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The European Union of Medical Specialists (UEMS) Section and Board of Pediatric surgery was founded more than 40 years ago. Since then major activities have been related to the improvement of quality of care of pediatric surgery in Europe. Remarkable success was achieved in the development of pediatric surgery as an independent specialty all over Europe. Other major successful activities of the UEMS Section and Board of Pediatric Surgery consisted of the development of a high-quality European examination and delineating a minimal common standard in pediatric surgery training in the form of European training requirements. Recommendations drawn from examination experience support that candidates who achieve weaker passes in part 1 may wish to consider more practice before attempting part 2 due to the weak correlation between the two scores. It may be helpful for candidates to consider having some experience working in an English-speaking clinical setting, if not truly fluent in English, to improve their chances of being successful in the part 2 examination. Other achievements of the Section were accreditation of the training centers in Europe and European Census in pediatric surgery project. All the aforementioned activities led to standardization and harmonization of pediatric surgery, as well as contributed to increasing quality of pediatric surgical service throughout Europe., Competing Interests: None declared., (Thieme. All rights reserved.)

Background:  Detailed data on the distribution of pediatric surgical institutions in Europe are sparse. Therefore, the Section and Board of Pediatric Surgery of the Union of European Medical Specialists (UEMS) and the European Pediatric Surgeons' Association (EUPSA) jointly organized the first official census of the European centers of pediatric surgery (PS)., Materials and Methods:  After obtaining a list of pediatric surgical centers in Europe, a specialized questionnaire was created and made available on the Internet. General, workload, staff, and ancillary data were collected for the centers., Results:  Total 215 out of 431 centers answered. PS center density is 1 in every 177,000 children or 1 in every 1,142,000 inhabitants. Approximately 77% of the centers (167 out of 215) are training centers in PS. Every center has an average inpatients procedures/year equal to 1,588, and 92.6 neonatal procedures/year. There are 3.9 surgeons/100,000 children, and 1,662 neonates for every surgeon. In average every EU surgeon performs 202 procedures/year, of which 11 procedures are on neonates. Trainees represent the 38% of the total workforce, and there are 2.3 trainees for every staff aged > 60 years., Conclusion:  The paper aims to offer a more grounded basis on which the future of PS in our Continent should be planned. Presented data will provide an invaluable help to all our colleagues, as well as national policy makers, to press for and to make better informed and well-grounded sound political choices in the field of PS., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Competing Interests: Conflict of Interest: None.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

The treatment outcomes in children with bladder/prostate rhabdomyosarcoma (B/P RMS) have considerably improved in the past few decades. Current protocols incorporate chemotherapy, radiotherapy, and surgery (radical or organ sparing). Using this multimodal and multidisciplinary approach, the cure rates for nonmetastatic RMS have gradually increased from 25% in the 1970s, and 70% in the 1990s, to over 80% in the 2000s. Surgery plays a role not only in the diagnosis and management of all stages of B/P RMS, but also in the urinary reconstruction and long-term follow-up. Furthermore, the tendency to avoid mutilating surgery and to preserve functional genitourinary tract has been noticeable. Authors describe the current status and future surgical treatment approaches and possibilities for patients with B/P RMS and how reconstructive urologic surgery in B/P RMS continues to evolve., (Georg Thieme Verlag KG Stuttgart · New York.)

Introduction: We present the experiences from two European centers performing the Foker technique (FT) of esophageal lengthening by axial traction and the Kimura advancement (KA) method of lengthening the upper pouch by extrathoracic resiting a spit fistula (SF) in children with long-gap esophageal atresia (LGEA, gap length > 5 cm)., Materials and Methods: A total of 15 children were treated (8 pure EA, 6 lower tracheoesophageal fistula [TEF], and 1 upper TEF). Gaps ranged from 5 to 14 cm. Nine children already had a SF. Patients were grouped according to the presence of a SF and the subsequent surgical strategy: Group A (no SF, n = 6) received FT on both pouches. Group B (with SF, n = 6) received KA of SF and FT of the lower pouch. Group C (with SF, n = 3) received closure of the SF and subsequent Foker traction (CSFT) on both pouches., Results: Group A: Primary repairs for all six children (mean age 3 months, gap length 6.5 cm) after a mean traction time of 3 weeks and a mean of 2.1 thoracotomies (range 2 to 3). Dilations were required in three out of six for anastomotic strictures with one perforation during the second dilation. Group B: All six children (mean age 16.4 months, gap length 9.5 cm) had a primary anastomosis, although for two it was significantly delayed (48 and 143 weeks traction time) because of infections. The number of thoracotomies ranged from 2 to 8 (mean 3.6). Leaks occurred in five out of six anastomoses (responsive to conservative management). Two children developed severe strictures, which required the anastomosis to be redone. In group C (mean age 10.6 months, gap length 6.5 cm), several major complications occurred. The three SF closures leaked (one iatrogenic) causing severe mediastinitis. CSFT was successful in only one case and the other two children had an esophageal replacement (stomach, jejunum). No deaths occurred in the series., Conclusion: FT of both pouches (group A) resulted in primary repairs of all six LGEA patients. The combination of KA and FT (group B) resulted in an equivalent rate of primary repairs, but with an increased number of thoracotomies and rate of complications compared with group A. CSFT (group C) resulted in a high failure rate. More data are needed (we propose a multicenter registry) to elucidate the safety and efficacy of each elongation technique and to establish an algorithm with clearer inclusion and exclusion criteria., (Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: Nonparasitic splenic cysts (NPSCs) are uncommon in children. The aim of this multinational and multicentric study was to present the authors' experience as well as the changing trends in the management of NPSCs over the last 25 years., Material and Methods: From 1981 to 2005, 50 children or adolescents were surgically treated for NPSCs in 6 paediatric surgical centres in four European countries. The medical records of these 50 patients with NPSCs were reviewed retrospectively., Results: Twenty-six male and 24 female patients were operated on. Age at surgery ranged from 1 to 17 years (mean 11.9). Seventeen patients were symptomatic. Six total (4 open and 2 laparoscopic) and 26 partial (22 open and 4 laparoscopic) splenectomies were performed. Laparoscopic fenestration or deroofing and open cystectomy was carried out in 9 patients, respectively. Histological findings revealed the lesion to be an epidermoid cyst (n = 28), a pseudocyst (n = 15) or a mesothelial cyst (n = 2). In 5 patients haemangioma or lymphangioma was the pathological diagnosis. At a mean follow-up of 2.9 years, residual cysts were found in 8 laparoscopically treated patients, 4 of whom required re-do laparoscopy or open surgery., Conclusions: Over the last two decades, the surgical treatment of NPSCs has changed from a formerly customary total splenectomy to spleen-conserving procedures, such as total cystectomy with or without partial splenectomy or partial cystectomy. These therapeutic modalities can be performed laparoscopically, if technically possible. Fenestration or deroofing of the cyst resulted in a high recurrence rate (7/9).

Forty-seven children treated in various Polish centers between 1985 and 1995 for primary malignant liver tumors were retrospectively analyzed. Hepatoblastoma (HB) prevailed--it was found in 39 cases. There were 6 hepatocarcinoma (HCC) cases and 2 cases of undifferentiated sarcoma (UDS). In 44% of HB patients the tumor involved both liver lobes. 18% of children with HB presented with pulmonary metastases at diagnosis. Chemotherapy was applied in 92% of cases (preoperatively in 67%). Tumor resection was performed in 56% of HB patients. Overall survival of patients with hepatoblastoma was 43.6%, while it was 50% for hepatocarcinoma and 100% for undifferentiated sarcoma (2 cases only). Mean observation time was 58 months. The hepatoblastoma subgroup, being the largest (83% of all cases), was analyzed separately for prognostic factors. Completeness of tumor excision strongly influenced survival. Involvement of both lobes of the liver and multifocality of the tumor were other adverse prognostic factors.

Unlabelled: In 4 years (1993-1996) 206 pts. with nephroblastoma were treated. All children were treated according to SIOP 93-01 protocol. Overall survival was 92%. In 27 cases hepatotoxic events occurred. In 10 cases, venoocclusive liver disease (VOD) was diagnosed. VOD is a syndrome associated with hepatomegaly, sudden weight gain or ascites and jaundice. It results from damage to the endothelium of hepatic venules and necrosis of central hepatocytes with subsequent proliferation of fibrous tissue and occlusion of the central hepatic veins. Dactinomycin is one of the drugs considered responsible for its development. Mean age of VOD patients was 4 yrs, however 3 of them were below 1 yr. In all cases, VOD occurred during postoperative chemotherapy (mean 16 th week of treatment). All patients received dactinomycin and vincristine. Five children with right kidney tumors underwent post-operative abdominal irradiation. Main VOD symptoms were hepatomegaly and ascites (80%). Hypertransaminasaemia, as well as, on ultrasound, gallbladder wall thickening and/or free abdominal fluid were observed. Median VOD duration was 27 days and its course was usually temporary and self-limiting. However, in 2 cases recurrent VOD episodes were noted. All children received supportive treatment only. In 6 cases, VOD resulted in chemotherapy delay or drug reductions, while in 4 others chemotherapy was completed preliminarily. Nevertheless it did not affect patients' outcome overall survival in VOD group was 90%., Conclusions: Total 5% VOD frequency is similar to other reports. Infants and children receiving abdominal irradiation seem to be at special risk of VOD development.

A case of hepatic inflammatory pseudotumor mimicking malignancy in a 4-year-old girl with the Papillon-Lefevre syndrome (PLS) is reported. Only recently, an association between this inherited syndrome and liver abscesses has been found. Its possible pathogenesis is discussed and immunologic defects resulting from the Papillon-Lefevre syndrome are presented. The development of inflammatory pseudotumor of the liver might be caused by immunologic disturbances and staphylococcal infection. The picture of the hepatic tumor on imaging in patients with PLS should be attributed rather to inflammatory than neoplastic process.

Between 1965 and 1990 five children with pheochromocytomas were treated. Clinical course, methods of diagnosis, treatment and results established by follow-up from 6 months to 20 years are presented. Hypertension and headaches occurred in all patients. One child with a malignant form of pheochromocytoma manifested central nervous symptoms imitating epilepsy and additionally diffuse pulmonary metastases were found. One patient had a family history of pheochromocytomas and presented with hypermetabolic state and neurovegetative symptoms. One child had multifocal tumors: two in the posterior mediastinum and one in the retroperitoneal space. All patients were treated by either enucleation of the tumor, tumor excision or adrenalectomy. One child died 12 years after surgery from the rapid progression of pulmonary metastases of pheochromoblastoma present at the time of diagnosis.