Acute acalculous cholecystitis with portal cavernoma: A case report with literature review.

Portal cavernoma cholangiopathy (PCC) refers to morphological changes in the intrahepatic, extrahepatic biliary system, along with the gallbladder (GB), induced by portal cavernoma (PC). Acute acalculous cholecystitis (AAC) represents an infrequent clinical manifestation of PCC. Given the inadequacy of documentation within medical literature, AAC may go undiagnosed among patients with PC presenting symptoms of right upper quadrant pain. The current study aims to report a case of acute acalculous cholecystitis secondary to portal cavernoma, focusing on radiological findings, with a brief review of literature.

Portal Vein Reconstruction-trans Jugular Intrahepatic Portosystemic Shunt and Portal Vein Stenting in Patients With Extrahepatic Portal Vein Obstruction.

Extrahepatic portal vein thrombosis (EHPVO) is an uncommon cause of portal hypertension. In the long term, patients may develop portal cavernoma cholangiopathy (PCC). Up to 30%-40% of patients with EHPVO may not have shuntable veins and are often difficult to manage surgically. Interventional treatment including portal vein recanalisation-trans jugular intrahepatic portosystemic shunt (PVRecan-TIPS) has been used for patients with EHPVO. However, PV reconstruction-trans jugular intrahepatic portosystemic shunt (PVRecon-TIPS) and portal vein stenting are novel techniques for managing such patients with EHPVO with non-shuntable venous anatomy. In contrast to PVRecan-TIPS, PV reconstruction-TIPS (PVRecon-TIPS) is performed through intrahepatic collaterals. Here we present six cases of PCC who presented with recurrent acute variceal bleeding (AVB) and or refractory biliary stricture. They did not have any shuntable veins. PVRecon-TIPS was performed for five patients whilst PV stenting was done in one. Amongst the six patients, one died of sepsis whilst one who developed hyponatremia and hepatic encephalopathy was salvaged with conservative management. Following the procedure, they were started on anti-coagulation. Decompression of cavernoma was documented in all other patients. Biliary changes improved completely in 40% of patients.

Portosystemic Shunt Surgery for Extrahepatic Portal Venous Obstruction Beyond Endoscopic Variceal Eradication: Two Decades of Pediatric Surgical Experience.

Background: This exclusively surgical series on pediatric extrahepatic portal venous obstruction (EHPVO) defines surgical indications beyond endoscopic eradication of esophageal varices (EEEV), the selection of an appropriate surgical procedure, and the long-term post-surgical outcome. Methods: EHPVO management protocol at the reporting institute has been endotherapy until esophageal variceal eradication and surgery for select adverse sequelae manifesting after EEEV. Results: One hundred and thirty-nine EHPVO cases underwent surgery for the following indications in combination: i) massive splenomegaly with severe hypersplenism (n = 132, 95%); ii) growth retardation (GR, n = 95, 68%); iii) isolated gastric (IGV) and ectopic varices (n = 49, 35%); iv) Portal cavernoma cholangiopathy (PCC) (n = 07, 5%). A portosystemic shunt (PSS) was performed in 119 (86%) cases. Types of PSS performed were as follows: central end-to-side splenorenal shunt with splenectomy (n = 104); side-to-side splenorenal shunt (n = 4); mesocaval shunt (n = 1); inferior mesenteric vein (IMV) to left renal vein shunt (n = 2); IMV to inferior vena cava shunt (n = 3); H-graft interposition splenorenal shunt (n = 1); spleno-adrenal shunt (n = 3); makeshift shunt (n = 1). Esophagogastric devascularization (n = 20, 14%) was opted for only for non-shuntable anatomy. At a median follow-up (FU) of 41 (range: 6-228) months, PSS block was detected in 13 (11%) cases, with recurrent variceal bleeding in 4 cases. PCC-related cholestasis regressed in 5 of 7 cases. Issues of splenomegaly were resolved, and growth z-scores improved significantly. Conclusions: Endotherapy for secondary prophylaxis until EEEV has resulted in a shift in surgical indications for EHPVO. Beyond EEEV, surgery was indicated predominantly for non-variceal sequelae, namely massive splenomegaly with severe hypersplenism, GR, and PCC. Varices warranted surgery infrequently but more often from sites less amenable to endotherapy, i.e., IGV and ectopic varices. The selection of PSS was tailored to anatomy and surgical indications. On long-term FU post surgery, PSS block was detected in 13% of patients. PCC-related cholestasis regressed in 71%, and issues of splenomegaly resolved with significantly improved growth Z scores.

The use of fully covered self-expandable metal stents in the endoscopic treatment of portal cavernoma cholangiopathy.

BACKGROUND AND AIMS: There are different therapeutic approaches for biliary strictures and reducing portal hypertension in patients with symptomatic portal cavernoma cholangiopathy (PCC). Endoscopic treatment includes endoscopic biliary sphincterotomy (EST), dilation of stricture with a biliary balloon, placement of plastic stent(s) and stone extraction. Fully covered self-expandable metal stent (FCSEMS) is placed as a rescuer in case of haemobilia seen after EST, dilation of stricture and removal of plastic stent rather than the stricture treatment itself. In this retrospective observational study, we sought to assess the clinical outcomes of FCSEMS as the initial treatment for PCC-related biliary strictures. MATERIALS AND METHODS: Twelve symptomatic patients with PCC both clinically and radiologically between July 2009 and February 2019 were examined. Magnetic resonance cholangiopancreatography (MRCP) and cholangiography were employed as the diagnostic imaging methods. Chandra-Sarin classification was used to distinguish between biliary abnormalities in terms of localization. Llop classification was used to group biliary abnormalities associated with PCC. Endoscopic partial sphincterotomy was performed in all the patients. If patients with dominant strictures 6-8-mm balloon dilation was first performed. This was followed by removal of the stones if exist. Finally, FCSEMS placed. The stents were removed 6-12 weeks later. RESULTS: The mean age of the patients was 40.9 ± 10.3 years, and 91.6% of the patients were male. Majority of the patients (n = 9) were noncirrhotic. Endoscopic retrograde cholangiopancreatography (ERCP) findings showed that 11 of the 12 patients were Chandra Type I and one was Chandra Type IIIa. All the 12 patients were Llop Grade 3. All patients had biliary involvement in the form of strictures. Stent placement was successful in all patients. FCSEMSs were retained for a median period of 45 days (30-60). Seven (58.3%) patients developed acute cholecystitis. There was no occurrence of bleeding or other complications associated with FCSEMS replacement or removal. All patients were asymptomatic during median 3 years (1-10) follow up period. CONCLUSIONS: FCSEMS placement is an effective method in biliary strictures in case of PCC. Acute cholecystitis is encountered frequently after FCSEMS, but majority of patients respond to the medical treatment. Patients should be followed in terms of the relapse of biliary strictures.

Feasibility of laparoscopic cholecystectomy for symptomatic gallstone disease with portal cavernoma: Can prior portal vein decompression be avoided?

Backgrounds/Aims: Biliary surgery in patients with extrahepatic portal vein obstruction with portal cavernoma (PC) is technically challenging, and associated with the risk of bleeding. Therefore, prior portal vein decompression is usually recommended before definitive biliary surgery. Only a few studies have so far reported the safety of isolated laparoscopic cholecystectomy. We aimed to evaluate our experience of laparoscopic cholecystectomy in patients with PC without prior portal decompression. Methods: Prospectively maintained data for patients with PC who underwent laparoscopic cholecystectomy for symptomatic gallstone disease without portal decompression were analyzed. Clinical features, imaging, intraoperative factors, conversion rate, complications of surgery, and long-term outcomes were assessed. Results: Sixteen patients underwent cholecystectomy without portal decompression from 2012 to 2021, of which interventions 14 were laparoscopic cholecystectomies. One patient required conversion (7.1%) to open surgery. Jaundice was present in 5 patients (35.7%), and underwent endoscopic stone clearance before surgery. Median intraoperative blood loss, operative time, and hospital stay were 100 mL (20-400 mL), 105 min (60-220 min), and 2 days (1-7 days), respectively. Blood transfusion was required in two patients (14.2%). Prior endoscopic or percutaneous intervention was associated with significant blood loss and prolonged intraoperative time. Conclusions: In centers with experience, prior portal decompression can be avoided in patients with PC requiring isolated cholecystectomy to treat gallstones or their complications. Laparoscopic surgery is safe and feasible for these patients, and gives excellent outcomes in the selected group.

Portal cavernoma in type 1 neurofibromatosis: A fortuitous or causal association?

Neurofibromatosis type 1 (NF-1) is a multisystem genetic disorder affecting the NF1 tumor suppressor gene. Patients typically develop superficial (cutaneous) and internal (plexiform) neurofibromas. The latter may rarely involve the liver locating in the hilum and encasing the portal vessels, leading to portal hypertension. Vascular abnormalities (NF-I vasculopathy) are a well-recognized manifestation of NF-1. Although the pathogenesis is not well-known, NF-1 vasculopathy involves arteries of both peripheral and cerebral territories, with venous thrombosis being exceptionally reported. Portal venous thrombosis (PVT) is the leading cause of portal hypertension in childhood and has been associated with several risk factors. Nevertheless, predisposing conditions remain unknown in more than 50% of the cases. The treatment options are limited, and its management is nonconsensual in the pediatric age. We report the case of a 9-year-old boy with clinically and genetically confirmed NF-1, diagnosed with portal venous cavernoma after an episode of gastrointestinal bleeding. There were no identifiable risk factors for PVT and intrahepatic peri-hilar plexiform neurofibroma was excluded by MRI imaging. To the best of our knowledge, this is the first report of PVT in NF-1. We speculate that NF-1 vasculopathy may have been a pathogenic factor, or instead, it was a fortuitous association.

Pregnancy outcomes in patients with portal cavernoma: A case series study.

OBJECTIVE: Non-cirrhotic portal vein cavernoma (PVC) is a cause of portal hypertension (PH) frequently affecting women of childbearing age. Cavernous transformation of the portal vein is frequently associated with prothrombotic disorders and often entails multiple hemodynamic changes, porto-collateral shunt development and thrombopenia, all of which can affect the course of pregnancy. Our aim was to evaluate the risk of PH-related complications and pregnancy outcomes in patients with PVC. METHODS: Retrospective case series study of patients with PVC undergoing pregnancy in a tertiary care hospital. RESULTS: Eight pregnancies fulfilled the eligibility criteria. All patients had a predisposing factor for PVC. One episode of variceal bleeding was reported at week 28. Six cesarean sections were scheduled to avoid labor while two urgent surgeries were indicated due to fetal distress and intrauterine growth restriction (IUGR). In all but one case, anticoagulation was prescribed after delivery. No hemorrhagic or thrombotic complications were reported. There were four cases of IUGR with no case of miscarriage or stillbirth. CONCLUSION: Pregnancy in patients with PVC has an overall favorable outcome albeit a higher risk of PH-derived complications, and IUGR may be expected. Hence, PVC must not be considered a contraindication for pregnancy although larger prospective studies are necessary.

Clinical outcomes and prognostic factors in non-cirrhotic non-neoplastic patients with portal vein thrombosis: A single-centre experience.

INTRODUCTION: The knowledge of natural history and prognostic factors of portal vein thrombosis (PVT) is still based on a limited number of studies. AIM: To describe our single-center experience with 79 consecutive non-neoplastic non-cirrhotic patients with PVT (15 recent/64 chronic PVT). RESULTS: Among patients with recent PVT, 7 received anticoagulation alone, 4 systemic thrombolysis, 3 direct thrombolysis through a TIPS and 1 TIPS alone. Portal recanalization was achieved in 11 patients. In patients with chronic PVT, the rate of variceal progression was high (20% at one year and 50% at two years). The thrombotic involvement of splenic and superior mesenteric veins was the only risk factor for variceal enlargement. The cumulative bleeding rates were 10% at one year and 20% at two years. A multisegmental thrombosis and large varices at entry and a previous variceal bleeding were the independent predictors for variceal bleeding. The cumulative rate of new thrombotic events was 14% at one year and 18% at two years. Eight patients died, 2 because of thrombotic events. There were no bleeding-related deaths. Two-year cumulative survival rate was 90%. CONCLUSIONS: Our study supports the importance of anticoagulation especially when a more extended thrombosis is present. Moreover, in patients with chronic PVT, the timing of follow-up endoscopy should be based on the extension of thrombosis and not, as in cirrhosis, on the size of varices at first endoscopy.

Cholestatic Jaundice Secondary to Portal Hypertensive Biliopathy Regarding a Case of Cavernous Transformation of the Portal Vein

Portal hypertensive biliopathy comprises the anatomical and functional abnormalities of the intra- and extrahepatic biliary tract, cystic duct, and gallbladder in patients with portal hypertension. The compromise of the bile duct usually occurs in portal obstruction due to the cavernous transformation of the portal vein (CTPV). We present a case of a young patient with a recent history of portal hypertension and CTPV who presented with an episode of cholestatic hepatitis. Studies documented an image of nodular appearance with extrinsic compression of the distal bile duct compatible with a tumor-like cavernoma. Effective endoscopic treatment was performed using endoscopic retrograde cholangiopancreatography (ERCP), sphincterotomy, and biliary stenting.

La biliopatía hipertensiva portal comprende las anomalías anatómicas y funcionales del tracto biliar intra- y extrahepático, el conducto cístico y la vesícula biliar en pacientes con hipertensión portal. El compromiso de la vía biliar suele presentarse en obstrucción portal debido a transformación cavernomatosa de la porta. Presentamos un caso de un paciente joven, con historia reciente de hipertensión portal y cavernomatosis de la porta, que presentó un episodio de hepatitis colestásica y en estudios se le documentó una imagen de apariencia nodular con compresión extrínseca de la vía biliar distal compatible con tumor-like cavernoma. En este caso se realizó un tratamiento endoscópico efectivo mediante colangiopancreatografía retrógrada endoscópica (CPRE), esfinterotomía y stent biliar.

Histological Changes in Portal Cavernoma Cholangiopathy.

INTRODUCTION: Portal cavernoma cholangiopathy (PCC)' refers to abnormalities of the extrahepatic and intrahepatic bile ducts in patients with portal hypertension. Although there is data on clinical and imaging aspects of PCC, the description of liver pathology has been strikingly deficient. The purpose of this study was to examine the histopathological characteristics of PCC. PATIENTS AND METHODS: A retrospective study of patients clinically diagnosed with extrahepatic portal vein obstruction (EHPVO) with portal cavernoma cholangiopathy, was conducted. Vascular anatomy was characterized by computerized tomographic angiography, and endoscopic retrograde cholangiography (ERC) and magnetic resonance cholangiography (MRC) were used to characterize the biliary anatomy. Histological features were analyzed by two hepatopathologists in a blinded manner, with mutual discussion to resolve any discrepancies. RESULTS: A total of 50 patients with portal cavernoma cholangiopathy were included in the study. The mean age of the patients was 26.2 ± 11.6 years. Radiologically, bilobar intrahepatic biliary dilatation was seen in 98% with common bile duct abnormality in 100% of patients, along with extrinsic ductal impressions in 77 % of cases. Liver tests were deranged total bilirubin 1.5 mg/dL (IQR 0.8-2.4) and alkaline phosphatase 109.5 IU/L (IQR 70-193). Histologically; dilated multiple portal venous channels (72%), hepatic artery thickening (70%). The presence of aberrant vascular channels around portal tracts (54%), elastosis of portal veins (50%), and bile ductular reaction in (44%) were the other prominent findings. A 12% of cases show focal thin bridges. Advanced fibrosis was not seen in any of the cases. One-fourth of the cases showed concomitant minimal to mild hepatocyte steatosis. CONCLUSIONS: Histologically, intrahepatic portal vein and portal tract abnormalities were noted in cases with portal cavernoma cholangiopathy, associated with mild derangement of liver tests.

Diagnostic and Therapeutic Challenges of Chronic Portal Vein Thrombosis Secondary to Myeloproliferative Neoplasm in Sub-Saharan Region: A Series of Cases and Literature Review.

Myeloproliferative neoplasms (MPN) are the commonest causes of non-cirrhotic abdominal vein thrombosis. MPN was observed in 30-40% with Budd Chiari Syndrome (BCS) and portal vein thrombosis (PVT), whereas this is the cause in only a minority of other types of venous thrombosis. Herein, we report 10 cases of chronic PVT secondary to Janus Kinase 2 gene (JAk 2) positive myeloproliferative neoplasm (MPN). The median age was 35 years with M:F 1:1. Portal hypertension was present at presentation in all of them. Nine patients have esophageal varices, of which 6 have bleeding at presentation. Classical MPN phenotype on complete blood count (CBC) was not present in 6 of them. Management of patients with anticoagulation was difficult with 4 patients experiencing bleeding during anticoagulation, and bleeding occurring despite the target international normalized ratio (INR) in 2 of them. Portal hypertension leading to hypersplenism and hemodilution may mask erythrocytosis and thrombocytosis, which are the main criteria used to diagnose MPN. The resulting absence of a typical MPN phenotype in routine laboratory studies alongside accessibility issues, and the need for a multidisciplinary treatment approach imparts a diagnostic as well as a therapeutic challenge for chronic PVT associated with Jak 2 positive MPN in sub-Saharan patients.

A case of portal biliopathy in a young patient with portal cavernoma secondary to neonatal umbilical vein catheterization.

Portal biliopathy (PB) refers to biliary obstruction caused by cavernous transformation of the portal vein (CTPV). CTPV occurs most frequently in patients with liver cirrhosis or malignancy. Less common causes include congenital malformations and neonatal umbilical vein cannulation. We present a case of portal biliopathy in a 28-year-old man with CTPV secondary to umbilical vein catheterization in neonatal age. The case illustrates portal biliopathy as a late complication of neonatal invasive procedures and highlights the importance of a multimodality imaging approach to achieve a prompt diagnosis.

A Young Male With Non-cirrhotic Cryptogenic Portal Cavernoma: An Authoritative Case Study.

The growth of several porto-portal collateral veins encircling an existing stenosed or obstructed entry vein is an uncommon condition known as portal cavernoma. It is traditionally shown as the entry vein thrombosis (portal vein thrombosis - PVT) outcome. A male of 25 years with stomach discomfort for three days that was acute, nonprogressive, and was not accompanied by fever, loose stools, or vomiting. After he had undergone abdominal ultrasonography, portal vein thrombosis was discovered, and based on no involvement of suprahepatic veins according to ultrasonography, Budd-Chiari syndrome was ruled out. It was accompanied by dilated periportal tortuous veins and visible mesenteric and peri-splenic collaterals. Moderate splenomegaly was also present. All these features on ultrasound were suggestive of the "portal cavernoma" formation. The patient is not an alcoholic and does not have any chronic, hereditary, or metabolic liver disease. Thrombophilia and cancer screening through tumor markers were also negative. We, with this, present a rare case of non-cirrhotic idiopathic portal cavernoma. This rare case contributes to advancing medical and scientific knowledge that will encourage further dialogue on the topic.

A Rare Case of Chronic Invasive Pulmonary Aspergillosis.

Invasive pulmonary aspergillosis (IPA) and invasive aspergillosis (IA) are two examples of the broad clinical spectrum of Aspergillus infection. It mainly affects severely immunocompromised hosts, while immunocompetent people can sometimes be affected, especially those receiving treatment in the intensive care unit (ICU) for emergency cases with few instances of chronic cases. The risk factors in ICU patients for aspergillosis include intubated patients receiving hot and humidified air, viral infections like covid, and influenza, and diseases like diabetes, chronic obstructive pulmonary disease, etc. A case of 35-year-old male reported to us with a complaint of stomach discomfort that was acute and non-progressive in the epigastric area, radiating to the back, not accompanied by fever, and not linked with loose stools/vomiting. In addition, the patient experienced a nonproductive cough for two days that was not associated with dyspnea or chest discomfort. He had a high-resolution computed tomography (HRCT) thorax, which revealed a single pulmonary nodule in the left lung's middle zone; histology of the same nodule biopsy material revealed that it was caused by Aspergillus. He had an abdominal ultrasound, which revealed portal vein thrombosis, dilated periportal tortuous veins, evident peri splenic and mesenteric collaterals, and significant splenomegaly - suggestive of portal cavernoma formation with chronic liver parenchymal disease. Our patient has a past history of alcohol use disorder for the last 15 years due to which the patient has had recurrent episodes of acute pancreatitis for the last three years which has now progressed to chronic pancreatitis, also the patient has been diabetic for the last 10 years on insulin for the same. A patient with multiple comorbidities, such as cirrhotic portal cavernoma, type 2 diabetes, diabetic neuropathy, and acute and chronic pancreatitis, is the subject of our case study on chronic IPA.

Benign Hepatic Nodules in Patients With Primary Extrahepatic Portal Vein Obstruction: Clinical and Magnetic Resonance Imaging Features.

Background/objective: To retrospectively evaluate the magnetic resonance imaging (MRI) features of benign hepatic nodules in patients with extrahepatic portal vein obstruction (EHPVO) and assess predictable features for their development. Methods: This retrospective observational study included 18 diagnosed patients of EHPVO who underwent contrast enhanced abdominal MRI at our Institute between June 2016 and May 2017, and who could be followed up for at least two years. The patients with liver nodules formed the study group (n = 8; 4 males, 4 females; mean age: 26.1 ± 10.9 years) and patients without liver nodules were controls (n = 10; 3 males, 7 females; mean age: 24.2 ± 15.1 years). Liver nodules were confirmed as benign by either biopsy or stability on follow up imaging. MRI features of liver nodules were assessed. Clinical details and imaging data of the study group were compared with controls to assess predictable features. Results: There was no statistically significant difference in age, gender, clinical characteristics and upper gastrointestinal endoscopic findings between the study and control groups. The size of the lienorenal collateral, left renal vein and superior mesenteric vein were significantly larger in the study group (P < 0.05). In the study group, the majority had multiple hepatic nodules with most of them being isointense on T1 (18/35; 51.4%) and T2-weighted images (16/35; 45.7%) and showing restriction of diffusion (21/35; 60%). All (n = 35) lesions showed arterial phase hyperenhancement and none showed washout in the venous phase. The patients in the control group did not develop any liver nodules during the follow-up period. Conclusion: Liver nodules in patients with EHPVO are likely to be benign and have characteristic MRI features. Significantly larger lienorenal collateral, left renal vein and superior mesenteric vein were associated with the development of these nodules.

Portal cavernoma cholangiopathy: Update and recommendations on diagnosis and management.

Portal cavernoma cholangiopathy is defined as an obstruction of the biliary system due to distended veins surrounding bile ducts that mainly occur in patients with extrahepatic portal venous obstruction. The periductal venous plexuses encircling the ducts can cause morphological changes which may or may not become symptomatic. Currently, non-invasive techniques such as ultrasonography, computed tomography, magnetic resonance cholangiopancreatography, and dynamic contrast enhanced magnetic resonance images are being used to diagnose this disorder. Only a few patients who have symptoms of biliary obstruction require drainage which might be accomplished using endoscopic stenting, decompression of the portal venous system usually via a lienorenal shunt, a difficult direct hepaticojejunostomy, and rarely a liver transplant.

Long-Term Outcomes of Endoscopic Management of Patients with Symptomatic Portal Cavernoma Cholangiopathy with No Shuntable Veins for Surgery or Failed Surgery.

Background and Aim: Shunt surgery is the definitive treatment for symptomatic patients with portal cavernoma cholangiopathy (PCC), but few patients are non-surgical candidates or fail to improve even after surgery. This study aims to analyze the long-term outcomes of endoscopic therapy in these patients. Methods: Retrospective review of a prospectively maintained database of all patients with symptomatic PCC managed with endoscopic retrograde cholangiography (ERC) followed by stent placement. Outcomes studied included number of biliary interventions, complications, resolution of stricture, development of decompensation and mortality. Results: Thirty-five patients (68.6% males, median age = 35 years) with a median follow-up duration of 46 months (12-112) were included in the analysis. Presentation was only jaundice in 51.4% cases while one-third (37.1%) of the patients presented with cholangitis. Patients underwent a total of 363 endoscopic sessions with a median of 9 procedures (3-29) per patient. Hemobilia was the most common complication of the procedure (6.06%). Ten (28.5%) patients required frequent stent exchanges. Patients who required frequent stent exchanges had higher number of cholangitis episodes and hospitalization. Secondary biliary cirrhosis developed in 4 (11.4%) patients and 2 (5.7%) patients had mortality. Of the 5 (14.3%) patients who were given a stent free trial, 3 patients required restenting due to redevelopment of symptoms. Conclusion: Patients with PCC without shuntable veins for surgery or those who failed to improve after surgery can be managed long-term with repeated endoscopic intervention with a slightly increased risk of non-fatal hemobilia.

Portal Cavernoma Cholangiopathy in Children: An Evaluation Using Magnetic Resonance Cholangiography and Endoscopic Ultrasound.

BACKGROUND: Portal cavernoma cholangiopathy (PCC) refers to abnormalities of the extra- and intrahepatic bile ducts in patients with portal cavernoma. The literature on PCC in children is very scarce. This study aimed at characterizing PCC in children with extrahepatic portal venous obstruction (EHPVO) using endoscopic ultrasound (EUS) and magnetic resonance cholangiography/portovenography (MRC/MRPV). METHODS: A total of 53 consecutive children diagnosed with EHPVO were prospectively evaluated for PCC using MRC/MRPV and EUS. Chandra classification was used for type of involvement and Llop classification for grading of severity. RESULTS: All 53 children (100%) had PCC changes on MRC/EUS, but none were symptomatic. Extrahepatic ducts (EHDs) and intrahepatic ducts were involved in majority (85%), and 58.5% had severe changes. Periductal thickening/irregularity (71%) was the commonest change in intrahepatic ducts, whereas irregular contour of the duct with scalloping (68%); common bile duct (CBD) angulation (62.3%) were the frequent changes in the EHDs. Increased CBD angulation predisposed to CBD strictures (P = 0.004). Both left and right branches of portal vein were replaced by collaterals in all children. Among the EUS biliary changes, para-pericholedochal, intrapancreatic, and intramural gall bladder collaterals had significant association with severity, with higher frequency of occurrence in children with the most severe Llop Grade. CONCLUSIONS: PCC develops early in the disease course of EHPVO, in children, but is asymptomatic despite severe changes. EUS biliary changes are more likely to be observed with increasing severity of PCC.

[Portal cavernoma at child about two observations]. / Cavernome portal chez l'enfant a propos de deux observations.

We report two observations of portal cavernoma diagnosed successively in Bamako and Dakar. The first is a 6-year-old male admitted to the service for ascites and abdominal pain. At admission the clinical parameters (weight, height, temperature, cranial perimeter and temperature) were within the norms for age. The clinical examination noted a moderate skin-mucosal pallor, asthenia. The biological assessment returned to moderate normochrome anemia with impaired pancreatic function while renal and hepatic functions were maintained. The abdominal scan performed after two low-contribution abdominal ultrasounds, objected signs in favor of a portal cavernoma with perisplenic and gastric varicose veins. The second is an 8-year-old male child born from an unborn marriage and from a followed pregnancy with premature delivery. His pathological history includes a notion of prematurity that required a stay in neonatology with umbilical catheterization and repeated abdominal pain. He had an acute abdominal episode in March 2015 justifying a surgical hospitalization for suspicion of appendicitis. At admission the clinical parameters (weight, height, temperature, cranial perimeter and temperature) were within the norms for age. The abdominal ultrasound prescribed for this was suggestive of portal cavernoma, later confirmed by abdominal computed tomography.

Nous rapportons deux observations de cavernome portal diagnostiqué successivement à Bamako et à Dakar. Le premier est un enfant de 6 ans de sexe masculin admis dans le service pour ascite et douleurs abdominales. L'examen clinique notait une pâleur cutanéo-muqueuse modérée, une asthénie. Le bilan biologique retrouvait une anémie modérée normochrome normocytaire avec une fonction pancréatique perturbée tandis que les fonctions rénales et hépatiques étaient conservées. Le scanner abdominal réalisé après deux échographies abdominales peu contributives, objectivait des signes en faveur d'un cavernome portal avec varice péri-splénique et gastrique. Le second est un enfant de 8 ans de sexe masculin né d'un mariage non consanguin et issu d'une grossesse suivie avec accouchement prématuré. Il est le 3e enfant de sa fratrie et scolarisé. On retrouve dans ses antécédents pathologiques une notion de prématurité ayant nécessitée un séjour en néonatologie avec cathétérisme ombilical et des douleurs abdominales à répétition. L'enfant a commencé à se plaindre de douleurs abdominales récurrentes vers l'âge de 6 ans. Douleurs de siège péri ombilical sans réveil nocturne dans un contexte de constipation chronique d'allure fonctionnelle. Il a fait un épisode abdominal aigu justifiant une hospitalisation en chirurgie pour suspicion d'appendicite. A l'admission les paramètres cliniques (poids, taille, température, périmètre crânien et température) étaient dans les normes pour l'âge. L'échographie abdominale prescrite à cet effet était évocatrice de cavernome porte, confirmé par la suite par la tomodensitométrie abdominale.

Case Report: Portal cavernoma related to multiple liver hydatidosis: A rare case of fatal cataclysmic haemorrhage.

Clinical presentation of liver hydatidosis can vary from asymptomatic forms to lethal complications. We report a rare case of a 27-year-old male from a rural Tunisian region who presented with large-abundance haematemesis, haemodynamic instability, and marked biological signs of hypersplenism. Endoscopy showed bleeding esophageal varicose veins that were ligated. Abdominal ultrasound concluded the presence of three type CE2 hydatic liver cysts causing portal cavernoma with signs of portal hypertension. Despite resuscitation, the patient died of massive rebleeding leading to haemorrhagic shock. Hepatic hydatid cyst should be considered as an indirect cause of gastrointestinal bleeding in endemic countries. Early abdominal ultrasound in varicose haemorrhage is essential in orienting the diagnosis.