Papillary fibroelastoma of the left atrium in a 3-year-old boy

A 3 year old boy presented with a cardiac murmur. Except for mildly impaired physical condition that was more likely due to asthmatic bronchitis, he was asymptomatic. Cardiac echocardiography disclosed a large tumor in the left atrium originating from the interatrial septum. The patient was immediately referred to surgery for excision of the tumor. Histological examination of the tumor showed a typical papillary fibroelastoma. The occurrence of a papillary fibroelastoma in a child is a rarity

Stem-cell-based, tissue engineered tracheal replacement in a child: a 2-year follow-up study.

Background Stem-cell-based, tissue engineered transplants might off er new therapeutic options for patients, including children, with failing organs. The reported replacement of an adult airway using stem cells on a biological scaff old with good results at 6 months supports this view. We describe the case of a child who received a stem-cell-based tracheal replacement and report fi ndings after 2 years of follow-up. Methods A 12-year-old boy was born with long-segment congenital tracheal stenosis and pulmonary sling. His airway had been maintained by metal stents, but, after failure, a cadaveric donor tracheal scaff old was decellularised. After a short course of granulocyte colony stimulating factor, bone marrow mesenchymal stem cells were retrieved preoperatively and seeded onto the scaff old, with patches of autologous epithelium. Topical human recombinant erythropoietin was applied to encourage angiogenesis, and transforming growth factor β to support chondrogenesis. Intravenous human recombinant erythropoietin was continued postoperatively. Outcomes were survival, morbidity, endoscopic appearance, cytology and proteomics of brushings, and peripheral blood counts. Findings The graft revascularised within 1 week after surgery. A strong neutrophil response was noted locally for the fi rst 8 weeks after surgery, which generated luminal DNA neutrophil extracellular traps. Cytological evidence of restoration of the epithelium was not evident until 1 year. The graft did not have biomechanical strength focally until 18 months, but the patient has not needed any medical intervention since then. 18 months after surgery, he had a normal chest CT scan and ventilation-perfusion scan and had grown 11 cm in height since the operation. At 2 years follow-up, he had a functional airway and had returned to school. Interpretation Follow-up of the fi rst paediatric, stem-cell-based, tissue-engineered transplant shows potential for this technology but also highlights the need for further research. Funding Great Ormond Street Hospital NHS Trust, The Royal Free Hampstead NHS Trust, University College Hospital NHS Foundation Trust, and Region of Tuscany

Understanding Interventional Bronchoscopy

Pediatric flexible bronchoscopy as a diagnostic modality has become a standard tool in the armamentarium of the modern pediatric pulmonologist. However the feasibility of interventions through flexible bronchoscopy is emerging slowly as it is limited not only by the size of the pediatric bronchoscope and its working channel but also by the demarcation lines between flexible and rigid bronchoscopy. We organize this chapter on interventional bronchoscopy into the following broad topics: Use of flexible bronchoscopy for acquisition of diagnostic material.Bronchoscopy for removal of obstructive, noxious, or damaging materials from the airway or the lung.Management of the narrowed or obstructed airway: debridement, dilation, and stenting.Use of bronchoscopy for other procedures including: sealing of fistulae and pneumothorax, control of diffuse alveolar hemorrhage, and segmental bronchography.New horizons: a discussion on recent reports on fetal bronchoscopy as a novel interventional approach