Rare Müllerian Anomaly: Complete Septate Uterus with Simultaneous Longitudinal and Transverse Vaginal Septa

Abstract Background We present a patient with primary amenorrhea and a rare combination of anomalies. She was found to have a septate uterus, double cervix, and a longitudinal and a low transverse vaginal septum. Case An 18-year-old girl with primary amenorrhea presented with severe monthly pelvic p...

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Bibliographic Details
Published in:Journal of pediatric & adolescent gynecology 2015-12, Vol.28 (6), p.e189-e191
Main Authors: Louden, Erica D., MD, PhD, Awonuga, Awoniyi O., MD, Gago, Laura A., MD, Singh, Manvinder, MD
Format: Article
Language:English
Subjects:
Adolescent
Amenorrhea - etiology
Female
Humans
Magnetic Resonance Imaging
Mullerian anomalies
Mullerian Ducts - abnormalities
Mullerian Ducts - surgery
Obstetrics and Gynecology
Pediatrics
Pelvic pain
Pelvic Pain - etiology
Pimary amenorrhea
Pregnancy
Septate uterus
Urogenital Abnormalities - diagnosis
Urogenital Abnormalities - surgery
Uterus - abnormalities
Uterus - surgery
Vagina - abnormalities
Vagina - surgery
Vaginal septum
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Summary:Abstract Background We present a patient with primary amenorrhea and a rare combination of anomalies. She was found to have a septate uterus, double cervix, and a longitudinal and a low transverse vaginal septum. Case An 18-year-old girl with primary amenorrhea presented with severe monthly pelvic pain. Examination and imaging revealed a thin transverse vaginal septum, complete septate uterus, double cervix, and a longitudinal vaginal septum. The transverse and longitudinal vaginal septa were excised and repaired. Summary and Conclusion Although repetitive pregnancy loss and preterm birth are associated with various Müllerian duct anomalies, clinicians should also be suspicious of the presented anomaly in cases of primary amenorrhea and cyclic pelvic pain. To our knowledge, this is the only case of simultaneous septate uterus with longitudinal and transverse vaginal septum and the second case of combined longitudinal and transverse septum, which caused primary amenorrhea. This rare anomaly further supports the bidirectional regression theory of Müllerian development.
ISSN:1083-3188
1873-4332
DOI:10.1016/j.jpag.2015.04.012