Advanced cystic fibrosis liver disease: Endovascular, endoscopic, radiologic, and surgical considerations.

Up to 90% of people with CF (pwCF) will have some form of hepatobiliary involvement. This manuscript aims to explore the different endovascular, endoscopic, radiological and surgical procedures available to diagnose and manage the most severe form of CF hepatobiliary involvement (CFHBI) known as advanced cystic fibrosis liver disease (aCFLD), seen in 10% of pwCF. These procedures and interventions include liver biopsy, hepatic venous pressure gradient measurement, gastrostomy tube placement to optimize nutrition, paracentesis, endoscopic variceal control of bleeding and portosystemic shunting before liver transplantation. By utilizing advanced diagnostic or surgical techniques, healthcare professionals of pwCF can more effectively manage patients with CFHBI and aCFLD and potentially improve patient outcomes.

Radiological and Surgical Treatments of Portal Hypertension.

Interventions for portal hypertension are continuously evolving and expanding beyond the realm of medical management. When complications such as varices and ascites persist despite conservative interventions, procedures including transjugular intrahepatic portosystemic shunt creation, transvenous obliteration, portal vein recanalization, splenic artery embolization, surgical shunt creation, and devascularization are all potential interventions detailed in this article. Selection of the optimal procedure to address the underlying cause, treat symptoms, and, in some cases, bridge to liver transplantation depends on the specific etiology of portal hypertension and the patient's comorbidities.

Diagnosis and management of Abernethy syndrome.

Abernethy syndrome (AS or extrahepatic portosystemic shunt) is an uncommon congenital malformation consisting of agenesis or hypoplasia of the portal vein (PV) in such a way that splanchnic venous blood drains directly into the systemic circulation through aberrant communications, resulting in a portosystemic shunt that bypasses the liver AS is an underdiagnosed condition with unknown incidence and complication rate given that symptoms are usually absent. AS identification is increasingly common because of improved imaging techniques, hence prognostic implications and clinical management need be understood. This editorial reviews the natural history of AS and its diagnostic-therapeutic implications, illustrating the process with a series of cases from our institution.

Surgical Treatment of Portal Hypertension in Children.

Introduction: Portal hypertension is a syndrome characterized by increased pressure in the portal vein system and can be caused by impaired blood flow in the portal vein, hepatic veins, or inferior vena cava. The main complications of this condition are bleeding from varicose veins of the esophagus (in our study in 100% of patients), splenomegaly with hypersplenism (in our study in 98% of patients), ascites (in our study in 1 patient). The main goal of treating portal hypertension is to prevent bleeding from esophageal varices. However, today the goal of surgical treatment of portal hypertension in children is not only to prevent the development of bleeding but also the possible restoration of intrahepatic blood flow. Materials and Methods: A retrospective analysis of the results of treatment of portal hypertension in 75 children (41 boys, 34 girls) operated in our Center for the period from 2019 to 2022 was carried out. The mean age of the patients was 7 ± 1 years. Sixty-nine patients had an extrahepatic form of portal hypertension, and 6 patients had an intrahepatic form (liver fibrosis). In 14 patients (18.6%), the operation was repeated (a vascular shunt was previously applied in another hospital; 4 children were operated on repeatedly). Results: A good result was obtained in all children, and the risk of bleeding from varicose veins of the esophagus was eliminated. Vascular bypass surgery was performed in all cases: mesoportal bypass in 17 (22.7%) patients, splenorenal bypass in 37 (49.3%) patients, mesocaval bypass in 21 (28%) patients. In 10 (13%) cases, repeated bypass surgery was required due to dysfunction or thrombosis of the previously performed bypass. In 14 (18.6%) patients with mesoportal shunts, blood flow in the liver was completely restored. Conclusions: The main method of surgical treatment of portal hypertension today is portosystemic bypass surgery, which effectively prevents bleeding from varicose veins of the esophagus. Mesoportal shunting is a definitive treatment for extrahepatic portal hypertension that restores portal perfusion of the liver.

Endoscopic Ultrasound-Guided Transgastric Shunt Obliteration for Recurrent Hepatic Encephalopathy.

INTRODUCTION: Occlusion of spontaneous portosystemic shunts (SPSSs) in patients with cirrhosis may be required in recurrent or refractory hepatic encephalopathy. We describe a novel method for occlusion of SPSS using endoscopic ultrasound (EUS). METHODS: EUS-guided transgastric shunt obliteration was performed by injecting glue and coils directly into SPSS. RESULTS: EUS-guided transgastric shunt obliteration was performed for 7 patients in 9 sessions. Complete cessation of Doppler flow was achieved in 6/7 cases. Adequate clinical response was observed in 6/7 patients. No procedure-related severe adverse events were seen. DISCUSSION: This novel technique is a potentially effective and efficient method for shunt obliteration.

Surgical management of extrahepatic portal vein obstruction in children: advantages of MesoRex shunt compared with distal splenorenal shunt.

PURPOSE: To review surgical management of extrahepatic portal vein obstruction (EHPVO) at Red Cross War Memorial Children's Hospital and compare MesoRex shunt (MRS) with distal splenorenal shunt (DSRS). METHODS: This is a single-centre retrospective review documenting pre- and post-operative data in 21 children. Twenty-two shunts were performed, 15 MRS and 7 DSRS, over an 18-year period. Patients were followed up for a mean of 11 years (range 2-18). Data analysis included demographics, albumin, prothrombin time (PT), partial thromboplastin time (PTT), International normalised ratio (INR), fibrinogen, total bilirubin, liver enzymes and platelets before the operation and 2 years after shunt surgery. RESULTS: One MRS thrombosed immediately post-surgery and the child was salvaged with DSRS. Variceal bleeding was controlled in both groups. Significant improvements were seen amongst MRS cohort in serum albumin, PT, PTT, and platelets and there was a mild improvement in serum fibrinogen. The DSRS cohort showed only a significant improvement in the platelet count. Neonatal umbilic vein catheterization (UVC) was a major risk for Rex vein obliteration. CONCLUSION: In EHPVO, MRS is superior to DSRS and improves liver synthetic function. DSRS does control variceal bleeding but should only be considered when MRS is not technically feasible or as a salvage procedure when MRS fails.

REX SHUNT IN CHILDREN: COULD DYSFUNCTION BE PROGNOSED? A RETROSPECTIVE ANALYSIS.

OBJECTIVE: The aim: To analyze retrospectively our experience of Rex shunt in children with symptomatic portal hypertension, its effect on hypersplenism regression and varices eradication, assess shunt survival and investigate risk factors, that could lead to shunt dysfunction and thrombosis. PATIENTS AND METHODS: Materials and methods: 24 children (16 males, 8 females) ,with portal hypertension included into the study. All surgeries were performed within single center in a period from January 2010 to March 2022. Follow up period was 6.75±1.19 years. RESULTS: Results: Age at diagnosis was 5.39±0.64 years. 5 (20.8%) had umbilical catheter in anamnesis. 16 (66.7%) manifested bleeding episodes as the first sign of portal hypertension. 9 (37.5%) of children manifested severe hypersplenism. Age at Rex shunting was 7.5±0.7 years. In 7 (31.8%) cases Rex shunt thrombosis occurred. 1 successful thrombectomy and 6 splenorenal shunting were performed. Kaplan-Meyer analysis showed Rex shunt survival 0.670 (95%CI 0.420-0.831). Logistic regression model indicated thrombocytes count (p=0.0423) and cytopenia (p=0.0272) as factors that could influence shunt thrombosis. Follow-up group included 18 patients. Spleen volume regression became significant by 1 p/o year p<0,05, thrombocytes significant increasement reached in 1 p/o months (p<0.01), varices involution was achieved by 1 p/o year (p<0,001). CONCLUSION: Conclusions: Rex shunt effectiveness in study group was 70.9%., shunt survival assessed 0.670 (95%CI 0.420-0.831). Rex shunt was effective in bleeding prophylaxis in all patients of follow up group. Preoperative thrombocytes count (p=0.0423) and cytopenia (p=0.0272) were detected as factors that could influence shunt thrombosis, that is to be considered in RS preoperative period and require following studies.

Congenital portopulmonary shunt in a cat.

A 9-year-old spayed female crossbreed cat with chief complaints of anorexia and hypersalivation had high serum concentrations of ammonia and fasting and postprandial total bile acid. Therefore, she was referred to our hospital. On the first evaluation, haematology, serum chemistry, radiography and ultrasonography findings suggested that she had a congenital portosystemic shunt. CT revealed a shunt vessel from the left gastric vein to the left pulmonary vein. During median celiotomy and sternotomy, gross findings and mesenteric portography revealed abnormal vessel shunting from the left gastric vein to the left pulmonary vein. Complete ligation of the shunt vessel was achieved. She recovered without any complications. Postoperative serum chemistry revealed that ammonia and total bile acid levels decreased to within the reference intervals. This report is the first to describe the clinical features and surgical outcome of a cat with a congenital portopulmonary shunt.

Cirrhosis-related Spontaneous Mesocaval Shunt.

Online supplemental material is available for this article.

The use of autologous peritoneum in surgery of portal hypertension: H-shape splenorenal shunt using simple layer peritoneal tube.

The management of portal hypertension complicated by iterative gastro-intestinal bleeding remains challenging, especially in a low-income environment. Interventional radiology and endoscopic treatments are not always accessible, and a definitive surgical option may prove to be lifesaving. We report a new technique of surgical portosystemic shunt that can be performed in all contexts. We describe the surgical technique of a H-shaped splenorenal shunt using autologous rolled up peritoneum as a vascular graft.

Functional side-to-side splenorenal shunts to treat extrahepatic portal vein thrombosis in children.

BACKGROUND: Surgical shunts are commonly used to manage complications resulting from extrahepatic portal vein thrombosis (EHPVT) in children. We describe a single-center experience utilizing a functional Side-to-Side Splenorenal Shunt (fSRS), created using either an enlarged inferior mesenteric vein (IMV) or left adrenal vein (LAV). METHODS: Pediatric patients with isolated EHPVT who were poor candidates for a Rex shunt and who underwent a fSRS procedure at our institution between 2003 and 2020 were reviewed. The pre/post shunt portosystemic gradient change, rates of early and late complications, postoperative shunt patency, and mortality were evaluated. RESULTS: Twelve EHPVT patients (mean age of 6.1 years) underwent a fSRS procedure. The mean portosystemic gradient change for the cohort was -11.7 mmHg (±4.9). There were no cases of recurrent variceal bleeding or episodes of shunt thrombosis reported after fSRS procedures. CONCLUSIONS: Surgical shunts continue to be an important adjunct in the treatment of complications related to EHPVT. The functional Side-to-Side Splenorenal Shunt is a safe alternative that is easy to perform, involves minimal dissection and requires only a single anastomosis.

A new variant of Abernethy malformation treated by transhepatic interventional closure: a case report.

BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic CPSS, also termed Abernethy malformations are associated with severe long-term complications including portopulmonary hypertension, liver atrophy, hyperammoniemia and hepatic encephalopathy. We report a hitherto undescribed variant of Abernethy malformation requiring an innovative approach for interventional treatment. CASE PRESENTATION: We describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months. CONCLUSIONS: Portal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers.

Portosystemic Shunt in Pediatric Living Donor Liver Transplant.

BACKGROUND: To evaluate the significance of portosystemic shunts and associated long-term outcomes in living donor liver transplant (LDLT) among pediatric patients. METHODS: Retrospective review of 121 pediatric patients who underwent LDLT between May 1994 and December 2015 at Taiwan Kaohsiung Chang Gung Memorial Hospital. Pre- and postoperative computed tomography images of the liver were reviewed, and portal vein complications were assessed. RESULTS: Ninety-seven pediatric patients were included in the study, and 70 had portosystemic shunts before transplant. Thirty-three patients have portal systemic shunt (PSS) 6 months after transplant (mean [SD] shunt size, 4.59 [1.98] mm). Thirty-seven patients' portosystemic shunts closed spontaneously (mean [SD] shunt size, 3.14 [1.06] mm). Smaller PSSs tend to close spontaneously with a cutoff point of 3.35 mm by receiver operating characteristic curve (P = .01). Patients with PSS have more portal vein complications than those without PSS (44.3% vs 11.1%, P = .02). Among PSS recipients, patients with portal vein complications tend to have larger PSS size (mean [SD], 4.14 [1.96] mm vs 3.59 [1.48] mm), although the difference is not statistically significant (P = .19). CONCLUSIONS: In pediatric patients, preoperative portosystemic shunts are significantly correlated with portal venous complications, some of which require minimal interventions after LDLT with good outcomes. Shunts larger than 3.35 mm tend to persist after transplant with increased portal venous complications.

A common celiacomesenteric trunk is a rare anatomic variant in dogs.

With the increasing use of CT and MRI for diagnostic imaging and planning of interventional procedures, it is important for veterinary radiologists to be familiar with variations in normal vascular anatomy and not mistake them for pathology. The arterial blood supply to the cranial abdominal viscera is provided by the celiac and the cranial mesenteric arteries. A common celiacomesenteric trunk (CMT) has been reported as a rare anatomical variant in dogs. The goals of this retrospective, observational, cross-sectional prevalence study were to determine the prevalence of a CMT in dogs with non-abdominal disease and compare it to the prevalence in dogs with portosystemic shunts (PSS). Magnetic resonance imaging studies of the thoracolumbar and lumbosacral spine in dogs that included the origin of the celiac and cranial mesenteric arteries and dual-phase CT angiography studies of the abdomen in dogs with portosystemic shunts were retrospectively reviewed by a veterinary student and a board-certified veterinary radiologist. The prevalence of a CMT was determined as the proportion of dogs diagnosed with this vascular anomaly in the MRI and CT group, respectively. Fisher's exact test was used to determine any association of a CMT with the concurrent presence of a PSS, sex, and breed size. A CMT was identified in seven of 606 (1.2%) MRI studies and in none of 47 abdominal CT studies. There was no association between the presence of a CMT and PSS (P = 1.000), sex (P = .4694), or breed size (P = 1.000). A CMT is a rare incidental finding in dogs.

Abernethy malformation: A comprehensive review.

Abernethy malformation is a rare condition in which portomesenteric blood bypasses the liver and drains into the systemic vein through a partial or complete shunt. It is categorised into two types on the basis of the shunt pattern between the portal vein and systemic vein. Abernethy malformation is associated with multiple congenital anomalies and acquired complications. A detailed understanding of anatomy and embryology is a prerequisite to interpret the imaging findings. Computed tomography and magnetic resonance angiography can delineate the shunt anatomy and evaluate the concomitant malformations. It is essential to differentiate Abernethy malformation from intrahepatic portosystemic shunts and acquired extrahepatic portosystemic shunts. Mild metabolic abnormalities are treated with dietary modifications and medical therapy. Definitive treatment is done in symptomatic patients. Generally, type I Abernethy patients undergo liver transplantation, and type II undergo shunt occlusion by surgery or transcatheter coiling.

Microbiota transplants from feces or gut content attenuated portal hypertension and portosystemic collaterals in cirrhotic rats.

Liver cirrhosis and portal hypertension is the end of chronic liver injury with hepatic, splanchnic and portosystemic collateral systems dysregulation. Liver injury is accompanied by gut dysbiosis whereas dysbiosis induces liver fibrosis, splanchnic angiogenesis and dysregulated vascular tones vice versa, making portal hypertension aggravated. It has been proved that intestinal microbiota transplantation alleviates dysbiosis. Nevertheless, the influences of microbiota transplantation on cirrhosis-related portal hypertension are not so clear. Liver cirrhosis with portal hypertension was induced by bile duct ligation (BDL) in rats. Sham rats were surgical controls. Rats randomly received vehicle, fecal or gut (terminal ileum) material transplantation. The results showed that microbiota transplantation from feces or gut material significantly reduced portal pressure in cirrhotic rats (P=0.010, 0.044). Hepatic resistance, vascular contractility, fibrosis and relevant protein expressions were not significantly different among cirrhotic rats. However, microbiota transplantation ameliorated splanchnic hyperdynamic flow and vasodilatation. Mesenteric angiogenesis, defined by whole mesenteric window vascular density, decreased in both transplantation groups and phosphorylated endothelial nitric-oxide synthase (eNOS) was down-regulated. Portosystemic shunts determined by splenorenal shunt (SRS) flow decreased in both transplantation groups (P=0.037, 0.032). Shunting severity assessed by microsphere distribution method showed consistent results. Compared with sham rats, cirrhotic rats lacked Lachnospiraceae. Both microbiota transplants increased Bifidobacterium. In conclusion, microbiota transplantation in cirrhotic rats reduced portal pressure, alleviated splanchnic hyperdynamic circulation and portosystemic shunts. The main beneficial effects may be focused on portosystemic collaterals-related events, such as hepatic encephalopathy and gastroesophageal variceal hemorrhage. Further clinical investigations are mandatory.