Cambio Angelo J, Evans Christopher P, Kurzrock Eric A
Department of Urology, University of California, Davis School of Medicine, Sacramento, CA 95817, USA.
BJU Int. 2008 Apr;101(7):804-8. doi: 10.1111/j.1464-410X.2007.07328.x. Epub 2008 Jan 8.
To better define the outcome and association of multicystic dysplastic kidney (MCDK) with hypertension, vesico-ureteric reflux (VUR), infection and cancer, as there is no consensus on the management of patients born with MCDK. The risk of cancer has dictated the surgical management of the disease in the past.
The Medline database was searched for articles published between 1965 and 2006 and written in the English language, and containing the keywords 'multicystic dysplastic kidney'.
The inclusion criteria were met by 105 reports that were subsequently analysed. Of MCDK, 60% regress or involute within 3 years. About 25% of patients will have VUR into the contralateral kidney, of which 90% is grade <or=3. The risk of urinary tract infection appears to be associated with VUR or coexistent abnormalities rather than the MCDK. The risk of hypertension is no greater than that in the general population and nephrectomy is usually not curative. The overall risk of Wilms' tumour developing in a MCDK is <1 in 2000. All reported Wilms' tumours were identified before 4 years of age and 70% presented as a palpable mass.
Published reports support the non-surgical management of MCDK. Common practice has been to remove palpable or growing MCDKs, although these represent a very small fraction of MCDKs. In theory, ultrasonographic surveillance until 4 years old might allow the earlier detection of a Wilms' tumour, and decrease the intensity of chemotherapy and improve prognosis. Previous reports do not prove or disprove this concept, and the appropriate frequency of surveillance is not evident.
由于对于患有多囊性发育不良肾（MCDK）的患儿的治疗尚无共识，故旨在更好地明确MCDK与高血压、膀胱输尿管反流（VUR）、感染及癌症之间的结局及关联。过去，癌症风险决定了该疾病的手术治疗方式。
检索Medline数据库中1965年至2006年间发表的英文文章，关键词为“多囊性发育不良肾”。
105篇报告符合纳入标准并随后进行了分析。MCDK中，60%在3年内会消退或 involute。约25%的患者会出现VUR反流至对侧肾脏，其中90%为≤3级。尿路感染风险似乎与VUR或并存异常有关，而非MCDK本身。高血压风险并不高于普通人群，肾切除术通常无法治愈。MCDK发生Wilms瘤的总体风险低于2000分之一。所有报告的Wilms瘤均在4岁前被发现，70%表现为可触及的肿块。
已发表的报告支持对MCDK采取非手术治疗。常见做法是切除可触及或增大的MCDK，尽管这些仅占MCDK的极小部分。理论上，超声监测至4岁可能有助于更早发现Wilms瘤，并降低化疗强度及改善预后。既往报告未证实或证伪这一概念，且监测的合适频率尚不明确。
