Hepatobiliary complications in sickle cell disease range from gallbladder stones to life-threatening acute hepatic sequestration. For simplicity, we classify them as biliary and hepatic complications.
Complications of the biliary tract are not uncommon among patients living with sickle cell disease. Those that carry HbSS are at a higher risk. These include cholelithiasis, acute cholecystitis, biliary sludge, acute choledocholithiasis.
Chronic haemolysis occurs very often in sickle cell disease. Due to this, unconjugated bilirubin increases in the blood. It may crystallise, gallstones and sludge eventually form.
The prevalence of gallstones among people living with sickle cell disease increases with age, from 12% in those aged 2 to 4 years to 43% by age 15 to 16 years, and a staggering 70%-75% among adults. They are asymptomatic in many instances. Clinicians often identify them accidentally during abdominal ultrasonography for other ailments. Gallstones predispose to acute gallbladder infections and inflammation. In the worst of the scenarios, they can lead to obstruction of the cystic or bile ducts and acute pancreatitis.
Acute cholecystitis (acute inflammation of the bladder) is not as common as cholelithiasis (gallbladder stones). The former occurs in about 10% among people living with sickle cell disease. It can occur with or without the presence of gallstones. Acute cholecystitis commonly presents as severe colicky right upper quadrant pain with abdominal tenderness.
When the gallstones are present in the common bile duct, it is choledocholithiasis, and symptoms a dull pain in the right upper quadrant, tender enlarged liver, and a rapidly increasing jaundice. Choledocholithiasis is prevalent at 5% among people living with sickle cell disease with gallstones who are asymptomatic. It is, however, 60% among those who are symptomatic with gallstones. In many cases, clinicians employ endoscopic retrograde cholangiopancreatography and sphincterotomy to remove these stones.
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There are two acute hepatic complications of sickle cell disease – sickle cell hepatopathy. They include acute hepatic sequestration and acute intrahepatic cholestasis.
Much as it is uncommon, suspect acute hepatic sequestration in any person living with sickle cell disease who presents with sudden acute right upper quadrant pain with hepatomegaly and a 2 g/dL or more decline in haemoglobin concentration. Acute hepatic sequestration occurs when sickled red cells are sequestered in the liver, so fast that the liver becomes engorged with blood, leading to its enlargement which adds strain to the liver capsule leading to the perceived pain. Acute hepatic sequestration is hard to diagnose since many people living with sickle cell disease have mild baseline hepatomegaly. Closely monitoring the increase in the size of the liver is crucial during the work-up of the possible causes of acute right upper quadrant pain among people living with sickle cell disease. It is worth noting that the liver enzymes mildly elevate. Rule out the other causes of acute hemolysis. Once a patient suffers acute hepatic sequestration, chances of recurrence are very high.
On the other hand, acute intrahepatic cholestasis is a suspect when a person living with sickle cell disease presents with;
-sudden onset right upper quadrant pain,
-light-coloured stools, and
-extreme hyperbilirubinemia without urobilinogenuria.
Often, such a patient comes with thrombocytopenia and coagulopathies. Clinically, the patient has cholestatic jaundice. Common bile duct obstruction or cholangitis are non-existent. When not treated promptly, acute intrahepatic cholestasis is fatal.
Now that you have a clearer picture of the hepatobiliary complications of sickle cell disease, here are the recommendations to help you appropriately manage such patients. We adopted them from the panel of experts about evidence-based management of sickle cell disease in the National Heart, Blood and Lung Institute.
- Treat acute cholecystitis in children and adults with SCD with antibiotics and surgical consultation.
- Treat asymptomatic gallstones with watchful waiting in children and adults with SCD. In those who develop symptoms specific to gallstones, treat with cholecystectomy. The laparoscopic approach is preferable if surgically feasible and available.
- Consult with a haematologist or a sickle cell expert for any possible transfusion before surgery.
- In children and adults with SCD and features of sickle cell hepatopathy, provide hydration, rest, close observation, and consult a sickle cell expert for further management.
- In children and adults with SCD and signs and features of sickle cell hepatopathy, obtain an urgent consultation with a sickle cell disease expert for diagnosis confirmation.
- In children and adults with SCD with confirmed sickle cell hepatopathy, perform simple or exchange transfusion.
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