Currarino syndrome (CS) is a rare condition that presents with any combination of a sacral defect, a presacral mass, and an anorectal malformation. This collection, referred to as Currarino's triad, may not necessarily present as all three abnormalities in the diagnosis of the syndrome. Anal canal duplication (ACD) is an even rarer occurrence. We present a case that lies on the CS spectrum with an associated ACD and discuss a complex surgical challenge that necessitated a customized management plan, devised through a multidisciplinary approach.
PURPOSE Oncovascular surgery is a term identifying vascular resection in the context of tumour resection which is still controversial in children. We present our experience and review of the literature.
METHODS A retrospective review of children who underwent abdominal oncovascular procedures in our Institution from 2018 to 2022 was conducted. Type of operation and post-operative outcome was described. Literature review on oncovascular surgery in children is presented.
RESULTS Seven cases were identified, mean age 8.25 years (9 months- 14 years): 2 bilateral paraganglioma, 2 Wilms tumour, 1 Pancreatoblastoma, 1 Solid Pseudopapillary Tumour of the Pancreas (SPN), and 1 Hepatoblastoma. Five procedures were performed on the Inferior Vena Cava: 3 patients underwent cavectomy with no reconstruction, one had a partial cavectomy with primary repair, and one had resection and reconstruction with a PTFE graft which complicated with leaking and infection. Two patients underwent a pancreaticoduodenectomy with Portal Vein resection and primary anastomosis. Five patients were completely resected (R0), 2 patients had microscopically positive margins (R1). All patients were alive at last follow up.
CONCLUSION Vascular resection can allow complete tumour resection in locally advanced paediatric tumours. Oncovascular surgery in children is feasible and may be beneficial in selected cases.
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