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.

Our results suggest that a chemotherapeutic regimen containing both AAP and CDDP with delayed NAC rescue has the potential to enhance chemotherapeutic efficacy while decreasing adverse effects. This would be a promising approach particularly for hepatoblastomas regardless of cellular CYP2E1 protein level but could also be beneficial in other malignancies.

IntroductionLaparoscopic appendectomy is a safe and feasible technique accepted by many surgeons as the gold standard approach for the treatment of acute appendicitis in children. Traditionally laparoscopic appendectomy requires the use of three ports. However, surgical techniques with fewer ports have been reported.AimTo evaluate the efficacy of laparoscopic appendectomy in children according to the proposed 3-step protocol using one, two or three ports.Material and methodsA total of 100 children with the diagnosis of acute appendicitis underwent laparoscopic appendectomy. Patients were treated according to the following protocol: transumbilical access with one 10 mm port using the laparoscope with working channel. The appendix was mobilized and delivered through the umbilical port and tied extracorporeally and removed. If the appendix was placed retrocecally or had adhesions, a second port was introduced. The appendix was mobilized and finally retrieved from the abdominal cavity through the camera port, and resected extracorporeally. In the cases of very short and gangrenous appendix and immobile colon, a third port was introduced and totally intra-abdominal appendectomy was performed. Patients were evaluated regarding the duration of the operation, and operative and postoperative complications.ResultsDuring the study period 100 children (58 males, 42 females) had laparoscopic appendectomy: 48 children by one-port technique (group I), 27 children by two-port technique (group II) and 25 children by three-port technique (group III). The mean operative time was 33 min (20-55 min) in group I, 39 min in group II (23-60 min), and 49 min (30-75 min) in group III. There were no intraoperative complications. Wound infections were recorded in 4 (8.3%) patients in group I, three (11.1%) in group II and four (16.0%) in group III. One patient in group III developed an abdominal abscess managed conservatively.ConclusionsOne-port laparoscopic appendectomy is a feasible technique in children. It allows 48% of children to have the operation. The addition of a second port allows one to mobilize the appendix and perform extracorporeal resection in an additional 27% of cases. These approaches have shorter operative time compared to 3-port technique. Laparoscopic extracorporeal appendectomy, especially one-port, is found to be cost effective and have excellent cosmetic results.

The laparoscopic upper pole HNU is a safe and feasible procedure, even in infants. It is associated with minimal morbidity, and the operative time is acceptable and not significantly longer in comparison with the open approach. LHNU reduces analgesic requirements and hospital stay. In our opinion it should be the preferred option for HNU in children.

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This article reports a case of a 7-year-old girl with Turner syndrome, treated with growth hormone (GH), who developed ovarian dysgerminoma. The patient karyotype was mosaic for chromosome Xq deletion: 46,X,del(X)(q22)/45,X. No Y chromosome sequences were present. Molecular studies revealed the presence of a driving mutation in exon 17 of the KIT gene in the neoplastic tissue, as well as Sonic-hedgehog (SHH) pathway activation at the protein level. The patient responded well to chemotherapy and remained in complete remission. This is the first case of dysgerminoma in a Turner syndrome patient with such oncogenic pathway.

Anorectal malformations (ARMs) occur in approximately 1 per 5000 live births. The most commonly used procedure for repair of high ARMs is posterior sagittal anorectoplasty (PSARP). This operation is performed entirely through a perineal approach. The first report of laparoscopically assisted anorectal pull-through (LAARP) for repair of ARMs was presented by Georgeson in 2000. The aim is presenting early experience with laparoscopically assisted anorectal pull-through technique in boys with high anorectal malformations. In the last 5 years 7 boys (9 months to 2 years old) with high ARMs were operated on using the LAARP technique. Laparoscopically the rectal pouch was exposed down to the urethral fistula, which was clipped and divided. Externally, the centre of the muscle complex was identified using an electrical stimulator. In the first 4 patients after a midline incision of 2 cm at the planned anoplasty site, a tunnel to the pelvis was created bluntly and dilated with Hegar probes under laparoscopic control. In the last 3 boys a minimal PSARP was done creating a channel into the pelvis. The separated rectum was pulled down and sutured to the perineum. Laparoscopic mobilization of the rectal pouch and fistula division was possible in all cases. There were no intraoperative complications except one ureteral injury. Patients were discharged home on post-operative day 5 to 7. The early results prove that LAARP, an alternative option to PSARP for treatment of imperforate anus, offers many advantages, including excellent visualization of the pelvic anatomical structures, accurate placement of the bowel into the muscle complex and a minimally invasive abdominal and perineal incision. It allows for shorter hospital stay and faster recovery. However, to compare the functional results against the standard procedure (PSARP), longer follow-up of all patients is necessary.