Abstract
Background
Müllerian aplasia or Mayer–Rokitansky–Küster–Hauser syndrome is described as congenital
absence of the proximal vagina with or without absence of the cervix and uterus, most
often recognized after the onset of primary amenorrhea.
Case
An 18-year-old woman presented to a free medical clinic in Arcahaie, Haiti with primary
amenorrhea, abdominal distention, and cyclic monthly abdominal pain. Physical exam
was significant for uterus palpable superior to the umbilicus, absence of vagina,
and rectal exam without palpable vagina or cervix. Transabdominal and transperineal
ultrasound examinations did not reveal hematocolpos. Exploratory laparotomy revealed
severe endometriosis with bilateral hematosalpinx, markedly distended uterus, no proximal
vagina, and normal ovaries. Uterine specimen was filled with blood and no clear cervix
was present.
Summary and Conclusion
Diagnosis of vaginal and cervical agenesis is complicated in low-resource settings
and treatment must be modified when subspecialty care and consistent follow-up are
not available.
Key Words
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References
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Article info
Publication history
Published online: November 21, 2018
Footnotes
The authors indicate no conflicts of interest.
Identification
Copyright
© 2018 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc.
