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近端输尿管闭锁,一种罕见的先天性异常——偶然发现:一例病例报告

Proximal ureteral atresia, a rare congenital anomaly-incidental finding: a case report.

作者信息

Bleve Cosimo, Conighi Maria Luisa, Fasoli Lorella, Bucci Valeria, Battaglino Francesco, Chiarenza Salvatore Fabio

机构信息

Department of Pediatric Surgery and Pediatric Minimally Invasive Surgery and New Technologies, San Bortolo Hospital, Vicenza, Italy.

出版信息

Transl Pediatr. 2017 Jan;6(1):67-71. doi: 10.21037/tp.2017.01.02.

Abstract

Ureteral atresia is a rare disease usually associated with a non-functioning dysplastic kidney. The condition may be unilateral or bilateral; focal, short or long and may involve any part of the ureter. Association with other urinary anomalies is rare. We report the case of a 10-month-old boy with prenatal diagnosis of multicystic right kidney. This suspicion was confirmed after birth by ultrasound and static scintigraphy; a right vesicoureteral reflux (VUR) was recorded at cystography. The boy presented a regular renal function but was hospitalized twice for suspected pyelonephritis between the 8th and the 10th month of life and were recorded occasional mild changes in blood pressure. Antibiotic prophylaxis was administered until surgery. When he was 10-month underwent retroperitoneoscopy to perform a nephroureterectomy finding a complete atresia of the upper third of the ureter with the blind end at the level of the uretero-pelvic-junction. The programmed surgery was performed. By a revision of literature, only few cases of imperforate distal ureter have been described. This condition is associated with a kidney dysplasia. The atresia of the ureter with no signs of infection in the dysplastic kidney may be unknown up to adulthood or throughout one's life. Prognosis usually depends on the severity of the obstruction.

摘要

输尿管闭锁是一种罕见疾病,通常与无功能的发育不良肾脏相关。该病症可能是单侧或双侧的;可为局灶性、短段或长段,可累及输尿管的任何部位。与其他泌尿系统异常同时存在的情况罕见。我们报告一例10个月大男童,产前诊断为多囊性右肾。出生后经超声和静态闪烁扫描证实了这一怀疑;膀胱造影显示存在右侧膀胱输尿管反流(VUR)。该男童肾功能正常,但在出生后第8至10个月期间因疑似肾盂肾炎住院两次,且记录到血压偶尔有轻微变化。在手术前一直给予抗生素预防治疗。10个月大时,他接受了后腹腔镜检查以进行肾输尿管切除术,发现输尿管上三分之一完全闭锁,盲端位于输尿管肾盂连接处水平。按计划进行了手术。通过文献复习,仅描述了少数远端输尿管闭锁的病例。这种情况与肾发育不良有关。发育不良肾脏中输尿管闭锁且无感染迹象的情况在成年前或一生中可能都不为人所知。预后通常取决于梗阻的严重程度。

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本文引用的文献

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Congenital intestinal atresia; observations on its origin.先天性肠闭锁;关于其起源的观察
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