Medical Mystery of the Week

 A previously healthy 25-year-old Romanian man develops the sudden onset of epigastric and right upper quadrant pain while lifting a heavy box. He describes a simultaneous warm feeling throughout his body accompanied by paresthesias involving his lips and extremities. Shortly thereafter his vision blurs and darkens, he vomits nonbilious material, and collapses in a state of unconsciousness. Emergency personnel find him to be unresponsive and diaphoretic with a blood pressure of 52/27 and a regular pulse rate of 90 beats per minute. He has been incontinent of urine. His blood pressure responds to plasma expanders and he eventually regains consciousness. Abdominal exam reveals generalized tenderness, greatest in the epigastrium and right upper quadrant. His abdominal CT scan is shown below.

CT abdomen

DIAGNOSIS: Anaphylaxis following leakage of an hepatic hydatid (echinococcal) cyst. Humans acquire the disease by ingesting infected embryonic eggs shed by the definitive hosts of E. granulosus - dogs and other canines. The eggs, which contain infectious oncospheres, penetrate the intestinal wall, enter the bloodstream, and lodge in the liver where they develop into hydatid cysts. Cysts can also occur in the brain, lung, bones, heart and muscle. About 25% of patients develop complications: cysts can rupture or leak their highly allergenic fluid into the peritoneal cavity precipitating, as in this patient, anaphylaxis; rupture into the biliary tree and cause fibrosis, obstruction, and enterocutaneous fistulas; cysts can also cause localizing symptoms when present in structures other than the liver. Treatment options include clinical observation, administration of antihelminthic agents (albendazole), percutaneous aspiration by means of the PAIR (puncture, aspirate, inject, and reaspirate) procedure, and surgery. This patient was treated with albendazole and praziquantel for one week, and then underwent surgery, which involved aspiration of the cyst, injection of hypertonic saline to lyse the organisms, reaspiration, and finally removal of the cavity.

This case was recently published in the NEJM (Murali MR, Uyeda JW, Tingpej B. 372:265-272, 2015) 

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