Congenital utero-cervical and vaginal atresia: a rare case report
DOI:
https://doi.org/10.18203/2320-1770.ijrcog20222338Keywords:
Cervical atresia, MURCS syndrome, Singapore flapAbstract
A genesis of cervix is a very rare developmental anomaly. It may be associated with vaginal agenesis. The usual age of presentation is teenage, with symptoms like cyclical abdominal pain and failure to attain menarche. Earlier, hysterectomy used to be the only option for such girls. Recently reconstructive surgery is gaining popularity. This is a case report presenting an eighteen-year-old girl with classical symptoms, diagnosed with both cervical and vaginal atresia, and managed by reconstructive surgery (Singapore flap method). It was a single step surgery done by abdomino-perineal approach. The girl attained menses and has been symptom free at one year follow-up. The postoperative complications were minor and managed conservatively. A patient of cervical atresia should be given the option of a reconstructive procedure even in the presence of vaginal agenesis. Utero-cervical anastomosis has shown good results in several studies. However, the reproductive potential has to be assessed by long term follow up.
References
Fujimoto VY, Miller JH, Klein NA, Soules MR. Congenital cervical atresia: report of seven cases and review of the literature. Am J Obstet Gynecol. 1997;177(6):1419-25.
Grimbizis GF, Tsalikis T, Mikos T, Papadopoulos N, Tarlatzis BC, Bontis JN. Successful end-to-end cervico-cervical anastomosis in a patient with congenital cervical fragmentation: case report. Hum Reprod. 2004;19(5):1204-10.
Rock JA, Roberts CP, Jones HW Jr. Congenital anomalies of the uterine cervix: lessons from 30 cases managed clinically by a common protocol. Fertil Steril. 2010;94(5):1858-63.
4 Combined Cervical and Vaginal Reconstruction With Prefabricated Pudendal Thigh Flap in a Case With Cervical and Vaginal Agenesis (MURCS Syndrome): A New and Original Technique. Ann Plastic Surg. 2008;61(1):88-93.
Wee JT, Joseph VT. A new technique of vaginal reconstruction using neurovascular pudendal-thigh flaps: a preliminary report. Plast Reconstr Surg. 1989;83(4):701-9.
Kriplani A, Kachhawa G, Awasthi D, Kulshrestha V. Laparoscopic-assisted uterovaginal anastomosis in congenital atresia of uterine cervix: follow-up study. J Minim Invasive Gynecol. 2012;19(4):477-84.
Fedele L, Bianchi S, Frontino G, Berlanda N, Montefusco S, Borruto F. Laparoscopically assisted uterovestibular anastomosis in patients with uterine cervix atresia and vaginal aplasia. Fertil Steril. 2008;89(1):212-6.
Deffarges JV, Haddad B, Musset R, Paniel BJ. Utero-vaginal anastomosis in women with uterine cervix atresia: long-term follow-up and reproductive performance. A study of 18 cases. Hum Reprod. 2001;16(8):1722-5.
Fein LA, Salgado CJ, Pearson JM. Singapore Flap (Pudendal Thigh Fasciocutaneous Flap) for Vaginal Reconstruction. In: Anh Tran T, Panthaki Z, Hoballah J, Thaller S. (eds) Operative Dictations in Plastic and Reconstructive Surgery. Springer, Cham. 2017.
