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Table 4 Literature studies on CD

From: Surgical management of Caroli disease in a low-mid income country: a single-center study and review of literature

Study

Fard-Aghaie et al. [19]

Lewin et al. [20]

Wabitsch et al. [21]

Fahrner et al. [22]

Yamaguchi et al. [23]

Mabrut et al. [24]

Year

2022

2021

2021

2019

2018

2013

Country

Germany

France

Germany

Germany

Switzerland

France

No of patients (Male/Female)

14 (6/8)

66 (40/26)

7 (3/4)

21 (7/14)

25 (15/10)

155 (89/66)

CD/CS

12/2

31/35

ND

6/13

23/2

125/30

Median Age

56,5

32,3

49

56

53,4

55,7

Comorbidities/medical history (%)

ND

Cholecystectomy (27%), sphincterotomy (18%), personal history of renal ciliopathies (18%), familial history of liver ciliopathies (9%)

Primary hypertension (28%), type II diabetes (28%), hypothyroidism (28%), paroxysmal atrial fibrillation (14%), Conn syndrome due to a cortical adrenal adenoma (14%), chronic obstructive pulmonary disease (14%), chronic gastritis (14%), arthritis (14%), DVT and pulmonary embolism (14%)

Previous surgery: cholecystectomy (28%), appendectomy (14%), surgical correction of a rectal prolapse (14%), stripping of lower-limb varices (14%), cataract operation (14%)

ND

Previous surgery: cholecystectomy (48%), choledochotomy (12%), surgical sphincterotomy (12%), biliodigestive anastomosis (12%), resection of a main bile duct diverticulum (4%)

Associated lesions: congenital hepatic fibrosis (8%), intrahepatic pancreatic ectopia (4%), renal cysts (4%)

Drainage of a hepatic abscess (4%)

Associated HBP disease: biliary lithiasis (56,8%), secondary biliary cirrhosis (47,7%), unilobar liver atrophy (14,2%), acute pancreatitis (3,2%), CHF (2,6%), NASH (1,3%), polycystic liver (1,3%), chronic pancreatitis (1,3%), autoimmune hepatitis (0,6%), undetermined, cholestasis (0,6%)

Previous surgery: cholecystectomy (42,6%), biliodigestive anastomosis (6,5%), choledochotomy (5,2%), Portosystemic shunt (2,6%), IHS extraction (1,9%), hepatectomy (1,3%)

Presentation (%)

Recurrent lithiasis (42%), recurrent pain (35%), recurrent cholangitis (14%), liver abscess (7%)

Severe cholangitis or jaundice (59%), abdominal pain (18%), asymptomatic (13%), pancreatitis (1%), hematemesis (7)

Biliary lithiasis (57%), cholangitis (42%), systemic sepsis (14%), pancreatic and liver cysts (14%)

Recurrent episodes of cholangitis (58%), pain (63%), fever (26%)

Asymptomatic (4%)

Biliary lithiasis (80%), ↑GGT and ALP (4%), cholangitis (4%), pain and recurrent jaundice (4%), acute cholecystitis (4%), acute pancreatitis (4%), hepatic abscess (4%)

Asymptomatic (10,3%)

Cholangitis (58,1%), abdominal pain (29%), jaundice (24,5%), acute pancreatitis (11%), loss of weight (6,5%), asthenia (6,5%), gastrointestinal bleeding (4.5%), ascites (3,2%), abdominal mass (1,3%), pruritus (2,6%), biliary peritonitis (0,6%), complicated delay between symptom and diagnosis (60,6%)

Imaging findings

ND

Bile duct dilations, ‘Dot sign’, biliary lithiasis, hepatomegaly, liver atrophy, liber abscess, biliary hamartoma, large regenerative nodules, signs of portal hypertension, renal cysts

Intrahepatic gallstones, bile duct dilations

ND

ND

“Central dot sign”

Unilobar (Left/Right)

14 (11/3)

21 (19/2)

7 (5/2)

12 (7/5)

25 (20/5)

107 (91/16)

Laparoscopic approach (n =)

ND

ND

6

ND

ND

ND

Open approach

(n =)

ND

ND

0

ND

ND

ND

Conversion to open surgery (n =)

ND

ND

1

ND

ND

ND

Interventions and treatments (%)

ERCP (78%), lithotripsy (7%), PTD (14%), left hepatectomy (64%), right hepatectomy (14%), left lateral sectionectomy (14%), segmentectomy (7%)

Conservative management (53%), hepatectomy (24%), liver transplantation (21%), biliodigestive anastomosis (3%)

Left lateral bisegmentectomy (28%), left hemihepatectomy (28%), left lobectomy (14%), posterolateral hepatectomy (14%), complete right hemihepatectomy (14%), bilio-jejunal anastomoses (14%)

ERCP (89%), stenting of the biliary tree (21%), left lateral segmentectomy (38%), left hepatectomy (33%), right hepatectomy (14%), monosegmentectomy (4%), LT (9%), hepaticojejunostomy (4%)

left lobectomy (40%), left hepatectomy (36%), biliojejunal anastomosis (24%), right hepatectomy (12%), segmentectomy (12%), right hepatectomy (12%), ERCP (8%), drainage of abscess (4%)

Unilateral left LR (81.1%), liver transplantation (18.9%), IHS extraction (32%), unilateral right LR (11.8%), biliary drainage (10.2%), bilobar liver resection (7.5%), surveillance (0,6%), other (6.1%)

Post-operative complications (%)

Bilioma (7%), postoperative bleeding (7%), bile leakage (7%)

Death (9%), severe cholangitis (3%), gastrointestinal hemorrhage (3%), postoperative complications after liver transplant (1%)

Seroma (28%), cholangitis (14%), left-sided pleural effusion (14%)

Biliary leakage (14%), pleural effusion (4%), urinary tract infection (4%)

Intra-abdominal abscess (4%), bilioma (4%), biliary fistula (4%), liver hematoma (4%), intra-abdominal collection (4%)

Biliary fistula (7%), mortality (2%), hepatic artery thrombosis (1,2%), primary nonfunction and acute rejection (1,2%), hemorrhage (0,9%)

Rate of CCA (%)

7%

1,5%

0%

19%

4%

5.2%