Surgical Management of Nonparasitic Liver Disease
Cystic
Herminio Sanchez, MD, Michel Gagner, MD, Ricardo L. Rossi, MD, Burlington, Massachusetts, Roger L. Jenkins, MD, W. David Lewis, MD, Boston,Massachusetts, J. Lawrence Munson, MD, John W. Braasch, MD, Burlington, Massachusetts
We report clinical features, surgical management, recurrences, and follow-up study of 12 patients with simple hepatic cyst, 11 patients with polycystic liver disease, and 19 patients with cystadenoma who were surgically treated over a 25-year period. The median age of patients was 48 years, and 37 women and 5 men were in the series. The most common presenting symptom and physical finding were chronic abdominal pain and tenderness in the right upper quadrant. The most commonly associated disease was polycystic kidney disease, which was an associated finding in 5 of the 11 patients with polycystic liver disease (45%). The most valuable diagnostic studies in all groups were computed tomography and ultrasonography. The location of the disease was bilobar in patients with polycystic liver disease, with a right lobe predominance in 18% of patients. The right lobe was also predominant in 83% of patients with simple hepatic cyst and 58% of patients with cystadenoma. Of all solitary cystic lesions in the left lobe, 75% of them were cystadenomas. Of the 66 surgical procedures performed, aspiration was associated with a failure rate of 100%; partial excision, a failure rate of 61%; and total excision and liver resection, a failure rate of 0%. Orthotopic liver transplantation was performed in three patients and was associated with two early deaths. Partial excision relieved symptoms in three patients (43%) with polycystic liver disease. Total excision, enucleation, or liver resection with cyst(s) is the treatment of choice for nonparasitic cystic lesions of the liver.
From the Department of General Surgery (HS, MG, RLR, JLM, JWB), Lahey Clinic Medical Center, Burlington, Massachusetts, and the Department of Surgery (RLJ, WDL), New England Deaconess Hospital, Boston, Massachusetts. Requests for reprints should be addressed to Ricardo L. Rossi, MD, Department of General Surgery, Lahey Clinic Medical Center, 41 Mall Road, Burlington, Massachusetts 01805. Presented at the 3 1st Annual Meeting of the Society for Surgery of the Alimentary Tract, San Antonio, Texas, May 15-l 6, 1990.
N
onparasitic cystic liver disease arises from developmental abnormalities in the liver parenchyma of the intrahepatic and extrahepatic biliary system. It is a rare clinical entity that is seen with increasing frequency because of the common use of computed tomography and ultrasonography [I-4]. As a result of the low incidence of this disease, uniform management has not been defined clearly. The standard surgical management has been to operate when patients are symptomatic or when cysts cause complications, such as torsion [5], hemorrhage [ 6], rupture [ 71, infection [7], malignant degeneration [8], portal hypertension [9], and obstructive jaundice [lo]. Our objective is to define the role of surgical management of nonparasitic cystic liver disease, including simple hepatic cyst, polycystic liver disease, and cystadenoma. PATIENTS
AND METHODS
The clinical records of patients who underwent a surgical procedure for nonparasitic cystic liver disease at the Lahey Clinic Medical Center and at the New England Deaconess Hospital between January 1964 and October 1989 were retrospectively reviewed. Length of follow-up study was obtained from clinical charts or by telephone questionnaire or by both means. RESULTS
Of the 42 patients studied, 37 (88%) were women. The median age at presentation for surgery was 48 years (range, 20 to 72 years). For comparison, the results have been divided into three main groups: simple hepatic cysts (12 patients, 83% women), polycystic liver disease (11 patients, 91% women), and liver cystadenomas (19 patients, 88% women). No differences in the sex ratio were apparent. Age analysis showed that the median age of patients with polycystic liver disease was only 1 year more than that of patients with simple cysts and 13 years more than that of patients with cystadenomas (55, 54, and 42 years, respectively). Symptoms and physical findings: The preoperative symptoms and physical findings are presented in Tables I and II. Chronic abdominal pain was the most common presenting complaint in all groups. It was present in 75% of patients with simple cysts, in 91% of patients with polycystic liver disease, and in 79% of patients with cystadenomas. In the group with simple hepatic cyst, nausea or vomiting or both were present in 17% of patients, whereas in the group with polycystic liver disease an abdominal mass, fever, or ascites was present in 18% of patients. In the cystadenoma group, sepsis and cholangitis were present in 21% of patients. Of note is that 16% of patients with cystadenomas were asymptomatic. During physical examination, the presence of an ab-
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TABLE I
TABLE II Symptoms
Physlcal Flndlngs
Symptom
Simple (n = 12)
Polycystic (n = 11)
Cystadenoma (n = 19)
Chronic abdominal pain Abdominal mass Nausea or vomiting or
9 (75%) 1(6%) 2(17%)
10 (91%) 2 (18%) -
15 (79%) 3 (16%) 2(11%)
born Sepsis and cholangftis Fever Ascites Early satiety Abdominal distention Acute abdominal pain Sack pain Upper gastrofntestlnel tract hemorrhage lntracystfc hemorrhage Encephalopamy Edema of lower extremitles Asymptomatic
-
1(8%) 1(8%) 1(80/o) 1(6%) -
4 (21%) -
2(18%) 2(18%) 1(90/b) 1(9%)
-
-
1(5%) 1(5%) 1(5%)
1(8%)
-
3 (16%)
Physical Finding Abdominal mass Tenderness In right upper quadrant Hepatomegaly Ascltes Massive abdominal distention Edema of lower extremities Signs of portal hypertension
Polycystic (n = 11)
Cystadenoma (n = 19)
5 (42%) 3 (25%)
6 (55%) 6 (55%)
3 (16%) 10 (53%)
-
4 (36%) 2 (18%) 1(9%)
2(11%,) -
-
1(9%)
f (5%)
-
1(9%)
1(5%)
TABLE IV Sire and Locatlon of Cysts
Size and Location
TABLE III Radlologlc Studies ;‘;$I;
Po;;cy&s)tlc cYstynma 0
Radiologic Study Computed tomography Ultrasonography Arteriography Liver scan Endoscopic retrograde cholangiopancreatography Inferior venacavography Percutaneous transhepatic cholangiography
7 (86%) 6 (75%) 6 (67%) 5 (40%) l(O%) -
5 9 2 2
(100%) (100%) (100%) (50%) -
18 (67%) 17 (59%) 12 (67%)
2 (0%) -
1 (0%) l(O%)
dominal mass was the predominant finding in 42% of patients with simple cysts and 55% of patients with polycystic liver disease. Tenderness in the right upper abdominal quadrant or epigastric tenderness was found in more than half of the patients with polycystic liver disease or cystadenoma. The physical findings of ascites, hepatomegaly, and other signs of portal hypertension were mostly associated with polycystic liver disease. Associated diseases: The most commonly associated disease was polycystic kidney disease, seen only with polycystic liver disease in 45% of patients. The second most commonly associated finding was gallbladder disease, which was present in 17% of patients with simple hepatic cysts and in 16% of patients with cystadenoma but which was absent in patients with polycystic liver disease. Overall, only 5 patients, or less than 12%, had abnormal results on preoperative laboratory studies.
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Size Average (cm) Range (cm) Location Bilobar Right lobe Dominant right lobe Left lobe
Simple (n = 12)
Polycystic (n = 11)
Cystadenoma (n = 19)
9 3-16
Variable 0.2-15
11 4-25
10(83%) 2(17%)
11 (100%) 2(18%) -
2(11%) 11(56%) 6 (32%)
l(O%) 3 (33%)
n = total number of procedures; % = percentage of procedures that were dlagnostlc or highly suggestive of the diagnosis.
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Simple (n = 12)
Radiologic studies: We reviewed the results of 100 radiologic studies in 42 patients. The number of procedures in the three groups, with the percentage of procedures that were diagnostic or highly suggestive of the diagnosis, are presented in Table III. Computed tomography was the radiologic study that produced the highest diagnostic accuracy in all groups, followed closely by ultrasonography. Arteriography was useful and was also highly diagnostic in contrast with the results of nuclear scans of the liver, which were suggestive in only 40% to 50% of patients. Inferior venacavography, endoscopic retrograde cholangiopancreatography, and percutaneous transhepatic cholangiography were only performed selectively. Anatomicopatbologic features: The average size and location of cysts are presented in Table IV. Cystadenomas averaged 11 cm in size, which is slightly larger than the average size for simple liver cysts. In polycystic liver disease, the size of cysts was variable, and symptoms related to the summation of multiple cysts. In the group with simple hepatic cysts, 10 patients had a solitary cyst, and 2 patients had paired cysts. Simple cysts were predominantly located in the right lobe of the liver (83% of patients). Polycystic liver disease was always bilobar but demonstrated a pattern of right lobar dominance in 18% of patients. Of the 19 patients with cystadenomas, 6 pa-
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TABLE V Surgical Management and Recurrences
Lesion Simple Number Recurrences Median time to recurrence (mo) Median time of nonrecurrence (mo) Polycystic Number Recurrences Median time to recurrence (mo) Median time of nonrecurrence (mo) Cystadenomas Number Recurrences Median time to recurrence (mo) Median time of nonrecurrence (mo)
Total Excision
Orthotopic Liver Transplantation
Aspiration
Partial Excision
1 1 (100%) 24
3 (38%) 26
-
11
31
7 4 (57%) la
-
-
-
-
48
20
-
6 6 (100%) 24 -
4
135
9 9 (100%) 7 -
a
Resection
a 7 (86%) 36 19
-
-
1 -
-
a
2
5
9
2’
12
-
-
-
-
26
-
1’
number = number of procedures for each category; recurrences = number of recurrences for each category; % = percentage of recurrences for that procedure in that category. * Denotes one death.
tients had lesions located in the left lobe (32%). In consideration of the relative frequency of simple cyst uersus cystadenoma in the left lobe, six of eight lesions (75%) were found to be cystadenomas. This finding was compared with the right lobe, where cystadenomas were found with equal frequency. No evidence of malignant degeneration was found in the histologic study of any specimen, Surgical procedures, recurrences, and follow-up study: Sixty-six procedures were performed on 42 pa-
tients on the basis of symptoms or uncertainty of diagnosis. Aspirations were performed either radiographically or at the time of surgical exploration. Marsupialization and fenestration were procedures considered to be forms of partial excision because a portion of cystic tissue was left behind. Total excision included any procedure that totally removed the cyst without considerable resection of surrounding hepatic tissue (i.e., enucleation). Liver resection included formal or extended lobectomy, segmentectomy, or wedge resection wherein a cuff or liver tissue around the cyst was removed. Results of treatment for the three groups are given in Table V. Recurrence with simple liver cyst and cystadenoma was simply defined as the radiographic recurrence of a cyst as opposed to the definition of recurrence in polycystic liver disease, which was based on recurrent symptoms. Simple cyst. Partial excision was the most common
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procedure (57% of patients), followed by total excision (29% of patients). Of those patients who underwent partial excision, 38% had recurrences, and of those patients who underwent total excision, none had recurrences. The median time (range) to recurrence for three patients in the partial excision group was 26 (9 to 60) months. Four patients in the total excision group were followed up for a median (range) time of 31 (12 to 360) months with no recurrences. One patient who had resection of the liver was reported to be free of recurrence after a follow-up period of 8 months. Polycysticdisease.Partial excision was the most common procedure, followed in frequency by aspiration. Aspiration was a poor treatment method for symptoms because of a 100% rate of recurrence, and partial excision was associated with a 57% rate of recurrence at a median (range) time of 18 (4 to 36) months of follow-up. However, partial excision benefited 43% of patients for a median follow-up of 135 (60 to 168) months or more than 10 years median follow-up. Liver resection was performed in two patients, and neither patient had recurrence of symp toms at 44 and 52 months of follow-up. Orthotopic liver transplantation was performed in two patients. One patient had severe hepatomegaly and portal hypertension with bleeding varices and ascites. She also had severe polycystic kidney disease and renal failure that necessitated dialysis. She died perioperatively of hemorrhagic complications. The second patient had severe disability
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frequent complications are intracystic hemorrhage [6], infection, portal hypertension [ 91, and jaundice [IO]. In addition, torsion [5], malignant degeneration [8], and spontaneous rupture [ 131 have been reported occasionally. Many methods have been used to treat patients with symptomatic hepatic cysts. Percutaneous aspiration or simple drainage is associated with a high rate of recurrence and can lead to serious complications, such as hemorrhage, sepsis, peritonitis caused by leakage of bile, and perforation of the gallbladder [10,14,15]. In our series, one patient had needle aspiration under ultrasonographic guidance, and the cyst recurred 2 years later. Aspiration can be used to alleviate symptoms momentarily, principally while the patient awaits definitive treatment and jaundice is present [10,15]. Bean and Rodan [ 161 suggested that percutaneous aspiration and direct injection of sterile 95% ethanol into the cyst cavity is the treatment of choice because no recurrence was found on follow-up examinations of 6 to 18 months. A longer period of follow-up study is needed to conclude whether this modality of treatment is safe. Marsupialization or other partial excision procedures may be used to alleviate symptoms, but these are also associated with a high rate of recurrence. This therapeutic choice is based on age, symptoms, and clinical status of the patient. The pathologic specimen should be inspected thoroughly to rule out cystadenoma or cystadenocarcinoma, and communication with the biliary tree should be excluded. When a communication is identified, complete excision is the treatment of choice. Complete excision, COMMENTS Simple hepatic cysts occur in approximately 5% of the enucleation, or pericystectomy is the recommended treatgeneral population. Small cysts are usually found inci- ment of choice for symptomatic simple hepatic cysts. No evidence of recurrent disease was seen in four patients dentally during radiologic examination or at laparotomy and rarely produce symptoms. Symptomatic patients are with a median follow-up period of 3 1 months. Occasionoften women in their 40s and 50s. The symptoms report- ally, a formal anatomic resection is required, but the ed are those of pressure on adjacent organs, chiefly ab-’ lesions are usually amenable to simple enucleation. Polycystic liver disease is a common manifestation of dominal pain or nausea and vomiting, or all three. On physical examination, an abdominal mass or tenderness polycystic kidney disease and is associated with an autosomal dominant inheritance. In an autopsy study at in the right upper abdominal quadrant is often found. These cysts are usually located in the right side of the UCLA Medical Center, adult polycystic liver disease was liver. Moschcowitz [II] postulated that aberrant ducts identified in 0.13% of all patients and in 93% of patients are formed during embryogenesis, resulting in cystic for- with polycystic kidney disease. However, of patients with polycystic liver disease, about 35% had an associated mations in the biliary tree. According to von Meyenburg [ 121, embryologic maldevelopment leads to excessive polycystic kidney disease [I 71. This is less than in our numbers of intralobular ducts that progressively accumu- series in which 45% of patients with polycystic liver dislate fluid and eventually become cystic. The cysts usually ease had polycystic kidney disease. Most patients were contain nonbilious fluid and are usually lined by cuboidal women, and the most common symptom was chronic epithelium surrounded by basement membrane and fi- abdominal pain. On physical examination, an abdominal brous tissue. In our series, 10 of 12 patients had cuboidal mass, tenderness in the right upper abdominal quadrant, epithelium; the others had fibrous walls and chronic in- or hepatomegaly was found. These cysts are multiple and of variable sizes and are flammatory changes. On ultrasonography, the cyst is unilocular, thin- typically located in both lobes. Occasionally, a dominant walled, smooth in contour, and anechoic, while on com- pattern of the right lobe is seen. The cysts contain a puted tomography, the cyst appears homogenous with transparent fluid similar to plasma in composition. von water density. Irregularities, septations, and calcifica- Meyenburg complexes are sometimes observed and may tions of the cyst wall are suggestive of an infectious, explain their embryologic cause [18]. Normal parenchyma is compressed and is sometimes difficult to identineoplastic, or traumatic cause [l-4]. The natural history of simple hepatic cysts is known, fy between cysts. Ultrasonography and computed tomogwith less than 5% having a complicated course. The most raphy are highly diagnostic and show multiple cysts in
from the liver enlargement and polycystic kidney disease with relatively good kidney function. This patient is alive and well, is free of symptoms after a follow-up of 20 months, and has normal liver and kidney function. Cystadenoma.Liver resection was the most common procedure, followed by aspiration and partial excision. An important observation was that when aspiration or partial excision was performed, almost all patients had recurrent disease in contrast to patients undergoing total excision or liver resection who had no recurrences. The median length of follow-up study for total excision was too short to draw conclusions (9 months), but the resection group had a median length of follow-up study of 26 months. One patient with jaundice from coexisting endstage sclerosing cholangitis and thrombosis of the portal vein had orthotopic liver transplantation and died postoperatively of acute hepatic failure secondary to immediate nonfunction of the hepatic allograft. When all the pathologic entities were combined and the success rates of treatment were compared, aspiration was associated with a failure rate of 100% and partial excision with a failure rate of 61%, while no failures in the total excision or liver resection groups occurred. The median times of follow-up study (excluding deaths) in each treatment group were 8 (1 to 84) months for aspiration, 26 (1 to 168) months for partial excision, 12 (2 to 360) months for total excision, 31 (3 to 67) months for liver resection, and 20 months for the single surviving liver transplant patient.
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both lobes with characteristics similar to simple hepatic cysts. Cystic disease is usually demonstrable also in one or both kidneys and occasionally in the pancreas. It has usually been accepted that polycystic liver disease is a benign process in which the prognosis is determined by the extent of the kidney disease. However, in a series [ 191 of more than 100 patients with polycystic liver disease followed up for 28 years, polycystic kidney disease resulted in little morbidity, causing only 4 deaths. This finding corroborates our series in which 5 of 11 patients had polycystic kidney disease that resulted in 1 instance of renal insufficiency. Hepatic failure directly attributable to cyst formation or compression has not been reported. This finding may be explained by the fact that the compressed liver parenchyma is histologically and morphologically normal. In fact, liver parenchyma measured by computed tomography and plastic agar models is preserved in polycystic liver disease despite massive cystic involvement [20]. Complications are usually those of polycystic kidney disease, and complications attributable to polycystic liver disease are portal hypertension, bleeding esophageal varices, ascites, cyst infection, cholangitis, and, rarely, malignant degeneration [ZI ,221. Surgical management of patients with polycystic liver disease is different from that for patients with simple cysts or cystadenomas because multiple cysts continue to grow and appear de nouo after treatment [ 171. Therefore, the therapeutic aim is to control the larger cysts and relieve symptoms. Aspiration can be performed for temporary relief, but symptoms recurred 100% of the time in our series, with a median time (range) for recurrence of 24 (3 to 84) months. The instillation of ethanol or Pantopaque in the cysts of polycystic liver disease, after aspiration, has been performed by other authors [16,23], but longer follow-up study is needed before widespread acceptance of this method. Lin et al [24] described a fenestration procedure that involves partial excision of superficial cysts and unroofing deeper cysts through the common wall of the superficial cyst. This procedure results in a decrease in the size of the cystic mass and relieves pain and discomfort. Complications are more frequent in a patient with a massively enlarged liver because of the distorted anatomy and the difficulty in identifying the plane of dissection [2.5,26]. In our experience with seven patients with partial excision, four patients had recurrent disease with a median time for recurrence of 18 months. However, benefit was noted in 43% of patients, and three patients had relief of symptoms for a median period of 135 months. Partial resection appears to be the treatment of choice for symptomatic patients. We noted no recurrence of symptoms in two patients with a mean follow-up period of 48 months. Nagorney ef al [27], from the Mayo Clinic, reviewed their experience with polycystic liver disease in seven patients undergoing multisegmental hepatic resection. Abdominal relief was remarkable in four patients and moderate in two patients, with a median follow-up period of 17 months. One operative death occurred in a patient who had renal transplantation, and sepsis, coagulopathy, and intracranial hemorrhage developed,
Finally, orthotopic liver transplantation can be of benefit in carefully selected patients. Patients with associated renal failure may benefit from simultaneous kidney transplantation. Cystadenoma is usually a large multiloculated cystic tumor [28]. The wall is made of fibrous tissue with biliary epithelium and associated chronic inflammatory changes. The lesions are located predominantly in the right lobe of the liver, but a third of them are in the left lobe. This left lobe incidence is higher than that in the group with symptomatic simple cyst. Most patients are women; the median age of those with these lesions is 42 years. Symptoms are mostly chronic abdominal pain. Sepsis or cholangitis may occur and suggests an infected cystadenoma. Physical examination most often reveals tenderness in the right upper abdominal quadrant and, less often, an abdominal mass. Computed tomography and ultrasonography are the most helpful examinations but have been diagnostic or highly suggestive only 60% of the time. Computed tomography demonstrates a regular thick-walled cyst with internal septations, and ultrasonography shows an anechoic mass with echogenic septations or papillary projections or both [29,30]. However, neither study can establish the diagnosis with certainty because some cystadenomas are unilocular [31]. Surgical management of cystadenomas has included aspiration, drainage, marsupialization, partial excision, enucleation, cystectomy, excision, and resection of the liver. Aspiration is associated with a recurrence rate of lOO%,and partial excision methods are associated with a recurrence rate of nearly 90%. The treatment of choice remains complete excision with or without associated resection of the liver. We have treated 17 patients with either excision or resection with no recurrences. This experience corroborates the experience of others [28,32]. Cystadenocarcinomas may arise from cystadenomas, although we have not seen microscopic malignant degeneration in our specimens [28,33,34]. In summary, the treatment of choice in the three groups is complete excision, enucleation, or resection when it is able to be performed safely. Partial excision can be used to alleviate symptoms in 43% of patients with polycystic liver disease. Aspiration can be used as a temporary measure to alleviate symptoms while preparing the patient for a more definite procedure. REFERENCES 1. Taylor KJ, Viscomi GN. Ultrasound diagnosis of cystic disease of the liver. J Clin Gastroenterol 1980; 2: 197-204. 2. Kuni CC, Johnson ML, Holmes JH. Polycystic liver disease. J Clin Ultrasound 1987; 6: 332-3. 3. Barnes PA, Thomas JL, Bernardino ME. Pitfalls in the diagnosis of hepatic cysts by computed tomography. Radiology 1981; 141: 129933. 4. Brick SH, Hill MC, Lande IM. The mistaken or indeterminate CT diagnosis of hepatic metastases: the value of sonography. AJR Am J Roentgen01 1987; 148: 723-6. 5. Davis CK, Schoffstall RO, Glass TF. Fatal complication of hepatic cystic disease. South Med J 1981; 74: 1409-l 1. 6. Flagg RS, Robinson DW. Solitary nonparasitic hepatic cysts:
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report of oldest known case and review of the literature. Arch Surg 1967; 95: 964-73. 7. Gesundheit N, Kent DL, Fawcett HD, Effron MK, Maffly RH. Infected liver cyst in a patient with polycystic kidney disease. West J Med 1982; 136: 246-9. 8. Richmond HG. Carcinoma arising in congenital cysts of the liver. J Path01 Bacterial 1956; 72: 681-3. 9. Ratcliffe PJ, Reeders S, Theaker JM. Bleeding oesophageal varices and hepatic dysfunction in adult polycystic kidney disease. Br Med J 1984; 288: 1330-l 10. Cappell MS. Obstructive jaundice from benign, nonparastic hepatic cysts: identification of risk factors and percutaneous aspiration for diagnosis and treatment. Am J Gastrcenterol 1988; 83: 936. 11. Moschcowitz E. Non-parasitic cysts (congenital) of the liver with a study of aberrant bile ducts. Am J Med Sci 1906; 13 1: 67499. 12. von Meyenburg H. fiber die Cystenleber. Beitr Path01 Anat Allgem Path01 1918; 64: 477-532. 13. Ayyash K, Haddad J. Spontaneous rupture of a solitary nonparasitic cyst of the liver: case report. Acta Chir Stand 1988; 154: 214-3. 14. Saini S, Mueller PR, Ferrucci JT Jr, Simeone JF, Wittenberg J, Butch RJ. Percutaneous aspiration of hepatic cysts does not provide definitive therapy. AJR Am J Roentgen01 1983; 141: 55960. 15. Litwin DE, Taylor BR, Langer B, Greig P. Nonparasitic cysts of the liver: the case for conservative surgical management. Ann Surg 1987; 205: 45-8. 16. Bean WJ, Rodan BA. Hepatic cysts: treatment with alcohol. AJR Am J Roentgen01 1985; 144: 237-41. 17. Thomsen HS, Thaysen JH. Frequency of hepatic cysts in adult polycystic kidney disease. Acta Med Stand 1988; 224: 381-4. 18. Melnick PJ. Polycystic liver: analysis of 70 cases. Arch Path01 1955; 59: 162-72. 19. Peltokallio V. Non-parasitic cysts of the liver: a clinical study of 117 cases. Ann Chir Gynaecol Suppl (Fenn) 1970; 174: l-63. 20. Everson GT, Scherzinger A, Berger-Leff N, et al: Polycystic liver disease: quantitation of parenchymal and cyst volumes from computed tomography images and clinical correlates of hepatic cysts. Hepatology 1988; 8: 1627-34. 21. Bloustein PA. Association of carcinoma with congenital cystic conditions of the liver and bile ducts. Am J Gastroenterol 1977; 67: 40-6. 22. DelGuercio E, Greco J, Kim KE, Chinitz J, Swartz C. Esophageal varices in adult patients with polycystic kidney and liver disease. N Engl J Med 1973; 289: 678-9. 23. Goldstein HM, Carlyle DR, Nelson RS. Treatment of symptomatic hepatic cyst by percutaneous instillation of Pantopaque. AJR Am J Rcentgenol 1976; 127: 850-3. 24. Lin TY, Chen CC, Wang SM. Treatment of non-parasitic cystic disease of the liver: a new approach to therapy with polycystic liver. Ann Surg 1968; 168: 921-7. 25. Tumage RH, Eckhauser FE, Knol JA, Thompson NW. Therapeutic dilemmas in patients with symptomatic polycystic liver disease. Am Surg 1988; 54: 365-72. 26. Armitage NC, Blumgart LH. Partial resection and fenestration in the treatment of polycystic liver disease. Br J Surg 1984; 7 1: 2424. 27. Nagorney DM, Torres VE, Rakela J, Newman KD, Gross JB Jr. Highly symptomatic polycystic liver disease: a role for hepatic resection [Abstract 06661. In: Proceedings of the World Congress of Surgery, Toronto, September 10-16, 1989; 121. 28. Ishak KG, Willis GW, Cummins SD, Bullock AA. Biliary cystadenoma and cystadenocarcinoma: report of 14 cases and review of the literature. Cancer 1977; 39: 322-38. 29. Forrest ME, Cho KJ, Shields JJ, Wicks JD, Silver TM, McCormick TL. Biliary cystadenomas: sonographic-angiographicpathologic correlations. AJR Am J Roentgen01 1980; 135: 723-7. 30. Federle MP, Filly RA, Moss AA. Cystic hepatic neoplasms: 118
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complementary roles of CT and sonography. AJR Am J Roentgenol 1981; 136: 345-8. 31. Edwards JD, Eckhauser FE, Knol JA, Strode1 WE, Appelman HD. Optimizing surgical management of symptomatic solitary hepatic cysts. Am Surg 1987; 53: 510-4. 32. Lewis WD, Jenkins RL, Rossi RL, Munson L, et al. Surgical treatment of biliary cystadenoma: a report of 15 cases. Arch Surg 1988; 123: 563-8. 33. Woods GL. Biliary cystadenocarcinoma: case report of hepatic malignancy originating in benign cystadenoma. Cancer 1981; 47: 2936-40. 34. Iemoto Y, Kondo Y, Nakano T, Tsuchiya K, Ohto M. Biliary cystadenocarcinoma diagnosed by liver biopsy performed under ultrasonographic guidance. Gastroenterology 1983; 84: 399-403. DISCUSSION Robert Hermann
(Cleveland, OH): Did any of your patients have jaundice? We have had at least one patient present with a biliary cystadenoma that caused jaundice. The cyst actually did not contain bile, but it was an hepatic cyst that compressed the hepatic duct. Second, for simple hepatic cysts, especially giant ones, it has always seemed to me that it is rather hazardous to resect the entire capsule. I have partially excised the outer cyst wall for the last 20 years and have marsupialized water-clear cysts into the peritoneal cavity, where the fluid is secreted and absorbed. We have had no recurrences in our experience. Would you comment on that? I am surprised that you recommend excision for simple hepatic cysts. I certainly agree that excision would be appropriate for cystadenomas and polycystic livers. Bernard Langer (Toronto, Canada): I would like to urge some caution regarding the advice that simple cysts should be treated by complete excision. It is important to emphasize that most simple cysts do not need surgical treatment. Those that do are usually large cysts, which have compressed liver parenchyma and often have vascular and ductal structures in their wall. For this reason, our preferred treatment is partial excision and oversewing of the parenchymal cyst wall edges. If a few of these recur, they are usually small and asymptomatic, and by definition do not require surgical treatment. In our experience, we have only had one out of 19 patients who required reoperation after initial treatment by this technique of wide unroofing and peritoneal marsupialization. Robert Hall (Indianapolis, IN): I want to ask about the use of alcohol. You only mentioned your two cases. Can you give us some experience from the literature? Our radiologists are quite enthusiastic about alcohol for simple cysts. If you can distinguish between simple cysts and cystadenoma, then, of course, you want to treat the cystadenoma surgically. Even if a trial with alcohol for simple cysts fails, is that harmful? As the second point, I would like to emphasize that resections of cystadenomas can be done between the cyst wall and the liver parenchyma. You don’t have to resect any liver parenchyma and very little bleeding occurs. Dr. Nagorney (Rochester, MN): I would like to focus my comments and questions on polycystic liver disease. We have recently operated on 15 patients with extensive
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polycystic liver disease, of which the clinical management of 9 patients will be detailed in Annals of Surgery ( 1990; 212: 30-7). Most patients have had extended hepatic resections. We have found that the clinical performance status of such patients can be significantly diminished because of the massive liver enlargement. However, extended resection affords clinical improvement without impairing liver function. I have several questions related to your approach to these patients. First, you showed that some of the patients with polycystic liver disease do have some liver segments spared of cysts. We have found that there are one or two segments, if not more, that are spared in continuity and this finding affords extended hepatic resection of symp’ tomatic cyst mass often up to 80% of the liver volume, which certainly will reduce abdominal mass. Have you recognized segmental liver sparing in your patients? Secondly, most of the patients with polycystic liver disease have autosomal dominant polycystic kidney disease with renal insufficiency of variable degree. We have difficulty in deciding which patients should undergo extended hepatic resection and which we should offer liver transplantation with or without renal transplantation. I think there is a definite role for liver transplantation, but selection criteria remain undefined. Would you perform transplantation in these patients? I don’t think you can conclude that no patient is a transplant candidate based on one postoperative death. And finally, one comment on cystadenomas. It is helpful if a computed tomographic scan (CT) shows septations in the solitary cyst, supporting a diagnosis of cystadenoma. There are cystadenomas either on ultrasound or on CT that lack septations and this adds confusion to the clinical approach of large solitary hepatic cysts. Have you seen cystadenomas without septations? Lawrence Way (San Francisco, CA): Inevitably, the following philosophy is espoused: Why not try alcohol injection and then decide on surgery if it is unsuccessful? In my experience, there is a pitfall in this general strategy. It is difficult to treat giant cysts in this manner, because they tend to become infected secondary to the irregular way in which they collapse. We have seen several patients who would have been simple surgical candidates at the outset, but who were manipulated several times, developed infected giant cysts, and became much more difficult surgical candidates. However, I don’t feel so strongly about this if the cyst is in the range of 5 to 6 cm. How do you manage a symptomatic cyst deep in the liver? Sometimes it is difficult to excise the cyst and the question arises: Should you do a hepatic lobectomy? We have treated several cysts like this by unroofing and packing the cavity with omentum. Do you agree that this is still a useful technique in some of these deep-seated, medium-sized cysts in the right lobe of the liver? The spectrum of patients with polycystic liver disease may be changing in recent years due to the early use of renal transplantation in these patients. We have encountered several elderly patients with this disease, whereas in
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the past we have only seen patients in the younger age group. When they are elderly, they are a good deal more fragile and often present with complications. As a result, the operation may not be tolerated as well. We had one elderly patient, treated with immunosuppression for her renal transplant, who developed a lymphoma within her liver. Would you comment on whether the spectrum of disease may be changing somewhat? Although we have not used it, Roux-Y decompression is a treatment option for this disease in the surgical literature. Would you comment on this? Onye Akwari (Durham, NC): A few years ago, we became curious about the propensity of cystadenomas to recur and established that the histology of the cystadenoma was important. If they had mesenchymal stroma in the wall, that seemed to be correlated not only with recurrence but also with the propensity for malignant change. Please comment on the histology of your cystadenomas and especially those that recurred. Michel Gagner (closing): Dr. Hermann, we had no patients with jaundice. For simple hepatic cyst, we find that enucleation or excision is easy to perform. Dr. Hall, we agree with Dr. Langer that simple cysts have to be operated on sometimes. Regarding Dr. Hall’s comment, Dr. Bean has instilled alcohol into some cysts, but his follow-up is only between 9 and 18 months. He had no recurrences. It is our belief that the follow-up period is not long enough to make any conclusion with the instillation of alcohol. We have seen some segmental sparing in patients with polycystic disease, Dr. Nagorney. We had two patients with unilocular cystadenomas. These patients were operated on because of the size of their cysts and the diagnosis of cystadenoma was made postoperatively. Dr. Way, regarding the alcohol injection, we only had limited experience in two patients. It is the opinion of our department that, if the cyst communicates with the biliary tract, sclerosis of the biliary tree may result. Regarding symptomatic deep cysts, we prefer to perform a hepatic resection according to anatomic planes. We think it is easier and creates less morbidity in these patients. In a series of our patients that were studied for 28 years, only four had significant renal failure. Only 4% or 5% of these patients develop complications from renal failure. We had no patients who had renal transplantation prior to surgery, so I cannot comment on that. Regarding malignancy and lymphoma, we have not encountered lymphomas with polycystic disease, and we have not encountered any patients with cystadenocarcinoma. Roux-Y drainage was performed in two patients in whom infections developed. We had to take down the Roux limb and excise the cyst in both of these patients. Dr. Akwari, there was no malignant degeneration in any of the cystadenomas or the simple cysts. We could not correlate papillary or other histologic patterns with the likelihood of recurrence. Cysts recurred in seven of the eight patients who underwent partial excision.
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Cystic
Herminio Sanchez, MD, Michel Gagner, MD, Ricardo L. Rossi, MD, Burlington, Massachusetts, Roger L. Jenkins, MD, W. David Lewis, MD, Boston,Massachusetts, J. Lawrence Munson, MD, John W. Braasch, MD, Burlington, Massachusetts
We report clinical features, surgical management, recurrences, and follow-up study of 12 patients with simple hepatic cyst, 11 patients with polycystic liver disease, and 19 patients with cystadenoma who were surgically treated over a 25-year period. The median age of patients was 48 years, and 37 women and 5 men were in the series. The most common presenting symptom and physical finding were chronic abdominal pain and tenderness in the right upper quadrant. The most commonly associated disease was polycystic kidney disease, which was an associated finding in 5 of the 11 patients with polycystic liver disease (45%). The most valuable diagnostic studies in all groups were computed tomography and ultrasonography. The location of the disease was bilobar in patients with polycystic liver disease, with a right lobe predominance in 18% of patients. The right lobe was also predominant in 83% of patients with simple hepatic cyst and 58% of patients with cystadenoma. Of all solitary cystic lesions in the left lobe, 75% of them were cystadenomas. Of the 66 surgical procedures performed, aspiration was associated with a failure rate of 100%; partial excision, a failure rate of 61%; and total excision and liver resection, a failure rate of 0%. Orthotopic liver transplantation was performed in three patients and was associated with two early deaths. Partial excision relieved symptoms in three patients (43%) with polycystic liver disease. Total excision, enucleation, or liver resection with cyst(s) is the treatment of choice for nonparasitic cystic lesions of the liver.
From the Department of General Surgery (HS, MG, RLR, JLM, JWB), Lahey Clinic Medical Center, Burlington, Massachusetts, and the Department of Surgery (RLJ, WDL), New England Deaconess Hospital, Boston, Massachusetts. Requests for reprints should be addressed to Ricardo L. Rossi, MD, Department of General Surgery, Lahey Clinic Medical Center, 41 Mall Road, Burlington, Massachusetts 01805. Presented at the 3 1st Annual Meeting of the Society for Surgery of the Alimentary Tract, San Antonio, Texas, May 15-l 6, 1990.
N
onparasitic cystic liver disease arises from developmental abnormalities in the liver parenchyma of the intrahepatic and extrahepatic biliary system. It is a rare clinical entity that is seen with increasing frequency because of the common use of computed tomography and ultrasonography [I-4]. As a result of the low incidence of this disease, uniform management has not been defined clearly. The standard surgical management has been to operate when patients are symptomatic or when cysts cause complications, such as torsion [5], hemorrhage [ 6], rupture [ 71, infection [7], malignant degeneration [8], portal hypertension [9], and obstructive jaundice [lo]. Our objective is to define the role of surgical management of nonparasitic cystic liver disease, including simple hepatic cyst, polycystic liver disease, and cystadenoma. PATIENTS
AND METHODS
The clinical records of patients who underwent a surgical procedure for nonparasitic cystic liver disease at the Lahey Clinic Medical Center and at the New England Deaconess Hospital between January 1964 and October 1989 were retrospectively reviewed. Length of follow-up study was obtained from clinical charts or by telephone questionnaire or by both means. RESULTS
Of the 42 patients studied, 37 (88%) were women. The median age at presentation for surgery was 48 years (range, 20 to 72 years). For comparison, the results have been divided into three main groups: simple hepatic cysts (12 patients, 83% women), polycystic liver disease (11 patients, 91% women), and liver cystadenomas (19 patients, 88% women). No differences in the sex ratio were apparent. Age analysis showed that the median age of patients with polycystic liver disease was only 1 year more than that of patients with simple cysts and 13 years more than that of patients with cystadenomas (55, 54, and 42 years, respectively). Symptoms and physical findings: The preoperative symptoms and physical findings are presented in Tables I and II. Chronic abdominal pain was the most common presenting complaint in all groups. It was present in 75% of patients with simple cysts, in 91% of patients with polycystic liver disease, and in 79% of patients with cystadenomas. In the group with simple hepatic cyst, nausea or vomiting or both were present in 17% of patients, whereas in the group with polycystic liver disease an abdominal mass, fever, or ascites was present in 18% of patients. In the cystadenoma group, sepsis and cholangitis were present in 21% of patients. Of note is that 16% of patients with cystadenomas were asymptomatic. During physical examination, the presence of an ab-
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TABLE I
TABLE II Symptoms
Physlcal Flndlngs
Symptom
Simple (n = 12)
Polycystic (n = 11)
Cystadenoma (n = 19)
Chronic abdominal pain Abdominal mass Nausea or vomiting or
9 (75%) 1(6%) 2(17%)
10 (91%) 2 (18%) -
15 (79%) 3 (16%) 2(11%)
born Sepsis and cholangftis Fever Ascites Early satiety Abdominal distention Acute abdominal pain Sack pain Upper gastrofntestlnel tract hemorrhage lntracystfc hemorrhage Encephalopamy Edema of lower extremitles Asymptomatic
-
1(8%) 1(8%) 1(80/o) 1(6%) -
4 (21%) -
2(18%) 2(18%) 1(90/b) 1(9%)
-
-
1(5%) 1(5%) 1(5%)
1(8%)
-
3 (16%)
Physical Finding Abdominal mass Tenderness In right upper quadrant Hepatomegaly Ascltes Massive abdominal distention Edema of lower extremities Signs of portal hypertension
Polycystic (n = 11)
Cystadenoma (n = 19)
5 (42%) 3 (25%)
6 (55%) 6 (55%)
3 (16%) 10 (53%)
-
4 (36%) 2 (18%) 1(9%)
2(11%,) -
-
1(9%)
f (5%)
-
1(9%)
1(5%)
TABLE IV Sire and Locatlon of Cysts
Size and Location
TABLE III Radlologlc Studies ;‘;$I;
Po;;cy&s)tlc cYstynma 0
Radiologic Study Computed tomography Ultrasonography Arteriography Liver scan Endoscopic retrograde cholangiopancreatography Inferior venacavography Percutaneous transhepatic cholangiography
7 (86%) 6 (75%) 6 (67%) 5 (40%) l(O%) -
5 9 2 2
(100%) (100%) (100%) (50%) -
18 (67%) 17 (59%) 12 (67%)
2 (0%) -
1 (0%) l(O%)
dominal mass was the predominant finding in 42% of patients with simple cysts and 55% of patients with polycystic liver disease. Tenderness in the right upper abdominal quadrant or epigastric tenderness was found in more than half of the patients with polycystic liver disease or cystadenoma. The physical findings of ascites, hepatomegaly, and other signs of portal hypertension were mostly associated with polycystic liver disease. Associated diseases: The most commonly associated disease was polycystic kidney disease, seen only with polycystic liver disease in 45% of patients. The second most commonly associated finding was gallbladder disease, which was present in 17% of patients with simple hepatic cysts and in 16% of patients with cystadenoma but which was absent in patients with polycystic liver disease. Overall, only 5 patients, or less than 12%, had abnormal results on preoperative laboratory studies.
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Size Average (cm) Range (cm) Location Bilobar Right lobe Dominant right lobe Left lobe
Simple (n = 12)
Polycystic (n = 11)
Cystadenoma (n = 19)
9 3-16
Variable 0.2-15
11 4-25
10(83%) 2(17%)
11 (100%) 2(18%) -
2(11%) 11(56%) 6 (32%)
l(O%) 3 (33%)
n = total number of procedures; % = percentage of procedures that were dlagnostlc or highly suggestive of the diagnosis.
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Simple (n = 12)
Radiologic studies: We reviewed the results of 100 radiologic studies in 42 patients. The number of procedures in the three groups, with the percentage of procedures that were diagnostic or highly suggestive of the diagnosis, are presented in Table III. Computed tomography was the radiologic study that produced the highest diagnostic accuracy in all groups, followed closely by ultrasonography. Arteriography was useful and was also highly diagnostic in contrast with the results of nuclear scans of the liver, which were suggestive in only 40% to 50% of patients. Inferior venacavography, endoscopic retrograde cholangiopancreatography, and percutaneous transhepatic cholangiography were only performed selectively. Anatomicopatbologic features: The average size and location of cysts are presented in Table IV. Cystadenomas averaged 11 cm in size, which is slightly larger than the average size for simple liver cysts. In polycystic liver disease, the size of cysts was variable, and symptoms related to the summation of multiple cysts. In the group with simple hepatic cysts, 10 patients had a solitary cyst, and 2 patients had paired cysts. Simple cysts were predominantly located in the right lobe of the liver (83% of patients). Polycystic liver disease was always bilobar but demonstrated a pattern of right lobar dominance in 18% of patients. Of the 19 patients with cystadenomas, 6 pa-
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TABLE V Surgical Management and Recurrences
Lesion Simple Number Recurrences Median time to recurrence (mo) Median time of nonrecurrence (mo) Polycystic Number Recurrences Median time to recurrence (mo) Median time of nonrecurrence (mo) Cystadenomas Number Recurrences Median time to recurrence (mo) Median time of nonrecurrence (mo)
Total Excision
Orthotopic Liver Transplantation
Aspiration
Partial Excision
1 1 (100%) 24
3 (38%) 26
-
11
31
7 4 (57%) la
-
-
-
-
48
20
-
6 6 (100%) 24 -
4
135
9 9 (100%) 7 -
a
Resection
a 7 (86%) 36 19
-
-
1 -
-
a
2
5
9
2’
12
-
-
-
-
26
-
1’
number = number of procedures for each category; recurrences = number of recurrences for each category; % = percentage of recurrences for that procedure in that category. * Denotes one death.
tients had lesions located in the left lobe (32%). In consideration of the relative frequency of simple cyst uersus cystadenoma in the left lobe, six of eight lesions (75%) were found to be cystadenomas. This finding was compared with the right lobe, where cystadenomas were found with equal frequency. No evidence of malignant degeneration was found in the histologic study of any specimen, Surgical procedures, recurrences, and follow-up study: Sixty-six procedures were performed on 42 pa-
tients on the basis of symptoms or uncertainty of diagnosis. Aspirations were performed either radiographically or at the time of surgical exploration. Marsupialization and fenestration were procedures considered to be forms of partial excision because a portion of cystic tissue was left behind. Total excision included any procedure that totally removed the cyst without considerable resection of surrounding hepatic tissue (i.e., enucleation). Liver resection included formal or extended lobectomy, segmentectomy, or wedge resection wherein a cuff or liver tissue around the cyst was removed. Results of treatment for the three groups are given in Table V. Recurrence with simple liver cyst and cystadenoma was simply defined as the radiographic recurrence of a cyst as opposed to the definition of recurrence in polycystic liver disease, which was based on recurrent symptoms. Simple cyst. Partial excision was the most common
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procedure (57% of patients), followed by total excision (29% of patients). Of those patients who underwent partial excision, 38% had recurrences, and of those patients who underwent total excision, none had recurrences. The median time (range) to recurrence for three patients in the partial excision group was 26 (9 to 60) months. Four patients in the total excision group were followed up for a median (range) time of 31 (12 to 360) months with no recurrences. One patient who had resection of the liver was reported to be free of recurrence after a follow-up period of 8 months. Polycysticdisease.Partial excision was the most common procedure, followed in frequency by aspiration. Aspiration was a poor treatment method for symptoms because of a 100% rate of recurrence, and partial excision was associated with a 57% rate of recurrence at a median (range) time of 18 (4 to 36) months of follow-up. However, partial excision benefited 43% of patients for a median follow-up of 135 (60 to 168) months or more than 10 years median follow-up. Liver resection was performed in two patients, and neither patient had recurrence of symp toms at 44 and 52 months of follow-up. Orthotopic liver transplantation was performed in two patients. One patient had severe hepatomegaly and portal hypertension with bleeding varices and ascites. She also had severe polycystic kidney disease and renal failure that necessitated dialysis. She died perioperatively of hemorrhagic complications. The second patient had severe disability
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frequent complications are intracystic hemorrhage [6], infection, portal hypertension [ 91, and jaundice [IO]. In addition, torsion [5], malignant degeneration [8], and spontaneous rupture [ 131 have been reported occasionally. Many methods have been used to treat patients with symptomatic hepatic cysts. Percutaneous aspiration or simple drainage is associated with a high rate of recurrence and can lead to serious complications, such as hemorrhage, sepsis, peritonitis caused by leakage of bile, and perforation of the gallbladder [10,14,15]. In our series, one patient had needle aspiration under ultrasonographic guidance, and the cyst recurred 2 years later. Aspiration can be used to alleviate symptoms momentarily, principally while the patient awaits definitive treatment and jaundice is present [10,15]. Bean and Rodan [ 161 suggested that percutaneous aspiration and direct injection of sterile 95% ethanol into the cyst cavity is the treatment of choice because no recurrence was found on follow-up examinations of 6 to 18 months. A longer period of follow-up study is needed to conclude whether this modality of treatment is safe. Marsupialization or other partial excision procedures may be used to alleviate symptoms, but these are also associated with a high rate of recurrence. This therapeutic choice is based on age, symptoms, and clinical status of the patient. The pathologic specimen should be inspected thoroughly to rule out cystadenoma or cystadenocarcinoma, and communication with the biliary tree should be excluded. When a communication is identified, complete excision is the treatment of choice. Complete excision, COMMENTS Simple hepatic cysts occur in approximately 5% of the enucleation, or pericystectomy is the recommended treatgeneral population. Small cysts are usually found inci- ment of choice for symptomatic simple hepatic cysts. No evidence of recurrent disease was seen in four patients dentally during radiologic examination or at laparotomy and rarely produce symptoms. Symptomatic patients are with a median follow-up period of 3 1 months. Occasionoften women in their 40s and 50s. The symptoms report- ally, a formal anatomic resection is required, but the ed are those of pressure on adjacent organs, chiefly ab-’ lesions are usually amenable to simple enucleation. Polycystic liver disease is a common manifestation of dominal pain or nausea and vomiting, or all three. On physical examination, an abdominal mass or tenderness polycystic kidney disease and is associated with an autosomal dominant inheritance. In an autopsy study at in the right upper abdominal quadrant is often found. These cysts are usually located in the right side of the UCLA Medical Center, adult polycystic liver disease was liver. Moschcowitz [II] postulated that aberrant ducts identified in 0.13% of all patients and in 93% of patients are formed during embryogenesis, resulting in cystic for- with polycystic kidney disease. However, of patients with polycystic liver disease, about 35% had an associated mations in the biliary tree. According to von Meyenburg [ 121, embryologic maldevelopment leads to excessive polycystic kidney disease [I 71. This is less than in our numbers of intralobular ducts that progressively accumu- series in which 45% of patients with polycystic liver dislate fluid and eventually become cystic. The cysts usually ease had polycystic kidney disease. Most patients were contain nonbilious fluid and are usually lined by cuboidal women, and the most common symptom was chronic epithelium surrounded by basement membrane and fi- abdominal pain. On physical examination, an abdominal brous tissue. In our series, 10 of 12 patients had cuboidal mass, tenderness in the right upper abdominal quadrant, epithelium; the others had fibrous walls and chronic in- or hepatomegaly was found. These cysts are multiple and of variable sizes and are flammatory changes. On ultrasonography, the cyst is unilocular, thin- typically located in both lobes. Occasionally, a dominant walled, smooth in contour, and anechoic, while on com- pattern of the right lobe is seen. The cysts contain a puted tomography, the cyst appears homogenous with transparent fluid similar to plasma in composition. von water density. Irregularities, septations, and calcifica- Meyenburg complexes are sometimes observed and may tions of the cyst wall are suggestive of an infectious, explain their embryologic cause [18]. Normal parenchyma is compressed and is sometimes difficult to identineoplastic, or traumatic cause [l-4]. The natural history of simple hepatic cysts is known, fy between cysts. Ultrasonography and computed tomogwith less than 5% having a complicated course. The most raphy are highly diagnostic and show multiple cysts in
from the liver enlargement and polycystic kidney disease with relatively good kidney function. This patient is alive and well, is free of symptoms after a follow-up of 20 months, and has normal liver and kidney function. Cystadenoma.Liver resection was the most common procedure, followed by aspiration and partial excision. An important observation was that when aspiration or partial excision was performed, almost all patients had recurrent disease in contrast to patients undergoing total excision or liver resection who had no recurrences. The median length of follow-up study for total excision was too short to draw conclusions (9 months), but the resection group had a median length of follow-up study of 26 months. One patient with jaundice from coexisting endstage sclerosing cholangitis and thrombosis of the portal vein had orthotopic liver transplantation and died postoperatively of acute hepatic failure secondary to immediate nonfunction of the hepatic allograft. When all the pathologic entities were combined and the success rates of treatment were compared, aspiration was associated with a failure rate of 100% and partial excision with a failure rate of 61%, while no failures in the total excision or liver resection groups occurred. The median times of follow-up study (excluding deaths) in each treatment group were 8 (1 to 84) months for aspiration, 26 (1 to 168) months for partial excision, 12 (2 to 360) months for total excision, 31 (3 to 67) months for liver resection, and 20 months for the single surviving liver transplant patient.
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both lobes with characteristics similar to simple hepatic cysts. Cystic disease is usually demonstrable also in one or both kidneys and occasionally in the pancreas. It has usually been accepted that polycystic liver disease is a benign process in which the prognosis is determined by the extent of the kidney disease. However, in a series [ 191 of more than 100 patients with polycystic liver disease followed up for 28 years, polycystic kidney disease resulted in little morbidity, causing only 4 deaths. This finding corroborates our series in which 5 of 11 patients had polycystic kidney disease that resulted in 1 instance of renal insufficiency. Hepatic failure directly attributable to cyst formation or compression has not been reported. This finding may be explained by the fact that the compressed liver parenchyma is histologically and morphologically normal. In fact, liver parenchyma measured by computed tomography and plastic agar models is preserved in polycystic liver disease despite massive cystic involvement [20]. Complications are usually those of polycystic kidney disease, and complications attributable to polycystic liver disease are portal hypertension, bleeding esophageal varices, ascites, cyst infection, cholangitis, and, rarely, malignant degeneration [ZI ,221. Surgical management of patients with polycystic liver disease is different from that for patients with simple cysts or cystadenomas because multiple cysts continue to grow and appear de nouo after treatment [ 171. Therefore, the therapeutic aim is to control the larger cysts and relieve symptoms. Aspiration can be performed for temporary relief, but symptoms recurred 100% of the time in our series, with a median time (range) for recurrence of 24 (3 to 84) months. The instillation of ethanol or Pantopaque in the cysts of polycystic liver disease, after aspiration, has been performed by other authors [16,23], but longer follow-up study is needed before widespread acceptance of this method. Lin et al [24] described a fenestration procedure that involves partial excision of superficial cysts and unroofing deeper cysts through the common wall of the superficial cyst. This procedure results in a decrease in the size of the cystic mass and relieves pain and discomfort. Complications are more frequent in a patient with a massively enlarged liver because of the distorted anatomy and the difficulty in identifying the plane of dissection [2.5,26]. In our experience with seven patients with partial excision, four patients had recurrent disease with a median time for recurrence of 18 months. However, benefit was noted in 43% of patients, and three patients had relief of symptoms for a median period of 135 months. Partial resection appears to be the treatment of choice for symptomatic patients. We noted no recurrence of symptoms in two patients with a mean follow-up period of 48 months. Nagorney ef al [27], from the Mayo Clinic, reviewed their experience with polycystic liver disease in seven patients undergoing multisegmental hepatic resection. Abdominal relief was remarkable in four patients and moderate in two patients, with a median follow-up period of 17 months. One operative death occurred in a patient who had renal transplantation, and sepsis, coagulopathy, and intracranial hemorrhage developed,
Finally, orthotopic liver transplantation can be of benefit in carefully selected patients. Patients with associated renal failure may benefit from simultaneous kidney transplantation. Cystadenoma is usually a large multiloculated cystic tumor [28]. The wall is made of fibrous tissue with biliary epithelium and associated chronic inflammatory changes. The lesions are located predominantly in the right lobe of the liver, but a third of them are in the left lobe. This left lobe incidence is higher than that in the group with symptomatic simple cyst. Most patients are women; the median age of those with these lesions is 42 years. Symptoms are mostly chronic abdominal pain. Sepsis or cholangitis may occur and suggests an infected cystadenoma. Physical examination most often reveals tenderness in the right upper abdominal quadrant and, less often, an abdominal mass. Computed tomography and ultrasonography are the most helpful examinations but have been diagnostic or highly suggestive only 60% of the time. Computed tomography demonstrates a regular thick-walled cyst with internal septations, and ultrasonography shows an anechoic mass with echogenic septations or papillary projections or both [29,30]. However, neither study can establish the diagnosis with certainty because some cystadenomas are unilocular [31]. Surgical management of cystadenomas has included aspiration, drainage, marsupialization, partial excision, enucleation, cystectomy, excision, and resection of the liver. Aspiration is associated with a recurrence rate of lOO%,and partial excision methods are associated with a recurrence rate of nearly 90%. The treatment of choice remains complete excision with or without associated resection of the liver. We have treated 17 patients with either excision or resection with no recurrences. This experience corroborates the experience of others [28,32]. Cystadenocarcinomas may arise from cystadenomas, although we have not seen microscopic malignant degeneration in our specimens [28,33,34]. In summary, the treatment of choice in the three groups is complete excision, enucleation, or resection when it is able to be performed safely. Partial excision can be used to alleviate symptoms in 43% of patients with polycystic liver disease. Aspiration can be used as a temporary measure to alleviate symptoms while preparing the patient for a more definite procedure. REFERENCES 1. Taylor KJ, Viscomi GN. Ultrasound diagnosis of cystic disease of the liver. J Clin Gastroenterol 1980; 2: 197-204. 2. Kuni CC, Johnson ML, Holmes JH. Polycystic liver disease. J Clin Ultrasound 1987; 6: 332-3. 3. Barnes PA, Thomas JL, Bernardino ME. Pitfalls in the diagnosis of hepatic cysts by computed tomography. Radiology 1981; 141: 129933. 4. Brick SH, Hill MC, Lande IM. The mistaken or indeterminate CT diagnosis of hepatic metastases: the value of sonography. AJR Am J Roentgen01 1987; 148: 723-6. 5. Davis CK, Schoffstall RO, Glass TF. Fatal complication of hepatic cystic disease. South Med J 1981; 74: 1409-l 1. 6. Flagg RS, Robinson DW. Solitary nonparasitic hepatic cysts:
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report of oldest known case and review of the literature. Arch Surg 1967; 95: 964-73. 7. Gesundheit N, Kent DL, Fawcett HD, Effron MK, Maffly RH. Infected liver cyst in a patient with polycystic kidney disease. West J Med 1982; 136: 246-9. 8. Richmond HG. Carcinoma arising in congenital cysts of the liver. J Path01 Bacterial 1956; 72: 681-3. 9. Ratcliffe PJ, Reeders S, Theaker JM. Bleeding oesophageal varices and hepatic dysfunction in adult polycystic kidney disease. Br Med J 1984; 288: 1330-l 10. Cappell MS. Obstructive jaundice from benign, nonparastic hepatic cysts: identification of risk factors and percutaneous aspiration for diagnosis and treatment. Am J Gastrcenterol 1988; 83: 936. 11. Moschcowitz E. Non-parasitic cysts (congenital) of the liver with a study of aberrant bile ducts. Am J Med Sci 1906; 13 1: 67499. 12. von Meyenburg H. fiber die Cystenleber. Beitr Path01 Anat Allgem Path01 1918; 64: 477-532. 13. Ayyash K, Haddad J. Spontaneous rupture of a solitary nonparasitic cyst of the liver: case report. Acta Chir Stand 1988; 154: 214-3. 14. Saini S, Mueller PR, Ferrucci JT Jr, Simeone JF, Wittenberg J, Butch RJ. Percutaneous aspiration of hepatic cysts does not provide definitive therapy. AJR Am J Roentgen01 1983; 141: 55960. 15. Litwin DE, Taylor BR, Langer B, Greig P. Nonparasitic cysts of the liver: the case for conservative surgical management. Ann Surg 1987; 205: 45-8. 16. Bean WJ, Rodan BA. Hepatic cysts: treatment with alcohol. AJR Am J Roentgen01 1985; 144: 237-41. 17. Thomsen HS, Thaysen JH. Frequency of hepatic cysts in adult polycystic kidney disease. Acta Med Stand 1988; 224: 381-4. 18. Melnick PJ. Polycystic liver: analysis of 70 cases. Arch Path01 1955; 59: 162-72. 19. Peltokallio V. Non-parasitic cysts of the liver: a clinical study of 117 cases. Ann Chir Gynaecol Suppl (Fenn) 1970; 174: l-63. 20. Everson GT, Scherzinger A, Berger-Leff N, et al: Polycystic liver disease: quantitation of parenchymal and cyst volumes from computed tomography images and clinical correlates of hepatic cysts. Hepatology 1988; 8: 1627-34. 21. Bloustein PA. Association of carcinoma with congenital cystic conditions of the liver and bile ducts. Am J Gastroenterol 1977; 67: 40-6. 22. DelGuercio E, Greco J, Kim KE, Chinitz J, Swartz C. Esophageal varices in adult patients with polycystic kidney and liver disease. N Engl J Med 1973; 289: 678-9. 23. Goldstein HM, Carlyle DR, Nelson RS. Treatment of symptomatic hepatic cyst by percutaneous instillation of Pantopaque. AJR Am J Rcentgenol 1976; 127: 850-3. 24. Lin TY, Chen CC, Wang SM. Treatment of non-parasitic cystic disease of the liver: a new approach to therapy with polycystic liver. Ann Surg 1968; 168: 921-7. 25. Tumage RH, Eckhauser FE, Knol JA, Thompson NW. Therapeutic dilemmas in patients with symptomatic polycystic liver disease. Am Surg 1988; 54: 365-72. 26. Armitage NC, Blumgart LH. Partial resection and fenestration in the treatment of polycystic liver disease. Br J Surg 1984; 7 1: 2424. 27. Nagorney DM, Torres VE, Rakela J, Newman KD, Gross JB Jr. Highly symptomatic polycystic liver disease: a role for hepatic resection [Abstract 06661. In: Proceedings of the World Congress of Surgery, Toronto, September 10-16, 1989; 121. 28. Ishak KG, Willis GW, Cummins SD, Bullock AA. Biliary cystadenoma and cystadenocarcinoma: report of 14 cases and review of the literature. Cancer 1977; 39: 322-38. 29. Forrest ME, Cho KJ, Shields JJ, Wicks JD, Silver TM, McCormick TL. Biliary cystadenomas: sonographic-angiographicpathologic correlations. AJR Am J Roentgen01 1980; 135: 723-7. 30. Federle MP, Filly RA, Moss AA. Cystic hepatic neoplasms: 118
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complementary roles of CT and sonography. AJR Am J Roentgenol 1981; 136: 345-8. 31. Edwards JD, Eckhauser FE, Knol JA, Strode1 WE, Appelman HD. Optimizing surgical management of symptomatic solitary hepatic cysts. Am Surg 1987; 53: 510-4. 32. Lewis WD, Jenkins RL, Rossi RL, Munson L, et al. Surgical treatment of biliary cystadenoma: a report of 15 cases. Arch Surg 1988; 123: 563-8. 33. Woods GL. Biliary cystadenocarcinoma: case report of hepatic malignancy originating in benign cystadenoma. Cancer 1981; 47: 2936-40. 34. Iemoto Y, Kondo Y, Nakano T, Tsuchiya K, Ohto M. Biliary cystadenocarcinoma diagnosed by liver biopsy performed under ultrasonographic guidance. Gastroenterology 1983; 84: 399-403. DISCUSSION Robert Hermann
(Cleveland, OH): Did any of your patients have jaundice? We have had at least one patient present with a biliary cystadenoma that caused jaundice. The cyst actually did not contain bile, but it was an hepatic cyst that compressed the hepatic duct. Second, for simple hepatic cysts, especially giant ones, it has always seemed to me that it is rather hazardous to resect the entire capsule. I have partially excised the outer cyst wall for the last 20 years and have marsupialized water-clear cysts into the peritoneal cavity, where the fluid is secreted and absorbed. We have had no recurrences in our experience. Would you comment on that? I am surprised that you recommend excision for simple hepatic cysts. I certainly agree that excision would be appropriate for cystadenomas and polycystic livers. Bernard Langer (Toronto, Canada): I would like to urge some caution regarding the advice that simple cysts should be treated by complete excision. It is important to emphasize that most simple cysts do not need surgical treatment. Those that do are usually large cysts, which have compressed liver parenchyma and often have vascular and ductal structures in their wall. For this reason, our preferred treatment is partial excision and oversewing of the parenchymal cyst wall edges. If a few of these recur, they are usually small and asymptomatic, and by definition do not require surgical treatment. In our experience, we have only had one out of 19 patients who required reoperation after initial treatment by this technique of wide unroofing and peritoneal marsupialization. Robert Hall (Indianapolis, IN): I want to ask about the use of alcohol. You only mentioned your two cases. Can you give us some experience from the literature? Our radiologists are quite enthusiastic about alcohol for simple cysts. If you can distinguish between simple cysts and cystadenoma, then, of course, you want to treat the cystadenoma surgically. Even if a trial with alcohol for simple cysts fails, is that harmful? As the second point, I would like to emphasize that resections of cystadenomas can be done between the cyst wall and the liver parenchyma. You don’t have to resect any liver parenchyma and very little bleeding occurs. Dr. Nagorney (Rochester, MN): I would like to focus my comments and questions on polycystic liver disease. We have recently operated on 15 patients with extensive
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polycystic liver disease, of which the clinical management of 9 patients will be detailed in Annals of Surgery ( 1990; 212: 30-7). Most patients have had extended hepatic resections. We have found that the clinical performance status of such patients can be significantly diminished because of the massive liver enlargement. However, extended resection affords clinical improvement without impairing liver function. I have several questions related to your approach to these patients. First, you showed that some of the patients with polycystic liver disease do have some liver segments spared of cysts. We have found that there are one or two segments, if not more, that are spared in continuity and this finding affords extended hepatic resection of symp’ tomatic cyst mass often up to 80% of the liver volume, which certainly will reduce abdominal mass. Have you recognized segmental liver sparing in your patients? Secondly, most of the patients with polycystic liver disease have autosomal dominant polycystic kidney disease with renal insufficiency of variable degree. We have difficulty in deciding which patients should undergo extended hepatic resection and which we should offer liver transplantation with or without renal transplantation. I think there is a definite role for liver transplantation, but selection criteria remain undefined. Would you perform transplantation in these patients? I don’t think you can conclude that no patient is a transplant candidate based on one postoperative death. And finally, one comment on cystadenomas. It is helpful if a computed tomographic scan (CT) shows septations in the solitary cyst, supporting a diagnosis of cystadenoma. There are cystadenomas either on ultrasound or on CT that lack septations and this adds confusion to the clinical approach of large solitary hepatic cysts. Have you seen cystadenomas without septations? Lawrence Way (San Francisco, CA): Inevitably, the following philosophy is espoused: Why not try alcohol injection and then decide on surgery if it is unsuccessful? In my experience, there is a pitfall in this general strategy. It is difficult to treat giant cysts in this manner, because they tend to become infected secondary to the irregular way in which they collapse. We have seen several patients who would have been simple surgical candidates at the outset, but who were manipulated several times, developed infected giant cysts, and became much more difficult surgical candidates. However, I don’t feel so strongly about this if the cyst is in the range of 5 to 6 cm. How do you manage a symptomatic cyst deep in the liver? Sometimes it is difficult to excise the cyst and the question arises: Should you do a hepatic lobectomy? We have treated several cysts like this by unroofing and packing the cavity with omentum. Do you agree that this is still a useful technique in some of these deep-seated, medium-sized cysts in the right lobe of the liver? The spectrum of patients with polycystic liver disease may be changing in recent years due to the early use of renal transplantation in these patients. We have encountered several elderly patients with this disease, whereas in
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the past we have only seen patients in the younger age group. When they are elderly, they are a good deal more fragile and often present with complications. As a result, the operation may not be tolerated as well. We had one elderly patient, treated with immunosuppression for her renal transplant, who developed a lymphoma within her liver. Would you comment on whether the spectrum of disease may be changing somewhat? Although we have not used it, Roux-Y decompression is a treatment option for this disease in the surgical literature. Would you comment on this? Onye Akwari (Durham, NC): A few years ago, we became curious about the propensity of cystadenomas to recur and established that the histology of the cystadenoma was important. If they had mesenchymal stroma in the wall, that seemed to be correlated not only with recurrence but also with the propensity for malignant change. Please comment on the histology of your cystadenomas and especially those that recurred. Michel Gagner (closing): Dr. Hermann, we had no patients with jaundice. For simple hepatic cyst, we find that enucleation or excision is easy to perform. Dr. Hall, we agree with Dr. Langer that simple cysts have to be operated on sometimes. Regarding Dr. Hall’s comment, Dr. Bean has instilled alcohol into some cysts, but his follow-up is only between 9 and 18 months. He had no recurrences. It is our belief that the follow-up period is not long enough to make any conclusion with the instillation of alcohol. We have seen some segmental sparing in patients with polycystic disease, Dr. Nagorney. We had two patients with unilocular cystadenomas. These patients were operated on because of the size of their cysts and the diagnosis of cystadenoma was made postoperatively. Dr. Way, regarding the alcohol injection, we only had limited experience in two patients. It is the opinion of our department that, if the cyst communicates with the biliary tract, sclerosis of the biliary tree may result. Regarding symptomatic deep cysts, we prefer to perform a hepatic resection according to anatomic planes. We think it is easier and creates less morbidity in these patients. In a series of our patients that were studied for 28 years, only four had significant renal failure. Only 4% or 5% of these patients develop complications from renal failure. We had no patients who had renal transplantation prior to surgery, so I cannot comment on that. Regarding malignancy and lymphoma, we have not encountered lymphomas with polycystic disease, and we have not encountered any patients with cystadenocarcinoma. Roux-Y drainage was performed in two patients in whom infections developed. We had to take down the Roux limb and excise the cyst in both of these patients. Dr. Akwari, there was no malignant degeneration in any of the cystadenomas or the simple cysts. We could not correlate papillary or other histologic patterns with the likelihood of recurrence. Cysts recurred in seven of the eight patients who underwent partial excision.
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