Eckoldt F, Woderich R, Wolke S, Heling K S, Stöver B, Tennstedt C
Klinik und Poliklinik for Kinderchirurgie, der Medizinischen Fakultät (Charité) der Humboldt--Universität zu Berlin, Germany.
J Matern Fetal Neonatal Med. 2003 Sep;14(3):177-86. doi: 10.1080/jmf.14.3.177.186.
Since the introduction of antenatal diagnostic screening, multicystic kidney dysplasia (MCKD) has been diagnosed in 1 of 4300 live births. We analyze our own experience and demonstrate a management regime based on these results and existing studies.
Retrospective data analysis was carried out in 110 patients, prenatally diagnosed with MCDK. A total of 93 patients with confirmed diagnosis of unilateral MCDK were born alive and followed up in our institutions.
A total of 110 unilateral cystic kidneys were prenatally diagnosed; 93 were confirmed postnatally. These children were retrospectively allocated to two treatment groups: 51 were operated upon; 42 were treated conservatively. A micturition cystourethrogram was performed 88 times, yielding 20 pathological findings. Vesicoureteral reflux was identified 11 times (12.5%). Eleven children (12%) had associated non-urological abnormalities. Cardiac and musculoskeletal malformations predominated. After 1995, nephrectomy was performed only when clinically indicated. Thirty-seven children were treated conservatively for between 4 months and 6 years (mean 33 months); 28 dysplastic kidneys exhibited size reduction. Complete involution was noted in 16 of 28 children between 7 and 29 months (mean 16.2 months).
This study provides evidence that neonatal nephrectomy of unilateral MCDK is rarely required. It also shows that the discussed malformation is not an isolated developmental abnormality. In a high proportion, associated urogenital abnormalities were present and therapeutically relevant, and determined the overall prognosis.
自从引入产前诊断筛查以来,多囊性肾发育不良(MCKD)在4300例活产婴儿中被诊断出1例。我们分析了自己的经验,并根据这些结果和现有研究展示了一种管理方案。
对110例产前诊断为MCDK的患者进行回顾性数据分析。共有93例确诊为单侧MCDK的患者存活并在我们的机构中接受随访。
共有110个单侧肾囊肿在产前被诊断出;93例在出生后得到确诊。这些儿童被回顾性地分为两个治疗组:51例接受了手术;42例接受了保守治疗。进行了88次排尿性膀胱尿道造影,发现20例病理结果。发现膀胱输尿管反流11次(12.5%)。11名儿童(12%)伴有非泌尿系统异常。以心脏和肌肉骨骼畸形为主。1995年以后,仅在临床指征明确时才进行肾切除术。37名儿童接受了4个月至6年的保守治疗(平均33个月);28个发育不良的肾脏体积缩小。28名儿童中有16名在7至29个月(平均16.2个月)之间完全消退。
本研究提供的证据表明,单侧MCDK很少需要进行新生儿肾切除术。它还表明,所讨论的畸形不是一种孤立的发育异常。在很大比例的病例中,存在相关的泌尿生殖系统异常且具有治疗相关性,并决定了总体预后。