Pop-Trajković Sonja, Ljubić Aleksandar, Antić Vladimir, Trenkić Milan
Klinicki centar Nis, Ginekolosko akuserska klinika, Nis, Srbija.
Vojnosanit Pregl. 2009 Sep;66(9):733-7. doi: 10.2298/vsp0909733p.
BACKGROUND/AIM: Multicystic dysplastic kidney represents a disorder in the fetus development presented prenatally of postnatally, this deserving special attention due to a risk of additional anomalies in children with this disorder. The aim of this study was to determine the incidence and type of common anomalies of the urinary system in the prenatal diagnosis of unilateral multicystic dysplastic kidney, and point out the necessity of postnatal diagnostic procedures in order to evaluate the state of the urinary system.
This retrospective-prospective study encompassed 38 cases of prenatally diagnosed unilateral fetal multicystic dysplastic kidney, presented to the Council for Fetal Anomalies from the Institute for Gynecology and Obstetrics of the Clinical Centre of Serbia and the University Children's Clinic within a three-year period. Associated anomalies were revealed by autopsy findings when pregnancy was terminated, ie resumed with clinical and operative findings of born children.
In every case of terminated pregnancy and death after birth the autopsy revealed additional renal or exstrarenal anomaly which were not prenataly detected. Postnatal evaluation of survived children with unilateral multicistic disease revealed that 31.4% of them have an anomaly of the contralateral kidney, 26.3% anomaly of the ipsilateral side, 13.2% anomaly of the lower portions of the urinary system and the same percent an additional extrarenal anomaly. The surgery was performed in 73.6% of children, more than half of the interventions were related to extrarenal anomaly. In 17% of children the kidney function was deteriorated after surgery.
Children suffering from unilateral multicystic dysplastic kidney have a greater chance of exhibiting an anomaly of the contralateral kidney and the urinary system in general. Therefore, they require thorough examination, both prenatally and postnatally. We propose obligatory serial professional prenatal ultrasound examinations, followed by postnatal ultrasound, isotope scan, and we especially emphasize the need for performing urinary cystouretherography, bearing in mind the high incidence of the vesicoureteral reflux of the contralateral kidney. In addition to nephrectomy, cytoscopy and colposcopy also need to be performed for the purpose of discovering possible hidden anomalies of the urogenital system.
背景/目的：多囊性发育不良肾是一种在胎儿发育过程中出现的疾病，可在产前或产后表现出来，由于患有这种疾病的儿童存在出现其他异常的风险，因此值得特别关注。本研究的目的是确定产前诊断单侧多囊性发育不良肾时泌尿系统常见异常的发生率和类型，并指出产后诊断程序对于评估泌尿系统状况的必要性。
这项回顾性前瞻性研究涵盖了38例产前诊断为单侧胎儿多囊性发育不良肾的病例，这些病例在三年期间内被提交至塞尔维亚临床中心妇产科研究所和大学儿童诊所的胎儿异常委员会。当妊娠终止时，通过尸检结果发现相关异常，对于出生的儿童，则通过临床和手术结果发现相关异常。
在每例终止妊娠和出生后死亡的病例中，尸检均发现了产前未检测到的额外肾脏或肾外异常。对存活的单侧多囊性疾病儿童进行产后评估发现，其中31.4%的儿童对侧肾存在异常，26.3%同侧存在异常，13.2%泌尿系统下部存在异常，另有13.2%存在额外的肾外异常。73.6%的儿童接受了手术，超过一半的手术与肾外异常有关。17%的儿童术后肾功能恶化。
患有单侧多囊性发育不良肾的儿童总体上更有可能出现对侧肾和泌尿系统异常。因此，他们在产前和产后都需要进行全面检查。我们建议进行强制性的系列专业产前超声检查，随后进行产后超声、同位素扫描，并且特别强调需要进行膀胱尿道造影，因为要考虑到对侧肾膀胱输尿管反流的高发生率。除了肾切除术外，还需要进行膀胱镜检查和阴道镜检查，以发现泌尿生殖系统可能存在的隐藏异常。
