Incidence and clinical course of radionecrosis in children with brain tumors. A 20-year longitudinal observational study.

Radionecrosis (RN) in children treated for brain tumors represents a potentially severe long-term complication. Its diagnosis is challenging, since magnetic resonance imaging (MRI) cannot clearly discriminate between RN and tumor recurrence. A retrospective single-center study was undertaken to describe the incidence and clinical course of RN in a cohort of 107 children treated with external radiotherapy (RT) for various brain tumors between 1992 and 2012. During a median follow-up of 4.6 years (range 0.29-20.1 years), RN was implied by suspicious MRI findings in in 5 children (4.7 %), 5-131 months after RT. Suspicion was confirmed histologically (1 patient) or substantiated by FDG positron-emission tomography (FDG-PET, 2 patients) or by FDG-PET and MR spectroscopy (1 patient). Before developing RN, all 5 patients had received cytotoxic chemotherapy in addition to RT. In addition to standard treatment protocols, 2 patients had received further chemotherapy for progression or relapse. Median radiation dose expressed as the biologically equivalent total dose applied in 2 Gy fractions (EQD2) was 51.7 Gy (range 51.0-60.0 Gy). At RN onset, 4 children presented with neurological symptoms. Treatment of RN included resection (n = 1), corticosteroids (n = 2) and a combination of corticosteroids, hyperbaric oxygen (HBO) and bevacizumab (n = 1). One patient with asymptomatic RN was not treated. Complete radiological regression of the lesions was observed in all patients. Clinical symptoms normalized in 3 patients, whereas 2 developed permanent severe neurological deficits. RN represents a severe long-term treatment complication in children with brain tumors. The spectrum of clinical presentation is wide; ranging from asymptomatic lesions to progressive neurological deterioration. FDG-PET and MR spectroscopy may be useful for distinguishing between RN and tumor recurrence. Treatment options in patients with symptomatic RN include conservative management (steroids, HBO, bevacizumab) and surgical resection.

Necrosis of a skin autograft after short-term treatment with sunitinib in a 14-year-old girl with metastatic alveolar soft part sarcoma of the thigh.

A 14-year-old girl was diagnosed with alveolar soft part sarcoma (ASPS) of the thigh and lung metastases. She underwent tumor resection and pulmonary metastasectomy followed by hyperfractionated local radiotherapy (44.8 Gy). A mesh graft transplant was used to cover the postoperative skin defect on the thigh. Since ASPS do not respond to conventional chemotherapy antiangiogenic treatment with peginterferon alfa-2b and thalidomide was started. Immunohistochemical analysis of tumor tissue showed expression of vascular endothelial growth factor receptors (VEGFR) 1, 2, 3, and platelet derived growth factor receptor (PDGFR)-alpha and -beta. Hence, additional treatment with multitargeted receptor tyrosine kinase inhibitor sunitinib (Sutent) was started on a compassionate use basis. 2 weeks later the patient presented with necrosis of the skin transplant requiring necrectomy and skin grafting. This case illustrates that drugs inhibiting vascular endothelial growth factor receptors have to be used very cautiously in cancer patients with severe pre-existing skin damage.

Modified Toupet wrap for gastroesophageal reflux in childhood.

UNLABELLED: It was the aim of our study to follow up our clientele of infants and children who had undergone a partial (posterior) Toupet wrap or modified Toupet wrap. METHODS: All 22 children who had undergone a posterior partial wrap within a 4-year period were followed up for 4.0 years (0.6-5.7 years). 36.4% of children had associated anomalies such as operated esophageal atresia, operated congenital diaphragmatic hernia, Gregg's syndrome or arthrogryposis multiplex congenita. 14% of children suffered from some form of neurological impairment. Prior to operation the 4 main examinations for detection and documentation of gastroesophageal reflux (GER) disease were carried out where possible. Five children underwent Toupet fundoplication and in 17 children a modified Toupet fundoplication with reinforcement of the wrap fixation using a Vicryl-mesh was applied. A pH-metric study and upper gastrointestinal series with reflux testing were done in all 22 children at least 6 months postoperatively. Successful control of GER was documented in 21 of 22 children (95.5%). In the remaining child a reoperation was necessary to correct recurrence of a sliding hiatal hernia and GER. In three children (13.6%) postoperative complications occurred within the first 2 weeks following fundoplication and were managed medically. Following the postoperative 24-hour pH-metric study and the upper gastrointestinal series with reflux testing, one child with normal results at these investigations was lost to follow-up. The remaining 21 children were followed up for another 10 months to 5 years. Four children (18.2%) were found to suffer from functional complications (mild dumping syndrome, retrosternal pain, vomiting during episodes of asthma, dysphagia). No gas bloat syndrome occurred within the follow-up interval and all children were able to belch and vomit. There was no mortality in our limited series. Our series indicates that the partial (posterior) Toupet wrap and the modified Toupet fundoplication are safe and effective procedures for surgical correction of GER in children which preserve the ability of infants and children to belch and vomit.

[Balloon dilatation of postoperative and congenital aortic isthmus stenoses]. / Ballondilatation postoperativer und nativer Aortenisthmusstenosen.

From July 1987 to December 1992, 52 patients underwent balloon-angioplasty of aortic coarctation at three units of pediatric cardiology in Austria (Graz = 35 patients, Innsbruck = 15 patients, and Vienna = 2 patients). 35 patients had postoperative and 17 had native coarctation. Mean age at intervention was 7 10/12 years with 2 patients under 1 year and 5 patients over 18 years old. The mean relation balloon diameter-coarctation diameter was 2.6 +/- 0.9. The blood pressure gradient between upper and lower extremities decreased from a mean of 44 +/- 16 mm Hg to 15 +/- 13 mm Hg (p < 0.0001). The diameter of the stenosed segment was increased from 5 +/- 3 mm to 8.5 +/- 3.5 mm (p < 0.0001). Native coarctation showed a significantly better result in respect to decrease of the gradient (36 +/- 12 mm Hg) than did postoperative coarctations (25 +/- 19 mm Hg) (p < 0.03). 13 patients did not respond adequately to angioplasty. 10 patients out of this group had tubular narrowings and belonged to the group of postoperative coarctations, whereas localized stenoses in native coarctations gave the best results. Localized wall irregularities were found in 4 patients with native coarctation. Balloon angioplasty of postoperative and native coarctations in childhood and adolescence is a secure and effective means of treatment and should be considered as therapy of first choice. The best results can be found in the group of the circumscript type of stenoses in native coarctation, whereas long and tubular stenoses in the group of postoperative coarctations give less satisfying results.