The correct answer is:

acute hepatic sequestration

Educational Objective:

Differentiate between causes of acute right upper quadrant pain in patients with sickle cell disease.

Key Point:

Abdominal complications causing right upper quadrant pain are common among patients with sickle cell disease, and several conditions may present similarly. Diagnosis requires careful assessment of laboratory work and imaging.

Explanation:

Abdominal complications in sickle cell disease are common, especially complications causing pain in the right upper quadrant. Cholecystitis, acute intrahepatic cholestasis, acute sickle hepatic crisis, and acute hepatic sequestration may present similarly. Differentiating between diagnoses requires evaluation of complete blood count, liver function tests, coagulation panel, and right upper quadrant ultrasonography.

Acute sickle hepatic crisis affects 10% of patients with sickle cell disease. Like cholecystitis, acute sickle hepatic crisis presents with right upper quadrant pain, fever, leukocytosis, and variable increases in serum transaminases and bilirubin levels; however, unlike cholecystitis, hepatomegaly also occurs. Treatment is supportive, with intravenous hydration and analgesia.

Acute hepatic sequestration occurs secondary to obstruction of sinusoidal flow by masses of sickled erythrocytes. In addition to right upper quadrant pain, fever, jaundice, and hepatomegaly, an acute drop in hemoglobin level with reticulocytosis occurs. Treatment focuses on transfusions to maintain blood volume.

Hemolysis precipitates the formation of pigmented gallstones in 70% of patients with sickle cell disease, increasing the risk of cholecystitis. Cholecystitis presents as right upper quadrant pain and tenderness, often with a positive Murphy sign, as well as leukocytosis and possibly mild transaminitis. Right upper quadrant ultrasonography will reveal gallstones and gallbladder distension, and, in the late stages, gallbladder wall thickening and pericholecystic fluid. As with adults, surgical consultation is indicated.

Acute intrahepatic cholestasis may represent a severe variant of acute sickle hepatic crisis. It occurs when widespread sickling within the hepatic sinusoids leads to vascular stasis and ischemia. Hypoxic damage leads to ballooning of hepatocytes and intracanalicular cholestasis. The presentation is initially similar to acute sickle hepatic crisis; however, striking jaundice develops, accompanied frequently by renal impairment, bleeding diathesis, and increasing encephalopathy. Patients with acute intrahepatic cholestasis often require exchange transfusion.

References :

Simon E, Long B, Koyfman A. Emergency medicine management of sickle cell disease complications; an evidence-based update. J Emerg Med,. 2016;51(4):370-381.

Subramaniam S, Chao JH. Managing acute complications of sickle cell disease in pediatric patients. Pediatr Emerg Med Pract. 2016;13(11):1-28.

Maloney PJ. Gastrointestinal Disorders. (Chapter 171) In: Walls R, et al. Rosen’s Emergency Medicine: Concepts and Clinical Practice. 9th ed., 2018: 2126 -2144.e2