Is Asphyxiating Thoracic Dystrophy (Jeune's Syndrome) Deadly and Should We Insist on Treating It? Reconstructive Surgery "On Demand".

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.

Poland Syndrome: Use of Vertical Expandable Prosthetic Titanium Rib System before Walking Age-A Case Report.

 To present a new therapy for Poland syndrome (PS) using a novel surgical approach: the vertical expandable prosthetic titanium rib (VEPTR) system.  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.  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.

Diagnosis and treatment of pleuropulmonary blastoma-single center experience.

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.

Emergency surgical treatment of cervico-mediastinal lymph-haemangioma in a 2-month-old infant, indicated by acute inner tumour haemorrhage.

Unlabelled: Cervico-Mediastinal Lymphangiomas (CML) are caused by atresia of the efferent lymphatics or lack of communication between lymphatics and venous channels. Cervico-Mediastinal Lymphangiomas in certain cases require emergency surgery to secure decompression of the airways. The purpose of this study is to present a multidisciplinary surgical approach in the treatment of urgently complicated Cervico-Mediastinal Lymphangiomas, and to asses the surgical tactics and satisfactory outcome after a two-stage operation.

[Surgical aspects of primary tuberculosis in children: clinical presentation and indications for surgical treatment].

Aim: During the last years a trend of increasing the cases with primary pulmonary tuberculosis (PPT) in our country is observed. The aim of this study is to analyze retrospectively the pediatric cases with PPT and to determinate the specific clinical features as well the indications for surgery.

Material And Methods: For a period of six years (2000-2005) thirteen children with PPT were treated at the Department of pediatric surgery.