- Email: [email protected]
Nodular regenerative hyperplasia of the liver, coeliac disease and Sjogren's syndrome in a child
Gastroentérologie Clinique et Biologique (2010) 34, 40—44
LETTERS TO THE EDITOR
Nodular regenerative hyperplasia of the liver, coeliac disease and Sjogren’s syndrome in a child Hyperplasie nodulaire régénérative du foie, maladie cœliaque et syndrome de Sjögren chez un enfant Introduction Nodular regenerative hyperplasia is an uncommon cause of portal hypertension. Microscopic features are characterized by diffuse micronodular transformation of the hepatic parenchyma without fibrous septa between the regenerative nodules [1]. The pathogenesis of nodular regenerative hyperplasia is not well known. It has been found to be associated with variety of conditions, such as collagen disorders, Felty’s syndrome, congestive heart failure, hematologic abnormalities, especially myeloproliferative disorders, metabolic diseases, neoplasms and drugs [2]. We report the case of a 10-year-old girl who presented with nodular regenerative hyperplasia with celiac disease and dry eye syndrome.
Case report A 10-year-old girl was admitted to manage anemia with recurrent abdominal pain. She had been born to a nonconsanguinous marriage. There was no history of jaundice or chronic family liver diseases. On physical examination, the girl was pale and icteric. She was shorter than average (— 2 SD) with hepatosplenomegaly. There was no ascites, oedema or melena. Examination of other systems was normal. Laboratory values were as follows: • complete blood count and peripheral blood smear showed anemia; • leucocytopenia and thrombocytopenia (hemoglobin = 4 g/dL, leucocytes count = 2700/mm3 , platelets = 53,000/ mm3 , mean corpuscular volume = 68 qm3 , reticulocyte count = 50,000/mm3 );
• • • • •
aspartate aminotransferase (AST) = 136 IU/L; alanine aminotransferase (ALT) = 200 IU/L; total bilirubin = 410 mol/L; direct bilirubin = 374 mol/L; prothrombin level = 87%.
Hemoglobin electrophoresis was normal, direct Coombs test was negative. Bone marrow biopsy was normocellular. Abdominal ultrasound revealed dysmorphic liver with atrophic right liver and hypertrophic left liver; the parenchyma was heterogeneous. There was also portal hypertension which was characterized by splenomegaly and collateral vascular formation. The hepatic veins and the portal vein were permeable. Upper gastrointestinal endoscopy showed grade 2 oesophageal varices with red signs and the patient was treated with beta-blockers (avlocardyl). Jaundice disappeared in two weeks and liver disturbances normalized rapidly within few weeks: AST = 43 IU/L, ALT = 27 IU/L, total bilirubin = 8 mol/L, gamma-glytamyl transpeptidase = 23 IU/L, alkaline phosphatase = 317 IU/L. Investigations for chronic liver diseases which might be associated with portal hypertension were performed: viral markers HBs antigen, antiHBs, anti-hepatitis C virus (HCV) antibodies and HCV-RNA were negative; antimitochondrial and antismooth muscle antibodies were negative. Alpha 1 antityripsin level was normal. Serum seruloplasmin levels and urinary excretion of copper in 24 hours were also normal. D penicillamine test was normal. Ophtalmological examination showed punctate keratitis; the patient complained of dry eyes and the Schirmer test identified a dry eye syndrome. Serum cortisol and ACTH levels were normal. Rheumatoid factor, antinuclear antibodies, IgG and IgM anticardiolipin antibodies and scleroderma (Scl-70) antibodies were negative. Because of the patient’s short stature, liver involvement and anemia, a celiac serology was performed and showed positive antigliadin, antiendomysium and antitissue transglutaminase antibodies. Duodenal biopsy demonstrated complete villous atrophy compatible with coeliac disease and the patient was prescribed a gluten-free diet. Finally, liver biopsy was performed at three years of follow-up and there were no signs of cirrhosis, a preserved liver architecture, a portal tract with normal appearance and size and thickening of liver-cell plates with two or three hepatocytes layers. These features
0399-8320/$ – see front matter © 2009 Elsevier Masson SAS. All rights reserved.
Letters to the editor were compatible with the diagnosis of nodular regenerative hyperplasia. At the seven-year follow-up, the patient had normal functional liver tests, no gastrointestinal bleeding but she still had portal hypertension and grade 2 oesophageal varices and her haemoglobin level was 10 g/dL.
Discussion Nodular regenerative hyperplasia is rare and generally appears in association with many other diseases [1]. It has been reported to occur with the same frequency in both sexes and can be observed in all ages and races. However, pediatric onset as in our case, is unusual. The disease can be described as broad hepatocellular nodule formation without fibrous septa between the nodules [1]. Nodular regenerative hyperplasia of the liver is a rare condition which usually (in 59% of case reports in the literature [3]) presents with stigmata of portal hypertension and its sequellae. Our patient had portal hypertension related to nodular regenerative hyperplasia. She presented with jaundice and abnormal functional liver tests, which disappeared rapidly after starting a gluten-free diet. She had pancytopenia due to hypersplenim and anemia due to several factors: celiac disease, hypersplenism and occult bleeding. Clinical and radiological signs of nodular regenerative hyperplasia are not very specific, and pathologic confirmation via needle biopsy or surgery is necessary to establish the diagnosis. In our case and at the initial presentation, the diagnostic of cirrhosis related to viral or autoimmune hepatitis or Wilson disease, seemed plausible. However, no signs of cirrhosis were found on liver needle biopsy. Systemic diseases (rheumatoid arthritis, Felty’s syndrome, progressive systemic sclerosis, systemic lupus erythematosus, polymyalgia rheumatica, polyarteritis nodosa), hematological diseases (myeloproliferative disorders, lymphoproliferative disorders, idiopathic thrombocytopenic purpura), glomerulonephritis, metabolic diseases, endocrine disorders (lymphocytic thyroiditis, diabetes mellitus) and lymphomas may occur alongside nodular regenerative hyperplasia [2—4]. The association of celiac disease or Sjögren’s syndrome with nodular regenerative hyperplasia has rarely been described. Our patient had coeliac disease and Sjögren’s syndrome associated with nodular regenerative hyperplasia. To our knowledge, this association has never been reported in adults or pediatric patients. The etiology of nodular regenerative hyperplasia is not fully understood and there are many theories explaining its pathogenesis. The critical lesion is obliterative portal venopathy, presenting with obstruction of terminal radicals of hepatic arterioles, and portal venules [5]. The resultant hepatic ischemia may result in nodular regenerative changes [3]. Nodular regenerative hyperplasia is thought to be a secondary and nonspecific tissue adaptation to heterogeneous distribution of blood flow and not a specific entity. The vascular hypothesis postulates that the basic pathologic lesion leading to nodular regenerative is obliteration or thrombus in the portal venous system leading to ischemic atrophy in the most vulnerable central areas [3]. There is support of the mechanism of hepatic obliteration angiopathy in nodular regenerative hyperplasia because many of its associated conditions later are known to cause vasculitis or nonvasculitis thrombosis [6]. Austin et al. described two
41 cases of nodular regenerative hyperplasia in individuals with adult coeliac disease and IgA anticardiolipin antibodies [7]. They suggested that T cell help from gluten specific T cells was responsible for driving the IgA autoantibody response to both transglutaminase and protein/phospholipid complexes, leading to the formation of IgA anticardiolipin. IgA anticardiolipin then trigger thrombosis in small portal vein radicles, which drain the inflamed small intestine, leading to liver injury with consequent hyperplasia of the surrounding tissue [7]. An alternative mechanism for the pathogenesis of nodular regenerative hyperplasia is that it is a proliferative disorder of the liver. A possible pathogenicity as a premalignant condition which progresses to hepatocyte dysplasia and hepatocellular carcinoma has also been attributed to nodular regenerative hyperplasia based on stronger association between nodular regenerative hyperplasia and dysplasia [6]. In addition, based on the study of CD8+ T-lymphocytes concentrations in liver biopsy, it has been suggested that some nodular regenerative hyperplasia cases associated or not with autoimmune or systemic diseases may result in chronic cytotoxic CD8+ T lymphocytes targeting the sinusoidal endothelial cells [7]. This hypothesis is likely in our patient who presented with two autoimmune diseases, celiac and Sjörgen’s syndrome in association with nodular regenerative hyperplasia; the absence of lymphocyte infiltration in the patient’s liver biopsy does not disprove this hypothesis. As with other forms of noncirrhotic hypertension, the prognosis is usually better than in patients with portal hypertension due to cirrhosis. Portal diversion helps relieve the symptoms of portal hypertension in patients [1], while liver transplantation is another alterative treatment for end stage nodular regenerative hyperplasia. After seven years of follow-up, our patient had normal functional liver tests and no upper GI bleeding. No other autoimmune manifestations were present.
Conflicts of interest None.
References [1] Dachman AH, Ros PR, Goodman ZD, Olmsted WW, Ishak KG. Nodular regenerative hyperplasia of the liver: clinical and radiologic observations. AJR Am J Roentgenol 1987;148:717—22. [2] Reshamwala PA, Kleiner DE, Heller T. Nodular regenerative hyperplasia: not all nodules are created equal. Hepatology 2006;44:7—14. [3] Wanless IR. Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2,500 autopsies and a new classification of benign hepatocellular nodules. Hepatology 1990;11:787—97. [4] Moran CA, Mullick FG, Ishak KG. Nodular regenerative hyperplasia of the liver in children. Am J Surg Pathol 1991;15:449—54. [5] Naber AH, Van Haelst U, Yap H. Nodular regenerative hyperplasia of the liver: an important cause of portal hypertension in noncirrhotic patients. J Hepatol 1991;12:94—9. [6] Colina F, Alberti N, Solis JA, Martinez-Tello FJ. Diffuse nodular regenerative hyperplasia of the liver (DNRH). A clinicopathologic study of 24 cases. Liver 1989;9:253—65.
42
Letters to the editor
[7] Austin A, Campbell E, Lane P, Elias E. Nodular regenerative hyperplasia of the liver and celiac disease: potential role of IgA anticardiolipin antibody. Gut 2004;53:1032—4.
F. Tinsa a,∗ I. Brini a A. El May b D. Bousnina a K. Boussetta a S. Bousnina a a Department of Pediatrics B of the Children’s Hospital of Tunis, boulevard 9-avril, Jabbary, 1007 Bab Saadoun, Tunisia b Department of Pathology, Salah Azaiez Institute, Tunis, Tunisia ∗
Corresponding author. E-mail address: [email protected] (F. Tinsa). Available online 17 September 2009 doi:10.1016/j.gcb.2009.07.035
Kyste du canal cystique : le type VI de la classification de Todani modifiée existe-t-il ? Cystic duct cyst: What about Todani’s VI? Introduction
Figure 1 Cholangiographie par résonance magnétique en coupes coronales épaisses pondérées T2 TE long. Formation liquidienne périvésiculaire semblant se raccorder au cholédoque. Kyste biliaire hépatique gauche.
n’existait pas d’anomalie des voies biliaires et les kystes biliaires et l’angiome hépatique étaient visualisés. La tomodensitométrie abdominale (en contraste spontané puis après injection de produit de contraste iodé, aux temps arté-
Les kystes des voies biliaires extrahépatiques sont rares, environ 1/200 000, avec une fréquence multipliée par 150 au Japon. La population est à nette prédominance féminine, et la découverte se fait à plus de 60 % durant l’enfance, à partir d’une symptomatologie digestive. Ces kystes ont été décrits par Vater dès 1723 et leur classification principale, dite de Todani, a été proposée complétant celle décrite en 1959 par Alonso. Elle a ensuite été modifiée en ajoutant notamment le type VI en 1991, pour les kystes du canal cystique. Nous rapportons le quatrième cas de la littérature.
Observation Une femme âgée de 77 ans se plaignait de douleurs abdominales épigastriques survenant uniquement vers deux heures du matin. Cette symptomatologie évoluait depuis environ 15 jours, mais était aussi apparue une dizaine d’années auparavant. En 1997, une échographie hépatobiliaire avait montré une vésicule collabée, sans autre anomalie significative. L’examen clinique, le bilan biologique et la fibroscopie œsogastroduodénale étaient normaux. L’échographie montrait une vésicule en réplétion incomplète malgré le jeûne et une image mixte, liquidienne et tissulaire, de 20 × 19 mm, contiguë. Les voies biliaires intra et extrahépatiques étaient fines et il existait plusieurs kystes biliaires simples et un angiome hépatique. La cholangiographie par résonance magnétique montrait une vésicule difficile à identifier, contiguë à une pseudocollection en franc hypersignal T2 liquidien (Fig. 1). Il
Figure 2 Coupe tomodensitométrique axiale après injection de produit de contraste iodé. Formation liquidienne extravésiculaire et extraduodénale. Respect du calibre de la voie biliaire principale. : vésicule biliaire ; : voie biliaire principale ; : kyste.
LETTERS TO THE EDITOR
Nodular regenerative hyperplasia of the liver, coeliac disease and Sjogren’s syndrome in a child Hyperplasie nodulaire régénérative du foie, maladie cœliaque et syndrome de Sjögren chez un enfant Introduction Nodular regenerative hyperplasia is an uncommon cause of portal hypertension. Microscopic features are characterized by diffuse micronodular transformation of the hepatic parenchyma without fibrous septa between the regenerative nodules [1]. The pathogenesis of nodular regenerative hyperplasia is not well known. It has been found to be associated with variety of conditions, such as collagen disorders, Felty’s syndrome, congestive heart failure, hematologic abnormalities, especially myeloproliferative disorders, metabolic diseases, neoplasms and drugs [2]. We report the case of a 10-year-old girl who presented with nodular regenerative hyperplasia with celiac disease and dry eye syndrome.
Case report A 10-year-old girl was admitted to manage anemia with recurrent abdominal pain. She had been born to a nonconsanguinous marriage. There was no history of jaundice or chronic family liver diseases. On physical examination, the girl was pale and icteric. She was shorter than average (— 2 SD) with hepatosplenomegaly. There was no ascites, oedema or melena. Examination of other systems was normal. Laboratory values were as follows: • complete blood count and peripheral blood smear showed anemia; • leucocytopenia and thrombocytopenia (hemoglobin = 4 g/dL, leucocytes count = 2700/mm3 , platelets = 53,000/ mm3 , mean corpuscular volume = 68 qm3 , reticulocyte count = 50,000/mm3 );
• • • • •
aspartate aminotransferase (AST) = 136 IU/L; alanine aminotransferase (ALT) = 200 IU/L; total bilirubin = 410 mol/L; direct bilirubin = 374 mol/L; prothrombin level = 87%.
Hemoglobin electrophoresis was normal, direct Coombs test was negative. Bone marrow biopsy was normocellular. Abdominal ultrasound revealed dysmorphic liver with atrophic right liver and hypertrophic left liver; the parenchyma was heterogeneous. There was also portal hypertension which was characterized by splenomegaly and collateral vascular formation. The hepatic veins and the portal vein were permeable. Upper gastrointestinal endoscopy showed grade 2 oesophageal varices with red signs and the patient was treated with beta-blockers (avlocardyl). Jaundice disappeared in two weeks and liver disturbances normalized rapidly within few weeks: AST = 43 IU/L, ALT = 27 IU/L, total bilirubin = 8 mol/L, gamma-glytamyl transpeptidase = 23 IU/L, alkaline phosphatase = 317 IU/L. Investigations for chronic liver diseases which might be associated with portal hypertension were performed: viral markers HBs antigen, antiHBs, anti-hepatitis C virus (HCV) antibodies and HCV-RNA were negative; antimitochondrial and antismooth muscle antibodies were negative. Alpha 1 antityripsin level was normal. Serum seruloplasmin levels and urinary excretion of copper in 24 hours were also normal. D penicillamine test was normal. Ophtalmological examination showed punctate keratitis; the patient complained of dry eyes and the Schirmer test identified a dry eye syndrome. Serum cortisol and ACTH levels were normal. Rheumatoid factor, antinuclear antibodies, IgG and IgM anticardiolipin antibodies and scleroderma (Scl-70) antibodies were negative. Because of the patient’s short stature, liver involvement and anemia, a celiac serology was performed and showed positive antigliadin, antiendomysium and antitissue transglutaminase antibodies. Duodenal biopsy demonstrated complete villous atrophy compatible with coeliac disease and the patient was prescribed a gluten-free diet. Finally, liver biopsy was performed at three years of follow-up and there were no signs of cirrhosis, a preserved liver architecture, a portal tract with normal appearance and size and thickening of liver-cell plates with two or three hepatocytes layers. These features
0399-8320/$ – see front matter © 2009 Elsevier Masson SAS. All rights reserved.
Letters to the editor were compatible with the diagnosis of nodular regenerative hyperplasia. At the seven-year follow-up, the patient had normal functional liver tests, no gastrointestinal bleeding but she still had portal hypertension and grade 2 oesophageal varices and her haemoglobin level was 10 g/dL.
Discussion Nodular regenerative hyperplasia is rare and generally appears in association with many other diseases [1]. It has been reported to occur with the same frequency in both sexes and can be observed in all ages and races. However, pediatric onset as in our case, is unusual. The disease can be described as broad hepatocellular nodule formation without fibrous septa between the nodules [1]. Nodular regenerative hyperplasia of the liver is a rare condition which usually (in 59% of case reports in the literature [3]) presents with stigmata of portal hypertension and its sequellae. Our patient had portal hypertension related to nodular regenerative hyperplasia. She presented with jaundice and abnormal functional liver tests, which disappeared rapidly after starting a gluten-free diet. She had pancytopenia due to hypersplenim and anemia due to several factors: celiac disease, hypersplenism and occult bleeding. Clinical and radiological signs of nodular regenerative hyperplasia are not very specific, and pathologic confirmation via needle biopsy or surgery is necessary to establish the diagnosis. In our case and at the initial presentation, the diagnostic of cirrhosis related to viral or autoimmune hepatitis or Wilson disease, seemed plausible. However, no signs of cirrhosis were found on liver needle biopsy. Systemic diseases (rheumatoid arthritis, Felty’s syndrome, progressive systemic sclerosis, systemic lupus erythematosus, polymyalgia rheumatica, polyarteritis nodosa), hematological diseases (myeloproliferative disorders, lymphoproliferative disorders, idiopathic thrombocytopenic purpura), glomerulonephritis, metabolic diseases, endocrine disorders (lymphocytic thyroiditis, diabetes mellitus) and lymphomas may occur alongside nodular regenerative hyperplasia [2—4]. The association of celiac disease or Sjögren’s syndrome with nodular regenerative hyperplasia has rarely been described. Our patient had coeliac disease and Sjögren’s syndrome associated with nodular regenerative hyperplasia. To our knowledge, this association has never been reported in adults or pediatric patients. The etiology of nodular regenerative hyperplasia is not fully understood and there are many theories explaining its pathogenesis. The critical lesion is obliterative portal venopathy, presenting with obstruction of terminal radicals of hepatic arterioles, and portal venules [5]. The resultant hepatic ischemia may result in nodular regenerative changes [3]. Nodular regenerative hyperplasia is thought to be a secondary and nonspecific tissue adaptation to heterogeneous distribution of blood flow and not a specific entity. The vascular hypothesis postulates that the basic pathologic lesion leading to nodular regenerative is obliteration or thrombus in the portal venous system leading to ischemic atrophy in the most vulnerable central areas [3]. There is support of the mechanism of hepatic obliteration angiopathy in nodular regenerative hyperplasia because many of its associated conditions later are known to cause vasculitis or nonvasculitis thrombosis [6]. Austin et al. described two
41 cases of nodular regenerative hyperplasia in individuals with adult coeliac disease and IgA anticardiolipin antibodies [7]. They suggested that T cell help from gluten specific T cells was responsible for driving the IgA autoantibody response to both transglutaminase and protein/phospholipid complexes, leading to the formation of IgA anticardiolipin. IgA anticardiolipin then trigger thrombosis in small portal vein radicles, which drain the inflamed small intestine, leading to liver injury with consequent hyperplasia of the surrounding tissue [7]. An alternative mechanism for the pathogenesis of nodular regenerative hyperplasia is that it is a proliferative disorder of the liver. A possible pathogenicity as a premalignant condition which progresses to hepatocyte dysplasia and hepatocellular carcinoma has also been attributed to nodular regenerative hyperplasia based on stronger association between nodular regenerative hyperplasia and dysplasia [6]. In addition, based on the study of CD8+ T-lymphocytes concentrations in liver biopsy, it has been suggested that some nodular regenerative hyperplasia cases associated or not with autoimmune or systemic diseases may result in chronic cytotoxic CD8+ T lymphocytes targeting the sinusoidal endothelial cells [7]. This hypothesis is likely in our patient who presented with two autoimmune diseases, celiac and Sjörgen’s syndrome in association with nodular regenerative hyperplasia; the absence of lymphocyte infiltration in the patient’s liver biopsy does not disprove this hypothesis. As with other forms of noncirrhotic hypertension, the prognosis is usually better than in patients with portal hypertension due to cirrhosis. Portal diversion helps relieve the symptoms of portal hypertension in patients [1], while liver transplantation is another alterative treatment for end stage nodular regenerative hyperplasia. After seven years of follow-up, our patient had normal functional liver tests and no upper GI bleeding. No other autoimmune manifestations were present.
Conflicts of interest None.
References [1] Dachman AH, Ros PR, Goodman ZD, Olmsted WW, Ishak KG. Nodular regenerative hyperplasia of the liver: clinical and radiologic observations. AJR Am J Roentgenol 1987;148:717—22. [2] Reshamwala PA, Kleiner DE, Heller T. Nodular regenerative hyperplasia: not all nodules are created equal. Hepatology 2006;44:7—14. [3] Wanless IR. Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2,500 autopsies and a new classification of benign hepatocellular nodules. Hepatology 1990;11:787—97. [4] Moran CA, Mullick FG, Ishak KG. Nodular regenerative hyperplasia of the liver in children. Am J Surg Pathol 1991;15:449—54. [5] Naber AH, Van Haelst U, Yap H. Nodular regenerative hyperplasia of the liver: an important cause of portal hypertension in noncirrhotic patients. J Hepatol 1991;12:94—9. [6] Colina F, Alberti N, Solis JA, Martinez-Tello FJ. Diffuse nodular regenerative hyperplasia of the liver (DNRH). A clinicopathologic study of 24 cases. Liver 1989;9:253—65.
42
Letters to the editor
[7] Austin A, Campbell E, Lane P, Elias E. Nodular regenerative hyperplasia of the liver and celiac disease: potential role of IgA anticardiolipin antibody. Gut 2004;53:1032—4.
F. Tinsa a,∗ I. Brini a A. El May b D. Bousnina a K. Boussetta a S. Bousnina a a Department of Pediatrics B of the Children’s Hospital of Tunis, boulevard 9-avril, Jabbary, 1007 Bab Saadoun, Tunisia b Department of Pathology, Salah Azaiez Institute, Tunis, Tunisia ∗
Corresponding author. E-mail address: [email protected] (F. Tinsa). Available online 17 September 2009 doi:10.1016/j.gcb.2009.07.035
Kyste du canal cystique : le type VI de la classification de Todani modifiée existe-t-il ? Cystic duct cyst: What about Todani’s VI? Introduction
Figure 1 Cholangiographie par résonance magnétique en coupes coronales épaisses pondérées T2 TE long. Formation liquidienne périvésiculaire semblant se raccorder au cholédoque. Kyste biliaire hépatique gauche.
n’existait pas d’anomalie des voies biliaires et les kystes biliaires et l’angiome hépatique étaient visualisés. La tomodensitométrie abdominale (en contraste spontané puis après injection de produit de contraste iodé, aux temps arté-
Les kystes des voies biliaires extrahépatiques sont rares, environ 1/200 000, avec une fréquence multipliée par 150 au Japon. La population est à nette prédominance féminine, et la découverte se fait à plus de 60 % durant l’enfance, à partir d’une symptomatologie digestive. Ces kystes ont été décrits par Vater dès 1723 et leur classification principale, dite de Todani, a été proposée complétant celle décrite en 1959 par Alonso. Elle a ensuite été modifiée en ajoutant notamment le type VI en 1991, pour les kystes du canal cystique. Nous rapportons le quatrième cas de la littérature.
Observation Une femme âgée de 77 ans se plaignait de douleurs abdominales épigastriques survenant uniquement vers deux heures du matin. Cette symptomatologie évoluait depuis environ 15 jours, mais était aussi apparue une dizaine d’années auparavant. En 1997, une échographie hépatobiliaire avait montré une vésicule collabée, sans autre anomalie significative. L’examen clinique, le bilan biologique et la fibroscopie œsogastroduodénale étaient normaux. L’échographie montrait une vésicule en réplétion incomplète malgré le jeûne et une image mixte, liquidienne et tissulaire, de 20 × 19 mm, contiguë. Les voies biliaires intra et extrahépatiques étaient fines et il existait plusieurs kystes biliaires simples et un angiome hépatique. La cholangiographie par résonance magnétique montrait une vésicule difficile à identifier, contiguë à une pseudocollection en franc hypersignal T2 liquidien (Fig. 1). Il
Figure 2 Coupe tomodensitométrique axiale après injection de produit de contraste iodé. Formation liquidienne extravésiculaire et extraduodénale. Respect du calibre de la voie biliaire principale. : vésicule biliaire ; : voie biliaire principale ; : kyste.