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We studied the ability of pre- and postcaptopril renal scintigraphy to predict renovascular disease (RVD) in children. Retrospective review of medical notes and radiology reports of all hypertensive children who had had both pre- and postcaptopril renal scintigraphy with [[sup.99m]Tc] dimercaptosuccinic acid (DMSA) and/or [[sup.99m]Tc] mercaptoacetyltriglycine (MAG3) and digital subtraction angiography (DSA). 81 children aged 1-18 (median 10) years were studied with 62% (51) having a diagnosis of RVD. Main renal artery disease, intrarenal disease, and both main and intrarenal artery disease were present in 25, 14, and 12 patients respectively. The isotope study accurately diagnosed RVD, confirmed by DSA, in 47% (24 of 51) children, with eight false positive studies. The sensitivity, specificity, and positive and negative predictive values of the isotope study to predict RVD were 48%, 73%, 76%, and 51%, respectively. Pre- and postcaptopril renal scintigraphy was unable to predict RVD in children. Keywords Renal isotope * Angiography * Scintigraphy * Vascular * Hypertension * Stenosis, Introduction The prevalence of hypertension in children is 1-3% and is caused mainly by renal disease in infants and young children [1]. However, with increased obesity in children, primary hypertension [...]

Achalasia is a rare esophageal motility disorder: its optimal treatment in children is still a matter of debate. Records of children treated for achalasia, over an 18-year period, were reviewed.Forty-eight children (median age at diagnosis 10 years; range 3-17 years) were identified. Twenty-eight patients were initially treated with Heller's myotomy (HM) and 20 with balloon dilatation (BD). At last follow-up (median 3 years; range 1-5.5 years), 43.8% (21/48) of children were symptom free. The number of asymptomatic children was significantly higher among those treated initially with HM compared to BD (HM 15/28, 53.6% BD 6/20, 30%, p < 0.05). All children who underwent BD required HM due to symptom recurrence. The median (range) total number of procedures was significantly higher in the BD group (BD 3 (1-7); HM 1 (1-5); p < 0.05) with a shorter time to the second intervention (BD 14 months, 95%CI 4-24; HM 58 months, 95%CI 38-79; p < 0.05). Of 108 procedures, esophageal perforation occurred in two children after HM (two out of 48 HM procedures in total, 4%) and one child after BD (1/60, 1.7%).Conclusion: Less than half of children with achalasia are symptom free after initial treatment with either BD or HM. HM, however, when performed as first procedure, provided longer symptom-free period and reduced need for subsequent intervention. What is Known: • Balloon dilatation (BD) and Heller's myotomy (HM) are safe and effective treatment options for achalasia. • Controversy, however, exists regarding the most effective initial therapeutic approach. What is New: • HM with or without fundoplication may represent the initial therapeutic approach of choice. • Initial BD may negatively affect the outcome of a subsequent HM. [ABSTRACT FROM AUTHOR]

Objectives: Renovascular disease (RVD) is found in about 10 % of secondary childhood hypertension. Digital subtraction angiography (DSA) is the gold standard to diagnose RVD. Non-invasive imaging methods like Doppler ultrasound (US), magnetic resonance angiography (MRA), and computed tomography angiography (CTA) are increasingly used. Our aim was to evaluate the role and accuracy of US, MRA, and CTA compared to DSA in diagnosing RVD in children. Patients and methods: A retrospective review of 127 children with suspected renovascular hypertension was performed in children referred to Great Ormond Street Hospital between 2006 and 2014 due to clinical suspicion of renovascular hypertension and/or findings on US and/or MRA or CTA. Results: Ninety-nine of 127 children (78 %) were diagnosed with renovascular disease and 80 were treated with percutaneous transluminal angioplasty during the same procedure. The median age at presentation was 5.6 (range, 2.5-10.6) years. Thirty-six children had unilateral renal artery stenosis in major extraparenchymal vessels, 47 bilateral stenosis and 16 intrarenal small vessel disease. US had a sensitivity of 63 % and specificity of 95 %. MRA and CTA were performed in 39 and 34 children, respectively. CTA sensitivity was slightly higher than that of MRA, 88 vs. 80 %, and specificity 81 vs. 63 %. Conclusions: The sensitivity of MRA and CTA is still too low to reliably rule out renovascular disease in children with a strong suspicion of this diagnosis. DSA remains the gold standard to diagnose renovascular hypertension and is recommended when clinical and laboratory criteria are highly suggestive of renovascular disease even with normal radiological investigations from non-invasive methods. [ABSTRACT FROM AUTHOR]

Objective Polyarteritis nodosa (PAN) is a rare disease of childhood. The aims of this study were to describe the clinical features, treatment, and outcome of systemic childhood PAN and to identify predictors of relapse. Methods A single-center retrospective medical records review of children with PAN fulfilling the European League Against Rheumatism (EULAR)/Paediatric Rheumatology European Society (PRES)/Paediatric Rheumatology International Trials Organisation (PRINTO) classification criteria who were seen over a 32-year period was performed. Data on demographic and clinical features, treatments, relapses (recurrence of clinical signs/symptoms or occurrence of new symptoms after initial remission requiring escalation or resumption of immunosuppressive therapy), and deaths were recorded. A disease activity score was retrospectively assigned using the Paediatric Vasculitis Activity Score (PVAS) instrument. Cox regression analysis was used to identify significant predictors of relapse. Results Sixty-nine children with PAN were identified; 55% were male, and their median age was 8.5 years (range 0.9-15.8 years). Their clinical features at presentation were fever (87%), myalgia (83%), skin (88%), renal (19%), severe gastrointestinal (GI) (10%), and neurologic (10%) involvement. The PVAS at presentation was 9 of 63 (range 4-24). Histopathologic analysis of the skin showed necrotizing vasculitis in biopsy samples from 40 of 50 children. Results of selective visceral arteriography suggested the presence of PAN in 96% of patients. Treatment included cyclophosphamide and corticosteroids (83%), plasma exchange (9%), and biologic agents (after 2002; 13%). The relapse rate was 35%, and the mortality rate was 4%. Severe GI involvement was associated with increased risk of relapse ( P = 0.031), while longer time to induce remission ( P = 0.022) and increased cumulative dose of cyclophosphamide ( P = 0.005) were associated with lower relapse risk. Conclusion Childhood PAN is a severe inflammatory disease of insidious onset and variable clinical presentation. Relapses occurred more frequently in those with severe GI involvement. A higher cumulative dose of cyclophosphamide was associated with a lower risk of relapse. [ABSTRACT FROM AUTHOR]

Aim "Buried bumper" is a complication of percutaneous gastrostomy related to the internal flange getting buried into the wall of the stomach. The aim of this study is to evaluate the management of this complication. Methods The surgical and interventional radiology database in our hospital from August 1999 to May 2011 was analyzed. There were 2,007 patients who underwent percutaneous gastrostomy insertion. Notes for patients with buried bumper were reviewed. A telephonic interview with the parents of these children was performed with focused assessment of the care of the gastrostomy tube before the episode of buried bumper. Continuous data are reported as median (range). Results Twenty children developed buried bumper after gastrostomy insertion. They had a primary diagnosis of neurological (n = 14), metabolic (n = 3), or endocrine (n = 3) disorders. The age at presentation was 5.7 years (2 to 18 years); 2.5 years (1 month to 5 years) after gastrostomy insertion. Ten children (50%) presented with symptoms related to buried bumper which included leakage around the gastrostomy (n = 4), pus, discharge or bleeding from the site (n = 5), stiffness on feeding (n = 3), and unable to push the flange (n = 1) (three children had more than one symptom). Ten children (50%) were asymptomatic and underwent routine change or removal of gastrostomy. In nine children, there was an attempt to remove the flange by interventional radiology but this was successful only in one. In the remaining 19 children, 4 had endoscopic removal while 15 children developed an inflammatory mass and required a laparotomy (n = 12) or laparoscopic-assisted excision (n = 3). Conclusion Buried bumper is a rare complication of percutaneous gastrostomy. Inadequate postoperative care without appropriate mobilization is a factor leading to this preventable complication. Endoscopic removal is possible, failing which laparoscopic surgery should be considered. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: To identify factors relevant to long-term outcome in newly diagnosed hepatoblastoma, and define subgroups for clinical research on tailoring treatment to the individual patient. Patients and methods: Between 1995 and 2006 the SIOPEL group conducted two clinical trials which established risk-adapted therapy for hepatoblastoma patients. Patients were stratified into high-risk (AFP<100ng/mL and/or PRETEXT IV and/or vascular invasion and/or extra-hepatic intra-abdominal disease (V+/P+/E+) and/or metastases) and standard-risk (all others). The hierarchy of these factors plus multifocality, PRETEXT III, AFP>1,200,000ng/mL, patient age, platelet count and histology were further explored. The outcome measure was event-free survival (EFS). Results: In 541 patients, reduced EFS correlated significantly with AFP<100ng/ml (hazard ratio [HR] 4.09, 95% confidence interval 2.16–7.75), AFP⩾1.2×106 ng/mL (2.48, 1.47–4.17), metastatic disease (3.02, 2.05–4.44), PRETEXT IV (2.15, 1.19–3.87), multifocality (1.59, 1.01–2.50), age>5years (2.76, 1.68–4.53); borderline with small cell undifferentiated (SCU) histology (2.29, 95% confidence interval 0.91–5.77); but not with PRETEXT III, age 30–60months, platelet count or V+/P+/E+. By using the significant factors and SCU to stratify the population, we have identified three distinct prognostic groups: PRETEXT I/II/III, and no other factors, have 3year EFS of 90%, PRETEXT IV and/or multifocal tumour and/or age>5years and/or AFP>1.2×106 have 3year EFS of 71% and SCU and/or AFP<100ng/mL and/or metastatic have a 3year EFS of 49%. Conclusion: Prognostic stratification for clinical research on newly diagnosed hepatoblastoma should take into consideration PRETEXT, metastatic disease, AFP, multifocality, age and SCU histology. [Copyright &y& Elsevier]

Mid aortic syndrome (MAS) is a rare condition often presenting with severe hypertension. It is characterized by narrowing of the abdominal aorta. We here describe a case of complete occlusion of the abdominal aorta presenting in infancy. This child presented at four months of age with heart failure and hypertension. CT angiogram showed total narrowing of the abdominal aorta. This was initially felt to be too severe for surgical treatment and he was planned for palliative care. We were however able to improve his blood pressure with antihypertensive agents and he underwent succesful angioplasty at five and a half months of age. He has required three further angioplasty procedures and still needs two antihypertensive agents to control his blood pressure. His renal function remains normal and at age six years he has excellent quality of life with normal growth and development. This case illustrates that the combination of medical treatment and angioplasty can give an excellent long-term treatment response even in children with extremely severe MAS. This boy now has normal blood pressure and has experienced normal growth, development and quality of life. [ABSTRACT FROM AUTHOR]

Background. We retrospectively reviewed the medical records of all patients who underwent surgery as part of the treatment of renovascular hypertension (RVH) at our centre between 1979 and 2008. [ABSTRACT FROM PUBLISHER]

SUMMARY Rhabdoid tumour of the kidney is an uncommon malignancy with a poor prognosis that usually occurs in childhood. Certain imaging features may help distinguish this lesion from the much more common Wilmstumour '. Extra-renal rhabdoid tumours appear to have no specific imaging characteristics. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Hyponatremia in a Child With Takayasu Arteritis: Questions and Answers" by S. Masavkar et al.

Abstract: Purpose: To assess the clinical activity of irinotecan as single drug in children with refractory or recurrent hepatoblastoma. Patients and methods: Four cycles of irinotecan were administered (20mg/m2/day intravenous (i.v.) infusion on days 1–5 and 8–12, every 21days) unless tumour progression occurred or resectability was achieved earlier. Tumour response was assessed according to modified SIOPEL and Response Evaluation Criteria In Solid Tumours (RECIST) criteria. Main end-points were best overall response rate (RR), early progression rate (EPR) and progression free survival (PFS). Results: Twenty-four eligible patients (median age 58.0months; 19 boys) were enrolled in the study (11 relapses, 13 refractory diseases). Of the 23 evaluable patients six had an overall partial response, 11 stable disease and six progressive disease, of which four were early progression (RR: 26%, EPR: 17%). In eight patients the residual tumour could be completely resected; seven patients became tumour free. At last follow-up 12 patients were alive (six with no evidence of disease, six with disease). PFS at 1year was 24%. Patients with relapse had a higher RR than patients with refractory disease (46% versus 8%) and patients with isolated lung lesions showed a better response than patients with other tumour localisations (50% versus 13%). The main grade 3–4 toxicities, diarrhoea and neutropenia, occurred in half of the patients. Conclusion: Irinotecan has a significant anti-tumour activity and acceptable toxicity in patients with relapsed hepatoblastoma and therefore should be considered for the treatment of these patients. Exploration of the role of irinotecan in the initial treatment of hepatoblastoma is warranted. [Copyright &y& Elsevier]

Abstract: Cisplatin-containing chemotherapy and complete surgical resection are both crucial in the cure of hepatoblastoma. Radical resection can be obtained either conventionally by partial hepatectomy or with orthotopic liver transplant, but the surgical approach to hepatoblastoma differs considerably across the world. Our main aim in this paper is to present the surgical recommendations of the Childhood Liver Tumour Strategy Group of the International Society of Paediatric Oncology (SIOPEL), as well as to stimulate international debate on this issue. We discuss biopsy, verification of resectability, resection principles, indications and potential contraindications for orthotopic liver transplant, as well as thoracic surgery for pulmonary metastases. We suggest that heroic liver resections with a high probability of leaving residual tumour should be avoided whenever possible. In such cases primary orthotopic liver transplant should be considered. Superior survival rates in hepatoblastoma patients who have received a primary transplant after a good response to chemotherapy support the strategy of avoiding partial hepatectomy in cases where radical resection appears difficult and doubtful. We recommend early referral to a transplant surgeon in cases of: (i) multifocal or large solitary PRETEXT IV (PRE Treatment EXTent of disease scoring system) hepatoblastoma involving all four sectors of the liver and (ii) unifocal, centrally located tumours involving main hilar structures or main hepatic veins. Because complete tumour resection is a prerequisite for cure, any strategy leading to an increased resection rate will result in improved survival. We advise the more frequent use of orthotopic liver transplant, as well as the standardisation of techniques for partial liver resection. These guidelines should not be seen as final, but rather as a starting point for further discussion between the various national and international liver tumour study groups. [Copyright &y& Elsevier]