J Gastroenterol 1996; 31:885-888 Journal of- - - -

Gastroenterology �9 Springer-Verlag 1996

Case Report

A long-surviving patient with recurrences of hepatic alveolar echinococcosis after traumatic intra-abdominal rupture

NAOKI SATO, TSUTOMU NAMIENO, HIROAKI TAKAHASHI, KENICHIRO YAMASHITA, TADASHI MATSUHISA, SHIGERU AOKI, and JUNICHI UCHINO

First Department of Surgery, Hokkaido University School of Medicine, N-14, W-5, Kita-Ku, Sapporo 060, Japan

Abstract: Alveolar echinococcosis of the liver (AEL) takes a progressive and malignant course. Intra- abdominal dissemination of the parasite has a miserable outcome. Complete resection of the lesion is indispens- able for the curative treatment of AEL. We experienced an extremely rare case of intra-abdominal rupture of AEL. The patient had repeated recurrences of AEL following the traumatic rupture of the lesion. After re- peated resections of the lesions and appropriate medica- tion, the patient is still alive more than 25 years since the initial onset of the disease. AEL differs from unilocular echinococcosis in terms of biological behavior. We com- pare the pathophysiology of the two conditions.

Key words: alveolar recurrence, distant dissemination

echinococcosis, liver, rupture, metastasis, intra-abdominal

Introduction

In unilocular echinococcosis of the liver, an isolated large non-malignant cyst is formed; in alveolar echin- ococcosis of the liver (AEL) by contrast, solid orange- yellowish, tumor-like lesions are formed and the course is indolent but progressively malignant. In the advanced stage of AEL, the lesions are calcified and often form abscesses with gray-yellowish or muddy exudate. The lesions can infiltrate the surrounding tissues and metas- tasize to distant organs; these findings are frequent in patients with associated abscesses. Focal infection and severe cholangitis may be induced as a result of direct perforation of the biliary tree and gastrointestinal tract. To date, intra-abdominal rupture, which may occur in

Offprint requests" to: N. Sato (Received Dec. 22, 1995; accepted May 7, 1996)

unilocular echinococcosis, has not been reported in AEL.

We experienced an extremely rare case of AEL in a patient in whom the lesion suddenly ruptured, this oc- curring during a Judo exercise. Subsequently, after sur- gery, larvae were implantated in the surgical wound, and intra-abdominal dissemination and splenic meta- stasis occurred. However, with repeated resections of the recurrent lesions and appropriate medication, the patient is still alive more than 25 years since the initial rupture. Here we discuss the pathophysiology and the mechanism of shock in AEL compared with features in unilocular echinococcosis.

Case report

The patient was a 19-year-old man, born in 1953, the son of dairy farmer in a rural area in Hokkaido, Japan. Alveolar echinococcosis was and still is prevalent in the area. The patient's father also suffered from the disease; his lesions involved three hepatic segments, the dia- phragm, and the retroperitoneum, the involvement be- ing detected on imaging in 1985 at age 65 years. He underwent exploratory laparotomy, but died of liver failure in the following year.

The present patient suddenly experienced diffuse ab- dominal pain in June 1972, after he was hit hard in the abdomen during a Judo exercise. He lost consciousness 10 min after the blow and rapidly decompensated due to shock. He underwent an emergency laparotomy at a neighboring hospital, the presumed preoperative diag- nosis being peritonitis caused by perforation of the ap- pendix. A large amount of dark-brown pus was found in the abdominal cavity during the laparotomy, but the appendix appeared normal. Intra-abdominal irrigation and drainage were performed, but the cause of the pus discharge during this operation (first operation) was not determined.

886 N. Sato et al.: Survival after rupture of AEL

Fig. 1. Rupture of the echinococcal lesion in the right lobe of the liver. A, Internal wall of the ruptured lesion; B, fluid content; C, cut surface of the lesion wall; D, normal liver

Fig. 3. Metastatic echinococcal lesion on lateral edge of the spleen

Fig. 2. Transabdominal ultrasound shows a circumferential hypoechoic and central hyperechoic mass 20 mm in size (white arrow) in the lower part of the spleen; lateral shadows are also observed

The surgical wound leaked continuously. The extrac- tive discharge f rom the wound continued for almost 1 year after the surgery. In December 1973, a huge ab- scess, measuring 18 • 22cm, was found in the right lobe of the liver. Thoraco laparo tomy was pe r fo rmed in January 1974, and the abscess ruptured again; a large amount of pus again flowed into the abdominal cavity (Fig. 1). Right lobec tomy of the liver and intra- abdominal irrigation were then also per formed (second operation).

The patient was relatively comfortable after the sec- ond surgery. Ten years later, in November 1984, he visited our out patient clinic, presenting with a pus-like exudation f rom the surgical wound. There was an en-

Fig. 4. Many pearl-like glossy masses (arrowheads') are ob- served in the pouch of Douglas. Splenectomy and combined resection of the seeded lesions were performed through a thoracolaparotomy

largement of the chest wall around the wound. On physical examination, an elastic firm prominence with an irregular surface was observed; this was approxi- mately 7 cm in diameter and located at the center of the thoracoabdominal surgical wound, at a site correspond- ing to the costal arch. An autolyzed dermal fistula was observed in the center of this prominence. The mass was neither indurated nor erythematous. The patient was admit ted to Hokka ido University Hospital for evaluation and t reatment . Ul t rasonography (US) re- vealed a small hypoechoic area, the long axis measuring

N. Sato et al.: Survival after rupture of AEL 887

33mm, but no capsule was found in the subcutaneous region above the right costal arch. Strong echoes with acoustic shadows were sporadically noted in this area. Computed tomography (CT) showed a regenerated and hypertrophic left lobe of the liver without evidence of AEL. US did, however, reveal a 20mm cystic mass on the lateral edge of the spleen (Fig. 2). Through careful evaluation of the patient's surgical records and US findings, and noting of his place of birth and family occupation, we diagnosed alveolar echinococcosis, the series of episodes being regarded as recurrences of the disease. We performed thoracolaparotomy along the old incision of the second surgery. A hemispheric lesion was found 8cm in size, involving the muscular layers of the chest wall. The splenic metastases were white, elastic hard, and encapsulated, and located in the lower part of the spleen (Fig. 3). In the abdominal cav-

ity, several small lesions (2-6 mlTl in size) were detected on the parietal side of the greater omentum, and many smaller lesions (1ram or less) were scattered through- out the mesentery and retroperitoneum. Many pearl- like glossy masses of various sizes, ranging from several millimeters to as large as 3 cm, were found in the pouch of Douglas (Fig. 4). Splenectomy was performed and the chest wall mass was resected, together with as many intra-abdominal lesions as possible (third operation). Histology of the resected specimen showed multiple cysts covered with a cuticular layer in dense connective tissue (Fig. 5).

The mass in the chest wall recurred 1 year after the third operation, and enzyme-linked immunosorbent as- say (ELISA) in 1985 showed serum positivity for AEL. The lesion grew and was again resected in 1986 (fourth operation). In 1987, although neither US nor CT showed abnormal findings the ELISA values were un- stable and the patient 's serum was positive for A E L by Western blot. He was therefore treated orally with albendazole (600mg/day) in cycles of 4-weeks' intake and 2-weeks' intermission. Treatment continued for 2 years, from 1992 to 1993. As of August 1995, no appar- ent lesions have been demonstrated by various imaging modalities and ELISA values have been lowered, from 0.63 ELISA values are relative ones without any units in 1992 to 0.44 in 1995 (cut-off value < 0.5). Western blot test was negative for A E L in 1994, and the patient is in good health and working as a dairy farmer (Table 1).

Fig. 5. Multiple cysts are seen in the specimen; they are cov- ered with a cuticular layer in dense fibrous connective tissue. H&E, x 50

Discuss ion

The pathology of A E L is fundamentally different from that of unilocular echinococeosis of the liver. It is im- portant for the physician to recognize the different

Table 1. Clinical course

Year 1953 1972 1974 t984 1986 1 9 8 7 1992-1993 1995

Age (years)

Medical history

Surgery

Laboratory results T-Bil (mg/dI) ALP (IU/I) GOT 0U) GPT (IU)

ELISA (cut-off value <0.5)

Western blot

born 19 21

Traumatic Liver mass rupture (origin unknown)

Laparotomy Right lobectomy and drainage of the liver

1.0 623

31 33

Chest wall Chest wall mass recurrence

lntra-abdominal dissemination

Thoracolaparotomy Local resection local resection and splenectomy

34 38 39 41

Treatment with Alive albendazole

0.8 0.7 0.8 0.8 581 654 566 681

30 24 28 33 29 27

0.89 0.72 0.63 0.44

Positive Positive Negative

ELISA, enzymeqinked immunosorbent assay

888 N. Sato et al.: Survival after rupture of AEL

pathogeneses of these entities. Unilocular echinococcus grows slowly in an expansive manner, forming a serous cyst at the pr imary site. I t usually induces a sensation of abdominal pressure; however, complications appear only after a long history of the disease. According to Kat tan ' s 2 report of a series of 94 patients, complications consisted of infection in 9 patients (9,6%), calcification in 5 (5.3%), intra-abdominat rupture in 3 (3.2%), and intra-thoracic rupture in 1 (1.0%). In other studies) ,4 cholangitis caused by constriction of the biliary trees was frequent and pancreatit is was induced due to ob- struction of the c om m on channel of the pancreat- obiliary duct.

Alveolar echinococcus also grows slowly, forming multiple hydatid and septal cysts. The pat ient is asymptomat ic for a long period, and once symptoms appear, the disease is usually quite advanced. Progres- sively destructive disorders of the biliary tract fre- quently cause cholangitis, and the parasite not only invades neighboring tissues, causing perforat ion of the digestive tract and hepa topulmonary fistulas but also metastasizes to the lung or brain.

In unilocular echinococcosis, spontaneous rupture is often experienced; the preceding cysts are not usually recognized. 5 When the cyst ruptures, anaphylactic shock occurs very quickly, frequently resulting in death sev- eral hours later. 6 The shock is considered to be due to a reaction of the host to fluid leaked f rom the hydatid cyst. Our patient had shown not evidence of either unilocular or alveolar echinococcosis until he exhibited hypotension shock after an abdominal blow. Alveolar echinococcosis was proven eventually by E L I S A and Western blot test. Rupture of the lesion brought about implantat ion and dissemination of the larvae, with re- peated recurrences. Consecutive resections of the re- current lesions were needed. In this situation, we would expect a progressive course, resulting in death.

We found no reports in the literature on the rupture of alveolar echinococcocal cysts of the liver, whether spontaneous or traumatic, and the pathogenesis of the associated shock is not clear. We believe that our pa- tient represents the first repor ted case of shock follow- ing the traumatic rupture of AEL. This shock may be similar to the anaphylactic shock that is manifested in the rupture of unilocular echinococcosis. With the echinococcus larvae in the unilocular type, the fluid con- tent of the cyst is serous. The alveolar type usually forms various types of cysts in which there are viable larvae and germinal cells, 7 The living parasites or germi- nal cells and the fluid content, whose characteristics are unknown, may be implicated in the anaphylactic shock. However , the precise mechanism of the shock remains unclear. In our patient, it is clear that the initial rupture of the cyst led to seeding of the incisional wound and

intraabdominal dissemination. Drainage alone is never effective in AEL, as implantat ion and dissemination of the larvae or germinal cells is frequent, eventually caus- ing death. Drainage is effective in the unilocular type, since such complications do not generally occur. Our patient suffered f rom repeated recurrences, the main cause likely being associated with rupture, incomplete resection, and inappropriate drainage. Comple te resec- tion of the involved areas is indispensable for the cura- tive t rea tment of AEL.

Albendazole inhibits growth of the A E L lesion? The serum concentration at a dose of 50mg/kg per day 9 reaches maximal level approximately 2h after dosing, and the half-life is 8h. 1~ Albendazole freely diffuses across the cell membrane of the parasite and undergoes first-pass metabol ism through the liver, forming alben- dazole sulphoxide. This agent inhibits glucose uptake by many helminth species both in vivo and in vitro, 11 probably associated with a compensa tory depletion of glycogen stores. However , at most, only 50% of patients treated with albendazole respond to the medi- cation. 12 In our patient, the agent was effective after the repeated resections of the remaining lesions.

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4. Mentes A, Batur Y, Ahmet E. Pancreatitis as a complication of a hydatid cyst. Jpn J Surg 1990;20:356-358.

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8. Sato N, Uchino J, Aoki S, et al. Efficacy of benzimidazole-lype drugs in alveolar echinococcosis (in Japanese with English ab- stract). Nippon 8hokakibyo Gakkai Zasshi (Jpn J Gastroenterol) 1994;91:1197-1204.

9. Morris DL, Chinnery JB, Georgeious G. Penetration of albendazole sulphoxide into bydatid cysts. Gut 1987;28:75-80.

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