Should cholecystectomy be performed concomitantly with splenectomy in children with sickle-cell disease?

Splenectomy and cholecystectomy are among the common surgical procedures required to treat complications of sickle-cell disease (SCD), and when performed separately have been shown to be safe and effective. To determine whether cholecystectomy be performed concomitantly with splenectomy (CSC) in these children, we studied a total of 130 children who underwent splenectomy for various hematologic diseases at our hospital. The most common indication was SCD. Ninety-nine patients (86 SCD and 13 sickle-B-thalassemia) underwent splenectomy and 19 (19.2%) (12 males and 7 females, mean age 13.4 years [7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18]) underwent CSC due to concomitant gallstones, which were asymptomatic in 13 cases. Those with an admission hemoglobin (Hb) of less than 10 g/dl were transfused with packed erythrocytes to increase their Hb to 10-12 g/dl and their hematocrit to 30%-40%. All patients received intravenous hydration the night before surgery at a rate of 1(1/2) times their maintenance rate, which was continued postoperatively until they were able to tolerate adequate oral fluid intake. The indications for splenectomy in those who had CSC were: acute splenic sequestration crisis in 12, splenic abscess in 3, hypersplenism in 2, and massive splenic infarction in 2. Eight patients had massive splenomegaly (spleen weight >1 kg). In 7 cases CSC was done through a left-upper-quadrant (LUQ) transverse incision, in the remaining 12 through an upper midline incision. There was no mortality and only 2 patients developed postoperative complications; a wound infection in 1 and a hematoma in the splenic bed in another. With good perioperative management, CSC is both safe and effective for children with SCD. Both procedures can be performed safely through an upper midline or a LUQ transverse incision, even in the presence of massive splenomegaly.     

Splenectomy and acute splenic sequestration crises in sickle cell disease

Acute splenic sequestration crises (ASSC) is one of the complications of sickle cell disease (SCD) that can be life-threatening due to loss of blood volume. Over a 5-year period, we have treated 19 patients ranging in age from 4 to 32 years with ASSC. There were 14 males and 5 females; 17 had homozygous SCD and the other 2 had sickle thalassemia. Two patients presented with severe anemia and acute circulatory collapse; 1 of them developed residual weakness of his limbs and decreased visual acuity. Nine patients underwent splenectomy after major episodes of sequestration while the remaining 10 had recurrent minor episodes of sequestration. The clinical features and the role of splenectomy are discussed.     

Acute splenic sequestration crises in Saudi children with sickle cell disease

Sickle cell disease (SCD) is a common disease in the Eastern Province of Saudi Arabia. Twenty per cent of the population have sickle cell trait and 1.75% have SCD. In the first 2 years of life, infection, hand-foot syndrome and acute splenic sequestration crisis (ASSC) are the most common complications of this disease. The classical characteristics of an ASSC, the subject of this paper, are sudden and rapid enlargement of the spleen, secondary to the massive pooling of red blood cells in the splenic sinusoids in a functioning spleen. Less common minor attacks of ASSC have been described recently. An ASSC is one of the most common causes of death in infants with SCD. The underlying cause and the precipitants of attacks of ASSC remain unknown. Seventeen children with ASSC were seen. Clinically, they had minor ASSC; three developed hypersplenism and four underwent splenectomy for recurrent attacks of ASSC. None of these 17 children had the classical ASSC described in black children where peripheral circulatory shock is encountered.     

Simultaneous acute splenic sequestration and transient aplastic crisis in children with sickle cell disease

Acute splenic sequestration crisis (ASSC) is a hematological emergency in young children with sickle cell disease (SCD), characterized by worsening anemia and splenomegaly, usually with reticulocytosis and thrombocytopenia. Transient aplastic crisis (TAC) due to parvovirus B19 infection occurs in older children with SCD, and typically manifests as worsening anemia with reticulocytopenia and no splenomegaly. Five older children with SCD (4 HbSC, 1 HbSS on hydroxyurea) developed ASSC concurrent with TAC and had a severe clinical course. Our cases suggest that older children with SCD and acute parvovirus infection should be monitored closely for splenomegaly and multi‐system dysfunction. Pediatr Blood Cancer 2009;53:479–481. © 2009 Wiley‐Liss, Inc.     

Postsplenectomy course in homozygous sickle cell disease

OBJECTIVE: To determine whether children with homozygous sickle cell (SS) disease and splenectomy are at greater risk of death, overwhelming septicemia, or other complications. METHODS: A total of 130 patients with SS treated by splenectomy (46 recurrent acute splenic sequestration, 84 chronic hypersplenism) over a 22.5-year period at the Sickle Cell Clinic of the University Hospital of the West Indies, Kingston, Jamaica, were compared with a control group matched for sex, age, and duration of follow-up in a retrospective review. Deaths and bacteremias were examined over the whole study period. Painful crises, acute chest syndromes, and febrile episodes were compared in the 90 patients completing 5 years of postsplenectomy follow-up. FINDINGS: Mortality and bacteremic episodes did not differ between the splenectomy and control groups. Painful crises were more common in the splenectomy group than in the control group (P =.01) but did not differ between splenectomy indications. Acute chest syndrome was more common in the splenectomy group than in the control group (P <.01) and was more common in the acute splenic sequestration group than in the hypersplenism group (P =.01). Febrile events did not differ between the groups or between the indications for splenectomy. CONCLUSION: Splenectomy does not increase the risk of death or bacteremic illness in patients with SS disease and, if otherwise indicated, should not be deferred for these reasons.     

Clinical events after surgical splenectomy in children with sickle cell anemia

Purpose  

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.     

Life threatening parvovirus B19 and herpes simplex virus associated acute myocardial dysfunction in a child with homozygous sickle cell disease

Human parvovirus (HPV) B19, a common infection, frequently causes transient red cell aplasia in children with hemolytic anemia, such as sickle cell disease (SCD). It was considered to be a self-limited condition, easily treated with blood transfusion. However, acute splenic sequestration, acute chest syndrome, nephrotic syndrome, and stroke have been reported in SCD patients following HPV B19 infection. We report a 3-year-old child with SCD who developed fulminant myocarditis following HPV B19-related aplastic crisis. The diagnosis of myocarditis should be considered in a patient with hemolytic anemia with an infection with HPV B19 who develops signs of cardiopulmonary failure despite correction of anemia.     

Acute complications in children with sickle cell disease: Prevention and management

Sickle cell disease (SCD) is a chronic, multi-system disease that requires comprehensive care. The sickling of red blood cells leads to hemolysis and vascular occlusion. Complications include hemolytic anemia, pain syndromes, and organ damage. Patterns of immigration and an increase in newborn screening mean that paediatric health care providers across Canada, in small and large centres alike, need to be knowledgeable about SCD. This statement focuses on principles of prevention, advocacy, and the rapid treatment of common acute complications. Guidance includes the current status of newborn screening, recommendations for immunizations and antibiotic prophylaxis, and an introduction to hydroxyurea, a medication that reduces both morbidity and mortality in children with SCD. Case vignettes demonstrate principles of care for common acute complications of SCD: vaso-occlusive episodes (VOE), acute chest syndrome (ACS), fever, splenic sequestration, aplastic crises, and stroke. Finally, principles of blood transfusion are highlighted, along with indications for both straight and exchange blood transfusions.     

Laparoscopic splenectomy and/or cholecystectomy for children with sickle cell disease

Background  In 1991, Delaitre reported the first laparoscopic splenectomy (LS). Since then LS has become the procedure of choice to treat hematological diseases requiring splenectomy. The Eastern province of Saudi Arabia is known to have a high incidence of hemoglobinopathies including sickle cell disease (SCD), which is known to be associated with complications necessitating splenectomy and/or cholecystectomy. This report describes our experience with LS and/or laparoscopic cholecystectomy (LC) for children with SCD. Patients and methods  The medical records of all children with SCD who had LS and/or LC were retrospectively reviewed for age, sex, indication for splenectomy, operative time, hospital stay, and post-operative complications. The results were compared to a similar group of children with SCD who had open splenectomy (OS) and/or open cholecystectomy (OC). Results  Over a period of 3.5 years (January 2005 and June 2008), a total of 45 children had LS with or without LC, 30 (66.7%) of them had SCD. Their age ranged from 2 to 12 years (mean 7 years). There were 16 males and 14 females. In all, LS was done because of recurrent splenic sequestration crisis except one who had a large spleen with multiple infarcts that was causing abdominal pain. The operative time ranged from 1.5 to 9 h (mean 2.75 h). Their hospital stay ranged from 3 to 9 days (mean 4.5 days). There was no mortality. Two patients (6.7%) required conversion to OS due to a large-sized spleen and severe adhesions in one and uncontrolled intra-operative bleeding in the other. The results were compared to a group of 120 children with SCD who had OS only (88) and OS with OC (32). From 1994 to 2006, a total of 55 children had LC only, 47 (26 M:21 F) of them (85.5%) had SCD. Their age ranged from 4 to 15 years (mean 11.4 years). The indications for cholecystectomy were: biliary dyspepsia (20), biliary colic (35), acute cholecystitis (5), obstructive jaundice (5), asymptomatic (6), and biliary pancreatitis (1). There was no mortality, but one (2.1%) required conversion to OC because of severe adhesions and another underwent postoperative exploration because of bleeding from an accessory cystic artery. The results were compared to a similar group of 27 children with SCD who underwent OC. Conclusions  With good peri-operative management, LS is feasible and safe in children with SCD and can be done concomitantly with cholecystectomy. Currently, it requires more operative time than the open approach. This is specially so for children with SCD who are known to have a large spleen with severe adhesions. It is, however, superior to OS with regard to duration of hospital stay, cosmetic appearance, post-operative complications, and post-operative recovery. LC is also safe in children with SCD. When compared with OC, it is associated with less post-operative complications, a shorter hospital stay, better cosmetic appearance and a faster recovery.     

Fetal haemoglobin and early manifestations of homozygous sickle cell disease.

The relevance of fetal haemoglobin (HbF) concentration to the development of early clinical manifestations of homozygous sickle (SS) disease has been investigated by examining the time to first occurrence and the proportional hazard of these complications in three groups of the HbF distribution at age 5 years. HbF was significantly related to dactylitis, painful crises, acute chest syndrome, and acute splenic sequestration. The relationship suggested that a critically low HbF concentration increased the risk, little difference in risk occurring between the medium and high HbF groups. The abdominal painful crisis and hypersplenism were not related to HbF concentration suggesting that the degree of sickling may not be important in their genesis. Parental education on acute splenic sequestration should be focused on children with HbF concentrations in the lowest part of the HbF distribution for age.     

Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series

The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non‐SCD cause of hepatopathy in a patient with viral hepatitis. These complications require a systematic approach to extensive evaluation and coordinated multidisciplinary care.     

Acute splenic sequestration in female children with sickle cell disease in the North of Jordan

The objective of this study was to evaluate the rate of acute splenic sequestration (ASSC) in patients with sickle beta-thalassaemia and sickle cell anaemia, the risk of recurrence in those who survive the first episode, and the relationship between ASSC episodes and subsequent hypersplenism. All patients with confirmed diagnosis of sickle cell disease at a tertiary referral teaching hospital, between January 1994 and December 2002 were interviewed and had their medical records reviewed. Seventy-seven patients with sickle cell disease were identified. Their ages ranged between 2 and 18 years (mean, 10.1 years). There were 35 females and 38 males. Thirty-seven (50.6%) had sickle beta-thalassaemia, and 36 (49.4%) had homozygous sickle cell anaemia. Of these, 26 had high level of Hb F and 11 had normal level of fetal haemoglobin (Hb F). Twenty-one patients (28%) had 63 episodes of acute splenic sequestration. Thirty-seven episodes were experienced by 12 patients with sickle beta-thalassaemia; of these 11 were major attacks with one fatality. Twenty-six episodes were experienced by nine patients with sickle cell anaemia. Splenomegaly and hypersplenism were greater in the acute splenic sequestration group than in the rest of the sickle cell anaemia patients, and the differences were extremely significant. ASSC was found in nine siblings of sickle beta-thalassaemia group, while none were found in the sickle cell anaemia group. The mean age of the first episode was significantly higher in sickle beta-thalassaemia, with significant differences in the levels of Hb F, Hb S, size of spleen and severity of crisis between both groups. In the sickle cell anaemia group the only significant difference between patients with and these without acute splenic sequestration was the difference in the size of spleen. In this study, the rate of ASSC in the sickle beta-thalassaemia patients was 32%, in contrast to 25% in the sickle cell anaemia patients. The risk of recurrence was about 70% in those who survived their first episodes. There was a close relationship between ASSC and subsequent hypersplenism. Important predictable factors for ASSC in sickle beta-thalassaemia patients were the presence of splenomegaly of more than 5 cm below the costal margin, history of acute splenic sequestration in siblings and high Hb F. Most of first episodes in sickle cell anaemia occur under the age of 2 years, while in sickle beta-thalassaemia the majority of patients have their first crisis at the age of > or =3.5 years.     

The spleen in the sickling disorders: an update

In early life, patients with sickle cell disease (SCD) can have acute, life-threatening emergencies related to splenic hypofunction (overwhelming bacterial sepsis), as well as anemic crises from acute splenic sequestration because of sudden pooling of blood in the spleen. The landmark penicillin prophylaxis study in 1985 showed a remarkable decrease in mortality from sepsis in young children with SCD who were treated with oral penicillin prophylaxis compared to placebo. Since that study, newborns are screened for SCD and placed on oral penicillin prophylaxis in nearly all of the United States, as well as in other countries where the disease is highly prevalent. The previously described permanent, complete and nearly universal “autosplenectomy” emerging by late childhood or early adulthood is now challenged by recent findings of reversibility of splenic dysfunction by the antisickling drug hydroxyurea or by successful allogeneic stem cell transplantation, even in older patients. Imaging techniques for hypofunction of the spleen are the most commonly used modalities to guide the clinician in decisions regarding medical or surgical management.     

Acute splenic rupture in an adult with homozygous sickle cell anemia treated with chronic transfusions

Splenic rupture is a very rare event in adult homozygous sickle cell patients. The authors describe a 19-year-old patient with homozygous sickle cell disease who experienced an acute splenic rupture crisis requiring emergent splenectomy. He had been receiving chronic blood transfusions regularly for 7 years secondary to a previous stroke. It is possible that these transfusions contributed to regeneration of splenic red pulp, which allowed a crisis to occur at an advanced age.     

Myocardial necrosis following general anesthesia in hemoglobin SC disease.

A 4-year-old girl with hemoglobin SC disease died following general anesthesia. Autopsy showed widespread intravascular sickling; staining with hematoxylin-basic fuchsin-picric acid demonstrated newly developed massive myocardial necrosis, a rarely documented finding. Anesthesia may produce conditions that provoke "crises" in patients with sickle hemoglobinopathies. Preoperative identification of sickle states and careful attention to hydration and oxygenation may minimize anesthetic risks in these patients.     

Resolution of chronic hepatic sequestration in a patient with homozygous sickle cell disease receiving hydroxyurea

Hepatic sequestration is an uncommon complication in patients with homozygous sickle cell disease. Although transfusion therapy has been effective for the acute condition, no definitive treatment of chronic hepatic sequestration has been identified. We describe a 17-year-old male patient with hemoglobin SS and chronic hepatic sequestration who was treated with long-term (60 months) hydroxyurea. After 36 months of HU therapy, the patient had both an excellent hematologic response and a resolution of hepatic sequestration, as evidenced by disappearance of clinical hepatomegaly, normalization of liver volume on serial computed tomography scans, as well as decreased sinusoidal dilatation and congestion and red blood cell sickling on liver biopsy. The findings in this case suggest that hydroxyurea may benefit patients who have unusual complications of sickle cell disease, such as chronic erythrocyte sickling in the liver.     

Splenic sequestration in patients with sickle cell disease.

Splenic sequestration is a potentially life threatening event that is characteristic for sickle cell disease. For reasons unknown a fraction of or even the entire blood volume is trapped in the splenic sinuses within a few hours and thus is no longer available for circulation. The result is splenomegaly, hypovolemia, anemia and extreme reticulocytosis. If the sequestered blood volume is very large the patient goes into fatal hypovolemic shock unless transfused instantly. If the sequestered volume is small there is a chance of spontaneous resolution. The etiology of splenic sequestration is not known. Children with homozygous sickle cell disease (HbSS) are at risk until age 6 years while individuals with compound heterozygous disease (HbSbetaThal, HbSC, HbSD) may develop splenic sequestration even in adulthood. Parents of infants and toddlers with sickle cell disease need to learn how to palpate the spleen in order to detect splenomegaly as early as possible and take the child to the hospital. Splenic sequestration with a drop in hemoglobin of more than 3 g/dl below the patient's usual hemoglobin level is a clear indication for splenectomy regardless of the patient's age as splenic sequestration tends to recur.     

CT imaging of splenic sequestration in sickle cell disease

Pooling of blood in the spleen is a frequent occurrence in children with sickle cell diseases, particularly in the first few years of life, resulting in what is termed “splenic sequestration crisis.” The spectrum of severity in this syndrome is wide, ranging from mild splenomegaly to massive enlargement, circulatory collapse, and even death. The diagnosis is usually clinical, based on the enlargement of the spleen with a drop in hemoglobin level by > 2 g/dl, and it is rare that imaging studies are ordered. However, in the patient who presents to the emergency department with non-specific findings of an acute abdomen, it is important to recognize the appearance of sequestration on imaging studies. We studied seven patients utilizing contrast-enhanced CT scans and found two distinct patterns – multiple, peripheral, non-enhancing low-density areas or large, diffuse areas of low density in the majority of the splenic tissue. Although radiological imaging is not always necessary to diagnose splenic sequestration, in those situations where this diagnosis is not immediately obvious, it makes an important clarifying contribution. Received: 11 May 2000/Accepted: 2 August 2000     

Developmental pattern of splenic dysfunction in sickle cell disorders

Splenic function in sickle hemoglobinopathy syndromes was assessed to determine the developmental pattern of splenic dysfunction. Nonvisualization of the spleen using technetium-99 metastable (99mTc) spleen scans correlated strongly with pocked (vesiculated) RBCs greater than or equal to 3.5%. Cross-sectional analysis of pocked RBC data from 2,086 patients showed differences in the developmental pattern of splenic dysfunction between several disorders. In hemoglobin SS disease (sickle cell anemia) and hemoglobin S beta(0) thalassemia, splenic dysfunction (greater than or equal to 3.5% pocked RBCs) often occurred in the first 6 to 12 months of life. In hemoglobin S beta(+) thalassemia, splenic dysfunction occurred less frequently and later. Splenic dysfunction in hemoglobin SC disease (sickle cell-hemoglobin C) was intermediate. The level of pocked RBCs was inversely associated with fetal hemoglobin (P less than .007) and directly associated with age (P less than or equal to .001). These patterns of splenic dysfunction reflect the known severity of hemolysis and intravascular sickling and are consistent with the epidemiology of severe bacterial meningitis and sepsis in these diseases. Serial measurement of pocked RBCs permits determination of the onset of splenic dysfunction and the time of increased susceptibility to severe bacterial infections.     

Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease.

A cord blood screening programme initiated in June 1973 had screened 68 000 normal deliveries by February 1979 with the detection of 216 cases of homozygous sickle cell disease. Regular review of these children in the Medical Research Council paediatric clinic has identified acute splenic sequestration as a major cause of morbidity and mortality in the first 5 years of life. In addition to classical episodes characterised by peripheral circulatory failure, minor episodes of increasing anaemia associated with an enlarging spleen and an active marrow were also common. These minor episodes appeared to have predictive value in children who later developed severe life-threatening episodes of acute splenic sequestration. Sequestration. Sustained hypersplenism was also appreciably more common in children developing minor or major episodes of acute splenic sequestration compared with those without such a history. It is proposed that the classification of acute splenic sequestration be expanded to include these minor episodes, and that consideration be given to prevention of recurrences by splenectomy particularly in patients who also develop sustained hypersplenism.