Kuwertz-Broeking E, Brinkmann O A, Von Lengerke H J, Sciuk J, Fruend S, Bulla M, Harms E, Hertle L
Department of Paediatric Nephrology, University Children's Hospital, Westphalian-Wilhelms University of Münster, Germany.
BJU Int. 2004 Feb;93(3):388-92. doi: 10.1111/j.1464-410x.2003.04623.x.
To report a retrospective study of unilateral multicystic dysplastic kidneys (MCDK) in children, assessing the contralateral kidneys and urinary tract, the functional consequences, and the urological and nephrological management and outcome, as unilateral MCDK is the most common cause of renal cystic disease in children, and malformations of the contralateral urinary tract and kidney (pelvi-ureteric obstruction, megaureter, reflux, renal dysplasia) have been reported.
The study included 97 patients (60 boys, 37 girls) with MCDK seen between 1985 and 1998; 82 were diagnosed in utero by ultrasonography (US). After birth, the diagnosis was verified by US, renal scanning (in 93) or intravenous urography (in four), and 89 (92%) had voiding cysto-urethrography (VCUG). Of the 97 children, 87 (90% had a mean (range) follow-up of 44.3 (15-115) months.
The MCDK was removed in 17 children; the follow-up of 75 children (five lost to follow-up) showed total involution of the MCDK in 25%, shrinkage in 60% and a stable size in 15%. None had any sign of malignancy. The contralateral kidney showed anomalies in 19 of 97 children (20%); 12 had a dilated renal pelvis (two with megaureter), six had a high echogenicity of the contralateral kidney (one had reflux, and two also pelvic dilatation). In only four of the 89 children was reflux found by VCUG; 16 of the 19 anomalies were detected by US. Five children needed surgery on the contralateral urinary tract (three a pyeloplasty, and one each a pyeloplasty plus ureteroneocystostomy, and an antireflux procedure). Of the contralateral kidneys 43% showed compensatory hypertrophy. There was mild renal insufficiency in three children; renal function seemed to be slightly impaired in many. Five infants had hypertension (four with spontaneous resolution) caused by renal scarring after pyelonephritis or inborn dysplasia of the contralateral kidney. There were symptomatic urinary tract infections in seven children.
US can be used safely to diagnose unilateral MCDKs and malformations of the contralateral urinary tract and kidney. In cases where US of the dysplastic kidney remains uncertain renal scintigraphy is necessary to detect the lack of renal function. The low rate of reflux makes routine VCUG unnecessary if the contralateral upper urinary tract and kidney appear to be normal on US. Nephrectomy of the dysplastic kidney in typical cases is also unnecessary. A long-term nephro-urological follow-up of children with MCDK is recommended.
报告一项关于儿童单侧多囊性发育不良肾（MCDK）的回顾性研究，评估对侧肾脏和尿路情况、功能后果以及泌尿外科和肾脏病学的管理与结局。因为单侧MCDK是儿童肾囊性疾病最常见的病因，且已有报道称对侧尿路和肾脏存在畸形（肾盂输尿管梗阻、巨输尿管、反流、肾发育不良）。
本研究纳入了1985年至1998年间诊治的97例MCDK患者（60例男孩，37例女孩）；其中82例在子宫内通过超声（US）诊断。出生后，通过US、肾扫描（93例）或静脉肾盂造影（4例）进行诊断核实，89例（92%）进行了排尿性膀胱尿道造影（VCUG）。97例儿童中，87例（90%）平均随访时间为44.3（15 - 115）个月。
17例儿童切除了MCDK；75例儿童（5例失访）的随访显示，MCDK完全消退的占25%，缩小的占60%，大小稳定的占15%。均无恶性肿瘤迹象。97例儿童中有19例（20%）对侧肾脏存在异常；12例肾盂扩张（2例合并巨输尿管），6例对侧肾脏回声增强（1例有反流，2例同时伴有肾盂扩张）。89例儿童中仅4例通过VCUG发现反流；19例异常中有16例通过US检测到。5例儿童需要对侧尿路手术（3例肾盂成形术，1例肾盂成形术加输尿管膀胱再植术，1例抗反流手术）。对侧肾脏43%显示有代偿性肥大。3例儿童有轻度肾功能不全；许多儿童肾功能似乎有轻微受损。5例婴儿因肾盂肾炎后肾瘢痕形成或对侧肾脏先天性发育不良而患高血压（4例自行缓解）。7例儿童有症状性尿路感染。
US可安全用于诊断单侧MCDK及对侧尿路和肾脏的畸形。在发育不良肾脏的超声检查结果不明确时，需要进行肾闪烁显像以检测肾功能缺失情况。如果对侧上尿路和肾脏在US检查中看起来正常，反流发生率低使得常规VCUG不必要。典型病例中发育不良肾脏的肾切除术也不必要。建议对MCDK儿童进行长期的肾脏 - 泌尿外科随访。
