Astrid Sarvis MD, Children’s National Health System

The Case
18 yo M HbSS, Protein S deficiency, Asthma and Chronic Headaches presenting with 2 days of worsening bilateral ankle pain who became confused, was randomly repeating phrases, and intermittently not oriented to place and idea. The patient was also found to have a total bili of 20.

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Denouement
GI and hematology consulted in the emergency room. The patient was presumed to have encephalopathy secondary to conjugated hyperbilirubinemia from gallstones vs hepatic failure. Bolus repeated, narcotics discontinued and PICU called for admission. GGT resulted 234. All other labs including urine tox screen, ammonia, tylenol level, aspirin level, amylase and lipase and coags wnl. The pt was reportedly back to baseline mental status on arrival to the PICU and his bilirubin level decreased to 13 with 9.0 unconjugated within 24 hours.

Abdominal ultrasound done the next day showed gallstones and sludge with no evidence of cholecystitis or stones in the common bile duct or other bile duct pathology. However surgery felt that the patient’s conjugated hyperbilirubinemia warranted urgent ERCP to rule out CBD stone. GI performed ERCP on day 2 of hospitalization which showed 2 CBD stones s/p balloon extraction. Surgery performed cholecystectomy 2 days later. The pt had a benign post-operative course and was discharged home the day after surgery.

Teaching Points
If mental status/confusion in sickle cell patient, maintain a low threshold to rule out CVA.  An unenhanced CT scan will localize an area of hemorrhage immediately, but may not become abnormal for up to six hours after an infarct.  Also consider a CT angiogram if an MRI cannot be obtained.  MRI demonstrates areas of abnormality within two to four hours following an infarct.

The prevalence of gallstones in SCD is directly related to the rate of hemolysis.  Gallstones occur in children as young as three to four years of age and are eventually found in approximately 70 percent of patients. Most patients are asymptomatic, and expectant management is appropriate in those without symptoms.

Information above from UpToDate