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梗阻性半阴道并同侧肾缺如（OHVIRA）综合征：一种罕见综合征的典型表现。

Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) syndrome: Typical presentation of a rare syndrome.

作者信息

Devanabanda Bhavana, Chatterton Bradley, Hassan Irfan Nazir, Patel Jay

机构信息

Department of Radiology, Cooperman Barnabas Medical Center, Rutgers RWJ Barnabas Health, 94 Old Short Hills Road, Livingston, NJ 07039, USA.

出版信息

Radiol Case Rep. 2023 May 11;18(7):2437-2440. doi: 10.1016/j.radcr.2023.04.016. eCollection 2023 Jul.

DOI:10.1016/j.radcr.2023.04.016
PMID:37235085
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10206379/
Abstract

Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) syndrome is a rare congenital malformation of the Mullerian duct. We report on a 34-year-old female who presented to the emergency department for cramping lower abdominal pain and pelvic pressure with vaginal spotting. Physical exam showed marked swelling in the right adnexa, and laboratory exams were within normal limits except for positive Coronavirus disease 2019 (COVID-19) status. Transvaginal ultrasound revealed 3 well circumscribed, round hypoechoic complex cystic lesions with arterial doppler detected within the peripheral walls. Magnetic resonance imaging of the abdomen and pelvis showed a right hemivagina, right hematosalpinx, right hematometra and right renal agenesis, compatible with OHVIRA syndrome. The patient was informed of elective surgical procedure but was unable to undergo surgery at this time secondary to COVID status. The patient was therefore recommended oral contraceptive therapy for suppression of menses and protection of endometrial lining.

摘要

梗阻性半阴道并同侧肾缺如（OHVIRA）综合征是一种罕见的苗勒管先天性畸形。我们报告一名34岁女性，因下腹部绞痛、盆腔压迫感伴阴道点滴出血就诊于急诊科。体格检查显示右附件区明显肿胀，实验室检查除2019冠状病毒病（COVID-19）呈阳性外均在正常范围内。经阴道超声显示3个边界清晰的圆形低回声复合囊性病变，外周壁可检测到动脉多普勒信号。腹部和盆腔磁共振成像显示右侧半阴道、右侧输卵管积血、右侧子宫积血和右侧肾缺如，符合OHVIRA综合征。患者被告知择期手术，但由于COVID状态，此时无法进行手术。因此，建议患者口服避孕药以抑制月经并保护子宫内膜。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/f74b0351816a/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/1c9edf5b45ee/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/c39f59290d33/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/fb8faa0a2395/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/32d424b71f4c/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/f74b0351816a/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/1c9edf5b45ee/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/c39f59290d33/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/fb8faa0a2395/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/32d424b71f4c/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8698/10206379/f74b0351816a/gr5.jpg

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Prz Menopauzalny. 2021 Apr;20(1):52-56. doi: 10.5114/pm.2021.104034. Epub 2021 Mar 1.
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Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report.罕见型赫尔林-韦纳-武德连综合征中成功的早产妊娠：病例报告。
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Clinical Implications of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in the Prepubertal Age Group.青春期前年龄组梗阻性半阴道并同侧肾异常（OHVIRA）综合征的临床意义
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Herlyn-Werner-Wunderlich syndrome: a case report.赫林-韦纳-温德利希综合征：一例报告。
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