Rare Course of Bilateral Congenital Diaphragmatic Hernia Treated Thoracoscopically-Case Report.

We present a rare case of metachronous bilateral congenital diaphragmatic hernia (CDH) in a newborn with additional malformations: macroglossia, pectus excavatum, and confirmed Simpson-Golabi-Behmel syndrome. We performed a successful thoracoscopic subsequent repair with a patch of the bilateral type C CDH. Despite using insufflation that should cause bilateral pneumothorax at first procedure there was no clinical signs of such a one. Contralateral CDH diagnosis was possible only on the basis of the symptoms that occurred after the first operation. After 13 months the patient is without recurrence. In selected patients, staged thoracoscopy may be a safe and feasible method of bilateral CDH treatment.

Assessment of Clinical Symptoms and Multichannel Intraluminal Impedance and pH Monitoring in Children After Thoracoscopic Repair of Esophageal Atresia and Distal Tracheoesophageal Fistula.

BACKGROUND: Motility disturbances of the esophagus and gastroesophageal reflux disease (GERD) are observed frequently in children after repair of congenital esophageal atresia with distal tracheoesophageal fistula (EA/TEF). Recently, in many pediatric surgical centers, thoracoscopic repair was introduced, which theoretically would change the postoperative course. OBJECTIVES: The goal of the study was to assess physical development, disease symptoms, and GERD symptom frequency in children who underwent thoracoscopic surgery of congenital EA/TEF. MATERIAL AND METHODS: The study comprised 22 children (14 boys and 8 girls), aged 16 to 79 months (average age 47.3 months) after surgery of EA/TEF. Clinical symptoms and physical development were analyzed. In 19 children, multichannel intraluminal esophageal impedance connected with pH-metry (MII/pH) was performed. In 11 patients, esophagogastroduodenoscopy with a histological study of mucosa samples was done. RESULTS: The most frequent symptoms were dysphagia, belching, cough and recurrent bronchitis. In 31.5% of the children, body mass deficiency was observed and in 28.6%, low body mass and short stature. Prematurity was present in half of the patients. Depending on the result of the MII/pH study, the children were divided into two groups: 10 children with GERD and 9 children without diagnosis of gastroesophageal reflux. In the 10 with GERD, acid reflux was diagnosed in 9 and non-acid reflux was diagnosed in one. MII/pH demonstrated statistically significant differences in the number of reflux episodes, reflux index, bolus exposure index, mean time of esophageal exposure and acid exposure and mean acid clearance time in children with GERD in comparison to children without this disease. In 36.4% of children who underwent endoscopy, esophagitis was diagnosed, esophageal stricture in 18% and gastric metaplasia in 9.1%. CONCLUSIONS: In children who underwent corrective thoracoscopic surgery of EA/TEF, GERD caused by motility disorders of the esophagus was frequently observed. These children require constant monitoring and early treatment of the complication.

Extrarenal nephroblastoma.

INTRODUCTION: Nephroblastoma is one of the most common solid tumours in children. The occurrence of extrarenal nephroblastoma is exceedingly rare. What can be defined as extrarenal Wilms' tumor must satisfy the following criteria: histologically confirmed nephroblastoma and extrarenal location. MATERIAL AND METHODS: Current data on extrarenal nephroblastoma based on a selective review of the literature. RESULTS: The retroperineal location is reported to be typical in males, whereas the inguinal region is believed to predominate in females. There are no characteristic manifestations of extrarenal nephroblastoma. The symptoms depend on the location and stage upon diagnosis. US, CT and MRI are used to detect tumours in the retroperiteneal space, inguinal, sacro-coccal and scrotal area. However, extrarenal nephroblastoma does not show characteristic radiological features. CONCLUSIONS: In absence of typical clinical presentation or conclusive imaging tests, the diagnosis is based on histology after the resection of the tumor. The recommended management of the extrarenal nephroblastoma is similar to the treatment of intrarenal nephroblastoma.