小儿肾外肾盏一例分析及文献复习

Extrarenal calyces in children： a case report and literature review

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。

Objective 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 - yearold girl complaining of paranavel 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.