Biliary rhabdomyosarcoma (RMS) is the most common biliary tumor in children. The management of affected patients contains unique challenges because of the rarity of this tumor entity and its critical location at the porta hepatis, which can make achievement of a radical resection very difficult. In a retrospective chart analysis we analysed children suffering from biliary RMS who were registered in three different CWS trials (CWS-96, CWS-2002P, and SoTiSaR registry). Seventeen patients (12 female, 5 male) with a median age of 4.3 years were assessed. The median follow-up was 42.2 months (10.7–202.5). The 5-year overall (OS) and event free survival (EFS) rates were 58% (45–71) and 47% (34–50), respectively. Patients > 10 years of age and those with alveolar histology had the worst prognosis (OS 0%). Patients with botryoid histology had an excellent survival (OS 100%) compared to those with non-botryoid histology (OS 38%, 22–54, p = 0.047). Microscopic complete tumor resection was achieved in almost all patients who received initial tumor biopsy followed by chemotherapy and delayed surgery. Positive predictive factors for survival of children with biliary RMS are age ≤ 10 years and botryoid tumor histology. Primary surgery with intention of tumor resection should be avoided.

Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung’s disease occurring in 3–10% of the cases. Only few studies reported the long-term clinical and metabolic outcomes of patients with TCA. The aim of this study was to evaluate the functional and metabolic long-term outcomes of children undergoing surgical treatment for TCA. A 15-year retrospective study was performed. Blood chemistry tests and stool analysis performed at the last follow-up visit were recorded. Height and weight development were assessed using the corresponding percentiles for age. Faecal continence and quality of life were evaluated using a detailed questionnaire. Eleven patients were included in the study. The median age at surgery was 6 months (range: 3–72 months). After histological confirmation, all patients underwent a total colectomy. Ileoanal anastomosis (n = 6), ileorectal anastomosis (n = 1), J-pouch (n = 1) and Duhamel procedure (n = 3) were performed. Temporary ileostomy was closed after a median of 8 weeks in 10/11 patients. After a median follow-up of 78 months (range: 27–199 months), all evaluated patients were continent. Height and weight were appropriate for age in only 5 patients. Vitamin B12 and folic acid serum levels were normal in all examined patients. Ten patients had normal hemoglobin serum levels. Seven patients had low transferrin saturation in serum. Hemoccult tests were negative in all examined patients. Despite complex postoperative courses in some cases, patients and parents showed good overall satisfaction in terms of quality of life. The majority of patients reported a good quality of life. This can result from the adaptation of the patients to certain disease states. The failure to thrive seems to be related with the extent of aganglionosis. The inclusion of these patients in interdisciplinary long-term follow-up care, in which pediatric surgeons, gastroenterologists, and dieticians are involved, is essential.

We report a single-center study of minimally invasive thoracoscopic lung surgery with pediatric patients. We performed a retrospective analysis of patients who underwent thoracoscopic lung surgery between 2004 and 2013. The procedures were divided into anatomic and non-anatomical lung resections. Seventy-six patients with a mean age of 6.5 years (range: 7 days-17 years) and a mean weight of 11 kg (range: 2.6-56.0 kg) received thoracoscopic lung surgery for tumor metastases (n = 20), sequestration/congenital adenomatoid malformation (19), cysts (12), aspergillomas (7), bullae (5), middle lobe syndrome (3), bronchiectasis (3), emphysema (2), and other reasons (5). Twenty-nine anatomical lung resections (Group I: lobectomies, segmentectomies) and 47 non-anatomical lung resections (Group II: wedge resections, lung tissue-sparing surgery) were performed. In 6 cases, preoperative CT-guided coiling was used to localize the lung lesions. Specimen removal was achieved using a widened (2 cm) trocar site. The operating times of Group I patients were longer compared than those of Group II patients (means: 154 and 68 min, respectively); conversion rates (8 versus 2), chest tube insertion rates (100% versus 51%), and postoperative ventilation (48% versus 13%) also differed. Thoracoscopic anatomical lung resections appear to be safe and effective in infants and children. In congenital lung diseases, the key to success is the intraoperative destruction of space-occupying lesions. Limitations exist in cases with infectious adhesions. Non-anatomical lung resections are technically easier and should remain standard in pediatric surgery. Limitations exist in cases of metastases, which are deep within the parenchyma and are not visible on the lung surface.