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Caroli's disease misdiagnosed as intraductal papillary neoplasm of the bile duct.

Gu DH, Park MS, Jung CH, Yoo YJ, Cho JY, Lee YH, Seo YS, Yim HJ, Um SH, Ryu HS - Clin Mol Hepatol (2015)

Bottom Line: Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development.Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient.Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea.

ABSTRACT
Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development. Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient. Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct. The asymptomatic patient was treated with liver segmentectomy.

No MeSH data available.


Related in: MedlinePlus

Microscopy findings of the resected specimen. Bile ducts were dilated and had thickened walls (asterisks). Ectasias result in a predisposition to repeated attacks of cholangitis with complications such as intrahepatic lithiasis, amyloidosis, and cholangiocarcinoma, but they were not present in this case. The lining epithelium (arrows) comprised columnar and cuboidal cells (hematoxylin and eosin stain, A: ×40, B: ×200).
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Figure 3: Microscopy findings of the resected specimen. Bile ducts were dilated and had thickened walls (asterisks). Ectasias result in a predisposition to repeated attacks of cholangitis with complications such as intrahepatic lithiasis, amyloidosis, and cholangiocarcinoma, but they were not present in this case. The lining epithelium (arrows) comprised columnar and cuboidal cells (hematoxylin and eosin stain, A: ×40, B: ×200).

Mentions: A 30-year-old man with no underlying diseases visited our hospital for further evaluation and treatment of a liver mass discovered on abdominal computed tomography (CT) scan at another hospital. This patient was asymptomatic on transfer to our hospital. The physical examination showed no abnormality, and the initial vital signs were as follows: body temperature, 36.9℃; pulse rate, 70 beats/min; respiratory rate, 20 breaths/min; and blood pressure, 120/70 mmHg. His height and body weight were 179 cm and 93.4 kg, respectively. He consumed 120-180 g of alcohol per week, and his medical history and familial history were unremarkable. Laboratory examinations showed a white blood cell count of 5,800/µL, hemoglobin level of 15.9 g/L, and platelet count of 272,000/µL. The only abnormal finding on the liver function test was mildly elevated alanine transaminase levels, and the results were as follows: serum aspartate transaminase, 42 IU/L; alanine transaminase, 76 IU/L; alkaline phosphatase, 58 IU/L; gamma-glutamyl transferase, 82 IU/L; total bilirubin, 0.69 mg/dL; albumin, 5.3 g/dL; and international normalized ratio, 0.98. CA 19-9 and α-fetoprotein for malignancy work-up was carried out; 5.7 IU/mL and 3.6 ng/mL, respectively. Results for viral markers including hepatitis B surface antigen and anti-hepatitis C virus were negative. Abdominal CT (Fig. 1A, B) performed at the other hospital and abdominal magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) (Fig. 1C, D) performed at our hospital revealed a localized bile duct dilatation measuring 2.0×0.8 cm in liver segment 5 without a demonstrable intraductal mass lesion and underlying steatosis of the liver. Although an obstructing lesion was not found on the images, we considered the duct obstructed with a mass or parasite proximal to the dilated ductal area because size of the dilated ductal site was very limited and could not find any evidence of other bile duct disease. Assuming that the lesion of the localized bile duct dilatation in liver segment 5 was due to an IPNB, we decided to perform surgery after consultation with the general surgery department because the possibility of malignancy with IPNB could not be excluded. He was transferred to the general surgery department and underwent segmentectomy of liver segment 5. Although the 16×7×16 cm resected liver specimen showed grossly unremarkable findings on the surface or inner surface (Fig. 2A), a diffuse parenchymal hemorrhagic soft dilated lesion was noted in the 4.5×3.5 cm cut surface, 0.5 cm from the resection margins in a serial section of the specimen (Fig. 2B). Histopathologic findings showed congenital dilatation of the intrahepatic bile ducts (Caroli's disease) with approximately 50% centrilobular macrovesicular type fatty change (Fig. 3). This patient is seen regularly at the outpatient department and has no complications 5 months after surgery.


Caroli's disease misdiagnosed as intraductal papillary neoplasm of the bile duct.

Gu DH, Park MS, Jung CH, Yoo YJ, Cho JY, Lee YH, Seo YS, Yim HJ, Um SH, Ryu HS - Clin Mol Hepatol (2015)

Microscopy findings of the resected specimen. Bile ducts were dilated and had thickened walls (asterisks). Ectasias result in a predisposition to repeated attacks of cholangitis with complications such as intrahepatic lithiasis, amyloidosis, and cholangiocarcinoma, but they were not present in this case. The lining epithelium (arrows) comprised columnar and cuboidal cells (hematoxylin and eosin stain, A: ×40, B: ×200).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
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Figure 3: Microscopy findings of the resected specimen. Bile ducts were dilated and had thickened walls (asterisks). Ectasias result in a predisposition to repeated attacks of cholangitis with complications such as intrahepatic lithiasis, amyloidosis, and cholangiocarcinoma, but they were not present in this case. The lining epithelium (arrows) comprised columnar and cuboidal cells (hematoxylin and eosin stain, A: ×40, B: ×200).
Mentions: A 30-year-old man with no underlying diseases visited our hospital for further evaluation and treatment of a liver mass discovered on abdominal computed tomography (CT) scan at another hospital. This patient was asymptomatic on transfer to our hospital. The physical examination showed no abnormality, and the initial vital signs were as follows: body temperature, 36.9℃; pulse rate, 70 beats/min; respiratory rate, 20 breaths/min; and blood pressure, 120/70 mmHg. His height and body weight were 179 cm and 93.4 kg, respectively. He consumed 120-180 g of alcohol per week, and his medical history and familial history were unremarkable. Laboratory examinations showed a white blood cell count of 5,800/µL, hemoglobin level of 15.9 g/L, and platelet count of 272,000/µL. The only abnormal finding on the liver function test was mildly elevated alanine transaminase levels, and the results were as follows: serum aspartate transaminase, 42 IU/L; alanine transaminase, 76 IU/L; alkaline phosphatase, 58 IU/L; gamma-glutamyl transferase, 82 IU/L; total bilirubin, 0.69 mg/dL; albumin, 5.3 g/dL; and international normalized ratio, 0.98. CA 19-9 and α-fetoprotein for malignancy work-up was carried out; 5.7 IU/mL and 3.6 ng/mL, respectively. Results for viral markers including hepatitis B surface antigen and anti-hepatitis C virus were negative. Abdominal CT (Fig. 1A, B) performed at the other hospital and abdominal magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) (Fig. 1C, D) performed at our hospital revealed a localized bile duct dilatation measuring 2.0×0.8 cm in liver segment 5 without a demonstrable intraductal mass lesion and underlying steatosis of the liver. Although an obstructing lesion was not found on the images, we considered the duct obstructed with a mass or parasite proximal to the dilated ductal area because size of the dilated ductal site was very limited and could not find any evidence of other bile duct disease. Assuming that the lesion of the localized bile duct dilatation in liver segment 5 was due to an IPNB, we decided to perform surgery after consultation with the general surgery department because the possibility of malignancy with IPNB could not be excluded. He was transferred to the general surgery department and underwent segmentectomy of liver segment 5. Although the 16×7×16 cm resected liver specimen showed grossly unremarkable findings on the surface or inner surface (Fig. 2A), a diffuse parenchymal hemorrhagic soft dilated lesion was noted in the 4.5×3.5 cm cut surface, 0.5 cm from the resection margins in a serial section of the specimen (Fig. 2B). Histopathologic findings showed congenital dilatation of the intrahepatic bile ducts (Caroli's disease) with approximately 50% centrilobular macrovesicular type fatty change (Fig. 3). This patient is seen regularly at the outpatient department and has no complications 5 months after surgery.

Bottom Line: Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development.Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient.Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea.

ABSTRACT
Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development. Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient. Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct. The asymptomatic patient was treated with liver segmentectomy.

No MeSH data available.


Related in: MedlinePlus