Congenital intrahepatic aorto-portal fistula presenting with cardiac failure

Congenital intrahepatic arterio-portal fistulae (cIAPF) are rare, high-flow vascular malformations that usually present with portal hypertension. They almost never cause heart failure, unless there is associated congenital heart disease or the ductus venosus in patent. We present an unusual case of IAPF in an 11-day-old boy, who presented with features of cardiac failure associated with increased N-terminal pro-brain natriuretic peptide (NT pro-BNP). The IAPF arose directly from the aorta, separated from the hepatic artery and divided to separately supply both left and right portal veins. The ductus venosus was occluded. The IAPF was treated with embolization of the aorto-portal fistula, accessed through a direct percutaneous puncture of the fistula. Embolization was associated with an immediate clinical improvement and a rapid and sustained normalization of the NT pro-BNP level. A similar re-presentation was noted and treated with repeat embolization. The child is well on follow-up. To our knowledge, this is the first case of cIAPF, which was presented with cardiac failure when the ductus venosus has closed and has been treated successfully with direct, percutaneous transhepatic embolization of the fistula, twice. Serial clinical follow-up and ultrasonographical examinations have proven to be an effective strategy to detect recurrent fistulae. </jats:p

Congenital intrahepatic aorto-portal fistula presenting with cardiac failure.

Congenital intrahepatic arterio-portal fistulae (cIAPF) are rare, high-flow vascular malformations that usually present with portal hypertension. They almost never cause heart failure, unless there is associated congenital heart disease or the ductus venosus in patent. We present an unusual case of IAPF in an 11-day-old boy, who presented with features of cardiac failure associated with increased N-terminal pro-brain natriuretic peptide (NT pro-BNP). The IAPF arose directly from the aorta, separated from the hepatic artery and divided to separately supply both left and right portal veins. The ductus venosus was occluded. The IAPF was treated with embolization of the aorto-portal fistula, accessed through a direct percutaneous puncture of the fistula. Embolization was associated with an immediate clinical improvement and a rapid and sustained normalization of the NT pro-BNP level. A similar re-presentation was noted and treated with repeat embolization. The child is well on follow-up. To our knowledge, this is the first case of cIAPF, which was presented with cardiac failure when the ductus venosus has closed and has been treated successfully with direct, percutaneous transhepatic embolization of the fistula, twice. Serial clinical follow-up and ultrasonographical examinations have proven to be an effective strategy to detect recurrent fistulae

Treatment Strategies for the Optimal Management of Locally Advanced Pancreatic Adenocarcinoma With Curative Intent: A Systematic Review.

Historically, locally advanced pancreatic adenocarcinoma (LAPDAC) was considered a palliative condition. Advances in treatment have resulted in studies reporting survival after neoadjuvant treatment and surgery similar to earlier disease stages. However, there is no consensus on optimal LAPDAC management. Our aim was a systematic review of published evidence on LAPDAC treatment strategies with curative intent. Twenty-eight studies defining LAPDAC as per established criteria and reporting outcomes after neoadjuvant treatment with a view to resection were included. Primary outcomes were resection rate and proportion of curative resections. Secondary outcomes were postoperative mortality, progression-free survival, and overall survival. Neoadjuvant treatment varied significantly, most common being the combination of folinic acid, fluorouracil, irontecan, and oxaliplatin. Median percentage of patients proceeding to surgery after completion of neoadjuvant pathway was 33.5%. Median resection rate was 25%. Median R0 resection was 80% of resected patients. These outcomes ranged 0% to 100% across studies. Ninety-day postoperative mortality ranged from 0% to 5%. Median progression-free and overall survival for resected patients were 12.9 and 30 months, respectively, versus 13.2 months overall survival for unresected patients. In conclusion, although there is wide variability in reported LAPDAC resection rates post-neoadjuvant chemotherapy, retrospective data suggest that neoadjuvant treatment followed by surgery results in improved survival

Peri-hilar cholangiocarcinoma: results from the UK nationwide CAPBIL study

BACKGROUND: The role of liver transplantation as a treatment option for de novo resectable peri-hilar cholangiocarcinoma (pCCA) is controversial. This study investigated the outcomes following resection of early-stage pCCA in the UK. METHODS: Patients undergoing resection for pCCA between 2014 and 2022 across 22 UK centres were included. Early-stage pCCA was defined as tumour size<3cm with no nodal disease (N0) on histopathology analysis. Clinical and survival data were collated. RESULTS: Of the 450 patients included, 138 patients underwent resection for early-stage pCCA. In the early-stage pCCA group, CD ≥ IIIa morbidity was 39.1 % (n = 54) and 90-day mortality was 10.1 % (n = 14). Sixty-four (46.4 %) patients received adjuvant chemotherapy, but this was reduced in those with CD ≥ IIIa morbidity (n = 17, 31.5 %). Early-stage tumours had a significantly lower vascular invasion (n = 57, 41.3 %) and R1 margin (n = 46, 33.3 %) compared to later-stage pCCA [62.2 % (n = 194) and 54.2 % (n = 169) respectively, p < 0.001). The median disease-free and overall survival was significantly better in patients with early-stage pCCA compared to more advanced tumours (p < 0.001). Male gender (p = 0.039) and Post-Hepatectomy Liver Failure (PHLF, p = 0.010) were associated with significantly worse disease-free survival, while biliary drainage (p = 0.013), PHLF (p < 0.001) and vascular invasion (p = 0.030) were associated with significantly poorer overall survival. CONCLUSION: Resection of early-stage pCCA tumours is associated with good clinical and survival outcomes in centralised HPB centres