Cystic fibrosis and portal hypertension: Distal splenorenal shunt can prevent the need for future liver transplant.

BACKGROUND: The management of portal hypertension (PHT) in children with well compensated cirrhosis and cystic fibrosis (CF) is controversial. We present our experience with distal splenorenal shunting (DSRS) for the treatment of PHT as an alternative to liver transplantation (LT). METHODS: Between 2008 and 2017, 5 CF children underwent a DSRS at a pediatric hepatobiliary and transplantation referral center. LT (n = 9) was reserved for patients with decompensated cirrhosis. Statistical analysis was done using the paired t-test (p < 0.05 considered significant). RESULTS: Mean PELD/MELD score was significantly lower for DSRS patients than LT (3 ±â€¯6 vs 28 ±â€¯4, p < 0.001). All 5 DSRS patients had grade III-IV varices. One bled prior to surgery. After DSRS, spleen size decreased significantly from 8.4 ±â€¯1.5 cm to 4.4 ±â€¯1.8 cm (p = 0.019). Mean platelet count remained stable (87.8 ±â€¯48 to 91.8 ±â€¯35, p = 0.9). There were no postoperative complications. No DSRS patient experienced variceal bleeding following shunt creation. Liver function tests remained stable in the DSRS group, and no patient required a liver transplant (median follow up 4.65 years, range 1.24-7.79). CONCLUSIONS: Patients with cystic fibrosis who have well-compensated cirrhosis and symptomatic portal hypertension can be palliated with distal splenorenal shunting and do not need liver transplants. These patients can undergo shunting with minimal morbidity. TYPE OF STUDY: Case series with no comparison group. LEVEL OF EVIDENCE: IV.

Transition from hospital to home following pediatric solid organ transplant: qualitative findings of parent experience.

Transplant providers are challenged to determine appropriate interventions for patients and families due to limited published research regarding the context of the post-discharge experience from the perspective of parents of transplanted children. The purpose of this study is to describe the parent perspective of the transition from hospital to home following their child's solid organ transplant. Within a mixed-methods design, 37 parents of pediatric heart, kidney, and liver transplant recipients from three pediatric hospitals responded to qualitative interview questions on the day of hospital discharge and three wk following hospital discharge. Insight to the discharge preparation process revealed necessary education components. Post-discharge themes were identified for coping, knowledge, and adherence. The parents' responses provide awareness as to specific stressors and concerns parents are faced with when their child is discharged from the hospital after solid organ transplant and opportunities for ways the transplant team can provide support.

Essential components of transition to adult transplant services: the transplant coordinators' perspective.

CONTEXT: Medical and surgical advancements have resulted in improved long-term survival of pediatric liver transplant recipients. As pediatric patients approach school age and adolescence, transplant centers are challenged to facilitate the process of transitioning from pediatric to adult centers. OBJECTIVE: To describe pediatric and adult liver transplant coordinators' perspective regarding practice for transitioning patients to adult-oriented transplant centers. DESIGN: Descriptive SETTING: Pediatric and adult liver transplant coordinators associated with Studies of Pediatric Liver Transplantation. PARTICIPANTS: A total of 35 pediatric liver transplant coordinators and 24 adult liver transplant coordinators completed the survey. METHOD: Investigator-developed survey to identify current practice for transition process and actual transfer of a patient from pediatric to adult care. RESULTS: Transplant coordinators play an integral role in the transition process, and study results highlight what experienced coordinators believe are important considerations for a successful transition process. Results also highlight the importance of communication and partnership between the pediatric and adult programs.

Graft histology characteristics in long-term survivors of pediatric liver transplantation.

The factors that influence the long-term histological outcome of transplanted liver allografts in children are not yet fully understood, and the role of surveillance biopsies in patients with normal graft function remains controversial. The aims of this study were to describe the long-term graft histology of pediatric liver transplant recipients surviving at least 3 years and to analyze factors correlating with long-term histological outcome. Histological slides of 63 long-term liver transplant recipients were assessed for inflammation and fibrosis. The histological findings were correlated with clinical, biochemical, serological, and radiological findings. A significant proportion of biopsies from these patients showed some type of histological abnormalities, with fibrosis being observed in 61 (97%) patients. Duration of transplantation of >6 years and > or =grade 2 inflammation were significantly associated with advanced fibrosis. We could not identify any correlation between > or =stage 3 fibrosis and donor age, cold and warm ischemia time, history of de novo autoimmune hepatitis, hepatic artery thrombosis, chronic rejection, or alanine aminotransferase, aspartate aminotransferase, and gamma-glutamyl transferase values. In conclusion, liver fibrosis appears to be a common finding in long-term pediatric liver transplant survivors. The cause of this fibrosis is uncertain, and normal alanine aminotransferase, aspartate aminotransferase, and gamma-glutamyl transferase levels do not exclude the presence of significant fibrosis.

Correction of extrahepatic portal vein thrombosis by the mesenteric to left portal vein bypass.

OBJECTIVE: The goal of this study was to determine the effectiveness of mesenteric vein to left portal vein bypass operation (MLPVB) in correcting extrahepatic portal vein thrombosis (EHPVT) in children. The treatment of idiopathic EHPVT has been primarily palliative, whereas MLPVB restores hepatic portal flow in patients with EHPVT. METHODS: Thirty-four children with symptomatic EHPVT underwent surgery with intent to perform MLPVB and were followed for up to 7 years. MLPVB was successful in 31 patients (91%), all of whom maintain patent vein grafts and have symptomatic relief of EHPVT in follow-up. All patients had complete relief from gastrointestinal bleeding. Patients with hypersplenism had significant increases in platelet and leukocyte counts and reduction in spleen size. Superior mesenteric vein flow increased from 119 +/- 66 mL/min before bypass to 447 +/- 225 mL/min (P < 0.0001) after surgery. Postoperative blood flow in the bypass graft expressed as a fraction of calculated ideal portal flow for size correlated inversely with age (P < 0.001). Left-portal vein diameter increased from 2.6 +/- 1.6 mm to 7.3 +/- 2.4 mm 2 years after surgery (P < 0.002). Liver volume increased from 703 +/- 349 cm3 to 799 +/- 351 cm3 1 week after surgery (P < 0.001). Prothrombin time improved to normal in all patients 1 year after surgery. CONCLUSIONS: MLPVB provides excellent relief of symptoms in children with idiopathic EHPVT and results in liver growth and normalization of coagulation parameters. This surgery is corrective and should be done at as early an age as possible.

Surgically restoring portal blood flow to the liver in children with primary extrahepatic portal vein thrombosis improves fluid neurocognitive ability.

OBJECTIVES: Children with primary extrahepatic portal vein thrombosis (EHPVT) have portal-systemic shunting, which may lead to disturbed neurocognitive function similar to portal-systemic encephalopathy (PSE) seen with chronic liver disease and cirrhosis. The functions most affected are those involving fluid cognitive ability, which comprise neurocognitive domains such as attention, processing speed, and short-term memory, that are particularly vulnerable to systemic illness or diffuse neurologic insult. We determined the fluid cognitive ability of children with EHPVT and whether surgically restoring portal blood flow by mesenteric left portal vein bypass (MLPVB) improved it. DESIGN: Twelve children with EHPVT and no overt PSE underwent comprehensive neurocognitive testing before and 1 year after undergoing surgery with intent to perform MLPVB. The evaluations sampled 4 functional domains at both time points: (1) neurobehavioral (behavior, emotional, executive functioning); (2) broad cognitive (intelligence, achievement); (3) fluid ability (attention, mental speed, working memory, memory encoding); and (4) visual motor (drawing, fine motor). Tasks in the fluid-ability and visual-motor domains were expected to be especially sensitive to adverse effects of EHPVT and to be most likely to show improvement with MLPVB. The test group consisted of 8 subjects who underwent successful MLPVB, and the comparison group was composed of 3 patients who received distal splenorenal shunts and one whose MLPVB failed. RESULTS: Both groups demonstrated similar fluid cognitive ability at initial evaluation. Successful MLPVB resulted in significantly improved fluid cognitive function: in the fluid cognitive domain, significant improvements were seen for the hit reaction time variability in the Conner's Continuous Performance Test, the attention scale of the Cognitive Assessment System, and immediate verbal memory in the Children's Memory Scale. In the visual-motor domain, z scores on the Grooved Pegboard Test improved. No improvement was observed in the comparison group. DISCUSSION: The results show that surgically restoring portal flow to the liver in children with primary EHPVT results in improved fluid cognitive ability. Subjects showed some neurocognitive abnormalities involving mainly fluid cognitive ability consistent with minimal PSE seen in adults with chronic liver disease. Cognitive defects in patients with minimal PSE seem to relate primarily to attention and fine motor skill, and although affected patients can function in everyday life, they are at risk for performance deficits in educational and vocational situations requiring the ability to pay close attention and react quickly (eg, driving, employment in manufacturing). The tests we administered in these domains are pediatric equivalents to measures used to detect minimal PSE in adults and should detect abnormalities in the same functional domains. Our results suggest that a narrow battery of tests could be used to detect minimal PSE in children in a manner similar to the 5-test battery used in adults, eliminating the need for the comprehensive and broad testing we performed. Our findings suggest that shunting of portal blood from the liver in primary EHPVT can result in PSE and question whether it is as benign a disease as previously thought. The importance of our findings is twofold. For understanding the pathophysiology of PSE, we have shown that restoring blood flow to the liver improves cognitive function in children with EHPVT. For therapy for EHPVT, it becomes clear that MLPVB is an excellent treatment option. It is effective for treating the complications of portal hypertension and provides effective portal blood flow that other medical and surgical therapies do not. The findings provide additional evidence that primary EHPVT should be considered curable by MLPVB. However, comparison of overall risks and benefits of MLPVB with those of other therapeutic options and longer-term outcome studies must be completed before MLPVB can be fully endorsed as the best treatment for EHPVT in children. CONCLUSIONS: Surgical restoration of portal venous flow to the liver in children with primary EHPVT by MLPVB improves fluid cognitive ability. MLPVB should be considered in treating primary EHPVT, because it corrects portal blood flow and could optimize learning potential.