European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (2) intraoperative imaging.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of intraoperative and postoperative complications. So far, only limited data regarding the best radiological approach to monitor children during liver transplantation is available. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra- and postoperative phase. This paper reports the responses related to intraoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: Intraoperative ultrasound (US) is used by all sites to assess the quality of the vascular anastomosis in order to ensure optimal perfusion of the liver transplant. Vessel depiction is commonly achieved using color Doppler (95.3%). Additional US-based techniques are employed by fewer centers (power angio mode, 28.6%; B-flow, 19%; contrast-enhanced US, 14.3%). Most centers prefer a collaborative approach, with surgeons responsible for probe handling, while radiologists operate the US machine (47.6%). Less commonly, the intraoperative US is performed by the surgeon alone (28.6%) or by the radiologist alone (23.8%). Timing of US, imaging frequency, and documentation practices vary among centers. CONCLUSION: Intraoperative US is consistently utilized across all sites during pediatric liver transplantation. However, considerable variations were observed in terms of the US setup, technique preferences, timing of controls, and documentation practices. These differences provide valuable insights for future optimization and harmonization studies.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (3) postoperative imaging.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of postoperative complications. So far, limited data is available regarding the best radiologic approach to monitor children after liver transplantation. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phases. This paper reports the responses related to postoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: All sites commence ultrasound (US) monitoring within 24 h after liver transplantation. Monitoring frequency varies across sites, ranging from every 8 h to 72 h in early, and from daily to sporadic use in late postoperative phases. Predefined US protocols are used by 73% of sites. This commonly includes gray scale, color Doppler, and quantitative flow assessment. Alternative flow imaging techniques, contrast-enhanced US, and elastography are applied at 31.8%, 18.2%, and 63.6% of sites, respectively. Computed tomography is performed at 86.4% of sites when clarification is needed. Magnetic resonance imaging is used for selected cases at 36.4% of sites, mainly for assessment of biliary abnormalities or when blood tests are abnormal. CONCLUSION: Diagnostic imaging is extensively used for postoperative surveillance of children after liver transplantation. While US is generally prioritized, substantial differences were noted in US protocol, timing, and monitoring frequency. The study highlights potential areas for future optimization and standardization of imaging, essential for conducting multicenter studies.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (1) pre-transplant evaluation.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment in end-stage liver disease. Imaging is a key element for successful organ-transplantation to assist surgical planning. So far, only limited data regarding the best radiological approach to prepare children for liver transplantation is available. OBJECTIVES: In an attempt to harmonize imaging surrounding pediatric liver transplantation, the European Society of Pediatric Radiology (ESPR) Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phase. This paper reports the responses on preoperative imaging. MATERIAL AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted and 22 institutions from 11 countries returned the survey. From 2018 to 2020, the participating centers collectively conducted 1,524 transplantations, with a median of 20 transplantations per center per annum (range, 8-60). RESULTS: Most sites (64%) consider ultrasound their preferred modality to define anatomy and to plan surgery in children before liver transplantation, and additional cross-sectional imaging is only used to answer specific questions (computed tomography [CT], 90.9%; magnetic resonance imaging [MRI], 54.5%). One-third of centers (31.8%) rely primarily on CT for pre-transplant evaluation. Imaging protocols differed substantially regarding applied CT scan ranges, number of contrast phases (range 1-4 phases), and applied MRI techniques. CONCLUSION: Diagnostic imaging is generally used in the work-up of children before liver transplantation. Substantial differences were noted regarding choice of modalities and protocols. We have identified starting points for future optimization and harmonization of the imaging approach to multicenter studies.

Bone Mineral Density and Vascular Calcification in Children and Young Adults With CKD 4 to 5 or on Dialysis.

Introduction: Older adults with chronic kidney disease (CKD) can have low bone mineral density (BMD) with concurrent vascular calcification. Mineral accrual by the growing skeleton may protect young people with CKD from extraosseous calcification. Our hypothesis was that children and young adults with increasing BMD do not develop vascular calcification. Methods: This was a multicenter longitudinal study in children and young people (5-30 years) with CKD stages 4 to 5 or on dialysis. BMD was assessed by tibial peripheral quantitative computed tomography (pQCT) and lumbar spine dual-energy X-ray absorptiometry (DXA). The following cardiovascular imaging tests were undertaken: cardiac computed tomography for coronary artery calcification (CAC), ultrasound for carotid intima media thickness z-score (cIMTz), pulse wave velocity z-score (PWVz), and carotid distensibility for arterial stiffness. All measures are presented as age-adjusted and sex-adjusted z-scores. Results: One hundred participants (median age 13.82 years) were assessed at baseline and 57 followed up after a median of 1.45 years. Trabecular BMD z-score (TrabBMDz) decreased (P = 0.01), and there was a nonsignificant decrease in cortical BMD z-score (CortBMDz) (P = 0.09). Median cIMTz and PWVz showed nonsignificant increase (P = 0.23 and P = 0.19, respectively). The annualized increase in TrabBMDz (ΔTrabBMDz) was an independent predictor of cIMTz increase (R 2 = 0.48, ß = 0.40, P = 0.03). Young people who demonstrated statural growth (n = 33) had lower ΔTrabBMDz and also attenuated vascular changes compared with those with static growth (n = 24). Conclusion: This hypothesis-generating study suggests that children and young adults with CKD or on dialysis may develop vascular calcification even as their BMD increases. A presumed buffering capacity of the growing skeleton may offer some protection against extraosseous calcification.

The burden of subclinical cardiovascular disease in children and young adults with chronic kidney disease and on dialysis.

BACKGROUND: Cardiovascular disease (CVD) is a common cause of morbidity and mortality even in young people with chronic kidney disease (CKD). We examined structural and functional CV changes in patients ˂30 years of age with CKD Stages 4 and 5 and on dialysis. METHODS: A total of 79 children and 21 young adults underwent cardiac computed tomography for coronary artery calcification (CAC), ultrasound for carotid intima-media thickness (cIMT), carotid-femoral pulse wave velocity (cfPWV) and echocardiography. Differences in structural (CAC, cIMT z-score, left ventricular mass index) and functional (carotid distensibility z-score and cfPWV z-score) measures were examined between CKD Stages 4 and 5 and dialysis patients. RESULTS: Overall, the cIMT z-score was elevated [median 2.17 (interquartile range 1.14-2.86)] and 10 (10%) had CAC. A total of 16/23 (69.5%) patients with CKD Stages 4 and 5 and 68/77 (88.3%) on dialysis had at least one structural or functional CV abnormality. There was no difference in the prevalence of structural abnormalities in CKD or dialysis cohorts, but functional abnormalities were more prevalent in patients on dialysis (P < 0.05). The presence of more than one structural abnormality was associated with a 4.5-fold increased odds of more than one functional abnormality (95% confidence interval 1.3-16.6; P < 0.05). Patients with structural and functional abnormalities [cIMT z-score >2 standard deviation (SD) or distensibility <-2 SD) had less carotid dilatation (lumen:wall cross-sectional area ratio) compared with those with normal cIMT and distensibility. CONCLUSIONS: There is a high burden of subclinical CVD in young CKD patients, with a greater prevalence of functional abnormalities in dialysis compared with CKD patients. Longitudinal studies are required to test these hypothesis-generating data and define the trajectory of CV changes in CKD.

Ultrasound-guided percutaneous insertion of Broviac lines in infants less than 5kg: Prospective study of 100 consecutive procedures.

AIM: Ultrasound-guided (USG) percutaneous insertion of Broviac lines (cuffed tunnelled silastic central venous catheters, TCVC) has increasingly been adopted throughout the UK. However, vascular access remains a challenge in small babies and in some units is still performed by open cutdown. Our vascular access team, established in 2004, consists of consultant surgeons, anaesthetists and interventional radiologists, who provide all permanent vascular access by the USG technique. We reviewed the outcome in our last 100 patients less than 5 kg. METHOD: A prospective database of TCVC insertions in patients <5 kg weight recorded age, gestation, weight, diagnosis, type of catheter and complications within 28 days of insertion. A standardised technique of USG insertion is used by all operators. RESULTS: One-hundred patients <5 kg had TCVC inserted between 1/1/2018 and 31/3/2020. Median age 46(range0-316)days, gestation 36.5(23-42)weeks, weight 3(0.66 to 5)kg. INDICATION: parenteral nutrition(75), long term antibiotics(14), cardiac medication(6), chemotherapy(3), other(2). All were tunnelled silicone lines of single 2.7fr(51) and 4.2fr(46) or double lumen 7fr(3). Uncomplicated insertion in 94/100 cases. In 6 patients difficulties were encountered with cannulating the vein. In 4 cases an experienced colleague was called and managed to cannulate the vein; in 1 case a new successful attempt was made on the opposite internal jugular vein, and in 1 the femoral vein was used. No patient required an open cutdown. There were no cases of line sepsis requiring removal but 1 replacement was required for blockage within 28days. CONCLUSION: The USG approach in infants<5 kg is safe and can be used exclusively for venous access even in the most tiny babies. It is, however, a technically challenging procedure therefore we would recommend establishing a consultant delivered vascular access team to provide this service. Open venous cutdown in a tertiary children's hospital is no longer necessary for the insertion of TCVC and should be abandoned altogether. LEVELS OF EVIDENCE: Level I Prognosis Study.

Interventional radiology and open surgery: An effective partnership for solid organ trauma.

BACKGROUND: Management algorithms of paediatric blunt abdominal solid organ injury (BASOI) are evolving to include interventional radiology, but there are few studies documenting the application and clinical outcomes of cases in children. METHODS: A retrospective case note review of all paediatric BASOI at a single Paediatric Major Trauma Centre was completed. CT scans and injuries have been retrospectively graded according to AAST guidelines. RESULTS: In the period February 2012 - October 2019, there were 106 children (median age 10.6 years (range 10 days - 16 years)) with BASOI. Of these, 71% (n = 75) suffered liver injuries, 29% (n = 31) spleen, and 27% (n = 29) renal. 95 children (89.6%) were treated with non-operative management, of which 15% (n = 14) went on to require secondary operative management (surgery, n = 1 & interventional radiology, n = 14). There were no deaths or loss of organ in the group which required secondary operative management, regardless of the grade of injury. CONCLUSION: The majority of BASOI can be successfully treated conservatively, but IR is a useful additional tool in management for all grades of injury and is complementary to open surgery. LEVEL OF EVIDENCE: Level IV Case Series.

Bilateral hydroureteronephrosis: A neonatal presentation of bladder duplication.

In a male neonate with bilateral hydroureteronephrosis, the most common surgical diagnosis is posterior urethral valves. This case report describes a male infant with the same presentation, but caused by a very uncommon congenital anomaly. The summation of different imaging modalities allowed a multidisciplinary team of colleagues to define the anatomy: bilateral duplex kidneys draining into separate urinary bladders. Only one of the bladders had an outlet, hence the obstructive uropathy to the right kidney led to total loss of function. The distended tortuous ureters produced a mass effect at presentation. This case acts as a reminder that complex congenital anomalies can mimic the presentation of more common conditions, and that they often require input from various specialists to diagnose the condition and guide its management.

Supratentorial vs infratentorial posterior calvarial distraction osteogenesis for the increase of ICV in children with syndromic or multi-suture craniosynostosis: a retrospective cohort study.

PURPOSE: Craniosynostosis is the premature and pathological fusion of calvarial sutures. One modality of surgical treatment of syndromic craniosynostosis is posterior calvarial distraction (PCD). This can be either supratentorial or infratentorial. Currently, supratentorial PCD may be regarded as safer but produces a smaller increase in calvarial volume compared to infratentorial PCD. This study quantifies and compares the effectiveness of supratentorial and infratentorial PCD to help guide surgical decision-making. METHODS: The CT and/or MRI scans of 47 cases of craniosynostosis who underwent PCD from the Birmingham Children's Hospital (BCH) were converted to sagittal series multi-planar reformatted (MPR) scans for the manual calculation of ICV. The 47 cases were classified as having undergone either supratentorial or infratentorial PCD using lateral plain film radiographs, with 28 and 32 pairs of pre- and post-operative CT/MRI scans reviewed respectively. RESULTS: A statistically significant difference between supratentorial and infratentorial PCD was observed for the increase in supratentorial volume (STV) (P = 0.0458) and total intracranial volume (TICV) (P = 0.0437), but not for the increase in infratentorial volume (ITV) (P = 0.0697). The relationship for each volume trended towards convergence but was not achieved before the physical limit of 30 mm distraction had been reached. Intraclass correlation coefficient values for agreement of MRI and CT scans for STV, ITV and total ICV were 0.852, 0.864 and 0.854 respectively. CONCLUSION: Our evidence suggests that supratentorial PCD is more effective for increasing ICV in a clinical setting. CT and MRI imaging modalities are acceptably clinically interchangeable for calculating ICV in craniosynostosis.

Fifteen-Year Single-Center Experience of Biliary Complications in Liver Trauma Patients: Changes in the Management of Posttraumatic Bile Leak.

INTRODUCTION: Management of posttraumatic bile leak has evolved over time in our unit, from endoscopic retrograde cholangiopancreatography (ERCP) stenting to intraperitoneal drainage (IPD) alone as first-line treatment for intraperitoneal bile leak. MATERIALS AND METHODS: Retrospective review of liver trauma patients from 2002 to 2017. Demographics, time and mode of diagnosis of bile leak, management, and outcome were analyzed of the box plot. RESULTS: In 118 patients, there were 28 traumatic bile leaks. Eighteen were free intraperitoneal and 10 were localized bilomas. The median time of diagnosis was 6 days following injury. The modes of diagnosis were preemptive hepatobiliary scintigraphy (18), computed tomography (CT) or ultrasound (7), and laparotomy (3). Free intraperitoneal biliary leak management included 11 IPD alone, 3 IPD plus ERCP, 2 IPD plus transcystic biliary stent (TBS), 1 operative cholangiogram, and 1 no intervention. Median time of IPD duration was 7 days (4-95) in IPD alone versus 14 days (6-40) in IPD + ERCP/TBS (p = 0.3). Median inpatient length of stay was 13 days (8-44) in IPD alone versus 12 days (8-22) in IPD + ERCP/TBS (p = 0.4). CONCLUSION: Placement of IPD alone, as first-line treatment, is safe and effective in the management of intraperitoneal bile leaks, avoiding the costs and potential complications of ERCP.

Liver Tumours in Children: The Hepatologist's View.

ABSTRACT: Diagnostic and therapeutic innovations have changed the way we now approach liver tumours in children and adolescents. Novel imaging tools, increasing awareness, and surveillance has led to early diagnosis of benign and malignant liver tumours. Multidisciplinary interventions have favourably altered the natural course in some liver tumours. The role of liver transplantation is expanding and has become fully integrated into today's therapeutic algorithms. Transarterial locoregional and ablation therapies have been successful in adults and are being explored in children to facilitate resectability and improve outcome. For the first time, North American, Japanese, and European experts have designed a global trial to optimize management of malignant liver tumours and aim to find signature molecular profiles that will translate to individualised treatment strategies.This article aims to offer an overview of recent advances in our understanding of liver tumours in children. It focuses on the paediatric hepatologist's view and their role in the multidisciplinary management of benign and malignant liver tumours.

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.

Morphology of vascular ring arch anomalies influences prognosis and management.

OBJECTIVE: This study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of symptomatic presentation and surgical intervention. METHODS: This was a retrospective observational study at a single cardiac unit. Individuals diagnosed with an aortic arch anomaly, either isolated or non-isolated, between June 2014 and September 2018 were included. The morphology of the aortic arch was established via analysis of postnatal echocardiography, CT or MRI scans. CT and magnetic resonance studies were evaluated for the presence of a Kommerell diverticulum in those with aberrant vessels. Case notes were reviewed for relevant clinical data. RESULTS: Of those with aberrant subclavian arteries, 24/79 (30.4%) were shown to have a Kommerell diverticulum. Additional forms of congenital heart disease were present in 133/227 (58.6%) individuals. Surgical division of the vascular ring was performed in 30/227 (13.2%), most commonly in the setting of a double aortic arch (70.8%). In those with aberrant subclavian arteries, no children without a Kommerell diverticulum were referred for surgery. In those with a Kommerell diverticulum confirmed on imaging, 11/24 underwent surgery. CONCLUSION: Individuals with a double aortic arch, or an aberrant subclavian artery arising from a Kommerell diverticulum, have the highest requirement for surgical intervention, especially in isolated anomalies. These individuals should remain under monitoring. The subjective nature of symptoms remains problematic. Longitudinal research is required further to understand the natural history of vascular rings and how it links to morphology.

Biodegradable Stents in the Management of Refractory Esophageal Strictures in Children.

Background: Management of refractory esophageal strictures in children is challenging and mostly involves frequent and prolonged dilatations that are associated with significant physical and psychological complications. Biodegradable stents (BDS) have recently been used and it is particularly attractive in children as stent removal is avoided due to their dissolution. Report of Cases: We report our experience with BDS use in 3 children with severe refractory esophageal strictures. Two of the patients had strictures as a result of caustic ingestion and 1 after long-gap esophageal atresia repair. Median number of dilatations prestent is 5 per year. Custom-made uncovered BDS were inserted under general anesthesia. Median follow-up period was 23 months. Results: Minor postprocedure complications occurred in 1 patient. Stent insertion led to temporary improvement in swallowing in all cases. However, all of the patients developed recurrent symptoms as severe as before stenting and need for dilatations remained unchanged. They also experienced mucosal hyperplasia that contributed to their recurrent symptoms. Conclusion: All of the cases in our series did not have a favorable outcome with BDS use. BDS is yet to be proven as an effective management option for refractory esophageal strictures in children from our experience as well as most other published reports.

An Unusual Combination of Double Inlet Left Ventricle With Discordant Ventriculoarterial Connections and Bilateral Arterial Ducts.

The presence of bilaterally persistent arterial ducts is an uncommon abnormality. Here, we describe the anatomy and successful management of an unusual patient with bilateral ducts in the setting of double inlet left ventricle, discordant ventriculoarterial connections, aortic atresia, and a severely hypoplastic and serpentine aortic arch.

Transjugular Intrahepatic Portosystemic Shunt Insertion for the Management of Portal Hypertension in Children.

OBJECTIVES: the aim of the study was to describe our 20-year experience with transjugular intrahepatic portosystemic shunt (TIPSS) procedures for children with resistant portal hypertension (PHTN). METHODS: Retrospective review of all children that had a TIPSS performed at Birmingham Children's Hospital from January 1, 1995 to January 1, 2015. RESULTS: Forty children underwent 42 attempted TIPSS for resistant PHTN with recurrent variceal bleeding (n = 35), refractory ascites (n = 4), and hypersplenism (n = 1). Median age at operation was 12 years (range 7 months-17 years). Thirty-four procedures were elective and 8 were emergency cases. TIPSS was established in 33 cases (79%). Median portal venous pressure reduction was 10 mmHg. Variceal bleeding ceased in 27 (96%) and ascites improved in all. Clinical improvement following TIPSS enabled 7 children to be bridged to transplantation and 7 others to become suitable for transplantation. The 1-year and 5-year survival with TIPSS was 57% and 35%, respectively. Child-Pugh score C was an independent risk factor for adverse outcome (Likelihood ratio (LR) = 8.0; 95% confidence interval (CI) 2.7 to 23.5; P = 0.001). There were 6 major complications: hepatic artery thrombosis and infarction (n = 1), hepatic artery pseudoaneurysm (n = 1), bile leak (n = 1), and hepatic encephalopathy (n = 3). Encephalopathy was resistant to medical treatment in 2 cases, necessitating staged closure in 1. Ten patients (30%) required intervention to maintain TIPSS patency. The 1-year and 5-year freedom from reintervention was 71% and 55%, respectively. CONCLUSIONS: A TIPSS is highly successful in controlling symptoms in children with resistant PHTN and facilitating liver transplantation. It is technically demanding and not without risk. Patients must be appropriately selected and counselled.

Truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage: a unique form of univentricular heart.

We present the first reported case of truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage into a retro-aortic innominate vein in a neonate in whom successful palliation was performed using a two-staged procedure. An early repair after presentation along with a re-routing of anomalous pulmonary venous drainage at the time of the second stage should be undertaken to optimize the patient's haemodynamic status prior to a Fontan completion.

Autonomic cardio-respiratory reflex reactions and superselective ophthalmic arterial chemotherapy for retinoblastoma.

OBJECTIVE: To describe our experience with superselective ophthalmic artery chemotherapy (SOAC) in retinoblastoma and to report the serious adverse cardio-respiratory reactions we have observed. METHODS: SOAC was performed using a standardized protocol for general anesthesia, ophthalmic artery catheterization, and pulsed infusion of melphalan. Adverse reactions were defined as those in which the patient required active treatment to maintain cardio-respiratory stability. RESULTS: Between December 2008 and May 2012, 54 eyes in 52 patients were treated. 143 catheterization procedures were performed, with a technical success rate of 93% (n = 133). There were no deaths or major complications. Adverse cardio-respiratory reactions developed during 35 procedures (24%; 95% CI, 18-32%). All reactions occurred during second or subsequent catheterization procedures (39%; 95% CI, .29-49%) and were characterized by hypoxia, reduced lung compliance, systemic hypotension and bradycardia. Adverse events were successfully treated in all patients. One procedure was abandoned due to prolonged hemodynamic instability. CONCLUSION: Adverse cardio-respiratory reactions are commonly observed in SOAC for retinoblastoma. We believe that the adverse clinical signs represent an autonomic reflex response, akin to the trigemino-cardiac or oculo-respiratory reflexes, and all patients should be considered at-risk. Reactions occur only during second or subsequent procedures and can be life-threatening. The routine use of intravenous atropine does not seem to have altered the incidence or severity of these reactions. Anesthetists and interventional neuroradiologists involved in SOAC must be vigilant to ensure adverse reactions, when they develop, are treated quickly and effectively.