Fig. 3.161 Alveolar hydatid disease involving both lobes of the liver in a 61-year-old Eskimo woman from northwestern Alaska. She was admitted to the hospital in 1970 for the fifth time for neurologic evaluation. Six weeks previously, she had an episode of clouded sensorium and bizarre behavior but returned to normal in a few days. Later she experienced a grand mal seizure. In 1960, she had a presumptive diagnosis of alveolar hydatid disease made at laparotomy: prior echinococcal titers had been positive. Upper gastrointestinal series in 1965 and 1968 showed enlargement of the left lobe of the liver and calcifications in both lobes (A). Radioisotope liver scans showed hepatomegaly with gross enlargement of the left lobe, in which there was a large mass with a central area of diminished uptake.
When admitted, she was cachectic but oriented. She had a mild fever of 100° and slight lateral nystagmus and ataxia. The liver was enlarged, chiefly in the epigastrium, but was not tender. The albumin-globulin ratio was reversed at 3.2:4.5 mg/ml. Echinococcal hemagglutination and flocculation titers were 1:4,096 and 1:320, respectively. Several lumbar punctures revealed normal pressures, 550-2,500 white blood cells per cu mm, of which 80% were lymphocytes, and protein which increased from 430 to 730 mg/100 ml. A pneumoencephalogram showed atrophy of cortical tissue and enlargement of the lateral ventricles and basilar cisterns. The fourth ventricle was not demonstrated. She had been on triple tuberculosis chemotherapy and steroids, but she gradually deteriorated and terminally developed left third, fourth, and seventh cranial nerve palsies.
At autopsy, the liver was markedly enlarged and the entire left lobe was replaced by a large cyst with a white fibrous wall 2-3 cm thick, lined by shaggy, yellow material, and with a large necrotic center (B). Hemisection of the right lobe of the liver revealed numerous white, somewhat lobulated, gritty nodules measuring 1 to 5 cm in diameter, one of which showed a necrotic center (C). A tumor-like mass was found in the left cerebellar-pontine angle and a similar lesion extended around the brain stem, deforming the midbrain and pons. These lesions resembled those in the liver and histologically showed the characteristic features of alveolar hydatid disease. (Courtesy of Dr. William Thompson, et al, and AJR, 1972).

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Fig. 3.162 Huge necrotic alveolar hydatid lesion in the liver of an Alaskan Eskimo who died from hepatic failure 6 months after these studies. (A) Tomogram of the right upper quadrant during an intravenous cholangiogram shows diffuse irregular calcifications within a huge mass in the midline of the liver which is displacing the common bile duct laterally (arrows). (B) A T-tube cholangiogram shows contrast medium has leaked from one of the hepatic ducts into this extensively necrotic, irregular, cystic lesion of the liver. Several branches of the right hepatic duct are filled, as well as the cystic duct and gallbladder (lower left). (C) An erect radiograph of the upper abdomen shows a prominent air-contrast level within this large lesion which has replaced most of the right lobe of the liver. The air above the contrast level shows the irregular necrotic nature of the lesion to good advantage. The right hemidiaphragm is markedly elevated. (D) An inferior vena cavogram shows complete obstruction of the inferior vena cava at the L2 level with collateral flow through dilated azygos, hemiazygos and lumbar veins. The irregular calcifications typical of E. multilocularis infection are again identified within the large liver lesion, which had extended posteriorly to block the vena cava. (Courtesy of Dr. William Thompson, et al, and AJR, 1972).

Fig. 3.163 Cholangiogram showing marked displacement of the common bile duct and right hepatic ducts towards the midline by a huge alveolar cystic lesion of the liver in an Eskimo. Some of the contrast medium has entered the necrotic lesion (arrow) via a branch from the right hepatic duct. The cystic duct and pancreatic ducts are well outlined. A few bizarre ring-like and irregular calcifications are seen within a portion of the liver lesion just to the right of the patient's midline (center of photo). (Courtesy of Dr. William Thompson, et al, and AJR, 1972).

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