Congenital scoliosis associated with agenesis of the uterine cervix. Case report

BMC Women's Health
Giorgio CarlomagnoMatteo Della Monica

Abstract

BACKGROUND: Alterations in the normal sequence of development of müllerian ducts lead to a wide spectrum of reproductive tract abnormalities. A rare form of lack of development, regarding a short tract of the müllerian ducts, leads to the isolated agenesis of the uterine cervix. Anomalies identified among patients with müllerian agenesis include skeletal deformities (i.e., scoliosis of the spine and Klippel-Feil anomaly). CASE PRESENTATION: A 46 years old woman presenting cyphoscoliosis and very low stature (120 cm - 3,93 feet), came to our observation for acute pelvic pain; she also reported primary amenorrhoea associated with cyclic pelvic pain. Clinical and imaging evaluation, evidenced a blind vaginal duct of normal length, left cystic adnexal mass, and enlarged uterus with hematometra. FSH, LH, 17beta estradiol and CA-125, karyotype and radiographic study of limbs and vertebral column were also evaluated.At laparotomy, a left ovarian cyst was found. Uterus ended at the isthmus; under this level a thin fibrous tissue band was found, joining the uterus to the vagina. Uterine cervix was replaced by fibrous tissue containing some dilated glands lined with müllerian epithelium. Karyotype resulted 46, XX. The described skeletal ...Continue Reading

Citations

Aug 16, 2014·The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians·Roberto RomeroYeon Mee Kim
Jun 21, 2014·Gynecological Endocrinology : the Official Journal of the International Society of Gynecological Endocrinology·David S Bombard, Shaker A Mousa

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BETA
hysterectomy
dissection
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