Presentation, treatment, and outcome of renovascular hypertension below 2 years of age.

Renovascular hypertension in most cases requires endovascular treatment and/or surgery. This is technically much more difficult in small children and there is very limited published knowledge in this age group. We here present treatment and outcome of young children with renovascular hypertension at our institution. Children below 2 years of age, with renovascular hypertension between January 1998 and March 2020 were retrospectively reviewed. Demographics and treatment modalities were noted. Primary outcome was blood pressure within a week after the procedures and at last available visit. Sixty-six angiographies were performed in 34 patients. Median age at time of first angiography was 1.03 (interquartile range (IQR) 0.4-1.4) years and systolic blood pressure at presentation 130 (IQR 130-150) mm Hg. Thirty-eight percent (13/34) of children were incidentally diagnosed and 18% (6/34) presented with heart failure. Twenty-six (76%) children had main renal artery stenosis and 17 (50%) mid-aortic syndrome. Seventeen (50%) children showed intrarenal, six (18%) mesenteric, and three (9%) cerebrovascular involvement. Twenty patients underwent 45 percutaneous transluminal angioplasty procedures and seven children surgeries. In 44% of the 16 patients who underwent only percutaneous transluminal angioplasty blood pressure was normalized, 38% had improvement on same or decreased treatment and 19% showed no improvement. Complications were seen in 7.5% (5/66) of angiographies. In four of the seven (57%) children who underwent surgery blood pressure was normalized, two had improved (29%) and one unchanged (14%) blood pressure. CONCLUSION: In small children with renovascular hypertension below the age of 2 years, percutaneous transluminal angioplasty caused significant improvement in blood pressure with low complication profile. Surgery can be recommended where percutaneous transluminal angioplasty and medical treatments failed. WHAT IS KNOWN: • Renovascular hypertension is diagnosed in all age groups from a few weeks of life until adulthood. • Both angioplasty and surgery are significantly more difficult to perform in small children and the published information on short and long-term outcome in these children is very scarce. WHAT IS NEW: • Children below the age of two years can safely and successfully undergo selective renal angiography and also safely be treated with angioplasty. • We here present a large group of babies and infants where angioplasty and in some cases surgery effectively and safely improved their blood pressure.

Pancreatic pseudocyst drainage in children by image-guided cystogastrostomy and stent insertion.

BACKGROUND: Endoscopic ultrasound is seldom available at paediatric centres; therefore drainage of pancreatic pseudocysts in children has traditionally been achieved by surgery. OBJECTIVE: This study assessed the feasibility and safety of performing image-guided internal drainage of pancreatic pseudocysts with a flanged self-expanding covered nitinol pancreatic pseudocyst drainage stent. MATERIALS AND METHODS: We conducted a retrospective case note review of children undergoing image-guided cystogastrostomy at two paediatric hospitals. Percutaneous access to the stomach was achieved via an existing gastrostomy tract or image-guided formation of a new tract. Under combined ultrasound, fluoroscopic or cone-beam CT guidance the pancreatic pseudocysts were punctured through the posterior wall of the stomach. A self-expanding covered nitinol stent was deployed to create a cystogastrostomy opening. RESULTS: Image-guided cystogastrostomy was performed in 6 children (4 male; median age 6 years, range 46 months to 15 years; median weight 18 kg, range 13.8-47 kg). Two children had prior failed attempts at surgical or endoscopic drainage. Median maximum cyst diameter was 11.5 cm (range 4.7-15.5 cm) pre-procedure. Technical success was 100%. There were no complications. There was complete pseudocyst resolution in five children and a small (2.1-cm) residual pseudocyst in one. Pseudocyst-related symptoms resolved in all children. CONCLUSION: Pancreatic pseudocyst drainage can be successfully performed in children by image-guided placement of a cystogastrostomy stent. In this cohort of six children there were no complications.

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.

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.

Can radiological assessment of abdominal computerized scans diagnose encapsulating peritoneal sclerosis in long-term peritoneal dialysis patients?

AIM: Encapsulating peritoneal sclerosis (EPS) is a rare but potentially devastating complication of long-term peritoneal dialysis (PD). Changes to the peritoneal membrane occur with duration of PD therapy. To determine the potential effect of prospective computerized tomography (CT) scanning, we reviewed the scans of patients who had developed EPS compared with those without EPS. METHODS: We retrospectively compared CT scans that had been prospectively performed in a screening program for PD patients after 4 years of PD and compared scans from 18 patients with confirmed EPS and 26 vintage matched controls without EPS. Anonymized scans were reported independently by two blinded experienced radiologists. RESULTS: Peritoneal thickening, calcification, bowel tethering, thickening and dilatation were significantly more commonly reported in the EPS group. Total combined radiological scores, also including septation within peritoneal fluid, were significantly higher in the EPS group and the greatest for those who died as a consequence of EPS. Simplified scoring based on presence or absence, then for a score of ≥3.0, gave a receiver operating characteristic value of 0.87 for EPS, with a sensitivity of 78% and specificity of 85%, respectively. Inter-observer agreement varied from poor to good, being the greatest for calcification and bowel dilatation and the lowest for peritoneal thickening. CONCLUSION: CT scan reporting can differentiate EPS from peritoneal changes associated with duration of PD therapy. Severity of abnormalities was associated with clinical outcomes. However, inter-observer agreement varies with different radiological appearances, and future studies are required to determine weighting of radiological changes to provide prognostic information for clinicians and patients.

Vesical Artery Embolization in Haemorrhagic Cystitis in Children.

Haemorrhagic cystitis is an uncommon and, in its severe form, potentially life-threatening complication of haematopoietic stem cell transplantation or cancer therapy in children. The severe form involves macroscopic haematuria with blood clots, urinary obstruction and/or renal impairment. There are many therapeutic options to treat acute haemorrhage, but only recombinant factor VII has a high level of clinical evidence in children. Supraselective vesical artery embolization (SVAE) is an increasingly used therapeutic procedure for controlling haemorrhage in adults, but is less commonly used in children. This might be due to several factors, such as the invasive nature of the procedure, lack of appropriate medical experience and possible long-term side effects. We present three cases of children successfully treated by means of effective SVAE.

Vascular anomalies of the head and neck in children.

Sixty percent of vascular anomalies in children are found in the head and neck. These lesions can present throughout antenatal, perinatal and childhood development. They broadly fall into two categories: vascular tumours and vascular malformations. Their clinical and, often, psychological impact is determined by both pathological type and location: many lesions follow an uncomplicated natural course and other more complex, extensive or progressive lesions can present a threat to life from mass effect, haemorrhage or large volume arteriovenous shunting. Vascular tumours include infantile haemangioma (IH), congenital haemangioma (CH) and kaposiform hemangioendothelioma (KH); of which IH is the most common. Management options for vascular tumours include conservative approaches, oral medications and surgical intervention as determined by tumour type, location and associated complications. Vascular malformations can be categorised into low flow and high flow lesions. Low flow lesions include capillary, venous and lymphatic malformations (LMs). High flow lesions describe the arteriovenous malformations (AVMs), a highly heterogeneous group of lesions which can present in a variety of ways-the mainstay of treatment for these dynamic lesions is endovascular or surgical obliteration. We provide a practical framework for clinical classification of vascular anomalies of the head and neck in children. We also explore principles of their clinical and radiological assessment along with management, highlighting the importance of a multi-disciplinary approach.

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.

Pulmonary ablation: a primer.

Percutaneous image-guided thermal ablation is safe and efficacious in achieving local control and improving outcome in the treatment of both early stage non-small-cell lung cancer and pulmonary metastatic disease, in which surgical treatment is precluded by comorbidity, poor cardiorespiratory reserve, or unfavorable disease distribution. Radiofrequency ablation is the most established technology, but new thermal ablation technologies such as microwave ablation and cryoablation may offer some advantages. The use of advanced techniques, such as induced pneumothorax and the popsicle stick technique, or combining thermal ablation with radiotherapy, widens the treatment options available to the multidisciplinary team. The intent of this article is to provide the reader with a practical knowledge base of pulmonary ablation by concentrating on indications, techniques, and follow-up.

EASL and mRECIST responses are independent prognostic factors for survival in hepatocellular cancer patients treated with transarterial embolization.

BACKGROUND & AIMS: Standard RECIST criteria may not be the optimal method to assess response to loco-regional therapy for hepatocellular cancer (HCC). EASL and mRECIST, which measure changes in arterialized tumor, have been proposed. Here we compare the three criteria and their associations with survival. METHODS: Response was determined using RECIST 1.1, EASL, and mRECIST criteria in 83 consecutive patients with HCC undergoing palliative therapy with transarterial (chemo) embolization. Results were compared at the first assessment after therapy. Cox regression and Kaplan-Meier survival analyses were used to explore differences in overall survival between the responders and non-responders defined by each method. RESULTS: There was a good correlation between EASL and mRECIST with overall response rates; 58% and 57%, and target lesion responses; 74% and 73%, respectively. There was a poor correlation with RECIST 1.1 with overall and target response rates of 7%. Overall and target lesion progression rates were similar for all three assessments; 27% and 2% for both EASL and mRECIST and 28% and 6% for RECIST 1.1. There was a significant association between survival and overall EASL and mRECIST responses, which was retained in multivariate analysis. EASL response was associated with a 44% risk reduction and mRECIST with a 42% reduction. There was no significant association between survival for RECIST 1.1 responses or target EASL and mRECIST responses. CONCLUSIONS: When measured at a single, early time point post-therapy, EASL and mRECIST overall response rates are associated with survival and should be used in preference to RECIST 1.1 or target responses.

The smaller bowel: imaging the small bowel in paediatric Crohn's disease.

Crohn's disease begins in childhood in 20% of cases. Imaging of the small bowel is needed for diagnosis and management and also to inform the clinician of the location, extent, and activity of disease. There are several modalities available to image the small bowel and the combined use of these is often required to optimise benefit. Methods available for imaging the small bowel include barium studies, sonography, CT, wireless capsule endoscopy, nuclear medicine studies, and MRI. Patient comfort is paramount in imaging paediatric patients. Therefore, non-invasive techniques are most likely to be successful. Furthermore, as children are at greatest risk of radiation induced malignancy, modalities which do not carry a radiation burden are preferable. This article discusses the methods available for imaging the small bowel in paediatric Crohn's disease and the relative merits of each modality.

Synergy between vascular targeting agents and antibody-directed therapy.

PURPOSE: Tumor heterogeneity necessitates the use of combined therapies. We have shown that combining antibody-directed therapy with antivascular agents converts a subcurative to a curative treatment. The purpose of this study was to investigate, by radioluminographic and microscopic techniques, the regional effects of the two complementary therapies. METHODS AND MATERIALS: Nude mice bearing colorectal tumors were injected with 125I-labeled anti-carcinoembryonic antigen antibody, and images were obtained for antibody distribution and modeling studies using radioluminography. For therapy studies, the mice were given radioimmunotherapy alone (131I-A5B7 anti-carcinoembryonic antigen antibody), the antivascular agent combretastatin A-4 3-0-phosphate (200 mg/kg), or both. Extra mice were used to study the regional tumor effects of these therapies over time: relevant histochemical procedures were performed on tissue sections to obtain composite digital microscopic images of apoptosis, blood vessels, perfusion, hypoxia, and morphology. RESULTS: Antibody distribution, modeling, and immunohistochemistry showed how radioimmunotherapy (7.4 MBq/40 microg antibody) effectively treated the outer, well-oxygenated tumor region only. Combretastatin A-4 3-0-phosphate treated the more hypoxic center, and in doing so altered the relationship between tumor parameters. CONCLUSION: The combined complementary therapies produced cures by destroying tumor regions with different pathophysiologies. Relating these regional therapeutic effects to the relevant tumor parameters microscopically allows optimization of therapy and improved translation to clinical trials.