Porto-sinusoidal vascular liver disorder with portal hypertension: Natural History and Long-Term Outcome.

BACKGROUND & AIMS: Current knowledge of the natural history of patients with porto-sinusoidal vascular disorder (PSVD) is derived from small studies. The aim of the present study was to determine natural history and prognostic factors using a large multicenter cohort of PSVD patients. METHODS: Retrospective multicentric study of PSVD patients and signs of portal hypertension (PH) prospectively registered in 27 centers. RESULTS: 587 patients were included, median age of 47 years and 38% were women. Four-hundred and one patient had an associated condition, that was graded as severe in 157. Median follow-up was 68 months. At diagnosis, 64% of patients were asymptomatic while 36% had a PH-related complication: PH-related bleeding in 112 patients; ascites in 117 and hepatic encephalopathy in 11. In those not presenting with bleeding, the incidence of first bleeding was of 15% at 5 years, with a 5-year rebleeding rate of 18%. Five-year cumulative incidence of new or worsening ascites was of 18% and of developing PVT of 16%. Fifty (8.5%) patients received a liver transplantation and 109 (19%) died, including 55 non-liver related death. Transplant-free survival was 97%, and 83% at 1 and 5 years. Variables independently associated with transplant-free survival were age, ascites, serum bilirubin, albumin and creatinine levels at diagnosis and severe associated conditions. This allowed the creation of a Nomogram that accurately predicted prognosis. CONCLUSIONS: Prognosis of PSVD is strongly determined by the severity of the associated underlying conditions and parameters of liver and renal function.

Incidence, predictive factors and clinical significance of development of portal vein thrombosis in cirrhosis: A prospective study.

BACKGROUND AND AIMS: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. There are few prospective studies validating risk factors for development of PVT. We analysed the incidence, factors associated with PVT development and its influence on cirrhosis decompensations and orthotopic liver transplant (OLT)-free survival. METHODS: In this prospective observational study between January 2014 and March 2019, 445 consecutive patients with chronic liver disease were screened and finally 241 with cirrhosis included. Factors associated with PVT development and its influence on cirrhosis decompensations and OLT-free survival by time dependent covariate coding were analysed. RESULTS: Majority of patients belonged to Child-Pugh class A 184 (76.3%) and the average MELD score was 10 ± 5. Previous cirrhosis decompensations occurred in 125 (52.1%), 63 (26.1%) were on NSBB and 59 (27.2%) had undergone banding for bleeding prophylaxis. Median follow-up was 29 (1-58) months. Cumulative incidence of PVT was 3.7% and 7.6% at 1 and 3 years. Previous decompensation of cirrhosis and low platelet counts but not NSBB independently predicted the development of PVT. During follow-up, 82/236 (34.7%) patients developed cirrhosis decompensations. OLT-free survival was 100% and 82.8% at 3 years, with and without PVT respectively. MELD score, but not PVT, independently predicted cirrhosis decompensations (HR 1.14; 95%CI:1.09-1.19) and OLT-free survival (HR 1.16;95%CI:1.11-1.21). CONCLUSION: Previous decompensations of cirrhosis and thrombocytopenia predict PVT development in cirrhosis suggesting a pathophysiologic role for severity of portal hypertension. PVT development did not independently predict cirrhosis decompensations or lower OLT-free survival.

Anticoagulation in Cirrhosis and Portal Vein Thrombosis Is Safe and Improves Prognosis in Advanced Cirrhosis.

BACKGROUND: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. AIMS: We analyzed the safety and effect of anticoagulant therapy (AT) on PVT recanalization and orthotopic liver transplant (OLT)-free survival. METHODS: Eighty consecutive patients from a prospective registry of cirrhosis and non-tumoral PVT at a tertiary center were analyzed. AT effect on PVT recanalization and OLT-free survival was determined by time-dependent Cox regression analysis. RESULTS: Average MELD score was 15 ± 7. Portal hypertension-related complications at PVT diagnosis were present in 65 (81.3%) patients. Isolated portal vein trunk/branch thrombosis was present in 53 (66.3%) patients. AT was started in 37 patients. AT was stopped in 17 (45.9%) patients, in 4 (10.8%) due to bleeding events. No variceal bleeding occurred while on AT. Anticoagulation was restarted in 6/17 (35.2%) patients due to rethrombosis. In 67 patients with adequate follow-up imaging, AT significantly increased the rate of PVT recanalization compared with those who did not receive anticoagulation [51.4% (18/35) vs 6/32 (18.8%), p = 0.005]. OLT-free survival after a median follow-up of 25 (1-146) months was 32 (40%). Although there was no significant effect of AT on overall OLT-free survival, OLT-free survival was higher among patients with MELD ≥ 15 receiving AT compared to those who did not (p = 0.011). Baseline MELD at PVT detection independently predicted PVT recanalization (HR 1.11, 95% CI 1.01-1.21, p = 0.027) and mortality/OLT (HR 1.12, 95% CI 1.05-1.19, p < 0.001). CONCLUSIONS: Although AT did not improve overall OLT-free survival, it was associated with higher survival in advanced cirrhosis. Anticoagulation increased PVT recanalization and should be maintained after PVT recanalization to avoid rethrombosis.

Endoscopic Management of Dysfunctioning Gastric Band after Sleeve Gastrectomy with the Luso-Cor® Esophageal Stent.

Sleeve gastrectomy (SG) can be aided by the addition of a calibration silicone ring, banded SG (BSG). It provides better weight loss than non-banded SG but with higher rate of adverse events. The aim of this case report is to further contribute to the knowledge of how to endoscopically manage these patients by placing a new esophageal stent (Luso-Cor®). A 58-year-old female with grade III obesity (weight 110 kg, BMI: 45.2 kg/m2) underwent SG in 2013. Due to the limited weight loss, a surgical calibration silicon ring was placed in 2017. In the following months, she developed recurrent and abundant postprandial regurgitation, achieving a minimum weight of 66 kg (BMI: 27.1 kg/m2). Gastroesophageal transit showed a stricture at the junction of the gastric corpus and antrum, causing gastric outlet obstruction. Endoscopy identified a regular luminal stenosis with normal mucosa, which allowed easy passage of the endoscope with slight pressure. Two sessions of endoscopic dilatation were performed, first with an 18-mm through-the-scope balloon and later with a 30-mm pneumatic balloon without symptomatic relief. A two-step endoscopic therapeutic approach was proposed to first promote intragastric ring erosion by placing a new partially covered metallic stent, Luso-Cor® esophageal stent 30/20/30 × 240 mm, and subsequently retrieve the stent, followed by cutting and retrieval of the ring. The proximal flare with a 30 mm diameter was placed in the distal esophagus and the distal edge in the prepyloric antrum. However, 2 weeks later, she complained of vomiting and abdominal fullness. Complete migration of the proximal flare of the stent into the remnant gastric fundus was seen on the contrast study. Endoscopy was performed, and the stent was easily removed. A blue calibration ring, partially eroded into the gastric lumen, was observed at the site of gastric tube stenosis. After stent removal, the patient was asymptomatic, and so conservative follow-up was decided. A follow-up endoscopy, performed 5 months later, showed complete reepithelization of the eroded ring. The patient remains asymptomatic after 3 years of follow-up and has regained weight up to 76 kg (BMI: 31.2 kg/m2). The efficacy of endoscopy on the management of ring-related adverse events has been previously reported. Small-case series describe the use of multiple pneumatic dilations or the deployment of plastic or covered metallic stents to cause erosion of the overlying mucosa, followed by cutting and retrieval of the ring. In conclusion, we believe that the mural pressure exerted by the Luso-Cor® esophageal stent, in the limited period it remained in situ, was sufficient to relieve the luminal pressure of the silicon ring, realigning the ring with the remnant gastric tube. This rare clinical entity highlights the potential role of specific metallic stents in the management of these patients.

A cirurgia bariátrica de gastrectomia vertical (sleeve gástrico) pode ser complementada pela adição de um anel restritivo de silicone ­ sleeve gástrico com anel de silastic. O acréscimo deste anel promove uma maior perda de peso, no entanto está associado a maior risco de eventos adversos. O objetivo da apresentação deste caso é contribuir para as diferentes técnicas úteis no tratamento das complicações relacionadas com o anel, através da utilização de uma prótese esofágica (Luso-Cor®).Uma doente de 58 anos, com obesidade grau III (peso 110 kg, IMC 45,2 kg/m2), foi submetida a um sleeve gástrico em 2013. Não apresentou perda de peso favorável e, em 2017, foi colocado um anel de silicone rodeando o tubo gástrico. Nos meses seguintes desenvolveu regurgitação pós-prandial recorrente e abundante, alcançando um peso mínimo de 66 kg (IMC 27,1 kg/m2). Realizou um trânsito gastroesofágico que revelou uma estenose na junção do corpo com o antro gástrico, com evidência de obstrução do esvaziamento gástrico. A endoscopia digestiva alta identificou uma estenose regular recoberta por mucosa sem lesões, com passagem do aparelho após pressão ligeira. Foram realizadas duas sessões de dilatação, inicialmente com balão trough-the-scope de 18 mm e posteriormente com balão pneumático de 30 mm. Os sintomas persistiram e, por esse motivo, foi decidido uma abordagem em dois tempos: primeiro promover a erosão intragástrica da banda para depois a seccionar e remover intraluminalmente. Nesse sentido, foi colocada uma prótese metálica esofágica parcialmente coberta, Luso-Cor® 30/20/30 × 240 mm. O segmento proximal da prótese com 30 mm de diâmetro foi colocado no esófago e o bordo distal da prótese ficou no antro pré-pilórico. No entanto, duas semanas depois, a doente queixou-se de vómitos e enfartamento precoce. O estudo radiográfico com contraste revelou migração distal da prótese, com deslocamento do segmento proximal para o corpo gástrico remanescente. A prótese foi removida endoscopicamente sem dificuldade e, na região da estenose, foi observado o anel de silicone parcialmente erosionado para o lúmen gástrico. Após remoção da prótese a doente evoluiu favoravelmente, sem novos sintomas, e, por esse motivo, foi decido seguimento sem novas intervenções. A endoscopia de seguimento, realizada cinco meses após, demonstrou reepitelização completa do anel parcialmente erosionado. A doente permanece assintomática após três anos de seguimento e voltou a ganhar peso (peso atual 76 kg, IMC 31,2 kg/m2). A eficácia da resolução endoscópica de estenoses relacionadas com anel de silicone no sleeve gástrico já foi relatada. Pequenas séries de casos utilizaram múltiplas sessões de dilatação com balão pneumático ou colocação de próteses plásticas ou metálicas cobertas para promover erosão intragástrica do anel e sua remoção. Acreditamos que a pressão mural exercida pela prótese Luso-Cor®, no curto tempo em que permaneceu in situ, foi suficiente para aliviar a obstrução, realinhando o seu diâmetro com o restante tubo gástrico. Através do relato desta entidade clínica rara, esperamos contribuir para o conhecimento das próteses metálicas específicas para o manejo destes doentes.

Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome.

BACKGROUND: Porto-sinusoidal vascular liver disorder (PSVD) is a rare disease that occasionally requires liver transplantation (LT), despite usually presenting preserved liver function. There remains a paucity of data pertaining to LT in PSVD. The aim was to identify features associated with post-LT outcomes in PSVD. METHODS: Retrospective multicentre study of 79 patients who received LT for PSVD. RESULTS: Median post-LT follow-up was 37 (range 1-261) mo. Refractory ascites 24 (30%), hepatic encephalopathy 16 (20%), and hepatopulmonary syndrome 13 (16.3%) were the most frequent indications for LT. Hepatocellular carcinoma was the indication in only 2 patients. Twenty-four patients died, 7 due to liver and 17 to non-liver related causes. Post-LT survival was 82.2%, 80.7%, and 68.6% at 1, 2, and 5 y, respectively. Post-LT survival was significantly better in patients without (n = 58) than in those with a persistent severe PSVD-associated condition (n = 21). Pre-LT hyperbilirubinemia levels and creatinine >100 µmol/L were also independently associated with poor survival. Six patients (7.6%) required a second LT. Recurrence of PSVD was confirmed by liver biopsy in only 1 patient and in 3 further patients it was likely. CONCLUSIONS: LT in PSVD is associated with an acceptable outcome in the absence of associated severe conditions. However, persistence of a severe associated condition, pre-LT high bilirubin levels, or creatinine >100 µmol/L impact outcome, and these are features that should be considered when evaluating PSVD patients for LT. PSVD recurrence is possible after LT and needs to be explored, at least, in cases of posttransplant portal hypertension.