BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder with an incidence of approximately 1 in 5000 in the general population. It is characterized by vasodilation, which affects specific organs, such as the skin, mucous membranes, brain, lungs, gastrointestinal tract, liver, and others. However, HHT rarely involves the portal venous system to cause serious clinical complications.
METHODS: A 68-year-old woman was admitted to the emergency department due to four consecutive days of abdominal pain and bloody stool and was subsequently diagnosed with HHT. Computed tomography angiography confirmed the presence of an arteriovenous fistula (AVFs). Considering this specific manifestation, whole exome sequencing was performed. After a comprehensive evaluation, a selective superior mesenteric artery embolization was prioritized to avoid intestinal ischemia. The postoperative symptoms of the patient were quickly relieved. Unfortunately, two months post-procedure the patient died from intestinal necrosis and abdominal infection related to remaining AVFs.
CONCLUSIONS: For patients with diffuse superior mesenteric AVFs, selective mesenteric arterial embolization may lead to positive short-term outcomes.

Congenital portosystemic shunt (CPS) is a developmental anomaly of the portal vein system. The disease can cause blood from the portal vein to flow into the vena cava, resulting in various atypical clinical manifestations. Pelvic congestion syndrome (PCS) caused by CPS is particularly rare. A young woman with PCS had an abnormal communicating branch of the left ovarian vein (OV). Her left OV drained normally into the left renal vein, and at the same time communicated with the portal vein, forming an extrahepatic portosystemic shunt. With embolization of her left OV, the patient was cured of PCS.

As the COVID-19 pandemic spread worldwide, case reports and small series identified its association with an increasing number of medical conditions including a propensity for thrombotic complications. And since the nephrotic syndrome is also a thrombophilic state, its co-occurrence with the SARS-CoV-2 infection is likely to be associated with an even higher risk of thrombosis, particularly in the presence of known or unknown additional risk factors. Lower extremity deep vein thrombosis (DVT) and pulmonary embolism (PE) are the most common manifestations of COVID-19-associated hypercoagulable state with other venous or arterial sites being much less frequently involved. Although splanchnic vein thrombosis (SVT) has been reported to be 25 times less common than usual site venous thromboembolism (VTE) and rarely occurs in nephrotic patients, it can have catastrophic consequences. A small number of SVT cases have been reported in COVID-19 infected patients in spite of their number exceeding 180 million worldwide.
An unvaccinated young adult male with steroid-dependent nephrotic syndrome (SDNS) who was in a complete nephrotic remission relapsed following contracting SARS-CoV-2 infection and developed abdominal pain and diarrhea. Abdominal US revealed portal vein thrombosis. The patient was anticoagulated, yet the SVT rapidly propagated to involve the spleno-mesenteric, intrahepatic and the right hepatic veins. In spite of mechanical thrombectomy, thrombolytics and anticoagulation, he developed mesenteric ischemia which progressed to gangrene leading to bowel resection and a complicated hospital course.
Our case highlights the potential for a catastrophic outcome when COVID-19 infection occurs in those with a concomitant hypercoagulable state and reminds us of the need for a careful assessment of abdominal symptoms in SARS-CoV-2 infected patients.

OBJECTIVE: The efficacy of sodium phenylbutyrate (SPB) for hyperammonemia associated with congenital portosystemic shunt (CPSS) remains unknown. We show the effectiveness of oral SPB.
METHODS: Our patient had CPSS with severe hypoplasia of extrahepatic portal veins. At 9 months of age, to assess the efficacy of oral SPB, we evaluated the 24 h fluctuations of venous ammonia levels. In the first two days without SPB, ammonia levels were above 80 μmol/L for half a day. On the third and fourth days, administration of oral SPB three times a day decreased ammonia to acceptable levels, except at midnight. On the fifth day, another oral SPB administration at 8 pm decreased ammonia at midnight. Low levels of branched-chain amino acids, as well as coagulation disturbances, were observed without apparent symptoms. At 12 months of age, he showed normal psychomotor development.
CONCLUSIONS: Oral SPB may be effective for hyperammonemia associated with CPSS.

In case of mechanical cholestasis, along with stagnation in the veins and bile ducts, lymph production increases, which contributes to the accumulation of ascitic fluid. In the peribiliaryareas, where the extramural biliary glands are located, an accumulation of lymphatic vessels has been observed in all the preparations.In these same areas, a contrast-enhanced microtomography showed transition of the ink-gelatin contrast agents (using gelatin and India ink) from the extramural part of the mucous glands to the lumen of the lymphatic capillaries and blood vessels.Lymphobiliary connections were visualized during the preparation of the sections of the portal triad of the lobar and sectoral portal tracts under a binocular microscope. More reliable evidence of the existence of lymphobiliary connections in the portal triad region was revealed on histological preparations stained with traditional methods.; Thus, anatomically, the area of the portal triad appears to be one of the alternative areas of lymphobiliary communications, which further confirms its high adaptive capacity in case of stagnation of bile.

BACKGROUND: Major pancreatic injuries are complex to treat, especially when combined with vascular and other critical organ injuries. This case-matched analysis assessed the influence of associated visceral vascular injuries on outcome in pancreatic injuries.
METHODS: A registered prospective database of 461 consecutive patients with pancreatic injuries was used to identify 68 patients with a Pancreatic Injury combined with a major visceral Vascular Injury (PIVI group) and were matched one-to-one by an independent blinded reviewer using a validated individual matching method to 68 similar Pancreatic Injury patients without a vascular injury (PI group). The two groups were compared using univariate and multivariate logistic regression analysis and outcome including complication rates, length of hospital stay and 90-day mortality rate was measured.
RESULTS: The two groups were well matched according to surgical intervention. Mortality in the PIVI group was 41% (n = 28) compared to 13% (n = 9) in the PI alone group (p = 0.000, OR 4.5, CI 1.00-10.5). On univariate analysis the PIVI group was significantly more likely to (i) be shocked on admission, (ii) have a RTS < 7.8, (iii) require damage control laparotomy, (iv) require a blood transfusion, both in frequency and volume, (v) develop a major postoperative complication and (vi) die. On multivariate analysis, the need for damage control laparotomy was a significant variable (p = 0.015, OR 7.95, CI 1.50-42.0) for mortality. Mortality of AAST grade 1 and 2 pancreatic injuries combined with a vascular injury was 18.5% (5/27) compared to an increased mortality of 56.1% (23/41) of AAST grade 3, 4 and 5 pancreatic injuries with vascular injuries (p = 0.0026).
CONCLUSIONS: This study confirms that pancreatic injuries associated with major visceral vascular injuries have a significantly higher complication and mortality rate than pancreatic injuries without vascular injuries and that the addition of a vascular injury with an increasing AAST grade of pancreatic injury exponentially compounds the mortality rate.

Pregnancy with massive splenomegaly is a rare entity and is associated with increased risk to both mother and foetus. There is paucity of studies in the literature to guide clinicians for the management of this condition.
We reviewed the course of pregnancy, maternal and foetal outcomes of 5 pregnant women with massive splenomegaly who were managed in our unit during 2015-16.
All 5 women had anaemia and thrombocytopenia, and had different causes for splenomegaly. One patient had chronic malaria, 2 had portal hypertension with cirrhosis and the remaining 2 had non-cirrhotic portal hypertension. Life-threatening complications were present in 2 patients; one of them had severe pre-eclampsia complicated by pulmonary oedema, cardiac arrest and the other patient developed spontaneous bacterial peritonitis. Intrauterine growth restriction and meconium-stained liquor were the most common perinatal complications. Two patients had vaginal delivery and 3 required emergency caesarean section. Postpartum haemorrhage was present in 2, and the hospital stay was prolonged in all the patients. All mothers and babies were discharged in a satisfactory condition.
Pregnancy with massive splenomegaly poses a challenge because of diverse aetiology and potentially adverse outcomes. Multidisciplinary care in a tertiary centre can help optimize the outcome.

BACKGROUND: Extensive thrombosis in the portal venous system caused by hypereosinophilic syndrome (HES) is rare, and there is no consensus on anticoagulant and thrombolytic treatments for arteriovenous thrombosis caused by HES.
METHODS: The clinical data of a patient with extensive thrombosis in his portal venous system (superior mesenteric, splenic, hepatic, and portal veins), renal artery thrombosis, and mesenteric thrombosis caused by HES with secondary gastrointestinal bleeding and intestinal necrosis were retrospectively analyzed. Before admission, his eosinophil count increased to 7.47 × 10/L, and HES had been confirmed via bone marrow cytology. The patient experienced fever, cough, abdominal pain, massive hematemesis, and hematochezia that developed in succession. Abdominal computed tomography showed portal vein and superior mesenteric vein thromboses.
METHODS: Hypereosinophilic syndrome; extensive thrombosis in the portal venous system; acute eosinophil-associated pneumonia; gastrointestinal bleeding; intestinal necrosis.
METHODS: The patient was first treated with methylprednisolone, plasma exchange/hemofiltration, and single or combined use of unfractionated heparin and argatroban for anticoagulation. He was also administered alteplase and urokinase, successively, for thrombolytic treatment. Once the thromboses finally disappeared, the patient underwent surgery to excise a necrotic intestinal canal.
RESULTS: The thromboses disappeared with these treatments, and the patient recovered after the necrotic intestinal canal was excised.
CONCLUSIONS: The clinical manifestations of HES are complex and varied, and this condition can cause severe and extensive arteriovenous thrombosis. Anticoagulation therapy and thrombolysis are necessary interventions, and appear to be safe and effective.

BACKGROUND: Hepatic portal venous gas (HPVG) is a rare radiological finding in which gas enters the portal venous system and it is associated in case of necrotizing colitis with a mortality of 75%. We report a case of iatrogenic HPVG with a review of literature.
METHODS: A 41 years old patient underwent total colectomy and ileal pouch- anal anastomosis with derivative ileostomy for a familiar adenomatous polyposis coli in June 2008. A stenosis of the pouch-anal anastomosis developed. The patient underwent several endoscopic dilations. A recurrence of the stenosis was observed. The patient underwent to several endoscopic procedure. After the last colonoscopy the patient showed a fever with abdominal pain. A CT scan showed little peri-anastomotic collections and massive hepatic portal venous gas.
CONCLUSIONS: The management of HPVG varied from surgical intervention to non-operative procedure. The surgical approach it\'s reserved to clinically unstable patients or those with evidence of peritonitis or bowel perforation. Stable patients, like those with an HPVG consequence of an endoscopic procedure, can be treated with non- operative management.
CONCLUSIONS: Our experience confirm that hepatic portal venous gas can be related to endoscopic procedure; thus, it can be managed on the basis of patient\'s general clinical conditions, and in selected cases it will disappear without therapeutic interventions with a good outcome.

Hematochezia is a rare clinical presentation of congenital extrahepatic portosystemic shunt (CEPS). We describe a series of three patients with type II CEPS presenting as hematochezia that were treated by catheter embolization, followed by a brief review of published articles. Hematochezia of the patients was due to the giant inferior mesenteric vein, superior rectal vein and colonic varices. The catheter embolization was successfully accomplished in all of the patients. After a mean follow-up of 27 months, no serious adverse effects were observed. For unexplained massive hematochezia, CEPS needs to be considered as a differential diagnosis. Based on our present results and the review of the literature, transcatheter permanent embolization of the giant inferior mesenteric vein might be an effective and safe treatment for type II CEPS.Level of Evidence Level 4, case series.