Ectopic varices account for 5% of variceal bleedings and occur outside the gastro-esophageal region. This review evaluates the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) for ectopic variceal management. A comprehensive search through PubMed, Scopus, Web of Science, and Embase was conducted until January 16, 2023, using relevant keywords. Case reports and case series with fewer than 10 patients on TIPS for ectopic variceal management were included. The quality assessment followed the Joanna Briggs Institute checklist for case reports. This systematic review evaluated 43 studies involving 50 patients with ectopic varices undergoing TIPS. Patients had a mean age of 54.3 years, half were female, and two were pregnant. Alcoholic liver disease (48%) and hepatitis C infection (26%) were common causes of portal hypertension. Ascites and splenomegaly were reported in 32% and 28% of the patients, respectively. Rectal, oral, and stomal variceal bleeding accounted for 62%, 16%, and 22% of the patients, respectively. Ectopic varices were mainly located in the duodenum (28%) and rectum (26%) regions. Complications affected 42% of the patients, re-bleeding in eleven and hepatic encephalopathy in seven. The follow-up lasted 12 months on average, and finally, 5 received a liver transplant. Mortality post-TIPS was 18%. Despite complications and a notable mortality rate, favorable outcomes were observed in almost half of the patients with ectopic variceal bleeding managed with TIPS. Further research is warranted to refine strategies and improve patient outcomes.

Esophagojejunal varices occurring after total gastrectomy are rare but potentially fatal in cases of variceal bleeding. Owing to their rarity, treatment strategies for this condition are not well established. Here, we describe the case of a 48-year-old woman who presented with hematemesis and melena. Four years prior, she underwent a total gastrectomy for gastric cancer. Esophagojejunal variceal bleeding supplied by a dilated jejunal vein, along with liver cirrhosis, was diagnosed as per endoscopy and computed tomography findings. Initial attempts at endoscopic therapy were unsuccessful. Subsequently, transjugular intrahepatic portosystemic shunt placement was performed to reduce the portal pressure gradient, resulting in the cessation of bleeding. At the 1-month follow-up endoscopy, the varices had resolved, and no rebleeding occurred during 6 months of follow-up. Transjugular intrahepatic portosystemic shunt placement may be considered as an effective treatment option for esophagojejunal variceal bleeding.

Budd-Chiari syndrome is an uncommon disorder characterized by occlusion of hepatic veins. It can lead to portal hypertension. Most common causes of this syndrome are hypercoagulability states. Transjugular intrahepatic portosystemic shunt is often not possible given the portal venous thrombosis. In these cases, direct intrahepatic portocaval shunt, involving the creation of an access between the portal vein and the systemic circulation via the intrahepatic inferior vena cava has proven to be a feasible alternative, and can improve portal hypertension in these patients. Herein, we present a case of a 37-year-old woman diagnosed with Budd-Chiari syndrome that was successfully treated with percutaneous ultrasound (US)-guided direct intrahepatic portocaval shunt.

Transjugular intrahepatic portosystemic shunt (TIPS) is a life-saving procedure for patients with severe portal hypertension and persistent variceal bleeding. Stent fracture is a rare and severe complication; however, its cause and mechanisms remain poorly defined. This case helps understand the factors contributing to its occurrence, complications, and subsequent poor outcomes. A 63-year-old male was presented with ruptured bare stent after a TIPS procedure. The upper edge of the bare stent was ruptured, and its fraction subsequently migrated to the entrance of the right atrium. Meanwhile, a mural thrombus was formed in the inferior vena cava. A surgery for the removal of free fracture was planned for preventing the form of pulmonary embolism. Before the surgery, the fracture was shifted to the right inferior pulmonary artery. Therefore, the surgery was canceled for further evaluation. Then, hematemesis suddenly occurred with a high possibility of variceal bleeding and/or gastric ulcer bleeding. Despite comprehensive treatments, the patient symptoms were still worsened with the development of chest tightness, shortness of breath, severe hypoxia, and heart failure. Finally, the patient succumbed to systemic multiorgan failure and death. Taken together, a ruptured unstable stent should be removed as early as the patient is hemodynamically stable, as it is difficult to balance between hemostasis therapy and anticoagulation treatment in patients with liver-cirrhosis-related severe portal hypertension. Physicians should be on high alert of the potential complications of bare stent rapture after TIPS.
Ruptured TIPS stent with a fatal consequence Unstable stent rupture is a life-threatening complication of TIPS and severely complicates the treatment of gastric ulcer bleeding. Early removal of the ruptured stent is necessary to prevent further complications.

门静脉海绵样变性是临床处理的难点，近年来，经颈静脉肝内门体分流术（TIPS）逐渐在门静脉海绵样变性的治疗中展现出优势，但技术难度相当高，技术成功率相对较低。现介绍1例超声内镜标记门静脉辅助下TIPS，是门静脉海绵样变性伴门静脉闭塞治疗的探索性尝试。.

UNASSIGNED: Parastomal variceal bleeding (PVB) is a recognized complication of ostomized patients in the presence of portal hypertension. However, since there are few reported cases, a therapeutic algorithm has not yet been established.
UNASSIGNED: A 63-year-old man, who had undergone a definitive colostomy, recurrently presented to the emergency department hemorrhage of bright red blood from his colostomy bag, initially assumed to be caused by stoma trauma. Accordingly, temporary success on local approaches such as direct compression, silver nitrate application and suture ligation was achieved. However, bleeding recurred, requiring transfusion of red blood cell concentrate and hospitalization. The patient\'s evaluation showed chronic liver disease with massive collateral circulation, particularly at the colostomy site. After a PVB with associated hypovolemic shock, the patient was submitted to a balloon-occluded retrograde transvenous obliteration (BRTO) procedure which stopped the bleeding successfully. The patient was subsequently proposed for a transjugular intrahepatic portosystemic shunt (TIPS) conjugated with percutaneous transhepatic obliteration (PTO). After an initial refusal by the patient, a new episode of self-limited PVB resulted in execution of the procedure. Four months later, in a routine consultation, the patient presented with grade II hepatic encephalopathy, successfully treated with medical therapy. After a 9-month follow-up, he remained clinically well and without further episodes of PVB or other adverse effects.
UNASSIGNED: This report highlights the importance of a high index of suspicion when dealing with significant stomal hemorrhage. Portal hypertension as an etiology of this entity may compel to a specific approach to prevent recurrence of bleeding, including conjugation of endovascular procedures. The authors pre-sent a case of PVB, initially submitted to a variety of treatment options including BRTO, which was successfully addressed with conjugated treatment of TIPS and PTO.
UNASSIGNED: A hemorragia de varizes periestomais é uma complicação conhecida de doentes ostomizados com hipertensão portal. Contudo, devido ao pequeno número de casos descritos, ainda não foi estabelecido um algoritmo terapêutico.
UNASSIGNED: Homem, 63 anos, com antecedentes de colostomia definitiva, recorre ao Serviço de Urgência recorrentemente por sangue vivo no saco de colostomia. Inicialmente, presumindo-se trauma do estoma, foi submetido a tratamentos locais, como compressão, aplicação de nitrato de prata e sutura, com sucesso temporário. Contudo, houve recorrência da hemorragia, com necessidade de suporte transfusional e hospitalização. A avaliação do doente evidenciou doença hepática crónica com circulação colateral exuberante, predominantemente junto da colostomia. Devido a hemorragia com choque hipovolémico, foi submetido a obliteração transvenosa retrógrada ocluída por balão (BRTO). Posteriormente, foi proposto para shunt portossistémico transjugular intra-hepático (TIPS) conjugado com obliteração transhepática percutânea (PTO). Após recusa inicial do doente, ocorreu novo episódio de hemorragia autolimitado, tendo o doente concordado em realizar o procedimento. Quatro meses depois, em consulta, apresentava sinais de encefalopatia hepática grau II, tendo sido controlada eficazmente com tratamento médico. Após nove meses de seguimento, mantém-se sem novos episódios de hemorragia ou efeitos adversos dos procedimentos.
UNASSIGNED: É necessário um alto índice de suspeição clínica ao abordar a hemorragia significativa do estoma. A hipertensão portal como etiologia exige uma abordagem específica para prevenir a recorrência da hemorragia, incluindo a conjugação de procedimentos endovasculares. Os autores apresentam o caso de um doente com hemorragia de varizes periestomais submetido inicialmente a vários tratamentos, incluindo BRTO e que foi tratado com sucesso com TIPS e PTO.

Budd-Chiari syndrome is a rare disease characterized by the obstruction of hepatic venous outflow. Stepwise treatment options aimed to relieve obstruction and prevent complications of Budd-Chiari syndrome are medical therapy, interventional recanalization, and surgery. Aggressive interventions for complicated Budd-Chiari syndrome are placement of a transjugular intrahepatic portosystemic shunt, surgical shunting, or liver transplantation. Although literature suggests differences in the presentation and management between Europe and Asia, cases documenting successful use of stepwise management of Budd-Chiari syndrome in Sub-Saharan Africa are scarce. A 47-year-old male on treatment for chronic hepatitis B presented with abdominal pain and distension for 2 weeks and findings of gross ascites without stigmata of chronic liver disease. Laboratory investigations performed showed anemia, elevated transaminases, coagulopathy, and renal dysfunction. Abdominal ultrasound and computed tomography abdominal scan revealed filling defects in intrahepatic veins and inferior vena cava extending to bilateral renal and external iliac veins. Extensive workup for thrombophilia and myeloproliferative disorders was negative. The diagnosis was hepatic dysfunction secondary to inferior vena cava obstruction due to a thrombus in the setting of extensive inferior vena cava thrombosis, and heparin was initiated. However, due to lack of recanalization with anticoagulation, we performed aspiration thrombectomy, balloon angioplasty, and local thrombolysis. Transjugular intrahepatic portosystemic shunt procedure was subsequently done due to hepatic venous congestion and refractory ascites. He was discharged on oral anticoagulation. Imaging exams performed 4 months later showed patent inferior vena cava and transjugular intrahepatic portosystemic shunt, good flows in the portal vein and resolution of ascites.

Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare plasma cell dyscrasia disease involving multiple organs combined with idiopathic non-cirrhotic portal hypertension. It has been reported only four times in the English literature. Here, we present the first case of a 62-year-old male POEMS syndrome patient with portal hypertension treated with the transjugular intrahepatic portosystemic shunt (TIPS), after he presented with a 10-day history of melena. The diagnosis of POEMS syndrome was given because the patient presented with polyneuropathy, monoclonal plasma cell proliferative disorder, sclerotic bone lesions, splenomegaly, lymphadenopathy, ascites, hypothyroidism, and hyperpigmentation. The presence of portal hypertension was confirmed by esophageal varices, congested and edematous stomach body, splenomegaly, and transudate ascites in which the serum-ascites albumin gradient of ascites fluid was over 11 g/L (a concentration considered to be associated with POEMS syndrome), as no other causes were found. The patient fasted and received conservative drug treatments on admission, but symptoms of melena soon recurred within 1 week after resuming his diet. After TIPS and venous embolization were performed, symptoms of bleeding were effectively controlled, while the patient subsequently developed hepatic encephalopathy, which ultimately led to death. The presence of gastrointestinal bleeding in POEMS syndrome with idiopathic non-cirrhotic portal hypertension indicates a poor prognosis. Given that this was the first patient to receive TIPS, and although the incidence of hepatic encephalopathy has increased, TIPS is still acceptable for refractory variceal bleeding.

Here, we report the case of patient cirrhosis with esophageal and gastric fundal varices who developed rare ectopic varices in the bilateral pulmonary hilar after repeated endoscopic treatments (tissue adhesive for gastric fundal varices + esophageal variceal ligation + esophageal variceal sclerotherapy) accompanied by serious shortness of breath. After transjugular intrahepatic portosystemic shunt + gastric coronary vein embolization, the shortness of breath was relieved, and the portography review indicated that the ectopic varices in the pulmonary hilar were significantly improved.

BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) is a method used to decrease portal hypertension. Biliary stricture is the rarest of the complications associated with this procedure with only 12 cases previously reported in the literature. None of these cases have documented the resolution of biliary stenosis induced by a stent graft. The only curative solutions reported are liver transplantation or bypassing the stenosis with an artificial biliary tract using advanced endoscopic techniques.
METHODS: This is the first reported case of biliary obstruction secondary to TIPS placement in a transplanted liver. In our patient, a portosystemic shunt was created to treat severe veno-occlusive liver graft disease manifesting itself primarily by fluid retention. A cholestatic liver lesion and cholangitis with abscesses developed due to a stent graft-induced stricture in the dorsal segment of the right hepatic duct and the stricture diminished following percutaneous drainage. Endoscopic drainage was performed after unsuccessful removal of the percutaneous catheter resulting in a bilio-cutaneous fistula. Although the liver graft now functions well, the stricture remains refractory even after 44 mo of treatment.
CONCLUSIONS: Biliary strictures caused by TIPS in both transplanted and native livers seem refractory to endoscopic treatment.