Extraperitoneal kidney transplantation: a comparison between children weighting ≤15 kg and >15 kg. Experience of a single institution.

Extraperitoneal approach is sometimes recommended for kidney transplantation (KT) in children weighting <15 kg. We hypothesized that this approach might be as successful as in patients with normal weight. Data of all consecutive KTs performed between 2013 and 2019 were retrospectively reviewed. Early outcomes and surgical complications were compared between children weighing ≤15 kg (low-weight (LW) group) and those weighing >15 kg (Normal-weight (NW) group). All the 108 KTs were performed through an extraperitoneal approach. The LW group included 31 patients (mean age 3.5 ± 1.4 years), whose mean weight was 11.1 ± 2.0 kg. In the LW group,-a primary graft nonfunction (PNGF) occurred in one patient (3.2%), surgical complications occurred in nine (29%), with four venous thrombosis. In the NW group, PNGF occurred in one case (1.3%), delayed graft function (DGF) in eight (10%), surgical complications in 11 (14%) with only one case of venous thrombosis. In both groups, no need for patch during wound closure and no wound dehiscence were reported. The extraperitoneal approach can be effectively used in LW children. No differences were observed in the overall complication rate (P = 0.10), except for the occurrence of venous thrombosis (P = 0.02). This might be related to patients' characteristics of the LW group.

Outcome and prognostic factors in high-risk childhood adrenocortical carcinomas: A report from the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT).

OBJECTIVES: The aim of this retrospective international analysis was to evaluate the role of risk factors in pediatric patients with adrenocortical carcinoma (ACC) observed in European countries (2000-2013) in an attempt to identify factors associated with poor prognosis. PROCEDURES: Data were retrieved from databases of Germany, France, Poland, and Italy, which form the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT). Patients were less than 18 years old, with at least one of the following tumor-related risk factors: metastases, volume more than 200 cm3 , Cushing syndrome, vascular or regional lymph node invasion, initial biopsy, or incomplete excision. Role of patients' age was also evaluated. RESULTS: Eighty-two patients were evaluated: 62 with localized disease and 20 with metastases. The 3-year progression-free survival (PFS) and overall survival (OS) were 39% and 55% for the whole population, respectively, and 51% and 73% for localized diseases, respectively. Concerning the whole population, PFS and OS were influenced by distant metastases, tumor volume, lymph node involvement, age, and presence of two or more risk factors. Factors significant only at OS were vascular involvement and incomplete surgery. At multivariable analysis, the main factors at PFS were volume more than 200 cm3 (hazard ratio [HR]: 2.6, 95% confidence interval [CI]: 1.18-5.70) and presence of distant metastases (HR: 8.26, 95% CI: 3.49-19.51). The OS was significantly influenced by the presence of metastases (P < 0.0001). Concerning patients with localized tumors, the only significant prognostic factor was volume more than 200 cm3 with a HR of 4.38 (95% CI: 1.60-12.00) for PFS and of 3.68 (95% CI: 1.02-13.30) for OS. CONCLUSIONS: Distant metastases and large tumor volume were the main unfavorable prognostic factors. Presence of two or more factors related to ACC was associated with an aggressive behavior of disease.

Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey.

BACKGROUND: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF. METHODS: Following approval of the Italian Society of Pediatric Surgery, a survey was sent to all Pediatric Surgery Units to retrospectively collect H-TEF treated in the period 2010-2022. Descriptive analysis was performed, and results are given as prevalence, mean ± standard deviation (SD), or median and interquartile range (IQR). RESULTS: The survey was sent to 65 units. Seventeen responded with one or more cases; 78 patients were diagnosed with H-TEF during the study period. Associated malformations were present in 43%, mostly cardiac (31%). The most frequent symptoms were cough (36%), bronchopneumonia (24%), and dysphagia (19%). H-TEF was detected by tracheobronchoscopy (90%), and/or upper GI (58%), and/or esophagoscopy (32%). The median age at diagnosis was 23 days (1 day-18 years). The most common approach was cervicotomy (76%), followed by thoracoscopy (14%) and thoracotomy (9%). The fistula underwent ligation and section of the fistula in 90% of the patients and clip closure and section in 9%. In one patient, the fistula was cauterized endoscopically. H-TEF preoperative cannulation was performed in 68% of cases, and a drain was placed in 26%. One month after surgery, 13% of the patients had mild persisting symptoms, mainly hypophonia. Recurrence occurred in 5%, and a second recurrence occurred in 1%. CONCLUSIONS: H-TEF prevalence was six cases/year, consistent with the expected rate of five cases/year in our country. The diagnosis was challenging, sometimes delayed, and, in most patients, required multiple examinations. Fistula ligation and section through cervicotomy were the most frequent treatment. Long-term outcomes are good, and recurrence is a rare event.

Surgical Approach of Wandering Spleen in Infants and Children: A Systematic Review.

Introduction: Splenopexy has been proposed as the treatment of choice in case of wandering spleen (WS). We report our experience and review the current literature focusing on surgical management and outcomes of children affected by WS. Materials and Methods: Data regarding demographics, clinical manifestations, diagnosis, and treatment of children treated for WS at our Institution were analyzed. Systematic review was registered on Prospero (CRD42018089971). Scientific databases were searched using defined keywords. Articles were selected using predefined exclusion and inclusion criteria. Analysis was conducted adding our center's cases. Results: One hundred sixty-six articles were included in the review, 197 cases were analyzed, 3 of which unpublished. Female/male ratio was 1.5:1 and median age at diagnosis was 8 years. Most frequent clinical manifestation was isolated abdominal pain (42.6%). Torsion of splenic pedicle was diagnosed in 56.3%. Among surgical procedures, 39% underwent splenopexy and 54.8% underwent splenectomy. In case of splenopexy, the most commonly used techniques were using of a mesh (45.5%) or creation of a retroperitoneal pouch (30.9%). In 48.2% of splenopexies, minimally invasive surgery (MIS) was used. Splenopexy was effective in 94.8% (88% considering only cases with a spleen torsion). Conclusion: WS is a rare condition potentially leading to torsion of the spleen. This entity has to be kept in mind as a differential diagnosis in case of abdominal pain. Splenopexy should be the treatment of choice; its success rate in terms of preserved spleens can be affected by the presence of a torted organ. Retroperitoneal pouch or mesh fixation are the most preferred techniques. Authors recommend MIS approach.

Thoracoscopy versus thoracotomy for congenital lung malformations treatment: A single center experience.

INTRODUCTION: Our aim is to compare thoracoscopy to thoracotomy in the treatment of congenital lung malformations (CLM) in children. MATERIALS AND METHODS: We report a retrospective monocentric cohort study. Patients treated at our Center for CLM (1991-2020) were divided in two groups: patients treated with video-assisted thoracoscopic surgery (VATS) and open thoracotomy (OT). Characteristics of the two groups were compared through statistical analysis (GraphPad Prism7). A p value less than .05 was considered statistically significant. RESULTS: One hundred six patients were included: 58 in VATS group, 48 in OT group. Prenatal diagnosis was possible in 73.6%. The most frequent surgical procedures were lobectomy (43.4%) and sequestrectomy (22.6%). All VATS patients underwent lung exclusion, mostly by endobronchial blocker (69%). Mean operative time was 146.1 min (±52.04 SD) in VATS and 159.2 (±46.53 SD) in OT (p = .1973). Conversion to OT was necessary in 20.6% of VATS patients, but decreased in the last 5 years (6.2%). There were not any intraoperative complication. Respectively in VATS and OT group, length of stay (LOS) was 4.5 days ± 3.6 SD versus 7.7 ± 3.4 SD (p < .0001), chest tube duration 2.8 days ± 3.4 SD versus 3.7 ± 2.4 SD (p < .0001), antibiotic treatment duration 3.7 days ± 4.7 SD versus 5 ± 2.6 SD (p = .1196). Postoperative complications were described in 22.6%. The commonest histological diagnosis (40.6%) was congenital pulmonary airway malformation. CONCLUSION: VATS resulted a feasible, effective and safe technique. Operative time and postoperative complications were similar in VATS and OT groups. VATS conversion rate decreased in time. VATS had a statistically significant shorter LOS and chest tube duration.

Exstrophy-Epispadias Complex Variants: A Hybrid Case.

The term exstrophy-epispadias complex refers to a group of midline defects ranging from epispadias to cloacal exstrophy. Bladder exstrophy is the most frequent malformation of this spectrum and it can present as a classical or a variant form. We report a case of a hybrid bladder exstrophy variant having some characteristics of both a duplicate bladder exstrophy and a superior vesical fistula.