Predicting clinically significant events in children with ureteropelvic junction obstruction.

Introduction: Ureteropelvic junction obstruction (UPJO) syndrome is one of the most common causes of neonatal hydronephrosis. Management varies from simple monitoring to surgical intervention, with indications differing between institutions. A consensus of 8 societies recently described a new Urinary Tract Dilation (UTD) classification which aims to standardize ultrasound description of hydronephrosis, but which is also supposed to have predictive value in children with hydronephrosis. Our aim was to compare, in a monocentric prospective cohort of children with UPJO, the ability of UTD to predict the occurrence of a clinically significant event within the first year of life, as compared to anteroposterior diameter of the renal pelvis (APD). Study design: We used a preexisting cohort of children followed in a prospective study on UPJO. A pediatric radiologist, blinded to the children's outcome, classified the last antenatal ultrasound and postnatal ultrasound according to the UTD-A and UTD-P classification. He also confirmed the APD-A and APD-P measures. We defined a clinically significant event as being: increased pelvic dilation (>5 mm) and/or the presence of a febrile urinary tract infection (fUTI) and/or impaired renal function on initial nuclear scan (<40%). We performed a ROC-AUC curve and Random Forest (RF) analysis to compare the ability of the APD-A, APD-P, UTD-A and UTD-P scores to predict a clinically significant event. Results: The cohort included 28 children. Clinically significant events were noted in 20 out of 28 patients: 13 children presented an increase >5 mm in dilation, 6 presented an episode of fUTI and 9 had impaired function of the affected kidney. APD-A was the most effective individual criterion for predicting the occurrence of a significant clinical event (AUC = 0.867). Conclusion: In our series, for children with UPJO, the most significant marker was prenatal APD >15 mm to predict an increase in dilation >5 mm.

Indocyanine green (ICG) fluorescent cholangiography during laparoscopic cholecystectomy using RUBINA™ technology: preliminary experience in two pediatric surgery centers.

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.

Management of Large Pediatric and Adolescent Ovarian Neoplasms with a Leak-Proof Extracorporeal Drainage Technique: Our Experience Using a Hybrid Minimally Invasive Approach.

STUDY OBJECTIVE: In a pediatric setting, laparoscopic management of large cystic ovarian neoplasms with low malignancy probability is not suitable, because of the mass size; nevertheless, an effort should be made to be as minimally invasive as possible, without violating the principles of oncologic surgery. We describe our experience in managing these neoplasms with leak-proof extracorporeal drainage through mini laparotomy, followed by cyst excision or oophorectomy. DESIGN: Case series study, describing interventions and outcomes. SETTING: Department of pediatric surgery in a tertiary pediatric and adult university hospital. PARTICIPANTS: Pediatric patients affected by large cystic ovarian mass. INTERVENTIONS: Hybrid minimally invasive approach using leak-proof extracorporeal drainage. MAIN OUTCOME MEASURES: Data on demographic characteristics, tumor marker values, and imaging findings were collected and analyzed. Outcome of surgical technique was evaluated and reported. RESULTS: Between 2011 and 2018, 17 patients (mean age, 10.2 years; range, 2-14 years) affected by large cystic ovarian mass, were eligible for this technique. All patients had negative preoperative tumor markers. Of the seventeen subjects, 13/17 girls (76%) underwent pelvic magnetic resonance imaging. No sign of lymphadenopathy or metastasis was found. Surgery was successful in all patients, with ovarian preservation in 5/17 cases (29.4%). Mean surgical time was 98 minutes; no intra-abdominal leakage of neoplasm content or postoperative complications occurred. Mature cystic teratoma was the most frequent histopathological diagnosis (71%). CONCLUSION: After a thorough patient selection, the management of large cystic ovarian neoplasms with leak-proof extracorporeal drainage performed through a mini laparotomy is a feasible and safe approach, with excellent cosmetic results. When achievable, ovarian-sparing surgery has to be considered.

Urogenital Abnormalities in Adenosine Deaminase Deficiency.

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 abnormalities, initiate early treatment, and prevent long-term complications.

Correction to: Acute epididymo-orchitis due to Salmonella Typhi in a young man from Bangladesh.

The original version of this article unfortunately contained a mistake. Arnaldo Caruso was not listed among the authors.

Acute epididymo-orchitis due to Salmonella Typhi in a young man from Bangladesh.

S. typhi infection rarely involves the genitourinary system. We report the first described case of acute epididymo-orchitis due to S. typhi in a 14-year-old boy from Bangladesh. A high index of suspicion should be maintained when evaluating patients coming from endemic countries also in case of unusual sites of infection.