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盘状肾,一种罕见且常被误诊的畸形:一例病例报告及影像学鉴别诊断

Pancake kidney, a rare and often misdiagnosed malformation: a case report and radiological differential diagnosis.

作者信息

Lomoro P, Simonetti I, Vinci G, Fichera V, Prevedoni Gorone M S

机构信息

Department of Diagnostic Medicine, Institute of Radiology IRCCS San Matteo University Hospital Foundation, Pavia, Italy.

Department of Advanced Biomedical Sciences, University of Naples Federico II, Naples, Italy.

出版信息

J Ultrasound. 2019 Jun;22(2):207-213. doi: 10.1007/s40477-018-0331-4. Epub 2018 Oct 25.

Abstract

Renal ectopia and fusion anomalies are Congenital Anomalies of the Kidney and the Urinary Tract (CAKUT) that are usually incidentally detected and asymptomatic. Patients affected present a higher risk of complications like recurrent urinary tract infections or obstruction. Pancake kidney (PK) is one of the rarest types of renal anomaly with complete fusion of the superior, mild and inferior poles of both kidneys in the pelvic cavity. Each kidney has its own excretory system with two ureters that do not cross the midline. In the asymptomatic cases, a conservative approach should be performed. Surgical management may be needed when urological problems occur. PK is often associated with congenital anomalies of other organs. Ultrasound is the first line radiological examination for the diagnosis and the follow-up of kidney malformations. The main sonographic findings suggesting PK diagnosis are a large and lobulated renal mass consisting of two fused lateral lobes without an intervening septum located in the pelvic cavity. Each lobe usually has a separate pelvicalyceal system, the renal pelvis is anteriorly placed and the ureters are usually short and enter the bladder normally without crosses the midline. Ultrasonography gives useful information on the morphology and volume of the organ, and on its vascularization through the use of the Color- and Power-Doppler. Computer Tomography and Magnetic Resonance Urography are second level techniques used to confirm the diagnosis and to evaluate the presence of other abnormalities. The knowledge of the imaging findings and the anatomy of congenital renal malformations is important to avoid diagnostic pitfalls and misinterpretations. We report the case of a 14-years old female with PK who was misdiagnosed with a horseshoe kidney (HSK) during an abdominal ultrasound.

摘要

肾异位和融合异常是先天性肾脏和尿路异常(CAKUT),通常是偶然发现且无症状的。受影响的患者出现复发性尿路感染或梗阻等并发症的风险较高。饼状肾(PK)是最罕见的肾异常类型之一,双肾的上极、中极和下极在盆腔内完全融合。每个肾脏都有自己的排泄系统,有两条输尿管且不越过中线。在无症状的情况下,应采取保守治疗方法。当出现泌尿系统问题时可能需要手术治疗。PK常与其他器官的先天性异常相关。超声是诊断和随访肾脏畸形的一线影像学检查。提示PK诊断的主要超声表现是盆腔内有一个由两个融合的外侧叶组成的大的分叶状肾肿块,中间无间隔。每个叶通常有一个独立的肾盂肾盏系统,肾盂位于前方,输尿管通常较短且正常进入膀胱而不越过中线。超声通过彩色多普勒和能量多普勒的应用,可提供有关器官形态、体积及其血管化的有用信息。计算机断层扫描和磁共振尿路造影是用于确诊和评估其他异常情况的二级技术。了解先天性肾畸形的影像学表现和解剖结构对于避免诊断失误和误解很重要。我们报告了一例14岁女性PK患者,其在腹部超声检查时被误诊为马蹄肾(HSK)。

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本文引用的文献

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Pancake kidney: when it is not a problem.饼状肾:何时并非问题所在
BJR Case Rep. 2018 Feb 16;4(3):20170117. doi: 10.1259/bjrcr.20170117. eCollection 2018 Mar.
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The pancake kidney.盘状肾。
Abdom Radiol (NY). 2019 Jan;44(1):381-382. doi: 10.1007/s00261-018-1706-x.
3
Crossed fused renal ectopia: case report and review of the literature.交叉融合型肾异位:病例报告及文献复习
J Ultrasound. 2017 Apr 4;20(4):333-337. doi: 10.1007/s40477-017-0245-6. eCollection 2017 Dec.
5
Pancake Kidney Found Inside Abdominal Cavity: Rare Case with Literature Review.腹腔内发现融合肾:罕见病例并文献复习
Urol Case Rep. 2017 May 5;13:123-125. doi: 10.1016/j.eucr.2016.11.020. eCollection 2017 Jul.
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Congenital Renal Fusion and Ectopia in the Trauma Patient.创伤患者中的先天性肾融合与异位
Case Rep Emerg Med. 2016;2016:5203872. doi: 10.1155/2016/5203872. Epub 2016 Nov 8.
7
Pancake kidney with cysts and a single ureter.伴有囊肿和单一输尿管的盘状肾。
Radiol Bras. 2016 Mar-Apr;49(2):127-8. doi: 10.1590/0100-3984.2015.0063.

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