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.

[Malignant neurogenic tumors with retroperitoneal and mediastinal locations in infants].

Introduction: Malignant neurogenic tumors /MNT/ are the third by frequency malignancies in children with mainly localization in the retroperitoneal space and posterior mediastinum.

The Aim: of this study is to compare the clinical presentation and the treatment results by young children with retroperitoneal and mediastinal MNT.

Clinical Material: For a period of 12 years (1993 -2004) 52 children aged fron 1 month to 3 years were admitted at the Department of Pediatric surgery-University Hospital "N.

[Non-Hodgkin's primary intestinal lymphoma - a cause of acute abdominal manifestation in children].

Aim: Lymphomas of the gastrointestinal tract are the most common type of primary extranodal lymphomas, accounting for 5 to 10% of all non-Hodgkin's lymphomas (NHL).

Patients And Methods: From January 1996 to November 2005, 10 patients with primary intestinal lymphomas were submitted with clinical signs of acute abdomen to the Pediatric surgical department in Sofia. The children presented with radiologically proven intussusception, ileal obstruction or peritonitis due to bowel perforation.

[Contemporary treatment of parapneumonic pleural complication in children: the role of Video - Assisted Thoracoscopic Surgery (VATS)].

Aim: To reveal the role of Video Assisted Thoracoscopic Surgery (VATS) in the treatment of different stages of pleural empyema in children.

Clinical Material: Between 2004 and 2006, 87 children aged from 11 months to 18 years were treated at the Department of Thoracic pediatric surgery for parapneumonic pleural complications (pleural empyema). The children were admitted in a period from 5 to 30 days (mean 11.

[Clinical manifestation of acute pancreatitis in children with caustic ingestion injury - the role of oxidative stress].

For a 10 years period (1996-2005) 66 children with severe caustic injuries of the esophagus and stomach were admitted at the Department of Pediatric Surgery. Subject of this article are 17 children with clinical, laboratory and intraoperative proven acute pancreatitis. The patients were admitted at the clinic 12 hours to 12 days after the ingestion of the corrosive agent.

[Pleuropulmonary blastoma in children: diagnosis and results of surgery and complex treatment].

This is a report on a retrospective study of experience had with complex treatment of pleuropulmonary blastoma in ten children aged 2-11 years, covering a 27-year period. In all patients diagnosis is made on the ground of clinical symptomatology, imaging methods and histological findings. All patients undergo surgery, as follows: tumor extirpation (n = 3), lobectomy (n = 2) and pneumonectomy (n = 5).

[Surgical tactics and surgical techniques in echinococcal cysts in the lung in children].

The differentiated surgical approach to hydatid lung cysts and the operative techniques used in 343 children treated in the Department of Pediatric Surgery over 24 years (1975-1998) are presented. The left lung is involved in 143 children (41.7%), the right--in 155 (45.

[The surgical treatment of pulmonary and associated echinococcosis in childhood].

Over a nine-year period (1990-1998), a total of 196 children with pulmonary and associated hydatid disease, aged 2 to 16 years, undergo treatment in the Department of Pediatric Thoracic Surgery--Emergency Medicine Institute "N. I. Pirogov"--Sofia.

[Traumatic lesions of the thoracic and abdominal organs in childhood].

This is a retrospective analysis of 651 children, aged 0 to 14 years, presenting chest and abdominal injuries, treated over a 6-year period in the section of pediatric surgery of the Emergency Medicine Institute "Pirogov". The results of studies on the etiopathogenesis of chest and abdominal trauma, patterns of associated lesions and quality of prehospital aid are discussed. Experience with the application of updated noninvasive and invasive methods of diagnosis, as well as the scope of surgical approach to the variety of lesions by resorting to organ sparing and organ salvaging operative procedures are analyzed.

[Bronchogenic cysts of the mediastinum in childhood].

Bronchogenic cysts are rare congenital anomalies result of abnormal development of the tracheobronchial tree. They develop in the lung parenchyma or in the mediastinum. For a period of 17 years (1975-1991) a total of 46 children with bronchogenic cysts have been treated; in 6 the cysts were localized in the mediastinum.

[The results of coloesophagoplasty in childhood].

Over a six-year period (1988 through 1993), a total of 380 children with congenital and acquired esophageal diseases undergo treatment in the Second Pediatric Surgery Clinic. Plastic replacement of esophagus using a graft from the colon is performed in 31 children. The indications and procedures for colo-esophagoplasty in children are discussed.

[Traumatic rupture of the diaphragm in combined trauma in childhood].

Blunt traumatic injuries to the diaphragm are comparatively rare and are always associated with other severe injuries to the chest, abdomen, head and extremities. In childhood they are exceptionally rare. No such complication in blunt abdominal and thoracic trauma has been observed at the Clinic of Pediatric Surgery of the "Pirogov" Emergency Hospital in Sofia for the last ten years.

[The diagnosis and surgical treatment of teratogenic tumors of the mediastinum in childhood].

Mediastinal tumors are leading tumors in pediatric chest oncology. Teratogenic mediastinal tumors are characteristic of early childhood. For a period of 7 years 4 children with teratogenic tumors of the mediastinum--7.