Ali S, Nunez V, Terkawi R, Katsoufis C, Abitbol C, Ruano R, Duara J, Fontanez-Nieves T
Division of Neonatology, University of Miami Miller School of Medicine, and Holtz Children's Hospital, Miami, FL, United States.
Division of Pediatric Nephrology, University of Miami Miller School of Medicine, and Holtz Children's Hospital, Miami, FL, United States.
Front Pediatr. 2025 May 8;13:1546561. doi: 10.3389/fped.2025.1546561. eCollection 2025.
Megalourethra is a rare congenital condition marked by dilation and elongation of the penile urethra, resulting from hypoplasia of either the corpus spongiosum or corpus cavernosa.
We describe a novel case of a male infant prenatally diagnosed with megalourethra who subsequently developed rapid-onset anhydramnios and advanced renal failure.
Unlike previously described cases, this case is unique due to the patient's abrupt progression to anhydramnios at 34 weeks and 5 days, despite having normal amniotic fluid levels prior to that. With late-onset anhydramnios, the severity of renal dysfunction was unexpected. Proximal urethrostomy requires further assessment as a potential intervention to successfully bypass the megalourethra and prevent infection. Long-term management is expected to include dialysis as a bridge to transplantation. Multiple corrective urological surgeries will be required to repair the urethra and restore penile function. Timely prenatal diagnosis of megalourethra or renal anomalies is essential for predicting long-term prognosis, as these conditions can lead to significant postnatal complications.
巨尿道是一种罕见的先天性疾病，其特征为阴茎尿道扩张和延长，由海绵体或海绵体发育不全引起。
我们描述了一例产前诊断为巨尿道的男婴的新病例，该男婴随后迅速出现羊水过少和晚期肾衰竭。
与先前报道的病例不同，该病例具有独特性，因为尽管在此之前羊水水平正常，但患者在34周零5天时突然进展为羊水过少。对于晚期羊水过少，肾功能障碍的严重程度出乎意料。近端尿道造口术作为一种潜在的干预措施，需要进一步评估，以成功绕过巨尿道并预防感染。长期管理预计包括透析作为移植的过渡。需要多次矫正性泌尿外科手术来修复尿道并恢复阴茎功能。巨尿道或肾脏异常的产前及时诊断对于预测长期预后至关重要，因为这些情况可能导致严重的产后并发症。
