Evidence of Chemoresponsiveness in Unresectable Metastatic Angiomatoid Fibrous Histiocytoma.

Angiomatoid fibrous histiocytoma (AFH) is a soft tissue neoplasm of intermediate biological potential. Typically a slow-growing tumor, it can recur locally. Rarely, it manifests as a soft tissue sarcoma capable of metastasis. When metastases are nonamenable to local therapy, it is believed uniformly fatal. We present 3 patients with metastatic AFH who demonstrated a sustained response to chemotherapy; including one who achieved complete remission with cryoablation. These cases reinforce the potential value of chemotherapy in some patients with unresectable metastatic AFH and provide the first case in the literature of cryoablation in AFH.

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.

Quality and readability of online patient information regarding sclerotherapy for venous malformations.

BACKGROUND: Patients often use the internet as a source of information about their condition and treatments. However, this information is unregulated and varies in quality. OBJECTIVE: To evaluate the readability and quality of online information for pediatric and adult patients and caregivers regarding sclerotherapy for venous malformations. MATERIALS AND METHODS: "Venous malformation sclerotherapy" was entered into Google, and results were reviewed until 20 sites that satisfied predefined inclusion criteria were identified. Scientific and non-patient-focused web pages were excluded. Readability was assessed using the Flesch Reading Ease Score and American Medical Association reading difficulty recommendations and quality was assessed using Journal of the American Medical Association standards and assessing if the site displayed HONcode (Health on the Net Code) certification. Assessment of the breadth of relevant information was made using a predefined checklist. RESULTS: Forty-nine search engine results were reviewed before 20 sites were identified for analysis. Average Flesch Reading Ease Score was 44 (range: 24.2-70.1), representing a "fairly difficult" reading level. None of the sites had a Flesch Reading Ease Score meeting the American Medical Association recommendation of 80-90. Only one site met all four Journal of the American Medical Association quality criteria (average: 2.1). None of the sites displayed a HONcode seal. The information most frequently found was: sclerotherapy is performed by radiologists, multiple treatments may be needed and surgery is an alternative treatment. CONCLUSION: Online information regarding sclerotherapy for venous malformations is heterogeneous in quality and breadth of information, and does not meet readability recommendations for patient information. Radiologists should be aware of and account for this when meeting patients.

Surgical jejunostomy and radiological gastro-jejunostomy tube feeding in children: risks, benefits and nutritional outcomes.

PURPOSE: Radiologically inserted gastrojejunal tubes (RGJ) and surgical jejunostomy (SJ) are established modes of jejunal feeding. The aim of the study is to review nutritional outcomes, complications and the practical consideration to enable patients and carers to make informed choice. METHODS: Retrospective review of patient notes with a RGJ or SJ in 2010, with detailed follow-up and review of the literature. RESULTS: Both RGJ and SJ are reliable modes to provide stable enteral nutrition. Both have complications and their own associated limitations. CONCLUSIONS: The choice has to be tailored to the individual patient, the social care available, the inherent medical disease and risk/benefit of repeated anaesthetic and radiation exposure. RGJ and SJ are important tools for nutritional management that achieve and maintain growth in a complex group of children. The risk and benefits should be reviewed for each individual patient.

Endovenous laser ablation therapy in children: applications and outcomes.

BACKGROUND: Endovenous laser ablation is well recognized as the first-line treatment for superficial venous reflux with varicose veins in adults. It is not widely reported and is not an established practice in pediatric patients. OBJECTIVE: To illustrate a variety of pediatric venous conditions in which endovenous laser ablation can be utilized and to demonstrate its feasibility and safety in children. MATERIALS AND METHODS: We conducted a retrospective review of endovenous laser ablation procedures performed between January 2007 and July 2014 at two large pediatric institutions. RESULTS: We included 35 patients (17 males) who underwent endovenous laser ablation to 43 veins. Median age at first treatment was 14 years (range: 3-18 years). Median weight was 56 kg (range: 19-97 kg). Underlying diagnoses were common venous malformation (15), Klippel-Trenaunay syndrome (8), superficial venous reflux with varicose veins (5), verrucous hemangioma-related phlebectasia (4), venous varix (2) and arteriovenous fistula (1). The most common aim of treatment was to facilitate sclerotherapy. Thirty-four patients had treatment in the lower limbs and one patient in an upper limb. Ten of the veins treated with endovenous laser ablation had an additional procedure performed to close the vein. Complications attributable to endovenous laser ablation occurred in two patients (6%). One patient experienced post-procedural pain and one patient developed a temporary sensory nerve injury. Median clinical follow-up was 13 months (range: 28 days-5.7 years). The aim of the treatment was achieved in 29 of the 35 (83%) patients. CONCLUSION: Endovenous laser ablation is technically feasible and safe in children. It can be used in the management of a range of pediatric venous diseases with good outcomes.

Neuropathy after sodium tetradecyl sulfate sclerotherapy of venous malformations in children.

PURPOSE: To estimate the risk of nerve injuries and assess outcomes after sodium tetradecyl sulfate (STS) sclerotherapy of venous malformations (VMs) in children. MATERIALS AND METHODS: Sclerotherapy is the treatment of choice for most VMs, but all sclerotherapy agents are associated with the risk of complications. Neuropathy is considered a rare but potentially serious complication of venous sclerotherapy. The institutional review board waived ethical approval for this retrospective review, in which 647 sclerotherapy procedures were performed in 204 patients (104 female and 100 male patients; mean age, 9 years 6 months [range, 6 months to 17 years 11 months]) as treatment for symptomatic VMs. Technical and clinical success of the treatment was evaluated. Complications were reviewed with a particular focus on nerve injury. Informed consent, specifying the risk of neuropathy, as well as pain, swelling, infection, risks of anesthesia, skin injury, nonresolution or worsening of symptoms, and possible need for further or multiple procedures, was obtained for all patients. Standard sclerotherapy techniques were used. Technical details of all procedures were recorded prospectively. Follow-up included immediate postprocedural assessment and outpatient clinic review. All nerve injuries were recorded. Patients were monitored and treated according to clinical need. Confidence intervals were calculated by using the Wilson method, without correction for continuity. RESULTS: Treatment was technically successful in 197 of 204 patients (96.6%), and clinical success was achieved in 174 of 204 (85.3%). Thirty-seven of the 647 procedures (5.7%) resulted in a complication, including 11 cases of excessive swelling, nine cases of skin injury, two patients with infection, and two with pain. Motor and/or sensory nerve injuries occurred after seven procedures (1.1%). Five of the seven children had undergone at least one previous sclerotherapy procedure. Neuropathy resolved spontaneously in four patients and partially recovered in three, of whom two underwent surgery. Surgery included debridement of necrotic tissue, carpal tunnel decompression, and external neurolysis. CONCLUSION: Nerve injury is an unusual but not rare complication of STS sclerotherapy. A degree of recovery, which may be complete, can be expected in most patients.

Ultrasound-guided transaxillary access for diagnostic and interventional arteriography in children.

PURPOSE: Most pediatric arteriograms are obtained from a femoral approach; alternative access sites are sometimes needed. Transaxillary arteriography is an established procedure in adults. The purpose of this study was to establish the feasibility and safety of this procedure in children. MATERIALS AND METHODS: All children who underwent arteriography involving axillary access during a 10-year period were reviewed for demographic details, indications, technical aspects of the procedure, and complications. Twenty-five procedures were performed in 19 children aged between 7 days and 15 years (median, 4.8 years). The children weighed between 2.6 and 47 kg (median, 15 kg). Indications for use of the axillary artery were absolute (including aortic or bilateral iliac artery occlusion) in 15 (60%) and relative (including more favorable angle for thoracic or abdominal intervention) in 10 (40%). Two procedures (8%) were purely diagnostic arteriography procedures. RESULTS: Transaxillary access was successful in all cases. The largest sheath used (7-F) had an outer diameter of 2.7 mm. One child died of a postoperative complication unrelated to the arterial access. There were no neurologic or bleeding complications related to the access site. Two access site complications (8%) occurred-one pseudoaneurysm and one axillary artery dissection-but neither resulted in permanent adverse sequelae. CONCLUSIONS: Ultrasound-guided transaxillary access is feasible for arteriography in children when femoral access is impossible or unfavorable for technical reasons. Although major complications are unlikely, this study does not exclude the possibility of subtle postoperative nerve injury.

Vascular anomalies: special considerations in children.

The diagnosis and treatment of vascular anomalies are a large part of the caseload for paediatric interventional radiologists. Although many of the principles of sclerotherapy and embolisation are the same in adult and paediatric practice, there are some key differences in the approach for children, including some longer term thinking about managing these chronic diseases and their impact on a growing child. Vascular tumours are not often seen in adult IR practice and the rarest can be life threatening; knowledge of the commonest types and the role IR can play in their management can be instrumental in ensuring that children get appropriate treatment in a timely manner. Vascular anomalies also encompass some conditions associated with complex overgrowth, a subject that often causes confusion and uncertainty for interventional radiologists. This paper presents a simplified and practical approach to this spectrum of disease.

Splenic Rupture Secondary to Vascular Ehlers-Danlos Syndrome Managed by Coil Embolization of the Splenic Artery.

Aim Atraumatic splenic rupture is uncommon and life-threatening. It may be related to underlying pathology and be the initial manifestation of the condition. Vascular Ehlers-Danlos syndrome (V-EDS) is a rare autosomal dominant collagen vascular disorder, associated with vessel fragility and rupture. We describe a child presenting with splenic rupture managed by embolization of the splenic artery. She was subsequently diagnosed with V-EDS. Case Description A 11-year-old girl with thalassemia trait presented with sudden onset of abdominal pain and hypovolemic shock. There was no history of trauma. Following resuscitation, abdominal computed tomography showed hemoperitoneum and active splenic arterial extravasation. Angiography demonstrated four bleeding points, from irregular vessels supplying the upper two-thirds of the spleen. These were not amenable to supraselective embolization. Therefore, coil embolization of the main splenic artery was performed, with no splenic supply seen on the postembolization angiogram. Her postoperative recovery was complicated by pancreatitis secondary to partial ischemia of the pancreatic tail. Subsequent extensive investigations excluded hematological, myeloproliferative, and infective causes for her splenic rupture. A safeguarding investigation was completed, with no pertinent factors identified. Findings of thin skin, abnormal bruising, and hypermobile joints raised a clinical suspicion of a connective tissue disorder. Genetic testing revealed a de novo mutation of the COL3A1 gene. Conclusions There are only four reports of V-EDS causing splenic rupture in the literature to date. These patients were all adults and only one had not previously been diagnosed with V-EDS. All underwent splenectomy. While V-EDS presenting with abdominal visceral rupture in children has been reported, this is the first report of a child with V-EDS presenting with splenic rupture. It is the only case of splenic rupture secondary to V-EDS that has been managed minimally invasively by embolization.

Haemoptysis and bronchial artery embolization in children.

Haemoptysis varies in significance from trivial to fatal. The most common causes in children are tuberculosis, fungal infections and bronchiectasis of any cause. In populations of European origin, cystic fibrosis is the most common predisposing factor. Chronic or life-threatening haemoptysis requires further investigation. Various forms of treatment are possible, and management is optimized when a multidisciplinary team is available. Bronchial artery embolization (BAE) is effective in controlling haemoptysis in most cases, although recurrent bleeding is not uncommon. BAE is often technically challenging, however, and requires angiographic skills that are not always available in children's hospitals. Although the procedure is usually regarded as relatively safe, complications are not uncommon and may be severe or even fatal.

Angiographic diagnosis of inflammatory bowel disease in patients presenting with gastrointestinal bleeding.

OBJECTIVE: The objective of our study was to review the final diagnosis and outcome in patients undergoing visceral angiography for gastrointestinal hemorrhage in whom a diagnosis of inflammatory bowel disease (IBD) had been suspected on the basis of angiographic findings. MATERIALS AND METHODS: A retrospective review was performed of all visceral angiography referrals over a 10-year period. During that time, 522 visceral angiograms were obtained for the investigation of acute or chronic gastrointestinal bleeding. IBD was reported as a possible or probable diagnosis based on the angiographic findings in 43 patients (8.2%) (male-to-female ratio, 23:20; age range, 11-79 years; mean age, 48.6 years). RESULTS: Active IBD was confirmed in 25 (58%) of these 43 individuals (male-to-female ratio, 15:10; mean age, 46.5 years), representing 4.8% of all patients referred for the investigation of gastrointestinal bleeding. In 14 of these individuals, a diagnosis of IBD had not been suspected previously. A diagnosis other than IBD was found to be the cause of the angiographic findings in seven subjects. In the 32 patients in whom a cause for the angiographic findings was identified, IBD was the diagnosis in 25 (78%). CONCLUSION: In a group of patients referred for angiography to investigate acute or chronic gastrointestinal bleeding, IBD can be expected to be the cause in approximately 5%. Radiologists should be aware of the angiographic abnormalities seen in patients with IBD and be prepared to consider this diagnosis even if the results of other previous investigations have been reported as normal.

Sclerotherapy treatment of orbital lymphatic malformations: a large single-centre experience.

BACKGROUND: Percutaneous sclerotherapy is an alternative to surgery for the treatment of orbital lymphatic malformations (LMs). We present a large series of patients undergoing sclerotherapy for macrocystic LMs with detailed visual acuity (VA) outcome data. METHODS: Data were collected prospectively in all patients with macrocystic orbital LMs undergoing sclerotherapy. Sclerotherapy was performed under general anaesthesia, instilling sodium tetradecyl sulfate under imaging control. Procedures were repeated at 2-week to 6-week intervals, depending on clinical response. Patients underwent ophthalmological assessment, ultrasound and/or MRI before and after treatment. PRIMARY OUTCOME MEASURE: change in maximal radiological diameter of the LM. SECONDARY OUTCOME MEASURE: change in VA after treatment. RESULTS: 29 patients underwent 71 procedures (1-8 procedures per patient) over 6.7 years. Mean age=7.31 years. 11 patients (37.9%) had undergone previous treatment, including excision biopsy, drainage and decompression. All patients presented with proptosis and/or pseudoptosis. 23 patients (79.3%) had decreased VA at presentation. Average follow-up was 21.8 months (range 3-75 months). All patients achieved a reduction in maximal lesion diameter of ≥50%, with complete radiological resolution in 51.7% (n=15). VA improved in 18/23 patients (78.2%). Average logMAR before treatment=0.43 (SD ±0.47); average after treatment=0.25 (SD ±0.32); p<0.01. There was one complication (1.4%): one patient required a lateral canthotomy for an intralesional haematoma 2 h after sclerotherapy. CONCLUSIONS: Sclerotherapy is a safe and highly effective treatment for orbital LMs with excellent VA outcomes. It should be considered as the first-line treatment for this condition.

Interventional radiology in infancy.

Interventional radiology (IR) is an emerging sub-speciality within paediatric medicine. In adult care, IR is largely centred on the management of vascular disease but in paediatric practice, IR applications are varied and increasingly innovative, making this an exciting field to be a part of. IR has a central role both in the day to day care of sick children, from long term IV access provision to feeding tube insertions, and in the acute management of critically ill infants, such as those with overwhelming liver disease, neonatal tumours and vascular malformations. Paediatric IR faces a unique set of challenges, developing or modifying techniques and equipment for use in very small patients, training professionals to take the speciality forward and, most importantly, convincing paediatricians and healthcare institutions to create opportunities for IR to make a difference.

Hepatobiliary intervention in children.

Various vascular and nonvascular hepatobiliary interventional radiology techniques are now commonly performed in children's hospitals. Although the procedures are broadly similar to interventional practice in adults, there are important differences in indications and technical aspects. This review describes the indications, techniques, and results of liver biopsy, hepatic and portal venous interventions and biliary interventions in children.

Paediatric interventional uroradiology.

Paediatric interventional uroradiology lies at the intersection of the disciplines of paediatric interventional radiology and paediatric endourology. Interdisciplinary collaboration has led to the development of new techniques and refinement of procedures adopted from adult practice. This article reviews the major procedures used in paediatric interventional uroradiology, with emphasis on nephrostomy, percutaneous nephrolithotomy, balloon-burst pyeloplasty, and antegrade ureteric stenting.