Congenital absence of uterine cervix
Keywords:Cervical agenesis, Dysgenesis, Congenital anomaly, Primary amenorrhea, Infertility, Ultrasonography
AbstractCervical agenesis or dysgenesis is an extremely rare congenital anomaly. Patients with congenital absence of the cervix present with primary amenorrhea and infertility. Though it poses a diagnostic challenge to the clinician, correct diagnosis prior to surgery is possible with the help of ultrasound. Early diagnosis offers significant advantages in patient care and effective presurgical planning. This case report reviews two cases of cervical agenesis diagnosed with the help of ultrasound and later confirmed with the help of MRI. Ultrasonography is the modality of choice to define the internal genital anatomy and helps us to classify the level of obstruction or aplasia in obstructive uterine anomalies.
Suganuma N, Furuhashi M, Moriwaki T, Tsukahara S, Ando T, Ishihara Y. Management of missed abortion in a patient with congenital cervical atresia. Fertil Steril. 2002;77:1071-3.
Markham SM, Parmley TH, Murphy AA, Huggins GR, Rock JA. Cervical agenesis combined with vaginal agenesis diagnosed by magnetic resonance imaging. Fertility and sterility. 1987;48(1):143-5.
Grimbizis GF, Tsalikis T, Mikos T, Papadopoulos N, Tarlatzis BC, Bontis J. Successful end to end cervicocervical anastomosis in a patient with congenital cervical fragmentation: case report. Hum Reprod. 2004;5:1204-10.
Valdes C, Malini S, Malinak LR. Sonography in the surgical management of vaginal and cervical atresia. Fertility and sterility. 1983;40(2):263-5.
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;16:1722-5.
Olive DL, Henderson DY. Endometriosis and mullerian anomalies. Obstetrics Gynecology. 1987;69(3):412-5.
Blask AR, Sanders RC, Gearhart JP. Obstructed uterovaginal anomalies: demonstration with sonography. Part I. Neonates and infants. Radiology. 1991;179(1):79-83.
Sherer DM, Beyth Y. Ultrasonographic diagnosis and assisted surgical management of hematotrachelos and hematometra due to uterine cervical atresia with associated vaginal agenesis. Journal of ultrasound in medicine: official journal of the American Institute of Ultrasound in Medicine. 1989;8(6):321.
Woelfer B, Salim R, Banerjee S, Elson J, Regan L, Jurkovic D. Repro-ductive outcomes in women with congenital uterine anomalies detectedby three-dimensional ultrasound screening. Obstet Gynecol. 2001;98:1099-103.
The American Fertility Society. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, Mullerian anomalies and intrauterine adhesions. Fertil Steril. 1988;49:944-55.
Buttram VC, Gibbons WE: Mullerian anomalies: a proposed classification (an analysis of 144 cases). Fertil Steril. 1979;32:40-6.
Xie Z, Zhang X, Liu J, Zhang N, Xiao H, Liu Y. Clinical characteristics of congenital cervical atresia based on anatomy and ultrasound: a retrospective study of 32 cases. Europ jour of medi rese. 2014;19(1):1.
Rock JA, Schlaff WD, Zacur HA, Jones HW Jr. The clinical management of congenital absence of the uterine cervix. Int J Gynaecol Obstet. 1984;22:231-5.
Vallerie AM, Breech LL. Update in Müllerian anomalies: diagnosis, management, and outcomes. Current Opinion in Obstetrics and Gynecology. 2010;22(5):381-7.
Acién P, Acién M, Ferrer MS. Complex malformations of the female genital tract. New types and revision of classification. Hum Reprod. 2004;19:2377-84.
Farber M, Marchant DJ. Congenital absence of the uterine cervix. American journal of obstetrics and gynecology. 1975;121(3):414-7.
Creighton SM, Davies MC, Cutner A. Laparoscopic management of cervical agenesis. Fertil Steril. 2006;85:1510.e13-5.