Approaches to the management of haemoptysis in young people with cystic fibrosis.

Haemoptysis occurs in up to 25 % of young people with Cystic fibrosis (CF) [1]. We undertook a literature review and described the management approach to haemoptysis in CF between 2010 and 2020 at an Australian tertiary paediatric centre, The Children's Hospital Westmead, Sydney, New South Wales, using a retrospective review of the medical records which identified 67 episodes. Sixty episodes met inclusion criteria, including 31 patients. Using the US CF Foundation guidelines, episodes were classified as scant (53.3 %), moderate (38.3 %) or massive (8.3 %). Fifty-two percent of patients were female, mean age at presentation was 15.4 years (SD+/- 2.4) and 58 % were homozygous for the Fdel508 genotype. Twelve episodes (9 patients) required bronchial artery embolization (BAE). BAE was used in all cases of massive haemoptysis 5/5 (100 %), 6/23 (22 %) episodes of moderate and 1/32 (3 %) episode of scant haemoptysis as an elective procedure for recurrent haemoptysis. Our literature review and institutional experience highlights the need for up-to-date management guidelines in the management of haemoptysis in Cystic Fibrosis. Based on our experience, we provide a proposed algorithm to help guide the management of haemoptysis in CF.

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.

Update in management of paediatric primary spontaneous pneumothorax.

Paediatric spontaneous pneumothorax (PSP) management continues to lack paediatric-specific guideline recommendations. There have been increasing reports of paediatric retrospective case studies supplemented by important well designed RCT (predominantly) adult studies. Taken together, these suggest that conservative management may have an increasing role to play in the management of PSP and that aspiration may have limited utility as a first line intervention. Our local experience, as part of a multicentre retrospective analysis and subsequent audit of management since, corroborates recent published data: it highlights an increasing trend towards conservative management in spontaneous pneumothorax with similar rates of recurrence, compared to intervention, and low use of aspiration with similarly low success rates. We have therefore updated our local practice guidelines and share these with readers. Specifically, we have removed aspiration in the management of primary spontaneous pneumothorax and reserved intervention for children who are clinically unstable or show evidence of increasing air leak irrespective of pneumothorax size. Whilst the success of this change in clinical practice will need to be reviewed in the next 5-10 years, the overall low incidence of the condition, demands a multicentre, and probably multinational, collaborative approach to allow the best chance of obtaining definitive evidence to guide clinical paediatric management.

A systematic review assessing incorporation of prophylactic splenic artery embolisation (pSAE) into trauma guidelines for the management of high-grade splenic injury.

BACKGROUND: Splenic artery embolisation (SAE) has become a vital strategy in the modern landscape of multidisciplinary trauma care, improving splenic salvage rates in patients with high-grade injury. However, due to a lack of prospective data there remains contention amongst stakeholders as to whether SAE should be performed at the time of presentation (prophylactic or pSAE), or whether patients should be observed, and SAE only used only if a patient re-bleeds. This systematic review aimed to assess published practice management guidelines which recommend pSAE, stratified according to their quality. METHODS: The study was registered and reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Medline, PubMed, Cochrane, Embase, and Google Scholar were searched by the study authors. Identified guidelines were graded according to the Appraisal of Guidelines Research and Evaluation II (AGREE-II) instrument. RESULTS: Database and internet searches identified 1006 results. After applying exclusion criteria, 28 guidelines were included. The use of pSAE was recommended in 15 guidelines (54%). This included 6 out of 9 guidelines that were high quality (66.7%), 4 out of 9 guidelines that were moderate quality (44.4%), and 3 out of 10 (30%) guidelines that were low quality, p = 0.275. CONCLUSIONS: This systematic review showed that recommendation of pSAE is more common in guidelines which are of high quality. However, there is vast heterogeneity of recommended practice guidelines, likely based on individual trauma systems rather than the available evidence. This reflects biases with interpretation of data and lack of multidisciplinary system inputs, including from interventional radiologists.

Exaggerated increase in cardiac output during exercise in patients with peripheral high-flow arteriovenous malformations.

PURPOSE: To test the hypothesis that cardiac output (Q) in patients with arteriovenous malformations (AVMs) is normal at rest and increases disproportionately during exercise. MATERIALS AND METHODS: Q was measured in eight patients with large peripheral AVMs and in nine healthy subjects using a noninvasive carbon dioxide (CO2)-based differential Fick method. Subjects were tested while seated at rest and during mild exercise (repeated leg straightening while sitting). Oxygen consumption (VO2) was monitored as an index of the degree of exercise. RESULTS: Average resting Q was similar between AVM patients and healthy subjects (7.40 L/min ± 3.29 vs 6.13 L/min ± 0.94, respectively, P = .29). During exercise, AVM patients showed a smaller increment in VO2 (0.50 L/min ± 0.11 vs 0.78 L/min ± 0.26, P = .012) but with more apparent effort and shortness of breath compared with healthy subjects. The change in Q per unit change in VO2 (ΔQ/ΔVO2) was greater in AVM patients than in healthy subjects (16.00 L/min ± 6.50 vs 9.79 L/min ± 5.33, P < .045). CONCLUSIONS: Exercise intolerance in AVM patients may be due to an imbalance in ΔQ/ΔVO2 resulting from increased shunting through the AVM. Exercise provocation may increase the sensitivity of Q in the clinical evaluation of AVM patients.

Embolization in neonates and infants.

Several conditions presenting in the neonatal and infant period benefit from embolization, including hemangioma, vascular shunts, and tumors. The physiological delicacy and small size of newborns create distinct challenges. This paper discusses embolization of these patients and illustrates the techniques involved.

Pediatric high-flow priapism and super-selective angiography--an Australian perspective.

High-flow priapism is an uncommon entity in the pediatric and adolescent population. It is usually caused by perineal trauma. Here we describe the experience of our institution in this condition over the past 10 years, the various treatment options available, and the successful application of super-selective angiographic embolization as our treatment modality of choice. Included here is the case of a 4-year-old boy (case 3) who, to our knowledge, is the youngest patient described with this condition in the literature.