Alavi-Dunn Nicole, Waisanen Kyle M, Marrara Joseph A, Zawerton Ash, Monteiro Ajit, Saade Kiana, Young Ezekiel
Urology, University at Buffalo, Buffalo, USA.
School of Medicine, Trinity School of Medicine, Kingstown, VCT.
Cureus. 2023 Jun 2;15(6):e39880. doi: 10.7759/cureus.39880. eCollection 2023 Jun.
Posterior urethral valves (PUV) are an uncommon urologic congenital anomaly in males often discovered antenatally and more rarely after birth. PUV can lead to obstructive nephropathy and voiding dysfunction, putting patients at increased risk for irreversible renal damage and subsequent progression to end-stage renal disease. Much of the renal damage caused by PUV is proportional to the amount of time that the kidney has been experiencing retrograde pressure. Although much debate exists within the field, spontaneous decompression within the collecting system (e.g., "pop-off" valve) such as urinoma formation or spontaneous ascites has been found to relieve pressure on and thus protect the kidney, decreasing the risk of progression to advanced stages of chronic kidney disease. Despite the significant mass effect on the renal parenchyma, the pressure-relieving function of urinoma formation is a net protective factor allowing renal function to be preserved. We report a unique case of antenatal detection of PUV in a male with postnatal complicated urinoma formation secondary to forniceal rupture. Remarkably, despite significant external compression of the kidney and the development of urosepsis from infection of the urinoma with a multidrug-resistant organism that required percutaneous drainage, renal function was preserved throughout the disease course. After ablation of the PUV and drainage of the septic urinoma, the patient recovered rapidly after intervention and was ultimately discharged in stable condition.
后尿道瓣膜(PUV)是男性中一种罕见的泌尿系统先天性异常,常在产前发现,产后发现的情况更为罕见。PUV可导致梗阻性肾病和排尿功能障碍,使患者发生不可逆肾损伤及随后进展至终末期肾病的风险增加。PUV所致的许多肾损伤与肾脏承受逆行压力的时间长短成正比。尽管该领域存在诸多争议,但已发现集合系统内的自发减压(如“安全阀”),如尿瘤形成或自发性腹水,可减轻对肾脏的压力,从而保护肾脏,降低进展至慢性肾病晚期的风险。尽管尿瘤对肾实质有显著的占位效应,但其减压功能是一个净保护因素,可使肾功能得以保留。我们报告了一例独特病例,一名男性胎儿在产前被检测出患有PUV,出生后因穹窿破裂继发复杂尿瘤形成。值得注意的是,尽管肾脏受到明显的外部压迫,且尿瘤被多重耐药菌感染导致尿源性败血症,需要经皮引流,但在整个病程中肾功能得以保留。在切除PUV并引流感染性尿瘤后,患者在干预后迅速康复,最终病情稳定出院。