Primary mediastinal malignancies are rare tumors and can originate from any mediastinal organ or tissue such as thymic, neurogenic, lymphatic, germinal, or mesenchymal. The authors reviewed all cases of primary pediatric mediastinal masses diagnosed over a 25-year period to determine the pattern of presentation, the histology, and the outcome of the surgical treatment. In this study, 120 primary pediatric mediastinal mass cases diagnosed between 1985 and 2011 are retrospectively evaluated according to their age, sex, symptoms, anatomical location, surgical treatment, and histopathological evaluation. The median age of the patients was 5.8 years. There were 34 benign and 86 malign tumors. Thirty patients were asymptomatic. Common symptoms in the patients were cough, dyspnea, fatigue, fever, abdomen pain, back pain, and neurological symptoms. According to their origins, they were presented as neurogenic tumors (38.3%), lymphomas (18.3%), undifferentiated sarcomas (15%), germ cell tumors (7.5%), and the other tumors (22%) thymic pathologies, lymphangiomas, rhabdomyosarcomas, lipomas, hemangiomas, and Wilms' tumor. Complete resection of the tumor was performed in 86 patients, partial resection of the tumor was the intervention in 11 patients. In 23 patients, biopsy was undertaken. Because of the high incidence of asymptomatic or nonspecific presentation such as the upper airway disease, the presentation of a mediastinal mass in children may be challenging. Neurogenic tumors or lymphomas are indicating surgery, if possible complete resection, for both benign and malignant conditions. Although surgery is the mainstay of therapy for most mediastinal tumors, an experienced multidisciplinary approach is necessary.

Due to the variability in the site of enteric duplications, a wide range of presenting symptoms can exist, which is challenging for diagnosis. In children with a diagnosis of acute abdomen, enteric duplication cysts should be considered, and these children should be further investigated for additional skeletal, urogenital, and gastrointestinal system pathologies. Surgical treatment depends on the site and type of the cyst.

OBJECTIVETo report experience of gender (re)assignment in genotypic female (46XX) patients with congenital adrenal hyperplasia (f‐CAH), a difficult and stressful experience if complicated with delayed presentation and inadvertent assignment.PATIENTS AND METHODSBetween 1983 and 2002, 70 patients with f‐CAH were counselled for gender assignment. The age at diagnosis and operation, the degree of virilization, parental consanguinity, the gender preference of the families, and the factors governing the decision‐making process were determined.RESULTSForty‐one (59%) patients presented after the neonatal period. All parents had already assumed or were advised of a gender for their children, based on the suggestive appearance of the external genitalia. Consequently, 49 patients were reared as female and 21 as ‘male’. Only nine of these ‘males’ could be reassigned as females (mean age at presentation 7.87 months, sd 10.42). Twelve children had to be reared as ‘male’ (mean age at presentation 55.8 months, sd 32.42) in compliance with the parents’ and the study group's decision, and appropriate masculinizing reconstructive surgery was undertaken. The difference in the mean age of those reassigned as female and those who remained ‘male’ was significant (P < 0.001). The parental consanguinity rate among the families was especially high in the ‘male’ patients.CONCLUSIONSIt is extremely difficult to correct the gender of patients with f‐CAH when they present at >2.5 years old. Furthermore, the delay in diagnosis and the male bias in choice of gender in our population might be a result of strong social pressures on families, influenced by cultural, traditional and economic factors.

BackgroundThis study aims to evaluate the outcomes of pulmonary metastasectomy resections in pediatric patients.MethodsWe retrospectively reviewed the medical records of 43 children who were operated on in the Pediatric Surgery Clinic between January 1988 and 2014. Forty-three children (26 boys; 17 girls; mean age 10 ± 4.24 years, range 6 months–18 years) who underwent pulmonary metastasectomy resection were included in the study. The patients were evaluated based on age, gender, history of disease, surgical procedures, complications, duration of hospitalization, duration of chest tube placement, and procedure outcome.ResultsIndications for pediatric resections were oncological. Metastasis was secondary to Wilms’ tumor in 14 patients, osteosarcoma in 7 patients, Ewing’s sarcoma in 5 patients, rhabdomyosarcoma in 5 patients, lymphoma in 3 patients, hepatoblastoma in 2 patients, and other tumors in 7 patients. A total of 59 thoracotomies were performed. Approaches utilized included unilateral posterolateral thoracotomy (n = 33), bilateral posterolateral thoracotomy (n = 8), and sternotomy (n = 2). Wedge resection was the procedure of choice (n = 44). In selected cases, 11 segmentectomies, 3 lobectomies, and 1 pneumonectomy were performed. There was no perioperative mortality. One patient suffered prolonged air leak and three patients from fever. All patients received chemotherapy. Radiotherapy was administered to 16 patients (37.2 %). Of those 16 patients, 7 had Wilms’ tumor, 6 had Ewing’s sarcoma/PNET, and 3 were rhabdomyosarcoma patients. During a median follow-up of 3 years, the overall survival was 74.4 %.ConclusionsMultidisciplinary treatment involving pediatric oncologists, surgeons, and radiation oncologists is necessary to obtain positive results in children who have pulmonary metastases of oncological diseases. Wedge resection is a suitable option for children because less lung tissue is resected.

During the past 25 years, 252 children underwent repair of pectus deformities. There were 195 male and 57 female patients, of whom 227 had pectus excavatum and 25 had pectus carinatum. Of the 252 patients, 113 underwent repair at between 2 and 5 years of age. Exercise limitation was reported by 51 %, and 32 % had frequent respiratory infections or asthma. Repair was performed through a transverse incision with subperiosteal resection of the lower four or five costal cartilages, from sternum to costochondral junction bilaterally. A transverse wedge osteotomy was made through the anterior table of the sternum, with fracture but no displacement of the posterior table. For children younger than 5 years (n = 108), the periosteal sheath of the fifth rib from each side was sewn together behind the sternal tip. For older patients (n = 136), a thin steel strut was used for sternal support for 6 months. There were no deaths within the first year. Complications included seroma (16), atelectasis (12), pneumothorax (three), and recurrent chest depression (three). More than 98 % of patients had improvements in exercise tolerance, endurance, respiratory symptoms, and cosmetic appearance; these improvements were considered excellent results. Operation at an early age with routine use of substernal support with minimal preoperative and postoperative testing has provided exceUent results at a low cost.