Mattioli Girolamo, Pini-Prato Alessio, Costanzo Sara, Avanzini Stefano, Rossi Valentina, Basile Angela, Ghiggeri Gian Marco, Magnasco Alberto, Leggio Samuele, Rapuzzi Giovanni, Jasonni Vincenzo
Pediatric Surgery Department, Gaslini Research Institute and Children Hospital, University of Genova, Largo G. Gaslini, 16100, Genoa, Italy.
Pediatr Surg Int. 2010 May;26(5):523-8. doi: 10.1007/s00383-010-2592-7. Epub 2010 Mar 26.
Little is reported in literature regarding correct management of benign lesions of the kidney. The aim of our study is to present a series of total and partial nephrectomies performed in the last 5 years and to discuss indications.
Patients with benign lesions who underwent nephrectomy and partial nephrectomy at our institution in the period 2003-2008 were retrospectively included in the study. Notes were carefully reviewed and demographic data, symptoms onset, preoperative diagnosis, investigations, medical and/or surgical treatment, postoperative complications and definitive histological reports were collected.
Forty procedures were performed. Twelve patients were preoperatively diagnosed of having multicystic dysplastic kidney (MCDK), which was confirmed in 10, whereas the remaining 28 patients of having severe dysplasia or hypodysplasia. Thirty-four patients underwent total nephrectomy, six underwent partial nephrectomy. Histopathological analysis confirmed segmental or complete abnormalities of the involved kidney in all cases. No malignancies were detected.
Our study confirmed the extremely low malignancy rate of MCDK and hypodysplastic kidneys. The 20% mismatch of pre- and post-operative diagnosis suggests a common aetiology and shared therapeutic strategies for MCDK and hypodysplasia. At present, there is no consensus regarding correct indications for nephrectomy in paediatric age. As nephrectomy seems not to provide any advantage over preservation, but surgical and anesthesiological risks, we should be prudent in preserving every asymptomatic poorly or non-functioning kidneys maintaining a strict follow-up. Randomised controlled studies on larger multicentric series are strongly warranted to define this topic.
关于肾脏良性病变的正确管理，文献报道较少。我们研究的目的是呈现过去5年中进行的一系列全肾切除术和部分肾切除术，并讨论其适应证。
回顾性纳入2003年至2008年期间在我院接受肾切除术和部分肾切除术的良性病变患者。仔细查阅病历，收集人口统计学数据、症状出现时间、术前诊断、检查、内科和/或外科治疗、术后并发症及最终组织学报告。
共进行了40例手术。12例患者术前诊断为多囊性发育不良肾（MCDK），其中10例得到证实，其余28例为重度发育异常或发育不全。34例患者接受了全肾切除术，6例接受了部分肾切除术。组织病理学分析证实所有病例受累肾脏均有节段性或完全性异常。未检测到恶性肿瘤。
我们的研究证实了MCDK和发育不全肾脏的极低恶性率。术前和术后诊断20%的不匹配表明MCDK和发育不全有共同的病因和共同的治疗策略。目前，关于儿童肾切除术的正确适应证尚无共识。由于肾切除术似乎并不比保留肾脏有任何优势，反而存在手术和麻醉风险，因此我们在保留每一个无症状的功能不良或无功能肾脏时应谨慎，并进行严格的随访。强烈需要对更大的多中心系列进行随机对照研究来明确这一问题。
