Clinical events after surgical splenectomy in children with sickle cell anemia

ArticleinPediatric Surgery International 26(5):495-500 · March 2010with10 Reads
DOI: 10.1007/s00383-010-2587-4 · Source: PubMed
Abstract
Despite advances in immune prophylaxis, sepsis remains the most feared complication following splenectomy for acute splenic sequestration crisis (ASSC) in children with sickle cell anemia (SCA). We seek to investigate the true prevalence of sepsis and other complications of splenectomy in this patient population. We reviewed the records of children with SCA (HbSS) who underwent splenectomy for ASSC between 1993 and 2008 at a single institution. Fifty-eight patients (33 males) at a median age of 2 years at splenectomy were included with an average post-splenectomy follow-up of 6.4 years (range 6 months-14 years). Thirty-seven patients (64%) underwent laparoscopic splenectomy, and acute chest syndrome (ACS) was the most common post-operative complication (6.9%). There was no difference in the incidence of sepsis pre- and post-splenectomy. The occurrence of vaso-occlusive pain crises (VOC) and ACS was significantly higher after splenectomy. In addition, 14 patients (24%) developed stroke (n = 5) or an abnormal transcranial Doppler (TCD) (n = 9) after splenectomy. Our data suggest that splenectomy can be safely performed in children with SCA given a low risk of sepsis. However, the increased incidence of VOC, ACS, and stroke or abnormal TCDs after splenectomy remains a concern.
    • "Removal of the spleen for major splenic injury at young age results in reduced bacterial phagocytosis and a diminished antigen response, resulting in a high risk of sepsis [6]. Younger children are at an increased risk of overwhelming post-splenectomy infection, which has a morbidity rate 5% and historical mortality rate of 25% [7]. The safety of NOM has been validated in many studies, with only very rare complications such as delayed rupture, abscess formation, pseudoaneurysm (PA) formation, and ruptured PA. "
    Article · Jan 2016
    • "This increased prevalence was not explained by known determinants of bone pain or chest syndrome, such as high haemoglobin or low HbF, raising the hypothesis that splenic complications, such as acute splenic sequestration, may be a predictive factor of disease severity. A higher incidence of severe complications in the pre-versus post-splenectomy period was also reported (Kalpatthi et al, 2010) in 58 children splenectomized at a median age of 2 years. However, considering the young age of these patients, a serious bias may well be the expected increasing incidence of these complications with age. "
    [Show abstract] [Hide abstract] ABSTRACT: The spleen has a combined function of immune defence and quality control of senescent or altered red cells. It is the first organ injured in sickle cell anaemia (SCA) with evidence of hyposplenism present before 12 months in the majority of children. Repeated splenic vaso-occlusion leads to fibrosis and progressive atrophy of the organ (autosplenectomy), which is generally complete by 5 years in SCA. The precise sequence of pathogenic events leading to hyposplenism is unknown. Splenic injury is generally silent and progressive. It can be clinically overt with acute splenic sequestration of red cells, an unpredictable and life-threatening complication in infants. Splenomegaly, with or without hypersplenism, can also occur and can coexist with loss of function. Hyposplenism increases the susceptibility of SCA children to infection with encapsulated bacteria, which is notably reduced by penicillin prophylaxis and immunization. Whether hyposplenism indirectly increases the risk of vaso-occlusion or other circulatory complications remains to be determined.
    Article · May 2014
    • "In children with sickle-cell crisis, splenectomy may be considered after the crisis has been abated by transfusion and medical treatment. The immediate risks seem similar to those associated with other indications for benign haematological disorders, but the long-term outcome is burdened by many vascular complications (Kokoska et al, 2004; Ghantous et al, 2008; Kalpatthi et al, 2010). Advice from expert centres is recommended. "
    [Show abstract] [Hide abstract] ABSTRACT: Splenectomy has represented a key treatment option in the treatment of many benign haematological diseases, including immune thrombocytopenia (ITP) and disorders associated with ongoing haemolysis (thalassaemia major and intermedia, sickle cell disease, and hereditary or acquired haemolytic anaemias). Improvements in surgical techniques have reduced perioperative complications and mortality. Preventive measures (new protein conjugate vaccines, antibiotic prophylaxis, and increased vigilance) are thought to greatly reduce the risk of overwhelming post-splenectomy infection (OPSI), although their implementation is inconsistent. Nevertheless, there is increasing documentation of the short- and long-term risks of splenectomy, which vary according to the underlying indication. Splenectomized patients are at increased risk of venous thromboembolism, particularly within the splenoportal system. The long-term thromboembolic risk is higher in haematological disorders associated with ongoing haemolysis, particularly in thalassaemia intermedia, which has led to a more conservative approach. In comparison, patients with ITP appear to be at lower risk of adverse effects of splenectomy, which maintains its place as the potentially most curative and safe second-line treatment. However, a splenectomy-sparing approach is also emerging for ITP, and recent guidelines recommend that this procedure is deferred until ≥ 12 months from ITP diagnosis, to allow sufficient time for possible remission.
    Article · May 2012
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