How do I treat acute recurrent cholecystitis in a patient with sickle cell disease?

Management of Acute Recurrent Cholecystitis in Patients with Sickle Cell Disease

For patients with sickle cell disease and acute recurrent cholecystitis, laparoscopic cholecystectomy is recommended as the definitive treatment after appropriate preoperative optimization with hydration, oxygenation, and consideration of blood transfusion. 1, 2

Initial Management of Acute Cholecystitis in SCD

Stabilization and Medical Management

• Aggressive hydration is crucial, with careful monitoring of fluid balance to prevent overhydration 3
• Maintain adequate oxygenation with continuous monitoring of oxygen saturation, administering oxygen therapy to maintain SpO2 above baseline or 96% (whichever is higher) 4
• For severe pain, administer parenteral opioids such as morphine by scheduled around-the-clock dosing or patient-controlled analgesia 3
• Obtain blood cultures if fever is present, and initiate antibiotics if temperature reaches ≥38.0°C or if there are signs of sepsis 5

Antibiotic Selection

• Initial antibiotic therapy should cover gram-negative and gram-positive bacteria 4
• For mild episodes, an aminopenicillin/beta-lactamase inhibitor is appropriate as first-line therapy 4
• For more severe cases, intravenous antibiotics such as piperacillin/tazobactam or third-generation cephalosporins with anaerobic coverage are recommended 4
• Ceftriaxone is an appropriate choice for biliary infections, particularly for surgical prophylaxis in cholecystectomy 6

Preoperative Optimization for SCD Patients

Blood Transfusion Considerations

• Evaluate the need for preoperative blood transfusion based on the patient's baseline hemoglobin level and clinical status 1, 2
• Simple transfusion is appropriate for most patients, while partial exchange transfusion may be necessary in selected cases 1
• The risks of transfusion (including allo-immunization, which occurs in 7-30% of SCD patients) must be carefully weighed against benefits 4

Temperature Management

• Maintain normothermia as hypothermia can lead to shivering and peripheral stasis, which increases sickling 3
• Increase ambient temperature in the operating theater and use warmed fluids during surgery 4

Definitive Management

Surgical Approach

• Laparoscopic cholecystectomy is the preferred approach for acute cholecystitis in SCD patients, with conversion rates as low as 5.7% 1
• Surgery should be performed within the first 5 days of presentation when possible 1
• Perform brush cytology and/or endoscopic biopsy to exclude malignancy if dominant strictures are present 4

Timing of Surgery

• Early intervention is preferred over conservative management due to the high risk of complications with emergency surgery in SCD patients 1, 7
• Even asymptomatic cholelithiasis should be considered for elective cholecystectomy in SCD patients due to the high risk of eventual complications and to simplify medical management by eliminating diagnostic confusion between acute cholecystitis and sickle cell hepatobiliary crisis 7

Postoperative Care

Thromboprophylaxis

• Patients with SCD have an increased risk of deep vein thrombosis; therefore, thromboprophylaxis should be used for all peri- and post-pubertal patients 4
• Early mobilization should be encouraged postoperatively 4

Infection Prevention

• Monitor intravenous cannula sites regularly for phlebitis and remove immediately if there are signs of redness or swelling 4
• Provide chest physiotherapy if the patient is unable to mobilize to prevent respiratory complications 4

Monitoring for Complications

• Be vigilant for signs of acute chest syndrome, which can be precipitated by surgery or infection 5
• Monitor for recurrent biliary tract obstruction, which occurs in approximately 20% of SCD patients despite cholecystectomy 8

Special Considerations

Recurrent Bacterial Cholangitis

• For patients with recurrent bacterial cholangitis due to complex intrahepatic cholangiopathy, prophylactic long-term antibiotics (e.g., co-trimoxazole) and rotation of antibiotics may be considered in exceptional circumstances 4
• Biliary cultures and multidisciplinary expert assessment with formal microbiology advice is recommended for these complex cases 4

Fungal Infections

• Be aware that Candida species may be isolated from bile in patients with advanced disease and high-grade stenosis 4
• Persistent biliary candidiasis is associated with reduced transplantation-free survival and increased frequency of cholangiocarcinoma 4

Common Pitfalls and Caveats

• Delayed recognition of complications can lead to rapid progression to serious outcomes 3
• Overtransfusion should be avoided as it can lead to hyperviscosity and increased sickling 4
• The presence of sickle cell disease may mask symptoms of cholecystitis, leading to delayed diagnosis 9
• Recurrent biliary tract obstruction is common in SCD patients (20%) and often presents as common bile duct obstruction by stone, despite cholecystectomy 8