Management of hepatic cysts (including hydatid disease)

Management of Hepatic Cysts

(Including Hydatid Disease) Juan M. Sarmiento, MD and David M. Nagorney, MD

epatic cysts are classically divided into parasitic and

H .nonparasitic types, with the latter most prevalent worldwide. Typically cysts cause no symptoms and seldom lead to liver dysfunction. Their clinical presence is precipitated by hemorrhage, infection, bile duct compression, portal hypertension, or, most commonly, expansion. The treatment of hepatic cysts should be individualized by cyst type and symptomatology. Cyst-related complications of jaundice, infection, or hemorrhage must be addressed during treatment. Because malignant potential is rare (with the exception of cystadenomas), malignancy should not be a prima~y concern. Our approach to treatment is based on these premises. 1 Many diagnostic modalities can define the presence and the extent of hepatic cysts. Ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) are the most useful imaging tests. US and CT are used routinely in the workup of these patients, with MRI reserved for complicated cases and polycystic liver disease. Treatment is undertaken based on cyst type by imaging, cyst number, intrahepatic site, and any complications present. Simple

Cysts

Simple cysts occur in 1% to 5% of the general population. Most are diagnosed in women, in whom symptoms are also more common. The indication for treatment is based on the presence of symptoms or complications. Asymptomatic patients require no therapy, regardless of the size of the cyst. Percutaneous cyst aspiration and alcohol sclerosis under US or CT guidance has evolved as an effective treatment for uncomplicated hepatic cysts. 2 Complicated cysts are best approached by an open method. Cyst aspiration and sclerosis is preferable for cysts with no or minimal capsular involvement or cysts in segments 7 and 8. When interventional radiologic expertise is unavailable, laparoscopic partial cystectomy (unroofing) is war-

From the Division of General and Gastroenterologic Surgery, Mayo Clinic and Mayo Foundation, Rochester. MN. Address reprint requests to David M Nagorney, MD, Professor of Surge U, Department of Surgery., Mayo Clinic, 200 First Street SW, Rochester, MN 55905 Copyright 2002, ElsevierScience(USA).All rights reserved. 1524 153X/02/0401-0006535.00/0 doi:10 1053/otgn.2002 30032

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ranted. Laparoscopy is preferred for anterior hepatic cysts (segments 2 to 6) or for cysts with at least 50% capsular surface area. Laparoscopic management has become the treatment of choice based on its applicability, efficacy, and low morbidity) Importantly, unroofing of cystadenomas, which are usually multiloculated but can be unilocular, should be avoided because of the high recurrence rate. Biopsy of the cyst wall provides the distinction. Biliary Cystadenoma Cystadenocarcinoma

and

These cysts are true neoplastic cysts. They can either be serous or mucinous, with the latter more common. They differ from simple cysts in their imaging features and prognosis. Cystadenomas can be differentiated radiologically from simple cysts by thicker walls and more prominent intraluminal septae. Cystadenocarcinomas are further characterized by mural nodularity. Cystadenomas are best treated by complete excision at laparotomy. 4

Echinococcal Cysts Echinococcus, or hydatid disease, is characterized by a worldwide distribution and frequent hepatic involvement. This disease is not prevalent in the United States. The capsule of the hydatid cysts in the liver comprises three different layers: the host capsule, consisting of compressed liver tissue and fibrous scar; the actual parasitic cysts, consisting of an outer laminated membrane of proteinaceous material of the cyst; and a germinal epithelium that gives rise to brood capsules that contain numerous protoscolices. These cysts are at high pressure, making them prone to enlargement or bile duct erosion. Patients are frequently asymptomatic. Often a routine chest x-ray that raises the suspicion of a cyst due to elevation of the right hemidiaphragm or the presence of subdiaphragmatic calcifications. Once the diagnosis is confirmed by further imaging and serology, treatment must be initiated. In general, all symptomatic patients and probably those patients with large asymptomatic cysts with minimal or no calcifications should be treated. Small or centrally located cysts are best observed. Recently, percutaneous aspiration and injection of scolicidal agents have proven safe and effective. 5 Operative or percutaneous intervention is indicated for symptomatic cysts; large, peripherally located, noncalcified hydatid cysts are ap-

Operative T e c h n i q u e s in General Surgery, Vol 4, No 1 (March), 2002: pp 76-87

Hepatic Cysts

proached operatively because of the unpredictable risk of major complications. If operation is indicated, preoperative antihistamines should be administered to prevent anaphylaxis. Albendazole or mebendazole also are administered preoperatively, e

Adult Polycystic Kidney and Liver Disease Adult polycystic kidney and liver disease (APKLD) is an autosomal dominant disease that adversely affects clinical performance status by producing progressive hepatomegaly in some patients. The cysts are variable in size and numerous, have a simple epithelial layer, and only rarely communicate with the biliary tree. Cyst distribution and extent varies from a few small cysts to total hepatic involvement, although nearly all patients have some parenchymal sparing. Although the cysts compress the hepatic parenchyma and produce hepatomegaly, liver function is preserved almost uniformly. Increased liver volume and adjacent visceral compression produce symptoms. Surgical treatment is dictated for patients who exhibit

77 significant impairment in clinical performance from massive hepatomegaly or patients with complications (e.g., bleeding, rupture, infection, jaundice). About 10% of patients with APKLD have intracranial aneurysms that should be addressed preoperatively, r In some patients with few dominant cysts (the minority), percutaneous cyst aspiration with alcohol sclerosis is appropriate. In patients with more diffuse disease, operative intervention is warranted. 8 The role of laparoscopy in this setting is limited to patients harboring only a few large peripheral cysts or some patients with medium-size cysts located anteriorly in the liver. 9 The surgical technique is the same as that described for simple cysts. However, most symptomatic patients require more extensive therapy. The choice between resection/fenestration and liver transplantation is undefined. We favor liver transplantation in patients who lack segmental sparing. If at least two adjacent segments are spared, we prefer resection/fenestration. ]

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SURGICAL TECHNIQUE Simple Cysts

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1 Laparoscopic unroofing of a simple cyst. Usually three ports are necessary, one each for the camera, grasper for the cyst wall, and cutting instrument (cautery, harmonic scalpel, or scissors). One of the working ports should be 10 mm to allow clip application. Placement varies according to the anatomic location and size of the cyst. This illustration shows the port sites that we use most often. After the laparoscope is introduced and the abdominal cavity inspected, the dome of the cyst is elevated with a grasper. The wall of the cyst is resected with electrocautery. We emphasize excision as close as possible to the cyst-liver tissue interface.

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Hepatic Cysts

2 After the cyst is incised, cyst contents are drained making the flaccid cyst wall easier to handle. Cytology of the aspirate is performed if indicated. It is important to resect as much of the wall of the cyst as possible to enhance retraction of the remnant edge of the cyst, thus preventing reapproximation of the rim by contraction which leads to cyst recurrence. Effaced ducts or vessels at the cyst-liver interface should be stapled or clipped. The cyst wall is removed and assessed microscopically.

3 After unroofing, the residual cyst wall is carefully inspected. Irregularities within the concavity of the cyst are biopsied. When less than 50% of the cyst has been removed, ablation of the remnant cyst lining by cautery, argon beam coagulation, or topical sclerosant may reduce the incidence of recurrence. Rarely, omentum can be placed within the cyst remnant to prevent recurrence.

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Not all simple cysts are amenable to laparoscopic therapy. Cysts located superiorly in segments 7 and 8 are very difficult to access without mobilizing the liverff This illustration shows a cyst in the right posterior segment. A right subcostal incision is indicated in these patients; we usually divide the triangular ligament and a portion of the coronary ligament to rotate the liver and bring the cyst closer to the wound. Using the nondominant hand, the surgeon pushes the liver toward the midline to facilitate exposure of the cyst. Following the same principles espoused in the laparoscopic approach, the cyst is resected. Alternatively, the cyst can be enucleated en toto.

5 The final result after resection shows a "clean" concavity of the cyst with basically no cyst wall above the level of the hepatic parenchyma. Occasionally, blood vessels or bile ducts can be seen in the cavity. No drains are necessary after cyst excision.

Hepatic Cysts

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Biliary Cystadenoma and Cystadenocarcinoma

6 A cystadenoma in the inferior portion of the liver.

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7 After mobilization of the liver is achieved, countertraction is imposed by the surgeon's nondominant hand. The interface between the cyst and the hepatic parenchyma is identified and developed. The cyst wall is sufficiently thick allowing the surgeon to dissect it away from the liver without rupture. With deeper dissection, compressed vessels and bile ducts become evident and are preserved. This dissection is continued circumferentially with enucleation of the cystadenoma. 1~ The cyst is sent for histologic analysis to exclude occult cystadenocarcinoma. If this finding is present, forreal hepatic lobectomy is indicated. 11

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8 When inadvertent injury of the bile ducts occurs, fine (4-0 or 5-0 absorbable) interrupted suture is used for repair. Abdominal drainage is optional. Complete excision of the cystadenoma eliminates the risk of recurrence. 4

Echinococcal Cysts

9 After routine abdominal exploration and liver exposure, the cyst is identified and isolated with saline-soaked gauze (20%) packs peripherally. This preventive measure may reduce the risk of anaphylactic reactions if cyst contents spill into the peritoneal cavity.

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83 Cyst is drained

Scolicidal solution is injected

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l 0 The cyst is decompressed to avoid inadvertent rupture during manipulation. Once the cyst is drained and bile duct communication is excluded, a scolicidal solution is instilled into the cyst. Adequate duration of exposure to the agent is necessary. Scolicidal instillation is contraindicated with bile duct rupture because of the risk of biliary sclerosis.

l 1 The cyst is opened, and the laminated cyst membrane and daughter cysts are removed manually, again avoiding spillage.

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Fibrous capsule Risk of injury to underlying vessels

Prefered method

12 It is not our practice to perform a cystopericystectomy ~2 because of the risk of hemorrhage and bile duct injury. We prefer to leave the host capsule behind, because it is noninfectious and because excision may lead to vascular or bile duct injury. We advise removal of the cyst, staying in the plane between the host capsule and the actual cyst to avoid unnecessary complications.

13 Final appearance of the cyst after excision. One must be very careful to identify bile duct injuries at this stage.

Hepatic Cysts

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14 The cyst cavity is obliterated with omenturn, and the site is drained externally by a suction catheter.

Adult Polycystic Kidney and Liver Disease

15 Diffuse involvement of the right lobe with some cysts in the left lobe (i.e., lobar sparing). In this particular patient, resection of the right lobe and fenestration of cysts in the left lobe is the procedure of choice. Generally, the cysts on the right lobe are small, and almost no gross hepatic parenchyma exists between the walls of the cysts. Resection of the involved lobe has only a minimal affect on hepatic function.

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16 Right hepatectomy in these patients follows the same principles as resection for other conditions. The lobar hepatic artery and portal vein are divided initially, as is the right hepatic vein if possible. Large adjacent lymphatics should also be ligated. Liberal cyst decompression around the vasculature facilitates control of the major vessels. Moreover, reduction of liver size by decompression improves mobility.

17 After vascular control is achieved, the liver is divided by sequential cyst fenestration along the planned transection plane. This maneuver offers the advantage of quick advancement through the liver. Use of a cavitron ultrasonic suction aspirator (CUSA) enhances the dissection because of its dual suction and cautery capability. Any vessel or duct encountered should be ligated with sutures to reduce the risk of hemorrhage and bile leaks.

Hepatic Cysts

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18 The location of the vessels and the bile ducts compressed in the hepatic parenchyma surrounding the cysts (inset). These structures must be suture ligated to prevent postoperative complications. After resection is complete, central lobar cysts are unroofed. This illustration shows the final result of this procedure. Because the remnant cyst wall maintains its secretory capacity, epithelial ablation by cautery or an equivalent is performed to reduce postoperative drainage. Moreover, ensuring that the interior vena cava and the remaining hepatic veins are free of cyst compression reduces the risk of postoperative ascites.

Summary

The management of liver cysts should be individualized by cyst type, symptoms, and associated complications. Percutaneous aspiration/ablation therapy and laparoscopic approaches are used most commonly and have proven efficacious for simple cysts. Management of specific diseases such as APKLD is more complicated and dictates treatment in centers with hepatobiliary and transplantation expertise.

REFERENCES 1. Nagorney DM: Surgical management of cystic disease of the liver. In Blumgart LH, Fong Y (eds): Surgery of the Liver and Biliary Tract. Philadelphia, PA, W.B. Saunders, 1995, pp 1261-1276 2. Van Sonnenberg E, Wroblicka JT, D'Agostin HB, et al: Symptomatic hepatic cysts: Percutaneous drainage and sclerosis. Radiology 190:387-392, 1994 3. Gigot JF, Legrand M, Hubens G, et al: Laparoscopic treatment of nonparasitic livers cysts: Adequate selection of patients and surgical technique. WorldJ Surg 20:556-561, 1996

4. Davies W, Chow M, Weiland L, et al: Intrahepatic biliary cystadenomas: An institutional experience with 37 cases. HPB 1:141, 1999. 5. Men S, Hekimoglu B, Yucesoy C, et al: Percutaneous treatment of hepatic hydatid cysts: An alternative to surgery. AmJ Roentgenol, 172:83-89, 1999 6. Turkcapar AG, Ersoz S, Gungor C, et al: Surgical treatment of hepatic hydatidosis combined with perioperative treatment with albendazol. EurJ Surg 163:923-928, 1997 7. Geevarghese SK, Powers T, Marsh J, et al: Screening for cerebral aneurysms in patients with polycystic liver disease. South Med J 92:1167-1170, 1999 8. Que F, Nagorney DM, Gross JB Jr, et al: Liver resection and cyst fenestration in the treatment of severe polycystic liver disease. Gastroenterology 108:487-494, 1995 9. Martin IJ, McKinley AJ, Currie EJ, et al: Tailoring the management of nonparasitic liver cysts. Ann Surg 228:167-172, 1998 10. Pinson CW, MunsonJL, Rossi RL, et al: Enucleation of intrahepatic biliary cystadenomas. Surg Gynecol Obstet 168:534-537, 1989 11. Ishak KG, Willis SGW, Cummins SD, et al: Biliary cystadenoma and cystadenocarcinoma. Report of 14 cases and review of the literature. Cancer 39:322-338, 1977 12. Mentes A: Hydatid liver disease: A perspective in treatment. Dig Dis 12:150-160, 1994