Clinical characteristics of congenital cervical atresia based on anatomy and ultrasound: a retrospective study of 32 cases

This study was a retrospective analysis of patients who presented with congenital cervical atresia at the Fuyang Clinical College of Anhui Medical University and the Fifth People’s Hospital of Fuyang City between March 1984 and September 2010. Written consent was provided by all patients undergoing surgical treatment.

All patients had initial symptoms of cyclic abdominal pain during adolescence. The clinical diagnoses of congenital cervical atresia were based on symptoms and abdominal ultrasound before surgery. Diagnoses based on intraoperative exploration were identical to preoperative clinical diagnoses and ultrasound results.

Specimens collected by hysterectomy and biopsy were examined, and their characteristics were recorded, to highlight anatomical characteristics.

Patient follow-up was conducted for 6 months to 14 years post-operatively. Each follow-up examination assessed cervical presence and dimensions, vaginal dimensions, reproductive outcomes, sexual satisfaction, dysmenorrhea occurrence, and associated abnormalities due to scarring, infection, or other pathologies.

A total of 32 patients aged 12 to 21 years with congenital cervical atresia were included in this study. Based on preoperative ultrasound (Figure 1), medical examination, intraoperative exploration, and anatomical and pathological specimen examination, the cases were divided into four types (Figure 2). In each case, ultrasound revealed a liquid accumulation in the uterine cavity that was significantly larger than the cervix. A total of 22 cases (68.8%) were classified as Type I cervical atresia, in which a unique hematoma cyst cavity was detected at the lower portion of the uterus (Figure 1A,B; Figure 3A). Two patients had a normal vaginal recess but with an atresic uterine cervix and a malformed uterine body. In the other 20 cases, no vagina was apparent. Five cases were classified as Type II cervical atresia (5/32, 15.6%), in which a short and solid cervix with a round end was detected (Figure 1C); however, this structure lacked uterosacral and cardinal ligament attachments to the lower uterine body. Two cases were classified as Type III cervical atresia (2/32, 6.3%), in which the lowest region of the uterus exhibited a long and solid cervix (Figure 1D,E). Three cases were classified as Type IV cervical atresia (3/32, 9.4%), in which no internal os was detected, and a blind lumen was found under the uterus (Figure 1F, Figure 2). Sagittal sections of the resected uterus of patients with Types II, III, and IV cervical atresia are shown as Figure 4.

Of the 22 patients with Type I cervical atresia, two patients with a blind vaginal canal underwent laparotomy and transvaginal top cervical plastic surgery, while a further 18 patients underwent vaginal and cervical reconstructive surgery, one followed by exploratory laparotomy and one with hysterectomy (owing to an infection sustained after surgery outside our hospital).

In Type I cases, follow-up revealed no dysmenorrhea within 6 months after surgery. In the 20 cases of complete vaginal atresia, two demonstrated vaginal stenosis after removing the vaginal model 4 months after surgery. One patient experienced dysmenorrhea and discomfort during sexual activity 7 years after surgery. A vaginal stenosis ring of 1.5 cm length was detected at the upper vagina, and the collar showed atresia due to scar hyperplasia; therefore, restorative surgery was performed. In one case, the vagina was too shallow, and in 16 cases, the vaginal canal was 8 to 10 cm deep and two fingers wide. Seven of ten patients were married within 3 to 13 years of surgery, and all reported satisfaction with their sex lives. Among these, four patients (4/7, 57.1%) experienced a natural pregnancy, of whom three delivered by lower uterine segment Cesarean section, and one experienced abortion at 8 weeks’ gestational age. Two patients with top vaginal atresia suffered from uterine malformation (complete uterus bicornis and double uterus). The patient with two uteri had one atresic cervix and experienced a natural pregnancy in the other uterus.

Among the five patients with Type II cervical atresia, one patient underwent vaginal and cervical reconstruction via abdominal and perineal surgery. During follow-up, the patient reported that she was satisfied with her sex life. However, her cervix gradually shifted to the left rear of the pubic area, and she continued to experience dysmenorrhea. The other four patients received a hysterectomy and vaginal reconstructive surgery.

Among the patients with Type III cervical atresia, two patients underwent hysterectomy and vaginal reconstructive surgery.

Among the patients with Type IV cervical atresia, two of the three patients underwent vaginal and cervical reconstruction via abdominal and perineal surgery. The depth and width of the vaginal canals were normal, and dysmenorrhea did not occur. One patient was married 5 years after surgery and had a natural pregnancy; however, spontaneous abortion occurred at 10 weeks’ gestational age. One patient with uterus unicornis had a hysterectomy and vaginal reconstructive surgery.

We studied hysterectomy specimens of Type I (one case), Type II (four cases), Type III (two cases), and Type IV cervical atresia, and biopsy specimens taken from the wall of the hematoma cyst in Type I (six cases) cervical atresia.

In Type I cervical atresia, the smooth muscle tissues of the hematoma cyst wall are separated by proliferating blood vessels and collagen fibers, with a small amount of lymphocytic infiltration. The cyst cavity surface is coated with cervical mucosa and endometrium (Figure 5). In Type II cervical atresia, the surface of the cavity consists of uterine endometrium and glands, which covers a disorderly hypogenetic smooth muscle layer. In one case, the uterine cavity extended downward, showing a short tubule and visible cervical mucosa. A retention cyst with an old hemorrhage in the atresic cervical tissues was observed (Figure 6). In Type III cervical atresia, the atresic cervix is composed of fibro-muscular tissue, fibrous tissue, and collagenous fibers, with scattered nerve fibers, some smooth muscle bundle, and small blood vessels. In one case, a local retention cyst and a focus of adenomyosis were seen (Figure 7). In Type IV cervical atresia, the uterine endometrium is replaced by cervical mucosa at the junction of the uterine body and cervix. The lower wall of the blind end of the cervical canal is mainly composed of collagenous fibers, with scattered small blood vessels and smooth muscle bundles (Figure 8).