Prevalence, management and efficacy of treatment in portal vein obstruction after paediatric liver transplantation: protocol of the retrospective international multicentre PORTAL registry.

INTRODUCTION: Portal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management practices and efficacy of treatment in patients with PVO. METHODS AND ANALYSIS: The Portal vein Obstruction Revascularisation Therapy After Liver transplantation registry will facilitate an international, retrospective, multicentre, observational study, with 25 centres around the world already actively involved. Paediatric patients (aged <18 years) with a diagnosed PVO between 1 January 2001 and 1 January 2021 after liver transplantation will be eligible for inclusion. The primary endpoints are the prevalence of PVO, primary and secondary patency after PVO intervention and current management practices. Secondary endpoints are patient and graft survival, severe complications of PVO and technical success of revascularisation techniques. ETHICS AND DISSEMINATION: Medical Ethics Review Board of the University Medical Center Groningen has approved the study (METc 2021/072). The results of this study will be disseminated via peer-reviewed publications and scientific presentations at national and international conferences. TRIAL REGISTRATION NUMBER: Netherlands Trial Register (NL9261).

[Retroperitoneal lymph node assessment in pediatric paratesticular rhabdomyosarcoma]. / La place du curage rétropéritonéal dans les Rhabdomyosarcomes para-testiculaires du jeune.

Paratesticular Rhabdomyosarcoma accounts for 7 to 11% of pediatric rhabdomyosarcomas. Children older than 10 years have a worse event-free survival (69 to 80% vs. 87 to 92%) than children younger than 10 years. In this location, the relapses are essentially in the retroperitoneal lymph nodes and are often fatal. In European protocols, the assessment of the retroperitoneal lymph nodes at diagnosis is made by imaging whereas it is performed by retroperitoneal lymph node dissection in the American protocols. This method has been proved to improve event-free survival in the group of patient older than 10 years with a tumour bigger than 5cm. In the next European protocol, when nodes will be enlarged a surgical biopsy will be performed, this will be irrespective of age or size, and when no nodes will be enlarged in patients older than 10 years, retroperitoneal lymph node assessment will be performed. Other techniques of lymph nodes assessment will be tested like sentinel node biopsies or PET-scan.

A new surgical approach of temporary ovarian transposition for children undergoing brachytherapy: technical assessment and dose evaluation.

PURPOSE: We developed a new technique of temporary ovarian transposition (OT) for prepubertal girls undergoing brachytherapy. The aim of this study was to describe it, assess its feasibility and safety and calculate the dose delivered to the ovary in order to prove its efficacy. METHODS: Sixteen prepubertal patients underwent temporary OT for brachytherapy at our center from March 2001 to December 2012. OT was done either by laparotomy or by laparoscopy. In all patients, the ovaries were grasped with an atraumatic forceps and mobilized above the iliac crest level as high as possible without any dissection or division of the ovarian ligaments or of the fallopian tube. They were sutured to the anterior abdominal wall by a transfixing stitch of non-dissolvable suture knotted on the outside of the patient on a pledget. RESULTS: Median age at surgery was 3 years (range: 2-9 years). The integrity of the fallopian tube was respected and not a single ligament was dissected or divided. None of the patients had intraoperative or postoperative complications. The stitches were retrieved after completion of irradiation and the ovaries in all the patients fell back into the pelvis. The calculated median radiation dose to the ovary was 1.4 Gy (range: 0.4-2.4 Gy). CONCLUSIONS: This surgical technique is simple and safe, either by laparotomy or by laparoscopy. It meets the radiation and physical constraints in prepubertal girls with vaginal or bladder RMS. However, longer follow-up is required to assess the ovarian function.