摘要目的 通过文献和病例回顾及复习,对小儿肾外肾盏畸形的流行病学特征、临床表现和诊治要点进行探讨.方法 回顾性分析2014年6月收治的一例以脐周疼痛为代主诉入院的5岁女肾外肾盏畸形患儿的发病、诊断和治疗过程,并对相关文献进行复习分析.结果 本例患儿经手术探查切除发育畸形肾脏,术后患儿随诊未出现异常体征.同时,我们对7篇中文文献、13篇英文文献报道进行分析.其中,中文文献7篇共10例,男4例,女6例;年龄13～61岁.英文文献13篇,共29例,检索到全文7篇共8例,男5例,女3例;年龄2～52岁.此病一般无特征性临床表现,发病率低,故易漏诊误诊.结论 肾外肾盏畸形是非常罕见的上尿路集合系统畸形,一般无特征性临床表现.静脉尿路造影、螺旋CT尿路造影、MRI尿路造影及肾核素扫描是诊断和术前评估的常用方法.无临床症状的肾外肾盏畸形只需定期观察随访即可;出现临床症状时,手术是治疗的唯一方法,但术前需详细评估双肾功能状态后,再选择适合的个体化手术治疗方案,预后一般较好.

abstractsObjective To explore the epidemiology,clinical manifestations and diagnostic essentials of pediatric extrarenal calyces and conduct the relevant literature review.Methods The clinical manifestations,diagnosis and treatment of a 5-year-old girl complaining of para-navel pain due to extrarenal calyces were retrospectively analyzed.And the relevant literature review was conducted.Results Congenital kidney was removed without any abnormal postoperative sign.Therefore,7 Chinese papers (n =10,4 males and 6 females with an age range of 13-61 years) and 13 English papers (7 full articles,n =8,5 males and 3 females with an age range of 2-52 years) were reviewed.It was prone to misdiagnosis due to a lack of specific manifestations.Conclusions As an extremely rare anomaly of upper urinary tract,extrarenal calyces may be easily misdiagnosed due to a lack of specific manifestations.IVU (intravenous urography),CTU (computed tomography urography),MRU (magnetic resonance urography) and renal dynamic imaging are vital for diagnosis and preoperative evaluations.Regular follow-ups are necessary and adequate for asymptomatic patients.Surgery remains a sole effective treatment for this congenital anomaly when clinical symptoms are examined for associated disease.The prognosis is excellent when renal function is evaluated before surgery and appropriate individual surgery selected.