Corrales J G, Elder J S
Department of Urology, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA.
J Urol. 1996 Apr;155(4):1398-401.
We characterize segmental multicystic dysplasia, in which there is a duplex collecting system with multicystic changes in the upper pole.
Three neonates with an abdominal mass and/or prenatal diagnosis of a multicystic kidney were evaluated. Postnatal ultrasound showed a classic multicystic kidney and small ipsilateral orthotopic ureterocele in all cases. All patients had ipsilateral grade V lower pole reflux and a renal scan showed functioning lower pole systems.
One patient underwent bilateral ureteroneocystostomy and another underwent upper pole nephrectomy. There has been reduction in the size of the segmental multicystic kidney in 2 patients and decrease in reflux grade in 2 (followup 2 to 5 years).
Newborns with an apparent multicystic kidney should undergo a voiding cystourethrogram and renal scan to confirm the diagnosis. Segmental multicystic kidney can be followed nonoperatively in most cases because the cysts tend to involute.
我们对节段性多囊性发育不良进行特征描述，其表现为双收集系统且上极存在多囊性改变。
对3例有腹部肿块和/或产前诊断为多囊肾的新生儿进行评估。产后超声显示所有病例均为典型的多囊肾及同侧小的原位输尿管囊肿。所有患者同侧下极均有V级反流，肾扫描显示下极系统有功能。
1例患者接受了双侧输尿管膀胱吻合术，另1例接受了上极肾切除术。2例患者节段性多囊肾体积缩小，2例反流程度减轻（随访2至5年）。
明显患有多囊肾的新生儿应进行排尿性膀胱尿道造影和肾扫描以确诊。大多数情况下节段性多囊肾可进行非手术随访，因为囊肿往往会自行消退。
