Non-traumatic bilateral epidural hematoma in a child with sickle cell anemia: A case report and a review of the literature.

OBJECTIVE: To report a rare case of spontaneous bilateral epidural hematoma (EDH) in a 10-year-old Nigerian child with sickle cell disease (SCD) and review the literature regarding this unusual complication. METHODS: We present a case of a pediatric patient with SCD who developed a spontaneous bilateral EDH and discuss the potential underlying mechanisms, management approaches, and outcomes. We also conducted a literature review of existing cases of spontaneous EDH in patients with SCD. RESULTS: Our patient initially presented with a subgaleal hematoma and underlying bilateral EDH, but she was sent home without any neurosurgical consultation. Two years later, she returned with altered consciousness and left-sided weakness, revealing an increased size of the EDH with a noticeable mass effect. She underwent a successful emergency bilateral craniotomy, with noticeable improvement in her level of consciousness and left-sided weakness post-operatively. In our literature review, we found 40 reported cases of spontaneous EDH in SCD patients, with a male predominance (82.5%). The average age of patients was 15.282 years. The most common hematoma location was bifrontal (20%) and the most reported symptom was headache (47.5%). Most patients (97.5%) were already known cases of SCD. Among those treated, 77.5% survived. CONCLUSION: Spontaneous bilateral EDH in SCD patients is an uncommon complication, with a variety of proposed pathophysiological mechanisms. Prompt recognition and appropriate management, either conservative or surgical, are crucial to improve outcomes. Our case and literature review underscore the importance of considering spontaneous EDH in SCD patients presenting with neurological symptoms, even in the absence of trauma. Further research is needed to elucidate the precise etiology, identify risk factors, and optimize management approaches for this rare complication in SCD patients.

Total hip replacement among sickle cell patients in a low-income country-Yemen.

PURPOSE: Sickle cell patients presented with progressive hip pain and limitation of daily activities, as evidenced by low preoperative hip scores and failed conservative therapy. Management of femoral head avascular necrosis (AVN) using total hip replacement (THR) in sickle cell disease (SCD) is widespread in developed countries, but it is still in its initiation stage in developing countries. The outcome of using cementless THR among SCD patients is still unknown with lack of published studies from regional countries. This study aimed to evaluate the outcome of using cementless primary THR among patients with sickle cell disease with end-stage hip avascular necrosis in Yemen. METHODS: Thirty cementless primary total hip arthroplasty (THA) were performed for AVN of the femoral head in 27 sickle cell patients, at Al.-Thawra Modern General Hospital-Sana'a, Yemen, from January 2018 to December 2022. RESULTS: The mean age of the patients was 27 ± five years (ranged 18-37 years) with a male to female ratio was 3:1. Steinberg staging for hip AVN was stage IV, one patient (3%); stage VI, thirteen patients (45%); and stage V, sixteen patients (51%). THR was on right side 14 (52%), left side 10 (37%), and bilateral 3 (11%). The implant used was ceramic on polyethylene acetabular liner. All patients showed improvement in Harris hip score from preoperative mean hip score was 25 ± 8 points to postoperative mean hip score was 88 ± 6 points at the last follow-up. Mean of the length of stay in hospital was 12.7 ± eight days (ranged from 4 to 32 days); the mean operating time was 107 ± 23 min. Three cases had superficial wound infection; four patients had five intraoperative fractures; two cases had pulmonary complications; one case had abdominal crisis. All patients had postoperative leg length discrepancy less than 2 cm. None had deep infection, nerve injury, wound hematoma, aseptic loosing, dislocation, DVT, heterotopic ossification, or death. CONCLUSION: THR in SCD has a good outcome using cementless THA with a low rate of complication in Yemen, a developing country.

Early splenectomy in a large cohort of children with sickle cell anemia: risks and consequences.

In children with sickle cell anemia (SCA), early splenic complications can require splenectomy, but the benefit-to-risk ratio and the age at which splenectomy may be safely performed remain unclear. To address this question, we analyzed the rate of post-splenectomy events in children with SCA splenectomized between 2000-2018 at the Robert Debré University Hospital, Paris, France. A total of 188 children underwent splenectomy, including 101 (11.9%) from our newborn cohort and 87 referred to our center. Median (Q1-Q3) age at splenectomy was 4.1 years (range 2.5-7.3 years), with 123 (65.4%) and 65 (34.6%) children splenectomized at ≥3 years of age or <3 years of age, respectively. Median postsplenectomy follow-up was 5.9 years (range 2.7-9.2 years) yielding 1192.6 patient-years (PY) of observation. Indications for splenectomy were mainly acute splenic sequestration (101 [53.7%]) and hypersplenism (75 [39.9%]). All patients received penicillin prophylaxis; 98.3% received 23-valent polysaccharic pneumococcal (PPV-23) vaccination, and 91.9% a median number of 4 (range 3-4) pneumococcal conjugate vaccine shots prior to splenectomy. Overall incidence of invasive bacterial infection and thrombo-embolic events were 0.005 / PY (no pneumococcal infections) and 0.003 / PY, respectively, regardless of age at splenectomy. There was an increased proportion of children with cerebral vasculopathy in children splenectomized <3 years of age (0.037 / PY vs. 0.011 / PY; P<0.01). A significantly greater proportion of splenectomized than non-splenectomized children were treated with hydroxycarbamide (77.2% vs. 50.1%; P<0.01), suggesting a more severe phenotype in children who present spleen complications. If indicated, splenectomy should not be delayed in children, provided recommended pneumococcal prophylaxis is available. Spleen complications in childhood may serve as a marker of severity.

Impact of sickle cell disease in shoulder arthroplasty: a matched cohort analysis.

BACKGROUND: Sickle cell disease (SCD) is a genetic disorder of abnormal hemoglobin synthesis that is known to cause glenohumeral avascular necrosis (AVN). Little has been published on the use of shoulder arthroplasty (SA) for the treatment of glenohumeral AVN in SCD. We report on the clinical and radiographic results and postoperative complications following SA in the patient cohort. METHODS: A retrospective review was performed identifying 17 primary SAs (9 hemiarthroplasties, 7 anatomic total SAs, and 1 reverse total SA) in patients with a confirmed diagnosis of SCD and a minimum of 2-year follow-up. This cohort was matched (1:2) according to age, sex, body mass index, type of prosthesis, and year of surgery with patients who had undergone hemiarthroplasty or total SA for osteoarthritis (OA) or reverse total SA for cuff tear arthropathy. Outcomes included the visual analog scale (VAS) for pain, American Shoulder and Elbow Surgeons score, range of motion, and strength measurements in forward elevation (FE), external rotation (ER), and internal rotation (IR). RESULTS: Our cohort included 9 (52.9%) men with a mean age of 43 yr. The average follow-up time was 5.9 yr. In patients with SCD, SA provided significant improvements in VAS pain (9.1-3.8; P < .001), FE (95°-128°; P < .001), ER (24°-38°; P < .001), IR score (3.2-5.2; P < .001), FE strength (4.2-4.8; P < .001), ER strength (4.1-4.7; P < .001), IR strength (4.1-4.7; P < .001), and American Shoulder and Elbow Surgeons scores (48.6-73.5; P < .001). When compared to the matched cohort, the SCD group demonstrated higher preoperative (9.1 vs. 3.8; P < .001) and postoperative VAS scores (3.8 vs. 1.3; P < .001). Other clinical outcomes demonstrated no significant differences. There were 5 (29%) complications, 2 (11.8%) episodes of sickle cell crisis, and 3 (18%) reoperations in the SCD cohort. When compared to the matched cohort, there were no statistical differences with respect to complications (29% vs. 12%; P = .140) or reoperations (18% vs. 12%; P = .387). CONCLUSIONS: SA is an effective treatment modality for glenohumeral AVN in patients with SCD. Patients may expect improvements in pain, function, motion, and strength. However, final postoperative pain relief may be less than those treated with SA without SCD. Unique perioperative management is necessary to mitigate the risk of postoperative sickle cell crises.

Femoral Head Core Decompression and Bone Marrow Concentrate Injection in Pediatric Sickle-cell Related Avascular Necrosis.

BACKGROUND: Femoral head avascular necrosis affects 10% to 40% of patients with sickle cell disease (SCD). The purpose of this study was to evaluate the results of femoral head core decompression and bone marrow aspirate concentrate (CD-BMAC) injection in pediatric patients with SCD. METHODS: Eligible patients were enrolled in this retrospective study of patients undergoing CD-BMAC. Patients with SCD who were younger than 18 at the time of surgery and had >1 year of follow-up were included in this analysis. Hips were staged based on the Ficat system by 2 raters. The visual analog score for pain, hip outcome score, modified Harris hip score, and the University of California, Los Angeles activity score were used as patient-reported outcome measures preoperatively, at 5 to 9 months postoperatively, and final follow-up. Treatment failure was defined as total hip arthroplasty or visual analog score >3 at the final follow-up. RESULTS: Twenty-three hips in 17 patients were included, with a median age at the time of surgery of 15.8 years (interquartile range: 13.1 to 17.8 y). Patients were followed for 4.25 ± 1.7 years. Ten hips showed a 1-stage increase in the Ficat stage at the final follow-up, whereas 11 retained the same stage, and 1 hip had a 1-stage regression. All patient-reported outcome measures showed significant improvement from the preoperative visit to short-term follow-up, but the preoperative to final follow-up improvement was not significant. Six treatment failures were recorded, including 3 total hip arthroplasties and 3 painful hips at the final follow-up. In a multivariate logistic regression model, only skeletal maturity (odds ratio = 16.2, 95% CI: 1.44-183.0, P = 0.024) and femoral head collapse (odds ratio = 12.0, 95% CI: 1.1-130.5, P = 0.041) were significant predictors of treatment failure. CONCLUSIONS: In the largest study on pediatric SCD patients undergoing CD-BMAC, we found that CD-BMAC injection offers significant improvement in pain and functional outcomes in the short term, with a very low risk for complications. Skeletal maturity and femoral head collapse were significant predictors of treatment failure. Patients with a collapsed femoral head experienced functional deterioration over time after initial improvement, whereas precollapse hips maintained their functional improvement up to the latest follow-up. LEVEL OF EVIDENCE: Level III, retrospective cohort study.

Modifying priming techniques in cardiopulmonary bypass circuit in known case of sickle-cell trait undergoing open heart surgery.

Modifying cardiopulmonary bypass (CPB) circuit's priming technique before the onset of CPB in cardiac patients known with sickle-cell disease or sickle cell trait has been observed to be of substantial significance in dealing with such challenges without having any life-threatening consequences. We modified our routine heparinized crystalloid priming of the CPB circuit with partial exchange transfusion by adding donor blood (packed red blood cells), fresh frozen plasma (FFP), and bicarbonate. This has helped us bring down the overall sickle cell hemoglobin in the blood thereby reducing its risk of sickling.

Survival of total hip arthroplasty (THA) in sickle cell disease.

BACKGROUND: The objective of this study was to evaluate the outcome of THA done in patients with sickle cell disease at a single center, and compare with rest of the world before we could recommend routine THA in SCD patients. METHODS: In 92 patients, 117 THAs were performed in sickle cell patients. Seventy-six (76) patients had homozygous hemoglobin (Hgb SS) disease, whereas sixteen had sickle cell trait (Hgb AS). Patients with Grade III Ficat and above avascular necrosis of the head of femur (ANFH) with cementless porous-coated proximal femur fixation were included in this analysis. Harris hip score was modified (MHHS) and used for assessment of improvement and visual analog scale score (VAS), for pain assessment. RESULT: VAS improved from average pre-operative mean of 7 (7-9) to 2.5 (1-5). The average pre-operative MHHS was 44 (32-63), and it improved to 79 (71-88) in the final mean follow-up of 12.4 (4-18) years. Nine patients (9.7%) had complications and revision surgery was required in seven (5.9%) for aseptic loosening. CONCLUSION: Our analysis shows that THA in sickle cell disease is safe with average 12 years of follow-up, indicating that surgery in these young patients (≤ 25 years) is safe and should not be delayed.

How does sickle cell disease affect the peri-operative outcome in patients undergoing total knee arthroplasty? A large-scale, National Inpatient Sample-based study.

INTRODUCTION: In view of the vaso-occlusive pathophysiology affecting osseous micro-circulation, sickle cell disease (SCD) is well known to present with diverse skeletal and arthritic manifestations. With prolonged life-expectancy over the past decades, there has been a progressive increase in the proportion of SCD patients requiring joint reconstructions. Owing to the paucity of evidence in the literature, the post-operative complication rates and outcome in these patients following total knee arthroplasty (TKA) are still largely unknown. METHODS: Based on the National Inpatient Sample (NIS) database (using ICD-10 CMP code), patients who underwent TKA between 2016 and 2019 were identified. The cohort were classified into two groups: A-those with SCD; and B-those without. The data on patients' demographics, co-morbidities, details regarding hospital stay including expenditure incurred, and complications were analyzed and compared. RESULTS: Overall, 558,361 patients underwent unilateral, primary TKA; among whom, 493 (0.1%) were known cases of SCD (group A). Group A included a significantly greater proportion of younger (60.14 ± 10.87 vs 66.72 ± 9.50 years; p < 0.001), male (77.3 vs 61.5%; p < 0.001); and African-American (88.2 vs 8.3%B; p < 0.001) patients, in comparison with group B. Group A patients were also at a significantly higher risk for longer duration of peri-operative hospital stay (p < 0.001), greater health-care costs incurred (p < 0.001), and greater need for alternative step-down health-care facilities (p < 0.001) following discharge. Among the SCD patients, 24.7%, 20.9% and 24.9% developed acute chest syndrome, pain crisis and splenic sequestration crisis, respectively during the peri-operative period. Group A patients had a statistically greater incidence of acute renal failure (ARF; p = 0.014), need for blood transfusion (p < 0.001) and deep vein thrombosis (DVT; p = 0.03) during the early admission period. CONCLUSION: The presence of SCD substantially lengthens the duration of hospital stay and enhances health care-associated expenditure in patients undergoing TKA. SCD patients are at significantly higher risk for systemic complications including acute chest syndrome, pain crisis, splenic sequestration crisis, acute renal failure, higher need for blood transfusions and deep venous thrombosis during the initial peri-operative period following TKA.

Guideline on the peri-operative management of patients with sickle cell disease: Guideline from the Association of Anaesthetists.

Sickle cell disease is a multisystem disease characterised by chronic haemolytic anaemia, painful vaso-occlusive crises and acute and chronic end-organ damage. It is one of the most common serious inherited single gene conditions worldwide and has a major impact on the health of affected individuals. Peri-operative complications are higher in patients with sickle cell disease compared with the general population and may be sickle or non-sickle-related. Complications may be reduced by meticulous peri-operative care and transfusion, but unnecessary transfusion should be avoided, particularly to reduce the risk of allo-immunisation. Planned surgery and anaesthesia for patients with sickle cell disease should ideally be undertaken in centres with experience in caring for these patients. In an emergency, advice should be sought from specialists with experience in sickle cell disease through the haemoglobinopathy network arrangements. Emerging data suggest that patients with sickle cell disease are at increased risk of COVID-19 infection but may have a relatively mild clinical course. Outcomes are determined by pre-existing comorbidities, as for the general population.

Liver transplantation for sickle cell disease: a systematic review.

BACKGROUND: Sickle cell disease is a group of autosomal recessive disorders characterised by haemolytic anaemia. Liver is one of the most affected organs, ranging from liver tests alterations to acute liver failure for which liver transplantation is the only life-saving treatment. METHODS: This study aims to make a systematic review of the current literature to evaluate indications, timing, and results of liver transplantation for patients affected by SCD. RESULTS: Twenty-nine patients in total were reported worldwide until 2018, the average patient age is 28.7 (0.42-56), all patients have a pre-transplant diagnosis of SCD. Cirrhosis at transplantation was present in six-teen (n = 16, 55.1%) patients. In ten patients (n = 10, 34.5%), acute liver failure arises from healthy liver and presented sickle cell intrahepatic cholestasis. Eleven patients (n = 11, 39.2%) died, three (n = 3, 10.7%) in the first postoperative month, and seven (n = 7, 25%) in the first year. Mean follow-up was 27 months (range: 7-96), one-year overall survival was 48.7%. DISCUSSION: Liver transplantation for SCD has been increasingly reported with encouraging results. Indications are presently reserved for acute liver failure arising both in healthy liver and end-stage liver disease.

Perioperative care of children with sickle cell disease: A systematic review and clinical recommendations.

Children with sickle cell disease (SCD) require specific perioperative care, and clinical practice in this area remains poorly defined. We aimed to conduct a systematic, PRISMA-based review of the literature, available clinical guidelines and practice recommendations. We also aimed to extract any valuable information for the "best of available-evidence"-based prevention of perioperative adverse events in children with SCD, and highlight the most urgent priorities in clinical research. As data sources, US National Library of Medicine, Medline, National Guideline Clearinghouse, International Guideline Network, TRIP databases were searched for any content until January 2019. We also included institutional, consortia and expert group guidelines. Included were reports/guidelines in English, French, German, and Italian. Excluded were reports on obstetrical and fetal management. We identified 202 reports/guidelines fulfilling the criteria outlined above. A majority focused on visceral, cardiovascular and orthopedic surgery procedures, and only five were multicenter randomized controlled trials and two prospective randomized studies. After grading of the quality of the evidence, the extracted data was summarized into clinical recommendations for daily practice. Additionally, we designed a risk-grading algorithm to identify contexts likely to be associated with adverse outcomes. In conclusion, we provide a systematic PRISMA-based review of the existing literature and ancillary practice and delineate a set of clinical recommendations and priorities for research.

Bilateral THA in the same sitting for avascular necrosis of the head of the femur in sickle cell patient: our African experience.

Sickle cell anemia is protective against the malaria protozoan. The heterozygous form of the disease is not fatal, and may cause musculoskeletal disorders when sickling occurs, and small vessels are occluded. When the head of the femur is involved, this may result in hip arthritis, often bilateral, at a young age. This article describes three patients in whom bilateral total hip arthroplasty (THA) was performed in the context of a humanitarian mission in Togo, Africa.

Total hip arthroplasty for osteonecrosis of the femoral head in sickle cell disease: a case series from our African experience.

Sickle cell disease causes osteonecrosis (20% to 50% of patients) and collapse of the femoral head that result in secondary osteoarthritis. Total hip arthroplasty (THA) is a valid alternative for these patients. We define the difficulties that can be encountered when undertaking THA in sickle cell disease patients and give advice on how to deal with these technically demanding procedures. We undertook total hip arthroplasty procedures on 12 patients (4 females and 8 males) with osteonecrosis of the femoral head. Two bilateral prostheses were performed. We had only one type of stem, only one type of acetabular cup and only 28 mm cobalt chrome heads. The procedures were performed through either an anterior or a direct lateral approach. The average size of the Cup was 46 (Versafit, Medacta), the average size of the femoral stem was 0 (Amistem, Medacta), the most used size of the modular head was a S. Standard stem that was used in nine patients, while three patients received a lateralizing stem. Three patients had periprosthetic fracture, treated by cerclage. Total hip replacement is an excellent alternative for patients with osteonecrosis from sickle cell disease. The preparation of the acetabulum and the femur is difficult and requires attention, time and appropriate equipment.

Hematopoietic stem cell transplantation for people with sickle cell disease.

BACKGROUND: Sickle cell disease is a genetic disorder involving a defect in the red blood cells due to its sickled hemoglobin. The main therapeutic interventions include preventive and supportive measures. Hematopoietic stem cell transplantations are carried out with the aim of replacing the defective cells and their progenitors (hematopoietic (i.e. blood forming) stem cells) in order to correct the disorder. This is an update of a previously published review. OBJECTIVES: To determine whether stem cell transplantation can improve survival and prevent symptoms and complications associated with sickle cell disease. To examine the risks of stem cell transplantation against the potential long-term gain for people with sickle cell disease. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Group's Haemoglobinopathies Trials Register complied from electronic searches of the Cochrane Central Register of Controlled Trials (CENTRAL) (updated each new issue of the Cochrane Library) and quarterly searches of MEDLINE. We also searched trial registries for ongoing trials up to April 2020. Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 09 December 2019. SELECTION CRITERIA: Randomized controlled and quasi-randomized trials that compared any method of stem cell transplantation with either each other or with any of the preventive or supportive interventions (e.g. periodic blood transfusion, use of hydroxyurea, antibiotics, pain relievers, supplemental oxygen) in people with sickle cell disease irrespective of the type of sickle cell disease, gender and setting. DATA COLLECTION AND ANALYSIS: No trials were eligible for inclusion in the review. MAIN RESULTS: We identified 12 potentially-eligible trials by the searches; we excluded 11 of these and the remaining trial is an ongoing trial that may be eligible for inclusion in a future version of the review. AUTHORS' CONCLUSIONS: Reports on the use of hematopoietic stem cell transplantation improving survival and preventing symptoms and complications associated with sickle cell disease are currently limited to observational and other less robust studies. We did not find any eligible randomized controlled trials assessing the benefit or risk of hematopoietic stem cell transplantations. However, there is an ongoing quasi-randomized trial comparing hematopoietic stem cell transplantation with standard care, Thus, this systematic review identifies the need for a multicentre randomized controlled trial assessing the benefits and possible risks of hematopoietic stem cell transplantations comparing sickle status and severity of disease in people with sickle cell disease.

Preoperative blood transfusions for sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Surgical interventions are more common in people with SCD, and occur at much younger ages than in the general population. Blood transfusions are frequently used prior to surgery and several regimens are used but there is no consensus over the best method or the necessity of transfusion in specific surgical cases. This is an update of a Cochrane Review. OBJECTIVES: To determine whether there is evidence that preoperative blood transfusion in people with SCD undergoing elective or emergency surgery reduces mortality and perioperative or sickle cell-related serious adverse events. To compare the effectiveness of different transfusion regimens (aggressive or conservative) if preoperative transfusions are indicated in people with SCD. SEARCH METHODS: We searched for relevant trials in the Cochrane Library, MEDLINE (from 1946), Embase (from 1974), the Transfusion Evidence Library (from 1980), and ongoing trial databases; all searches current to 28 January 2020 We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register: 19 September 2019. SELECTION CRITERIA: All randomised controlled trials and quasi-randomised controlled trials comparing preoperative blood transfusion regimens to different regimens or no transfusion in people with SCD undergoing elective or emergency surgery. There was no restriction by outcomes examined, language or publication status. DATA COLLECTION AND ANALYSIS: Two authors independently assessed trial eligibility and the risk of bias and extracted data. MAIN RESULTS: Three trials with 990 participants were eligible for inclusion in the review. There were no ongoing trials identified. These trials were conducted between 1988 and 2011. The majority of people included had haemoglobin (Hb) SS SCD. The majority of surgical procedures were considered low or intermediate risk for developing sickle cell-related complications. Aggressive versus simple red blood cell transfusions One trial (551 participants) compared an aggressive transfusion regimen (decreasing sickle haemoglobin to less than 30%) to a simple transfusion regimen (increasing haemoglobin to 100 g/L). This trial re-randomised participants and therefore quantitative analysis was only possible on two subsets of data: participants undergoing cholecystectomy (230 participants); and participants undergoing tonsillectomy or adenoidectomy surgeries (107 participants). Data were not combined as we do not know if any participant received both surgeries. Overall, the quality of the evidence was very low across different outcomes according to GRADE methodology. This was due to the trial being at high risk of bias primarily due to lack of blinding, indirectness and the outcome estimates being imprecise. Cholecystectomy subgroup results are reported in the abstract. Results for both subgroups were similar. There was no difference in all-cause mortality between people receiving aggressive transfusions and those receiving conservative transfusions. No deaths occurred in either subgroup. There were no differences between the aggressive transfusion group and conservative transfusion group in the number of people developing: • an acute chest syndrome, risk ratio (RR) 0.84 (95% confidence interval (CI) 0.38 to 1.84) (one trial, 230 participants, very low-quality evidence); • vaso-occlusive crisis, risk ratio 0.30 (95% CI 0.09 to 1.04) (one trial, 230 participants, very low quality evidence); • serious infection, risk ratio 1.75 (95% CI 0.59 to 5.18) (one trial, 230 participants, very low-quality evidence); • any perioperative complications, RR 0.75 (95% CI 0.36 to 1.55) (one trial, 230 participants, very low-quality evidence); • a transfusion-related complication, RR 1.85 (95% CI 0.89 to 3.88) (one trial, 230 participants, very low-quality evidence). Preoperative transfusion versus no preoperative transfusion Two trials (434 participants) compared a preoperative transfusion plus standard care to a group receiving standard care. Overall, the quality of the evidence was low to very low across different outcomes according to GRADE methodology. This was due to the trials being at high risk of bias due to lack of blinding, and outcome estimates being imprecise. One trial was stopped early because more people in the no transfusion arm developed an acute chest syndrome. There was no difference in all-cause mortality between people receiving preoperative transfusions and those receiving no preoperative transfusions (two trials, 434 participants, no deaths occurred). There was significant heterogeneity between the two trials in the number of people developing an acute chest syndrome, a meta-analysis was therefore not performed. One trial showed a reduced number of people developing acute chest syndrome between people receiving preoperative transfusions and those receiving no preoperative transfusions, risk ratio 0.11 (95% confidence interval 0.01 to 0.80) (65 participants), whereas the other trial did not, RR 4.81 (95% CI 0.23 to 99.61) (369 participants). There were no differences between the preoperative transfusion groups and the groups without preoperative transfusion in the number of people developing: • a vaso-occlusive crisis, Peto odds ratio (OR) 1.91 (95% confidence interval 0.61 to 6.04) (two trials, 434 participants, very low-quality evidence). • a serious infection, Peto OR 1.29 (95% CI 0.29 to 5.71) (two trials, 434 participants, very low-quality evidence); • any perioperative complications, RR 0.24 (95% CI 0.03 to 2.05) (one trial, 65 participants, low-quality evidence). There was an increase in the number of people developing circulatory overload in those receiving preoperative transfusions compared to those not receiving preoperative transfusions in one of the two trials, and no events were seen in the other trial (no meta-analysis performed). AUTHORS' CONCLUSIONS: There is insufficient evidence from randomised trials to determine whether conservative preoperative blood transfusion is as effective as aggressive preoperative blood transfusion in preventing sickle-related or surgery-related complications in people with HbSS disease. There is very low quality evidence that preoperative blood transfusion may prevent development of acute chest syndrome. Due to lack of evidence this review cannot comment on management for people with HbSC or HbSß+ disease or for those with high baseline haemoglobin concentrations.

Total Hip Arthroplasty in Patients With Sickle Cell Disease: A Comprehensive Systematic Review.

BACKGROUND: Total hip arthroplasty (THA) is a common treatment for end-stage osteonecrosis of the hip in patients with sickle cell disease (SCD). This patient population presents unique challenges in the perioperative period. This systematic review aims to investigate the existing literature on the outcomes, complications, and survivorship of primary THA in SCD patients. METHODS: A systematic search using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines was performed of PubMed, MEDLINE, EMBASE, and Cochrane databases for clinical studies on THA in SCD patients. Studies on primary THA in SCD patients with a mean follow-up greater than 90 days were included. RESULTS: Sixteen studies containing 5193 SCD patients met criteria for inclusion. The Coleman Quality of Evidence score ranged from poor to moderate. SCD patients had a significant increase in Harris Hip Scores and Merle d'Aubigne Scores after undergoing THA. Compared to non-SCD patients, SCD patients had increased hospital length-of-stay, 30-day and 90-day readmission rates, and rates of medical complications, including pain crises, acute chest syndrome, cardiac complications, sepsis, and mortality. SCD patients also had increased rates of surgical complications, including wound complications, infection, periprosthetic fracture, and aseptic loosening. Overall, THA revision rates were higher in SCD patients relative to those with primary osteoarthritis. CONCLUSION: THA remains an effective treatment modality for osteonecrosis of the hip in SCD patients. However, these patients are at increased risk of medical and surgical complications. Surgeons should be aware of the unique challenges in this patient population when counseling and managing these patients in the perioperative period.

Outcomes of a preoperative risk-based transfusion assignment protocol in sickle cell disease patients: a single-center retrospective study from Saudi Arabia.

Many patients with sickle cell disease (SCD) need surgical management during their lifetime. The best approach for preoperative transfusion in SCD is still to be determined. In this single-center retrospective study, we included HBSS/HBS-Beta0-thalassemia patients younger than 16 years of age who underwent surgery between January 2008 and July 2019. Preoperative transfusion assignment (PTA) was based on SCD severity and surgical risk. Patients were assigned to no transfusion, simple transfusion, or exchange transfusion. A total of 284 patients were identified and 66 (23%) underwent 78 procedures. Mean age at the time of procedure was 8 (5-11) years, mean baseline hemoglobin was 8.5 (7.8-9.3) g/dl, and mean hemoglobin F was 18.4 ± 8.2%. SCD severity was low-risk in 57 (73%) and high-risk in 21 (27%) patients. Surgical risk was low-risk in 20 (25.6%) and medium-risk in 58 (74.4%) procedures. PTA was no transfusion in 17 (22%), simple transfusion in 40 (51%), and exchange transfusion in 21 (27%) procedures. Postoperative complications occurred in five (6.4%) of procedures only in the simple transfusion group (three acute chest syndrome, one hemolytic anemia, one pain crisis) undergoing medium-risk surgery. Preoperative risk-based transfusion assignment is feasible. Despite a high baseline hemoglobin level in the no transfusion group, none of the patients developed postoperative complications. It is possible that the high baseline hemoglobin F phenotype was protective and indicates the need to study the risk/benefit of interventions used in this phenotype.

Catastrophic sickling crisis in patient undergoing cardiac transplantation with sickle cell trait.

There is debate in the literature regarding management of patients with sickle cell trait (SCT) undergoing cardiac surgery, since it is recognized that cardiopulmonary bypass presents many precipitating risk factors for a sickling crisis. Despite this, many report successful outcomes without any modification to perioperative management. A 49-year-old woman with SCT (HbS 38%) with postpartum cardiomyopathy underwent cardiac transplantation. The patient was cooled to 34.0°C and retrograde cold blood cardioplegia was infused continuously. The cold ischemic time was 219 minutes and warm ischemic time 46 minutes. After weaning from bypass, she developed global cardiac dysfunction requiring veno-arterial extracorporeal membrane oxygenation. The circuit suddenly stopped, requiring emergency reinstitution of bypass; the circuit had clotted. Transesophageal-echocardiogram revealed thrombus within the left atrium and ventricle. There was no recovery of cardiac function and the patient developed multiorgan failure. At postmortem there was extensive myocardial infarction with evidence of widespread catastrophic intravascular red-cell sickling. This case highlights the danger of complacency in patients with SCT, offering a learning opportunity for the cardiothoracic community to highlight the most serious complication that can occur in this group of patients. We have learned that SCT and cardiac surgery is not a benign combination.

Acute Chest Syndrome After Splenectomy in Children With Sickle Cell Disease.

BACKGROUND: Individuals with sickle cell disease (SCD) are at high risk of developing life-threatening complications, particularly acute chest syndrome (ACS) postoperatively. The perioperative factors associated with the development of ACS in children with SCD after splenectomy have not been clearly identified. MATERIALS AND METHODS: We retrospectively reviewed medical records of all children who underwent splenectomy at our institution between 1997 and 2017 with the goal of identifying perioperative factors associated with postoperative ACS. Categorical and noncategorical variables were compared using Fisher's exact test and Student's two-tailed t-test, respectively. RESULTS: Sixty-five patients with SCD underwent splenectomy at a median of 4.0 (interquartile range [IQR] 2.0-8.0) years of age. A laparoscopic approach was used for 64 (98.5%) patients. Fifty-six (86.2%) underwent laparoscopic total splenectomy, and eight (12.3%) underwent laparoscopic partial splenectomy, of which two were converted to open. One had an open partial splenectomy (1.5%). Of the 65 patients, 10 (15.4%) developed ACS with a mean time to diagnosis of 49.0 ± 34.5 h. Children who developed ACS had a higher postoperative median pain score of 6.8 (IQR 5.1-9.1) versus 2.7 (IQR 1.6-4.2), P < 0.001, higher median pain score area under the curve 111.5 (IQR 76.9-169.1) versus 47.3 (IQR 30.5-78.3), P = 0.01, and received more total morphine equivalents (median 1.4 [IQR 0.4-2.7] versus 0.5 [IQR 0.3-0.9] mg/kg, respectively; P = 0.003), compared with children who did not develop ACS. CONCLUSIONS: Significant postoperative pain may be an early sign of ACS that could be worsened by opioid use, supporting the investigation of nonopioid pain control options in this patient population.

Splenectomy is not associated with a higher tricuspid regurgitant jet velocity in people with sickle cell anemia.

BACKGROUND: Vascular complications such as pulmonary hypertension (PH) occur at an increased rate following splenectomy in patients with various hemolytic blood disorders including thalassemia. The goal of this retrospective cross-sectional analysis was to assess the independent association of splenectomy with an elevated tricuspid regurgitation velocity (TRV) in people with homozygous sickle cell disease (HbSS). TRV is a noninvasive screening test for PH and a surrogate marker of prognosis in sickle cell disease (SCD). PROCEDURE: Data were obtained from the multicenter Walk-PHaSST (treatment of pulmonary hypertension and sickle cell disease with sildenafil therapy) study of PH (NCT00492531). We compared TRV in the cohort of patients with HbSS who were surgically splenectomized with patients who were not surgically splenectomized. RESULTS: We found no significant differences in TRV between the two groups. CONCLUSIONS: The lack of difference in TRV between the two groups is most likely because members of the comparator nonsurgical group in many cases experienced autoinfarction of the spleen in childhood. Splenectomy does not seem to confer additional risk for the development of a higher TRV in HbSS, unlike in patients with thalassemia or other hemolytic anemias. This could be an important consideration when weighing the risks and benefits of splenectomy in patients with HbSS.