ScintigraphicFeaturesof Choledochal

Ernest Camponovo, James L. Buck, and Walter E. Drane Department ofRadio/ogy, Nationa/ Naval Medical Center, Bethesda, Mary/and; Department ofRadio/ogic Pathology, Armed Forces Institute ofPatho/ogy, Washington, DC; Department ofRadio/ogy and Nuclear Medicine, Uniformed Services University ofthe Health Sciences, Bethesda,Mary/and

Thescintigraphicappearancesof 12 surgicallyprovencasesofcholedochalcystwere retrospectivelyreviewed.In sevenof 12 cases,radionuclideaccumulatedin the choledochal cyst(i.e.,thedilatedcommonbileduct)inlessthan1 hr.Inthreeadditionalcases,delayed accumulation(1—24hr)withinthecystwasseen.Intwoofthe12 cases,noductalactivity appeared and the diagnosis of ChOIedOChaIcyst could not be made, although in one of these two casesdelayedimageswerenot obtained.Otherfrequentfindingsincludeddelayedor nonvisualizationofthegallbladder(11 of 12) andtheappearanceof prominentintrahepatic ducts(fiveof 12).Weconcludethat hepatobiliaryscintigraphyis a noninvasivetest usefulin the diagnosisof chOledOchalcyst.

J Nucl Med 30:622-628, 1989

holedochal cyst is an uncommon disease, usually MATERIALS AND METhODS felt to be congenital in origin, represented by extrahe We reviewedthe files of the Departmentof Radiologic patic dilatation involving principally the Pathologyat the Armed Forces Institute of Pathology and common duct. Until the last decade, the diagnosis found 12 cases of pathologicallyproven choledochal cyst that was only occasionally made preoperatively as a result had been evaluated preoperatively with technetium-99m ofthe variabilityofthe presentationsofaffected patients (99mTc)hepatobiliary scans. The cases dated from 1980 to (1—10).The failure to consider the diagnosis preopera 1987 and were collected from 12 different institutions. All tively has been associated with poor surgicalresultsand scans were performed using technetium-99m (@mTc)imino increasedmorbidityand mortality(11). diacetic acid derivatives, including N-(2,6-diethylphenylcar The gold standard for choledochal cyst diagnosis is bamoylmethyl) iminodiacetic acid (DEIDA), N-(2,6-di direct cholangiography, but such tests are, unfortu methylphenycarbamoylmethyl)iminodiaceticacid(HIDA), p nately, invasive. The advent of computed tomography isopropylacetanilido iminodiacetic acid (PIPIDA), and di isopropyl iminodiacetic acid (DISIDA). From the clinical (CT) and ultrasound has dramatically increased the summary available in each case, the patient's age, sex, and ability of radiologists to detect the cystic abnormality degreeofjaundice, if present,was recorded.The scintigrams by noninvasive means. However, unless the lesion can were reviewedand the time of appearance,if any, of activity be proven with these techniques to be partofthe biliary within the choledochal cyst, the gallbladderand the gut was tract, a definitive diagnosis of choledochal cyst cannot recorded. In one case, images were obtained up to only 1 hr be made and additional tests are still required (3,12— afterinjection despite the fact that the bile ducts were not yet 20). In this situation, biliary scintigraphy will usually visualized. Follow-up images obtained up to 24 hr afterinjec provide a definitive and noninvasive diagnosis. tion were obtained in all other cases of delayed or nonvisual In this review the scintigraphic appearances of 12 ization of the ducts. Flow and/or blood-pool images, when cases of choledochal cyst are described and compared available, were analyzed for the presence of a photopenic region corresponding to the choledochal cyst. The scinti to those cases that have been previously reported. graphic appearance of intrahepatic ductal prominence was also recorded, if present. Additional images including ultra sonographs, cholangiographs, computed tomographs, and gross photographs of the surgical specimen were reviewed ReceivedJune9, 1988;revisionacceptedNov. 22, 1988. when available,but not specificallyanalyzed in this study. For reprintscontact:James L. Buck, MD, EditorialOffice, Armed Forces Institute of Pathology, Room 4018, Washington, RESULTS DC20306-6000. * Present address: Department of Radiology, University of Our resultsare tabulated in Table 1. Note that 11 of FloridaCollegeof Medicine,Gainesville,FL 32610. 12 patients were female with an average age of 7.1 yr.

622 Camponovo,Buck,andDrane TheJournalof NuclearMedicine TABLE I clinicalandScintigraphicFindingsin I 2 Casesof CholedochalCyst Earliestvisualization CaseDilatedGutducts1 no. Agents Ag& Sex Jaundicet Cyst Gallbladder. hrNo2HIDA 12 M No(0.8) <1 hr <1 hr<1 @ hrYes3DEIDA 16 F Yes Nonvis.©1 hr Nonvis.4 1 hrNonvis.4 ©1 hrNo4PIPIDA 1 F No(0.9) 24 hr 24 hr24 hrYes5HIDA 14 F Yes(10.) 4 hr <24 hr<24 @ hrNo6HIDA 0.1 F Yes(7.7) 24hr(minimal) Nonvis. 24hrNonvis. ©24 @ hrNo7DISIDA 21 F Yes <1 hr Nonvis. 1 hr<1 2.5Yeshrhr8HIDA 2 F No (0.8) <1 hr Nonvis.*@ 2.5Nonvis.t @

@ hrYes9PIPIDA 2.3 F No(0.2) <1 hr NorMs. 24 hr24 @ hrNo10DISIDA 2.2 F No <1 hr Nonvis.4 1 hr<1 @ hrNo11DISIDA 0.1 F Yes(11.7) Nonvis. 24hr Nonvis. 24hrNonvis. 24 @ hrYes12DISIDA 12 F No <1 hr Nonvis.4 1 hr<1 hrYes. DISIDA 2.3 F No <1 hr Nonvis.t@ 1hr<1

years.tThe age is expressed in

parentheses.4Wt@ known, the total levels expressed as mg/i 00 ml is induded in Indicates that additional delayed films up to 24 hr were not Obtained.

Clinicaljaundicewaspresentat the time of the study and represents one of the conditions included in the in five of 12 cases, and had been present previously in spectrum of hepatobiliary fibrocystic disease. Other as one additional case (Case 1). sociatedconditionsincludeCarolis' disease,congenital Seven of our 12 cases demonstrated visualization of hepatic fibrosis, infantile polycystic , simple the dilated common within 1hrafterinjection hepatic cyst(s), and biiary atresia (1—3,5—8,21).The (Figs. 1 and 2). One case showed cyst activity between most widely accepted classification system of choledo 1 and 12 hr, and two more between 12 and 24 hr (Fig. chal cyst is that ofAlonso-Lej, with type I, representing 3). There was one case of total nonvisualization of the cystic dilatation of the main portion of the common cyst at 24 hr (Fig. 4). In the final case, no ductal activity bile duct, being by farthe most common ( 7). All of our was visible at 1 hr, but images beyond 1 hr were not cases were type I. Neither divertic available. Whether in this case subsequent delayed films ulum (type II choledochal cyst) nor choledochocele would have demonstrated extrahepatic biiary activity (type III choledochal cyst) was studied in this series. cannot be determined. The etiology of choledochal cyst is debated, but there Normal visualization of the gallbladder was uncom are two predominant theories. One of these holds that mon. Only one case demonstrated gallbladdervisuali there is prenatal biliary obstruction associated with a zation in less than 1 hr (Fig. 2B). Two cases visualized congenital weakness of the common bile duct produc at greaterthan 3 hr, and nine cases did not demonstrate ing dilatation (6). The other maintains that there is an gallbladder visualization at all (Fig. lC), although in six anomalous development ofthe distalcommon bile duct of these cases 24-hr delayed films were not obtained. and such that there is a long common The time of visualization of gut activity also varied. channel allowing reflux of pancreatic juice into the Five cases showed early (<1 hr) gut activity (Fig. 2B), common bile duct resultingin weakening and dilatation three demonstrated activity at >1 hr, and in four cases ofthe duct wall (22). no gut activity was visible (Fig. lC), although in two of Choledochal cyst is much more commonly seen in these cases 24-hr delayed films were not obtained. females and usually presents in the firsttwo decades of In four cases, flow studies or blood-pool images were life (1,2,4,8,10,23—25), though patients may present in available for review and a photopenic defect corre the third and fourth decade (26). It has also been sponding to the choledochal cyst was demonstrated in diagnosed prenatally (27). The age and sex of the each case. In six instances, intrahepatic duct promi patientsin ourseriesmatchesthoseofpreviousstudies. nence was visible on the scintigrams(Fig. 1B). Thereis a predilection forOrientalswith approximately one-third ofthe ‘@-l,000reported cases having occurred in Japan, China, or Korea (1,6,8,10,15,28,29). How DISCUSSION ever, to our knowledge, none of our patients were Oriental. Choledochal cyst is an uncommon anomaly defined The presenting signs and symptoms can include al by congenital dilatation ofthe extrahepaticbiliarytract most any combination of biliary obstruction, gastroin

Volume30 •Number5 •May1989 623 @. @ A ..@.. B

L)Lr n ADD PWR = .-@ 50d :@ GAl =

C D

FIGURE 1 Case 8. Promptvisualizationof choledochalcyst withoutgallbladderor gut visualization.A: Blood-poolphase images reveala photopenicarea (arrow)belowthe livercorrespondingto the choledochalcyst. B:At 1 hr after injection,activity withinthecholedochalcyst(arrow)isclearlyvisible.Activitywithindilatedlefthepaticducts(arrowhead)isalsonoted. C: At 2.5 hr after injection,the amountof activitywithinthe choledochalcyst has increased,but no gallbladderor gut activity has appeared. This nonvisualization persisted up to 24 hr. D: A transverse sonogram through the ports hepatis reveals the superior extent of the choledochal cyst (arrowhead) communicating with dilated intrahepatic ducts (arrows).

testinal obstruction, , , pain, cyst were limited to plain film findings ofan abdominal cholangitis, or bile peritonitis. The classic clinical triad mass, indirect signs of abdominal mass on UGI, or ofjaundice, abdominal mass and pain is seen in only contrast delineation of the cyst by oral, intravenous, or 20—30%of cases. This variable presentation of chole percutaneous cholangiography (4,8,9,12,33). Oral and dochal cyst along with its relative rarity, has made intravenous cholangiography, though occasionally di preoperative consideration of this condition uncom agnostic in patients with no or minimal jaundice, are mon (1—4,8,9,12,13,15,19,23—26,28,30—31).There is nondiagnostic with bilirubin levels >3 mg %, and intra evidence that failureto consider choledochal cyst in the venous cholangiographyis associated with a significant preoperative assessment can increase surgical morbidity risk of severe allergic reaction. Percutaneous cholangi and mortality (11). Additionally, failureto diagnose or ography is sensitive and specific, but invasive and ex adequately treat the condition has been associated pensive. with an increasedincidence ofcomplications, including More recently, the diagnosis of choledochal cyst has cholangitis, choledocholithiasis , bile duct been made with CT and ultrasound. These methods are perforation and, especially, typicallyusedwhenbiliaryobstructionor an abdominal (2,4,8,13,25,32). mass is suspected, and will almost always identify the In the past, methods for the diagnosis of choledochal cystic mass (12,14—17). However, although a specific

624 Camponovo,Buck,andDrane TheJournalofNudearMedicine A B

FIGURE 2 Case1. Promptvisualizationof cho ledochalcyst with prompt gallbladder and gut activityvisualization.A: Ac tivity has accumulatedwithin the choledochalcyst (arrow) 20 mm after injection.B:Sixtyminutesafterinjec tion the choledochal cyst remains well visualized.Gallbladder(arrow) and gut activityare alsoseen. C: An intraoperative cholangiogram dem onstratingthe choledochalcyst gall bladder and doudenum correlates with the 60-mm scintigraphic image. Theanomalouscommonchannel(ar row)formed by the common bile duct and pancreaticduct is seen. diagnosis of choledochal cyst may be made by com In the past, the “classic―scintigraphic appearance puted tomography (CT) or ultrasound in some cases of choledochal cyst was that of an early photon defi (20,23,31), in other casestheseanatomically oriented cient area that “filled-in―on late (>2 hr) images tests may be unable to differentiate choledochal (12,25,28,30—32).This appearanceof late cyst activity from other cystic lesions such as simple hepatic cysts, wasseenin onlythreeofour 12cases(Fig.1C),although pancreaticpseudocysts,duodenal duplication cysts, and had delayed images been obtained in Case 2, visualiza hepatic arteryaneurysms, all ofwhich may presentwith tion might have been seen. The pattern seen more symptoms of pain, jaundice, and abdominal mass. frequently in our experience, occurring in seven of our Since 1970, nuclear medicine methods have been 12 cases, was that ofearly appearance ofactivity within used to diagnose choledochal cyst, first with iodine-13l grossly dilated extrahepatic ducts (Figs. 3B, 2A). Why Rose Bengal, and later with [@“Tc]IDAcompounds this pattern was found much more frequently in our (12,13,15,1 7,25,28—32).The imino-diacetic acid family series is not clear. Regardless,the prompt visualization of 99mTcligands provide good imaging statistics and of the extrahepatic ducts reflects the fact that the ob allow functional assessment of the biliary tract even struction that may have contributed to the formation with bilirubin levels >20 mg % (34—37).These radio of the choledochal cyst in utero does not necessarily pharmaceuticals allow a definitive diagnosis of chole persist. Note that there was not a close correlation dochal cyst, and frequently define other anatomic or between the time of visualization of the extrahepatic physiologic abnormalities of the hepatobiliarysystem. activity and the presence ofjaundice. One of the four

Volume30 •Number5 •May I 989 625 @. @*

,,

A B

4

dl C

FIGURE 3 Case 4. Delayedvisualizationof choledochalcyst. A: Initially,there is a suggestionof photopenicmass(arrows)in the portahepatis,butnoevidenceofextrahepaticactivity.B:A subsequentimage,Obtained4 hrafterinjection,demonstrates activitywithinthecholedochalcyst(arrows).Gutandgallbladderactivityarenotyetvisible.C:At 24hrafterinjection, both gut andgallbladderactivity(arrow)are visible.D: A percutaneoustranshepaticcholangiogramdemonstratesthe choledochalcyst (asterisk)andthe gallbladder(arrow).Contrastwithinthe renalcollectingsystemand uretersis also seen. patients with jaundice demonstrated prompt extrahe nuclide because of persistent or recurrent high grade patic ductal visualization while delayed visualization biiary obstruction. In this situation, a definitive diag was seen in one of the five patients without jaundice. nosis of choledochal cyst cannot be made scintigraphi In the final pattern,the cyst neveraccumulates radio cally. Unfortunately, this pattern seems particularly

626 Camponovo,Buck,andDrane TheJournalofNuclearMedicine A

FIGURE 4 Case 10. Persistent nonvisualization of choledochal cyst. A: Twenty-four hours after injection, no bile duct activity is seen.Thediagnosisof choledochalcystcannotbeestablished,althoughtherelativelyphotopenicareain theporta hepatis(arrows)suggeststhe diagnosis.B: A longitudinallyorientedsonogramof the portahepatisidentifiesthe superioraspectof the choledochalcyst communicatingwith the remainderof the biliarytract (arrows).The separate cysticstructureanteriorto the choledochalcyst is the gallbladder. likely to occur in infants who present with jaundice. ductal dilatation (i.e., Carolis' disease) and congenital Our case of complete nonvisualization of the choledo extrahepaticductal dilatation (i.e. choledochal cyst) has chal cyst at 24 hr occurred in a jaundiced infant 6 wk been previously described (12,34). Han et al. described of age. In a second case, also involving a 6-wk-old girl, this same scintigraphic appearance in two cases prey only minimal activity was seen in the choledochal cyst ously. However, in the last of our cases, the prominent at 24 hr. In the former case, phenobarbital was not ducts wereactuallyproven to be normal by sonography, administered prior to the scintigram and it is possible emphasizing scintigraphy's lack of specificity in assess that extrahepatic activity would have been seen had the ing mild to moderate changes in ductal diameter. hepatocellular function been improved with this mcdi We believe that hepatobiliary scintigraphy with cation. Nevertheless, seems likely that in infants with [99mTc]IDAagents provides important information in choledochal cyst an incorrect diagnosis ofbiliary atresia choledochal cyst in the majority of cases and should be can be made ifthe interpreteris unawareofthe presence utilized whenever CT and sonography are unable to of a cystic or avascularmass in the porta hepatis based establish a definitive diagnosis. In our experience, on ultrasound or blood-pool phase scintigraphy. prompt appearance of radionucide within the dilated In our series, as in a previously reported series (28), extrahepatic bile duct representing the choledochal cyst delayed or nonvisualization of the gallbladder was very is the most common scintigraphic appearance. In other common. Cholecystectomy is frequently performed at cases, delayed images may be required to demonstrate the time ofcholedochal cystectomy. Ofthe six patients this accumulation of activity and establish the diagno in our series with delayed visualization of the gallblad sis. In some cases, however, particularly in infants, no der, three were found to have chronic cholecystitis, and extrahepaticactivity will be seen even on 24-hr delayed three were found to have normal galibladders. There images. In these cases either ultrasound or flow/blood fore, although in some cases cholecystitis may have pool imaging is requiredto distinguish choledochal cyst contributed to the delayed gallbladdervisualization, in from . others this may have simply represented an alteration in the pattern of biliary flow due to the reservoireffect of the choledochal cyst. The same phenomenon may also explain the delayed gut visualization in two patients without jaundice and with prompt visualization of the ACKNOWLEDGMENTS choledochal cyst. The authors thank the many radiologistsand pathologists In five of our cases the intrahepatic ducts appeared who have contributed material to our department and Ms. prominent and in four ofthese cases dilated ducts were Bonnie Yelverton who assisted in preparationof the manu confirmed by ultrasound examination. The lack of script. jaundice in one of these cases suggests that the intra The opinions or assertions contained herein are the private hepatic dilatation is part ofthe congenital anomaly and views of the authorsand are not to be construed as official or not just a manifestation ofextrahepatic biiary obstruc as reflectingthe views ofThe Department ofthe Army, Navy, tion. The association between congenital intrahepatic or the Department of Defense.

Volume30 •Number5 •May1989 627 REFERENCES with bile duct dilatation: sonography and 99mTcIDA cholescintigraphy. Am J Roentgeno/ 1981; 136:1075— 1079. 1. Chen WJ, Chang CH, Hung WT. Congenital chole 20. Buck JL, Ros PR, Moser RP Jr. Specific sonographic dochal cyst: with observations on rupture of the cysts diagnosis of choledochal cyst: sonographic-cholangio and intrahepatic ductal dilatation. J Pediatr Surg graphic correlation. Radio/ogy 1987; 165:285. 1973;8:529—538. 21. Matsumoto Y, Uchida K, Nakase A, Honjo I. Clini 2. BarlowB,Tabor E, Blanc WA, et al. Choledochal cyst: copathologic classification of congenital cystic dilata a reviewof 19cases.J Pediatr 1976;89:934—940. tion of the common bile duct. Am J Surg 1977; 3. Markle BM, Potter BM, Majd M. Thejaundiced infant 134:569—574. and child. Semin Ultrasound 1980; 1:123—133. 22. Babbitt DP, Starshak RJ, Clemett AR. Choledochal 4. Bass EM, Cremin BJ. Choledochal cysts: a clinical and cyst: a concept ofetiology. Am JRoentgeno/Rad Ther radiological evaluation of 21 cases. Pediatr Radiol Nuc/Med 1973;119:57. 1976;5:81—85. 23. Reuter K, Raptopoulos VS, Cantelmo N, et al. Diag 5. Longmire WP Jr, Mandiola SA,Gordon HE. Congen nosis of a choledochal cyst by ultrasound. Radiology ital cystic disease of the liver and biliary system. Ann 1980; 136:437—438. Surg1971;174:711—726. 24. Sherman P, Kolster E, Davies C, et al. Choledochal 6. Miyano T, Suruga K, Chen SC. A clinicopathologic cysts: heterogeneity of clinical presentation. J Pediatr study of choledochal cyst. WorldJ Surg 1980; 4:231— Gastroentero/Nutr1986;5:867—872. 238. 25. Park CH, Garafola JH, O'Hara AE. Preoperative di 7. Alonso-Lej F, Revers WB, Pessagno DJ. Congenital agnosis of asymptomatic choledochal cyst by rose choledochal cyst with a report oftwo, and an analysis bengal liver scan. J Nuc/ Med 1973; 15:310—311. of94cases. IntAbstSurg 1959; 108:1. 26. Oldham KT, Hart Mi, White iT. Choledochal cysts 8. Lee 55, Mm PC, Kim GS, Hong PW. Choledochal presenting in late childhood and adulthood. Am J cyst. Arch Surg 1969; 99:19—28. Surg1981;141:568—571. 9. Rosenfield N, Griscom NT. Choledochal cysts: roent 27. Wiedman MA, Tan A, Martinez CJ. Fetal sonography genographic techniques. Radiology 1975; 114:113— and neonatal scintigraphy of a choledochal cyst. J 119. Nuc/Med 1985; 26:893—896. 10. Todani T, Watanabe Y, Narusue M, et al. Congenital 28. Huang MJ, Liaw TF. Intravenous cholescintigraphy bile duct cysts. Classification, operative procedures, using Tc-99m-Labeled agents in the diagnosis of cho and reviewofthirty-seven cases including cancer aris ledochalcyst.JNuc/Med 1982;23:113—116. ing from choledochal cyst. Am JSurg 1977; 134:263— 29. Oshiumi Y, Nakayama C, Morita K, et al. Serial 269. scintigraphyofcholedochal cystsusing ‘311-rosebengal 11. Tsardakeas E, Robnett AH. Congenital cystic dilata and ‘31I-bromsulphalein.Am J Roentgeno/ 1977; tion ofthe common bile duct. Arch Surg 1956; 72:311. 128:769—771. 12. Piyachon C, Poshyachinda M, Dhitavat V. Hepato 30. Williams LE, Fisher JH, Courtney RA, Darling DB. scintigraphy, arteriography, and ultrasonography in Preoperativediagnosisof choledochalcyst by hepato preoperative diagnosis of choledochal cyst. Am J scintigraphy. NEJM 1970; 283:85—86. Roentgeno/1976;127:520—523. 31. Nakata H, Nobe T, Takahashi M, et al. Case report. 13. Fleischer AC, Born ML, Kirchner SG, et al. Comple Choledochalcyst.JCAT 1981;5:99—101. mentary use of 99mTcHIDA and upper abdominal 32. Paramsothy M, Somasundram K. Technetium 99m- sonography in diagnosing a choledochal cyst. South diethyl-IDA hepatobiliaryscintigraphy in the pre MedJ 1980;73:1651—1653. operative diagnosis of choledochal cyst. Br J Radio/ 14. Brodey PA, Fisch AE, Fertig 5, Roberts GS. Case 1981;54:1104—1107. Report.Computedtomographyof choledochocele.J 33. Scholz FJ, Carrera GF, Larsen CR. The choledocho ComputAssist Tomogr 1984; 8:162—164. cele:correlation of radiological,clinical and patholog 15. Klein GM, Frost SS. A case report. Newer imaging icalfindings.Radio/ogy1976;118:25—28. modalities for the preoperative diagnosis of choledo 34. Sty JR, Glicklich M, Babbitt DP, Starshak RJ. Tech chal cyst. Am J Gastroentero/ 1981; 76:148—152. netium-99m biliary imaging in pediatric surgical prob 16. Pollack M, Shirkhoda A, Charnsangavej C. Case re lems. J Pediatr Surg 198 1; 16:686—690. port. Computed tomography of choledochocele. J 35. Pauwels 5, Steels M, Piret L, Beckers C. Clinical ComputAssist Tomogr 1985; 9:360—362. evaluation of Tc-99m-diethyl-IDA in hepatobiliary 17. Gates GF, Miller JH. Combined radionuclide and disorders. JNuc/Med 1978; 19:783—788. ultrasonic assessment of upper abdominal masses in 36. Majd M, Altman RP, Reba RC. Hepatobiliary scintig children. Am JRoentgeno/ 1977; 128:773—780. raphy with Tc-99m PIPIDA in infants and children. J 18. Kirks DR, Coleman RE, Filston HC, et al. An imaging Nuc/Med 1979; 20:680. approach to persistent neonataljaundice. Am JRoent 37. Rosenthall L, Shaffer EA, Lisbona R, Pare P. Diag genol1984;142:461—465. nosis of hepatobiliary disease by @“Tc-HIDAchole 19. Han BK, Babcock DS, Gelfand MH. Choledochal cyst scintigraphy. Radiology 1978; 126:467—474.

628 Camponovo,Buck,andDrane The Journalof NuclearMedicine