Department of Obstetrics and Gynecology, Maternal-Fetal Medicine Unit, Van Education and Research Hospital, Van, Turkey.
Department of Urology, Pediatric Urology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey.
J Obstet Gynaecol. 2021 Oct;41(7):1071-1075. doi: 10.1080/01443615.2020.1845631. Epub 2021 Jan 16.
We reviewed the records of 144 patients. The mean gestational age at first US diagnosis was 27.5 ± 4.3 weeks. An anomaly of the contralateral kidney was detected in 25% of cases. An extrarenal anomaly was detected in 13.8%. Karyotype analysis was performed in 16.6% of cases and revealed trisomy 18 in 2 cases with extrarenal defects. Karyotype analysis was normal in all the patients who had isolated multicystic dysplastic kidney (MCDK). The diagnostic accuracy of prenatal ultrasound was 92.2%. Contralateral kidney anomaly was detected 33.9% of patients, and half of these were vesicoureteral reflux. Antihypertensive therapy was required in 2.6% of cases. Nephrectomy was performed in 8%, and partial or total involution of MCDK was achieved in 33.9% of patients. MCDK can be accurately diagnosed by prenatal sonography, and prognosis depends on extrarenal and contralateral renal abnormalities. In isolated cases, require of surgery is rare, and serial follow-up is suggested to determine involution.Impact statement Multicystic dysplastic kidney (MCDK) is one of the most renal anomalies and is associated with numerous renal and extrarenal abnormalities. It can lead to severe consequences in the neonatal period. The accuracy of prenatal ultrasonography is excellent for detecting MCDK. In isolated unilateral cases, chromosomal aberrations are low, and the majority of them involute spontaneously. A periodic follow-up of the contralateral kidney is mandatory due to an increased risk of an anomaly. Genital anomaly risk is increased in males. Detailed evaluation and follow-up of the contralateral kidney are crucial for counselling in isolated cases. Karyotype analysis in isolated unilateral MCDK is debateable. Postnatal prognosis is encountering, and the majority of patients have no requirement of surgery.
我们回顾了 144 例患者的记录。首次 US 诊断的平均胎龄为 27.5±4.3 周。在 25%的病例中检测到对侧肾脏异常。在 13.8%的病例中检测到肾外异常。对 16.6%的病例进行了核型分析，发现 2 例有肾外缺陷的病例存在 18 三体。所有孤立性多囊性发育不良肾（MCDK）患者的核型分析均正常。产前超声的诊断准确性为 92.2%。对侧肾脏异常在 33.9%的患者中被发现，其中一半为输尿管反流。2.6%的病例需要抗高血压治疗。8%的患者进行了肾切除术，33.9%的患者实现了 MCDK 的部分或完全退化。产前超声可以准确诊断 MCDK，预后取决于肾外和对侧肾脏异常。在孤立性病例中，手术需求很少，建议进行连续随访以确定退化。
影响陈述 MCDK 是最常见的肾脏异常之一，与许多肾脏和肾外异常相关。它可能导致新生儿期的严重后果。产前超声对 MCDK 的检测准确性很高。在孤立性单侧病例中，染色体异常率较低，其中大多数可自发退化。由于对侧肾脏异常的风险增加，必须定期随访对侧肾脏。男性的生殖器异常风险增加。详细评估和随访对侧肾脏对于孤立性病例的咨询至关重要。孤立性单侧 MCDK 的核型分析存在争议。产后预后良好，大多数患者无需手术。
