Beyond image defined risk factors (IDRFs): a delphi survey highlighting definition of the surgical complexity index (SCI) in neuroblastoma.

PURPOSE: Preoperative evaluation of Image Defined Risk Factors (IDRFs) in neuroblastoma (NB) is crucial for determining suitability for upfront resection or tumor biopsy. IDRFs do not all carry the same weighting in predicting tumor complexity and surgical risk. In this study we aimed to assess and categorize a surgical complexity (Surgical Complexity Index, SCI) in NB resection. METHODS: A panel of 15 surgeons was involved in an electronic Delphi consensus survey to identify and score a set of shared items predictive and/or indicative of surgical complexity, including the number of preoperative IDRFs. A shared agreement included the achievement of at least 75% consensus focused on a single or two close risk categories. RESULTS: After 3 Delphi rounds, agreement was established on 25/27 items (92.6%). A severity score was established for each item ranging from 0 to 3 with an overall SCI range varying from a minimum score of zero to a maximum score of 29 points for any given patient. CONCLUSIONS: A consensus on a SCI to stratify the risks related to neuroblastoma tumor resection was established by the panel experts. This index will now be deployed to critically assign a better severity score to IDRFs involved in NB surgery.

Minimally invasive surgery for neuroblastic tumours: A SIOPEN multicentre study: Proposal for guidelines.

INTRODUCTION: Surgery plays a key role in the management of Neuroblastic tumours (NB), where the standard approach is open surgery, while minimally invasive surgery (MIS) may be considered an option in selected cases. The indication(s) and morbidity of MIS remain undetermined due to small number of reported studies. The aim of this study was to critically address the contemporary indications, morbidity and overall survival (OS) and propose guidelines exploring the utility of MIS for NB. MATERIALS & METHODS: A SIOPEN study where data of patients with NB who underwent MIS between 2005 and 2018, including demographics, tumour features, imaging, complications, follow up and survival, were extracted and then analysed. RESULTS: A total of 222 patients from 16 centres were identified. The majority were adrenal gland origin (54%) compared to abdominal non-adrenal and pelvic (16%) and thoracic (30%). Complete and near complete macroscopic resection (>95%) was achieved in 95%, with 10% of cases having conversion to open surgery. Complications were reported in 10% within 30 days of surgery. The presence of IDRF (30%) and/or tumour volume >75 ml were risk factors for conversion and complications in multivariate analysis. Overall mortality was 8.5%. CONCLUSIONS: MIS for NB showed that it is a secure approach allowing more than 95% resection. The presence of IDRFs was not an absolute contraindication for MIS. Conversion to open surgery and overall complication rates were low, however they become significant if tumour volume >75 mL. Based on these data, we propose new MIS guidelines for neuroblastic tumours.

Frozen-section examination in the management of paediatric testicular lesions.

PURPOSE: Paediatric testicular and para-testicular lesions have traditionally been managed according to adult protocols. Testis-sparing surgery (TSS) has gained popularity as it has become apparent benign lesions predominate in childhood. Frozen-section examination (FSE) for intra-operative diagnosis has been extensively utilised in adults, though its use in paediatric practice remains limited. We reviewed our experience of FSE in paediatric patients with an aim to identify the utility and efficacy of this tool in the management of testicular and para-testicular pathology. METHODS: A retrospective, single-centre review of paediatric patients who underwent intra-operative FSE for a range of testicular and para-testicular lesions was performed. FSE results were compared to final pathology. TSS was performed if appropriate, and was utilised in adolescent patients, and in lesions with a diameter greater than 20 mm. RESULTS: Nine males underwent FSE from 2013 to 2020. Median age at surgery was 9 years (range 1-15). Eight (89%) patients had benign pathology. FSE result correlated with the final pathological examination in 100% of cases. FSE facilitated TSS in 7/9 cases. CONCLUSION: FSE has 100% diagnostic accuracy for paediatric testicular and para-testicular pathology. We would recommend all lesions be evaluated by FSE to guide intra-operative decision making and facilitate TSS in appropriate cases.

The peripheral portacath provides safe and convenient venous access in pediatric and adolescent patients.

INTRODUCTION: Adolescent patients with chronic conditions rely on permanent venous access for safe treatment and supportive care. Traditionally this is provided by a central venous access device (CVAD) e.g. Hickmann catheter or totally implanted subcutaneous port or also called Port-a-Cath (PaC). We reviewed the patient experience, safety and feasibility of insertion of peripheral inserted implanted subcutaneous port (peripheral PaC). METHODS: Medical records of patients who underwent insertion of peripheral PaC under ultrasound guidance at our institution since between 2012-2017 were reviewed to ascertain specific details including duration of insertion and complication rate. Short structured questionnaires were used to assess nursing and patient satisfaction. RESULTS: Twenty eight peripheral PaC were inserted at our institution. There were 17 female and 11 male patients aged between 12.3 and 18.7 years (median = 16.1). Six were inserted under local anesthetic (LA) in patients who were not fit for general owing to mediastinal mass or lung disease. At the time of analysis 2 PaCs remained in situ with a median duration of 8 months (range 3-48). Removal of 26 PaCs was under LA in 15 cases and under GA in 11. Complications were observed in 9 cases but only necessitated early removal or replacement in 3 cases (displacement and disconnection) and repositioning in 1 case. Thrombosis was seen in 2 patients who required systemic anticoagulation but had complete resolution. CONCLUSION: This study shows that the use of peripheral PaC is safe. The feedback from patients and nursing staff supports the use of the peripheral PaC. We are exploring additional patient groups that might benefit from this device.

Solitary rectal ulcer syndrome in children. A report of three cases.

Solitary rectal ulcer syndrome (SRUS) is a rare condition in children. It is well recognised in the adult literature. We report our experience with 3 patients; two boys presented at a very young age (18 and 24 months, respectively) with a clinical picture simulating inflammatory bowel diseases. The other patient was a fifteen-year-old girl presenting with severe rectal stricture as a result of the syndrome, which is the first to be reported in the paediatric literature.

Gynaecomastia in the adolescent: a surgically relevant condition.

BACKGROUND/PURPOSE: Gynaecomastia is a benign condition which is commonly seen in adolescent males. The aim of this study is to assess the complications and the cosmetic outcome of surgical treatment in the management of adolescent gynaecomastia. METHODS: Thirty-nine adolescent males had subcutaneous mastectomy for gynaecomastia at Booth Hall and Royal Manchester Children's Hospitals between 1995 - 2001 inclusive. A retrospective analysis was performed and in addition to chart review, personal and telephone interviews were conducted to assess the patients' satisfaction with the cosmetic results of the procedure. RESULTS: Thirty-four patients (87%) had bilateral mastectomies and the remaining five (13%) had a unilateral procedure. The technique used was the standard subcutaneous mastectomy via a circumareolar skin incision (10). Early postoperative complications included haematoma (n = 3), seroma formation (n = 1), and wound infection (n = 1). Late complications included unequal breast sizes (n = 3) and redundant baggy appearance of the skin (n = 1). We contacted thirty-three patients to assess their satisfaction with the cosmetic results of the procedure. Twenty-nine (88%) were satisfied with their cosmetic appearance, compared to four (12%) reported "not satisfied". CONCLUSION: Gynaecomastia in adolescent males is common and the majority of cases regress spontaneously within one year. Surgery is indicated if the condition fails to regress or if it is causing sufficient embarrassment to interfere with the patient's social life. Based on our experience, we believe that subcutaneous mastectomy through a circumareolar skin incision is a valid procedure for the treatment for adolescent gynaecomastia and provides satisfactory cosmetic results for this age group.

Hyperammonemia with complex urinary tract anomaly: a case report.

Hyperammonemia has been reported rarely in the pediatric age group in systemically ill patients. All cases resulted from infections with urea splitting organisms, which are more common among patients who have undergone surgical procedures on the urinary tract. The authors report for the first time in the pediatric literature, one patient who presented with hyperammonemic encephalopathy that resulted from urinary tract infection with Staphylococcus epidermidis and Corynebacterium sp.