Komplikationen und Management einer Echinococcuscyste der Leber

A 24-year-old woman, who had recently emigrated to the United States from Kyrgyzstan, presented with a 6- to 8- month history of mild epigastric and right upper quadrant discomfort. The patient had no jaundice, fevers, chills or nausea. Imaging at an outside facility showed a 12×12×11-cm encapsulated cyst in the right hepatic lobe, and echinococcal serologies were positive. The patient was started on albendazole therapy, and later underwent percutaneous drainage and attempted cyst ablation with hypertonic saline administration, the Percutaneous Aspiration, Infusion of scolicidal agents and Reaspiration (PAIR) procedure. During this procedure the cyst was self-contained with no evidence of communication between the cyst and the biliary system. The patient had serial ablations with hypertonic saline and drainage, resulting in some decrease in the size of the cyst.
Five months after the fourth and last procedure she was admitted to our hospital with increasing abdominal pain and jaundice, attributed to either partial obstruction from the cyst or cholestasis secondary to isoniazid therapy (initiated for a positive PPD test 7 months before admission). Abdominal CT showed a septated 10.6×8.8-cm right hepatic lobe rim-enhancing hypodensity compatible with the echinococcal cyst and mild intrahepatic biliary ductal dilatation (A), and ductal dilatation of the proximal common bile duct (CBD) to approximately 1.8 cm. The irregularly walled cyst appeared to communicate with the CBD with suspected extrusion of cystic membranes into the CBD.

An ERCP (B) showed extravasation of contrast from the right intrahepatic duct into the intrahepatic cyst and mild intra- and extrahepatic ductal dilation. A biliary stent was placed, resulting in purulent drainage into the duodenum. A percutaneous catheter placed into the abscess resulted in improvement in the patient's clinical symptoms.

The patient later had a partial hepatic resection (C, in situ cyst within the liver with a transected percutaneous catheter is shown exiting the parenchyma).

The specimen (D) contained an 11×6.3×4.0-cm cavity circumscribed with a focally hemorrhagic fibrous wall. The lining of the cavity was golden tan and slightly undulated. Focal peliosis hepatis and an acellular hyalin-laminated cyst wall were also noted. Surgical pathology found no scolices within the cyst specimen.

Hydatid disease is caused by a cestode called Echinococcus granulosus and can be found worldwide, especially in grazing areas where dogs (the definitive host) have access to intermediate hosts such as sheep, goats, other herbivores, and occasionally humans. This condition is rarely found in the U.S., but is endemic in Central Asia, South America, and the Mediterranean basin.1 Clinical management of hepatic cysts often includes albendazole therapy in combination with either surgery or the PAIR procedure. With larger liver cysts (diameter >10 cm), such as the one in this patient, resection remains the preferred option.
In this patient, the repeated manipulations of the echinococcal cyst with PAIR and hypertonic saline allowed a fistula to form with the biliary system, resulting in her clinical deterioration. It is important to remember that using protoscolicidal agents in the setting of a biliary fistula can cause sclerosing cholangitis or pancreatitis.2 Surgical management remains an important early intervention for patients with complex larger cysts.

References

1 J. Eckert and P. Deplazes, Biological, epidemiological and clinical aspects of echinococcosis, a zoonosis of increasing concern, Clin Microbiol Rev 17 (2004), pp. 107–135.
2 W.S. Kammerer and P.M. Schantz, Echinococcal disease, Infect Dis Clin North Am 7 (1993), p. 605.

Yiqing Sheng and David A. Gerber
Journal of the American College of Surgons Volume 206, Issu 6, June 2008, Pages 1222-1223