DOCSLIB.ORG

An Unusual Type of Biliar Cyst: a Case Report

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
An Unusual Type of Biliar Cyst: a Case Report 788 Gidi ADG, et al. , 2016; 15 (5): 788-794 CASE REPORT September-October, Vol. 15 No. 5, 2016: 788-794 The Official Journal of the Mexican Association of Hepatology, the Latin-American Association for Study of the Liver and the Canadian Association for the Study of the Liver An unusual type of biliar cyst: A case report Alejandro D. G. Gidi,* Mario A. González-Chávez,* Eduardo Villegas-Tovar,*,, Víctor Visag-Castillo,* Juan P. Pantoja-Millan,* Francisco M. Vélez-Pérez,* Fernando Cano-García,** Alan G. Contreras* * Department of Surgery, Medica Sur Hospital, Mexico City. ** Department of Pathology, Medica Sur Hospital, Mexico City. ABSTRACT We present the case of a 56-yr-old woman with vague abdominal pain of approximately 5 months duration. An ultrasound study showed moderate dilation of the common bile duct. Magnetic resonance cholangiopancreatography confirmed a cystic dilatation of the right hepatic duct with intra and extra hepatic component. The patient underwent right hepatectomy and complete excision of the cyst. Microscopically, the cyst wall was formed by fibrous tissue with mild acute and chronic inflammatory infiltrate, the inner surface showed a single layer of columnar epithelium and extensive squamous metaplasia without atypia, wich expressed p63 and high mo- lecular weight cytoqueratin (34BE12). Key words.words.Key Choledochal cyst. Bile duct cysts. Classification. Management. INTRODUCTION There was no evidence of jaundice or palpable abdomi- nal mass on physical examination. She had mild tender- Choledochal cysts (CC) are rare medical conditions ness at the right upper quadrant but no Murphy’s sign. An and consist of cystic dilatations of the extrahepatic biliary ultrasound study showed moderate dilation of the com- tree, intrahepatic biliary ducts or both, with an incidence mon bile duct and a large cystic mass of unclear origin in in the western population of 1 in 100,000-150,000 live the liver, there was no certainty that the cyst continue births, although the incidence has been reported to be as to the intrahepatic bile tract (Figure 1). A magnetic reso- high as 1 in 13,500 births in the United States and 1 in nance cholangiopancreatography was obtained to help 15,000 births in Australia.1 The rate is remarkably higher clarify the anatomy. The magnetic resonance cholangio- in Asian populations with a reported incidence of 1 in 1000, and about two thirds of cases occur in Japan. The rea- son for this Asian preponderance is still unclear. There is also an unexplained female:male preponderance, com- monly reported as 4:1 or 3:1.2. CC are classified into five main types with several sub- types. Anatomical configurations, which do not readily fall into the Todani modification, have been described.3 CYSTCYSTCYST CASE REPORT A 56-yr-old woman presented initially complaining of vague abdominal pain of approximately 5 months duration. She denied nausea, vomiting, or weight loss, and had a normal ap- petite. She had no other medical problems, took no medica- Figure 1.1.Figure Ultrasound showing moderate dilation of the common bile duct tions regularly, and she had no previous surgical procedures. and a large cystic mass. Arrow showing gallbladder. Manuscript received: January 26, 2016. Manuscript accepted: April 06, 2016. DOI:10.5604/16652681.1212618. An unusual type of biliar cyst. , 2016; 15 (5): 788-794 789 Figure 2.2.Figure Magnetic resonance cholangiopancreatography confirmed a cys- tic dilatation of the right hepatic duct (arrow) next to the gallbladder (point of Figure 4. In the surgical exploration post-resection of the gallbladder, a arrow); the head of the pancreas appeared normal. cystic mass under the right hepatic duct was observed (arrow). tomy was performed (Figure 4). There were no intraoper- ative complications. The specimen consisted of an 18.2 cm liver and biliary duct segment; a 5.6 cm unilocular cyst was identified on the right hepatic duct, the external surface was regular and green, in the inner surface solid areas were not identified. Microscopically, the cyst wall was formed by fibrous tissue with mild acute and chronic in- flammatory infiltrate, the inner surface showed a single layer of columnar epithelium and extensive squamous metaplasia without atypia, which expressed p63 and high molecular weight cytoqueratin (34BE12). The residual in- ner parenchymal was normal (Figure 5). The patient expe- rienced a full recovery and was discharged on postoperative day 5. DISCUSSION Figure 3.3.Figure MR cholangiopancreatography show cystic dilatation of right bile CC are rare congenital cystic dilation of the biliary duct. tract, first described by Vater and Ezler in 1723.4 They present primarily in female infants and young children and pancreatography showed a cystic dilatation of the right he- are more prevalent in East Asian populations. Although be- patic duct with intra and extra hepatic component, the nign, CC can be associated with serious complications in- head of the pancreas appeared normal, as did the stomach, cluding malignant transformation, cholangitis, pancreatitis, kidney, liver, and adrenal gland. There were no other ab- and cholelithiasis.5 normalities (Figures 2 and 3). The patient underwent ex- Approximately 80% of the CC are diagnosed in infants ploratory laparotomy through a supraumbilical middle and young children within the first decade of life. The in- line incision. The gallbladder was removed and a sacular cidence of CC ranges from 1 in 100,000 to 1 in 150,000 dilatation of the right hepatic duct was observed after the individuals in Western countries and 1 in 13,000 individu- removal of the gallblader. The right bile duct was transect- als in Japan.6 CC are 4 times more common in females.1,6 ed distally, at the level of the confluence. Small duct Although the exact etiology is unknown, anomalous pan- stones were found inside the right hepatic duct. The left creaticobiliary duct union (APBDU) is seen in 30% to and common bile ducts remained intact. A right hepatec- 70% of all CC where the common bile duct (CBD) and 790 Gidi ADG, et al. , 2016; 15 (5): 788-794 AAA BBB CCC DDD Figure 5. The photomicrograph shows the fibrous cystic wall (A) wich inner sur- face is covered by columnar epithelium (B) with extensive squamous metaplasia (C) The metaplastic cells expressed p63 (D) and high molecular weight cytoque- ratin. pancreatic duct junction occurs outside the duodenum, al- patic or common bile duct; type III (also known as lowing reflux of pancreatic fluid into the biliary tree.7 choledochocele), an intraduodenal diverticulum of the It is well accepted that a CC is a premalignant state. distal common bile duct; type IV, intrahepatic and extra- The overall risk of cancer has been reported to be 10%- hepatic fusiform cysts (IVA), or multiple extrahepatic 15%, and increases with age. The risk rises from 2.3% in cysts (IVB); type V (also known as Caroli’s disease), mul- patients aged 20-30 years to 75% in patients aged 70-80 tiple intrahepatic cysts. The classification of bile duct years, and histopathology shows increasing dysplasia with cysts is important as it guides the clinical management and increasing age. Distribution of the types of cancer found may provide prognostic information.10 in patients with CC are as follows: adenocarcinoma In a recent review of the literature, the relative frequen- 73%-84%, anaplastic carcinoma 10%, undifferentiated car- cy of bile duct cysts using the Todani classification is type cinoma 5%-7%, squamous cell carcinoma 5% and other I (78%), type II (3%), type III (3%), type IV (15%), and carcinoma 1.5%.8 type V (1%). Intrahepatic variants of bile duct cysts with Bile duct cysts are classified based on location, extent isolated saccular or fusiform cysts arising from the main and shape of the cystic anomaly within the bile duct sys- lobar bile ducts near the confluence have been noted anec- tem. Currently, Todani’s modification3 of the Alonso-Lej dotally and are not classifiable by current typing schemes. classification9 is most commonly used which includes Whether these cysts are true bile duct cysts or cystadeno- both extrahepatic and intrahepatic bile duct cysts and the ma variants isn unk own.11 variants of Caroli’s disease (Figure 6). This classification CC in our patient was classified as Todani type IV, but includes five types: type I, a solitary fusiform or saccular the morphology of the cysts was unusual. The cyst only dilation of the common hepatic and common bile duct; took part of the right hepatic duct and its location was to- type II, a supraduodenal diverticulum of the common he- tally intrahepatic. Because of this, it is not in any classifi- An unusual type of biliar cyst. , 2016; 15 (5): 788-794 791 Figure 6. Todani’s classification of choledochal cysts. cation and malignant potential was unknown so a wider Abdominal pain is the most common symptom in adult resection was considered. This variant of CC has not been patients, with an incidence ranging from 78% to 90%. recognized previously and we propose the classification of Jaundice, nausea, and cholangitis are found in 40%-50% of this variant under Todani’s system, as type IVC. Surgeons adults. Cholangitis or pancreatitis is a common symptom should be aware of the possibility of variant anatomy in in adult. Dilatation of the bile ducts and stricture cause choledochal cysts, which is outside the normal classifica- bile stasis, stone or sludge formation, resulting in ascend- tion for this condition. ing cholangitis or pancreatitis.12 Clinical presentation varies with age, and 80% of the With the increased use of axial imaging, more CC are be- patients present before the age of 10 years. Children usual- ing diagnosed as incidental findings. CC diagnosis is typi- ly have signs and symptoms, but adults are asymptomatic.
Load more

Recommended publications
  • Clinical Classification of Caroli's Disease: an Analysis of 30 Patients
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector DOI:10.1111/hpb.12330 HPB ORIGINAL ARTICLE Clinical classification of Caroli's disease: an analysis of 30 patients Zhong-Xia Wang1,2*, Yong-Gang Li2*, Rui-Lin Wang2, Yong-Wu Li3, Zhi-Yan Li3, Li-Fu Wang2, Hui-Ying Yang2, Yun Zhu2, Yao Wang2, Yun-Feng Bai2, Ting-Ting He2, Xiao-Feng Zhang2 & Xiao-He Xiao1,2 1Department of Graduate School, 301 Hospital, 2Integrative Medical Centre, and 3Imaging Centre, 302 Hospital, Beijing, China Abstract Background: Caroli's disease (CD) is a rare congenital disorder. The early diagnosis of the disease and differentiation of types I and II are of extreme importance to patient survival. This study was designed to review and discuss observations in 30 patients with CD and to clarify the clinical characteristics of the disease. Methods: The demographic and clinical features, laboratory indicators, imaging findings and pathology results for 30 patients with CD were reviewed retrospectively. Results: Caroli's disease can occur at any age. The average age of onset in the study cohort was 24 years. Patients who presented with symptoms before the age of 40 years were more likely to develop type II CD. Approximately one-third of patients presented without positive signs at original diagnosis and most of these patients were found to have type I CD on pathology. Anaemia, leucopoenia and thrombocytopoenia were more frequent in patients with type II than type I CD. Magnetic resonance cholangiopancreatography (MRCP) and computed tomography (CT) examinations were most useful in diagnosing CD.
    [Show full text]
  • Biliary Tract
    2016-06-16 The role of cytology in management of diseases of hepatobiliary ducts • Diagnosis in patients with radiologically/clinically detected lesions • Screening of dysplasia/CIS/cancer in risk groups biliary tract cytology • Preoperative evaluation of the candidates for liver transplantation (Patients with cytological low-grade and high-grade Mehmet Akif Demir, MD dysplasia/adenocarcinoma are currently referred for liver transplantation Sahlgrenska University Hospital in some institutions). Gothenburg Sweden Sarajevo 18th June 2016 • Diagnosis of the benign lesions and infestations False positive findings • majority of false positive cases have a Low sensitivity but high specificity! background of primary sclerosing cholangitis. – lymphoplasmacytic sclerosing pancreatitis and cholangitis, – primary sclerosing cholangitis, – granulomatous disease, – non-specific fibrosis/inflammation – stone disease. False negative findings • Repeat brushing increases the diagnostic yield and should be performed when sampling • Poor sampling biliary strictures with a cytology brush at ERCP. • Lack of diagnostic criteria for dysplasia-carcinoma in situ • Difficulties in recognition of special tumour types – well-differentiated cholangiocarcinoma with tubular architecture • Predictors of positive yield include – gastric foveolar type cholangiocarcinoma with mucin-producing – tumour cells. older age, •Underestimating the significance of the smear background – mass size >1 cm, and – stricture length of >1 cm. •The causes of false negative cytology –sampling
    [Show full text]
  • ULTRASOUND STUDY GUIDE • Technical Knowledge O Physics And
    ULTRASOUND STUDY GUIDE Technical knowledge o Physics and Safety, understand the following: 1) Physics of sound interactions in the body. 2) How transducers work, how the image is created, and what physical properties are being displayed. 3) Relative strengths and weaknesses of different transducers including various aspects of resolution. Sound properties and interactions Reflection Attenuation Scattering Refraction Absorption Acoustic impedance Speed of sound Wavelength Other . Transducer fundamentals Transmit frequencies Transducer components Transducer types Transducer pros and cons Other . Beam formation Focusing Steering Other . Imaging modes and display 2D 3D 4D Panoramic imaging Compound imaging Harmonic imaging Elastography Contrast imaging Scanning modes o 2D o 3D o 4D o M-mode o Doppler o Other Image orientation Other . Image resolution Axial Lateral Elevational / Azimuthal Temporal Contrast Penetration vs. resolution Other . System Controls - Know the function of the controls listed below and be able to recognize them in the list of scan parameters shown on the image monitor Gain Time gain compensation Power output Focal zone Transmit frequency Depth Width Zoom / Magnification Dynamic range Frame rate Line density Frame averaging / persistence Other . Doppler / Flow imaging – Be familiar with the terminology used to describe Doppler exams. Be able to interpret and optimize the images. Be able to recognize artifacts, know their significance, and know what produces them. Doppler
    [Show full text]
  • Solitary Cystic Dilatation of the Intrahepatic Bile
    Short reports 617 Solitary cystic dilatation of the intrahepatic bile duct J Clin Pathol: first published as 10.1136/jcp.50.7.617 on 1 July 1997. Downloaded from K Ohmoto, M Shimizu, Y Iguchi, S Yamamoto, M Murakami, T Tsunoda Abstract presented with neither a pertinent family A 31 year old man was hospitalised with history nor a personal history of blood transfu- general fatigue and epigastric pain. Ab- sion, tattooing, or drug abuse. On physical dominalultrasonography, computedtomo- examination, there was a slight tenderness in graphy, and magnetic resonance imaging the epigastrium and right hypochondrium, and showed a cystic lesion in the left lobe ofthe the liver was slightly enlarged. Results of liver liver. Endoscopic retrograde cholangio- function tests were: total bilirubin, 2.1 mg/l pancreatography and percutaneous trans- (normal value 0.2-1.0); alkaline phosphate, hepatic cholangiography revealed a 127 IU/l (28-84); 7 glutamyltranspeptidase localised dilatation ofthe intrahepatic bile 314 IU/l (4-30); aspartate aminotransferase duct without any obstruction. However, a 117 IU/l (7-20); and alanine aminotransferase large mass of mucinous material was 425 IU/l (7-28). Virus markers were negative noted in the saccular intrahepatic duct for hepatitis A, B, and C. Abdominal ultra- and the common bile duct. There was no sonography, computed tomography, and mag- evidence of a choledochal cyst, anomalous netic resonance imaging showed a cystic lesion pancreaticobiliary ductal union, or con- in the left lobe of the liver. Choledochal cysts genital cystic change of the kidneys. A and anomalous pancreaticobiliary ductal union possible diagnosis ofmucinous cystic neo- were not demonstrated on endoscopic retro- plasm of the intrahepatic bile duct was grade cholangiopancreatography.
    [Show full text]
  • Department of Pathology & Laboratory Medicine
    F DEPARTMENT OF PATHOLOGY & LABORATORY MEDICINE Residency Procedure Manual Katie Dennis, MD, Residency Program Director Garth Fraga, MD and Sharad Mathur, MD, Associate Residency Program Directors Kim Ates, M.Ed., Residency/Fellowship Program Coordinator Revised October 2016 http://www.acgme.org/Portals/0/PFAssets/ProgramRequirements/300_pathology_2016.pdf 1 KUMC Pathology Residency Manual http://www.kumc.edu/Documents/gme/Web%20Ready%20Version%205.4%20%2012.2015.pdf 2 KUMC Pathology Residency Manual Table of Contents MISSION, GOALS AND PHILOSOPHY .....................................................................................................................5 RESIDENCY EDUCATIONAL PROGRAM .................................................................................................................6 PROGRAM STRUCTURE ........................................................................................................................................ 12 PGY-SPECIFIC GOALS ........................................................................................................................................... 14 GENERAL RESIDENCY GOALS ............................................................................................................................. 20 PRACTICE-BASED LEARNING AND IMPROVEMENT (PBLI) ............................................................................... 22 DIDACTIC SESSIONS AND CONFERENCES ........................................................................................................
    [Show full text]
  • Autosomal Dominant Polycystic Kidney Disease with Anticipation and Caroli's Disease Associated with a PKD1 Mutation Rapid Communication
    CORE Metadata, citation and similar papers at core.ac.uk Provided by Elsevier - Publisher Connector Kidney International, Vol. 52 (1997), pp.33—38 Autosomal dominant polycystic kidney disease with anticipation and Caroli's disease associated with a PKD1 mutation Rapid Communication ROSER Toiu&, CELIA BADENAS, ALEJANDRO DARNELL, CoNcEPcIO BRi, ANGELS ESCORSELL, and XAVIER ESTIVILL Nephrology Service, Genetics Service, Sonography Section of the Radiology Service, and Hepatology Service, Hospital ClInic, Barcelona, Spain Autosomal dominant polycystic kidney disease with anticipation and (ESRD) than PKD1 patients [14]. The PKD1 transcript consists Caroli's disease associated with a PKD1 mutation. Autosomal dominant of 14,148 bp, distributed among 46 exons, spanning 52 kb. An polycystic kidney disease (ADPKD) is the most common renal hereditary interesting feature of this gene is that all but 3.5 kb at the 3'end disorder. Clinical expression of ADPKDshowsinterfamilial and intrafa- milial variability. We screened for mutations the 3' region of the PKD1 of the transcript is encoded by a region repeated several times, gene, from exon 43 to exon 46, in a family showing anticipation and proximally in the same chromosome. Until now very few muta- Caroli's disease and have found a 28 base pairs deletion in exon 46tions [15—19] have been reported in the PKDI gene, mainly due to (12801de128) and a new DNA variant in exon 43 (12184 C to G conserving the this fact, and most of them are located in the non-repeated Ala 3991) segregating with the disease. The mutation should result in a3'region. Only one of these mutations has been reported in more protein 44aminoacids longer than the wild-type PKD1.
    [Show full text]
  • Cystic Disease of the Liver and Biliary Tract Gut: First Published As 10.1136/Gut.32.Suppl.S116 on 1 September 1991
    S116 GutSupplement, 1991, S116-S122 Cystic disease of the liver and biliary tract Gut: first published as 10.1136/gut.32.Suppl.S116 on 1 September 1991. Downloaded from A Forbes, I M Murray-Lyon Abstract ances, aspiration for microbiological and cyto- The widespread availability of ultrasound logical examination is warranted. Several reports imaging has led to more frequent recognition - (for example,5 and our own unpublished obser- of cystic disease affecting the liver and biliary vations) of needle diagnosis of unsuspected hy- tract. There is a wide range ofpossible causes. datid disease, and even therapy by ultrasound Cystic disease of infective origin is usually guided transcutaneous injection of sclerosant,67 caused by an Echinococcal species, or as the indicate that ifthe transhepatic route is taken the sequel of a treated amoebic or pyogenic risk ofmorbidity is low. abscess. The clinical and radiological features Distinction of abscess from cyst is relatively are often then distinctive and will not be dwelt simple if an abscess has viscous echo dense upon in this review, except in respect of their contents with a thick wall and densely com- contribution to the differential diagnosis of pressed surrounding hepatic parenchyma. Per- non-infective disorders. The principal non- cutaneous aspiration allows confirmation of the infective cysts can be conveniently divided diagnosis, provides material for microbiological between the simple cyst, the polycystic syn- examination, and may be of major therapeutic dromes (usually with coexistent renal disease), benefit. Positive blood cultures or amoebic Caroli's syndrome, and choledochal cysts. serology may, however, render aspiration super- The overlap between constituent members of fluous, given that small single abscesses can be these groups, and the association of cystic effectively managed with systemic antimicro- disease with hepatic fibrosis (especially with bials alone.
    [Show full text]
  • Body Systems Syllabus
    Body Systems Syllabus This syllabus defines the learning competencies, the clinical conditions and normal variants for each body system that trainees are expected to know and demonstrate proficiency in by the end of their training. The clinical conditions and normal variants are categorised into levels of knowledge as defined below. Contents • Definitions 161 ¡ Learning Competencies 162 ¡ Normal Variants 162 ¡ Condition Categories 162 • Abdominal Imaging 162 ¡ Normal Variants 165 ¡ Adult Clinical Conditions 166 • Cardiothoracic Imaging 171 ¡ Learning Competencies 171 ¡ Normal Variants 174 SYSTEMS BODY ¡ Adult Clinical Conditions 174 • Extracranial Head & Neck Imaging 178 ¡ Learning Competencies 178 ¡ Neuro/ENT imaging Normal Variants 180 ¡ Extracranial Head & Neck Imaging Clinical Conditions 181 • Neuroradiology 188 ¡ Learning Competencies 188 ¡ Adult Clinical Conditions 190 • Musculoskeletal Imaging 193 ¡ Learning Competencies 193 ¡ Normal Variants 195 ¡ Adult Clinical Conditions 196 • Paediatric Imaging 211 ¡ Learning Competencies 211 ¡ Paediatric Clinical Conditions 214 • Breast Imaging 222 ¡ Learning Competencies 222 ¡ Breast Normal Variants 225 ¡ Breast Clinical Conditions 225 • Obstetric & Gynaecological Imaging 227 ¡ Learning Competencies 227 ¡ O&G Normal Variants 229 ¡ Clinical Conditions 229 • Vascular Imaging & Interventional Radiology 236 ¡ Learning Competencies 236 ¡ VIR Normal Variants 238 ¡ Adult Clinical Conditions 239 © 2014 RANZCR. Radiodiagnosis Training Program – Curriculum Version 2.2 Page 161 Learning Competencies
    [Show full text]
  • Pediatric Pathology Major Category Code Headings 1 Perinatal
    updated 8/20/2021 Pediatric Pathology Page 1 of 25 Pediatric Pathology Major Category Code Headings Revised 8/17/2021 1 Perinatal Pathology: Placental-maternal-fetal relationships in pregnancy 70000 2 Perinatal Pathology: Fetal/Neonatal pathophysiology 70445 3 General Pathologic Principles and Syndromes, NOS 70645 4 Cardiovascular System, NOS 70815 5 Respiratory System and Mediastinum, NOS 71050 6 Central Nervous System, NOS 71255 7 Skin, NOS 71455 8 Special Senses – Eye and Ear 71680 9 Alimentary Tract, NOS 71800 10 Hepatobiliary System and Pancreas, NOS 72225 11 Kidney and Urinary System, NOS 72585 12 Endocrine system, excluding ovary and testis, NOS 72825 Hematopoietic system, including bone marrow, lymph nodes, thymus, spleen 13 and other lymphoid tissues 72945 14 Breast, NOS 73220 15 Female reproductive system, NOS 73275 16 Disorders of sexual development (Intersex disorders), NOS 73445 17 Male reproductive system, NOS 73530 18 Soft tissue, peripheral nerve and muscle, NOS 73690 19 Skeletal system, NOS 74005 20 Diagnostic/Technical Procedures, Laboratory Management 74120 21 Admin. & Management, LIS, QA, Lab Planning, Regulations & Safety 74775 22 Forensic Pathology, NOS 74850 Pediatric Pathology Page 2 of 25 Pediatric Pathology 1 Perinatal Pathology: Placental-maternal-fetal relationships in pregnancy 70000 A Conception 70005 1 Gametogenesis 70010 2 Fertilization 70015 3 Implantation 70020 B Normal embryonic and fetal development, NOS 70025 1 Embryologic processes 70030 2 Normal histology of fetal organs 70035 C Pregnancy physiology
    [Show full text]
  • Ultrasound Findings in Peliosis Hepatis
    Ultrasound findings in peliosis hepatis Yi Dong1, Wen-Ping Wang1, Adrian Lim2, Won Jae Lee3,4, Dirk-Andre Clevert5, Michael Höpfner6, Andrea Tannapfel7, Christoph Frank Dietrich8 1Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China; 2Department of Imaging, Imperial College London and Healthcare NHS Trust, Charing Cross Hospital Campus, London, UK; 3Department of Radiology and Center for Imaging Science, ORIGINAL ARTICLE Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul; 4Department https://doi.org/10.14366/usg.20162 of Health Science and Technology and Medical Device Management and Research, Samsung pISSN: 2288-5919 • eISSN: 2288-5943 Advanced Institute for Health Science and Technology, Sungkyunkwan University, Seoul, Ultrasonography 2021;40:546-554 Korea; 5Interdisciplinary Ultrasound-Center, Department of Radiology, University of Munich- Grosshadern Campus, Munich; 6Department Gastroenterologie, Klinik für Innere Medizin, Agaplesion Diakonie Kliniken Kassel, Kassel; 7Institut für Pathologie, Ruhr-Universität, Bochum, Germany; 8Department Allgemeine Innere Medizin (DAIM), Kliniken Beau Site, Received: October 10, 2020 Salem und Permanence, Hirslanden, Bern, Switzerland Revised: February 20, 2021 Accepted: February 22, 2021 Correspondence to: Christoph Frank Dietrich, MD, PhD, MBA, Department Allgemeine Innere Purpose: The aim of this study was to retrospectively evaluate contrast-enhanced ultrasound Medizin (DAIM), Kliniken Beau Site, (CEUS) findings in patients with peliosis hepatis (PH). Salem und Permanence, Hirslanden, 3036 Bern, Switzerland Methods: A retrospective analysis was conducted of CEUS features in 24 patients with Tel. +41-79-834-7180 histopathologically confirmed PH (11 men and 13 women; mean age, 32.4±7.1 years; range, Fax. +41-31-337-6000 28 to 41 years). All lesions were histologically proven, either by core needle biopsy (n=10) or by E-mail: [email protected] hepatic surgery (n=14).
    [Show full text]
  • Congenital Cholestatic Syndromes: What Happens When Children Grow Up?
    10094_Ling.qxd 26/10/2007 11:48 AM Page 743 REVIEW Congenital cholestatic syndromes: What happens when children grow up? Simon C Ling MB ChB SC Ling. Congenital cholestatic syndromes: What happens Les syndromes cholostatiques congénitaux : when children grow up? Can J Gastroenterol 2007; Que se passe-t-il lorsque l’enfant grandit ? 21(11):743-751. Bien que les progrès dans la prise en charge des enfants atteints d’une Although advances in the management of children with congenital cholestase congénitale aient permis à bon nombre d’entre eux de survivre cholestasis have enabled many to survive into adulthood with their jusqu’à l’âge adulte avec leur foie d’origine, ces pathologies, même les plus native livers, even the most common of these conditions remains rare courantes, demeurent rares en hépathologie pour adultes. Parmi les quatre in adult hepatology practice. Among four congenital cholestatic syn- syndromes cholostatiques congénitaux (atrésie des voies biliaires, syn- dromes (biliary atresia, Alagille syndrome, Caroli disease and con- drome d’alagille, maladie de Caroli et fibrose hépatique congéniale, genital hepatic fibrosis, and progressive familial intrahepatic cholestase intrahépatique héréditaire évolutive), les données publiées sur cholestasis), the published data on outcomes of the syndromes into l’issue des syndromes à l’âge adulte laissent prévoir tout un spectre de adulthood suggest that a spectrum of severity of liver disease can be gravité de maladie hépatique, en passant par la cirrhose (presque uni- expected, from cirrhosis (almost universal in adults with biliary atre- verselle chez les adultes atteints d’atrésie des voies biliaires qui n’ont pas sia who have not required liver transplantation) to mild and subclin- subi de greffe hépatique) jusqu’à une maladie légère et subclinique (p.
    [Show full text]
  • Jaundice in Infants and Children
    431 ULTRASOUND CLINICS Ultrasound Clin 1 (2006) 431–441 Jaundice in Infants and Children Marilyn J. Siegel, MD - Sonographic examination Spontaneous perforation of the - Overview of cholestatic diseases extrahepatic bile ducts - Neonatal jaundice Inspissated bile syndrome Biliary atresia - Jaundice in older infants and children Neonatal hepatitis syndrome Caroli disease Additional imaging studies to Byler disease differentiate atresia and hepatitis Hepatocellular diseases Alagille syndrome Inflammatory diseases of the biliary ducts Choledochal cyst Biliary tract obstruction - References Real-time sonography remains the screening echogenicity of the normal liver is low to medium study of choice for the evaluation of jaundice in and homogeneous, and the central portal venous infants and children and it is an important tool in vasculature is easily seen (Fig. 1). In the neonate differentiating between obstructive and nonob- and young infant, the hepatic parenchyma and re- structive causes of jaundice [1,2]. The causes of nal cortex are equally echogenic. In individuals 6 cholestasis are multiple, but the three major causes months of age and older, the liver usually is more are hepatitis, biliary atresia, and choledochal cyst. echogenic than the kidney. The patency and flow Other causes include neoplastic processes, cirrhosis, direction of the hepatic vessels should be do- and strictures. cumented with pulsed and color Doppler interroga- This article reviews the common congenital and tion. The liver and adjacent area should also be acquired causes of jaundice in the pediatric patient evaluated for evidence of end-stage liver disease, in- and describes the sonographic findings associated cluding collateral channels (varices), hepatofugal with these conditions. The role of correlative imag- flow, and ascites.
    [Show full text]