Schlomer Bruce, Rodriguez Esequiel, Baskin Laurence
University of Texas Southwestern and Children's Medical Center, Dallas, TX, USA.
Children's Hospital of Central California, Madera, CA, USA.
J Pediatr Urol. 2015 Apr;11(2):77.e1-6. doi: 10.1016/j.jpurol.2014.12.004. Epub 2015 Feb 26.
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare syndrome characterized by mullerian and renal anomalies. Renal agenesis is classically considered to be part of the definition of OHVIRA syndrome. There are increasing reports that the lack of an ipsilateral kidney on imaging studies is not from true renal agenesis but renal dysplasia and atrophy. We have observed cases where this ipsilateral dysplastic and atrophic kidney leads to clinical symptoms. The objective of this study is to report cases of OHVIRA syndrome where an ipsilateral dysplastic and atrophic kidney led to clinical symptoms, and to raise awareness in the pediatric urology community of this variant of OHVIRA syndrome.
All cases of OHVIRA syndrome with an ipsilateral dysplastic and atrophic kidney were identified from January 2010 to June 2013. The patient's presentation, clinical course, surgical management, radiologic findings, and pathologic finding were reviewed.
There were three cases of OHVIRA syndrome with a symptomatic ipsilateral dysplastic and atrophic kidney identified. All three of these dysplastic and atrophic kidneys had an ectopic ureter to the obstructed hemivagina and led to persistent vaginal drainage after resection of the vaginal septum. These dysplastic and atrophic kidneys were not visualized on any imaging studies. Laparoscopic removal of the dysplastic and atrophic kidney led to cessation of vaginal drainage in all cases.
This study reports additional cases to the literature that do not fit the classic definition of OHVIRA syndrome. In the cases in this study, there was no ipsilateral renal agenesis, but an ipsilateral dysplastic and atrophic kidney with an ectopic ureter to the obstructed hemivagina. In addition, these cases demonstrate that the dysplastic and atrophic kidney may cause symptoms such as persistent vaginal drainage after resection of vaginal septum. The dysplastic and atrophic kidneys may not be detected by any imaging modality including MRI and DMSA scan and may be ectopic in location. All three of our cases had persistent vaginal drainage after resection of the vaginal septum which was cured by removal of the dysplastic and atrophic kidney ipsilateral to the obstructed hemivagina. The limitations of this study include the small number of patients and retrospective nature. While the classic definition of OHVIRA syndrome includes ipsilateral renal agenesis, there is increasing evidence that the absence of a kidney on imaging is due to renal dysplasia and atrophy and not true renal agenesis. In addition, we have observed that this dysplastic and atrophic kidney may cause clinically significant issues such as persistent vaginal drainage through an ectopic ureter to the ipsilateral hemivagina after resection of vaginal septum. This small dysplastic and atrophic kidney may not be visualized on imaging studies and laparoscopy can be diagnostic and therapeutic if indicated. Ipsilateral renal anomalies, not ipsilateral renal agenesis, should be considered part of the definition of OHVIRA syndrome. Pediatric urologists need to be aware of the spectrum of renal anomalies in OHVIRA syndrome.
梗阻性半阴道并同侧肾缺如（OHVIRA）综合征是一种罕见的综合征，其特征为苗勒管和肾脏异常。传统上认为肾缺如是OHVIRA综合征定义的一部分。越来越多的报道称，影像学检查中同侧肾脏缺失并非真正的肾缺如，而是肾发育不良和萎缩。我们观察到一些病例，其中同侧发育不良和萎缩的肾脏导致了临床症状。本研究的目的是报告OHVIRA综合征病例，其中同侧发育不良和萎缩的肾脏导致了临床症状，并提高小儿泌尿外科界对这种OHVIRA综合征变体的认识。
从2010年1月至2013年6月确定所有伴有同侧发育不良和萎缩肾脏的OHVIRA综合征病例。回顾了患者的临床表现、临床病程、手术治疗、影像学检查结果和病理检查结果。
确定了3例伴有有症状的同侧发育不良和萎缩肾脏的OHVIRA综合征病例。所有这3个发育不良和萎缩的肾脏都有一条异位输尿管通向梗阻性半阴道，并在切除阴道隔后导致持续性阴道引流。这些发育不良和萎缩的肾脏在任何影像学检查中均未显影。腹腔镜切除发育不良和萎缩的肾脏后，所有病例的阴道引流均停止。
本研究向文献中报告了不符合OHVIRA综合征经典定义的其他病例。在本研究的病例中，不存在同侧肾缺如，而是有一个同侧发育不良和萎缩的肾脏，伴有一条通向梗阻性半阴道的异位输尿管。此外，这些病例表明，发育不良和萎缩的肾脏可能导致诸如切除阴道隔后持续性阴道引流等症状。发育不良和萎缩的肾脏可能无法通过包括MRI和二巯基丁二酸（DMSA）扫描在内的任何影像学检查发现，并且可能位于异位。我们所有3例病例在切除阴道隔后均有持续性阴道引流，通过切除与梗阻性半阴道同侧的发育不良和萎缩的肾脏得以治愈。本研究的局限性包括患者数量少和回顾性研究性质。虽然OHVIRA综合征的经典定义包括同侧肾缺如，但越来越多的证据表明，影像学检查中肾脏缺失是由于肾发育不良和萎缩，而非真正的肾缺如。此外，我们观察到这种发育不良和萎缩的肾脏可能导致临床上的重要问题，如切除阴道隔后通过通向同侧半阴道的异位输尿管出现持续性阴道引流。这个小的发育不良和萎缩的肾脏可能在影像学检查中未显影，并且如果有指征，腹腔镜检查可具有诊断和治疗作用。同侧肾脏异常，而非同侧肾缺如，应被视为OHVIRA综合征定义的一部分。小儿泌尿外科医生需要了解OHVIRA综合征中肾脏异常的范围。
