The clinical management of congenital absence of the uterine cervix

Abstract

Eight women with cervical atresia were evaluated and treated for symptoms of pelvic pain. Two distinct anatomic variations of cervical atresia were observed among these patients. A fibrous cord, with islands of endocervical glands, was noted in two patients surgically treated with the creation of a cervicovaginal fistula through the fibrous cord. Alternatively, a complete absence of the cervical stroma required a uterovaginal anastomosis in two women. One of the two women with the cervicovaginal fistula continues to menstruate. Both women, after the anastomosis, required additional surgery to maintain menstrual flow and ultimately received hysterectomy. Subsequently, four women with complete congenital absence of the cervical stroma were treated primarily with the removal of the uterine fundus. Although menstrual function may be maintained in patients with some cervical stroma, no pregnancies have occurred. Thus, removal of the uterine fundus remains the treatment of choice, relieving symptoms and avoiding additional surgery to maintain uterovaginal patency.

Ultrasonographic diagnosis and assisted surgical management of hematotrachelos and hematometra due to uterine cervical atresia with associated vaginal agenesis.

“…In five of six patients with congenital cervical atresia (two of which had combined vaginal agenesis) described by Farber, 1 and in four patients reported by Rock et al 2 conservative surgical procedures similar, but not identical, to ours succeeded in creating an avenue for egress of the menstruum. In the current era of in-vitro fertilization, the reproductive potential of these cases, treated conservatively, is conceivably enhanced, hence the importance of correct and complete diagnosis prior to surgery.…”

Congenital absence of uterine cervix

“…10 Cervical atresia has been further classified into different types as follows 5,[11][12][13] (i) The cervical body is intact with obstruction of the cervical os (ii) The cervical body consists of a fibrous band (iii) Fragmented portions of the cervix are noted (iv) The midportion of the cervix is hypoplastic with a bulbous tip. Imaging modalities should assess the nature of cervical remnant precisely as the reconstructive surgery is based on the anatomy observed.…”

Partial Cervical Agenesis and Complete Vaginal Atresia

“…There have been three cases discussed in the literature of sepsis-related death secondary to cervical agenesis. In these cases, the patients were initially thought to have a high transverse vaginal septum and thus were treated by the creation of a neovagina and establishment of communication with the uterine cavity 3,4 . Similar to our two patients, they all initially had normal periods however all patients represented to hospitals with severe infections requiring hysterectomy secondary to infectious morbidity 3 .…”

“…In these cases, the patients were initially thought to have a high transverse vaginal septum and thus were treated by the creation of a neovagina and establishment of communication with the uterine cavity 3,4 . Similar to our two patients, they all initially had normal periods however all patients represented to hospitals with severe infections requiring hysterectomy secondary to infectious morbidity 3 . One such author reported that the patient continued to decline, developing multi-system organ failure and died days after a hysterectomy 4 .…”