From the Guidelines
Patients with sickle cell anemia (SCA) commonly develop a gallbladder with pigment gallstones, often referred to as a "sickle cell gallbladder". These gallstones form due to the chronic hemolysis that occurs in SCA, resulting in increased bilirubin production, as noted in the context of chronic manifestations of SCD 1. The excess bilirubin is processed by the liver and excreted into bile, where it can precipitate and form pigment stones. Key characteristics of these gallstones include:
- Typically appearing black or dark brown
- Composed primarily of calcium bilirubinate
- Often beginning to form in childhood, with prevalence increasing with age
- Found in 30-70% of SCA patients by adulthood The gallbladder itself may show signs of chronic inflammation and wall thickening due to the presence of these stones. Evaluation for gallstones is indicated for a child with SCD and right upper quadrant pain, frequent episodes of vomiting, and/or worsening jaundice, as suggested by guidelines for health supervision in children with SCD 1. Management typically involves cholecystectomy for symptomatic cases.
From the Research
Gallbladder Type in SCA Patients
- The type of gallbladder in SCA patients is often associated with gallstones, which are usually pigment stones 2.
- Studies have shown that patients with SCA are at a higher risk of developing gallstones, with a frequency of gallstones increasing with age 3, 4.
- The presence of gallstones in SCA patients can lead to complications such as chronic cholecystitis, biliary obstruction, and increased morbidity 4, 5, 2.
- Elective cholecystectomy is often recommended for SCA patients with gallstones to prevent these complications and reduce morbidity 3, 4, 5, 6.
Characteristics of Gallbladder Disease in SCA Patients
- Gallbladder disease in SCA patients is often characterized by chronic cholecystitis, which can have a variable radiologic appearance 4.
- Patients with SCA and gallbladder disease may not always exhibit symptoms, and histopathologic evidence of cholecystitis does not always correlate with clinical symptoms 5.
- Ultrasound examination is often used to diagnose gallbladder abnormalities in SCA patients, and can detect sludge or stones in a significant proportion of patients 4, 5.
Treatment and Management of Gallbladder Disease in SCA Patients
- Elective cholecystectomy is a common treatment for gallbladder disease in SCA patients, and can be safely performed with judicious use of preoperative transfusion 3, 4, 5, 6.
- Early cholecystectomy is advisable to avoid the morbid sequelae of acute cholecystitis and peroperative sickle cell crisis 6.
- Laparoscopic cholecystectomy may be a suitable option for SCA patients with gallbladder disease, and can reduce hospital stay and morbidity 5.