Hydatid Disease of the Abdomen and Other Locations

World J. Surg. 29, 1161 1165 (2005) DOI: 10.1007/s00268-005-7775-3 Hydatid Disease of the Abdomen and Other Locations Alexandra K. Tsaroucha, M.D. Ph.D., 1 Alexandros C. Polychronidis, M.D. Ph.D., 1 Nikolaos Lyrantzopoulos, M.D. Ph.D., 2 Michail S. Pitiakoudis, M.D. Ph.D., 1 Anastasios J. Karayiannakis, M.D. Ph.D., 1 Konstantinos J. Manolas, M.D. Ph.D., 2 Constantinos E. Simopoulos, M.D. Ph.D. 1 1 2nd Department of Surgery and Department of Experimental Surgery, Medical School, Democritus University of Thrace, 6 I. Kaviri Street, Alexandroupolis, 68100 Greece 2 1st Department of Surgery, Medical School, Democritus University of Thrace, 6 I. Kaviri Street, Alexandroupolis, 68100 Greece Published Online: August 11, 2005 Abstract. We present patients treated for hydatid disease in our hospital (in northeastern Greece) over the last 20 years. In the period from 1984 to 2003, a total of 135 patients (54 male, 81 female) were treated for echinococcal disease (age 15 85 years). In 111 (82.2%) patients only the liver was affected; 9 (6.7%) patients had concomitant hepatic and extrahepatic hydatid disease; and 15 (11.1%) patients had only extrahepatic disease. Clinical symptoms in patients with hepatic locations of the disease included abdominal pain localized in the epigastrium or right upper quadrant of the abdomen, tenderness, hepatomegaly with palpable abdominal mass, jaundice, fever, and anaphylactic reaction. All the patients were treated surgically. Surgical techniques included partial cystectomy and drainage, cystectomy and capitonage, cystectomy and omentoplasty, only drainage, left lateral hepatectomy, total pericystectomy, and laparoscopic pericystectomy. Rupture into the bile duct was managed by T-tube drainage or biliodigestive anastomosis. Symptoms and surgical treatment for extrahepatic cysts varied according to the location of the cyst. The median cyst diameter of all patients was 11 cm. The postoperative complication rate was 17.0%. Two patients died (1.5%). The median hospital stay was 18 days. The recurrence rate was 6.7%. The study suggests that treatment of this benign disease should be the less radical surgical technique combined with pre- and postoperative anthelmintic administration. The surgical treatment should be combined with careful use of scolicidal fluids and aspiration of the cyst to avoid contamination and minimize the risk of recurrence. The echinococcal disease, caused by Echinococcus granulosus, is still a problem with worldwide distribution. The disease is particularly common in the Mediterranean region. Echinococcal cysts are mostly located in the liver (60 70% of cases) followed by the lung (10 25% of cases), although they can also develop in other organs in the abdominal cavity and, rarely, anywhere in the body[1 4]. Hydatid cysts may remain asymptomatic for years, until causing local symptoms due to pressure by the cyst or systemic allergic reactions. Pain in the right upper quadrant of the abdomen or the epigastrium is the most common symptom, although hepatomegaly and a palpable mass are also common with Correspondence to: Alexandra K. Tsaroucha, M.D. Ph.D., e-mail: tsihrin@otenet.gr hepatic cysts [65]. Billiary obstruction, a generalized toxic reaction due to hydatid cyst rupture, and secondary infection are the most common complications of liver echinococcosis [7]. Hydatid disease in extrahepatic locations usually follows a silent clinical course unless it grows and produces pressure symptoms or develops complications [43]. Complications may include local pressure, rupture into the pleural or the peritoneal cavities, secondary infection, and an allergic reaction. Plain abdominal radiographs may show calcification of the cystic wall. Ultrasonography (US) and computed tomography (CT) are sensitive methods for diagnosing echinococcal cysts [61]. Biologic and serologic tests have also been used for diagnosis, but their sensitivity and reliability are low [1]. The treatment of choice for the hydatid disease is surgery for all locations of the echinococcal cyst. Surgical treatment of hydatid disease in combination with anthelmintic chemotherapy (albendazole or mebendazole) has been presented as an effective therapy since the mid-1970s [7 10]. Chemotherapy alone has failed in many cases [7]. For liver echinococcosis various surgical techniques have been proposed, ranging from hepatectomy, to complete resection of the cyst, to minimally invasive procedures [11 21]. Effective surgical treatment of the liver hydatid cyst does not necessarily imply a radical approach such as liver resection and total pericystectomy [159]. The operative strategy is important and should be tailored to each patient depending on the cyst location. In this study, we present patients treated for hydatidosis in our hospital (northeastern Greece) over the last 20 years. The emphasis is on symptoms, location of the cyst, complications, surgical treatment, adjuvant therapy, and hospital stay. Patients and Methods During the period from 1984 to 2003, the medical records of 135 patients treated for hydatid disease in our institution were reviewed retrospectively. Of these patients 54 (40.0%) were male and 81 (60.0%) female. Patient age ranged from 15 to 85 years (mean SD 47.4 16.7 years). Most of the patients (n = 103) were farmers (76.3%).

1162 World J. Surg. Vol. 29, No. 9, September 2005 Table 1. Extrahepatic locations of the ecchinococcal cyst. Cyst location No. of patients with synchronous liver location Symptoms Surgical treatment Lung (n = 4) 4 Chest pain, coughing, hydatidemesis Lobectomy/cystectomy Spleen (n = 4) 1 Palpable abdominal mass, tenderness Splenectomy on left upper quadrant Peritoneal cavity (n = 6) 3 Atypical abdominal pain, asymptomatic Cyst excition Kidney (n = 5) 0 Pain, hematuria, palpable mass 4 Pericystectomies, 1 nephrectomy Retroperitoneal space (n = 2) 0 Atypical abdominal pain, asymptomatic Cyst excision Pericardium (n = 1) 1 Thoracic pain, cough, dyspnea Cyst excision Gallbladder (n = 1) 0 Atypical abdominal pain Cholecystectomy Thyroid gland (n = 1) 0 Palpable nodule in the gland Thyroidectomy Preoperative diagnosis was established by the history (i.e., occupation, contact with animals, level of education), clinical examination, US, and CT. All patients underwent radiologic evaluation of the lung with chest radiography. The treatment for all patients was surgical. In patients treated over the last 10 years, additional anthelmintic chemotherapy with albendazole was administered. Statistical analysis of the data was performed using the Statistical Package for the Social Sciences (SPSS), version 11.0 (SPSS, Chicago, IL, USA). Because the distribution of some continuous variables did not appear to be normal, a Kolmogorov-Smirnov test for normality was performed. Normally distributed continuous variables were expressed as the mean standard deviation, and nonnormally distributed variables were expressed as the median and range. Categoric variables were expressed as frequencies (and percentage). The v 2 test and the Kruskal-Wallis test were used, respectively, to compare complication rates and hospital stay between patients with different surgical procedures. All tests were two-tailed, and statistical significance was considered for p < 0.05. Results In 111 (82.2%) of the 135 patients only the liver was affected, and 9 (6.7%) patients had concomitant hepatic and extrahepatic disease. Thus, a total of 120 patients had hepatic disease. In 83 of the 120 patients the hydatid cyst was in the right lobe of the liver (among them one patient had two cysts and four patients had three cysts each); in 36 of the 120 patients the cyst was in the left lobe (two patients had two cysts each); and in one patient the cyst was in the caudate lobe. There were a total of 131 hepatic cysts in 120 patients. Altogether, 24 (17.8%) patients had hydatid cysts in other locations, including the 9 (6.7%) patients with concomitant hepatic and extrahepatic disease and 15 (11.1%) with extrahepatic disease only (Table 1). Clinical symptoms for hepatic disease are summarized in Table 2. Abdominal pain and tenderness of the abdomen were the most predominant symptoms in patients with hydatid disease of the liver. Twenty-one patients (17.5%) were asymptomatic, and the hepatic hydatid cyst was revealed during a diagnostic evaluation for another reason. The predominant symptom in patients with an extrahepatic location was atypical pain; other symptoms varied according to the localization of the cyst (Table 1). Complications of the hydatid cyst of the liver included acute cholangitis, infection, and rupture in the peritoneal cavity. Acute cholangitis occurred in 22 (18.3%) of the 120 patients, caused by a hydatid cyst that ruptured into the biliary tract. These 22 patients Table 2. Symptoms and signs of hepatic hydatidosis. Symptom No. of patients Abdominal pain 84 70.0 Atypical abdominal tenderness 98 81.7 Hepatomegaly with abdominal mass 36 30.0 Jaundice 22 18.3 Fever 11 9.2 Anaphylactic reaction 8 6.7 No symptoms 21 17.5 Percent of 120 patients had jaundice and suffered from chronic epigastric pain. The hydatid disease was not diagnosed prior to rapture in 11 of these 22 patients. Infection of the cyst with high fever ( 38 C and higher) was presented in 11 (9.2%) of the 120 patients. Rupture of the cyst in the peritoneal cavity occurred in three patients followed by anaphylactic reaction (two liver cysts, one peritoneal cyst). Anaphylactic reaction also occurred in other five patients. One patient had an unusual complication, upper gastrointestinal bleeding, because of erosion of the stomach wall by a cyst in the left lobe. The median cyst diameter in all patients was 11 cm (range 3 35 cm). All patients were treated surgically (Table 3). The surgical treatment was either radical (i.e., liver resection or total pericystectomy) or conservative. Radical excision was indicated for small, peripherally located cysts; however, in most cases the size of the cyst and the location did not permit a radical operation. The conservative surgical technique (Table 3) used depended on the preoperative complication, the age of the patient, the location of the cyst, the size of the cyst, the number of cysts, and the intraoperative findings. The rupture into the bile duct and the cystobiliary communication were managed by T-tube drainage or biliodigestive anastomosis. Biliodigestive anastomosis was performed for patients over 50 years old who had a dilated common bile duct (20 mm diameter); whereas for patients younger than age 50 with a narrow common bile duct, T-tube drainage was performed. The operative procedures performed in patients with obstructive jaundice (22 patients) included cholecystectomy, choledochotomy with T-tube drainage or with choledochoduodenostomy. Partial cystectomy and drainage of the cavity and choledochotomy with T-tube drainage was performed in nine patients, and in four patients only drainage of the hydatid cyst and cholecystectomy, and choledochotomy with T-tube drainage were performed. In the remaining nine patients partial cystectomy/ drainage of the hepatic cyst and choledochoduodenostomy were performed. The additional procedures performed in patients with cystobiliary communication are also presented in Table 3. In

Tsaroucha et al.: Hydatid Disease 1163 Table 3. Surgical techniques in hepatic ecchinococcosis. Surgical technique No. of hydatid cysts Percent of 131 hepatic cysts Conservative Partial cystectomy drainage (group A) 46 35.1 Cystectomy capitonage (group B) 32 24.4 Cystectomy omentoplasty (group C) 25 19.1 Drainage only (group D) 17 13.0 Radical Liver resection (left lateral lobectomy) 4 3.0 Total pericystectomy 6 4.6 Laparoscopic pericystectomy 1 0.8 Additional surgical techniques in patients with obstructive jaundice Cholecystectomy, choledochotomy, and T-tube drainage 13 Cholecystectomy and choledochoduodenostomy 9 patients with infected cysts, only drainage of the hydatid cyst cavity was performed. The methods of treatment of the hydatid disease in extrahepatic locations were: splenectomy in four patients, nephrectomy in one patient, and pericystectomy in four patients with kidney cysts. The patient with hydatid cyst of the thyroid gland underwent thyroidectomy. Cyst excision was performed in nine patients (Table 1) with cysts in the pericardium, the peritoneal cavity, and the retroperitoneal space. Lobectomy or cystectomy was performed in the four patients with lung cysts, and cholecystectomy was performed in the patient with a cyst in the gallbladder. The patients with concomitant cysts in the liver and lung or the pericardium were operated on first for the extrahepatic cysts and then at a later time for the hepatic ones. For the last 10 years the surgical management of echinococcal disease in our hospital is in combination with preoperative and postoperative anthelmintic albendazole administration. Of the 135 patients, 47 (34.8%) received preoperative and postoperative therapy with albendazole to a total daily dose 500 to 800 mg. The duration of the preoperative treatment was 2 weeks, and the drug was administered postoperatively for 3 to 4 months, depending on side effects. A minimum follow-up period was 1 year. There is no recurrence of the disease in this group of patients. Of the 135 patients, 23 (17.0%) presented 27 perioperative or postoperative complications (or both): in one patient pneumonia, in five patients atelectasis, in three patients deep vein thrombosis, in six patients wound infection, in two patients subphrenic abscess, in one patient infrahepatic hematoma, and in nine patients prolonged tube drainage and a long-lasting biliary fistula. The cause of the prolonged tube drainage was bile leak or infection of the residual cyst cavity. Five of these nine patients developed long-lasting biliary fistulas, which closed spontaneously after 3 to 5 months. One patient with a large retroperitoneal cyst developed postoperative fistula and was treated surgically. Relaparotomy was required in 3 of the 135 cases (2.2%). The median hospital stay was 18 days (range 12 42 days) except for the patient who underwent laparoscopic excision and was discharged on the third day. There were no significant differences in hospital stay or complication rates between the four conservative surgical techniques used (groups A D, Table 3). The median hospital stay for each group was for group A 20 days (range 15 42 days), for group B 18 days (12 38 days), for group C 19 days (12 32 days), and for group D 18 days (16 26 days) (Kruskal-Wallis test; p = 0.511). The complication rates were for group A 23.3%, group B 19.4%, group C 16.7%, and group D Fig. 1. Echinococcosis incidence in Greece. 18.8% (v 2 test: p = 0.925). Because of the small number of patients with radical treatment, they were not included in the statistical analysis. In the present study two patients died (mortality 1.5%). One died because of an anaphylactic reaction after cyst rupture. The other patient had a perioperative death because of massive pulmonary embolism. Recurrence can be asymptomatic, so it may be diagnosed only in those patients who come for follow-up. In this study, only 49 (36.3%) of the patients returned to the hospital for follow-up. The total recurrence rate was 6.7% (nine patients). Four patients had recurrence in the liver, two in the abdominal wall, two in the peritoneal cavity, and one in the peritoneal cavity close to the hilus of the spleen. None of these patients had been given adjuvant therapy with albendazole. Discussion Epidemiologic data by the National Statistical Service of Greece show the annual incidence (number of patients per year in a population of 100,000 people) of echinococcosis decreasing in the country over the years. This index of morbidity has changed from more than 16 in 1970 to less than 10 in recent years (Fig. 1). Nevertheless, there is no question that hydatidosis is still a public health problem in Greece, affecting certain groups of the population and being more serious in certain parts of the country. It is noted that most of the patients (76.3%) in this study were farmers. In addition, reported morbidity rates in the region of Thrace,

1164 World J. Surg. Vol. 29, No. 9, September 2005 in northeastern Greece (where our hospital is located), are the highest in the country. Thus, the diagnosis and treatment of the echinococcal disease are still important for our physicians. Of the treated hydatid cysts in the general population, 9.5% to 30.0% are asymptomatic [2016]; and often the first symptom is a complication. A frequent complication is rupture of the hepatic hydatid cyst into the biliary tract. In the present study, 21 (17.5%) patients were asymptomatic; 22 (18.3%) with hepatic hydatid cysts had obstructive jaundice because of rupture into the biliary tract, and in 11 of them the hydatid disease was unknown before this complication. Methods for treating intrabiliary rupture in patients with hepatic hydatid cysts are choledochoduodenostomy, T-tube drainage, and endoscopic retrograde cholangiopancreatography [22 24]. Of the 22 patients with this complication, 9 underwent choledochoduodenostomy and 13 T-tube drainage. In addition to clinical evaluation, the diagnosis of hydatid disease is based mostly on imaging with US [1]. However, CT should always be performed before any surgical intervention [1], especially in the case of an uncommon location of the disease. In our study, US and CT were the main diagnostic methods and provided the basic information in most patients. A serious diagnostic problem is still the unusual location of primary hydatid disease [2 254 29]. In this study, two patients with unusually located primary hydatid cysts were misdiagnosed. One patient with a primary hydatid cyst in the thyroid gland was diagnosed as having a cold nodule and was treated with thyroidectomy. Secondarily, the patient was examined, and no other echinococcal cyst was found. Another female patient with an intraperitoneal cyst was diagnosed as having an ovarian cyst; after examination, this patient also did not have a cyst at any other location. Biologic and serologic tests have been used, although their sensitivity and reliability are low [1]. In our cases, diagnosis was based mainly on imaging; only in cases with differential diagnostic problems were biologic or serologic tests also performed. The Casoni test was used for the first 10 years of the study, but because of low sensitivity this test is not used anymore. In recent years, we have used the indirect hemaglutination test and specific immunoglobulin E antibodies based on an enzyme-linked immunosorbent assay (ELISA). Among these two methods, the first is the less reliable. Surgery, conservative or radical, is still the treatment of choice for all locations of the disease [2018]. Until now it has not been clearly stated in the literature if there are benefits to minimal surgery compared to radical surgical treatment [30]. In this study, no significant impact of the surgical technique on duration of hospitalization and postoperative complications was found. Surgery with adjuvant therapy seems to remain the optimal method of treatment [2118]. Laparoscopy is considered to be another alternative for treating hydatid disease of the liver [3231]. In this study, the laparoscopic approach was used in only one patient with a small hepatic echinococcal cyst. Secondary hydatidosis, a result of spillage of cystic fluid during surgery, can be minimized using various techniques or methods [3317]. Various scolicidal agents have been used over the years, but most have shown limited effect and have produced adverse reactions [3433]. Hypertonic saline, one of the most commonly used scolicidal agents, has been tested at various concentrations and exposure times [33]. In our hospital, 15% hypertonic saline was used and has not shown any toxic effects. However, the recurrence rate of the disease in our study suggests that it is not an ideal agent; our recommendation is that this agent be used together with pre- and postoperative albendazole or mebendazole. In addition, careful aspiration of most of the hydatid cyst fluid before injecting the scolicidal agent into the cyst is recommended. An aspiration technique has been proposed that employs an aspiration apparatus that can contain and remove the cyst fluid by suction, thereby minimizing the risk of peritoneal contamination [17]. Reported recurrence rates in the literature vary from 6.6% to 22% [3635]. The recurrence rate in the present study was 6.7% for 49 patients (36.3%) who returned to the hospital for follow-up. Conclusions Treatment of human echinococcosis should be the less radical surgical technique. 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