Two Cases of Herlyn-Werner-Wunderlich Syndrome
1
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Herlyn-Werner-Wunderlich (HWW) syndrome—also known as obstructed hemivagina and ipsilateral renal anomaly—is a rare congenital anomaly of Müllerian ducts and Wolffian ducts which is classically characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis; however, there are various combinations of uterine anomalies, obstruction sites, and renal anomalies that can occur as anatomic variants of HWW syndrome. Most patients show symptoms of recurrent lower abdominal pain or pelvic mass after the onset of menarche. Diagnosis can be delayed due to regular menstruation through non-obstructed hemivagina, which can lead to complications such as endometriosis, pyocolpos, pelvic inflammatory disease, and infertility. Here, we report two cases of females who presented dysmenorrhea and pelvic mass after the onset of puberty and were diagnosed with HWW syndrome through pelvic MRI. A review of the relevant literature was also provided.
Abstract
Herlyn-Werner-Wunderlich (HWW) syndrome—also known as obstructed hemivagina and ipsilateral renal anomaly—is a rare congenital anomaly of Müllerian ducts and Wolffian ducts which is classically characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis; however, there are various combinations of uterine anomalies, obstruction sites, and renal anomalies that can occur as anatomic variants of HWW syndrome. Most patients show symptoms of recurrent lower abdominal pain or pelvic mass after the onset of menarche. Diagnosis can be delayed due to regular menstruation through non-obstructed hemivagina, which can lead to complications such as endometriosis, pyocolpos, pelvic inflammatory disease, and infertility. Here, we report two cases of females who presented dysmenorrhea and pelvic mass after the onset of puberty and were diagnosed with HWW syndrome through pelvic MRI. A review of the relevant literature was also provided.
참고문헌 (10)
- Del Vescovo R, "Herlyn-Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis", BMC Med Imaging, 12, pp.4~ (2012)
- Orazi C, "Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases", Pediatr Radiol, 37, pp.657~665 (2007)
- Khaladkar SM, "The Herlyn-Werner-Wunderlich syndrome-a case report with radiological review", Pol J Radiol, 81, pp.395~400 (2016)
- Li X, "Herlyn-Werner-Wunderlich syndrome and its complications : a report of two cases and literature review", Radiol Case Rep, 16, pp.2319~2324 (2021)
- Jha S, "Diverse presentations of OHVIRA syndrome: a case series", Clin Pediatr (Phila), 60, pp.6~8 (2021)
- Lin TB, "Obstructed hemivagina and ipsilateral renal anomaly with uterus didelphys and vaginal discharge", Taiwan J Obstet Gynecol, 52, pp.593~596 (2013)
- Cox D, "Herlyn-Werner-Wunderlich syndrome : a rare presentation with pyocolpos", J Radiol Case Rep, 6, pp.9~15 (2012)
- Troiano RN, "Mullerian duct anomalies : imaging and clinical issues", Radiology, 233, pp.19~34 (2004)
- Zhang H, "Herlyn-Werner-Wunderlich syndrome : diverse presentations and diagnosis on MRI", Clin Radiol, 75, pp.480.e17~480.e25 (2020)
- 안자혜, "주산기에 Herlyn-Werner-Wunderlich 증후군으로 진단된 2례", Neonatal medicine, 20(1), pp.159~166 (2013), 대한신생아학회