Rhabdomyosarcomas (RMS) of the kidney is extremely rare. We are reporting one case of embrional rhabdomyosarcoma localized in a child renal pelvis, focusing on the rarity of the site and role of imaging. An 8-years-old boy was admitted to the hospital with abdominal pain and hematuria. Ultrasonography revealed a right ureteropyelectasy including an echogenic image that extended from the renal pelvis towards inferior basin with no hypervascularization, simulating a clot. The urography-magnetic resonance imaging (Uro-MRI) showed two different components of the lesion: the first one simulating a clot; the second one detected in the papillary region and extended to the inferior calices having tumor-like features. A percutaneous ultrasound-guided renal biopsy revealed a botryoid embryonal rhabdomyosarcoma. The RMS is a complex childhood malignancy. This complexity is reflected in the radiological assessment that remains highly challenging and in the absense of treatment guidelines for unusual sites.
Objective This report documents the authors' experiences in the management of “complex” jejunoileal atresia (JIA) and provides a review of the recent literature on “simple” and “complex” JIA.
Materials and Methods This is a retrospective study of eight cases of “complex” JIA managed at the Pediatric Surgical Unit of Infermi Hospital in Rimini from 2002 to 2012. The inclusion criteria are all cases of JIA associated with distal bowel deformities and Types IIIb or IV. One patient had gastroschisis.
Results The authors of this study performed primary anastomosis on three patients and enterostomies on five patients. In one case in which a patient presented with gastroschisis, the V.A.C. Therapy System (KCI Medical Ltd., Langford Locks, Kidlington, UK) was used to close the abdominal defect. All patients needed central venous catheter (CVC). Total parenteral nutrition (TPN) was administered for a mean of 12 days. Oral feeding was introduced on mean day 7 (7.71 ± 3.40 standard deviation). Patients with enterostomy began extracorporeal stool transport on mean day 14. No outcomes resulted in short bowel syndrome (SBS). The mortality rate was zero. The authors of this study performed more enterostomies and CVC insertion than other authors in “complex” JIA and reported a percentage of SBS, complications of TPN, and start of oral feeding comparable to “simple” case reported by other authors.
Conclusions The results demonstrate that the complexity of JIA alone is not associated to a worsening prognosis than simple atresia if the surgical and clinical approach is as conservative as possible.
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