Laparoscopic management of a catecholamine-secreting paraganglioma in a 15-year-old boy
Epublication WebSurg.com, May 2016;16(05). URL: http://websurg.com/doi/vd01en4740
We report the case of the surgical removal of a paravesical paraganglioma located on the right vesicoureteric junction in a 15-year-old boy who was screened positive for SDHB gene mutation, which his father suffers from. Indeed, his dad died of a metastatic paraganglioma, notably including bone and cerebral metastases. The patient has been complaining of major headaches for some time, which were triggered off during urination to the point that he held in urine as long as possible during daytime in order to avoid urinating, and this seemed to be immediately related to the onset of severe headaches. The tumor location allowed to correctly understand the phenomenon intraoperatively since every bladder mobilization would induce abrupt bouts of high blood pressure. Resting blood pressure did not evidence any particular anomaly. However, there were high fluctuations in blood pressure, notably when headaches were reported. The tumor was located exactly at the vesicoureteric junction. It necessitated the resection of the lower ureter and the placement of a vesical patch. Complete surgical resection allowed to eliminate all symptoms. The patient’s clinical and biological work-up is strictly normal more than one year after the intervention. The resection was performed without paying attention to the potential difficulties related to the type of reconstructive surgery which entailed. Reconstruction of the vesicoureteric junction was achieved without any major problem on a slightly reduced bladder. A minimal vesicoureteric reimplantation was performed according to the Lich-Gregoir technique, with bladder closure onto a vesical drain and a vesicoureteric double J catheter. Immediate outcomes were uneventful and control performed more than one year postoperatively testified to the absence of tumor recurrence and to the very good functioning of the vesicoureteric junction.