Anesthesiological management in patients with sickle cell disease

We report on the general anesthesia in the case of an orthopedic surgery conducted on two brothers with sickle cell anemia. After concerted planning with a pediatric hematologist concerning the perioperative management an exchange transfusion was conducted ambulatorily before surgery. The following anesthesia under continuous fluid infusion, warming and regular hemoglobin and temperature control could be conducted without problems. One of the boys was treated with two heterologous red cell packs after an unexpected hemoglobin drop intraoperatively. The postoperative course was uneventful, especially concerning sickle cell relevant complications. The case reports are intended to show up the pathogenesis of patient-relevant sickle cell crises and critically evaluate central points of anesthesiological management, especially the choice of preoperative transfusion.     

Cardiopulmonary bypass in sickle cell anaemia. Report of five cases

Five patients with sickle cell haemoglobinopathies underwent open heart surgery. At the start of the cardiopulmonary bypass the patient's circulating blood volume was separately drained into a bag, spun down and retransfused into the patient at the end of the operation. Moderate hypothermia, aortic cross clamping, topical hypothermia and cold crystalloid cardioplegia were used in all patients. None of the patients had transfusions in the postoperative period. No macroscopic or microscopic evidence of haemolysis were seen, nor haematuria or other clinical evidence of sickling. There was no evidence of wound infection and all were discharged home at a mean of 12.6 days. It is concluded that in cases of sickle cell anaemia exchange transfusion at the beginning of cardiopulmonary bypass followed by retransfusion of the red cell free blood can be used safely, permitting the performance of standard open heart procedures.     

Assessment of perioperative transfusion therapy and complications in sickle cell disease patients undergoing surgery

BACKGROUND: Perioperative blood transfusion is usually given to sickle cell disease patients to reduce or prevent perioperative morbidity. Assessment of such a practice was the subject of our study. METHODS: A retrospective one year survey of sickle cell disease patients undergoing surgery at Salmaniya Medical Complex, Bahrain was conducted. The medical records were reviewed to characterize the surgical procedure, transfusion management and perioperative complications. RESULTS: 85 sickle cell disease patients who underwent surgery were studied. Preoperatively, 21.2% had exchange transfusion (ETX), 24.7% had simple transfusions (STX) and 54.1% had no transfusion (NTX). 14.1% of all patients had postoperative complications, and 50% of those, had complications from the laparoscopic cholecystectomy group. The incidence of sickle cell crisis postoperatively was 22.2% in ETX group, 9.5% in STX group and 4.34% in the NTX group. The incidence of acute chest syndrome postoperatively was found to be 5.55% in the ETX group, 4.76% in the STX group and 4.34% in the NTX group. No intraoperative complications were recorded in all groups. All patients who had postoperative complications had a preoperative HBSS > 40%. CONCLUSION: Exchange transfusion does not prevent perioperative complications of sickle cell disease patients. HBSS > 40% carries a higher risk of postoperative complications.     

Can exchange transfusions treat postoperative intrahepatic colestasis in patients with sickle cell anemia?

BACKGROUND: Although the most common cause of liver failure (LF) in hematologic patients is viral hepatitis, several episodes of sickle cell intrahepatic cholestasis (IHC) have been reported as rare but potentially causative of fulminant LF. Reviewing the literature, we have presented a single case of intrahepatic cholestasis after major liver resection, which was effectively treated by exchange transfusion. METHODS: Serial hemoglobin S, D levels and liver enzymes were monitored postoperatively. RESULTS: Although the patient's intra- and postoperative courses were uneventful, an increased serum bilirubin was identified to be due to intrahepatic sinusoid congestion and subsequent cholestasis. Exchange transfusion was required to maintain HbS below 20% and reverse bilirubin levels to normal values. CONCLUSION: Sickle cell anemia is a rare cause of cholestasis after major hepatic surgery. To our knowledge, this case is the only documented incidence of IHC following major hepatectomy that was effectively treated with exchange transfusion.     

Perioperative Überwachung der Hämoglobinfraktionen bei homozygoter Sichelzellanämie

This case report presents the perioperative management of double-sided hip arthroplasty in a patient (female, 25 years old) homozygous for sickle cell anemia (SS). The fraction of sickle hemoglobin (Hb S) to total hemoglobin was monitored with an automated cation exchange microcolumn chromatography. The main purpose of ion exchange chromatography is to measure glycated hemoglobin A (HbA1c) in diabetic patients. Furthermore, as a by-product, this test enables the quantitative assessment of aberrant hemoglobin molecules such as sickle cell hemoglobin Hb S with sufficient precision and selectivity. The standard method, hemoglobin-electrophoresis is more complicated and not generally available. For the perioperative estimation of the risk for sickle cell related complications and as a guide for transfusion therapy, knowledge of preoperative Hb S level is essential. In this case report, the clinical use of a rapid laboratory test at low costs with common equipment in a patient with known homozygous sickle cell anemia is demonstrated.     

Mitral Valve Replacement in Sickle C Disease Using Intraoperative Exchange Transfusion

Abstract Sickle C (SC) disease is a relatively uncommon hematologic disorder that poses special challenges when the patient requires a major surgical procedure. In particular, those who have a history of hemolytic crises require some type of intervention, usually homologous transfusion, to decrease the level of circulating hemoglobin S (HbS) and prevent intraoperative sickle crisis. We describe a 25-year-old man with SC disease and a history of multiple sickle cell crises who underwent mitral valve replacement using intraoperative exchange transfusion to decrease his HbS level from 53% to 7%. (J Card Surg 1998; 73:48–50)     

Evaluation of clinical and laboratory correlates of sickle leg ulcers

The exact mechanism for the occurrence of sickle leg ulcers (SLUs) has not been fully explained, although, popular opinion supports a multifactorial etio‐pathogenetic process. Leg ulceration in sickle cell is a chronic and debilitating condition which is difficult to treat and may worsen the psychosocial impact of this illness. This study aims to evaluate the laboratory and clinical correlates of SLUs. One hundred sixty‐seven patients who had been diagnosed with sickle cell anemia (homozygous S) had their steady‐state hemoglobin concentration (Hb), hematocrit, white cell count, platelet count, serum bilirubin, and aspartate transaminase (AST) as well as frequency of crisis per annum evaluated with respect to their relationship to the occurrence of leg ulcers. They were aged 6–53 years (mean age 24.3 years), and prevalence of leg ulcer was found to be 2.75 per 1000 (2.54 per 1000 in females and 2.83 per 1000 in males). The independent sample t‐test showed a significant difference in the serum AST levels in those with SLU (p = 0.029), though a positive correlation did not exist. Other predictors of disease severity found to have positive relationship with each other were the AST and total serum bilirubin 0.207 (p = 0.012); Hb and age 0.130 (p = 0.035); Hb and white cell count ?0.159 (p = 0.010), white cell count and age ?0.113 (p = 0.018). SLUs do not occur in patients with severe disease in sickle cell. The clinical and laboratory indicators of severe sickle cell disease do not correlate positively with the occurrence of SLU. Serum AST may have a relationship with leg ulceration in these patients. Environmental factors most likely play a major part in the etiopathogenesis of leg ulcer and this may require further studies in different sociocultural settings.     

Liver transplantation in a patient with S(beta)o-thalassemia

BACKGROUND: Patients presenting sickle cell disease may develop different types of hepatic complications. Intrahepatic cholestasis is a potentially fatal complication of the disease, and sometimes the only possible solution is transplantation. Postoperative transfusion management has not yet been well established. In this report, we describe the transfusional program of a patient presenting sickle cell disease and intrahepatic cholestasis who underwent liver transplantation 2 years ago. METHODS: Data were obtained from the chart and the blood bank records. RESULTS: The liver transplantation was performed successfully. Despite mild allograft dysfunction 3 months after surgery, secondary to intrahepatic sickling, the patient has been doing well with the transfusional management adopted (sickle-cell hemoglobin <20%). CONCLUSION: Sickle cell disease should not be a criterion for exclusion from liver transplantation. Regular transfusion with monitoring of sickle-cell hemoglobin is a very important measure to minimize the risk of intrahepatic sickling and possible rejection.     

Blood transfusion in patients with sickle cell disease requiring laparoscopic cholecystectomy

Background:

Surgery in patients with sickle cell disease is associated with high morbidity. To reduce this high morbidity, different preoperative transfusion regimens were introduced. However, blood transfusion is associated with problems. This prospective study aims to establish the safety of conducting laparoscopic cholecystectomy without transfusion in sickle cell disease patients.

Methods:

Forty patients (16 males and 24 females; mean age 26.6 years) undergoing laparoscopic cholecystectomy for cholelithiasis were divided into 2 matched groups: Group I “no transfusion” (n=24 patients; 60%) and Group II “transfusion” (n=16; 40%). In Group II, 9 patients (22.5%) received a simple transfusion and 7 (17.5%) a partial exchange transfusion.

Results:

Group II patients had significantly higher levels of Hb-S prior to transfusion. They developed a significantly higher complication rate (25% vs. 0%) and subsequently longer hospital stay (3.9±2 vs. 2.1±1.4). Moreover, there was no significant difference in the complications between the simple transfusion and partial exchange transfusion subgroups.

Conclusion:

Surgery in SCD patients is safe without a preoperative blood transfusion. Moreover, preoperative blood transfusion is associated with significantly higher postoperative complications and longer hospital stay. Hence, a “no transfusion” policy is recommended.     

Coronary artery bypass graft surgery in a patient with haemoglobin SC disease

Patients with sickle cell disease who undergo surgery are generally considered to be at greater risk of peri-operative complications than otherwise healthy patients. We report a case of a woman with haemoglobin SC disease undergoing coronary artery bypass grafting. She was successfully managed with pre-operative exchange transfusion and normothermic cardiopulmonary bypass.     

The role of laparoscopic cholecystectomy in the management of acute cholecystitis in patients with sickle cell disease

BACKGROUND: As emergency surgery in sickle cell disease patients is associated with high morbidity, the aim of the study was to assess the safety of laparoscopic cholecystectomy in the acute state for these patients. METHODS: Over a 5-year period, April 1994 till December 1998, 35 sickle cell patients with acute cholecystitis had laparoscopic cholecystectomy within the first 5 days of presentation. A retrospective study of these was undertaken. RESULTS: Thirty-five patients were diagnosed as having acute cholecystitis with sickle cell disease. There were 26 female and 9 male patients; 5 patients needed preoperative and 1 patient needed postoperative endoscopic retrograde cholangiopancreatography. Twenty-seven patients needed simple transfusion and 8 needed partial exchange; conversion was necessary in two cases (5.7%). The mean hospital stay was 5.3 days and the complication rate was 17.5%. CONCLUSIONS: Because of the lack of significant complications, we believe that laparoscopic cholecystectomy for acute cholecystitis is safe and recommended in experienced hands with adequate preoperative preparation for patients with sickle cell disease.     

Management of the Dialysis Patient with Sickle Cell Disease

While patients with sickle cell disease currently constitute a very small minority of the US dialysis population (0.1%), there is anticipated growth of this group as the life expectancy of those with sickle cell disease (SCD) increases. SCD patients suffer a high burden of morbidity, which is enhanced by the presence of end‐stage renal disease (ESRD). In this review, we discuss the pathophysiology of SCD and the basic tenets of its management with focus on the dialysis patient with SCD. Anemia in dialysis patients with SCD is a unique challenge. The hemoglobin target in SCD dialysis patients with ESRD should not exceed 10 g/dl. SCD patients, and particularly those on dialysis, are likely to be poorly responsive to erythropoietin‐stimulating agent (ESA) therapy and might be at increased risk for vaso‐occlusive crisis (VOC) with ESA. Iron chelation and hydroyxurea therapy require special considerations and modifications in dialysis patients with SCD. There are theoretical advantages to both hemodialysis (HD) and peritoneal dialysis (PD) in SCD patients. With HD, there is a secure vascular access available for both standard and exchange blood transfusion in patients who need them. With PD, the absence of an acute rise in hematocrit with ultrafiltration (UF) might offer lower risk of VOC. During VOC, reduction in UF goals should be considered but administration of intravenous fluids should be reserved only for clear cases of volume depletion. Finally, renal transplantation appears to confer a survival advantage to dialysis in SCD patients and should be pursued when possible.     

Acute chest syndrome after abdominal surgery in children with sickle cell disease: Is a laparoscopic approach better?

BACKGROUND/PURPOSE: Acute chest syndrome (ACS) is the leading cause of hospitalization and death among patients with sickle cell disease (SCD). Surgery is a risk factor for the development of ACS. It has been suggested that laparoscopic surgery could diminish the risk of sickle-related complications; therefore, more procedures may be encouraged in asymptomatic patients. The goal of the authors was to determine the incidence of postoperative ACS and assess for predisposing factors in all sickle cell patients undergoing abdominal surgery. METHODS: A retrospective analysis of all sickle cell patients receiving abdominal surgery (open and laparoscopic) between 1994 and 1998 was conducted. Data pertaining to demographics, perioperative clinical status, postoperative care, and outcome were collected and analyzed using Student's t test or chi(2) where appropriate. RESULTS: Fifty-four children underwent 62 procedures (35 abdominal and 27 extracavitary). All abdominal cases were either cholecystectomy or splenectomy (22 laparoscopic and 13 open). ACS occurred in 7 of 62 (11.3%) overall, and all were in abdominal cases 7 of 35 (20%). ACS occurred in 5 of 22 (22.7%) laparoscopic cases and 2 of 13 (15.4%) open cases. Operating time was significantly longer in the laparoscopic group compared with open cases (P <.05). A higher percentage of patients who had ACS had at least 1 previous episode (71.4% v 39.3%; P value not significant) and a smaller percentage of ACS patients received a preoperative blood transfusion (14.3% v 32.1%; P value not significant). Postoperative hospitalization was prolonged if ACS occurred (9 +/- 2 v 3 +/- 2 days; P <.05). CONCLUSIONS: Abdominal surgery carries a significantly high risk (20%) of ACS. Laparoscopy does not decrease the incidence of ACS compared with open approach. Predisposing factors were not significant in predicting postoperative ACS. There is considerable morbidity and potential cost implications in patients with ACS.     

Cardiopulmonary bypass for surgical correction of congenital heart disease in children with sickle cell disease: a case series

We present a series of three children with sickle cell disease aged 3 months, 3 weeks and 18 months, all presenting for cardiac surgery requiring cardiopulmonary bypass. The cardiac lesions were atrioventricular septal defect, transposition of the great arteries and ventricular septal defect, with sickle cell loads of 35%, 11% and 39% respectively at presentation. We calculated that the bypass circuit would provide sufficient volume to decrease sickle cell levels to safe values, so we decided to proceed to bypass without pre-operative exchange transfusion, and modified the bypass technique so as to avoid the likely stimulants of a sickle cell crisis. Haemoglobin S levels after the start of bypass were significantly lower than before bypass, and remained low throughout the case and into the second postoperative day. By adopting this approach, we feel that we achieved a successful outcome with minimal distress to the children and their families.     

Bilateral total knee replacement with tourniquets in a homozygous sickle cell patient

A 27-yr-old male patient, with homozygous sickle cell disease was scheduled for bilateral total knee replacement under tourniquet. The use of tourniquet in sickle cell patients is not without hazard. After preoperative exchange transfusion, total knee replacement was performed. The patient tolerated the procedure well. Patients with sickle cell disease should not be denied the benefit of a tourniquet if hematological correction has been undertaken. IMPLICATIONS: The use of a tourniquet in patients with sickle cell is controversial. The author describes a case of bilateral total knee replacement performed using a tourniquet in a patient with sickle cell disease.     

Guideline on the peri-operative management of patients with sickle cell disease

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.     

Operative blood loss and use of blood products after laparoscopic and conventional open colorectal operations

HYPOTHESIS: Blood loss, measured by estimated blood loss, drop in hemoglobin levels, and transfusion requirements, is lower in patients undergoing laparoscopic colectomy compared with patients undergoing conventional open colectomy. DESIGN: Case-matched study. SETTING: A university hospital. PATIENTS: Patients undergoing laparoscopic colectomy between January 2000 and December 2001 were matched in a prospective database for age, sex, comorbidity, and surgical procedure with patients undergoing open colectomy during the same period. MAIN OUTCOME MEASURES: Estimated blood loss, drop in hemoglobin levels, and transfusion requirements after surgery were compared. RESULTS: One hundred forty-seven patients undergoing the same operation using either an open or laparoscopic approach could be matched for age, sex, and diagnosis related grouping. There was no significant difference in American Society of Anesthesiologists class, body mass index, or preoperative and postoperative hemoglobin levels, but the open colectomy group required significantly more units of blood (P =.003) to maintain similar hemoglobin levels after surgery. Estimated blood loss (P<.001) and the number of patients who received transfusions on the day of surgery (P =.002), during the first 48 hours after surgery (P =.005), and during the entire hospital stay (P =.003) were significantly higher in the open colectomy group. CONCLUSION: A laparoscopic approach for colorectal surgery led to significantly less blood loss than matched open colectomy cases.     

Exchange transfusions in sepsis after the pediatric open heart surgery]

We experienced 2 cases of septic children after open heart surgery. Both of them recovered by using exchange blood transfusion technique. We use irradiated and dialysed fresh blood for exchange blood transfusion. After this procedure, they recovered from sepsis, as the datas improving, white blood cell reduced from 19,700 +/- 3,710 to 8,200 +/- 2,360, CRP reduced from 5.46 +/- 1.65 to 1.89 +/- 0.70, T-Bil reduced from 7.61 +/- 2.66 to 3.02 +/- 0.89, and BUN reduced from 525.92 +/- 6.64 to 19.76 +/- 5.34. Furthermore, blood pressure and urine volume were stable between exchange blood transfusion, although after open heart surgery. Therefore this procedure has benefits for the compromised, septic patients, performed open heart surgery, because of its stability of the circulating circumstances. And using the irradiated and dialysed fresh blood provides stable condition eventhough under high dose catecholamine use.     

Preoperative Epoetin Alfa in Colorectal Surgery: A Randomized,Controlled Study

Background Colorectal cancer patients are often anemic before surgery, and this leads to an increased requirement for allogeneic blood transfusion. This may result in transfusion-induced immunosuppression, which in turn leads to increased morbidity and possibly an increased rate of tumor relapse. We investigated the possible benefits of perioperative epoetin alfa administration in anemic patients to correct hemoglobin levels and reduce transfusion needs.Methods A total of 223 colorectal cancer patients with anemia scheduled for surgery were randomized to a group that received epoetin alfa 150 or 300 IU/kg/day subcutaneously for 12 days (day -10 to +1) or to a control group. All received iron (200 mg/day by mouth) for 10 days before surgery. Hemoglobin levels, hematocrit, and the number of blood units transfused were recorded.Results A total of 204 patients were eligible for analysis. Mean hemoglobin levels and hematocrit were significantly higher in the 300 IU/kg group than in the control group, both 1 day before surgery (hemoglobin, P = .008; hematocrit, P = .0005) and 1 day after surgery (hemoglobin, P = .011; hematocrit, P = .0008). Blood loss during and after surgery was similar in all groups. Patients who received epoetin alfa 300 IU/kg required significantly fewer perioperative transfusion units than control patients (.81 vs. 1.32; P = .016) and significantly fewer postoperative units (.87 vs. 1.33; P = .023). There were no significant differences in the number of units in the 150 IU/kg group.Conclusions Preoperative epoetin alfa (300 IU/day) increases hemoglobin levels and hematocrit in colorectal surgery patients. These effects are associated with a reduced need for perioperative and postoperative transfusions.See Appendix 1 for a list of investigators in the Hellenic Surgical Oncology Perioperative EPO Study Group.     

Relationship between changes in thyroid hormone level and severity of the postoperative course in neonates undergoing open-heart surgery

Background: Our aim was to determine whether the changes in thyroid function after open‐heart surgery in neonates depend on the postoperative course. Methods: Twenty neonates undergoing open‐heart surgery for congenital heart disease were prospectively studied in the cardiac intensive care unit of a university‐affiliated children's hospital. The patients were divided into two groups by level of inotropic support (high or mild). Results: The groups were similar in age, bypass time and ultrafiltration volume. In both groups, there was a significant reduction in levels of thyroid‐stimulating hormone and FT₄ at 24 h postoperatively. However, in the high inotropic support group, FT₄ was lower for a longer time. This group also had a significantly higher score on The Pediatric Risk of Mortality (PRISM; P < 0.042) and a longer duration of ventilation (P < 0.014). Conclusions: Neonates after open‐heart surgery undergo changes in thyroid function characteristic of euthyroid sick syndrome. The degree of hypothyroxinemia may be related to the severity of illness and the postoperative course.