Pulmonary Hydatid Disease: Report of 100 Patients

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1 Pulmonary Hydatid Disease: Report of 100 Patients Aydin Aytaq, M.D., Yurdakul Yurdakul, M.D., CoSkun Ikizler, M.D., Riistem Olga, M.D., and Argun Saylam, M.D. ABSTRACT One hundred patients with pulmonary hydatid disease underwent thoracotomy and operative removal of the cysts. Of the 60 men and 40 women, most were between 3 and19 years old. Cough, fever, dyspnea, and chest pain were the prominent symptoms in the majority of cases. Intact hydatid cysts were found in 67 patients and infected or ruptured cysts in 33. The Casoni skin test, Weinberg reaction, and eosinophilia were found to be unreliable diagnostic criteria and therefore were not used routinely in our patients. Roentgenological examination was the most valuable diagnostic aid. A single lobe was affected in 72 patients. Unilateral multiple foci were present in 15 patients and bilateral multiple foci in 13. Cystectomy and capitonnage were the preferred operative procedures in most cases. Pulmonary resection was necessary in only a limited number of patients. We conclude that conservative surgical methods such as cystectomy (with or without capitonnage) are preferable, especially for children, whose residual lung parenchyma has great capacity for expansion. The mortality rate among our 100 patients was 2%; both died of cardiac arrest during operation. Hydatid disease is endemic in some areas of the world, notably the Mediterranean countries, the Middle East, South America, and Australia. Lung is the second most common focus for this disease, after liver. Roentgenographic signs are usually diagnostic in pulmonary hydatidosis, but the Casoni skin test, Weinberg reaction, and eosinophilia are not specific and reliable for making the diagnosis. In children it is especially important to use conservative surgical methods, preserving pulmonary parenchyma with or without capitonnage of the residual cavity. Because the From the Department of Pediatric Thoracic and Cardiovascular Surgery, Hacettepe University Hospitals, Ankara, Turkey. Accepted for publication Mar 11, Address reprint requests to Dr. AytaC, Department of Pediatric Thoracic and Cardiovascular Surgery, Hacettepe University Hospitals, Hacettepe, Ankara, Turkey. lung parenchyma in children has a great capacity for expansion, it is possible to leave just a small area of parenchyma after the cyst is removed. One hundred patients with pulmonary hydatidosis operated upon between 1964 and 1975 form the basis of this paper. Clinical Material and Methods Most of our patients were children; our youngest patient was 3 years old and the oldest, 61 (Table 1). Ninety percent of them were from underdeveloped rural areas. Sixty patients were male and 40 were female. In 67 cases the cysts were intact and uncomplicated. Fifteen cysts were infected, 6 were ruptured, and 12 were both ruptured and infected. Seventy-two patients showed unilobar localization, and the others had unilateral or bilateral multiple cysts (Table 2). The most prominent symptoms in our series were cough, fever, dyspnea, and chest pain (Table 3). Symptoms due to concomitant extrapulmonary cysts were also present in some instances. Four patients were completely symptomless, their cysts being detected on routine chest roentgenograms. The Casoni skin test and Weinberg reaction were performed in only a small number of patients; therefore these tests were not evaluated in this series. Diagnosis was made routinely by chest roentgenogram, except in 1 patient in whom malignant lymphoma was suspected due to bilateral pulmonary opacities (see Fig 4). Cystectomy and capitonnage were the most commonly used surgical techniques (Table 4). Deactivation of the cyst by 3% NaCl solution was performed before a cystotomy was done during cystectomy. Figures 1 to 5 show some of the more interesting chest roentgenograms and intraoperative pictures. Results Ninety-eight patients are in good condition following complete recovery. Two patients died in 145

2 146 The Annals of Thoracic Surgery Vol23 No 2 February 1977 Table 1. Age Distribution of 100 Patients with Hydatid Cysts Table 4. Surgical Methods Used in 100 Patients with Hydatid Cystsa Age (Yr) No. of Patients Procedure No. Performed Table 2. Location of Hydatid Cysts in 100 Patientsa Location Single lobe Right upper lobe Right middle lobe Right lower lobe Left upper lobe Left lower lobe Unilateral multiple foci Bilateral multiple foci No. of.patients instances hydatid cysts were also present in extrapulmonary organs (liver, 4; liver and omentum, 1; liver and spleen, 1; spleen, 3; brain, 2). Cystectomy and capitonnage 101 Lobectomy 14 Cystectomy without capitonnage 5 Wedge resection 5 Enucleation and capitonnage 3 Segmentectomy 2 amore than one procedure was performed in some patients and the same procedure was sometimes performed more than once in a single patient due to multiple hydatidoses. the operating room, both from cardiac arrest during anesthesia. No recurrences have been seen on control chest roentgenograms during long-term follow-up of the living patients. Comment Hydatid disease is caused by either Echinococcus granulosus or E. alveolaris. E. granulosus is the common cause of hydatid lung disease; the alveolar type is very rare, only a few cases of lung metastases secondary to hepatic lesions having been reported 131. Hydatid cyst of the lung has a growth rate of 1 or 2 cm in diameter per year [lo]. Infestation of the embryo is the main route of infection. The embryo passes through the Fig I. Typical bilateral hydatid cysts in a 9-year-old boy. Table 3. Prominent Symptoms in 100 Patients with Hyda tid Cysts Symptom No. of Patients Cough 45 Fever 21 Dyspnea 13 Chest pain 10 Symptoms due to concomitant extrapulmonary hydatidosis 4 Hepatic cyst-abdominal pain 2 Brain cyst-headache 1 Brain cyst-hemiplegia 1 Detection during a routine chest roentgenogram (symptomless) 4 Expectoration of cystic fluid 2 Anaphylactic shock 1

3 147 Aytac et ai: Pulmonary Hydatid Disease Fig2. "Water-lily sign" in a case of ruptured hydatid cyst of the left lung in a 16-year-oldgirl. Fig3. (A) Ruptured hydatid cyst of the left lung in a 6-year-oldgirl. (B) Same patient, left lateral view. A duodenal wall into either the portal vein or the periduodenal and perigastric lymphatics. The entry of the portal vein into the liver accounts for the predominance of this location. It is through this route also that pulmonary cysts develop secondary to hepatic lesions. Periduodenal and perigastric lymphatic channels, which connect with the thoracomediastinal lymphatics and the thoracic duct, become infested, explaining the development of pulmonary lesions in the absence of hepatic cysts [lo]. Animals are hosts in the life cycle of echinococci, and hydatidosis is commonly encountered in animal (sheep, cow) feeding areas, especially in persons in contact with dogs; in a series of 577 cases from Turkey, 69% of the patients were from the animal-feeding and peasant population [BI. In Turkey, 4% of the dogs, 36% of the cows, and 26% of the sheep display hydatid disease [41. A direct route of infection in pulmonary hydatidosis is through inhalation of air particles containing dried microfragments of animal excreta carrying echinococci. The incidence of hydatidosis is 1:2,000 in the Turkish population, and 1% of the patients admitted to our surgical clinics have the disease 141. Pulmonary hydatidosis is most common in men in the second and third decades of life; other locations are more common in women in these decades [151. The incidence of pulmo- B

4 148 The Annals of Thoracic Surgery Vol23 No 2 February 1977 A B Fig4. (A)Bilateral multiple hydatidcysts of the lungs in an 8-year-old boy. Malignant lymphoma was suspected preoperatively. ( B ) Same patient, postoperative view. Figs. Giant hydatidcyst in therightupperlobeinan 8-year-old boy. (A)Operative view. The cyst was removed by enucleation. ( B ) Same patient. The removedspecimen. A B

5 149 Aytac et al: Pulmonary Hydatid Disease nary hydatid cysts is high in the first decade, as seen in our series. Pulmonary hydatid cysts can be located in any pulmonary lobe and can consist of multiple foci in one or both lungs. The right lower lobe is the most frequently attacked area of the lung, as seen in a review of the literature 18, 101; this is thought to be caused by the abundance of pulmonary flow to this lobe compared with other portions of the lung [lo]. In a series of 577 patients in Turkey, the right lung was attacked in 60.1%, the left in 31.1%, and both lungs in 8.8% of the patients [81. Unilateral multiple foci are reported in 20% of the patients [151. Other locations for hydatid cysts on the chest wall and in the chest cavity are the diaphragm, intercostal muscles, thoracic vertebrae, breasts, mediastinum, pericardium, and cardiac chambers. Locations of echinococcal cysts in a series from Turkey are shown in Table 5 [15]. Intrabronchial rupture, anaphylactic reaction, rupture into the pleural cavity with hydropneumothorax, rupture into the mediastinum, fatal asphyxia resulting from sudden occlusion of the bronchus or trachea by the expectorated germinative membrane, infection, lung abscess, and bronchiectatic changes in the lung parenchyma are the possible complications of pulmonary hydatid cysts. Infected hepatic or splenic echinococcosis may be complicated by transdiaphragmatic fistulas between these organs and the lung parenchyma or bronchi. About 2.5 to 3% of patients with hepatic Table 5. Locations of Hydafid Cysts in the Turkish Population Location Liver 54 Lungs 35 Peritoneum 5.9 Spleen 1.9 Muscles 1.8 Kidney 0.8 Nervous system 0.2 Breasts 0.2 Pancreas 0.1 Miscellaneous (cardiac, osseous, etc) 0.1 Percent of Patients with Echinococcosis echinococcosis display bronchobiliary fistulas and bile expectoration [l, 4, 5, 113. Signs and Symptoms About 5% of the patients are symptom free [lo]. Symptoms consist mainly of cough, fever, dyspnea, sweating, and chest pain. Evacuation of white cystic fluid and scoleces in the sputum sometimes occurs when cysts rupture and may lead to allergic and anaphylactic reactions requiring immediate treatment. Clubbing may be present in infected cases [171, as may pneumonitis. In some patients transdiaphragmatic rupture of a splenic 151 or hepatic [l, 4,111 cyst occurs with signs of pneumonitis at the bases of the lungs. Hydropneumothorax may be the first sign of a ruptured cyst. Other kinds of pulmonary masses or infection should be borne in mind during the differential diagnosis. Calcification of the cyst wall is seldom present on roentgenograms [lo]. Hydatid cyst may lead to Pancoast s syndrome when located at the apex of the lung [12, 131. Expectoration of bile may be a manifestation of bronchobiliary fistula in a ruptured and infected hepatic echinococcosis 11, 4, 111. Symptoms of concomitant extrapulmonary hydatid cysts may predominate over the symptoms of pulmonary hydatidosis. Nervous disorders can be observed when cysts arise in the thoracic vertebrae [6]. Similarly, abdominal pain and distention may appear in cases of hepatic and splenic cysts. Diagnosis Chest roentgenograms displaying oval or spherical masses in the lung fields are diagnostic of hydatid disease. A floating membrane called the water-lily sign is seen with an air-fluid level in ruptured cysts (see Figs 2, 3). Roentgenographic signs are generally diagnostic in countries where hydatid disease is common. Tumors of the lung and mediastinum as well as pulmonary hydatid emboli secondary to hepatic or cardiac echinococcosis [9] also should be kept in mind in making the differential diagnosis. Diaphragmatic (Bochdalek) hernia may rarely be misdiagnosed as hydatid cyst at the base of the lung. We have encountered 2 such patients in whom the liver had herniated through the diaphragmatic defect.

6 150 The Annals of Thoracic Surgery Vol23 No 2 February 1977 The Casoni skin test, the Weinberg reaction, and eosinophilia are not specific in making the diagnosis. We have encountered a number of patients with negative reactions and insignificant eosinophilia. Therefore, we do not use these tests for diagnosis. Orueta and co-workers [7] reported the Casoni skin test as 70% positive, the Weinberg reaction as 33% positive, and eosinophilia as 22% positive in their 23-year experience with pulmonary hydatidosis. Kagan and co-workers stated that serological reactions are positive in 85% of hepatic and 33 to 50% of pulmonary hydatid cysts [161. In large series in Turkey the Casoni skin test has been positive in 50%, the Weinberg reaction has been elicited in 30%, and significant eosinophilia has been seen in 28% of patients with pulmonary echinococcosis [8, 141. The Casoni skin test may be positive in pulmonary carcinoma and tuberculoma, and significant eosinophilia may be present in all kinds of allergic disorders [141. Treatment Prevention of hydatidosis is more important than treatment in countries where this disease is endemic. Hygienic conditions should be carefully maintained. Animals can be treated with bunamidine hydrochloride, as reported by Gemmell and Shearer [161. Multiple solitary cysts or alveolar forms in a patient are handled in much the same way as malignant lesions, owing to the difficulty in removing them. Rarely, expectoration of the cystic fluid and germinative membrane may lead to spontaneous healing of the residual cavity in some of the small cysts. We have encountered 1 such patient in our clinic (l0/o). Operation is the treatment of choice for pulmonary hydatid cysts. Conservative surgical methods that preserve lung parenchyma are usually preferred. Resection is indicated for some of the ruptured and infected cysts, in the presence of bronchiectatic changes secondary to infection or if the cysts are giant Emergency operation is indicated when cysts have ruptured into the pleura. Cysts that have ruptured into the bronchus are treated with postural drainage and antibiotics for at least 10 to 15 days and later are subjected to operation [171. Immediate medical treatment to prevent anaphylaxis should be initiated with ruptured cysts. Penicillin is the antibiotic of choice in pulmonary hy datidosis. Enucleation, introduced by Barrett [21, is generally used for small cysts. In some cases removal of the cyst is not followed by capitonnage, and only air leaks are sutured [lo, 181. Removal of the cyst with capitonnage of the residual cavity with 3-0 chromic sutures is the usual method of choice in our clinic. We aspirate the cystic material after sponges and towels moistened with 3% NaCl have been applied around the operative field to prevent implantation of scoleces into the pleural cavity. A quantity of cystic fluid is aspirated and the same quantity of 3% NaCl is injected into the cyst. Some surgeons use 1% formaldehyde in lieu of 3% NaCl for deactivation of cysts. Saline solution is safer than formaldehyde, because formaldehyde has a necrotizing effect as it penetrates the lung parenchyma through air leaks around the cyst. A waiting period of about three to five minutes is necessary for the hypertonic saline solution to deactivate the scoleces. After this, cystic fluid is evacuated as completely as possible, the cyst is removed with the germinative membrane, and the residual cavity is capitonnated. Although spilling of hydatid scoleces is inevitable in most patients, except those subjected to enucleation or pulmonary resection, implantation of the cyst in the pleural cavity is extremely uncommon, as determined from longterm follow-up of our patients. LeRoux [6] reported no recurrence four years after iatrogenic rupture of pulmonary hydatid cysts. Sar- Sam [lo] stated that some cysts are not mature enough and that others are too old to produce scoleces of adequate quality and quantity, thus preventing the development of a second cyst by local implantation. Wedge resection, segmentectomy, lobectomy, or pneumonectomy may be performed in selected patients. Lower lobe excision is usually indicated in patients with fistulas between the lungs and liver or spleen References 1. Amir-Jahed AK, Sadrieh M, Farpour A, et al: Thoracobilia: a surgical complication of hepatic

7 151 Aytac et al: Pulmonary Hydatid Disease echinococcosis and amebiasis. Ann Thorac Surg 14:198, Barrett NR: Removal of simple univesicular pulmonary hydatid cyst. Lancet 2:234, Doiru AM: Echinococcose alveolaire chez l'homme: a propos de 9 cas cliniques. Lyon Chir 67:371, Giirsoy H: A case of hepatic echinococcosis causing bronchobiliary fistula along with other various complications. J Turk Hydatid Assoc 9:39, Gursoy H: A case of hydatid disease of spleen with rupture into the bronchus. J Turk Hydatid Assoc 9:33, LeRoux BT: Pulmonary hydatid disease. Thorax 27:365, Orueta A, Fau LF, Montero A, et al: Surgical treatment of hydatid cysts of the lung. Br J Dis Chest 68:183, Oztaskent R, Amato E: Analysis of 577 cases of pulmonary hydatid cysts (in Turkish). Tuberkiiloz-Toraks 18:281, Purriel P, Tomalino D, Muras 0, et al: Embolismo pulmonar hidatico: estudio sobre 4 observaciones personales revision de la literatura. Torax 19:164, Sarsam A: Surgery of pulmonary hydatid cysts: review of 155 cases. J Thorac Cardiovasc Surg 62:663, Saylam A, Ersoy U, Barig I, et al: Thoracobiliary fistulas: report of six cases. Br J Dis Chest 68:264, Sili NM, Boratav M: A case of hydatid cyst of the lung showing Pancoast syndrome (in Turkish). Tuberkuloz 24:50, Stathatos C, Kontaxis AN, Zafiracopoulos P: Pancoast's syndrome due to hydatid cysts of the thoracic outlet. J Thorac Cardiovasc Surg 58:764, Tokgoz K: Studies on pulmonary hydatid cysts in our clinics (in Turkish). Turk Hidatid Derg Say 17:50, Ulker M, Okur N: The state of unilocular disease of hydatid cysts in Turkey. J Turk Hydatid Assoc 9:5, Wolcott MW, Harris SH, Briggs JN, et al: Hydatid disease of the lung. J Thorac Cardiovasc Surg 62:465, Xanthakis D, Efthimiadis M, Papadakis G, et al: Hydatid disease of the chest: report of 91 patients surgically treated. Thorax 27:517, Yacoubian HD, Dajani T: Preliminary report on a new method of surgical management of hydatid cysts of the lung. Ann Surg 157:618, 1963