|Year : 2021 | Volume
| Issue : 1 | Page : 24-26
Portal biliopathy in a Nigerian: A pictorial case report of a rare finding
Aderemi O Oluyemi1, Sumedh K Waghmare2, Raymond A Kuti3
1 ReMay Consultancy and Medical Services, Ikeja, Nigeria
2 Aster Imaging Center, Deira, Dubai, UAE
3 Prism Diagnostics, Surulere, Lagos, Nigeria
|Date of Submission||05-May-2020|
|Date of Acceptance||18-Sep-2020|
|Date of Web Publication||22-Apr-2021|
Dr. Aderemi O Oluyemi
ReMay Consultancy and Medical Services, Ikeja, Lagos State
Portal biliopathy (PB) was well delineated as a distinct clinical entity in the 1990s. Since then, science has much improved in diagnosing and managing this condition. Currently, magnetic resonance imaging and cholangiopancreatography have largely replaced endoscopic retrograde cholangiopancreatography as the most important tool in PB diagnosis. Our resource-limited environment has not allowed for optimal availability and uptake of this tool in general but when it is deployed, as in this index case, it can yield great and accurate dividend. This report is the first such scientific documentation of PB in a Nigerian adult. It seeks to show the striking radiological image appearances upon which diagnosis is based. We hope that the article will help increase the index of suspicion for such a rare diagnosis in our locality and that it will encourage the more widespread deployment of such essential and beneficial radiological tools in disease diagnosis.
Keywords: Magnetic resonance imaging., Nigeria, portal biliopathy, portal cavernoma
|How to cite this article:|
Oluyemi AO, Waghmare SK, Kuti RA. Portal biliopathy in a Nigerian: A pictorial case report of a rare finding. West Afr J Radiol 2021;28:24-6
| Introduction|| |
The term “Portal Biliopathy” (PB) was coined by Sarin et al. it has since stuck as the official description of this new clinical entity first brought to scientific attention in the early 1990s. It is defined as abnormalities of the extrahepatic and intrahepatic bile ducts, gallbladder, and cystic duct, observed in patients with portal cavernomatous (PvC), most commonly as a result of extrahepatic portal venous obstruction (EHPVO)., This distinction is important as there are biliary changes that are observed with idiopathic portal hypertension as well. Though they appear similar, there is a general, there is a general consensus that the term PB be limited to changes associated with the consequences of PvC formation. The use of radiological diagnostic tools (magnetic resonance imaging and cholangiopancreatography, MRI/MRCP) is crucial in this disease as the majority of patients have no significant symptomatic presentation and the heterogeneous clinical presentation of affected individuals can be misleading (as it does have several important mimics.), thus contributing to delayed diagnosis and consequent morbidity and mortality., Very rarely does scientific literature contain any documented cases from black Africa and this case represents a first from our environment. The power of radiological diagnostic tools is highlighted as well as is the desire of the authors to see greater availability, adoption, and deployment of MRI and MRCP in our locality.
| Case Report|| |
A 60-year-old female who was referred for gastroscopy in a Lagos, Nigeria-based private endoscopy center by her general practitioner. She had an 8-month long history of dull but intermittent abdominal pain centered in the epigastrium. She had been prescribed various proton pump inhibitors and had several courses of anti-Helicobacter pylori medications though her stool antigen and breath test screens had been negative. An additional symptom of feeling of a lump in the left side of the abdomen and easy satiety had prompted the GP to send her for the procedure. The finding of esophageal varices (grade 1) was considered interesting and a call was put through for her GP to consider a detailed probing into the status of the liver. Her liver function tests showed mild elevation of the alkaline phosphatase (about 1.5 times the upper limit of normal) and conjugated hyperbilirubinemia but gamma-glutamyl transferase was not assayed for. An abdominal ultrasound scan (without Doppler) was carried out. This showed an enlarged spleen and liver with vascular abnormalities in the porta hepatis. A subsequent MRI/MRCP was then carried out. This confirmed the splenomegaly, showed an absence of liver cirrhosis and the presence of EHPVO (as seen with the blockage of the portal vein and its subsequent replacement by portal cavenomatous (PvC) transformation). PvC was clearly demonstrated with the presence of dilated and tortuous collateral venous vessels in the periportal regions and in the porta hepatis as indicated on the MRI images by tortuous T2 dark flow areas. The accompanying fibrosis is clearly shown as well [Figure 1]a and [Figure 1]b. [Figure 1]c and coronal T2 sequences images in [Figure 2]a,[Figure 2]b,[Figure 2]c are included to demonstrate the obstruction of the common bile duct (CBD) in the mid one third as well as the consequent dilatation of the extra and intrahepatic ducts. [Figure 3] is a coronal 3D thin MRCP sequence and demonstrates the above obstruction of the CBD as well as the striking normalcy of the unaffected distal portion.
|Figure 1: (a) Axial T2 STIR. Image reveals the absence flow void in the portal vein, which is replaced by intermediate signal fibrotic tissue. (b) Axial T2 STIR. The image reveals tortuous T2 dark flow void areas representing periportal vascular collaterals formation. (c) Axial T2 STIR. The image reveals dilated hepatic duct|
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|Figure 2: Coronal T2 sequence. (a-c) reveal dilated hepatic ducts with the abrupt termination of the common bile duct in the mid 1/3rd portion by tortuous collaterals. Note is made of gross splenomegaly|
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|Figure 3: Coronal 3D thin magnetic resonance cholangiopancreatography sequence reveals that dilated hepatic and proximal common bile duct with the abrupt termination of the common bile duct in mid 1/3rd portion by tortuous collaterals. The distal portion of the common bile duct is normal|
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The diagnosis was thus clinched and she was referred back to her GP with a consideration for onward expert care.
| Discussion|| |
The causes of EHPVO have been well documented as resulting in PvC. In the index case, the underlying cause was not determined. The resultant formation of PvC is the key phenomenon in the description of P.B. PvC leads to extrinsic compression, resulting in the reduction of the diameter of the intrahepatic and extrahepatic bile ducts. There are also underlying inflammatory changes, resulting in peribiliary fibrosis as seen in our patient [Figure 1] and [Figure 2]. The spectrum of changes on radiological probing includes features of portal hypertension such as splenomegaly, demonstration of the PvC along with EHPVO and there may be fibrosis-thought to be caused by compression-related ischemic changes., Others include extrinsic compression and caliber irregularity of the CBD, dilation of one or both of the hepatic ducts, or the common hepatic duct with possible ectactic appearance. These diagnostic changes were clearly demonstrated in our index case [Figure 1],[Figure 2],[Figure 3]. Clinically, the majority of patients have no significant symptomatic presentation thus the importance of radiological tools in the definitive diagnosis of P.B. cannot be overemphasized., In India, the condition is not uncommon and mostly presents in children, with varices and other features of elevated portal pressures. As per treatment, only symptomatic patients are treated. Usually, the endoscopic biliary intervention (EBI) is the first line of treatment in those with biliary obstructive symptoms. This intervention is considered ample and carries less risks., Other measures such as surgery may be considered in those that fail EBI therapy or those that live in far off places or in remote areas, who cannot make the repeated commutes that EBI sessions will demand. When surgery is indicated, it is usually multistaged. An initial decompressive shunt surgery before biliary bypass provides early symptomatic relief and may preclude the need for biliary bypass surgery.,
The case report shows the power of detailed imagery that is obtained with advanced radiological tools and thus, their usefulness in diagnosis in a Nigerian patient. We also hope that this documentation will help increase the index of suspicion for this disease among clinicians in our locality. It is said that poor awareness, lack of availability, and prohibitive cost have markedly impaired the adoption of these impactful radiological tools for the benefit of the local population.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Sarin SK, Bhatia V, Makwane U. Portal biliopathy in extra hepatic portal vein obstruction. Indian J Gastroenterol 1992;2:A82.
Khuroo MS, Rather AA, Khuroo NS, Khuroo MS. Portal biliopathy. World J Gastroenterol 2016;22:7973-82.
Baskan O, Erol C, Sahingoz Y. Portal biliopathy, magnetic resonance imaging and magnetic resonance cholangiopancreatography findings: A case series. Gastroenterol Rep (Oxf) 2016;4:68-72.
Khan AN, Uzoka K, Macdonald S, Hammond AA, Venyo AK. Portal biliopathy: A review of imaging features of nine patients. Int J Clini Med 2017;8:604-17.
Khuroo MS, Yattoo GN, Zargar SA, Javid G, Dar MY, Khan BA, et al
. Biliary abnormalities associated with extrahepatic portal venous obstruction. Hepatology 1993;17:807-13.
Agarwal AK, Sharma D, Singh S, Agarwal S, Girish SP. Portal biliopathy: A study of 39 surgically treated patients. HPB (Oxford) 2011;13:33-9.
[Figure 1], [Figure 2], [Figure 3]