The Australian New Zealand Consortium in Children, Adolescents, and Young Adults Oncofertility action plan.

International and national oncofertility networks, including the US-led Oncofertility Consortium, FertiProtekt, and the Danish Network, have played pivotal roles in advancing the discipline of oncofertility over the last decade. Many other countries lack a shared approach to pediatric oncofertility health service delivery. This study aims to describe baseline oncofertility practices at Australian New Zealand Children's Haematology/Oncology Group centers in 2019-2021, describe binational priorities for care, and propose a 5-year action plan for best practice to be implemented by the newly formed Australian New Zealand Consortium in Children, Adolescents, and Young Adults (CAYA) Oncofertility (ANZCO).

Mediastinal Nontuberculous Mycobacterial Infection in Children: A Multidisciplinary Approach.

BACKGROUND: Mediastinal infections due to nontuberculous mycobacteria remain an exceedingly rare entity. Most cases in the published literature do not include pediatric patients. Due to their clinical infrequency, poor response to antimicrobial therapy and often precarious anatomical location, the optimal management of these lesions can be challenging. METHODS: Retrospective medical record review of 4 pediatric cases of mediastinal nontuberculous mycobacteria infection was undertaken. Each child presented with nonspecific respiratory symptoms, including significant acute airway obstruction and required a range of investigations to confirm the diagnosis. Nonresponsiveness to conservative measures and antimycobacterial therapy ultimately resulted in surgical intervention to obtain clinical improvement. RESULTS: All 4 children had extensive evaluation and multidisciplinary involvement in otolaryngology, respiratory medicine, pediatric surgery, infectious diseases and cardiothoracic surgery. They all eventually had their disease debulked via thoracotomy in addition to prolonged antimycobacterial therapy, with successful clinical outcomes. CONCLUSIONS: Mediastinal nontuberculous mycobacteria infections in the pediatric population are rare and diagnostically challenging. A high clinical suspicion should be maintained, and multidisciplinary input sought. Targeted surgery with adjuvant medical therapy can reduce disease burden with minimal long-term morbidity.

Congenital mesoblastic nephroma: review of current management and outcomes in a single centre.

BACKGROUND: Congenital mesoblastic nephroma (CMN) is a rare tumour of the kidney with an overall excellent prognosis. Once considered a benign tumour, it is now recognized to carry a risk of recurrence and metastases with subsequent poor outcomes. The potential for genetic aberrations such as ETV6-NTRK3 fusion raises the potential for targeted treatments in certain patients. The optimum mode and frequency of surveillance is unclear. This study aims to assess this institution's experience with CMN and long-term outcomes. METHODS: A single centre retrospective review was performed of all confirmed cases of CMN between October 2001 and January 2021. RESULTS: Nine cases of CMN in patients under 12 months of age were identified. The histopathology, management and outcomes of these patients are discussed. CONCLUSION: CMN overall has a very good prognosis, but a subgroup does exist that will have poor outcomes. It is difficult to accurately identify this group to target adjuvant therapy.

Massive, benign, cystic ovarian tumors: A technical modification for minimally invasive resection.

The majority of large, cystic ovarian tumors presenting in children are benign and amenable to ovarian sparing surgery (OSS). Laparoscopy is impractical in these cases and when attempted has been associated with a high rate of intraperitoneal fluid spill. We present a modified technique for controlled cyst decompression that allows delivery of the ovary through minilaparotomy and subsequent OSS. Criteria that must be met for the procedure to be undertaken are discussed.

Is fundoplication required after the Foker procedure for long gap esophageal atresia?

BACKGROUND: Fundoplication has been performed almost universally in children treated with the Foker procedure (FP) for long gap esophageal atresia (LGEA). We report our experience with pharmacological management and endoscopic surveillance rather than early routine fundoplication in infants treated with the FP. METHODS: A retrospective chart review was performed of all children treated with the Foker procedure at our institution. RESULTS: Nine children have undergone the FP since 2007. Median time between FP and definitive anastomoses was 22days. All nine children kept their native esophagus. There were three minor anastomotic leaks, all treated nonoperatively. All children required dilatation of anastomotic strictures (range 2-15). All have been treated with proton pump inhibitors. Three children had eosinophilic esophagitis and one had Barrett's esophagus. Only two children in this series have undergone fundoplication, which was performed for symptomatic and persistent erosive esophagitis. CONCLUSION: The question of early versus delayed fundoplication in LGEA patients managed with the FP remains unanswered. Our series demonstrates that it is possible to achieve good long-term outcomes when the operation is reserved for children with gastroesophageal reflux disease resistant to maximal medical therapy. LEVEL OF EVIDENCE: IV.

Outcomes of pulmonary metastases in hepatoblastoma--is the prognosis always poor?

BACKGROUND: Hepatoblastoma is a rare tumour accounting for approximately 1% of all paediatric malignancies. Hepatoblastoma complicated by pulmonary metastatic disease continues to cause management difficulties due to a lack of robust evidence and treatment guidelines. METHOD: This series is the experience of a tertiary paediatric referral centre. Patients were prospectively enlisted, and their charts were retrospectively reviewed. RESULTS: Thirty-seven patients were treated for hepatoblastoma from 1995 to 2012 inclusive. The overall survival was 34/37(91.9%). Eight patients had lung metastases at diagnosis (LMD) and twenty-nine did not (NLMD). Two-year EFS was 62.5% in the LMD group and 89.3% in the NLMD group (p=0.078). Overall survival in the LMD and NLMD groups was 100% and 89.7%, respectively (p=0.389). Two patients in the LMD group required multiple thoracic resections to achieve cure. Within the NLMD group, two patients developed lung metastases whilst on treatment, and both of these patients died. CONCLUSION: In this series, children presenting with lung metastases had a higher risk of relapse but excellent overall survival. However, children who developed pulmonary disease during treatment had a poor prognosis. We advocate aggressive surgical treatment of pulmonary hepatoblastoma to achieve cure.

Multiseptate gallbladder in an asymptomatic child.

A one-year-old child being investigated for urinary tract infection was diagnosed with a multiseptate gallbladder. The patient remains asymptomatic, and investigations demonstrate no associated anomalies. Forty-three cases, including 13 cases in children were identified in the literature. Their presentation and management were reviewed.