Congenital Absence of the Uterine Cervix
Congenital cervical atresia is a rare anomaly of the mullerian tract. It is characterized by failure of canalization of the cervical tissue and may be associated with both vaginal agenesis and uterine duplication abnormalities. An initial report in 1900 by Ludwig described this abnormality and an attempt at the creation of a uterine/vaginal fistula by the use of dilators. No follow-up was given in that report. In one of the earliest detailed descriptions, Napoleao in 1931 described congenital atresia of the cervix in a woman of 37 years. In this patient, recurrent abdominal pain had been present since age 15.1 The report described marked abdominal swelling with a normal vagina. At the time of surgery, partial cervical atresia was noted. In a second case in 1936, Mongaardin operated on a patient who had a partial atresia of the cervix. In this case a supracervical hysterectomy was performed. Isolated case reports continued to appear in the literature. In 1948, Maliphant used the expression gynatresia to denote the absence of a cervix in specific and any obstruction of congenital origin of menstrual flow in general.2 Early reports of attempts to form a fistula between the uterus and vagina with a variety of techniques were unsuccessful. Reports of significant postoperative morbidity and isolated cases of mortality led to the abandonment of organ-sparing reconstruction. The general recommendation in the literature through the 1960s and 1970s was a hysterectomy for congenital absence of the cervix.3
Added to this was a growing concern for infection. Several deaths from sepsis were reported further entrenching the opinion of the necessity for hysterectomy. These recommendations were also made at a time prior to effective assisted reproductive technologies such as in vitro fertilization, zygote intrafallopian transfer, and transmyometrial embryo transfer techniques. These technologies forced a re-evaluation of treatment options.