Department of Pediatric Surgery, Christian Medical College, Vellore, India.
Department of Pediatric Surgery, Christian Medical College, Vellore, India.
J Pediatr Urol. 2019 Aug;15(4):315-321. doi: 10.1016/j.jpurol.2019.06.019. Epub 2019 Jun 26.
Crossed fused renal ectopia is a rare congenital malformation, wherein both kidneys are present unilaterally, with the ureter of the crossed kidney opening into the bladder on the contralateral side. It has varied presentation from incidental detection to renal impairment. In this largest series of crossed fused renal ectopia, the authors assessed the clinical profile of these children and also attempted to shed light on the challenges in the surgical management.
It is a retrospective study conducted from January 2009 to July 2018, among patients evaluated for crossed fused ectopia, in the Department of Pediatric Surgery, Christian Medical College, Vellore. Electronic medical records of 36 children were reviewed. Imaging modalities and operative interventions were recorded. Results were analyzed.
A total of 36 children were found to have crossed fused ectopia, with male preponderance. Most children presented within one year of age with urinary tract infection being the commonest cause. The most common associated anomaly was anorectal malformation. Ultrasound abdomen and pelvis, micturating cystourethrogram, and radionucleotide scans were the imaging modalities preferred for diagnosis and follow-up. Left-to-right ectopia was more common with inferior ectopic variant being the most common. The predominant urological problems include pelviureteric junction obstruction, vesicoureteric junction obstruction, and vescioureteric reflux. Ureteric re-implant was the most common surgery performed followed by pyeloplasty. Pelvi-pelvostomy, uretero-ureterostomy, bladder augment with Mitrofanoff, and other procedures were performed for select cases.
Crossed fused renal ectopia is a challenging entity which requires individualized management plans based on the predominant urological anomaly and the functional status. Surgical options are diverse and are guided toward the symptomatic urological problem with focus on preserving the renal function. The long-term prognosis is good in these children.
交叉融合肾异位是一种罕见的先天性畸形，其中双侧肾脏单侧存在，交叉肾脏的输尿管开口于对侧膀胱。它的表现从偶然发现到肾功能损害不等。在这个最大的交叉融合肾异位系列中，作者评估了这些儿童的临床特征，并试图阐明手术管理中的挑战。
这是一项回顾性研究，于 2009 年 1 月至 2018 年 7 月在维洛尔基督教医学院小儿外科对交叉融合异位患者进行，共回顾了 36 名儿童的电子病历。记录了影像学检查和手术干预措施。对结果进行了分析。
共发现 36 名儿童存在交叉融合异位，男性居多。大多数儿童在一岁以内出现症状，尿路感染是最常见的病因。最常见的合并畸形是肛门直肠畸形。超声腹部和骨盆、排尿性膀胱尿道造影和放射性核素扫描是诊断和随访的首选影像学方法。左侧到右侧异位更为常见，下异位变体最为常见。主要的泌尿科问题包括肾盂输尿管连接部梗阻、膀胱输尿管连接部梗阻和膀胱输尿管反流。输尿管再植入是最常见的手术，其次是肾盂成形术。对于特定病例，还进行了肾盂肾盂吻合术、输尿管输尿管吻合术、膀胱增大术（伴 Mitrofanoff 技术）和其他手术。
交叉融合肾异位是一种具有挑战性的实体，需要根据主要的泌尿科异常和功能状态制定个体化的管理计划。手术选择多种多样，旨在针对有症状的泌尿科问题，同时注重保护肾功能。这些儿童的长期预后良好。
