Congenital aphallia is a rare anomaly with little supporting literature and controversial management. The aim of this review is to assess the most recent literature with a focus on staged management of these cases. We performed a PubMed search of all English literature in the past 10 years using the term aphallia. Twenty-three articles were identified of which six were excluded. A further three papers meeting our criteria were found in the references to papers initially identified. We found that management can be staged in three phases: short, intermediate and long-term. We conclude that optimal short-term management centers on resuscitation and urinary diversion as necessary, intermediate-term management entails urethrorectal fistula division, urethrostomy and neophallus creation and long-term management results in successful neophalloplasty, urethroplasty, prosthetic implant and continued protection of the upper urinary tracts with a Mitrofanoff. All this within a multidisciplinary team ensuring shared decision-making with the patient and their family.
Background: Optimal management of neuroblastoma depends on accurate risk stratification at diagnosis. Many low- and middle-income countries lack access to specific genetic tests used globally for this purpose.Aim: To determine whether socioeconomic factors predict prognosis in neuroblastoma and could therefore provide alternative measures for risk stratification in resource-constrained settings.Setting: The three main paediatric oncology units in Johannesburg, South Africa: Charlotte Maxeke Johannesburg Academic Hospital, Chris Hani Baragwanath Academic Hospital and Wits Donald Gordon Medical Centre.Methods: This retrospective record review included 145 patients presenting with biopsy-proven neuroblastoma between 01 January 2000 and 31 December 2018. Kaplan–Meier survival analysis was performed in relation to biological and socioeconomic factors, the latter including parental employment status, nationality, and distance of residence from treating facility. Cox proportional hazards regression analysis assessed the significance and effect of these prognostic factors.Results: Factors with significant effect on survival were age below 18 months (p 0.0001), extra-abdominal primary tumour site (p = 0.02), lower stage (p 0.001), serum ferritin level 0.0001) and favourable International Neuroblastoma Pathological Committee histology (p 0.0001), race (p = 0.005), nationality (p = 0.05) and paternal employment (p = 0.02). The association between distance from treating facility and stage at diagnosis was not significant (Tb = 0.108, p = 0.06).Conclusion: Biological factors exert a great influence on neuroblastoma survival than the socioeconomic factors analysed. This suggests that tumour biology exerts an overriding influence on prognosis in neuroblastoma.
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