Introduction: Sacrococcygeal teratoma (SCT) is a rare congenital tumour. The risk of malignancy and recurrence is not well defined. Previous studies are small and report differing conclusions about the timing of surgery and the duration of follow-up. The authors studied the risk of malignant transformation and SCT recurrence after surgery to address these gaps. Methods: This was a global retrospective cohort study. Data of consecutive SCT patients was obtained from 145 institutes in 62 countries. Malignant transformation, defined as malignancy at initial resection, malignant recurrence or death due to malignancy, and its risk factors were analysed. Results: Of the 3612 included patients, 3407 entered analysis. The risk of malignant transformation of the initial tumour was 3.3, 5.1, 10.1, and 32.9% at age 3 months, 6 months, 1 year, and 2 years, respectively. After 6 years, the censored risk of malignancy (64%) did not further increase. Recurrent SCT was diagnosed in 349 (10.2%) children with 126 (36.1%) malignant recurrences. Risk factors for recurrence were Altman type II [odds ratio (OR): 1.6, 95% confidence interval (CI): 1.2–2.2], Altman type III (OR: 1.6, 95% CI: 1.2–2.3), initial immature histology (OR: 1.9, 95% CI: 1.4–2.6), and initial malignant histology (OR: 4.0, 95% CI: 2.9–5.4). Conclusion: The risk of malignancy at initial resection in SCT increases with age reaching a plateau at 6 years of age. Recurrence after resection occurred in 10% of patients and 36% of these were malignant at that time. Altman type II or type III, and immature or malignant histology were associated with recurrence. Level of evidence: Level III.

Aim :Evaluate postoperative urinary continence in patients withcongenital adrenal hyperplasia(CAH)with intermediate (IT)and high urogenital sinus (UGS) who underwent a UGS mobilization maneuver . Methods: We called IT to those that although needing an aggressive dissection to get to the vagina, still have enough urethra proximal to the vaginal confluence. Very low variants are excluded from this analysis. Dissection always started in the posterior wall of the UGS with an aggressive separation from the anterior rectal wall. If the wide portion of the vagina was reached dissection stopped and the UGS opened ventrally widening to the introitus. Nineteen patients were treated using this maneuver (Group1). When more dissection was required the anterior wall of the UGS was dissected and carefully freed from the low retropubic space. Then the UGS was opened either ventrally or dorsally. Thirty three patients required this approach (Group 2) .Combined procedures were used in 3 patients with high UGS (Group 3). Results : Mean age at the time of the repair and length of the UGS were 12.2 years (4 months to 18 years) and 3.75 cm (3 -8 cm)for G 1; 8 years (5 months to 17 years) and 6.34 cm ( 4-12 cm) in G2 and 8,3 years (2 -14 y) and 11.5cm (11-12cm) in G3. All patients had been regularly followed. Mean age at last follow up of 14.3y, 17y and 9.9y for groups 1, 2 and 3 respectively. All patients continue to void normally and are continent. All patients have 2 separate visible orifices in the vulva. Only 3 are sexually active. Conclusion: UGS mobilization for vaginoplasty in girls with CAH does not compromise voiding function or urinary continence,

Nineteen newborns with prenatal diagnosis of ovarian cysts were referred to our institution during a 6-year period. Only those cysts more than 5-cm in diameter were included. Nine cases presented simple cysts and 7 of them underwent needle aspiration, 5 under ultrasound guidance and 2 with laparoscopic assistance. In one case an open surgical cystectomy was performed and the other had spontaneous resolution. No oophorectomies were performed in this group. Ten patients had complicated ultrasound pattern (septa, fluid/debris, calcification). From this group, 8 patients had ovarian torsion and 6 underwent laparoscopic oophorectomy (one bilateral). Open surgical oophorectomy was performed in 2 patients early in the series. Two cases were misdiagnosed. In one case a simple cyst appeared as complicated because of associated meconium peritonitis with calcifications. The other case underwent an initial laparoscopy that unveiled an intestinal duplication that was excised under open surgery. Mean operative time for laparoscopic approach was of 40 minutes and all patients left the hospital within 24 hours. We conclude that it is possible to preserve the neonatal ovary in noncomplicated cysts. Both laparoscopic and ultrasound-guided needle aspiration are safe and minimally invasive procedures for simple cyst-definitive treatment. Initial laparoscopic approach is the procedure of choice for complicated cysts thus providing diagnostic and treatment capabilities.

Introduction: The use of minimal invasive surgery (MIS) simulation models has been included into our residency program curricula and hands-on courses since 2013. We have previously presented our low-cost tracheoesophageal fistula esophageal atresia simulation model for training in IPEG 2013. The Society of American Gastrointestinal and Endoscopic Surgeons describes telementoring as a relationship, facilitated by telecommunication technology, in which an expert provides guidance to a less experienced learner from a remote location. MIS procedures are an ideal platform for real-time transmission and thus apply telementoring for surgery. We started a project to look at the efficacy of telementoring starting with simulation. Aim: The aim of this 5 minute video is to present a low-cost simulation model for training MIS repair of duodenal atresia (DA) and our first steps with combining telementoring technology. Material and Methods: The base model was introduced into the abdominal cavity of a rubber doll that resembles a 3 kg newborn. We use silicone finger covers and tubular latex balloons (as proximal and distal duodenum and bowel). The cost of materials was less than $50US. Video endoscopic equipment and 3 mm instruments were used. We reproduced all steps of the procedure: dissection of the proximal pouch, finding the distal duodenum below the transverse mesocolon, opening of both blind duodenal ends and performance of a duodenoduodenal anastomosis. We use a validation sheet to evaluate the performance: number of errors and type of injuries, quality of the anastomosis, and time of practice. A video camera was connected with a videoconference equipment through Internet under 1 MB for telementoring. Two pediatric surgery fellows were mentored by three faculties with focus in both the use of the lens and performance of the correct steps to repair a DA. Both of them had previously attended a basic and advanced MIS hands-on programs in our institution and developed skills for advanced suturing and working in small spaces. Mentors and mentees had a human and academic previous relationship. The development checklist sheet was filled after consensus. Results: The procedure was completed without injuries in 75 minutes. Interaction was effective without need to assist them in person. Discussion: Telementoring MIS simulation could assist in the provision of teaching advanced skills worldwide. No competing financial interests exist. Runtime of video: 5 mins This video was presented at 25th IPEG annual meeting, May 24–28 2016, Fukuoka Japan.

Background: Persistent müllerian duct syndrome (PMDS) is characterized by the persistence of müllerian duct derivatives in otherwise normally virilized 46,XY males. Biallelic mutations of the anti-müllerian hormone (AMH) and AMH receptor type 2 (AMHR2) genes lead to PMDS type 1 and 2, respectively. Aim: The aims of the study were to report the clinical, hormonal, and genetic findings in a patient with PMDS and discuss surgical strategies to achieve successful orchidopexy. Results: A 4-year-old boy was evaluated after the incidental finding of müllerian derivates during laparoscopy for nonpalpable gonads. Karyotype was 46,XY and laboratory tests revealed normal serum gonadotropin and androgen levels but undetectable serum AMH levels. PMDS was suspected. Molecular analysis revealed a novel variant c.902_929del in exon 5 and a previously reported mutation (c.367C>T) in exon 1 of the AMH gene. Successful orchidopexy was performed in two sequential surgeries in which the müllerian duct structure was preserved and divided to protect the vascular supply to the gonads. Histological evaluation of the testicular biopsy showed mild signs of dysgenesis. Doppler ultrasound showed blood flow in both testes positioned in the scrotum 1.5 years after surgery. Conclusion: PMDS is a rare entity that requires a high index of suspicion (from surgeons) when evaluating a patient with bilateral cryptorchidism. Surgical treatment is challenging and long-term follow-up is essential. Histological evaluation of the testis deserves further investigation.