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

Abstract

Background: To explore the clinical characteristics of congenital cervical atresia.

Methods: This retrospective analysis included 32 cases of congenital cervical atresia treated from March 1984 to September 2010. The anatomic location, ultrasonic features, surgical treatments, and outcomes were recorded.

Results: Based on clinical characteristics observed during preoperative ultrasound and intraoperative exploration, congenital cervical atresia was divided into four types. Type I (n?=?22/32, 68.8%) is incomplete cervical atresia. Type II (n?=?5/32, 15.6%) defines a short and solid cervix with a round end; the structure lacked uterosacral and cardinal ligament attachments to the lower uterine body. Type III (n?=?2/32, 6.3%) is complete cervical atresia, in which the lowest region of the uterus exhibited a long and solid cervix. Type IV (n?=?3/32, 9.4%) defines the absence of a uterine isthmus, in which no internal os was detected, and a blind lumen was found under the uterus.

Conclusions: Observations of clinical characteristics of congenital cervical atresia based on the anatomy and ultrasound may inform diagnosis and treatment strategy.

Figure 1

Characteristics of congenital cervical atresia in preoperative ultrasonography. (A, B) Type I congenital cervical atresia, showing the unique shape of a Type 1 uterus and uterine isthmus. (C) Type II congenital cervical, atresia showing a hematocele in the uterine cavity and a thickened lower uterine cavity (blind end) and wall. (D) Type III congenital cervical atresia, showing a sinus-like cervix becoming narrower from the top to the bottom; the end of the cervix was significantly enlarged. (E) Type III congenital cervical atresia, showing a long and solid cervix, with no enlarged end. (F) Type IV congenital cervical atresia, showing thickening of the wall of the cervical canal and a blind end. The liquid anechoic area of the uterine cavity was bigger than that of the cervix.

Figure 2

Diagrammatic anatomical comparison between the traditional cervix classification for abnormal development and the proposed cervical atresia typing (coronal uterus view).

Figure 3

Type I cervical atresia before, during, and after tracheloplasty. (A) Ultrasonic image of Type I congenital cervical atresia. (B) After the hematoma was discharged, the uterine isthmus cysts became smaller, and the wall consisted of 5 to 6 mm of muscle tissue. (C) Fifteen days after the operation, the volume of the uterine isthmus measured 2.1 cm?×?1.5 cm. The arrow in the figure points to the vagina outside the cervix. (D) Long-term follow-up showed that the cervix resembled that of a normal nulliparous woman.

Figure 4

Sagittal section of the resected uterus of patients with Types II(A), III(B), and IV(C) cervical atresia. Blue circle indicates uterine cavity, red circle indicates uterine cervix.

Figure 5

Type I cervical atresia. (A) The black arrow indicates smooth muscle. The yellow arrow indicates infiltrated lymphocytes. The red arrow indicates hyperplastic vascular and collagen fiber tissue (HE?×?100). (B) The red arrow indicates endometrial glands. The black arrow indicates cervical mucosa (HE?×?100). (C) The red arrow indicates collagen fiber tissue. The black arrow indicates cervical mucosal epithelium (HE?×?40). (D) The red arrow indicates cervical mucosal epithelium (HE?×?400).

Figure 6

Type II cervical atresia. (A) Endometrium and glands on the surface of the uterine cavity (HE?×?40). (B) Disorderly and hypogenetic smooth muscle below the uterine cavity (HE?×?40). (C) Dysplastic smooth muscle (HE?×?100). (D) Muscular layer in the adenomyosis (HE?×?40).

Figure 7

Type III cervical atresia. (A) The red arrow indicates cysts in the cervix. The black arrow indicates a lesion of the endometrial glands (HE?×?40). (B) The red arrow indicates an old hemorrhage (HE?×?40). (C) The red arrow indicates nerve fibers. The black arrow indicates microvessels (HE?×?100). (D) The red arrow indicates endometrial glands (HE?×?100).

Figure 8

Type IV cervical atresia. (A) Cervical canal tissue. Red and blue arrows indicate cervical canal glands. The black arrow indicates a nerve fiber (HE?×?100). (B) The red arrow indicates regularly arranged well-developed smooth muscle tissue (HE?×?100). (C) Histologicol intemal os of the uterus. The yellow arrow indicates endometrial tissue. The red arrow indicates cervical canal tissue (HE?×?40). (D) The red arrow indicates endometrial glands (HE?×?400).

Figure 9

Vaginoplasty and tracheloplasty performed through a perineal approach in Type I cervical atresia patients. (A) Coronal plane of pelvic cavity in Type I patients. (B) The lower blind end of the uterus is opened and the hematocele is discharged. (C) The uterine isthmus wound muscular layer is retracted and the mucosal wound edge is correspondingly drooped. (D) The apical wound edge of the vaginal transplanted skin flap and the wound edge of the cervical muscular layer are stitched by interrupted suture. (E) The vaginal model is implanted. (The wound edge of the cervical mucosa was outward turned. The surface of the created cervical canal was smooth).