Background Recently, we reported the feasibility of indocyanine green (ICG) near-infrared fluorescence (NIRF) imaging to identify extrahepatic biliary anatomy during laparoscopic cholecystectomy (LC) in pediatric patients. This paper aimed to describe the use of a new technology, RUBINA™, to perform intra-operative ICG fluorescent cholangiography (FC) in pediatric LC. Methods During the last year, ICG-FC was performed during LC using the new technology RUBINA™ in two pediatric surgery units. The ICG dosage was 0.35 mg/Kg and the median timing of administration was 15.6 h prior to surgery. Patient baseline, intra-operative details, rate of biliary anatomy identification, utilization ease, and surgical outcomes were assessed. Results Thirteen patients (11 girls), with median age at surgery of 12.9 years, underwent LC using the new RUBINA™ technology. Six patients (46.1%) had associated comorbidities and five (38.5%) were practicing drug therapy. Pre-operative workup included ultrasound (n = 13) and cholangio-MRI (n = 5), excluding biliary and/or vascular anatomical anomalies. One patient needed conversion to open surgery and was excluded from the study. The median operative time was 96.9 min (range 55–180). Technical failure of intra-operative ICG-NIRF visualization occurred in 2/12 patients (16.7%). In the other cases, ICG-NIRF allowed to identify biliary/vascular anatomic anomalies in 4/12 (33.3%), including Moynihan's hump of the right hepatic artery (n = 1), supravescicular bile duct (n = 1), and short cystic duct (n = 2). No allergic or adverse reactions to ICG, post-operative complications, or reoperations were reported. Conclusion Our preliminary experience suggested that the new RUBINA™ technology was very effective to perform ICG-FC during LC in pediatric patients. The advantages of this technology include the possibility to overlay the ICG-NIRF data onto the standard white light image and provide surgeons a constant fluorescence imaging of the target anatomy to assess position of critical biliary structures or presence of anatomical anomalies and safely perform the operation.

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Background Improved survival in ADA-SCID patients is revealing new aspects of the systemic disorder. Although increasing numbers of reports describe the systemic manifestations of adenosine deaminase deficiency, currently there are no studies in the literature evaluating genital development and pubertal progress in these patients.Methods We collected retrospective data on urogenital system and pubertal development of 86 ADA-SCID patients followed in the period 2000-2017 at the Great Ormond Street Hospital (UK) and 5 centers in Italy. In particular, we recorded clinical history and visits, and routine blood tests and ultrasound scans were performed as part of patients' follow-up. Results and Discussion We found a higher frequency of congenital and acquired undescended testes compared with healthy children (congenital, 22% in our sample, 0.5-4% described in healthy children; acquired, 16% in our sample, 1-3% in healthy children), mostly requiring orchidopexy. No urogenital abnormalities were noted in females. Spontaneous pubertal development occurred in the majority of female and male patients with a few cases of precocious or delayed puberty; no patient presented high FSH values. Neither ADA-SCID nor treatment performed (PEG-ADA, BMT, or GT) affected pubertal development or gonadic function. Conclusion In summary, this report describes a high prevalence of cryptorchidism in a cohort of male ADA-SCID patients which could represent an additional systemic manifestation of ADA-SCID. Considering the impact urogenital and pubertal abnormalities can have on patients' quality of life, we feel it is essential to include urogenital evaluation in ADA-SCID patients to detect any

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