What peri‑operative complications occur if a patient with sickle cell disease undergoes surgery without pre‑operative transfusion?

Perioperative Complications in Sickle Cell Disease Without Pre-operative Transfusion

Patients with sickle cell disease who undergo surgery without pre-operative transfusion face significantly elevated risks of vaso-occlusive crisis, acute chest syndrome, stroke, acute kidney injury, and death, with overall peri-operative complication rates substantially higher than the general surgical population. 1

Risk Stratification by Surgical Procedure

The complication risk varies dramatically based on surgical complexity:

Low-Risk Procedures

• Surgery involving eyes, skin, nose, ears, distal extremities, dental, perineal, and inguinal areas (e.g., inguinal hernia repair, myringotomy, dilatation and curettage) 1
• For HbSS/HbSβ0 patients with baseline Hb ≥90 g/L, proceeding without transfusion may be reasonable for these procedures 1
• However, even in low-risk surgery, untransfused HbSS patients had a 12.9% complication rate versus 4.8% with transfusion 2

Moderate-Risk Procedures

• Surgery involving throat, neck, spine, proximal extremities, genito-urinary system, and intra-abdominal areas (caesarean section, splenectomy, cholecystectomy, hip replacement) 1
• Pulmonary complications are especially prevalent after thoracotomy, laparotomy, or tonsillectomy/adenoidectomy (31% complication rate) 3
• For these procedures, proceeding without transfusion significantly increases risk 1

High-Risk Procedures

• All genotypes require exchange transfusion targeting Hb of 100 g/L regardless of baseline hemoglobin 1
• Proceeding without transfusion is strongly contraindicated 1

Specific Complications Without Pre-operative Transfusion

Sickle Cell-Related Complications

Vaso-occlusive crisis is the most common complication:

• Triggered by hypoxia, hypothermia, acidosis, dehydration, and pain—all common perioperative stressors 1
• Patients are at increased risk throughout the perioperative period, with the majority of complications occurring postoperatively 1

Acute chest syndrome develops in approximately 4-5% of surgical patients across all transfusion groups 4, 5:

• Can develop rapidly and requires immediate escalation to intensive care 1
• Regular SpO2 monitoring provides early warning 5
• Risk is particularly high after thoracic or upper abdominal procedures 3

Stroke can occur as an acute perioperative complication:

• Any acute neurologic symptom beyond transient mild headache requires urgent evaluation 5
• Patients may require emergency exchange transfusion 1

Acute kidney injury is a recognized perioperative complication 6:

• Exacerbated by volume depletion, which patients with sickle cell disease are particularly vulnerable to due to impaired urinary concentrating ability 5

Non-Sickle Cell Complications

Postoperative infections occur at higher rates:

• Patients have functional hyposplenism making them susceptible to bacterial infection from encapsulated organisms 1
• Any fever ≥38.0°C requires immediate blood cultures and broad-spectrum antibiotics (e.g., ceftriaxone) 7

Venous thromboembolism risk is elevated:

• Thromboprophylaxis should be routine for all peri- and post-pubertal patients 5

Congestive heart failure and cardiovascular complications are more common 6

Mortality Risk

Overall 30-day mortality is 1.1%, with 0.3% directly related to surgery/anesthesia 2:

• This is substantially higher than the general surgical population 1
• No deaths occurred in patients younger than 14 years in one large study 2
• Maternal mortality in pregnant patients with sickle cell disease is 1-3%, far exceeding general obstetric mortality 1

Evidence on Transfusion Benefit

The evidence strongly supports pre-operative transfusion for most procedures:

• For HbSS patients undergoing low-risk procedures, perioperative transfusion reduced complications from 12.9% to 4.8% 2
• For HbSC patients, pre-operative transfusion was beneficial for all surgical risk levels 2
• One study found 14.1% overall postoperative complication rate, with notably all patients who developed complications had preoperative HbS >40% 4

However, the same study paradoxically found higher crisis rates in exchange-transfused patients (22.2%) versus non-transfused (4.3%), though this likely reflects selection bias as sicker patients received transfusions 4.

Emergency Surgery Considerations

For emergency surgery with Hb ≥90 g/L and low surgical risk, it is reasonable to proceed without delay and transfuse intra- or postoperatively if necessary 1:

• If Hb is low, give simple top-up transfusion to target 100 g/L provided this will not delay surgery 1
• Blood should be available on site even if transfusion is not initially planned 1

Critical Pitfall

The risks of proceeding without blood transfusion are higher than in non-sickle patients, particularly for moderate- and high-risk procedures 1. The decision to forego transfusion should only be made after careful discussion with a haematologist, documentation of risks, and consideration of alternatives such as pre-operative hydroxycarbamide or erythropoietin-stimulating agents 1.