Gut 1993; 34: 853-856

CASE REPORTS

Non-communicating multiseptate gall bladder andcholedochal cyst: a case report and review ofpublications

C E L Tan, E R Howard, M Driver, I M Murray-Lyon

AbstractA 14 year old girl with multiseptate gail bladderand cystic dilatation of the biliary tree ispresented. This is the 20th published case

report of patients with multiseptate gail

bladder and only the second to be associatedwith a choledochal cyst. The cystic spaces ofthe gail bladder did not communicate with theneck ofthe gall bladder or the rest of the biliarytree, and this unusual feature has not been pre-

viously described. A multiseptate gall bladderwith a normal biliary tree commonly causes

symptoms suggestive ofcholecystitis, althoughgall stones are seldom present. Diagnosis isconfirmed by an oral cholecystogram or ultra-sound scan that may show the fine intraluminalseptae, and these features should be looked forin patients with biliary symptoms withoutbiliary calculi. Cholecystectomy is curative forthe isolated gall bladder anomaly but hepatico-jejunostomy may be necessary for an associ-ated choledochal cyst.(Gut 1993; 34: 853-856)

Department of SurgeryCE L TanE R Howard

Department ofHistopathology, King'sCollege Hospital,LondonM Driver

Department ofGastroenterology,Charing Cross Hospital,LondonI M Murray-LyonCorrespondence to:E R Howard, Department ofSurgery, King's CollegeHospital, Denmark Hill,London SE5.Accepted for publication22 September 1992

Multiseptate gall bladders are rare anomalies thatwere first described in 1963 by Simon andTandon. 'Eighteen further cases (Tables I and II)have now been reported. In one there was coexis-tent fusiform dilatation of the extrahepatic bileducts and a long common pancreaticobiliarychannel.2 We report a second case of a multi-septate gall bladder associated with cystic dilata-tion of the common bile duct. No communica-tion was found between the loculi of the gallbladder and the lumen of the rest of the biliarytract.

Case reportA 14 year old girl from Qatar presented with a

one year history of intermittent pyrexia, occur-

ring two to three times a month and oftenreaching up to 40°C. This was accompanied byrigors, nausea, and some generalised abdominaldiscomfort, but there was no vomiting, diar-rhoea, or jaundice.At presentation she was well nourished and

TABLE I Case reports ofmultiseptate gall bladder (adults)

Duration Gall bladderReports Age (y) Sex Symptoms ofsymptoms contents Surgery

Simon, Tandon (1963)' 32 F Upper abdominal pain--back 7 y Viscid bile CBigg (1964)' 38 M RHC pain--scapula 8 weeks NR CSachsse et al (1968)" 50 M Upper abdominal pain 2 y -Bhagavan, et al (1970)" 27 F RHC pain-epigastric 2 y Viscid bile CCroce (1973)' 45 F Epigastric pain 5 months Several stones CCodinach, et al (1975)' 28 F RHC pain--epigastric 11 y One stone CShaw, et al (1975)'4 31 F RHC pain-shoulder 6 months NR CKonishi, etal (1975)" 51 F Epigastric pain 31 y NR CJena, et al (1977)" 28 F RHC pain-shoulder 5 months Green bile COkuda, etal (1979)'4 37 M RHC pain 22y Brown bile CAlawneh, et al (1981)9 44 F RHC pain-back 20 y Pea sized stones CToombs, et al (1982)"3 22 F RHC pain 2 days NR COlivia, etal (1985)' 24 F RHC pain 4 weeks NR CLev-Toaff, et al (1987)4 30 M Symptom free (pancreatitis) - -

23 F Symptom free - -Vasinrapee, et al (1990)' 24 M RHC pain NR NR NR

No patient had jaundice and all bile ducts were normal.RHC= right upper quadrant; N=normal; NR=not reported; C= cholecystectomy.

TABLE II Case reports ofmultiseptate gall bladder (children)

Duration Gall bladderReports Age (y) Sex Symptoms ofsymptoms contents Surgery

Haslam, et al (1966)" 15 F RHC pain-epigastric 4 y Normal bile CPery, et al (1985)' 8 F Recurrent jaundice, bile ducts 45 y NR C+CD

dilatedFremond, et al (1989)" 13 F RHC pain NR NR CPresent case 14 F Recurrent pyrexia, bile ducts 1 y Clear mucus C+HJ

dilated

CD=choledochoduodenostomy; HJ=hepaticojejunostomy. Other abbreviations as for Table I.

853

on February 23, 2021 by guest. P

rotected by copyright.http://gut.bm

j.com/

Gut: first published as 10.1136/gut.34.6.853 on 1 June 1993. D

ownloaded from

Tan, Howard, Driver, Murray-Lyon

B

B

afebrile, with no signs of chronic liver disease.Her haemoglobin concentration was 9-5 g/dl, andwhite cell and platelet counts were normal.Serum electrolytes were normal. Serum bilirubinconcentration was 4 ,umol/l, alkaline phosphataseactivity 217 IU/1, y glutamyl transpeptidaseactivity 226 IU/l (raised), aspartate transaminaseactivity 30 IU/1, albumin concentration 39 g/dl,and International Normalised Ratio 1-08.An ultrasound scan of the abdomen showed

cystic dilatation of the common bile duct withsome dilatation of the intrahepatic duct, andthese findings were confirmed by endoscopicretrograde cholangiopancreatography (Fig1(A)). Surgery was recommended for treatmentofthe choledochal cyst that was thought to be thecause of recurrent cholangitis.

FINDINGS AT OPERATIONThe body and fundus of the gall bladder were

divided into cystic spaces by multiple septa. Thecysts were distended with clear mucus (Fig 2),which showed a normal amylase concentration of8 IU/1. The neck of the gall bladder containedbile and did not communicate with the multi-septate body. An operative cholangiogram con-

firmed the findings of endoscopic retrogradecholangiopancreatography, and also showed thatthere was no opacification of the cystic portion ofthe gall bladder (Fig 1(B)). Bile aspirated fromthe common bile duct contained high amylaseactivity (800 IU/1). The pancreas and liver weremacroscopically normal. The multiseptate gallbladder was excised and the common bile ductwas transected and removed together with thecholedochal cyst. A hepaticojejunostomy was

performed with a 45 cm Roux en Y loop ofjejunum and a tru-cut biopsy of the liver was

taken. The patient made an uncomplicatedrecovery and was discharged from hospital on the

13th day after operation. She has remainedanicteric and well for one year after operation.

HISTOPATHOLOGYThe gall bladder showed an irregular lobularexternal appearance. Slicing showed small cysticspaces measuring up to 0 7 cm in diameter, someof which contained minute amounts of inspis-sated bile. Figure 2(A) shows the histologicalfindings.The cystically dilated bile duct measured 6 cm

in length and up to 2 cm in diameter. The wallwas composed of chronically inflamed col-lagenous fibrous tissue (Fig 2(B)). The liverbiopsy showed moderate expansion of the portaltracts together with some ductular proliferationand active chronic inflammation. Periductalfibrosis was present indicating ascending infec-tion.

DiscussionA review of the 20 cases described to date shows a

female preponderance (female:male= 3:1) and a

mean age of 29 (range 8-51) years. Four of thepatients were younger than 16 years of age. Rightupper quadrant pain suggestive of biliary colicwas the presenting complaint in 60%, commonlywith radiation to the epigastrium, right shoulder,or back. Duration of symptoms averaged 6-6years. Jaundice was absent in all of the cases withundilated bile ducts. Of the two patients withbiliary duct dilatation, the eight year old girlreported by Pery et al presented with recurrent

jaundice,2 and our patient had recurrent pyrexiabut remained anicteric.Two patients did not complain of biliary

symptoms and their gall bladder abnormalitieswere found during ultrasonography for pyelo-nephritis and pancreatitis.4 The patient who had

Figure 1: Endoscopicretrograde cholangiogram(A) and explanatorydiagram (B) in a 14 year oldgirl. The extrahepatic andintrahepatic ducts showgeneralised dilatation (<-).Only the neck ofthe gallbladder opacifies ( t ). Thepancreatic duct is not seen.The line A-B represents theplane of the histology sectionin Fig 2A.

854

on February 23, 2021 by guest. P

rotected by copyright.http://gut.bm

j.com/

Gut: first published as 10.1136/gut.34.6.853 on 1 June 1993. D

ownloaded from

Non-communicating multiseptate gall bladder and choledochal cyst: a case report and review ofpublications

: i* - * :.....

** . a .....:.

I

n

Figure 2: (A) Section through the body ofthe gall bladder showing cystic spaces. The wallsare composed ofcollagenous fibrous tissue with focal acute on chronic inflammation. Somecysts are lined by hyperplastic tall columnar epithelium (*), others by low cuboidal epithelium(*). Smooth muscle fibres and chronic inflammatory changes are present in the septa ( t,

haematoxylin and eosin. Originally x 7).(B) Sectionfrom the wall of the choledochal cyst shows the lining is predominantly ofcollagenous fibrous tissue without epithelium (o-). In one area ofa cleft lined by cuboidalepithelium is seen ( t ), beneath it are some smooth musclefibres. (T , haematoxylin and eosin:originally x 7). Inset: high power view ofthe cuboidal epithelium. The apparentmultilayering is due to oblique cutting (haematoxylin and eosin. Originally x 100).

ultrasound for pancreatitis eventually died fromsevere haemorrhagic pancreatitis attributed toalcohol. A non-inflammed multiseptate gallbladder was found on necropsy.

Oral cholecystography was the usual examina-tion in earlier cases, and showed thin linearradiolucent septa within the gall bladder, com-

municating cysts, and good contractility in sixpatients. Olivia et al reported the first case

diagnosed with ultrasonography,5 and Lev-Toaffet al described the sonographic features ofmultiple, fine, non-shadowing septations thatbridge the lumen of the gall bladder.4 A 'clover

leaf pattern has been described in scans of the'Tc diisopropylimino diacetic acid radio-nuclide.6Knight stated that 'the multiseptate gall

bladder is usually associated with multiple cal-culi."' Only three of the 20 cases of true multi-septate disease, however, showed stones7 andthese cases should not be confused with thecommoner findings of one to three gall bladdersepta that may be associated with stones in up to22% of cases.3 Gall bladders containing one tothree septa are not unusual, and were recognisedin 3-8% of 2437 oral cholecystograms and intra-venous cholangiograms.The external appearance of multiseptate gall

bladders varies from normal to bosselated. Theyfeel spongy and when cut open show a honey-comb of multiple cysts ranging from 2-16 mm indiameter. Small intercommunicating openings inthe septa may be found. Eleven of the 17 gallbladders that were removed showed mild tomoderate chronic cholecystitis. It is not easy toaccount for the symptoms in the condition.Acute inflammation has not been reported and acomplete absence of any chronic inflammatorychange was noted in six cases.4 15 Our patientdid show areas of acute on chronic inflammationand mucosal ulceration but these changes mayhave been caused by the episodes of cholangitisthat were secondary to the choledochal cyst.Our patient was unusual in that the multi-

septated fundus and body of the gall bladder hadlost communication with the neck of the gallbladder and the rest of the biliary tree. This mayhave been an acquired phenomenon caused bythe inflammation and mucosal proliferation inthe gall bladder. The multiseptated portion wasdistended with clear mucus containing only 8 U/Iof amylase, but the biliary duct amylase was 100times higher, which confirmed the absence ofcommunication between the bile duct and gallbladder. High biliary amylase concentrations incholedochal cysts are usually associated withcommon pancreaticobiliary channels althoughthis was not found in our patient.The gall bladder in early gestation is composed

of three cell layers, an inner endodermal epi-thelium and two outer mesodermal layers thatlater become the tunica muscularis and thesubserosa. When epithelial proliferation occursin the second month of fetal life, the small hollowgall bladder vesicle becomes a solid tube. Vacuo-lisation of the solid endoderm then follows, andintraepithelial clefts coalesce to form the lumenof the gall bladder.'6 It has been suggested thatincomplete vacuolisation of the solid gall bladderbud is responsible for the formation of gallbladder septa.' 13 This does not explain thepresence ofthe smooth muscle fibres found in thesepta, as according to this theory they should beentirely endodermal in origin.Bhagavan et al proposed that the wrinkling or

infolding of the gall bladder bud found in catsand guinea pigs during the solid stage ofdevelop-ment" may occur in the human fetus." Thisprocess draws mesoderm into the gall bladderand when subsequent vacuolisation occurs, theclefts may fail to coalesce. The remaining intra-luminal endoderm and mesoderm could give riseto fibromuscular septa lined by epithelium.

855

on February 23, 2021 by guest. P

rotected by copyright.http://gut.bm

j.com/

Gut: first published as 10.1136/gut.34.6.853 on 1 June 1993. D

ownloaded from

856 Tan, Howvard, Driver, Murray-Lyon

A third hypothesis based on the mode offormation of the 'Phrygian cap' suggests that agall bladder may in its solid stage grow morerapidly than its gall bladder bed, and may thus besubjected to bending and kinking.'8 The nextphase of intraepithelial clefting is then incom-plete, and intraluminal septa result. " Thesetheories, however, do not explain the bile ductdilatation, found in two cases, which is usuallyconsidered to be the result of a defect in embryo-genesis of the bile ducts.9 20

Multiseptate disease of the gall bladder doesnot seem to be associated either with severeinflammation or gall stones, and seems to repre-sent a different entity from the single or doubleseptated gall bladder in which chronic chole-cystitis and gall stones often occur. The associa-tion with congenital anomalies ofthe bile ducts intwo cases may be evidence for an embryologicalevent in the formation of multiseptate gallbladder.

We thank Dr J G Moscoso for the photomicrographs, and Mrs IHarris and Mr H Khawaja for translating German and Frenchreferences.

1 Simon M, Tandon BN, Multiseptate gall bladder. A casereport. Radiology 1%3; 80: 84-6.

2 Pery M, Kaftori JK, Marvan H, Sweed Y, Kerna H. Ultra-sonographic appearance of multiseptate gall bladder. Reportof a case with coexisting choledochal cyst. Joumal ofClinicalUltrasound 1985; 13: 570-3.

3 Spech RJ, Hummer N, Braun H. Septierte Gallenblasen. MedKlin 1975; 70: 941-6.

4 Lev-Toaff AS, Friedman AC, Rindsberg SN, Caroline DF,Maurer AH, Radecki PD. Multiseptate gall bladder: in-cidental diagnosis on sonography. American rournal ofRoentgenology, Radium Therapy, and NuclearMedicine 1987;148: 1119-20.

5 Olivia IO, Moran MR, Sanchez FL, Alonso AG. Multi-septategall bladder. Int Surg 1985; 70: 83-8.

6 Vasinrapee P, Linden K, Cook RE. Multiseptate gall bladderdemonstrated on Tc-99m hepatobiliary imaging. Clin NuclMed 1990; 15: 272.

7 Croce EJ. The multiseptate gall bladder. Arch Surg 1973; 107:104-5.

8 Codinach N, Sarles H, Sahel J, Alemanno J. La vesiculebiliaire multiseptale: presentation d'un cas. ArchivesFrancaisesdesMaladiesdel'AppareilDigestif 1975;64: 431-5.

9 Alawneh I, Baranzanchi I, Jacobi K. Mehrfach SeptierteGallenblase als Ursache von Koliken. Med Klin 1981; 76:190-2.

10 Knight S. Anomalies of the gall bladder, bile ducts, andarteries. In: Smith R, Sherlock S, eds. Surgery of the gallbladder and bile ducts. 2nd ed. London: Butterworths, 1981:105.

11 Bhagavan BS, Amin PB, Land AS, Weinberg T. Multiseptategall bladder. Embryogenetic hypothesis. Arch Pathol 1970;89: 382-5.

12 Jena PK, Hardie RA, Hobsley M. Multiseptate hypoplasticgall bladder. BrJ Surg 1977; 64: 192-3.

13 Haslam RHA, Gayler BW, Ebert PA. Multiseptate gall-bladder. A cause of recurrent abdominal pain in childhood.AmJ Dis Chil 1966; 112: 600-3.

14 Okuda K, Nakajima M, Nakayama M, Nomura F. Multi-septate gall bladder: report of a case with a review ofliterature. Acta Hepato-Gastroenterologica 1979; 26: 70-5.

15 Fremond B, Stasik C, Jouan H, Marcorelles P, Chapuis M,Treguier C, et al. La vesicule multicloisonnee: une mal-formation rare des voies biliares. Chir Pediatr 1989; 30:292-4.

16 Moore KL. Development ofthe liver and biliary apparatus. In:Moor KL, ed. The developing human. Clinically orientedembryology. 4th ed. Philadelphia: Saunders WB, 1988:223-4.

17 Boyden EA. Accessory gallbladder; embryological and com-parative study of aberrant biliary vesicles occurring in manand domestic mammals. American Journal ofRoentgenology,Radium Therapy, and Nuclear Medicine 1935; 33: 589-602.

18 Boyden EA. "Phrygian cap" in cholecystography; congenitalanomaly of the gall bladder. AmJ_ Anat 1926; 38: 177-222.

19 Howard ER. Choledochal cysts. In: Howard ER, ed. Surgery ofliver disease in children. Oxford: Butterworth-Heinnimann,1991: 78-9.

20 O'Neill JA Jr. Choledochal cyst. In: Welch KJ, Randolph JG,RavitchMM, O'Neill JA Jr, Rowe MI, eds. Pediatric surgery.Chicago: Year Book Medical Publishers, 1986: 1056.

21 BiggRL. Multiseptate gall bladder. ArchSurg 1964; 88: 501-2.22 Sachsse VW, Hiller W. Zur vielfach septierten Gallenblase;

Kasuistischer Beitrag. Fortschritte auf dem Gebiete derRoentgenstrahlen und der Nuclearmedizin 1968; 108: 547-8.

23 Toombs BD, Foucar E, Rowlands BJ, Strax R. Multiseptategall bladder. South MedJ 1982; 75: 610-2.

24 Shaw RB, Donato CA, Douglas DD, Sass JK, Montegut FJ.Multiseptate gall bladder diagnosed during pregnancy.Am Surg 1975; 41: 818-22.

25 Konishi F, Saito H, Asano A. A case of multiseptate gallbladder. Japanese Journal of Gastroenterology 1975; 72:1252-6.

on February 23, 2021 by guest. P

rotected by copyright.http://gut.bm

j.com/

Gut: first published as 10.1136/gut.34.6.853 on 1 June 1993. D

ownloaded from