page 2 of 13 - ijhpd · 2018-12-07 · 136 pain, jaundice and fever due to biliary stone disease,...

Manuscript Accepted Peer Reviewed | Early View Article

of 13

Early View Article: Online published version of an accepted article before publication in the final form.

Journal Name: International Journal of Hepatobiliary and Pancreatic Diseases (IJHPD)

Type of Article: Case Series

Title: Low Convergence of Hepatic Ducts: A Rare Extrahepatic Biliary Tree Anatomical Variation

Authors: Awang Dahlan Dayang Azzyati, Nik Abdullah Nik Azim, Julaihi Rokayah

doi: To be assigned

Early view version published: July 17, 2015

How to cite the article: Azzyati A.D.D, Azim N.A.N, Rokayah J. Low Convergence of

Hepatic Ducts: A Rare Extrahepatic Biliary Tree Anatomical Variation. International

Journal of Hepatobiliary and Pancreatic Diseases (IJHPD). Forthcoming 2015.

Disclaimer: This manuscript has been accepted for publication. This is a pdf file of the Early View Article. The Early View Article is an online published version of an accepted article before publication in the final form. The proof of this manuscript will be sent to the authors for corrections after which this manuscript will undergo content check, copyediting/proofreading and content formatting to conform to journal’s requirements. Please note that during the above publication processes errors in content or presentation may be discovered which will be rectified during manuscript processing. These errors may affect the contents of this manuscript and final published version of this manuscript may be extensively different in content and layout than this Early View Article.


of 13

TYPE OF ARTICLE: Case Series 1

2

TITLE: Low Convergence of Hepatic Ducts: A Rare Extrahepatic Biliary Tree 3

Anatomical Variation 4

5

AUTHORS: 6

Awang Dahlan, Dayang Azzyati1, MBBS 7

Nik Abdullah, Nik Azim2, MD 8

Julaihi, Rokayah3, MBBS 9

10

AFFILIATIONS: 11

1Surgical Registrar, Department of Surgery, Sarawak General Hospital, Kuching, 12

Sarawak, Malaysia, [email protected] 13

2Consultant Hepatobiliary Surgeon and Head of Department, Department of Surgery, 14

Sarawak General Hospital, Kuching, Sarawak, Malaysia, [email protected] 15

3General Surgeon, Department of Surgery, Sarawak General Hospital, Kuching, 16

Sarawak, Malaysia, [email protected] 17

18

CORRESPONDING AUTHOR DETAILS: 19

Dayang Azzyati Awang Dahlan 20

Complete Mailing Address: Lot 9265, Lorong Kenanga 5, Jalan Kenanga, Gita, Petra 21

Jaya, Kuching, Sarawak, Malaysia - 93050 22

Phone number: +060168788094 23

Email: [email protected] 24

Fax number: +06082419495 25

26

SHORT RUNNING TITLE: Rare Low Convergence of Hepatic Ducts 27

28

GUARANTOR OF SUBMISSION : The corresponding author is the guarantor of 29

submission 30

31


of 13



34

ABSTRACT 35

Introduction: 36

Low convergence of hepatic ducts is extremely rare. Here, the right and the left 37

hepatic ducts course down toward the second part of the duodenum separately and 38

converge just proximal to the ampulla of Vater, forming a short common bile duct. 39

Case Series: 40

We describe 2 cases of a 43-year-old gentleman and a 53-year-old lady with this 41

anatomical anomaly who presented with obstructive jaundice. Endoscopic retrograde 42

cholangiopancreaticography (ERCP) demonstrated low convergence of the hepatic 43

ducts. Both cases were associated with biliary ectasia and complicated with primary 44

ductal stones. Excision of the anomalous extrahepatic bile ducts with 45

hepaticoenterostomy was performed for one of the cases, however the other was 46

lost to follow-up. 47

Conclusion: 48

Definitive operative intervention seems prudent despite the lack of consensus in the 49

management of such anomaly, especially when there are concomitant biliary ectasia 50

and ductal stones. 51

52

Keywords: Hepatic ducts, extrahepatic biliary tract, low convergence, distal 53

bifurcation, biliary stone disease 54

55

56

57

58

59

60


of 13



INTRODUCTION 63

Anatomical variations of the biliary tree are not uncommon and numerous anomalies 64

have been described in the literature. The right and the left hepatic ducts converge to 65

form the common hepatic duct just after exiting the liver hilum in 60%-70% of the 66

cases[1]. However, low convergence of the hepatic ducts is extremely rare and the 67

exact incidence is unknown with review of the literature resulted in only a few case 68

reports [2,3]. Here, the right and the left hepatic ducts course down toward the 69

second part of the duodenum separately and converge just proximal to the ampulla 70

of Vater, forming a short common bile duct. This may become significant during 71

operative intervention as failure to recognize such anomaly may lead to iatrogenic 72

biliary injury. 73

74

CASE SERIES 75

Case 1 76

A 43-year-old gentleman presented with epigastric pain and obstructive jaundice. 77

Ultrasonography (USG) showed a normal liver and gallbladder with dilated CBD and 78

intrahepatic ducts (IHD) secondary to choledocholithiasis. ERCP confirmed these 79

findings (Figure 1). ERCP with attempted stone extraction was challenging, made 80

difficult by the presence of a huge pre-pyloric gastric ulcer. Thus a biliary stent was 81

inserted to temporarily relieve the obstruction. He underwent open cholecystectomy 82

and CBD exploration. Intra-operatively, the CBD was dilated with multiple soft stones 83

within. Cholangioscopy confirmed no residual stones. Intra-operative biliary stenting 84

was done. He recovered uneventfully and the biliary stents were removed 2 months 85

later. ERCP revealed the presence of low convergence of the hepatic ducts (Figure 86

2). Patient was counselled for operative intervention but was not keen and 87

subsequently was lost to follow-up. 88

89

90

91


of 13

Case 2 92

A 53-year-old lady who had previous open cholecystectomy for empyema of the 93

gallbladder 16 years ago presented with epigastric pain and obstructive jaundice. 94

ERCP revealed low convergence of the hepatic ducts with a large stone in the left 95

hepatic duct (Figure 3). Both the hepatic ducts and the CBD were grossly dilated and 96

ectatic necessitating biliary stenting. Computed tomography (CT) showed normal 97

liver, mild dilatation of the IHD with marked dilatation of the left and right hepatic 98

ducts and CBD. Cholangiojejunostomy was performed and intra-operatively, both the 99

hepatic ducts and CBD were dilated with 1 large stone in the left hepatic duct, 100

measuring 2.5 cm x 1.5 cm and 2 smaller stones in the right hepatic duct, measuring 101

1.0 cm x 1.0 cm. Both hepatic ducts were transected as proximal as possible close 102

to the lower liver edge. The medial edges of the ducts were sutured with interrupted 103

polyglyconate sutures MaxonTM 4/0 to fashion a single duct and a retrocolic 104

cholangiojejunostomy was created (Figure 4 and Figure 5). Histopathological 105

examination of the hepatic ducts and CBD showed chronic inflammation. 106

Subsequent follow-ups found she was well with no stone recurrence. 107

108

DISCUSSION 109

Anatomical variations of the biliary tree are not uncommon and numerous anomalies 110

have been described in the literature, based on observation from imaging studies, 111

operative reports and autopsy discoveries, in as high as 47% of the cases [4]. These 112

anomalies may be minor or major with variable clinical significance. 113

The numerous anatomical variations and anomalies are attributable to the underlying 114

complexity of the embryological development of the liver and biliary tree. By the 5th 115

week of intrauterine life, the liver, biliary tract, gallbladder and pancreas are 116

recognizable structures. During this period, lengthening of the common duct occurs. 117

However, the lumen is occluded by the rapidly proliferating epithelial cells. By the 118

end of the 5th week, recanalization occurs and this start from the proximal portion 119

progressing toward the distal portion of the lumen. By the 8th week, the common duct 120

becomes patent and by the 12th week, bile starts to flow from the liver to the second 121

part of the duodenum [5]. 122


of 13

The right and the left hepatic ducts converge to form the common hepatic duct just 123

after exiting the liver hilum in 60%-70% of the cases [1]. Low convergence of the 124

hepatic ducts is extremely rare and resulted in longer than usual right and left 125

hepatic ducts with resultant shorter than usual common bile duct. The common 126

hepatic duct may or may not be present, depending on the site of insertion of the 127

cystic duct. Frequently, the site of insertion of the cystic duct is anomalous too. 128

Similar to one of our case, the cystic duct may insert to the right hepatic duct. 129

Insertion to the left hepatic duct or low insertion to the more distal portion of the 130

common bile ductal has been reported too [2,3]. 131

Such anomalies are postulated to be the result of embryological malformations, 132

either due to incomplete recanalization or maldivision of the extrahepatic ductal 133

portion of the embryonic hepatic diverticulum [5,6]. 134

Clinical presentation of such biliary tree anomalies includes recurrent abdominal 135

pain, jaundice and fever due to biliary stone disease, cholangitis or pancreatitis. 136

Frequently, these anomalies are discovered during the investigations and 137

management of the presenting symptoms. The importance of recognizing such 138

anomalies during surgical procedures such as laparoscopic cholecystectomy, is 139

emphasized as failure to do so may result in iatrogenic biliary tract injury with 140

resultant bile leak and ductal stricture [7,8]. 141

Both of our cases underwent open cholecystectomy under emergency setting as part 142

of the management of their presenting symptoms. However, intra-operatively the 143

anomaly was missed. This may be due to the presence of inflammatory and 144

oedematous tissues or fibrous adhesions surrounding the extrahepatic bile ducts 145

making the anomaly less obvious. For the first case, the initial ERCP did not reveal 146

obvious low convergence due to the orientation of the film. This was subsequently 147

detected in the later ERCP. 148

Biliary tree anomalies may predispose to formation of biliary stones [9]. Both of our 149

patients presented with complications of primary ductal stone. In our cases, we 150

believe such anomalous arrangement may cause abnormal bile flow or bile stasis. 151

We postulated that the long hepatic ducts lie in the edge of the lesser omentum 152

unsupported by solid liver parenchyma and an increase in the intraluminal pressure 153

(ie secondary to stone obstruction) will easily stretches the ductal wall and causes 154


of 13

ductal dilatation. This perpetuates the vicious cycle of biliary stasis, infection, further 155

stones formation and obstruction. 156

The presence of recurrent and chronic biliary stones is associated with carcinoma of 157

the biliary tract in the long term [10-11]. Biliary stones may initiate the hyperplasia-158

carcinoma sequence. As mentioned, it causes bile obstruction and stasis 159

predisposing to infection. These conditions induce chronic inflammation via 160

prolonged exposure of the epithelial cells to bile acids and pancreatic enzymes in the 161

bile. These cause increased cellular proliferation, initiating the multistep progression 162

from epithelial hyperplasia to metaplasia, then dysplasia and finally carcinoma of the 163

biliary tract [10,12]. 164

Hence, a definitive operative intervention seems prudent despite the lack of 165

consensus in the management of such anomalies, especially in cases with 166

concomitant biliary ectasia and ductal stones. We felt that the decision for definitive 167

management was challenging due to the scarcity of similar cases in literature. We 168

decided to extrapolate the management from the management of adult choledochal 169

cyst. Total excision of the extrahepatic bile duct and gallbladder followed by 170

hepaticoenterostomy is the treatment of choice. 171

172

CONCLUSION 173

Low convergence of hepatic ducts is extremely rare. Such anomaly may be 174

associated with biliary ectasia and ductal stones. Not only it predisposes the patients 175

to recurrent obstruction, infection and pancreatitis, in the long term it may also lead 176

to carcinoma of the biliary tract. Definitive operative intervention seems prudent, 177

especially in our cases where there are concomitant biliary ectasia and ductal 178

stones. 179

180

CONFLICT OF INTEREST 181

The authors declare no conflict of interest. 182

183

AUTHOR’S CONTRIBUTIONS 184

Dayang Azzyati Awang Dahlan 185


of 13

Main contributions to conception and design, acquisition of data, analysis and 186

interpretation of data, drafting the article, critical revision of the article, and final 187

approval of the version to be published 188

189

Nik Azim Nik Abdullah 190

Substantial contributions to conception and design, acquisition of data, analysis and 191

interpretation of data, drafting the article, critical revision of the article, final approval 192

of the version to be published 193

Rokayah Julaihi 194

Substantial contributions to conception and design, acquisition of data, analysis and 195

interpretation of data, drafting the article, critical revision of the article, final approval 196

of the version to be published 197

198

REFERENCES 199

200 1. Crawford JM. Development of the intrahepatic biliary tree. Semin Liver Dis 201

2002;22(3):213-226. 202

2. Koruk I, Aydin U, Koruk S, Aydinli M. A newly defined biliary anatomic variation. 203

Turk J Gastroenterol 2011;22(2):232-233. 204

3. Hafner M, Schofl R, Gangl A. A rare anomaly of the biliary tree: The interhepatic 205

duct. Gastrointest Endosc 1997;45(6):523-525. 206

4. Lamah M, Dickson GH. Congenital anatomical abnormalities of the extrahepatic 207

biliary duct: A personal audit. Surg Radiol Anat 1999;21(5):325-327. 208

5. Ando H. Embryology of the biliary tract. Dig Surg 2010; 27:87-89. 209

6. Roskams T, Desmet V. Embryology of extra- and intrahepatic bile ducts, the 210

ductal plate. Anat Rec (Hoboken) 2008;291(6):628-635. 211

7. Talpur KA, Laghari AA, Yousfani SA, Malik AM, Memon AI, Khan SA. Anatomical 212

variations and congenital anomalies of extra hepatic biliary system encountered 213

during laparoscopic cholecystectomy. J Pak Med Assoc 2010;60(2):89-93. 214

8. Hasan MM, Reza E, Khan MR, Laila SZ, Rahman F, Mamun MH. Anatomical and 215

congenital anomalies of extra hepatic biliary system encountered during 216

cholecystectomy. Mymensingh Med J 2013;22(1):20-26. 217


of 13

9. Kubota Y, Yamaguchi T, Tani K, Takaoka M, Fujimura K, Ogura M, et al. 218

Anatomical variation of pancreaticobiliary ducts in biliary stone diseases. Abdom 219

Imaging 1993;18(2):145-149. 220

10. Holzinger F, Z’graggen K, Buchler MW. Mechanisms of biliary carcinogenesis: A 221

pathogenetic multi-stage cascade towards cholangiocarcinoma. Ann Oncol 222

1999;10 Suppl 4:122-126. 223

11. Donato F, Gelatti U, Tagger A, Favret M, Ribero ML, Callea F, et al. Intrahepatic 224

cholangiocarcinoma and hepatitis C and B virus infection, alcohol intake, and 225

hepatolithiasis: A case-control study in Italy. Cancer Causes Control 226

2001;12(10):959-964. 227

12. Ohta T, Nakagawa T, Ueda N, Nakamura T, Akiyama T, Ueno K, Miyazaki I. 228

Mucosal dysplasia of the liver and the intraductal variant of peripheral 229

cholangiocarcinoma in hepatolithiasis. Cancer 1991;68(10):2217-2223. 230

231

ABBREVIATIONS 232

ERCP - Endoscopic retrograde cholangiopancreaticography 233

CBD - Common bile duct 234

IHD - Intrahepatic ducts 235

CT - Computed tomography 236

237

FIGURE LEGENDS 238

Figure 1: Cholangiogram demonstrating the insertion of the cystic duct into the right 239

hepatic duct. 240

Figure 2: Cholangiogram demonstrating the long right and left hepatic duct 241

converging to form the short common bile duct; note that the intrahepatic ducts are 242

no longer dilated. 243

Figure 3: Cholangiogram demonstrating the grossly dilated right and left hepatic 244

ducts. 245

Figure 4: Intraoperative image showing the grossly dilated right and left hepatic 246

ducts. 247

Figure 5: Medial edges of the ducts sutured to fashion a single duct before creation 248

of the retrocolic cholangiojejunostomy. 249


of 13

250

251

252

253

Figure 1: Cholangiogram demonstrating the insertion of the cystic duct into the right 254

hepatic duct. 255

256

257


of 13

258

Figure 2: Cholangiogram demonstrating the long right and left hepatic duct 259

converging to form the short common bile duct; note that the intrahepatic ducts are 260

no longer dilated. 261

262


of 13

263

Figure 3: Cholangiogram demonstrating the grossly dilated right and left hepatic 264

ducts. 265

266


of 13

267

Figure 4: Intraoperative image showing the grossly dilated right and left hepatic 268

ducts. 269

270

271

Figure 5: Medial edges of the ducts sutured to fashion a single duct before creation 272

of the retrocolic cholangiojejunostomy. 273

274

275

276

277

278

page 2 of 13 - ijhpd · 2018-12-07 · 136 pain, jaundice and fever due to biliary stone disease,...

Documents