Hydatid Disease of the Liver

Jose Luis Barros, MD, Madrid, Spain

Hydatid disease is a parasitic infection carried by the larval stage of Echinococcus granulosus, a small tapeworm that follows a life cycle in nature [1,2]. Man acquires the disease by ingestion of parasitic eggs which develop into hydatid cysts, usually in the liver or the lung and less commonly in other or- gans.

A hydatid cyst consists of a double layered par- asitic wall enclosing specific fluid. The outer layer is thick and is composed of hyalin substance; the inner layer is the germinal membrane of the parasite. The bulk of the cyst is made up of specific clear fluid which contains free scolices, detached from the ger- minal layer. This corresponds to the univesicular cyst, typical of almost all hydatid cysts of the lung. Hydatid cysts in the liver are most commonly mul- tivesicular. The cyst exists basically as a foreign body. As it grows, the reaction of the host leads to the for- mation of a pericystic wall (adventitia), particularly thick in the liver.

Cllnlcal Material

We reviewed our experience in the surgical management of hydatid disease of the liver in 212 patients over the past eighteen years. Ages ranged between six and seventy-two years; 58 per cent of patients were female and 42 per cent male. Incidence was similar in both lobes of the liver, and approximately 80 per cent of the cysts were multivesicular. There was more than one cyst of the liver in 72 patients, and 21 had associated intraabdominal hydatidasis. The number of cysts operated on exceeded considerably the number of patients.

There was associate pathologic disease of the biliary tract in forty-seven patients. (Table I.) The most common complication was rupture into the bile ducts (36 patients). In addition, other large, intact cysts often had small biliary communications with the pericystic cavity. Secondary bacterial infection was observed in twenty-seven patients with ruptured cysts. Two patients had an acute clinical presentation with abdominal pain and anaphylaxis sec- ondary to traumatic rupture of a cyst into the peritoneal cavity. A large cyst of the dome of the liver with thoracic involvement (hepatopuhnonary cyst) was present in twenty patients. A twenty-four year old patient was admitted with

From the Department of Surgery, Hospital Provincial. Madrid, Spain. Reprint requeste shwld be addressed to Jose Luis Barros, MD, Deparmmnt

Of Surgery, Hospital Provincial, Medrid. Spain. Presented at the Fifty-Eighth Annual Meeting of the New England Surgical

Society, Portsmouth, New Hampshke, September 30-October 2, 1977.

bleeding esophageal varices secondary to chronic com- pression of the portal vein by multiple hydatid cysts of the liver. (Table I.)

Upper abdominal pain was the most common symptom. A palpable maas or hepatomegaly was present in two thirds of the patients; for this reason, in Spain as in other endemic regions, percutaneous needle biopsy should not be per- formed until hydatid disease is ruled out. Other clinical manifestations were related to complications of the cysts (jaundice, fever, and so on).

The intradermal test (Casoni) and the complement fixation test (Weinberg) were used routinely. The intra- dermal test was positive in approximately 75 per cent of the patients and the complement test in 80 per cent. Re- sults of the former often remain positive for many years after treatment of the disease; those of the latter usually become negative within months.

Liver scanning with radioactive isotopes was the most helpful routine diagnostic aid. Results were positive in 91 per cent of the patients; false-negative results were usually ascribable to the small size of the cysts. In recent years we have been using ultrasonic echography. Chest films, gas- trointestinal series, cholangiography, and urography were routine preoperative studies. Although selective hepatic arteriography was used as a diagnostic tool for some time, we have abandoned this procedure except in some selected cases.

TABLE I Preoperative Complications in 212 Patients

No. of Patients

Biliary tract disease 47 (22%) Cystic rupture into the bile ducts 36 Gallbladder and/or common duct 9

stones Fibrosis of the papilla 4 External bile fistulas 4

Bacterial infedtion 27 (12.7%) lntracystic 26 Subphrenic abscess 1

lntraperitoneal rupture 23 (10.8%) Acute (anaphylactic shock) 2 k$ltiple intraperitoneal cysts 21

Hepatopulmonary cyst 20 (9.5%) Lung involvement, intact cyst 11 Perlc);stobronchial flstulas (hydatid 3

vomit) Biliptysis 4’ Rupture into the pleural cavity 2

Portal hypertension and gastrointestinal 1 1 (0.45%) bleeding

Total 124 94 (44%)+

l 2 with massive hemoptysis. t Many patients had more than 1 complicalion.

Vdumo 135, Aprlt 1978 597

Surgical Management

Surgery is the only effective treatment for hydatid disease of any location. A variety of surgical proce- dures has been proposed for treatment of the several forms of the disease. We strongly advocate the most conservative technics.

The most common method used (148 patients, 69.8 per cent) consisted of removing the parasite (cys- tectomy) and treatment of the residual pericystic cavity. The operative field must be carefully pro- tected of hydatid fluid spillage with packs and wa- terproof towels. The cyst is entered at its most ac- cessible part with a trocar connected with a three-way stopcock’(Dew’s modification), and hydatid fluid is aspirated by suction. If the aspirate is crystal-clear, ethyl alcohol is injected and aspirated repeatedly to kill the living parasites. If the aspirate is not clear (possible biliary communication), no irrigation is performed. The pericystic wall is then incised, and the parasitic membranes are meticulously removed. Small communications with the biliary system should be closed. The redundant portion of fibrous peri- cystic wall is trimmed away, and the edges are over- sewn with a running catgut suture and approximated loosely to reduce the volume of dead space. External drainage of the residual cavity is done, using a wide gauge rubber tube brought out through a stab wound and connected with a bag for gravity drainage; su- prahepatic and subhepatic spaces are also drained in most cases. Tubes are removed within a few days in the absence of bile leakage or any discharge.

Pericystectomy (total pericystectomy) involves en block resection of the intact cyst with the entire pericystic layer and was employed in twenty-eight patients (13.2 per cent), with three technical variants. The simplest form of pericystectomy involves exci- sion of the lesion along the outer boundaries of the pericyst, including a minimum amount of liver pa- renchyma. It was performed in twelve patients with pedunculated cysts of minimal surface contact with the liver. The second technical variant was wedge resection, including a minimal amount of liver tissue. This was done in nine patients with small cysts. The third variant of pericystectomy was left lateral seg- mentectomy, a procedure included here because the cysts occupied most of the segment. This was done in seven patients.

Biliary tract disease was present in forty-seven patients (22 per cent). Intraoperative studies with x-rays and colorant (methylene blue) were done routinely. Cholecystectomy was performed in nine patients, removal of common duct stones in three, and evacuation of intraductal membranes in four. Sphincteroplasty was done in four of these patients;

two of them had chronic external bile fistulas from previous operations for hydatid disease, and both were cured after removal of duct stones overlooked during the first operation, associating sphinctero- plasty and excision of the fistulous tracts.

Internal drainage into the gastrointestinal tract was performed in fourteen patients (6.6 per cent) with major bile fistulas to the pericystic cavity. A wide anastomosis was established between the per- icystic opening and the digestive tract. Pericystoje- junostomy, with a Roux-en-Y anastomosis of jeju- num, was performed in eleven patients, and peri- cystogastrostomy was performed in three. These technics are not feasible with cysts of the dome of the liver; in these cases, external closed drainage is in- dicated.

Intrathoracic evolution of a cyst of the dome of the liver (hepatopulmonary cyst) was observed in twenty patients (9.5 per cent). Their disease is characterized by adhesion of the adventitia to the diaphragm, which undergoes progressive atrophy from pressure of the growing hepatic cyst. The negative intratho- racic pressure favors the progressive encroachment upon the chest. In eleven of these patients, the cyst was intact. The lesion was approached through the chest and, after disconnection of the involved lung, the cyst was usually treated transdiaphragmatically. A drainage tube was brought out through a stab wound in the abdominal wall, the diaphragm was closed, and the pleural cavity was drained.

Three hepatopulmonary cysts ruptured into the bronchial tree. Excision of the right basal pyramid was required in one case, and two were managed by wedge resection of the lung.

In another four patients there were simultaneous communications with the bronchial and the biliary trees, manifested clinically by bile in the sputum (biliptysis). All were seriously ill and presented with extensive pneumonitis and abscesses. An associated biliary obstructive problem was observed in these four patients (parasitic membranes, gallstones, or fibrosis of the papilla). Two of these patients with major biliptysis required emergency operation for massive hemoptysis. The approach was through the chest and the abdomen in the same operative stage. Lower or middle lobectomy of the lung was required in these patients.

Free rupture of the hepatopulmonary cyst into the pleural cavity occurred in two patients (0.9 per cent), and both required emergency surgery. In one there was severe empyema and in the other a predominant bilithorax.

In cases of multiple intraabdominal hydatidosis, only symptomatic cysts were treated. Systemic an- tibiotics were used only in complicated cases.

599 The American Journal 01 Surgery

Hydatid Dlse~~se of the Liver

We took special care in treating the diaphragm. Inflammatory adhesions were always freed to facili- tate the diaphragmatic movements with respiration. This measure contributes to the effectiveness of the respiratory physiotherapy and decreases the post- operative pulmonary complications.

Results and Complications

The results were excellent in 96 per cent of patients with simple cysts, and the mean hospital stay in this group was two weeks. Most postoperative compli- cations were observed in patients with complicated cysts. (Table II.) There were eight deaths (3.8 per cent of the whole series); four occurred in patients with ruptured hepatopulmonary cysts; one was due to anaphylactic shock after traumatic rupture of a cyst; and two were due to terminal hepatic failure (cirrhosis) and bleeding esophageal varices. One in- traoperative death was ascribed to Formalin@ toxicity early in our series.

External bile fistulas were observed in eight pa- tients; two were persistent and required a second operation. Postoperative infections in twenty-nine patients included eleven pulmonary infections, ten wound sepses, five intraperitoneal abscesses that required surgical drainage, and three infections of the residual cavity managed conservatively. Recurrent intraabdominal disease is a late complication that occurred in eighteen patients (8.5 per cent). The period apparently free of disease ranged between twenty-two months and fourteen years.

Comments

Hydatid disease of the liver (E granulosus) is a benign disease curable by conservative surgical procedures in a high percentage of cases. Removal of the parasite is an operative step common to all technics. Concerning the treatment of the residual pericystic cavity, the classic methods are marsupi- alization and capsulorrhaphy without drainage

[L21. Marsupialization consists of suturing the edges of

the opened pericystic wall to the skin. This procedure is prone to many postoperative complications, in- cluding secondary hemorrhage, chronic suppuration, external bile fistulas, postlaparotomy hernia, and a very long postoperative course. We strongly oppose this technic.

The other classic method, capsulorrhaphy without drainage, is still employed, but several modifications are more commonly used, such as tight closure of the cavity after filling it with saline, capitonnage, excision of the redundant portion of pericystic wall, omen- toplasty, and a variety of methods of internal and

TABLE II Postoperative Compllcatlons In 212 Patlents

No. of Patients

Deaths Hepatopulmonary cysts Portal hypertension and gastrointestinal

bleeding Acute intraperitoneal rupture

(anaphylaxis) Hepatic failure (cirrhosis) Formalin toxicity (?)

External bile fistulas Transient Persistent

Infection of the residual cavity lntraperitoneal abscess Wound infection Pneumonia Recurrent hydatld disease

a (3.8%) 4

1

1 1

a (3.8%) 6 2

3 (1.4%) 5 (2.3%)

10 (4.7%) 11 (5.2%) ia (8.5%)

external drainage 11-101. In our experience, partial pericystectomy has afforded excellent results in 148 of our patients. It is of utmost importance to secure an effective external closed drainage. Unexpected bile leaks occasionally occur after surgery of simple cysts.

The various forms of pericystectomy (total peri- cystectomy) should be considered as previously in- dicated. Their indiscriminate application would lead to a high incidence of postoperative bleeding, bile leakage, and other hepatic complications. In recent years, major hepatic resection (lobectomy) has been proposed as the preferred method for elective treatment of hydatid disease of the liver [II,12]. We strongly condemn this method because it is associ- ated with a higher mortality than any of the conser- vative procedures. We deemed it, necessary to per- form major hepatic resection in only two of our pa- tients; both presented severe sequelae of previous marsupialization procedures performed elsewhere.

The most common complication (17 per cent, of our patients) of hydatid cysts of the liver was rupture into the bile ducts. Typically, the patient with this com- plication presents with pain in the right, upper quadrant of the abdomen and intermittent or per- sistent jaundice; enlargement of the liver or a pal- pable hepatic mass are fairly common findings on physical examination. Anaphylactic manifestations are unusual in our experience. Secondary bacterial infection with cholangitis, liver abscess, and septi- cemia often occurs.

There were twenty hepatopulmonary cysts with right thoracic involvement which deserve special consideration, since this form is associated with the highest mortality and presents problems of surgical management [13,14]. The cyst can often be ap- proached through a right thoracotomy, and in the presence of obstructive biliary disease (such as bil- iptysis or jaundice), laparotomy should be done in the

voluma IS5, AWill 599

Barros

same operative stage. There were no deaths in eleven patients with intact hepatopulmonary cysts; in contrast, four deaths were observed among nine pa- tients who presented with ruptures into the bronchial tree, bile ducts, or pleural cavity. Occasionally there are calcific deposits over the hepatic region on x-ray films. Some patterns of calcification, such as the “egg shell,” are generally considered degenerating or dead hydatid cysts [3,5]. We consider surgery only when the patient presents with symptoms attributable to the calcified cyst.

Recurrent intraabdominal disease is a grave problem. Multiple operations may be required to treat symptomatic cysts. Long-term follow-up of most of our patients revealed an intraabdominal re- currence rate of 8.5 per cent. Since many of these secondary cysts may take years to become evident, we advise follow-up visits at yearly intervals.

Summary

Our experience in the surgical management of hydatid disease of the liver in 212 patients over the past eighteen years is reviewed. The most frequent postoperative complications and mortality rates of elective and emergency procedures are presented, and the more frequently utilized operative technics are described.

In the great majority of patients conservatism was the rule in excision of solitary or multiple cysts. It is important to establish whether or not hepatic cysts communicate with the biliary tree. In these cases, enteroanastomoses (such as cystjejunostomy or cystgastrostomy) may be utilized depending on the position of the cyst. Any associated biliary disease (such as lithiasis or fibrosis) should be taken care of at the same time. External cystic drainage (marsu- pialization) is contraindicated because of the high incidence of chronic external biliary fistula, secon- dary hemorrhage, sepsis, and postlaparotomy hernia. In those patients in whom the cyst has penetrated the diaphragm and communicates with the lung, treat- ment should be carried out in one stage whenever possible.

References

1. Arce J: Hydatid disease (hydatidosis); pathology and treatment. Arch Surg 42: 1, 194 1.

2. Rornerc+Tor~es R, Campbell JR: An interpretative review of the surgical treatment of hydatid disease. Surg Gynecol Obstet 121: 851,1985.

3. SaMi F: Surgery of Hydatid Disease. Philadelphia, Wi3 Saunders, 1976.

4. Burlui D, Condiesco M, Manesco G: Anastomose directe kysto-gastrique et kystoduodenale dans le traitement du kyste hydatique du foie. Lyon Chir 64: 220, 1968.

5. Dew H: Operative treatment of hydatid cysts of the liver. Surg

Gynecol Obstet 48: 239, 1929. 6. Fagarasanu I: Pericystogastrostomy: internal drainage in the

treatment of certain hydatid cysts of the liver. Br J Surg 53: 624, 1978.

7. Jidejian Y: Collective review of hydatid disease. J Int Co/l Surg 28: 125, 1957.

8. Liaras M: Les grandes lignes du traitment du kyste hydatique du foie. Lyon Chir 65: 506, 1969.

9. Narbona 6: Quiste hidaditidico hep&ico. Comentarios a la experiencia personal de ciento un cases. Ann Hosp Prov Vale&a 1: 53. 1974.

10. Papadimitriou J, Mandrekas A: The surgical treatment of hydatid disease of the liver. Br J Surg 57: 431, 1970.

11. Hicken NF, McAllister AJ, Carlquist JM, Madsen F: Echino- coccosis of the liver and lungs. Analysis of nineteen cases. Am J Surg 112: 823, 1966.

12. Pissiotis CA, Wander JU, Condon RE: Surgical treatment of hydatki disease. Prevention of complications and recwence. Arch Surg 104: 454. 1972.

13. Reventos J, Nogueras FM, Rius X, Lorenzo T: Hydatid disease of the liver with thoracic involvement. Surg Gyneco/ Obstet 143: 570, 1978.

14. Yacoubian MD Thoracic problems associated with hydatid cyst of the dome of the liver. Surgery 79: 544, 1976.

Discussion

John Braasch (Boston, MA): I present one complica- tion of treatment of this disease. A cyst was injected with a sclerosing agent with rather alarming results, because the cyst communicated with the extrahepatic biliary tree. When this patient came to us there was obliteration of the right and common hepatic ducts. (Slide) This operative cholangiogram obtained at exploration demonstrates that needle puncture had been made at this point into the left hepatic system (only this left system remains), and the jejunum anastomosed to the area of the hilus, but not to the left duct. We had to partially amputate the left lobe and perform left hepatojejunostomy to that point. Certainly, before sclerosing therapy is carried out, the possibility of cystic communication with the ductal system must be eliminated.

Hermes C. Grill0 (Boston, MA): A technic for man- agement of echinococcus cysts, which may be a very useful adjunct, was described by Dr. Farrokh Saidi (Surgery of Hydatid Disease, Philadelphia, WB Saunders, 1976). A specially made funnel is placed on the dome of the cyst and fused to the cyst cryogenically. Carbon dioxide is passed through fine tubing around the open base of the funnel, creating the low temperature necessary to freeze the funnel to the cyst. This produces a nonleaking connection, and the cyst is drained through the funnel without spillage. After the scolices and cyst wall have been extracted, a cystocidai solution is poured into the cavity. Dr. Saidi recommends 0.5 per cent silver nitrate. The funnel is removed by thawing, and the rim of necrotic tissue around the edge is excised.

I first used this method in a patient in whom infection developed in a hepatic echinococcal cyst after biliary sur- gery. It was an enormous cyst which presented both in the dome of the right lobe of the liver and in the porta hepatis. The cyst was approached transthoracically by detaching the diaphragm peripherally. Drains were placed in the cavity and led out subcostally. Recovery was complete.

600 The American Journal of Surgery