Therapeutic approaches for portal biliopathy: A systematic review

Table 1 Characteristics of four stages in portal biliopathy natural history

Stage	Portal cavernoma	Biliopathy	LFTs	Symptoms	Complications
Preclinic	Yes	No	Normal	No	No
Asymptomatic	Yes	Early changes	Normal or abnormal	No	No
Symptomatic	Yes	Advanced changes	Abnormal	Yes	No
Complicated	Yes	Advanced changes	Abnormal	Yes	Yes

LFTs: Liver function tests.

Table 2 Endoscopic management of portal biliopathy: summary of case series

Ref.	No. of patients	Biliary abnormalities	First treatment	Follow-up	Further treatments	Complications/outcome
Bhatia et al[27], 1995	4 symptomatic	Stenosis + stones 3 CBD stones 4	ES + SE + NBD 4	3-8 mo	Multiple ERCP 4	None
Perlemuter et al[20], 1996	8 symptomatic	Stenosis 8 CBD stones 2 Cholangitis 1	ES+NBD 3 BD 1 PSS 1 UDCA 2	6-60 mo	Multiple ES 1	Death 2 (cholangitis 1; stroke 1)
Condat et al[3], 2003	7 symptomatic	Cholecystitis/right hypocondrial pain 4 Cholangitis 1 Stenosis 2	Cholecystostomy + SE 1 Stent 1 BA + PTBD 1 UDCA 4	4-25 mo	-	Haemobilia 1
Sezgin et al[28], 2003	10 symptomatic	Stenosis 9 IE stones 1	ES + stent 10 NBD 4 BD 4	3.3 yr (range 1-7)	Multiple ERCP 5	Haemobilia 1 Cholangitis 5 Death 1
Dumortier et al[22], 2003	6 symptomatic	Stenosis 5 CBD stones 2	ES 5 BD 5 SE 2 Stent 1	10 mo (range 2 d-18 mo)	Multiple ERCP + PSS 4	Cholangitis 1 Cholecystitis 4
Khare et al[25], 2005	13 symptomatic	Stenosis 10 CBD stones 8	Stent 4 BD 6 SE 4	-	PSS 8 BA 1 Multiple ERCP 2 Splenectomy + devasc 2	Death 1
Dhiman et al[29], 2007	12 symptomatic	Stenosis 7 CBD stones 5 CBD varices 1 Mirizzi’ssdr 1	PSS 5 ES 3 ES + BD 2 Stent 4	19 mo (6-132 mo)	Multiple ERCP in pts initially treated with stent	Cholangitis in 2 pts treated with stent
Vibert et al[30], 2007	19 symptomatic	IE biliary dilation 9 IE stones 7 CBD stones 4	PSS group: PTBD 1 SRS 10 NPSS group: ES + stent 2 ES + SE 1 PTBD 6 BA 4	19 pts 8.3 yr	PSS group: BA 5 NPSS group PTBD 1 after ERCP and 4 after BA	Resolution 17 Death 3
Oo et al[17], 2009	13 symptomatic	Stenosis 13 CBD stones 10 GB stones 11	UDCA 1 Stent 7 ES + SE 1 Failed ERCP 4	2 yr (1-18 aa)	Metallic stent 3 Stent exchange 2 PSS 3 (2 TIPS, 1 surgical) LT 1	Haemobilia 2 Sepsis 3
Llop et al[1], 2011	14 symptomatic	Stenosis 14 CBD stones 6 GB stones 2	ES + SE 6 ES + UDCA 2 Cholecystectomy 2	-	Multiple ERCP 1 BA 1	-
Saraswat et al[24], 2013	20 symptomatic	Stenosis 20 CBD stones 8 GB stones 6	ES + SE 8 Stent 9 BD + stent 11	18 mesi (range 3-188)	Multiple ERCP 11	In 130 procedures: Cholangitis 40 Haemobilia 9
Ramchandani et al[31], 2013	5 symptomatic	CBD stenosis 2 CHD stenosis 1 CBD stones 2	Metallic stent 1 Plastic stent 2 BD + stent 1 Intracholedocal lithotripsy 1	6-7 mo	SRS 2 Stent Exchange 1	- Resolution 5
Cellich et al[23], 2015	8 symptomatic 1 asymptomatic	Stenosis 7 CBD stones 3 GB stones 1	ES 7 BD 4 SE 2 Stent 7	-	PSS 1 Stent exchange 3 BA 3	Cholangitis 3 Haemobilia 1

CBD: Common bile duct; GB: Gallbladder; IE: Intrahepatic; ES: Endoscopic sphincterotomy; SE: Stone extraction; NBD: Nasobiliary drainage; BD: Balloon dilation; PSS: Porto-systemic shunt; NPSS: No porto-systemic shunt; UDCA: Ursodeoxycholic acid; ERCP: Endoscopic retrograde cholangio-pancreatography; BA: Bilioenteric anastomosis; SRS: Splenorenal shunt; LT: Liver transplantation; TIPS: Transjugular intrahepatic porto-systemic shunt.

Table 3 Endoscopic management of portal biliopathy: summary of case reports

Ref.	Patients	Biliary abnormalities	First treatment	Follow-up	Further treatment	Complications
Mörk et al[32], 1998	2	CBD stenosis 2	Stent 2	-	Multiple Stent exchange + PSS 1	Cholangitis 1
Solmi et al[33], 1998	1	Stenosis	Stent	-	-	-
Mutignani et al[34], 2002	3	CBD stenosis 3	Stent 3	-	PSS 3	Haemobilia 3
Perego et al[35], 2003	1	Stenosis + CBD stones	- Stent	3 yr	Multiple stent exchange→PTBD + dilation and SE	-
Umphress et al[15], 2004	M, 62 yr	IE and CBD stones	ES + SE + stent	1 yr	Stent exchange and cholecistectomy	-
Guerrero Hernández et al[36], 2005	M, 29 yrs	CBD stenosis	ERBD + Sugiura	-	-	-
Rosenthal et al[37], 2008	F, 44 yr	CBD stenosis and stones	Stent + SE	-	PSS	-
Ajayi et al[38], 2009	F, 13 yr	Stenosis	ES + BD + stent	6 mo	Multiple stent exchange	-
Layec et al[39], 2009	F, 74 yr	Stenosis	Metallic stent	18 mo	Metallic stent	Haemobilia
Sharma et al[40], 2009	M, 35 yr F, 30 yr M, 25 yr	CBD stenosis 3	ES+SE 3	-	-	Haemobilia 3
Vasiliadis et al[41], 2009	F, 39 yr	CBD stenosis	ES	19 mo	Multiple stent exchange→PSS + cholecistectomy	-
Cantù et al[42], 2010	M, 31 yr	CBD stenosis	ES + stent	4 yr	-	-
Martinez et al[43], 2011	M, 34 yr	IE diltaions	Stent	-	-	-
Mistry et al[44], 2012	M, 28 yr	CBD stenosis	BD + BA + PTBD	-	Percutaneous transhepatic-gastrostomy	Haemobilia
Alam et al[45], 2012	M, 30 yr F, 19 yr	CBD stenosis 2 GB stones 1 CBD stones 1	PTBD Stent	-	Cholecistectomy	-
D’Souza et al[46], 2013	M, 49 yr	CBD stenosis	Stent + BD (after stent removal)	-	PSS + splenectomy	Haemobilia (post-biopsy)
Bernon et al[47], 2014	M, 36 yr	CBD stenosis and stones	SE + stent	1 yr	Stent exchange→PTBD→cholecistectomy, intrahepatic 3 semgment bypass	Cholangitis
Hyun et al[26], 2015	M, 33 yr	CBD stenosis	ERBD→PTBD + portal stent	3 mo	-	Haemobilia

CBD: Common bile duct; ES: Endoscopic sphincterotomy; SE: Stone extraction; BD: Balloon dilation; PSS: Porto-systemic shunt; IE: Intrahepatic; BA: Bilioenteric anastomosis; ERBD: Endoscopic retrograde biliary drainage; PTBD: Percutaneous transhepatic biliary drainage.

Table 4 Surgical management of portal biliopathy: summary of case series and case reports

Ref.	No. of patients	Biliary abnormalities	First treatment	Follow-up	Further treatments	Complications/outcome
Chaudhary et al[53], 1998	9 symptomatic	Stenosis: 2 CBD stones: 2	BA 2 SRS 7	9 pts -	BA 2 Stent 1 ES + SE 2	Death 1 Resolution 7
Condat et al[3], 2003	7 symptomatic	Cholecystitis/right hypocondrial pain 4 Cholangitis 1 Stenosis 2	Cholecystostomy + ERCP 1 Stent 1 BA+PTBD 1	4-25 mo	-	Haemobilia 1
Gauthier-Villars et al[19], 2005	8 symptomatic (pediatric)	Stenosis 6 Biliarydilation 8	- PSS 8	8 pts 4.5-15 yr	-	Complete resolution 7 Partial resolution 1
Khare et al[25], 2005	13 symptomatic	A: Stenosis 5 B: CBD stones 3 C: Stenosis + CBD stones 5	A: PSS 4; BA 1 B: ERCP 2; PSS + BA 1 C: Unsuccessful ERCP 4; PSS 3; Splenectomy + devasc 2	-	A: Multiple ERCP: 1 B: Splenectomy 1 (post-ERCP) C: Multiple ERCP 2; BA 1; Splenectomy + BA 1	Death 1
Vibert et al[30], 2007	19 symptomatic	IE biliary dilation 9 IE stones 7 CBD stones 4	PSS group: PTBD 1 SRS 10 NPSS group: ES + stent 2 ES + SE 1 PTBD 6 BA 4	19 pts 4-30 mo	PSS group: BA 5 NPSS group PTBD 1 after ERCP and 4 after BA	Resolution 17 Death 3
Dhiman et al[2], 2007	12 symptomatic	Stenosis: 7 CBD stones: 5 Choledochal varices 2 Mirizzi’ssyndrome 1	PSS 5 ES 3 ES + dilation 2 Stent 4	19 mo (range 6-132)	Multiple stent exchange in pts initially treated with stent	Cholangitis in 2 pts treated with stent
D’Souza et al[54], 2009	1 symptomatic	CBD stenosis + GB stones	Pre-surgery stent→PSS + BA (single stage)	18 mo	-	Resolution
Camerlo et al[51], 2010	3 symptomatic	Stenosis 3	PSS 3 Stent 1 (pre-PSS)	3 pts 2-13 yr	-	Resolution 3
Agarwal et al[55], 2011	39 symptomatic	Stenosis 15 CBD stones 7 GB stones 12 IE dilation 39	SRS 37 BA 2	37 pts 32 mo	ES ± SE 10 BA 12 ES + cholecystectomy 1	Resolution 35
Chattopadhyay et al[52], 2012	24 symptomatic 32 asymptomatic	CBD stenosis 3 Multiple stenosis 5 IE dilation 14 CBD stones 7 GB stones 11	ERCP pre-surgery 12 PSS 40 Splenectomy + devasc. 16	43 pts 48 mo (range 14-120)	ES + SE 2 Multiple ES + stent 5 BA 2	Resolution 38 Death 1
Suárez et al[56], 2013	3 symptomatic	Stenosis 3	UDCA 1 BA 1	-	-	-
Bhatia et al[57], 2014	2 symptomatic	GB stones	Cholecistectomy	-	-	Resolution
Liu et al[58], 2015	18	Stenosis 6 Dilations 6 Stenosis + dilations 6	PSS 18	-	-	Resolution 15

CBD: Common bile duct; GB: Gallbladder; IE: Intrahepatic; ES: Endoscopic sphincterotomy; SE: Stone extraction; BD: Balloon dilation; PSS: Porto-systemic shunt; NPSS: No porto-systemic shunt; UDCA: Ursodeoxycholic acid; ERCP: Endoscopic retrograde cholangio-pancreatography; BA: Bilioenteric anastomosis; SRS: Splenorenal shunt; FU: Follow-up.