Congenital Philip CYSTIC Choledochal

DISEASES

M. Hatfield, OF THE

Diseases of the Gallbladder and Bile Ducts

M.D., Francis J. Scholz, BILE

DUCTS

Cyst

Choledochal cysts are rare, not exceeding one caseper two million people.23In Western cultures, they are four tcmfive times more frequent in women than in men. but there is no sex predilection among Orientals, in whom the disorder is more prevalent.2 The great majority (83%))of the 403 patients collected by Alonso-Lej et al.2 were lessthan 30 years of age; 18% were recognized in the tirst year of life. The pathogcnesisis poorly understood. Most patients appear to have both a weaknessin the common duct wall and distal obstruction. “The first factor is always congenital, while the second, usually congenital.,can alsobe acquired.“’ Among the eight casesreported from the Lahey Clinic, the obstruction appeared to be congenital in four (hypoplasia and obliteration) and acquired in four (fibrosis of the sphincter of Oddi or a web).46 An accurate preoperative diagnosishasbeen the exception rather than the rule. The classicaltriad of a palpable right upper quadrant mass,pain, and jaundice is present in no more than two-thirds of the patients.43 Before ultrasound, rose bengal scanning. and computerized tomography were available, the diagnosiswas seldomestablished.In only 15.5%of the 232 casescollected by Tsardakas and Robnett43 in 1956 was the diagnosismadeon plain abdominal radiographsand barium studies.

Philip M. Hatfield., M.D.: Radiologist, Department of Radiology, Lahey Clinic Foundation, and Radiologist, Department of Radiology. New England Baptist Hospital, Boston, Mass. Francis J. Scholz, M.D.: Radiologist, Departmen t of Diagnostic Radiology, Lahey Clinic Foundation, and Clinical instructor in Radiology, Harvard Medical School, Boston, Mass. Robert E. Wise, M.D.: Chairman. Department of Diagnostic Radiology, Lahev Clinic Foundation, Chairman, Department of Radiology, New England Baptist Hospital, and Clinical Professor of Radiology, Boston University School of Medicine, Boston, Mass. Reprint requests .should be addressed to Philip M. Hatfield, M.D., 605 Commonwealth Avenue, Boston, Mass. 022 15. 0 1976 by Grune & Stratton, Inc.

Seminars

in Roentgenology,

Vol.

Xl,

No.

4 (October),

1976

M.D., and Robert E. Wise, M.D.

On occasion, a rounded right upper quadrant massis evident on the plain film (Fig. I). The secondportion of the duodenum may be displaced either medially (Fig. 2) or laterally on the upper gastrointestinal examination. In the absence of jaundice, the cyst may occasionally be opacified by means of oral cholecystographyz7 or intravenous cholangiography.‘9~2S~3’~32~36~39~42 It may be necessary to prolong intravenous chottngiography beyond the customary 7 hr to achieve diagnostic quality opacification (Fig. 212). If the patient is jaundiced. radioactive rose bcngai scanning.48 echography, or computerized tomography may be diagnostic. Complications include malignant degeneration, calculi (Fig. 3). cyst rupture.“” and portal hypertension.‘55’6 These ale rarely demonstrated preoperativety.

The origin of this rare anomaly conrlnues to generate considerable interest but has not been resolved fully, asreflected by the variety of apellations it has been accorded. Although the term choledochocele is preferred, the disorder hasalso been called intraduodenal duplication cyst, enterogenouscyst of the ampulla of Vater, divcrticulum of the common bile duct, enterogenous cyst of the duodenum, and communicating enterogenous cyst.41 There have been 16 patients with chotedochocele reported. including 9 women and 7 men. The average age at the time of diagnosiswas 33 years; 7 were lessthan 20 years old. The majority of patients experience episodic pain. jaundice. and nauseaand vomiting. On Gl series,a slightly compressibleor mobile polypoid defect is demonstratedin the regionof the ampulla of Vater. The diagnosiscan be made if the sac opacifies on intravenous cholangiography, as it often does (Fig. 4). The similarity to a ureterocele is striking. Unlike intraluminal diverticulum of the duodenum, the choledochocele doesnot fill from the duodenum during an upper GI series but instead appearsasa filling defect. Two types have been demonstrated. In the more common one, “the common bile duct terminated in the cyst and the cyst drained directly into the 235

HATFIELD,

236

SCHOLZ,

AND

WISE

not entirely clear, the condition appears to represent an advanced form of the more common small diverticulum-like outpouchings of the distal common bile duct illustrated in Fig. 5. While this may be a logical explanation, it does not account for the fact that the majority of choledochoceles are lined by duodenal and not common duct epithelium. Solitary (Nonparasitic)

Fig. 1. Choledochal Note the large round mass (arrows).

cyst. right

Plain abdominal upper quadrant

radiograph. soft-tissue

duodenum through an aperture in its wall. . . . In the other type, the cyst drained into the adjacent intramural portion of the common duct and out via the papilla of Vater.“41 Although the pathogenesis of choledochocele is

Fig. 2. Choledochal cyst. (A) Tomogrzm during of the cyst (arrows). (B) After ingestion of barium, the duodenum (between arrows) is evident.

intravenous indentation

Liver Cyst

Solitary liver cyst is uncommon, occurring with approximately the same frequency as choledochal cyst.23 About 3.50 cases were reported through 1972.3 Women are affected twice as often as men. Although those cysts that become manifest clinically are usually diagnosed in the fifth decade or later, the majority remain silent. Only 12 of the 38 solitary liver cysts reported from the Mayo Clinic produced symptoms; the rest were incidental findings at surgery or autopsy.26 The silent cysts averaged 2.6 cm in diameter and the symptomatic ones 10.8 cm. The diagnosis is less often made in childhood.‘* Griscom found only two liver cysts among the 117 cases of neonatal abdominal mass reported from Children’s Hospital in Boston.18 In

cholangiography, and slight medial

4-hr delayed displacement

film. There is opacification of the proximal portion

of

CONGENITAL

Fig. 3. operative

DISEASES

Choledochal cholangiogram.

OF

cyst

THE

containing

Fig. 4. Choledochocele. Tomogram cholangiography. Note the sac-like common bile duct (arrows).

GALLBLADDER

calculi

(arrows).

during intravenous termination of

the

AND

BILE

(A)

Tomogram

237

DUCTS

during

intravenous

cholangiography.

(6)

T-tube

one instance, a cyst that grew to gigantic proportions in utero seriously interfered with parturition.38 The symptomatic patient usually experiences recurrent dull right upper quadrant pain or notes an enlarging abdominal mass. At least four instances of rupture of a solitary liver cysl are also on record. On occasion, a cyst arising from the surface of the liver presents with acute abdominal pain caused by torsion of its pedicle.23 Jaundice is uncommon, resulting either from extrinsic compression of the common bile duct10~29 or from intrinsic obstruction by a rare intraluminal cyst.4s Malignant degeneration within a solitary liver cyst is also rare but has been observed on at least two occasions.3’40 The etiology is usually unclear. Simple unilocular cysts may have any type of epithelium.26 The majority are probably congenital in origin, deriving either from a congenital bile duct rest or an aberrant duct obstructed by injury or inflammation.23 Localization and characterization of the mass are easily accomplished with a 99mTc-sulfur colloid liver scan (Fig. 6), ultrasound (Fig. 7A), or by computerized tomography (Fig. 7B). lnt ravenous

238

HATFIELD,

SCHOLZ,

Fig. 5. Small tal common bile cholangiography tienk.

cholangiography is invaluable for documenting the degree of duct displacement (Fig. 6A), thereby reducing the chances of inadvertent injury at operation. Angiography, which may be useful in distinguishing the thin uniform wall of a nonparasitic cyst from the thicker, more vascular rim of an echinococcal cyst, is usually unnecessary.

Fig. 6. Large cholangiogram.

solitary (6) Liver

liver cyst scan.

displacing

the

intrahepatic

duct in

AND

WISE

?iculum of the dis(arrows) on T-t ube two different pa-

Cystadenoma Although multicystic cystadenoma and papillary cystadenoma are rare, probably representing true neoplasms arising from the bile ducts rather than congenital lesions, they must be considered in the differential diagnosis of solitary cystic lesions of

and

extrahepatic

ducts

(arrows)

to the

left.

(A)

Intravenous

CONGENITAL

DISEASES

OF

THE

GALLBLADDER

AND

BILE

DUCTS

239

Fig. 7. Large liver cyst filling the entire right midabdomen. (A) Ultrasound scan and (B) computerized tomogram. A homogenous fluid-filled cyst is indicated (arrows). Unproved case.

the liver. At least 30 multicystic cystadenomas have been reported. They range in diameter from 1.5 to 25 cm.% The papillary cystadenoma is even less common.7,23~37 The multicystic adenoma is not distinguishable radiographically from solitary congenital liver cyst. The appearance of the injected papillary cystadenoma is pathognomonic because of the unique frond-like inner lining of the cyst (Fig. 8). Malignant degeneration within a papillary cystadenoma has been observed on two occasions.W Polycystic Disease of the Liver Polycystic disease of the liver is noteworthy primarily for its lack of clinical significance. Even though replacement of the hepatic parenchyma by cysts of varying size may be extensive, liver function rarely if ever deteriorates significantly. The

great majority of polycystic livers are 1101 detected before autopsy.*l Although polycystic disease may be confined to the liver, it is more commonly associated with polycystic disease of the kidneys and other organs, such as the pancreas and spleen. Recognition ot liver cysts may be important in distinguishing polycystic disease of the kidneys from multiple simple renal cysts, which are not associated with hepatic cystic disease. Liver cysts can be documented during the total-body opacification phase of infusion nephrotomography and by 99mT~sulfur colloid liver scanning.21 NONCYSTIC DISEASES BILE DUCTS

OF THE

Ductal Ectasia Two forms of ductal ectasia have been recognized. The simpler and rarer of the two. Caroli

HATFIELD,

240

Fig. 8. papiilary

Papillary cystadenoma inner lin/ng if the cyst.

of the

liver.

Tubocystogram

disease, was first described in 1958.6 It is usually not diagnosed until adulthood, although the patient may have experienced repeated bouts of fever and pain beginning in childhood. Intrahepatic calculi may develop, but jaundice is rare. Microscopically, Caroli disease is characterized by a lack of periportal fibrosis and inflammatory reaction. The second form of ductal ectasia, first described by Grumbach et al. in 1954,20 is more common and has a graver prognosis than Caroli disease. It is properly referred to as congenital hepatic fibrosis with ductal ectasia6 and not as Caroli disease. Usually cirrhosis is evident from birth and the patient dies at a young age from complications of portal hypertension. Survival into the third decade is the exception. Although their clinical course differs, the two forms of ductal ectasia have much in common. Both occur more frequently in men, both may have a familial distribution, both occur in association with medullary sponge kidneys in approximately 80% of instances, and both are indistinguishable radiographically and pathologically except for the periportal fibrosis. These similarities suggest that they represent two extremes of a single disease process. The pathogenesis of ductal ectasia has not been established. The suggestion by Glenn and McSherry r’ that the primary defect is hypoplasia or aplasia of the fibromuscular components of the

in

AP

and

lateral

projections

SCHOLZ,

demonstrates

AND

the

WISE

frond-like

submucosal and subserosal layers of the duct wall is not unreasonable. The diagnosis can be suggested by intravenous cholangiography with tomography if sufficient contrast material accumulates within the dilated duct segments. 33 More often, operati ve or T-tube cholangiography is necessary to demonstrate the characteristic “lollipop tree” appearance of the dilated ducts and to establish the diagnosis with certainty. Intravenous pyelography is useful to demonstrate the presence of medullary sponge kidneys, and a 99mTc-sulfur colloid liver scan will help exclude portal hypertension. Figure 9 illustrates a unique example of minimal segmental dilatation of the intrahepatic ducts, probably representing a forme fruste of ductal ectasia. Except for recurrent episodes of right upper quadrant pain, this patient was asymptomatic. Surgical exploration demonstrated no other abnormalities. The liver biopsy revealed only mild periportal fibrosis. Biliary A tresia Biliary atresia is included in a discussion of congenital diseases of the bile ducts since it was for some time believed to develop in utero. The consensus today is that biliary atresia is not a congenital defect but develops postpartum. It is probably “an acute or subacute, and subsequently, chronic inflammatory process which involves the

CONGENITAL

DISEASES

OF

THE

Fig. 9. Diverticula-like sacculations intrahepatic ducts, probably representing ant of ductal ectasia. T-tube cholangiogram.

GALLBLADDER

of the a vari-

AND

BILE

DUCTS

241

242

HATFIELD,

extrahepatic ducts and surrounding tissues(and possibly also the intrahepatic ducts) with an obliteration of the lumen which is often segmental and irregular in distribution.“24 In fact, biliary atresia and neonatal hepatitis may well represent opposite extremes of the samedisease.According to Bill et al.,4 biliary atresiais basically a hepatitis with a component of sclerosingcholangitis of the extrahepatic ducts. Thus, clinical, radiographic, and pathologic distinction between neonatal hepatitis and biliary atresia is difficult, if not impossible.To those seekinga more detailed discussion of this topic, a number of references are avai]ab]e,‘,8,9,11,23-25

CongenitalStrictures Congenital strictures of the bile ducts constitute only a small fraction of the total number of biliary strictures; the great majority result from prior surgical trauma.23 Only 0.5% of the 958 biliary strictures treated at the Lahey Clinic were congenital in origin.@v4’ Radiographically, strictures are best demonstrated by operative cholangiography. In the one example of congenital stricture that we have encountered in recent years, a short segment of common duct showed a uniform narrowing (Fig. IO). Proximal dilatation was absent since the patient had had a bypass procedure (cholecystojejunostomy) at an early age. When the anastomosisbecamepartially obstructed, a secondoperation wasnecessaryto excise the strictured segment.

SCHOLZ,

AND

WISE

GALLBLADDER

The majority of clinically significant congenital variations in the gallbladder were discussedin our previous article in Part I of this Seminar.22Except for these anatomic variants, choristomais the only noteworthy congenital disorder affecting the gallbladder. Examples of ectopic pancreas,gastric and intestinal glands, and prostatic and hepatic tissue within the gallbladderhave been reported sporadically.’ Ectopic pancreatic tissue is a common finding. Elfving I4 found aberrant pancreatic tissue,usually in the stomach or small bowel, in almost 2% of autopsies. Ectopic pancreas in the gallbladder is rare, occurring only once in the 2 12 casesof aberrant pancreasreported from the Mayo Clinic.13 To the best of our knowledge this oddity hasbeen demonstrated radiographically on only one occasion.35 The small nodule was in no way distinguishable radiographically from other fixed gallbladder defects, such as cholesterol polyp, adenoma,and embeddedcalculus. Aberrant gastric tissuein the gallbladder containing chief and parietal cellshasbeenidentified in at least seven patients.’ The casereported by Brunton’ is of interest in that the choristoma was somewhatpolypoid and larger (1 X 2 cm) than the usual benign fixed defect, closely resembling carcinoma of the gallbladder. ACKNOWLEDGMENT With the exception of Figs. 4 and 7, all illustrations are reproduced by permission of Williams & Wilkins.s

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sia: Comments on the frequency of potentially operable cases. J Pediatr 69:21-29, 1966 9. Danks DM, Clarke AM, Jones PG, et al: Extrahepatic biliary atresia. Further comments on potentially operable cases. J Pediatr 3:584-592, 1968 10. Dardik H, Glotzer P, Silver C: Congenital hepatic cyst causing jaundice: Report of a case and analogies with respiratory malformations. Ann Surg 159:585-592, 1964 11. DeLorimier AA: Surgical management of neonatal jaundice. N Engl J Med 288:1284-1286, 1973 12. Desser PL, Smith S: Nonparasitic liver cysts in children. J Pediatr 49:297-305, 1956 13. Dolan RV, ReMine WH, Dockerty MB: The fate of heterotopic pancreatic tissue. Arch Surg 109:762-765, 1974 14. Elfving G: Heterotopic pancreatic tissue in the gallbladder wall. Acta Chir Stand 118:32-36, 1959 15. Fonkalsrud EW, Boles ET Jr: Choledochal cysts

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DISEASES

OF

TtiE

GALLBLADDER

in infancy and childhood. Surg Gynecol Obstet 121:733742, 1965 16. Gillis DA, Sergeant CK: Prolonged biliary obstruction and massive gastrointestinal bleeding secondary to choledochal cyst. Surgery 52:391-393, 1962 17. Glenn I:, McSherry CK: Congenital segmental cystic dilatation of the biliary ductal system. Ann Surg 177: 705-713.1973 18. Criscom NT: The roentgenology of neonatal abdominal masses. Am J Roentgen01 93:447-463, 1965 19. Grove WJ: Ref:ognition and treatment of congenital choledochal cysts. Arch Surg 75:443-449, 1957 20. Grumbach R, Bourillon J, Auvert JP: La maladie fibrokystique du foic avec hypertension portale chez l’ent‘ant. Sem Hop Paris: Archives d’Anatomie Pathologique, A. 74:30, 1954. Quoted by Caroli J (Ref. 6) 21. Hatfield PM, Pfister RC: Splenicand hepatic evaluation during infusion nephrotomography; a potential source of considerable diagnostic information. Am J Roentgen01 119:687-691. 1973 22. Hatfield PM, Wise RR: Anatomic variation in the gallbladder and bile ducts. Semin Roentgen01 11 : 157164, 1976 23. Hatfield PM, Wise RE: Radiology of the Gallbladder and Bile Ducts. Baltimore, Williams & Wilkins, 1976 24. Ilays DM: Biliary atresia: The current state of confusion. Surg Clin North Am 53:1257-1274, 1973 25. Hays DM, Woolley MM, Snyder WH Jr, et al: Diagnosis of biliary atresia: Relative accuracy of percuopen liver biopsy, and operative tancous liver biopsy, cholanpiography. J Pediatr 71:598-607, 1967 26. Henson SW Jr, Gray HK, Dockerty MB: Benign rumors of the liver; solitary cysts. Surg Gynecol Obstet 103:607-612. 1956 27. Hoffer PB, Petasnick JP: Choledochal cysts demonstrated by oral cholqxystopraphy. Radiology 93:871-872, 1969 28. Hogarth J, Laird RC: Congenital cystic malformation of the bile ducts; report of a case and review of related literature. Can Med Assoc J 95:57-61, 1966 29. Hudson EK: Obstructive hepatic cyst. Am J Gastroenterol

jaundice from solitary 39: 161-164, 1963

30. Jackson BT, Saunders P: Perforated choledochus cyst. Br J Surg 58:38-42, 1971 31. Kanoui 1’: Fnorme kyste du CholCdoque mis en

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&idence par l’angiocholagraphie intraveineusc. Arch Ma1 App Digest 44:545-548, 1955 32. Liebner EJ: Roentgenographic study of’ congenital choledochal cysts, pre- and postoperative analysis of five cases. Am J Roentgen01 80:950-960, 1958 33. Mall JC, Ghahremani GG, Boyer JL: Caroli’s disease associated with congenital hepatic fibrosis and renal tubular ectasia. A case report. Gagtrocntcrology 66: 1029-1035. 1974 34. Marsh JL. Dahms B, Longmirc WP Jr: Cystadenoma and cystadenocarcinoma of the biliary system. Arch Surg 109:4143,1974 35. Martinez LO, Gregg M: Aberrant pancreas in the gallbladder. J Can Assoc Radio1 24:234-235. 1973 36. McKirdie M: Choledochal cyst: ;I ca\c report. West J Surg 64:598-600, 1956 37. Moore SW, McElwee RS, Romiti C: Benign tumors of the biliary tract. JAMA 150:999-1002. 1952 38. Munroe HS Jr: Solitary nonparasitic cyst\ of the liver. Ann Surg 116:751-762, 1942 39. Paulino I:: Dilitacao con&its do colcdoco. Rev Brasil Cirurg 3 1:402-404, 1956 40. Richmond HG: Carcinoma arismg m congenital cysts of the liver. J Pathol 72:681-683, 1956 41. Scholz l:J. Carrera GF. Larsen CR: The cholcdochocele; correlation of radiological. clinical, and pathological tindings. Radiology 118:25-28, 1976 42. Silberman EL, Glaessner TS: Roentgen features ot congenital cystic dilatation of the common bile duct: A report of two cases. Radiology 82~470-475, 1964 43. Tsardakas EN, Robnett AH: Congeniral cystic dilatation of the common bile duct; report of 3 caxs. analysis of 57 casts, and review of literature. .Arch Surg 72:311327. 1956 44. Warren KW, McDonald WM: l:acts and fiction rcgarding strictures of the cxtrahepatic bile duct>. 4nn Sure 159:996-lOlO, 45. Warren tiW, Polk RC: Benign cysts of the liver and biliary tract. Surg Clin North Am 38:707-728, 1958 46. Warren KW, Kune GA, Hardy KJ: Biliary duct cysts. Surp Clin North Am 48:567-577, 1968 47. Warren KW, Mountain JC. Middell Al: Management of strictures of the biliary tract. Surp Clin North Am 5 I : 711-731,197l 48. Williams LE, Fisher JH, Courtney RA, et al: Preoperative diagnosis of choledochal cyst by hepatoscintography. N I-ngl J Med 283:85-86. 1970