Distal vaginal agenesis (DVA) is a rare form of female genital tract malformation that presents as cryptomenorrhea. It results from the failure of the urogenital sinus to form the caudal portion of the vagina. Through a thorough history, physical examination and appropriate imaging studies, an accurate diagnosis is integral in selecting the correct intervention for the patient. This is a case of distal vaginal agenesis in a 10-year-old nulligravid, who presented with fecal retention from an abdominopelvic mass. The patient had no bowel movement for four days, and abdominal enlargement. On inspection, there was a 12.0cm x 10.0cm palpable abdominal mass. Inspection of the external genitalia, the introitus appeared concave, with no appreciable introital opening. On digital rectal examination, an anterior bulge was palpated 0.5 cm from the anal verge. A pull-through vaginoplasty was performed with an unremarkable post-operative course. The patient was discharged with a patent vagina and resolution of her gastrointestinal symptoms. On follow-up, the patient had monthly menstruation after surgery with no recurrence of her gastrointestinal symptoms.

Human ; Female ; Child: 6-12 Yrs Old ; Congenital Abnormalities ; Digital Rectal Examination ; Defecation

In Mayer-Rokitansky-K0ster-Hauser (MRKH) syndrome, the development of the uterus and some parts of the vagina is either completely absent or reduced. It is a rare congenital anomaly, and affects one in 4,000-5,000 female births and commonly presents as primary amenorrhea. Approximately 6% - 10% of these patients with MRKH syndrome report persistent pelvic pain, which may be attributed to the presence of myomas, endometriosis, adenomyosis or hematometra caused by a functioning endometrial tissue in a uterine remnant. This paper presents the case of a 37 year old nulligravid who experienced severe cyclic hypogastric pain, and was subsequently diagnosed with MRKH syndrome with adenomyosis. Clinical evaluation and definitive management of the index case are discussed.
Human ; Female ; Adult: 25-44 yrs old ; mullerian failure ; mullerian aplasia ; adenomyosis ; pelvin pain