Prevalence, management and efficacy of treatment in portal vein obstruction after paediatric liver transplantation: protocol of the retrospective international multicentre PORTAL registry.

INTRODUCTION: Portal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management practices and efficacy of treatment in patients with PVO. METHODS AND ANALYSIS: The Portal vein Obstruction Revascularisation Therapy After Liver transplantation registry will facilitate an international, retrospective, multicentre, observational study, with 25 centres around the world already actively involved. Paediatric patients (aged <18 years) with a diagnosed PVO between 1 January 2001 and 1 January 2021 after liver transplantation will be eligible for inclusion. The primary endpoints are the prevalence of PVO, primary and secondary patency after PVO intervention and current management practices. Secondary endpoints are patient and graft survival, severe complications of PVO and technical success of revascularisation techniques. ETHICS AND DISSEMINATION: Medical Ethics Review Board of the University Medical Center Groningen has approved the study (METc 2021/072). The results of this study will be disseminated via peer-reviewed publications and scientific presentations at national and international conferences. TRIAL REGISTRATION NUMBER: Netherlands Trial Register (NL9261).

Quality of life of children with neurological impairment who receive a fundoplication for gastroesophageal reflux disease.

BACKGROUND: Children with neurological impairment (NI) commonly have gastroesophageal reflux disease (GERD) treated with a fundoplication. The impact of this procedure on quality of life is poorly understood. OBJECTIVES: To examine the quality of life of children with NI who have received a fundoplication for GERD and of their caregivers. METHODS: The study was a prospective cohort study of children with NI and GERD who underwent a fundoplication at a children's hospital between January 1, 2005, and July 7, 2006. Quality of life of the children was assessed with the Child Health Questionnaire (CHQ) and of the caregivers with the Short-Form Health Survey Status (SF-36) and Parenting Stress Index (PSI), both at baseline and 1 month after fundoplication. Functional status was assessed using the WeeFIM. Repeated-measures analyses were performed. RESULTS: Forty-four of the 63 parents (70%) were enrolled. The median WeeFIM score was 31.2 versus the age-normal score of 83 (P = .001). Compared with the baseline scores, mean CHQ scores improved over 1 month in the domains of bodily pain (32.8 vs. 47.5, P = .01), role limitations-physical (30.6 vs. 56.6, P = .01), mental health (62.7 vs. 70.6, P = .01), family limitation of activities (43.3 vs. 55.1, P = .03), and parental time (43.0 vs. 55.3, P = .03). The parental SF-36 domain of vitality improved from baseline over 1 month (41.3 vs. 48.2, P = .001), but there were no changes from baseline in Parenting Stress scores. CONCLUSIONS: Parents reported that the quality of life of children with NI who receive a fundoplication for GERD was improved from baseline in several domains 1 month after surgery. The quality of life and stress of caregivers did not improve in nearly all domains, at least in the short term.

Long-term, randomized comparison of balloon angioplasty and surgery for native coarctation of the aorta in childhood.

BACKGROUND: The purpose of this study was to compare the long-term outcomes of children randomized to surgery or balloon angioplasty (BA) for native coarctation (CoA). A prior randomized, short-term comparison of BA and surgery for native CoA in 36 children demonstrated equivalent relief of obstruction. The risk of aneurysm formation and possibly restenosis was higher among patients treated with BA. METHODS AND RESULTS: Blood pressure, residual aortic obstruction, and exercise performance were evaluated. Need for repeat intervention was reviewed. Aortic arch anatomy was assessed with magnetic resonance angiography. For subjects who were not available to return for evaluation, the most recent clinical record was utilized. Among the 36 subjects initially randomized, 21 returned for evaluation (11 BA, 10 surgery). The average time since initial intervention to evaluation for all subjects was 10.6+/-4.7 years for BA subjects and 11.3+/-3.7 years for surgical subjects. Resting blood pressure, CoA gradient, exercise performance, MRI analysis of the aortic arch, and need for repeat interventions were not different for the 2 treatment strategies. There was a higher incidence of aneurysm formation (35% versus 0%) and a greater difference in blood pressure between the right and left legs with exercise among BA subjects. Some aneurysms developed late, first being detected more than 5 years after the initial intervention. Only 50% of BA subjects remained free of both aneurysm formation and repeat intervention compared with 87.5% of surgical subjects (P=0.03). CONCLUSIONS: BA for the treatment of childhood CoA is associated with a higher incidence of aneurysm formation and iliofemoral artery injury than surgery. These differences should be considered when undertaking treatment for native CoA during childhood.

Percutaneous cholecysto-cholangiography in the diagnosis of obstructive jaundice in infants.

BACKGROUND: Once it is established that a jaundiced infant has an elevated direct bilirubin level, the principal diagnostic concern is the differentiation of hepatocellular from obstructive cholestasis, of disorders of physiology from disorders of anatomy, and of disease that is managed medically from disease that is managed surgically. Traditional tests such as ultrasonography, liver biopsy, and technotium 99m HIDA scan are often not sufficiently discriminating. General anesthesia is required for invasive imaging with endoscopic retrograde cholangio pancreatography (ERCP) or operative cholangiogram. The authors describe a facile alternative using percutaneous cholecystocholangiography (PCC) with intravenous sedation. METHODS: Nine cholestatic infants underwent PCC (age, 27 to 73 days; mean, 44 days) after ultrasoundscan, liver biopsy, and (99mTc)HIDA scan failed to provide a definitive diagnosis. RESULTS: In the 4 infants without complete biliary filling, we found biliary atresia (3) and biliary hypoplasia (1). The biliary tree was completely opacified in 5 infants with the following diagnosis: neonatal hepatitis (2), duplication of the gallbladder (1), choledochocele (1), total parenteral nutrition (TPN) cholestasis (1). There were no complications. CONCLUSIONS: When the etiology of cholestasis remains elusive after traditional firstline tests, PCC has proven to be an accurate simple alternative in differentiating obstructive from hepatocellular causes of infantile cholestatic jaundice.

Gastric trichobezoar: an important cause of abdominal pain presenting to the pediatric emergency department.

Abdominal pain is a common presenting complaint of children seen in urgent care settings. It is the manifestation of a wide variety of disease processes ranging from benign to immediately life-threatening. Gastric bezoars are among the etiologies of chronic childhood abdominal pain that, when undiagnosed, may result acutely in serious complications, including gastric ulceration, bleeding and perforation, intussusception, and small bowel obstruction. To reinforce the importance of including this entity in the differential diagnosis of abdominal pain, we present the case of a 10-year-old girl with a history of chronic epigastric complaints who was ultimately presented with acute small bowel obstruction following fragmentation and distal migration of her gastric trichobezoar. Finally, we review and briefly summarize the current literature regarding the etiology, diagnosis, and management of this disorder in children.