Laparoscopic splenectomy in a minimal resource setting: a case series from the Caribbean

This study examines the perioperative outcome of laparoscopic splenectomy in a minimal resources setting in the Caribbean. Seventeen consecutive patients who underwent elective laparoscopic splenectomies by a single surgeon from August 2003 to December 2008 were studied. Data collected included patients' demographics, indications for surgery, operative time, complications, and outcome. 88% were females, the median age being 33.3 years. Idiopathic thrombocytopenic purpura was the most common indication (82%) in majority of the cases. Other indications included hereditary spherocytosis, hypersplenism, and metastatic disease. The mean operative time was 88.8 minutes and the mean hospital length of stay was 3.3 days. One case was converted to open splenectomy. Five patients experienced postoperative complications. There was no mortality. Despite limited blood banking facilities and hematology support, laparoscopic splenectomy may be safe in minimal resources setting, if cases are selected carefully and performed by an experienced surgeon with short operative times

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 (Au)

Septicemia caused by Salmonella infection: an overlooked complication of sickle cell disease

OBJECTIVE: To describe the characteristics of salmonella infections in sickle cell disease and to compare the features of osteomyelitis and those with bacteremia/septicemia without obvious bone changes. To search for risk factors for osteomyelitis, and to draw attention to the frequency and significance of salmonella bacteremia/septicemia. STUDY DESIGN:A retrospective review of all salmonella isolations from the blood, pus, or aspirates during a 22-year period. SETTING: The sickle cell clinic at the University Hospital of the West Indies, Kingston, Jamaica. SUBJECTS: Patients with all genotypes of sickle cell diseases. Incidence data and the frequency of associated cholelithiasis were derived for the cohort study based on follow-up of all children detected by neonatal screening. MAIN OUTCOME MEASURES: Osteomyelitis and bacteremia/septicemia. RESULTS: Of 55 patients with salmonella infections, 25 initially had osteomyelitis and 27 had bacteremia/septicemia. Three of the first group later had bacteremias for a total of 30 episodes of bacteremia/septicemia, and 4 of the second group later had osteomyelitis for a total of 32 episodes of osteomyelitis. The incidence of salmonella infection was 8.6 percent by 15 years and 96 percent of infections occurred before the age of 10 years. Preceding episodes of avascular necrosis of bone were frequent (p < 0.006) in patients with osteomyelitis. Patients with osteomyelitis were not more prone to gallstones. High fever (temperature > or = 40 degrees C or 104 degrees F) occurred in 41 percent, and occasionally marked bone marrow suppression mimicked the aplastic crisis. Twenty Salmonella serotypes were isolated; Salmonella enteritidis accounted for 36 percent of infections, but no serotype difference occurred between those with osteomyelitis and those with bacteremia/septicemia. There were no deaths in the 32 patients with osteomyelitis, but 7 (23 percent) of 30 patients with septicemia died. CONCLUSIONS: Anti-salmonella prophylaxis requires assessment in the management of bone necrosis. Anti-salmonella agents may be indicated in undiagnosed septic conditions in sickle cell disease pending culture results.(AU)

The effect of splenectomy for hypersplenism on whole body protein turnover, resting metabolic rate and growth in sickle cell disease

OBJECTIVE: To determine whether, in the same individual, an observed fall in whole body protein turnover following splenectomy in children with hypersplenism and homozygous sickle cell (SS) disease is associated with a measurable fall in resting metabolic rate (RMR) and an increase in rate of growth. SUBJECTS: Six children (5 SS disease, 1 S beta degree thalassaemia), aged 68 to 126 months, were studied before and after splenectomy for hypersplenism. DESIGN: Protein turnover was measured by the end product method using prime/intermittent oral doses of 15N-glycine and RMR by indirect calorimetry before preoperative transfusion and repeated at least eight weeks after splenectomy. Height and weight velocities were measured over six month periods before and after splenectomy. SETTING: University Hospital of the West Indies in Jamaica and the Medical Research Laboratories (Jamaica). RESULTS: After splenectomy protein turnover fell significantly by 30 percent and RMR by 34 kJ/kg/d. Mean weight velocity which was below normal before surgery, z = -2.3, improved significantly after surgery, z = 0.7, (p = 0.03). Height velocity increase in two children but the mean height velocity did not change following splenectomy. The reduction in protein turnover was estimated to account for 62 percent of the fall in RMR. CONCLUSION: This study confirms that there is a significant reduction in energy expenditure following splenectomy for hypersplenism in SS disease. A reduction in protein turnover was a major contributor to the saving in energy, although it is not clear whether it accounted for all. In the present group of children the energy saved was associated with an improvement in the wasting present before splenectomy.(AU)

Acceleration in linear growth after splenectomy for hypersplenism in homozygous sickle cell disease

Chronic hypersplenism in homozygous sickle cell (SS) disease markedly increases haemolysis and the resulting erythropoietic expansion is likely to have a high metabolic cost. Splenectomy for hypersplenism in SS disease is followed by highly significant changes in haematological indices and an increase in height, but not weight, velocity. This pattern is similar to that observed in the trichuris dysentery syndrome after treatment, and differs from the increase in both height and weight velocity that follow nutritional supplementation of severely malnourished children. It is postulated that accelerated linear growth after the reduction in erythropoietic stress may implicate a specific nutrient deficiency in hypersplenic children with SS (AU)

Plasmodium falciparum infection of splenectomized and intact Guyanan Saimiri monkeys

Spleen-intact and splenectomized Saimiri monkeys of Guyanan origin were examined for their potential suitability for Plasmodium falciparum protection studies. The animal could be readily infected with adapted strains of P. falciparium (Indochina 1/CDC and Uganda Palo Alto FUP strains), but spontaneously recovered without drug treatment and without development of severe clinical disease. In intact animals, peak parasitemia prior to recovery generally ranged from 0.1 percent to 10 percent, whereas in splenectomized animals the peak parasitemia was generally higher so that some animals were given drug treatment to assist in recovery from infection. In reinfection studies, previously infected spleen-intact monkeys demonstrated sterile immunity to the homologous parasite strain but not to a heterologous strain. However, in monkeys infected with the heterologous strain, the peak parasitemia was less than in the first infection and of shorter duration. Splenectomized animals did not demonstrate sterile immunity although the peak parasitemia achieved was less than in the previous infection of each of these monkeys. While the lack of major clinical disease indicated that these monkeys did not provide a good animal model for human malaria, the development of protective immunity was consistent with a useful role in evaluating candidate vaccine antigens (AU)

Inflammatory pseudotumour of the spleen: report of a case and review of the literature

A case of inflammatory pseudotumour of the spleen in a 34-year-old female patient is described. We have briefly reviewed the literature on the subject and discussed aetiopathogenesis, pre-operative differential diagnosis and treatment modalities (AU)

Wiskott-Aldrich Syndrome in a Jamaican

A 3-week-old boy presented with severe thrombocytopenia and recurrent infections. He was subsequently found to have Wiskott-Aldrich Syndrome, a rare X-linked disorder. Splenectomy was performed and he died of post-splenectomy sepsis despite prophylatic antibiotic therapy. This is the first recorded case in the English-speaking Caribbean. (AU)

The effect of splenectomy on whole body protein turnover in homozygous sickle cell disease

In individuals with homozygous sickle cell (SS) disease, haemolysis creates a demand for increased red cell production. In children with hypersplenism haemolysis may be further increased and in such cases the excessive red cell production may be reduced by splenectomy (1). Whole body protein turnover is increased in SS disease and a large part of this increase is the consequence of increased turnover of red blood cells (2). Whole body protein turnover has therefore been measured in children with SS disease before and after splenectomy to find out if the reduction of the haemolytic rate following splenectomy was associated with a decrease in protein turnover. (AU)

Lessons from a cohort study - the case for early detection of sickle-cell disease - abstract

A cord blood screening programme from 1973 to 1981 identified all cases with sickle-cell disease among 100,000 consecutive normal deliveries. Follow-up of childern with homozygous sickle-cell (SS) disease revealed a 15 per cent mortality in the first 10 years of life, one-third of which occurred in the first year of life. The principal causes of early mortality were identified as acute chest syndrome, acute splenic sequestration and pneumococcal septicaemia. Following a parental education programme in the significance and domiciliary diagnosis of acute splenic sequestration, and a policy of prophylatic splenectomy in recurrent life-threatening episodes, the mortality from this group of complications has fallen by approximately 90 per cent. The early institution of prophylactic penicillin therapy has significantly reduced the risks of pneumococcal septicaemia. The acute chest syndrome may also be more effectively treated if the underlying diagnosis of haemoglobinopathy is known. Neonatal screening for sickle-cell disease is a technically simple procedure which, by allowing the early institution of educational and prophylactic programmes, may have a profound effect on morbidity and mortality of the disease. It should be instituted throughout the Caribbean (AU)

Whole-body protein turnover before and after splenectomy in children with homozygous sickle cell disease - abstract

Splenomegaly in sickle-cell disease is associated with excessive red cell destruction. We previously found whole body protein turnover and energy expenditure to be significantly higher in adults patients with sickle-cell disease compared to normals. The increase could be largely due to the increased turnover of the red cell, in particular the protein haemoglobin, in this disease state. We therefore measured whole body protein turnover in four children with sickle-cell disease, before and after splenectomy, to find out if the reduction in haemolysis that follows splenectomy would be reflected in a reduction in protein turnover. Protein turnover was measured with an oral primed intermittent infusion of 15N-glycine over 12-16 hours on the standard hospital diet. Isotopic excretion in urinary urea and ammonia were used to calculate protein turnover. (Table Included) Protein turnover was reduced by 23-47 percent after splenectomy. This reduction is presumably due to the fall in the excessive haemolysis that is associated with enlarged spleen. From a regression of protein turnover against energy expenditure in our previous study, it is estimated that the reduction in turnover after splenectomy could result in a theoretical saving of energy after splenectomy could be associated with the growth spurt that generally follow operation (AU)

Treatment of sickle cell disease in early childhood in Jamaica

The Jamaican sickle cell cohort study, based on neonatal diagnosis of all cases of sickle cell disease among 100 000 consecutive births, has identified acute splenic sequestration (ASS) and pneumoccocal disease as the most important complications in early life. The etiology of ASS is unknown and prophylaxis is therefore not possible. For first attacks, attention has been directed to parental education to achieve earlier diagnosis. Recurrent attacks may be prevented by prophylactic splenectomy. A controlled trial on the prevention of pneumococcal disease has indicated many pneumococcal septicemias in children given the 14 valent pneumococcal vaccine between the ages of 6 months and 3 years. No pneumococcal isolations occurred during the same period in children given monthly long-acting prophylactic penicillin. A controlled trial of foliate supplementation for 1 year in children aged 6 months to 4 years indicated no difference between control and treatment groups in hemoglobin levels or weight and height velocity. The MVC was 4 fl less in the supplemented group. A controlled trial of feeder vessel photocoagulation in the therapy of proliferative retionpathy indicated significantly less vitreous hemorrhage in treated patients but choroidal neovascularization was a common complication of xenon arc therapy and retinal tears commonly followed the use of Argon laser. A new trial of scatter therapy is in progress. (AU)

Role of splenectomy in homozygous sickle cell disease in childhood

60 Jamaican children with homozygous sickle cell (SS) disease underwent splenectomy, 14 for prophylaxis against recurrent acute splenic sequestration and 46 for treatment of sustained hypersplenism. Age at operation varied from 9 months to 27 years (median 6 years), with a total of 369 years of patient-observation. None of the 3 patients who died, at ages 2«, 6«, and 21 years, had received prophylaxis against infection. Overwhelming sepsis was possible but not confirmed in the first two deaths which occurred 11 months and 2« years after operation; the third died from chronic renal failure 11 years after splenectomy. After operation, there were no confirmed cases of pneumococcal septicaemia or meningitis, and the commonest clinical event was the acute chest syndrome. (Summary)

The role of splenectomy in homozygous sickle cell disease in childhood - abstract

The persistence of splenomegaly in children with homozygous sickle cell (SS) disease can pose difficult management problems for the clinician. In the young child the prognosis of acute spenic sequestration (ASS) is worse if superimposed on hypersplenism and in the older child the development of chronic hypersplenism may have a wide range of detrimental effects on the child's health. Elective splenectomy abolishes the risk of further acute, or chronic, sequestration but the increased risk of fulminant sepsis following splenectomy and the known propensity to spontaneous atrophy counsels for conservative mangement. To understand better the role of splenectomy in SS disease the outcome of elective splenectomy in 60 Jamaican children is reviewed. The age at time of operation ranged from 9 months to 17 years, with a mean of 6 years and the period of follow up ranged from 1 month to 19 years. The principal indications were recurrent ASS under the age of 2 years and persistent hypersplenism in those over this age. There was no mortality or significant morbidity during the operative period. Two deaths occurred, one from pneumonia 11 months post operatively and one from unknown causes 30 months post operatively. The haematological response to splenectomy was striking, with significant sustained increases in haemoglobin, platelets and red cell survival times, and fall in reticulocyte count. In the last five years prophylaxis with penicillin and/or pneumococcal and Haemophilus influenzae vaccine has been given to most patients and may have improved the prognosis, but in 54 patients splenectomy precede this period. Elective splenectomy with modern surgical and anaesthetic techniques carries minimal morbidity and with careful patient selection (two episodes of ASS, or sustained hypersplenism) the procedure may confer considerable benefit on selected children with SS disease (AU)

Leukaemic recticuloendotheliosis (hairy cell leukaemia): value of splenectomy

The clinical and pathological features of four cases of leukaemic reticuloendotheliosis or hairy cell leukaemia observed at the University Hospital of the West Indies are described. This is the first report of the condition from Jamaica. The value of splenectomy is emphasized and the relevant literature is reviewed (AU)

The accessory spleen in a Surinam study

Attention is drawn to the incidence of accessory spleens in Suriname. In a post-mortem study at the Mortuarium of the Academic Hospital Paramaribo, consecutive examinations were performed to locate nodules around and near the spleen and the pancreas-tail. Histologic sections from the accessory spleens and from the main spleens were routinely prepared and microscopically examined. The hematoxylin and the eosin methods were used for staining. Between 1 January 1976 and 31 August 1978, 518 effective post-mortem examinations were performed (neonatal age to elderly). Accessory spleens occurred in 57 cases (11.0 per cent). The racial distribution was: 1 of 8 Creoles, 1 of 12 East-Indians, 1 of 12 Javanese and 1 of 6 others. These cases seemed unrelated to other congenital anomalities. The histologic diagnosis in each case was of a normal accessory spleen, but accessory spleens often mimic changes in the main spleen. It is reccommended that an effort should be made to locate and remove any accessory spleen, especially when splenectomy is performed for hypersplenism and other hematologic disorders

Splenic histiocytosis associated with thrombocytopenia

Two young Jamaican women of Chinese descent with idiopathic thrombocytopenic purpura were found to have lipid-laden histiocytes in their spleens; their condition has improved following splenectomy. The electron microscopic appearances of the spleens suggest that platelet destruction contributed to the accumulation of lipid (AU)

Beta-thalassaemia Hb E disease

B-thalassaemia Hb E disease occurring in a young Indian girl in Jamaica is described. Three years after splenectomy there is sustained clinical improvement and blood transfusions have not been necessary. The indications for splenectomy are discussed in relation to the pathology of the anaemia as revealed by radio-isotope studies of blood volume and red cell survival (AU)

The acute abdomen during pregnancy: report of three unusual cases

The details of three cases are presented in which surgical emergencies arose during the course of pregnancy. These consisted of torsion of the spleen, traumatic rupture of the spleen and a volvulus with internal hernia involving large and small bowel. Details of management are discussed as well as the implications of such conditions during pregnancy. (AU)