AIM: To evaluate the role of endoscopic retrograde cholangiopancreatography (ERCP) in patients with sickle cell disease (SCD).

Two hundred and twenty four patients with SCD underwent 240 ERCP procedures. There were 144 males and 80 females. Their mean age was 22.4 years (5-70 years). Their mean hemoglobin S (HbS) was 76.8% (64.7%-92.3%) and their mean hemoglobin F (HbF) was 20.4% (5.1%-34.0%). Their mean total bilirubin was 224 g/L (55-395 g/L). Their mean direct bilirubin was 134 g/L (40-263 g/L). Their mean alkaline phosphatase was 486 IU/mL (81-1189 IU/mL) (Normal: 50-136 IU/mL). Their mean alanine transaminase (ALT) was 234.3 IU/mL (50-761 IU/mL) (Normal: 30-56 IU/mL) and their mean aspartate transaminase (AST) was 206.3 IU/mL (63-317 IU/mL) (Normal: 15-37 IU/mL). The indications for ERCP were: CJ only in 97, CJ and dilated bile ducts on ultrasound in 103, and CJ and bile duct stones on ultrasound in 42. The ERCP findings in each of these three groups are shown in Tables ​Tables1,1, ​,2,2, ​,3.3. In those with CJ only, there was a group of 13 patients (16.5%) with dilated bile ducts without an obstructive cause. In those with CJ and dilated bile ducts on ultrasound, there was a group of 28 patients (27.2%) with dilated bile ducts without an obstructive cause. In those with CJ and bile duct stones on ultrasound, there was a group of 14 patients (33.3%) with dilated bile ducts without an obstructive cause. In total 55 patients (24.6%) had dilated bile ducts without an obstructive cause or previous history of biliary duct stones (Figures ​(Figures11 and ​and22).

Sickle cell disease is one of the common hemoglobinopathies in the Eastern Province of Saudi Arabia, where the frequency of Sickle cell trait can reach as high as 25% in some areas[^1-3]. One of the common manifestations of SCD is jaundice, which can be caused by a variety of hepatobiliary diseases including CJ[^3-5]. There are certain causes of CJ that are SCD related. One of these causes is intrahepatic sickling of RBC[^4,5,9]. This is also called hepatic crisis or hepatic sequestration (sickle cell hepatopathy)[^4,5]. This can lead to cholestasis and a clinical picture that can resemble extrahepatic bile duct obstruction which causes diagnostic and therapeutic dilemmas. Sickle cell intrahepatic cholestasis on the other hand is a more serious condition, characterized by acute onset of hepatomegaly, hyperbilirubinemia, coagulopathy, and acute liver failure[^4,5]. Early identification of these is important as the process of sickling can be reversed by hydration and simple, or in severe cases, exchange blood transfusion. There is also a high frequency of cholelithiasis and choledocholithiasis in patients with SCD[^3,6-8]. The frequency of cholelithiasis in patients with SCD is variable, ranging from 4% to 55%, and this increases with age[^2,3,6-8]. In the general population with cholelithiasis, the incidence of common bile duct (CBD) stones has been reported to be 10%-15%, whereas in those with SCD it ranges from 18%-30%[^10,11]. Due to this high incidence, routine intraoperative cholangiography has been advocated[¹⁰]. With the recent advances in laparoscopic cholecystectomy (LC), exclusion of CBD stones prior to LC is of great importance. ERCP has been shown to be valuable, both for the diagnosis and management of CBD stones, in patients with SCD who are undergoing or have undergone LC[^12-14]. ERCP is also of great importance in evaluating SCD patients with CJ, whether this is due to intrahepatic or extrahepatic causes. Ultrasound is a simple, non invasive imaging technique, and although gallstones and intrahepatic and extrahepatic bile duct dilatation are readily detected by ultrasound, common bile duct stones might be missed. ERCP, on the other hand, is more invasive but is the procedure of choice in suspected cases of extrahepatic bile duct obstruction. It provides direct visualization of the biliary tree and demonstrates the site and nature of the obstruction in more than 90% of patients. ERCP also provides therapeutic interventions, including endoscopic sphincterotomy and stone extraction, dilatation of strictures, and placement of stents and biliary drainage catheters[^15-19]. This was the case in our series, where we found ERCP valuable both as a diagnostic and therapeutic procedure. The majority of bile duct stones (95.4%) in our series were removed via ERCP. ERCP however was normal and unnecessary in a significant number of our patients (27%) with SCD and CJ. This was specially so in those who presented with CJ only (53.2%). These patients most likely had CJ secondary to intrahepatic sickling of RBC. Hepatic crisis and hepatic sequestration resemble each other clinically, and the only differentiating point between the two is a sudden drop in hematocrit, as well as a sudden increase in liver size in those with hepatic sequestration[^4,5]. Sickle cell intrahepatic cholestasis, on the other hand, is a more serious and often fatal complication of SCD where there is widespread intrahepatic sickling within the liver sinusoids leading to their blockage, vascular stasis with hepatic ischemia, and the striking feature is the highly elevated plasma bilirubin level[^4,5,12,13]. It is possible that our patients with CJ and normal ERCP represent a benign variant of intrahepatic cholestasis or a form of what is called benign hyperbilirubinemia[⁵]. All our patients had hyperbilirubinemia, but their plasma AST, ALT and alkaline phosphatase levels were only moderately elevated. To support this, these patients subsequently recovered with conservative treatment including observation, hydration and, where indicated, blood transfusion. To overcome this and decrease the number of patients with normal ERCP, patients with CJ only should be evaluated further prior to ERCP, including endoscopic ultrasound (EUS) and magnetic resonance cholangiopancreatography (MRCP). However, these investigations are not readily available, and in these situations a period of observation and conservative management is to be advocated.