Pleuropulmonary blastoma (PPB) is a rare and potentially aggressive intrathoracic disembryonic neoplasm typically occurring in children less than 6 years of age. We assessed the relative incidence, clinical characteristics, treatment outcome, and the prognostic factors for long-term survival in patients with PPB treated at our institution over a 25-year period, and compared these data with reports in the literature. From 1985 to 2010, 11 children (4 males and 7 females), with a median age of 5.4 years (range, 1-12 years) were treated at our hospital. Here we described the main characteristics of these patients, the diagnostic methods, and treatment modalities used. During a median follow-up period of 80, 9 months, the overall survival (OS) and disease-free survival (DFS) rates were 54, 6% and 45, 5%, respectively. Two patients survived for more than 20 years. The main prognostic factors for long-term survival were the diseases type I and II and treatment with radical surgery. Our results show that in order to improve the prognosis of patients with PPB a timely in our opinion and accurate diagnosis needs to be established and treatment should be offered according to the disease type and extend of dissemination.

Our aim is to present the treatment of one of the skeletal manifestations of Jeune's syndrome (JS), the hypoplastic chest, which can result in thoracic insufficiency syndrome and present “on-demand” stage surgical technique using mandible locking plate system for the fixation of ribs. The diagnosis “Jeune's syndrome” was presented clinically in a 3-month-old girl from a family in which the first child died of JS at the age of 18 months. After close follow-up for several months and preoperative planning, we decided to make reconstructive chest operation with atypical use of a double-angled mandible locking plate for fixation. The plate was shaped as a “crown” to ensure the three dimension stability, from the dorsal part of the most curved ribs (paravertebrally) to the sternum after the resection of this area. Operation was done at the period of worsened breathing. For nearly 1 year, the rib cage preserved its stability and the child was in good condition. During the next 3 months, the upper part of the deformation started to grow inward fast. Second operation was “on demand,” and the implants used were mandible locking plates curved anterolaterally to effectuate extension of the rib cage and the sternum. In both the reconstructive operations, we spared the rectus and pectoral muscles and achieved good enlargement of the thoracic volume. The postoperative period is smooth and the child is active, without complications. We believe that in the future, the treatment should be “on demand” according to the course of the illness and the results of the follow-up examinations and adequate to the progress of chest wall deformity.

Aim To present a new therapy for Poland syndrome (PS) using a novel surgical approach: the vertical expandable prosthetic titanium rib (VEPTR) system. Methods The VEPTR system rib-to-rib variant was used to enhance the chest wall and vertebral column support in a young patient before walking age. Case Report We present a 12-month-old infant diagnosed with left-sided PS at the age of 6 months associated with missing ribs, scoliosis, and absence of the left pectoral muscles. Because of four missing ribs, paradoxical breathing was present. In addition, the left scapula was protruding into the chest due to the missing rib support. Scoliosis was caused by a left-sided nonsegmented bar of the thoracic spine. Results We decided to use the VEPTR system before the patient reached walking age to prevent progression of column deformation and future pulmonary problems. To improve the spinal deformity, to stabilize the thorax, and to improve thoracic function, we performed the operation at 1 year of age. At 10-month follow-up, the patient was reevaluated. The construction was still stable and scoliosis had not deteriorated. Conclusion The VEPTR system is a choice of treatment in young patients with PS to prevent late complications after a child reaches walking age.

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SummaryPneumonia is an inflammatory lung disorder characterized by consolidation due to presence of exudates in the alveolar spaces. Most pneumonias can be effectively treated with appropriate oral antibiotics, with intravenous antibiotics being reserved for those with severe infections. We present two cases of girls admitted in our clinic with pneumonia where our conventional therapy was not sufficient. Case 1: A 15-year-old girl with cystic fibrosis, with left lobular pneumonia, for which an aggressive conservative treatment was initiated. After significant improvement, sudden detorioration and pneumothorax of the left lung occurred. She was transferred to the surgical department for intervention. Due to failure to respond to initial drainage she underwent thoracotomy and resection of the left lower lobe of the lung. The histology result confirmed gangrene. Case 2: A four-year old girl was treated for pneumonia in the right lung with aggressive intravenous antibiotic. After temporary improvement sudden deterioration was observed. The patient was transferred to the surgery department, where pulmonary gangrene was confirmed. After the lower lobe of the right lung was resected, she was discharged in good health. The careful follow up, accurate diagnosis and correct medication choice are crucial for reducing the complications of “common” pneumonia.