpt :
Our primary search yielded 703 citations, with 141 abstracts identified as relevant. After secondary review, 93 studies were included in the final report.5-97 There were no randomized clinical trials.
To compare the effectiveness of different types of splenectomy in children with congenital hemolytic anemias.
We constructed key questions that addressed outcomes relevant to clinicians and families on effects of partial or total splenectomy, including hematologic effect, splenic function, and the risk of adverse events. We identified from Pubmed and Embase 703 studies that evaluated different types of splenectomy and accepted 93 studies that satisfied entry criteria. We graded the quality of each report and summarized the overall strength of research evidence for each key question.
We did not identify any randomized clinical trials. All types of splenectomy have favorable clinical outcomes in most diseases. We did not identify any hematologic advantage of laparoscopy compared with laparotomy. Adverse events are uncommon in most studies and are minimized with use of laparoscopy.
There is a need for randomized clinical trials and improved data collection of different types of splenectomy in congenital hemolytic anemias. Outcomes studied should address the concerns of families and clinicians to assess the risks and benefits of various treatments.
Partial Splenic Embolization in Paediatric Sickle Cell Disease Patients with Hypersplenism
2024, CardioVascular and Interventional Radiology
Relationship between genotype variants and the age of first acute splenic sequestration in patients with sickle cell disease in a tertiary center of Saudi Arabia: A retrospective study
2022, Journal of Applied Hematology
Sensitivity of alternative measures of functioning and wellbeing for adults with sickle cell disease: Comparison of PROMIS® to ASCQ-Me<sup>SM</sup>
2017, Health and Quality of Life Outcomes

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Original articlePartial splenectomy prevents splenic sequestration crises in sickle cell disease

Abstract

Purpose

Methods

Results

Conclusions

Section snippets

Methods

Results

Discussion

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Pediatr Clin North Am

Sickle cell anemia in the pediatric intensive care unit: novel approaches for managing life-threatening complications

AACN Clin Issues

Original article
Partial splenectomy prevents splenic sequestration crises in sickle cell disease