Fostering research in pediatric interventional radiology: needs assessment and suggestions for support.

BACKGROUND: Due to the rarity of pediatric diseases, collaborative research is the key to maximizing the impact of research studies. A research needs assessment survey was created to support initiatives to foster pediatric interventional radiology research. OBJECTIVE: To assess the status of pediatric interventional radiology research, identify perceived barriers, obtain community input on areas of research/education/support, and create metrics for evaluating changes/responses to programmatic initiatives. MATERIALS AND METHODS: A survey link was sent to approximately 275 members of the Society for Pediatric Interventional Radiology (SPIR) between May and October 2020. Data was collected using a web-based interface. Data collected included practice setting, clinical role, research experience, research barriers, and suggestions for future initiatives. RESULTS: Fifty-nine surveys were analyzed with a staff physician survey response rate of 28% (56/198). A wide range of practice sizes from 15 countries were represented. Respondents were predominantly staff physicians (95%; 56/59) with an average of 11 years (range: 1-25 years) of clinical experience working at academic or freestanding children's hospitals. A total of 100% (59/59) had research experience, and 70% (41/58) had published research with a mean of 30 peer-reviewed publications (range: 1-200). For job security, 56% (33/59) of respondents were expected or required to publish, but only 19% (11/58) had research support staff, and 42% (25/59) had protected research time, but of those, 36% (9/25) got the time "sometimes or never." Lack of support staff, established collaborative processes, and education were identified as top barriers to performing research. CONCLUSIONS: The needs assessment survey demonstrated active research output despite several identified barriers. There is a widespread interest within the pediatric interventional radiology community for collaborative research.

Revision of meso-Rex bypass utilizing a collateral vein in a patient with portal steal phenomenon after liver transplant: A case report.

INTRODUCTION: In children with extrahepatic portal vein obstruction or those who develop portal vein thrombosis after liver transplant, the use of Meso-Rex Bypass (MRB) creates a more physiological state by redirecting mesenteric blood flow back into the intrahepatic portal system via a venous conduit. PRESENTATION OF CASE: A 3-year-old female with biliary atresia associated with polysplenia syndrome and a surgical history of Kasai portoenterostomy procedure, and an ABO incompatible whole liver transplant. Within a year after transplant she presented with prehepatic portal hypertension, that was treated with MRB using a deceased donor ABO compatible iliac vein as conduit. Six months later, she was taken to the operating room for bypass revision, during the procedure the MRB showed no flow and no thrombus, and a large splenorenal collateral vein that was causing a portal perfusion steal phenomenon was observed. After dissecting the collateral vein, an 8 cm x8 mm segment of this vessel was used as an autologous conduit to re-do the Rex. DISCUSSION: Failed of MRB can be attributed to portal steal phenomenon, hypercoagulable disorders, bypass contraction or kinking. In this case we believe the culprit to be the former. When there is a history of longstanding portal hypertension, large collaterals develop; thus, intraoperative portal vein flow measurement is critical and ligation of large collaterals during liver transplantation and MRB should be performed to avoid portal steal phenomenon postprocedure. CONCLUSION: Using a collateral vein as an alternative autologous venous conduit is a feasible option that can have durable success.

Challenges and Opportunities for Continued Success and Growth of Pediatric Interventional Radiology: A Communiqué From the Society for Pediatric Interventional Radiology.

OBJECTIVE: Pediatric interventional radiology (PIR) is a dynamic and growing subspecialty. We will detail our need to evolve like the emerging therapies and innovative imaging options that we provide to children. CONCLUSION: New interventional radiology training pathways, maintaining competency with small volumes of complex procedures, limited availability of pediatric-specific equipment, questions about the safety of sedation in the developing brain, and the dearth of PIR opportunities outside of North America provide challenges and opportunities for the vibrant community of PIR practitioners.

Ocular dysmotility after intra-arterial chemotherapy for retinoblastoma.

We report the case of a 15-month-old boy with retinoblastoma who developed exotropia secondary to a right medial rectus infarct after intra-arterial chemotherapy. He had unilateral sporadic group C tumor (International Classification of Retinoblastoma) and was treated with intra-arterial melphalan. One week after the first session of intra-ophthalmic arterial melphalan chemotherapy, he was noted to have orbital congestion, exotropia, and right adduction limitation. Magnetic resonance imaging was suggestive of a right medial rectus infarct. The tumor showed a good response to intra-arterial chemotherapy but the exotropia persisted.

Magnamosis: a novel technique for the management of rectal atresia.

We report a case of rectal atresia treated using magnets to create a rectal anastomosis. This minimally invasive technique is straightforward and effective for the treatment of rectal atresia in children.

Bronchial artery pseudoaneurysm as an unsuspected cause of hemoptysis in a pediatric patient.

OBJECTIVE: Hemoptysis in the pediatric population may be caused by foreign body aspiration, cystic fibrosis, bronchiectasis, or infection. Vascular causes are uncommon. We present a rare cause of hemoptysis related to a bronchial artery pseudoaneurysm. METHODS: We report the case of a child with a bronchial artery pseudoaneurysm causing hemoptysis and describe the clinical evaluation, treatment, and outcome. RESULTS: A 12-year-old girl presented to a tertiary children's hospital with a history of daily, intermittent, moderate-volume hemoptysis. Rigid bronchoscopy showed a fresh clot occluding the right bronchus intermedius. Computed tomography angiogram was concerning for mild external vascular compression of the right mainstem bronchus. A bronchial arteriogram showed a right mid-bronchial pseudoaneurysm, which was embolized without complication. On repeat bronchoscopy, thrombus was removed from the bronchus intermedius with no new active bleeding. The patient was discharged in stable condition and did not have any more episodes of hemoptysis. Additional medical work-up did not reveal another source of the patient's bleeding. CONCLUSION: Hemoptysis in the pediatric population can be inflammatory, infectious, or due to systemic disease. Although extremely rare, bronchial artery pseudoaneurysm should be considered in cases of moderate to severe intermittent hemoptysis without another identifiable cause. Bronchial angiography can be both diagnostic and therapeutic.

Sclerotherapy for intra-abdominal lymphatic malformations in children.

PURPOSE: Sclerotherapy is well described as a treatment for lymphatic malformations (LMs) in the head, neck, and other soft tissue areas. This study aims to evaluate the effectiveness of intralesional sclerotherapy as primary treatment for intra-abdominal LMs in children. METHODS: We conducted a retrospective review from 2008 to 2012 of all children with intra-abdominal LMs treated with sclerotherapy at our tertiary children's hospital. RESULTS: In this study, five patients underwent sclerotherapy as a primary intervention for intra-abdominal LMs. The ages of these patients ranged from 12 to 52 months at the time of initial treatment. Doxycycline was used as the primary sclerotherapy agent. The patients required between three and five (median 3) sclerosing treatments over a period that ranged from 5 to 366 days (median 28). No child has required an operation and all LMs have decreased in size. The median decease in maximum diameter is 62% (21-67). Complete resolution has not been attained but all have experienced symptomatic relief with a median follow-up of 3 (1-24) months. CONCLUSIONS: Sclerotherapy is an effective first-line therapy for intra-abdominal LMs in the pediatric population and should be considered when treating these difficult lesions.

Non-operative treatment versus percutaneous drainage of pancreatic pseudocysts in children.

PURPOSE: The objective of this study was to characterize the clinical course and outcomes of children with pancreatic pseudocysts that were initially treated non-operatively or with percutaneous drainage. METHODS: A retrospective review of children with pancreatic pseudocysts over a 12-year period was completed. Categorical variables were compared using Fischer's exact method and the Student's t test was used to compare continuous variables. Analysis was done using logistic and linear regression models. RESULTS: Thirty-six children met the criteria for pancreatic pseudocyst and 33 children were treated either non-operatively or with percutaneous drainage. Of the 22 children managed non-operatively, 17 required no additional intervention (77 %) and five required surgery. Operative procedures were: Frey procedure (3), distal pancreatectomy (1), and cystgastrostomy (1). Eight of the 11 children treated with initial percutaneous drainage required no additional treatment (72 %). The other three children underwent distal pancreatectomy. Success of non-operative management or percutaneous drainage was not dependent on size or complexity of the pseudocyst Logistic regression did not identify any patient demographic (gender, age, and weight), etiologic (trauma, non-traumatic pancreatitis) or pseudocyst characteristic (size, septations) that predicted failure of non-operative therapy. CONCLUSIONS: In children, pancreatic pseudocysts can frequently be managed without surgery regardless of size or complexity of the pseudocyst. When an intervention is needed, percutaneous drainage can be performed successfully, avoiding the need for major surgical intervention in the majority of patients.

Efficacy and safety during formulation switch of a pasteurized VWF/FVIII concentrate: results from an Italian prospective observational study in patients with von Willebrand disease.

Von Willebrand disease (VWD) is an inherited bleeding disorder caused by the quantitative or qualitative deficiency of von Willebrand factor (VWF). Replacement therapy with plasma-derived VWF/factor VIII (FVIII) concentrates is required in patients unresponsive to desmopressin. To assess the efficacy, safety and ease of use of a new, volume-reduced (VR) formulation of VWF/FVIII concentrate Haemate(®) P in patients requiring treatment for bleeding or prophylaxis for recurrent bleeding or for invasive procedures. Pharmacoeconomic variables were also recorded. Data were analysed using descriptive statistics. This was a multicentre, prospective, observational study. Consecutively enrolled patients received Haemate(®) P VR according to their needs, and were followed for 24 months. Of the 121 patients enrolled, 25.6% had type 3 VWD and more than 40% had severe disease. All patients were followed for 2 years, for a total of 521 visits. On-demand treatment was given to 61.9% of patients, secondary long-term prophylaxis to 25.6% and prophylaxis for surgery, dental or invasive procedures to 45.5%. The response to treatment was rated as good to excellent in >93-99% of interventions. The new formulation was well tolerated by all patients with no report of drug-related adverse events. The switch to volume-reduced Haemate(®) P was easy to perform and infusion duration was decreased twofold compared with the previous formulation. Volume-reduced Haemate(®) P was at least as effective and well-tolerated as the previous formulation.

Safe treatment of pulmonary hypertension with bosentan in a patient with moyamoya disease and cerebral ischemia.

A 7-year-old girl with Down syndrome and moderately severe pulmonary hypertension experienced a stroke while being treated with a calcium channel blocker. Angiography identified bilateral stenosis of the supraclinoid internal carotid arteries, stenosis or occlusion of the proximal anterior and middle cerebral arteries, and occlusion of the left posterior cerebral artery. She underwent surgery to enhance collateral blood flow to vulnerable areas of the brain. Her pulmonary hypertension therapy was changed to an oral endothelin receptor antagonist. She developed excellent collateral blood flow through external carotid arteries to each cerebral hemisphere and an improvement in blood flow through the right internal carotid artery. This case suggests that bosentan can be used safely in children with moyamoya disease. Additional studies are needed to determine whether endothelin receptor antagonists may influence the progression of moyamoya disease or the development of collateral cerebral blood flow following surgery.

Effects of secondary prophylaxis started in adolescent and adult haemophiliacs.

While primary prophylaxis is a well-established and recommended method of care delivery for children with severe haemophilia, fewer studies have documented the benefits of secondary prophylaxis started in adolescence or adulthood. To evaluate the role of secondary prophylaxis started in adolescent and adult severe haemophiliacs, a retrospective observational cohort study was conducted in 10 Italian Centres that investigated 84 haemophiliacs who had bled frequently and had thus switched from on-demand to prophylactic treatment during adolescence (n = 30) or adulthood (n = 54). The consumption of clotting factor concentrates, the orthopaedic and radiological scores, quality of life and disease-related morbidity were compared before and after starting secondary prophylaxis. Prophylaxis reduced the mean annual number of total and joint bleeds (35.8 vs. 4.2 and 32.4 vs. 3.3; P < 0.01) and of days lost from work/school (34.6 vs. 3.0, P < 0.01). A statistically significant reduction in the orthopaedic score was observed during prophylaxis in adolescents, but not in the whole cohort. Patients used more factor concentrates with corresponding higher costs on prophylaxis, but experienced a better quality of life. With respect to on-demand treatment, higher factor consumption and cost of secondary prophylaxis were balanced by marked clinical benefits and greater well-being in this cohort of adolescent/adult haemophiliacs.

Current treatment of pediatric empyema.

Pneumonia with complicated parapneumonic effusion and empyema is increasing in incidence and continues to be a source of morbidity in children seen in our institution. Current diagnostic modalities include chest radiographs and CT scanning with ultrasound being helpful in some situations. Exact management of empyema remains controversial. Although open thoracotomy drainage is well accepted in children, video-assisted thoracoscopic surgery (VATS) drainage has become more prevalent in the current era. Over the last 4 years, we have treated 58 children with intrapleural placement of pigtail catheters and administration fibrinolytics consisting of tissue plasminogen activator (tPA). Successful drainage and resolution of 54 of the 58 effusions was achieved with percutaneous methods alone. There was no mortality or 30-day recurrence. Mean hospital stay was 9.1 days (range 5 to 21) and mean chest catheter removal was 6 days post placement (range 1.5 to 20). Of the four patients that failed percutaneous tube therapy, 3 underwent video assisted thoracic surgery (VATS), and one had open thoracotomy with decortication. Based on our experience, tPA administered through a small bore chest tube for drainage of complicated parapneumonic effusions has become our standard practice. We reserve VATS for treatment failures and open thoracotomy and decortication for patients with VATS failure.

Management of complicated parapneumonic effusions in children.

Pneumonia with complicated parapneumonic effusion is a significant source of morbidity in children seen in our institution. This affords us the opportunity to evaluate new treatment options. In an effort to ensure that we provide quality care to these pediatric patients presenting with complicated parapneumonic effusions, we performed a retrospective review of patient records as well as our interventional radiology database. Fifty-eight patients were identified who were treated with intrapleural placement of pigtail catheters and administration of tPA. Successful drainage and resolution of 54 of the 58 effusions were achieved with percutaneous methods alone. There was no mortality or 30-day recurrence. Mean hospital stay was 9.1 days (range 5-21). On average, the chest catheter was removed on day 6 postplacement (range 1.5-20). tPA was administered intrapleurally, utilizing a standardized hospital protocol developed conjointly by Interventional Radiology and Thoracic Surgery. Patients were afebrile within 72 hours. In most patients, one catheter was placed. However, five patients had more than one catheter placed initially. Of the four patients that failed percutaneous tube therapy, three underwent video-assisted thoracic surgery (VATS) and one had open thoracotomy with decortication. The complication associated with this treatment was an average drop in hemoglobin of 2 g/mL. Based on our experience, tPA administered through a small-bore chest tube for drainage of complicated parapneumonic effusions has become our standard practice.

Methylene blue-stained autologous blood for needle localization and thoracoscopic resection of deep pulmonary nodules.

BACKGROUND/PURPOSE: Video-assisted thoracoscopic surgery (VATS) has used a variety of preoperative techniques to localize deep pulmonary nodules including wires, plain methylene blue, colored collagen, indigo carmine, India ink, and barium. The authors describe their experience with a computed tomography (CT)-guided localization technique using autologous blood stained with methylene blue. METHODS: The authors reviewed retrospectively children who had pulmonary nodules localized using CT guidance with a mixture containing 3 mL autologous blood stained with 0.3 mL methylene blue. Nodules were resected by standard VATS technique. Postoperative chest tube drainage was performed selectively. RESULTS: Nineteen procedures were performed in 17 children (average age, 11 years). Operating time (range, 21 to 171 minutes) varied depending on the number of nodules resected. All resections were diagnostic, and 80% represented malignancy. Lesions averaged 0.9 cm in size (range, 0.3 to 3 cm) with an average pulmonary depth of 0.8 cm (range, 0.1 cm to 1.8 cm). One patient required conversion to an open thoracotomy because of malfunction of the endoscopic stapler. Forty percent of the children received chest tubes, and 53% were discharged home the same day. CONCLUSION: VATS diagnostic resection of deep pulmonary nodules preoperatively localized with methylene blue stained autologous blood is safe and effective.

A novel approach to search for identity by descent in small samples of patients and controls from the same mendelian breeding unit: a pilot study on myopia.

Autosomal dominant high myopia, a genetic disorder already mapped to region 18p11.31, is common in Carloforte (Sardinia, Italy), an isolated village of 8,000 inhabitants descending from a founder group of 300 in the early 1700s. Fifteen myopic propositi and 36 normal controls were selected for not having ancestors in common at least up to the grandparental generation, although still descendants of the original founders. All subjects were genotyped for 14 markers located on autosome 18 at a resolution of about 10 cM. Allelic distributions were found to be similar at all tested loci in propositi and controls, except for the candidate marker D18S63 known to segregate in close linkage association with high myopia. In particular, the frequency of allele 85 among the propositi was almost double that of the controls (Fisher's exact test, p = 0.037). The association is more striking when the frequency of the genotype 85/85 in the two groups is compared (Fisher's exact test, p = 0.005). This conclusion was further evaluated through a bootstrap analysis by computing the overall probability of the observed data under the null hypothesis (i.e. no difference between the two groups in frequency distributions for the chromosome 18 markers). Again, marker D18S63 was found to have a sample probability lower than 0.004, which is significant at the 0.05 level after correcting for simultaneous testing of multiple loci. The study demonstrates the efficiency of our novel strategy to detect identity by descent (IBD) in small numbers of patients and controls when they are both part of well-defined Mendelian breeding units (MBUs). The iterative application of our strategy in separate MBUs is expected to become the method of choice to evaluate the ever-growing number of reported associations between candidate genes and multifactorial traits and diseases.

Deaths from asthma in Italy (1974-1988): is there a relationship with changing pharmacological approaches?

In this article we consider the relationship between asthma mortality rates, obtained from the Italian National Institute of Statistics (ISTAT), and the doses of all antiasthmatic drugs except systemic steroids sold in Italy in the years 1974-1988. The total asthma mortality rate showed three different trends: it decreased slowly until 1978 (period A); increased 10-fold from 1979 to 1985, rising from 0.30 to 4.17/100,000 (period B); and remained stable until 1988 (period C). More than half of the deaths in 1988 occurred in people 75 years of age or more. Men died more in the older age groups, while the mortality of women prevailed in the 35- to 54-year age group. In the 5- to 34-year age group the rate rose from 0.01 in 1978 to 0.21 /100,000 in 1986. Coding changes due to the 9th revision of the International Classification of Disease, adopted in Italy in 1979, probably increased the number of deaths being attributed to asthma in case of contemporary mention of bronchitis, a common diagnosis in older men, which showed the greatest increase in mortality. Increased prevalence and awareness of asthma may also have played a role. Although international comparisons strongly suggest undertreatment of asthma in Italy, the doses of anti-asthma drugs sold in Italy grew from 276 to 1,080 million from 1974 to 1985. During period B xanthine sales rose sevenfold and grew from 6.5 to 23.3% of the total doses, along with a twofold increase in beta 2-agonist and cromolyn sales. Period C was characterized by stable total doses (1155 million in 1988), with increases only in antiinflammatory and preventive drug sales. The increase in asthma deaths in Italy has been striking despite the contemporary rise in sales of all antiasthma drugs, particularly of beta 2-agonist metered aerosols and xanthine tablets. The increase in antiinflammatory and preventive drug sales may have contributed to the stabilization of asthma deaths during period C.